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rare tumors ISSN 2036-3605 ı eISSN 2036-3613 ı www.pagepress.org/rt Editor-in-Chief: Robert C. Miller, USA Volume 1, 2009

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RARE TUMORS ISSN 2036-3605 - eISSN 2036-3613

Editor-in-Chief Robert C. Miller, Rochester, MN, USA

Editorial Staff Nicole Pezzolo, Managing Editor Cristiana Poggi, Production Editor Anne Freckleton, Copy Editor Jeanette Mitchell, Copy Editor Filippo Lossani, Technical Support

Editorial Board Prakash Adhikari, Department of ENT and Head and Neck Surgery, GMS Memorial Academy of ENT and Head and Neck Studies, TU Teaching Hospital, Kathmandu, Nepal Rafael Álvarez-González, Graduate School of Biomedical Sciences, University of North Texas, Healt Science Center at Forth Worth, Forth Worth, Texas, USA Armando Bartolazzi, Cellular and Molecular Tumor Pathology Laboratory, Cancer Center Karolinska, Karolinska Hospital, Stockholm, Sweden Archit Bhatt, Department of Neurology and Ophtalmology, Clinical Center East Lansing, East Lansing, Michigan, USA Kevin Camphausen, Radiation Oncology Branch, National Cancer Institute, Bethesda, Maryland, USA Joseph R. Carver, Abramson Cancer Center, University of Pennsylvania, Pennsylvania, USA William Cho, Department of Clinical Oncology, Queen Elizabeth Hospital, Hong Kong Domenico Coppola, Anatomic Pathology and Neuroendocrine Cancer Research Divisions, Moffit Cancer Center and Research Institute, Tampa, Florida, USA Undurti N. Fams Das, UND Life Sciences, Shaker Heights, Ohio, USA Ozsahin E. Mahmut, Department of Radiooncology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland Jimmy Thomas Efird, Centre of Health Vulnerable Populations, University of Carolina, Greensboro, Carolina, USA Amany Elwakkad, National Research Center, Cairo, Egypt Gulgun Engin, Department of Radiology, Faculty of Medicine, Istanbul University, Istanbul, Turkey Guy Eslick, Harvard School of Public Health, MA, USA Patricia D. Evilliers, Department of Anatomic Pathology, University of Alabama at Birmingham, Birmingham, USA Luis E. Fayad, Department of Lymphoma & Myeloma, University of Texas MD Anderson Cancer Center, Houston, Texas, USA Faris Farassati, Department of Medicine, University of Kansas School of Medicine, Kansas City, Kansas, USA Ali Gholamrezanezhad, Research Institute for Nuclear Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran Samy Lewiz Habib, Department of Medicine, Division of Nephrology, University of Texas Health Science Center, San Antonio, Texas, USA Paul H. Hartel, Broaddus Hospital of Davis Health System, Elkins, West Virginia, USA Ghulam Sarwar Hashmi, Department of Oral and Maxillofacial Surgery, Z.A. Dental College, Medical Colony, A.M.U. Aligarh, India Joseph Herman, Department of Oncology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins School of Medicine, Baltimore Maryland USA Kanya Honoki, Department of Orthopedic Surgery, Nara Medical University, Nara, Japan Charles Hsu, Division of Plastic and Reconstructive Surgery, Stanford University Medical Center, Palo Alto, CA, USA Sergio Huerta, Dallas VA Medical Center Surgical Services, Dallas, Texas, USA Abdul Hussain, Minimal Access Unit, General Surgery Department, Princess Royal University Hospital, London, UK Rafael Jimenez, Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA Bleddyn Jones, Gray Institute for Radiation Oncology and Biology, University of Oxford, Oxford, UK Honoki Kanya, Department of Orthopedic Surgery, Nara Medical University, Nara, Japan Babak Kateb, Intraoperative Surgical Planning Society, Los Angeles, CA, USA Sunali Khanna, Department of Oral Medicine & Radiology, Nair Hospital Dental College, Mumbai, India Boris Kirshtein, Department of Surgery, Soroka University Medical Center, Beer

Sheva, Israel Antoniades Konstantinos, School of Dentistry, Aristotle University of Thessaloniki, Thessaloniki, Greece Masafumi Koshiyama, Department of Ob/Gy at Otsu Red Cross Hospital, Otsu, Japan Sunil Krishnan, Radiation Oncology, Gastrointestinal Translational Research, M.D. Anderson Cancer Center, Houston, TX, USA Calin Lazar, Department of Plastic and Reconstructive Surgery, Rouen University Hospital, Rouen, France Wei Li, Institute of Biomedical Engineering, School of Control Science and Engineering, Shandong University, Shandong, China Simon Lo, Department of Radiation Medicine, The Ohio State University Arthur G. James Cancer Hospital, Columbus, OH, USA Lorenzo Lo Muzio, Surgical Sciences Department, Faculty of Medicine, University of Foggia, Foggia, Italy Paulette M. Fauceglia, Roswell Park Cancer Institute, Buffalo, New York, USA Sridhar Mani, Departments of Medicine and Molecular Genetics, Division of Oncology, Albert Einstein School of Medicine, New York, USA Hiroyuki Matsubayashi, Shizuoka Cancer Center, Shizuoka-ken, Japan Toshihiro Matsuo, Department of Artificial Joints and Biomaterial, Hiroshima University, Hiroshima, Japan Axel Merseburger, Department of Urology, Eberhard-Karls-University, Tübingen, Germany Oliver Micke, Department of Radiotherapy and Radiation Oncology, Franziskus Hospital, Bielefeld, Germany Rene Mirimanoff, Service de Radio-Oncologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland Luca Morelli, Operative Unit of Patological Anatomy and Cytological Diagnostics, P.O. S. Chiara, Hospital of Trento, Trento, Italy Charbel D. Moussallem, Orthpedic Surgery, Faculty of Medical Sciences, Lebanese University, Beirut, Lebanon Hidenari Nagai, Division of Gastroenterology and Hepatology, Toho University Medical Center, Omori Hospital, Tokyo, Japan Tan Dat Ngyuen, Department of Radiation Oncology, Institut Jean-Godinot, Reims, France Athanasios Papatsoris, University of Athens, Athens, Greece Nicholas A. Pavlidis, Department of Medical Oncology, School of Medicine, University of Ioannina, Ioannina, Greece Yi Chu Pei, Department of Surgical Pathology, Changhua Christian Hospital, Changhua, Taiwan Shahid Pervez, Department of Pathology and Microbiology, Agakhan University Hospital, Karachi, Pakistan Camillo Porta, IRCCS San Matteo University Hospital Foundation, Pavia, Italy Fernando Quevedo, Division of Medical Oncology, Mayo Clinic, Rochester, MN, USA Malcolm Schinstine, Division of Pathology, Hilo Medical Center Laboratories, Hilo, Hawaii, USA Andrzej Semczuk, Lublin Medical University, Lublin, P oland Khan Shah Alam, Department of Orthopaedics, All India Institute of Medical Sciences Ansari Nagar, New Delhi, India Mark G. Shrime, Department of Otolaryngology/Head and Neck Surgery, Boston University Medical Center, Boston, Massachusetts, USA Vernon Keith Sondak, Department of Cutaneous Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA Giuseppe Spriano, Department of Otolaryngology/Head and Neck Surgery, National Cancer Institute Regina Elena, Rome, Italy Keith Stubbs, University of Western Australia, Perth, Australia Fabio Tavora, Department of Genitourinary Pathology, Armed Forces Institute of Pathology, Washington, DC, USA Juliette Thariat, Department of Radiation Oncology, Anti Cancer Center AntoineLacassagne, University Nice Sophia-Antipolis, Nice, France Takeshi Tomonaga, Department of Molecular Diagnosis, Graduate School of Medicine, Chiba University, Chiba Japan Mark Gerard Trombetta, Department of Radiation Oncology, West Penn Allegheny Health System Allegheny General Hospital, PA, USA Lyuba Varticovski, Laboratory of Human Carcinogenesis, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA Salvador Villa I Freixa, Department of Radiation Oncology, Hospital Universitari Germans Trías, Badalona, Catalunya, Spain Takuya Watanabe, Department of Internal Medicine and Gastroenterology, Medical Hospital, The Nippon Dental University of Life Dentistry at Nigata, Nigata, Japan Shigeru Yamada, Research Center for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba, Japan Leandra Náira Zambelli Ramalho, Department of Pathology, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Ribeirdo Preto, SP, Brazil

RARE TUMORS 2009; Volume 1 Metastatic pleomorphic sarcoma to left atrium Ammar H. Hawasli, Rachael Cayce, Trung Luong, Evelyn Taiwo, Michael N. Feliciano, Sharon C. Reimold, John M. DiMaio, Barbara B. Haley..........................................................................................................................................................................................................................................................1 Squamous cell carcinoma of the scrotum in a Nigerian: case report Jerome E. Azike, N.O. Chukwujama, T.C. Oguike...................................................................................................................................................................................................4 Extraosseous osteosarcoma in Ibadan: case series over a 20-year period Temitope O. Alonge, Henry A. Obamuyide, Gabriel Olabiyi Ogun......................................................................................................................................................................6 A multimodal approach to the treatment of bilateral choroidal metastases from thyroid carcinoma Maria Grazia Fabrini, Federica Genovesi-Ebert, Franco Perrone, Mario De Liguoro, Clara Giovannetti, Fausto Bogazzi, Stanislao Rizzo, Enio Martino, Luca Cionini.......................................................................................................................................................................................................................................9 Pleomorphic sarcoma metastatic to the duodenum? Fabio R. Tavora, Allen P. Burke ...............................................................................................................................................................................................................................12 Complete small bowel obstruction caused by metastasis from primary nasopharyngeal carcinoma Chi Pan Lau, Edwin Pun Hui, Anthony Tak-Cheung Chan .................................................................................................................................................................................16 Diagnosis of vulvar lesions by non-invasive optical analysis: a pilot study Anne-Therese Vlastos, Igor Charvet, Ilaria Dellacasa, Federica Capanna, Marie-Françoise Pelte, Philippe Thueler, Michel Saint-Ghislain, Christian Depeursinge, Paolo Meda.............................................................................................................................................................................18 Congenital giant melanocytic nevi Ghulam S. Hashmi, Syed S. Ahmed, Shahla Khan ................................................................................................................................................................................................23 Intramedullary capillary hemangioma of the thoracic spine: case report and review of the literature Rahul Kasukurthi, Wilson Z. Ray, Spiros L. Blackburn, Eriks A. Lusis, Paul Santiago..................................................................................................................................26 A case report of surgical debulking for a huge mass of elephantiasis neuromatosa Manabu Hoshi, Makoto Ieguchi, Susumu Taguchi, Shinya Yamasaki.............................................................................................................................................................29 Value of centrifugated liquid-based cytology by Papanicolaou and May-Grünwald in oral epithelial cells Hussain Gadelkarim Ahmed, Ali Mahmmoud Edris, Eneel Ahmed Mohmed, Mohammed Omer M. Hussein ..........................................................................................31 Retroperitoneal lipoma arising from the urinary bladder Shingo Ukita, Masafumi Koshiyama, Megumi Ohnaka, Naoyuki Miyagawa, Yukio Yamanishi, Fumitomo Nishimura, Michikazu Nagura, Tomoko Kim, Masaya Hirose, Tomoyuki Shirase, Hisato Kobayashi, Hiroshi Ozasa ..............................................................................................34 Primary breast lymphomas Olivier Julen, Ilaria Dellacasa, Marie-Françoise Pelte, Bettina Borish, Christine Bouchardy, Federica Capanna, Georges Vlastos, Jean-Bernard Dubuisson, Anne-Thérèse Vlastos..................................................................................................................................................................................................36 Neuroblastoma occurring in a 38-year old Nigerian man: a rare finding Martin A. Nzegwu, Aloy Aghaji................................................................................................................................................................................................................................42 Primary lymphoepithelial carcinoma of the parotid gland in a North African woman Soumaya Ben Abdelkrim, Amel Trabelsi, Faten Hammedi, Monia Omezzine, Soumaya Rammeh, Atef Ben Abdelkader, Badreddine Sriha .......................................................................................................................................................................................................................................................44 A review of the history, epidemiology and treatment of squamous cell carcinoma of the scrotum Jerome E. Azike ..........................................................................................................................................................................................................................................................47 Assessment of cytological atypia, AgNOR and nuclear area in epithelial cells of normal oral mucosa exposed to toombak and smoking Hussain Gadelkarim Ahmed, Abd-Elraheem Ali Babiker ...................................................................................................................................................................................50 Desmoplastic small round cell tumor: impact of 18F-FDG PET induced treatment strategy in a patient with long-term outcome Dorra Ben-Sellem, Kun-Lun Liu, Sébastien Cimarelli, André Constantinesco, Alessio Imperiale ..............................................................................................................53 [Rare Tumors 2009; volume 1]

Epithelioid hemangioendothelioma of the temporal artery presenting as temporal arteritis: case report and literature review Dina El Demellawy, Ahmed Nasr, Salem Alowami ..............................................................................................................................................................................................56 Retrorectal epidermoid cyst with unusually elevated serum SCC level, initially diagnosed as an ovarian tumor Masaru Hayashi, Shigeki Tomita, Takahiro Fujimori, Hitoshi Nagata, Keiichi Kubota, Akiko Shoda, Kazumi Tada, Nobuaki Kosaka, Ichio Fukasawa, Noriyuki Inaba ...........................................................................................................................................................................................................................59 Metastatic rectal cancer to the breast Hsiao C. Li, Prapti Patel, Payal Kapur, Sergio Huerta .........................................................................................................................................................................................63 Plexiform neurofibroma in the hepatic hilum associated with neurofibromatosis type 1: a case report Sojun Hoshimoto, Zenichi Morise, Chinatsu Takeura, Masahiro Ikeda, Tadashi Kagawa, Yoshinao Tanahashi, Yasuhiro Okabe, Yoshikazu Mizoguchi, Atsushi Sugioka .................................................................................................................................................................................................................66 Cystadenofibroma of the rete ovarii: a case report with review of literature Manisha Ram, Abdel Abdulla, Khalil Razvi, Ivilina Pandeva, Awatif Al-Nafussi..........................................................................................................................................69 Angiomyomatous hamartoma: a rare case report with review of the literature Manisha Ram, Nazar Alsanjari, Naseem Ansari...................................................................................................................................................................................................75 Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation Munir R. Tanas and Brian P. Rubin ........................................................................................................................................................................................................................79 Malignant Triton tumor in the retroperitoneal space associated with neurofibromatosis type 1: a case study Sojun Hoshimoto, Zenichi Morise, Chinatsu Takeura, Masahiro Ikeda,Tadashi Kagawa, Yoshinao Tanahashi, Yasuhiro Okabe, Yoshikazu Mizoguchi, Atsushi Sugioka ................................................................................................................................................................................................................82 A case of primary renal angiosarcoma Kazuhiko Yoshida, Fumio Ito, Hayakazu Nakazawa, Yoshiko Maeda, Hikaru Tomoe, Motohiko Aiba..................................................................................................85 A legacy of tinnitus: multiple head and neck paragangliomas Tricia M.M. Tan, Emma C.I. Hatfield, Rajesh V. Thakker, Eamonn R. Maher, Karim Meeran, Niamh M. Martin, Jeremy J. Turner......................................................88 Soft tissue mixed tumor of the hand Hiroshi Shimosawa, Michiro Susa, Takayuki Honma, Eiichi Hiraishi, Hiroshi Sakihara .........................................................................................................................90 Primary sarcoma of the liver and transplantation: a case study and literature review Benjamin Bismuth, Hélène Castel, Emmanuel Boleslawski, David Buob, Marc Lambert, Nicole Declerck,Valérie Canva, Eli-Serge Zafrani, Philippe Mathurin, François-René Pruvot, Sébastien Dharancy ......................................................................................................................................93 Pitfalls in neuroendocrine tumor diagnosis Emilio Bajetta, Marco Platania ..............................................................................................................................................................................................................................96 Hepatic angiosarcoma five years following spontaneous intraperitoneal bleed of a hepatic mass Jessica L. Cioffi-Pretti, Alexandra N. Kalof, George Ebert, Laurence E. McCahill .........................................................................................................................................98 Colonic cancer in adolescents. A report of three cases M.A.C. Odike, A.E. Dongo, E.F. Alufohai, A.I. Odike............................................................................................................................................................................................102 Tumors and tumor-like lesions of the heart valves Shi-Min Yuan, Hua Jing, Jacob Lavee ..................................................................................................................................................................................................................105 Treatment outcome of maxillary sinus cancer Hye Sung Won, Sang Hoon Chun, Bum-soo Kim, So Ryoung Chung, Ie Ryung Yoo, Chan-Kwon Jung, Yeon-Sil Kim, Dong-il Sun, Min Sik Kim, Jin-Hyoung Kang ......................................................................................................................................................................................................110 Invasive neuroendocrine tumor of the kidney: a case report Ephrem O. Olweny, Michael H. Hsieh, Jill C. Buckley, Jack W. McAninch.....................................................................................................................................................115 Bilateral angiosarcoma of the breast in a fourteen-year-old child Albertus N. van Geel, Michael A. den Bakker......................................................................................................................................................................................................117 Primary extrauterine endometrial stromal sarcoma: response to hormone therapy Gunjal Garg, Awoniyi O. Awonuga, Eugene P. Toy .............................................................................................................................................................................................119

[Rare Tumors 2009; volume 1]

The managament of rare nasal mass-nasal dermoid sinus cysts: open rhinoplasty Emel Cadalli Tatar, Ömer Tarik Selçuk, Güleser Saylam, Ali Özdek, Hakan Korkmaz ..............................................................................................................................121 Lipid-rich histology in a basal-type immuno-profile breast carcinoma: a clinicopathological histochemical and immunohistochemical analysis of a case Serena Russo, Diana Coppola, Paola Vinaccia, Antonella Siciliano, Francesca Baldassarre, Giovanni Battista, Giuseppe Pisani.................................................124 Synchronous malignant B-cell lymphoma and gastric tubular adenocarcinoma associated with paraneoplastic cutaneous vasculitis: hypereosinophilic syndrome with mixed cryoglobulinemia is an important sign of paraneoplastic syndrome Kazuhisa Nozawa, Hiroshi Kaneko, Tomoyasu Itoh, Yoko Katsura, Masaaki Noguchi, Fujihiko Suzuki, Yoshinari Takasaki, Hideoki Ogawa, Kenji Takamori, Iwao Sekigawa.............................................................................................................................................................................................128 Hemangiopericytomas of the spine: case report and review of the literature Chad D. Cole Meic H. Schmidt...............................................................................................................................................................................................................................132 Cervical intramedullary schwannoma: a case report and review of the literature Jardel Mendonça Nicácio, José Carlos Rodrigues Jr, Marcos Henrique Lima Galles, Igor Vilela Faquini, Clemente Augusto de Brito Pereira, Mario Ganau.............................................................................................................................................................................................137 Primary mediastinal giant cell tumor Judd Goldberg, Shameen Azizad, Jela Bandovic, Arfa Khan............................................................................................................................................................................141 Benign multicystic mesothelioma: a case report of three sisters Eve M. Bernstein, Alison Tate, Dan Arin Silasi, Thomas Rutherford...............................................................................................................................................................143 Congenital infantile digital fibromatosis: a case report and review of the literature Valérie Failla, Odile Wauters, Nazli Nikkels-Tassoudji, Alain Carlier, Josette André, Arjen F. Nikkels....................................................................................................146 Giant mesenteric cystic lymphangioma presenting with abdominal pain and masquerading as a gynecologic malignancy John Maa, Christianne Wa, Adnan Jaigirdir, Soo-Jin Cho, Carlos U. Corvera ..............................................................................................................................................148 Malignant peritoneal mesothelioma. Is there a new treatment? Kakil Ibrahim Rasul, David J. Kerr .......................................................................................................................................................................................................................150 Intracystic papillary carcinoma of the breast in a 21-year old premenopausal Nigerian woman: a case report Ivy N. Umanah, Akpan S. Okpongette ..................................................................................................................................................................................................................155 Diagnostic confusion resulting from CD56 expression by cutaneous myeloid sarcoma Thanh Ho, Franklin Sedarat, Nagesh Rao, Sheeja T. Pullarkat........................................................................................................................................................................156 The epidemiology of malignant giant cell tumors of bone: an analysis of data from the Surveillance, Epidemiology and End Results Program (1975-2004) Jennifer L. Beebe-Dimmer, Karynsa Cetin, Jon P. Fryzek, Scott M. Schuetze, Kendra Schwartz ...............................................................................................................159 Temsirolimus in the treatment of renal cell carcinoma associated with Xp11.2 translocation/TFE gene fusion proteins: a case report and review of literature Jigarkumar Parikh, Teresa Coleman....................................................................................................................................................................................................................164 Metastasizing pleomorphic adenoma presenting as an asymptomatic kidney tumor twenty-nine years after parotidectomy – urological viewpoint and overview of the literature to date Jan Ebbing, Carolin Blind, Harald Stein, Kurt Miller, Christoph Loddenkemper ........................................................................................................................................167 A case of primary mucosa-associated lymphoid tissue lymphoma of the prostate Noriko Koga, Masanori Noguchi, Fukuko Moriya, Kouichi Ohshima, Nobuyuki Yoshitake, Kei Matsuoka, Jan Ebbing, Carolin Blind, Harald Stein, Kurt Miller, Christoph Loddenkemper.........................................................................................................................................................................................169 Re: Koga et al. A case of primary mucosa-associated lymphoid tissue lymphoma of the prostate Damien C. Weber......................................................................................................................................................................................................................................................171 A clinical and molecular project on gonadoblastoma needs international collaboration Nicolas Kalfa, Olivier Maillet, Charles Sultan....................................................................................................................................................................................................172 Primary NK/T cell lymphoma nasal type of the stomach with skin involvement: a case report Sebastian Kobold, Hartmut Merz, Markus Tiemann, Carolina Mahuad, Carsten Bokemeyer, Irmtraut Koop, Walter Fiedler............................................................173

[Rare Tumors 2009; volume 1]

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[Rare Tumors 2009; volume 1]

Rare Tumors 2009; volume 1:e1

Metastatic pleomorphic sarcoma to left atrium Ammar H. Hawasli,1 Rachael Cayce,2 Trung Luong,2 Evelyn Taiwo,1 Michael N. Feliciano3, Sharon C. Reimold,2,4 John M. DiMaio,5 Barbara B. Haley1,2 Departments of 1Hematology-Oncology, 2 Internal Medicine, 3Pathology, 4 Cardiology, and 5Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA

Abstract Although several thousand patients are diagnosed with sarcoma annually in the United States, metastases to the heart are very uncommon. In this case report, an overall low frequency cancer presents masquerading with common cardiac symptomology. This case illustrates the importance for detailed diagnostic cardiac evaluations and heightened suspicion by physicians to consider metastatic disease to the heart in cancer patients with cardiovascular complications. Also discussed is a review of surgical and chemotherapeutic options for this problem.

strength, focal tenderness over the sacrum, pitting edema in the lower extremities and a murmur of mitral regurgitation with basilar crackles and dullness. Computed tomography (CT) showed a wedge-shaped splenic infarct and magnetic resonance imaging (MRI) of the spine demonstrated osseous metastatic disease involving the vertebral bodies, with epidural and neuroforaminal tumor extension in the sacrum. A TTE was performed in anticipation of chemotherapy with adriamycin and revealed a large vegetation attached to the anterior leaflet of the mitral valve with prolapse into the left atrium and ventricle (Figure 1A, arrow) and hypoechoic regions within the cardiac tissue (Figure 1A, arrowhead). The patient was taken to the operating room to remove the vegetation, biopsy the hypoechoic mass and replace the mitral valve. A 9 cm vegetation was excised from the anterior mitral valve leaflet (Figure 1B, left and middle). Intraoperatively, the patient was noted to have diffuse disease throughout the atrial septum and mitral valve (Figure 1B, right) and the procedure was terminated. Post-operative cardiac MRI showed a lobulated cardiac mass infiltrating the atrial septum, the posterior aortic wall and the posterior aspect of the anterior mitral valve annulus. In the axial plane, the mass measured at least 5.3 x 2.2 cm and extended to the superior portion of the left atrium near the entry of the right superior pulmonary vein

Correspondence: Dr. Barbara B. Haley, The Harold C. Simmons Comprehensive Cancer Center, University of Texas Southwestern Medical Center at Dallas, Dallas, TX, USA E-mail: [email protected] Key words: sarcoma, cardiac metastases, tumor, cancer. Received for publication: 1 May 2009. Accepted for publication: 5 May 2009. Acknowledgement: we would like to thank Dr. John Bagwell and Leah Gaither for their assistance. This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0) ©Copyright A.H. Hawasli et al., 2009 Rare Tumors 2009; 1:e1 doi:10.4081/rt.2009.e1

(Figure 1C; mass indicated with arrowheads). Histology of the atrial mass revealed a highgrade pleomorphic sarcoma, metastatic to heart. Low power frozen sections of the atrial wall showed hypercellularity (Figure 2A). Medium and high power frozen (Figure 2B and C) and permanent (Figure 2D) sections showed tumor giant cells (indicated with arrowheads) among spindled and epithelioid

Introduction A 53-year-old Caucasian gentleman presented with progressive lower back pain, urinary and bowel incontinence, and lower extremity swelling over a one month period. He had been diagnosed six months prior at an outside institution with a high-grade pleomorphic sarcoma with focal myxoid and epithelioid elements of the duodenum. Small bowel resection was performed because of bowel obstruction. Perioperatively, the patient suffered a myocardial infarction (MI) due to focal narrowing of the left anterior descending artery and a drugeluting stent was placed during cardiac catheterization. Post-operative transthoracic echocardiogram (TTE) was interpreted as mild mitral valve thickening, with flail chordae tendineae and an estimated ejection fraction of 35-40%. After discharge, the patient was lost to follow-up.

Case report On presentation to our facility, physical examination revealed normal lower extremity

Figure 1. Echocardiography (A) performed before excision of vegitation (B) and postoperative cardiac MRI (C).

[Rare Tumors 2009; 1:e1]

[page 1]

Case Report tumor cells. The atrial mass was immunohistochemically positive for vimentin, confirming mesenchymal differentiation (Figure 2E). Immunohistochemical stains for other markers, including CD117, AE1/AE3, desmin, myogenin, caldesmon, S100, HMB-34, calretinin, CD31 and CD34 were negative (Figure 2F). A diagnosis of metastatic cardiac sarcoma was established and treatment with gemcitabine and docetaxel was initiated. Adriamycin-based therapy was not considered secondary to the patient’s reduced cardiac function (37% ejection fraction). Five weeks after initiating chemotherapy, the patient was admitted for worsening lower extremity edema but denied shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, chest pains, palpitations, weight gain, or symptoms of transient ischemic attacks. Electrocardiogram revealed atrial flutter at 119 beats per minute with 2:1 block and the old anterolateral infarct. The patient declined cardioconversion and was treated with amiodarone. At the time of preparation of this report, the patient was tolerating palliative gemcitabine and docetaxel chemotherapy.

Discussion Soft-tissue sarcomas are a diverse mix of cancers with an incidence of 10,390 new cases diagnosed annually in the United States.1 Despite aggressive treatment with surgery, radiation and chemotherapy, outcomes remain suboptimal as 3,680 (35%) soft-tissue sarcoma patients die annually.1 Death secondary to metastases is common and sites of metastases vary depending on the type of soft-tissue sarcoma with most types showing a predilection for the lungs. Metastatic involvement of the heart by soft-tissue sarcoma has been reported in the literature but most cases of cardiac involvement occur in the setting of widespread metastases.2 Our patient is among the first reported cases of an epithelioid high-grade pleomorphic sarcoma metastatic to the heart in absence of known widespread metastases. Familiarity with the unique characteristics of the soft-tissue sarcomas will enable a physician to identify expected patterns of metastatic spread. One should be suspicious of cardiac metastases in a soft-tissue sarcoma patient who suddenly develops unexplained congestive heart failure, new cardiac murmurs, arrhythmias or embolic phenomena. For such patients, metastatic cardiac disease should be included in the differential diagnosis and further evaluation with electrocardiograms, echocardiograms, and cardiac MRI should be made. Accurate imaging of cardiac cancer is very important in the multimodal medical and surgical management of metastatic cardiac [page 2]

Figure 2. Histology of atrial mass.

sarcoma.3 In this patient, cardiac metastasis was only incidentally discovered during prechemotherapy cardiac evaluation. However, several aspects of this patient’s clinical course suggested the presence of this diagnosis. The patient’s initial myocardial infarction prompted cardiac catheterization, which showed luminal narrowing assumed to be secondary to atherosclerotic disease. Yet, on repeat studies, the patient had no evidence of significant atherosclerotic disease. The patient’s infarct likely resulted from an embolic event originating from the large mitral valve vegetation or from extrinsic compression of the artery by tumor. The splenic lesions noted on CT scans likely represent distant emboli with infarction. Finally, review of the outside hospital TTE abnormalities noted after the patient’s perioperative MI indicated that he had cardiac metastases at initial presentation. A high degree of suspicion for cardiac involvement by sarcoma may have hastened a correct diagnosis.

Conclusions Management of metastatic sarcoma to the heart remains a difficult task. Some reports discuss surgical resection of cardiac metastatic lesions with variable success,4,5 while others report the use of cardiac transplantation.6 In this case, surgical excision was attempted, but the extensive infiltrating nature of the tumor prohibited complete debridement leaving systemic therapy as the only viable option. There are several generally accepted systemic therapies for metastatic sarcoma with most regimens including doxorubicin as a single agent7 or in combination with other drugs. The most widely employed combination chemotherapy regimens are doxorubicin/dacarbazine,8,9 doxorubicin/ifoxfamide/mesna10,11 mesna/doxorubicin/ifosfamide/dacarbazine,12 ifosfamide/epi [Rare Tumors 2009; 1:e1]

rubicin/mesna,13 and gemcitabine/docetaxel.14-16 However, the use of doxorubicin was felt to be contraindicated due to the depressed cardiac function in this patient. If a sarcoma expresses CD117, imatinib would be a viable treatment option due to the drug’s oral administration, favorable toxicity profile, and anti-tumor activity on some subsets of sarcoma, particularly gastrointestinal stromal tumors.17-19 Expression of this marker is felt to be favorable due to this relationship. Thus far, reports do not associate imatinib with induction of cardiac failure.20 Unfortunately our patient’s cardiac tumor did not express CD117 and the drug was not a treatment option. Since metastatic sarcoma to the heart is a rare occurrence, no prospective or randomized trials exist to help guide treatment. In fact, there are no data to show whether systemic treatment of unresectable cardiac sarcomas significantly improves duration or quality of life. In the setting of congestive or restrictive heart failure, many of the first-line chemotherapy options are not feasible for fear of therapy worsening cardiac function. In our case, gemcitabine/docetaxel chemotherapy was felt to be an active regimen with controllable toxicity and without potential devastating cardiac side effects. Tolerance to the treatment regimen thus far has been acceptable and the patient is continuing this chemotherapy.

References 1. Jemal A, Siegel R, Ward E, et al. Cancer statistics, 2008. CA Cancer J Clin 2008;58: 71-96. 2. Catton C. The management of malignant cardiac tumors: clinical considerations. Semin Diagn Pathol 2008;25:69-75. 3. Yuan SM, Shinfeld A, Lavee J, et al. Imaging morphology of cardiac tumours. Card-

Case Report iol J 2009;16:26-35. 4. Harting MT, Messner GN, Gregoric ID, Frazier OH. Sarcoma metastatic to the right ventricle: surgical intervention followed by prolonged survival. Tex Heart Inst J 2004;31:93-5. 5. Kono T, Amano J, Sakaguchi M, Kitahara H. Successful resection of cardiac metastatic liposarcoma extending into the SVC, right atrium, and right ventricle. J Card Surg 2005;20:364-5. 6. Goldstein DJ, Oz MC, Rose EA, et al. Experience with heart transplantation for cardiac tumors. J Heart Lung Transplant 1995;14:382-6. 7. Tierney J Fea. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 1997;350:1647-54. 8. Antman K, Crowley J, Balcerzak SP, et al. An intergroup phase III randomized study of doxorubicin and dacarbazine with or without ifosfamide and mesna in advanced soft tissue and bone sarcomas. J Clin Oncol 1993;11:1276-85. 9. Zalupski M, Metch B, Balcerzak S, et al. Phase III comparison of doxorubicin and dacarbazine given by bolus versus infusion in patients with soft-tissue sarcomas:

10.

11.

12.

13.

14.

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a Southwest Oncology Group study. J Natl Cancer Inst 1991;83:926-32. Edmonson JH, Ryan LM, Blum RH, et al. Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. J Clin Oncol 1993;11:1269-75. Grobmyer SR, Maki RG, Demetri GD, et al. Neo-adjuvant chemotherapy for primary high-grade extremity soft tissue sarcoma. Ann Oncol 2004;15:1667-72. Elias A, Ryan L, Sulkes A, et al. Response to mesna, doxorubicin, ifosfamide, and dacarbazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy. J Clin Oncol 1989;7:1208-16. Frustaci S, Gherlinzoni F, De Paoli A, et al. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial. J Clin Oncol 2001;19: 1238-47. Hensley ML, Maki R, Venkatraman E, et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 2002;20: 2824-31. Leu KM, Ostruszka LJ, Shewach D, et al. Laboratory and clinical evidence of synergis-

[Rare Tumors 2009; 1:e1]

16.

17.

18.

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tic cytotoxicity of sequential treatment with gemcitabine followed by docetaxel in the treatment of sarcoma. J Clin Oncol 2004;22: 1706-12. Maki RG. Gemcitabine and docetaxel in metastatic sarcoma: past, present, and future. Oncologist 2007;12:999-1006. Verweij J, Casali PG, Zalcberg J, et al. Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet 2004;364: 1127-34. Blanke CD, Demetri GD, von Mehren M, et al. Long-term results from a randomized phase II trial of standard- versus higherdose imatinib mesylate for patients with unresectable or metastatic gastrointestinal stromal tumors expressing KIT. J Clin Oncol 2008;26:620-5. Demetri GD, von Mehren M, Blanke CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 2002;347:472-80. Verweij J, Casali PG, Kotasek D, et al. Imatinib does not induce cardiac left ventricular failure in gastrointestinal stromal tumours patients: analysis of EORTC-ISGAGITG study 62005. Eur J Cancer 2007;43: 974-8.

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Rare Tumors 2009; volume 1:e2

Squamous cell carcinoma of the scrotum in a Nigerian: case report Jerome E. Azike,1,2 N.O. Chukwujama,1,2 T.C. Oguike1,2 1

Department of Surgery, College of Medicine and Health Sciences, Imo State University, Orlu, Imo State, Nigeria; 2 Imo State University Teaching Hospital, Orlu, Imo State, Nigeria

Abstract Squamous cell carcinoma of the scrotum is rare and to the best of our knowledge has never been reported from Nigeria. We report on a case thought to be occupation-related in a 42-year old Nigerian taxi driver who had previously been an automobile mechanic and later a long-haul truck driver. He presented with a stage D disease and only palliation was feasible.

Case report A 42-year old taxi driver who had been a car mechanic for four years 25 years earlier presented in 2007 with a ten-year history of a small pruritic painless nodule on his ventral scrotal surface which had ulcerated but failed to heal. He infrequently washed his work-outfit while an auto-mechanic and admitted to its frequent soiling, sometimes up to his underwear, with used engine oil while at work. He took a bath at the close of work each day; however, his personal hygiene outside the workplace was good. On examination he was pale, had a foulsmelling, grotesque, fungating, ulcero-proliferative mass with rolled edges which involved the whole scrotum and proximal third of the penile shaft, extending 5 cm to the right thigh antero-medially and 3 cm of the anal verge. The scrotum was fixed to the pubis. There were bilateral inguinal lymphadenopathy involving both the vertical and horizontal chains. He looked otherwise well nourished and was ambulant. He was also rather depressed emotionally. Ultrasonography revealed a hepatomegaly and para-aortic lymphadenopathy, the largest 2.8 cm in diameter. His general condition was optimized and biopsy confirmed the diagnosis. He had palliative scrotectomy with bilateral orchidectomy and subsequently cisplatinumbased combination chemotherapy, after he had been counseled and informed consent [page 4]

obtained. Histopathological review showed scrotal squamous cell carcinoma with testicular involvement (Figure 1). He succumbed to the disease after seven months.

Correspondence: Jerome E. Azike, MBBS, FWACS, Department of Surgery, College of Medicine & Health Sciences, Imo State University, Orlu, Nigeria. E-mail: [email protected]

Discussion

Key words: cancer of the scrotum, occupational cancer, automobile mechanic, used engine oil.

The scrotum is a seven-layer pouch which invests the testes, testicular adnexae, and distal spermatic cord. The scrotal lymphatics drain into the corresponding superficial inguinal nodes. Anastomoses exist between the lymphatics of the contralateral network across the median raphe. Tumors have been reported that arise out of virtually any of the components of the scrotal wall.1 Squamous cell carcinoma of the scrotum is now exceedingly rare. It was the first malignancy linked to occupational exposure.1-2 Previously, it most commonly resulted from exposure to environmental carcinogens such as chimney soot, tars, paraffin, and some petroleum products. Currently most cases result from poor hygiene and chronic inflammation.3 Used engine oils have elevated polycyclic aromatic hydrocarbons which tend to be greater for petrol engines than for diesel engines. Prolonged and repeated contact with such oils can cause skin and scrotal cancer. Car mechanics are at potential risk from used engine oil.4 Best occupational health practices are invaluable at the workplace for those at risk to

Received for publication: 2 May 2009. Accepted for publication: 8 May 2009. This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0) ©Copyright J.E. Azike et al., 2009 Rare Tumors 2009; 1:e2 doi:10.4081/rt.2009.e2

minimize exposure. Prognosis correlates with extent of nodal involvement with virtually no survivors if iliac nodes are involved.3,5 Ability to achieve a negative margin at the time of initial surgery is an important prognostic factor as adjunctive therapy such as radiotherapy or chemotherapy or both has not proven useful.1 In conclusion, the index patient presented very late when even surgery, chemotherapy and radiotherapy could not possibly offer any meaningful improvement in terms of outcome. We believe this case will raise the awareness in our environment of the existence of this disease and improve the index of suspicion among practicing surgeons and oncologists.

Figure 1. a, b, c, d. Histology of the various sections of the tumor; (a) Normal skin and contiguous areas the well differentiated squamous cell carcinoma invading the surrounding deeper tissue; (b) High magnification of the malignant squamous cells; (c) Remnants of the testicular tubules being invaded by the tumor; (d) The malignant squamous cells invading the deeper testes.

[Rare Tumors 2009; 1:e2]

Case Report

References 1. Rowland RG, Herman JR. Tumors and Infectious Diseases of the Testis, Epididymis, and Scrotum. In Gillenwater JY, Grayhack JT, Howards SS and Mitchell

ME, eds. Adult and Pediatric Urology, Philadelphia: Lippincott, Williams and Wilkins; 2002. 2. Waldron HA. A brief history of scrotal cancer. Br J Ind Med 1983;40:390-401. 3. Presti JC Jr. Genital Tumors. In Tanagho EA, McAnich JW, editors. Smith’s General Urology. New York: Mc Graw Hill 2008:375-87.

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4. Wyre Borough Council, Lancashire U.K., Health and Safety Factsheet – No. 12 (internet), 2002. Available from: http:// www.wyrebc.gov.uk (Cited 5th July, 2008) 5. Ray B, Whitmore WF Jr. Experience with Carcinoma of the Scrotum. J Urol 1977; 117:741-5.

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Rare Tumors 2009; volume 1:e3

Extraosseous osteosarcoma in Ibadan: case series over a 20-year period Temitope O. Alonge,1 Henry A. Obamuyide,2 Gabriel Olabiyi Ogun3 1

Consultant Orthopedic Surgeon/Senior Lecturer, Department of Orthopedics and Trauma, University College Hospital, and College of Medicine, University of Ibadan, Ibadan, Nigeria; 2 Resident in Surgery, Department of Surgery, University College Hospital, Ibadan, Nigeria; 3 Consultant Pathologist/Lecturer, Department of Pathology, College of Medicine, and University College Hospital, Ibadan, Nigeria

Abstract Extraosseous osteosarcoma (EOO) is a rare form of sarcoma. There have been few reports of cases and outcome from an African population. Out of 112 cases of sarcomas seen at the UCH, Ibadan between 1986-2005, 5 were EOO. All presented late on account of initial excision without histology and outcomes were poor. EOO occurs in the black population of SubSaharan Africa. The outlook for these patients is still bleak.

Introduction Extraosseous osteosarcoma (EOO) is a malignant mesenchymal neoplasm that produces osteoid, bone or chondroid material and is located in the soft tissue without attachment to the skeleton as determined by imaging modalities or inspection during surgical operative procedure.1 EOO is a rare type of tumor making up only 1% of all soft tissue sarcomas2,3 and 4% of all osteosarcomas.4 Wilson reported the first case of EOO in 19415 and since then fewer than 300 cases have been reported in the English Language literature.6 The tumor is most common in middle-aged and elderly patients. A previous study showed that EOO is a doxorubicin-resistant lesion with a poor prognosis and a form of soft tissue sarcoma that should be viewed by clinicians as clinically and therapeutically distinct from osseous osteosarcoma.6 The single most important criteria for diagnosis of this tumor is the presence of neoplastic osteoid and bone; sometimes with neoplastic cartilage.1,5 The histological variants of EOO include osteoblastic, chondroblastic, fibroblas[page 6]

tic, osteoclastic or giant cell type, telangiectatic, small cell or well differentiated forms. The immunohistochemical profile includes the expression of osteocalcin and osteonectin which are specific for osseous osteosarcomas, but a study by Fanburg-Smith et al.7 showed that osteocalcin is highly sensitive for EOO neoplastic cells.1 Closely related differentials include calcified hematoma, myositis ossificans, synovial sarcoma, epithelial sarcoma, liposarcoma or malignant fibrous histiocytoma.

Methods All sarcomas recorded at our institution between January 1986 and December 2005 were compared and extraosseous variety identified. Their charts were selected and reviewed for relevant data. Out of 112 cases of osteosarcoma recorded in our hospital between January 1986 and December 2005, 5 cases were extraosseous. This series presents the 5 cases of EOO recorded at our institution during the 20-year period from 1986-2005 and this includes one patient reported previously by Ogundiran et al.8

Case Series Case #1 A 16-year-old male student who presented with a 4-year history of progressive left ankle swelling and pain. He had excision of a mass in his left ankle at a private facility without histological evaluation a year prior to presentation at the University College Hospital (UCH), Ibadan. On presentation at the UCH, examination revealed a 10x7 cm mass over the left lateral malleolus that was tender, differentially warm, with both cystic and solid areas. No significant popliteal or inguinal lymphadenopathy was noted. Laboratory studies were essentially normal. He had a local excision of the mass and the operative finding was a 10x7 cm multiloculated brownish soft tissue mass over the left lateral malleolus covered with a fibrous capsule with a pedicle arising from the ankle joint. There was associated erosion of the adjoining bone and the histology report revealed soft tissue osteosarcoma. He was lost to follow-up but represented two years later with recurrence.

Case #2 A 70-year-old man who presented with a 1-year history of a painful scalp swelling associated with bleeding. A similar swelling had recently grown on the right temporal region prior to presentation at the UCH. Incisional [Rare Tumors 2009; 1:e3]

Correspondence: Temitope O. Alonge, Consultant Orthopedic Surgeon/Senior Lecturer, Department of Orthopedics and Trauma, University College Hospital and College of Medicine, University of Ibadan, Ibadan, Nigeria E-mail: [email protected] Key words: extraosseous osteogenic sarcomas, Sub-Saharan Africa, histology. Received for publication: 3 May 2009. Accepted for publication: 5 May 2009. Contributions: TOA conceived, designed, supervised and helped to draft the manuscript and approved the final version; HAO participated in the design, gathered the data, drafted the manuscript and approved the final copy; GOO reviewed the manuscript for critical content and approved the final copy. This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0) ©Copyright T.O. Alonge et al., 2009 Rare Tumors 2009; 1:e3 doi:10.4081/rt.2009.e3

biopsy and histology of the initial mass at the source of referral revealed an extraskeletal osteosarcoma. Examination revealed a 15x7 cm left frontoparietal mass with overlying scar. He also had a smaller temporal mass. Both were tender and hemorrhagic. Skull X-ray showed an extensive soft tissue swelling extending from the left frontal to the left parietal region. There was an associated increase in the density of the underlying subcutaneous tissue but no calcifications. Routine laboratory studies were within normal limits. He had radiotherapy but later represented with facial recurrence after two months.

Case #3 A 21-year-old man with a 1-year history of a painful left upper back swelling and 4 month history of progressive weakness of both legs which progressed to paraplegia. He had a biopsy of the mass at a private facility five months before presentation at the UCH but no histological evaluation of the mass was carried out. Examination at the UCH revealed a left periscapular fluctuant mass with overlying hypertrophic scar which was also attached to underlying structures. He also had a T5 paraplegia. Chest X-ray showed a left paravertebral soft tissue mass lesion with evidence of destruction of the 5th rib and left sided pulmonary effusion. Urinary Bence Jones protein was negative, the full blood count was normal but the ESR was elevated. Two pleural aspirates were hemorrhagic and CT myelography showed an isolated left sided isodense lesion

Case Report in the spinal cord with extensive erosion of the ribs as well as infiltration into the left pleural cavity. Incisional biopsy of the left chest wall lesion revealed osteogenic sarcoma. Chemotherapy was to be administered but the patient could not afford it and he was subsequently lost to follow-up.

Case #4 A 20-year-old man who presented with an 11-month history of progressive soft tissue swelling of the right thigh associated with pain. The soft tissue mass was excised initially at a private facility but no histological evaluation of the specimen was carried out. Examination at the UCH revealed a massively swollen right thigh with multiple areas of ulceration. Biopsy and histology at the UCH revealed extraosseous osteogenic sarcoma. The patient was given a hemostatic dose of radiotherapy but was subsequently lost to follow-up.

Case #5 A 40-year-old housewife who presented with a 20-month history of painful left breast lump which had been excised previously at another hospital without histological evaluation, but recurred eight months before presentation at UCH. Examination revealed a globular left breast mass which was warm, multinodular, fixed to the pectoralis fascia and which measured 20x18 cm. There was associated ipsilateral axillary lymph node enlargement. Other systems were essentially normal. A clinical diagnosis of locally advanced cancer of the left breast was made. A core needle biopsy revealed an osteogenic sarcoma which was confirmed by the histology of the mastectomy specimen. She was scheduled for postoperative radiotherapy but defaulted and died six months post-mastectomy.

Discussion The exact cause of EOO is unknown and it is presumed to be mainly an idiopathic condition. Unlike osseous osteosarcoma (OOO), it has not been documented in siblings or in association with hereditary retinoblastoma but risk factors associated with the development of this tumor include previous exposure to radiation, such as X-rays and radioactive thorium dioxide (Thorothrast).9 Other associated factors suggested in literature in the development of EOO include trauma, the assessment and evaluation of which is difficult and controversial.10 Some cases have been associated with intramuscular injection11 while some EOO have been reported to follow myositis ossificans.12,13

A common mode of presentation is as a swelling of insidious onset with associated pain in one-third of patients but often the tumor grows to a large size before the patient seeks medical advice.14 It may ulcerate with growth, but that usually occurs after biopsy or attempts at excision and the tumor usually occurs in middle-aged and elderly patients.15 The anatomic location of EOO is usually the muscles of the thigh, which are the most commonly affected; the large muscles around the pelvic and shoulder girdles and the retroperitoneum, though rare locations like the thyroid gland, penis, mediastinum and the kidney are occasionally encountered.1,12,15,17,18 At the Memorial Sloan-Kettering cancer center, a review of 48 cases of EOO during 1950-1983 showed a median age at diagnosis of 51 years (range, 6-80 years).14 The most common primary sites were the thighs and buttocks (54.2%) with preponderance in patients aged 50 years and above and slightly more common in males (58%) than in females.12 In the report by Chung and Enzinger, EOO occurs principally as a soft tissue mass involving an extremity with a predilection for the thighs (lower extremity 46.6%; upper extremity 20.5%) and the retroperitoneum in 17%. In most cases, the tumor was deep seated and firmly attached to the fascia, but occasionally they are freely movable and confined to the subcutis or dermis. The duration of symptoms prior to presentation ranges from two weeks to 25 years (median six months). Prior trauma to the site was observed in 12.5% and irradiation to the affected site in 5.7% of cases.12 Although osseous osteosarcoma (OOO) occurs predominantly in the first two decades of life, EOO are rarely encountered under 40 years of age.15 However, in this case series, 3 of the 5 patients were aged 21 years and under in keeping with other cases which have been documented in the pediatric age group.16 Imaging modalities by either plain conventional radiograph, CT scan or MRI of the soft tissue is essential to rule out any continuity of tumor with bone. If adjacent bone shows radiological changes of involvement by the tumors, it is most likely to be originating from the bone rather than from the soft tissue. A soft tissue mass with spotty to massive calcification without adjacent bone involvement is one of the classic radiographic appearances of this tumor.15 EOO is a difficult disease to treat and the optimum therapy has not been fully determined due to the relative rarity of these tumors. Advances in the care of these patients will require disease-specific clinical trials.6 A wide local excision, with at least 5cm margin of normal tissues should be the treatment of choice.15 The local recurrence-free survival rate observed in a recent study6 suggests that patients with extremity EOO can be treated with limb salvage operative procedures. [Rare Tumors 2009; 1:e3]

However, if these are not possible due to the anatomic location of the tumor, amputation is recommended.19 In a review of 48 cases of EOO and 39 trials of chemotherapeutic agents reported by Sordillo et al. they showed that no patient had a major response.14 The average 5-year survival rate in 5 previous studies ranges from 1525%2,9 and the response to multimodality therapy is not as good as for OOO.14 Tumor size (