Indian Journal of Basic & Applied Medical Research; December 2013: Issue-1, Vol.-3, P.33-36
Case Report :
“Malignant mixed mullerian tumor of the uterus : a case report” Dr. Tekwani Deepa T, Dr. Joshi Sneha R, Dr.Smita Pathak, Dr Mangala Nagare, Dr.Ashwin Bihade, Dr. Deepak Kendre Department of Pathology, Maharashtra Institute of Medical Education & Research (MIMER), Talegaon Dabhade, Pune , India Corresponding author: Dr. Deepa Tekwani ; Email:
[email protected]
ABSTRACT: Malignant Mixed Mullerian Tumor (MMMT) of the uterus is an uncommon (2% – 5%), extremely aggressive neoplasm histologically composed of malignant epithelial and mesenchymal elements. They are found predominantly in postmenopausal women, presenting with uterine bleeding and enlargement. The present case is of a 70 year old post-menopausal female who had per vaginal bleeding since 2 months. Ultrasonography revealed an ill-defined heterogenous soft tissue mass, suggesting possibility of neoplastic pathology of the uterus.Diagnostic curettage was done, which on histopathological examination showed Malignant Mixed Mullerian Tumor with heterologous elements. Panhysterectomy was done, and the diagnosis of Malignant Mixed Mullerian Tumor of the uterus was confirmed. KEYWORDS: Malignant Mixed Mullerian Tumor, Uterus, Postmenopausal.
INTRODUCTION
CASE REPORT
Malignant Mixed Mullerian Tumors (MMMTs) of
A 70 Year old, multiparous woman presented with
the uterus are rare, high grade neoplasms
post-menopausal per vaginal bleeding since 2
comprising only 2 to 5% of all tumors derived from
months.
1
the body of the uterus. It is a biphasic neoplasm
P/S & P/V Examination: - bulky uterus.
comprising of both carcinomatous (epithelial
Chest X-ray - NAD
tissue) &sarcomatous (connective tissue) compo-
USG: - Revealed an ill- defined heterogeneous soft
nents. It is divided into 2 types, homologous (in
tissue mass of about 5×4.8 cm in the uterine body,
which the sarcomatous component is made up of
suggesting a possibility of neoplastic pathology.
tissues found in the uterus such as endometrial,
Diagnostic curettage was done & sample was sent
fibrous &/ or smooth muscle tissue) & a
to Dept.of Pathology. Histopathological exami-
heterologous type (made up of tissues not found in
nation showed a Malignant Mixed Mullerian
uterus, such as cartilage, skeletal muscle &/ or
Tumor with Heterologous Elements. This was
1
bone). In the present report, we describe a case of
followed by surgery. The type of operation was
Malignant Mixed Mullerian Tumor of the Uterus
Total Abdominal Hysterectomy with Bilateral
with heterologous elements.
Salphingo- Oophorectomy In pathologic evaluation, grossly, the tumor was polypoid,
friable
with
areas
of
haemmo-
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Indian Journal of Basic & Applied Medical Research; December 2013: Issue-1, Vol.-3, P.33-36
rhages&necrosis, measuring 8.5×4×3.5cm & filling
with MMMT of uterine cervix have been reported
the uterine cavity. The tumor was arising from the
as well.5 In our case the tumor was arising from
postero-lateral uterine wall. The endometrialcavity
posterior-lateral uterine wall along with three tiny
also showed 3 polypoidalmasses, largest measuring
polypoidal
1 cm in diameter, which on cut section showed
MMMT is most often made postoperatively by
cystic spaces containing mucinous Fluid.
histological examination. However, pre-operative
(Fig 1 & 2).
diagnosis of uterine MMMT will facilitate the
Light microscopy of the mass showed a heteogeno-
planning of appropriate surgical management with
usmalignant neoplasm with biphasic pattern. The
adjuvant therapy.
epithelial parts of the tumor consisted of glandular
show MMMT to be a heterogenous, hypodense, ill-
component with papillary pattern at places &focal
defined mass filling the uterine cavity.3
squamous differentiation. Furthermore, sarcom-
Grossly MMMTs are almost invariably fleshy,
atous areas consisting ofspindleoval nuclei focally
necrotic, haemorrhagic, polypoidal growths that
arranged in bundles accompanied by foci of chond-
often filled the uterine cavity.
ro-sarcoma were found. Microscopic examination
gross
of other 3 polypoidalmasses showed similar histo-
microscopic features ofMMMTs are a mixture of
pathological features. (Fig no:3,4&5).
carcinomatous and sarcomatouselements resulting
Histopathological examination ofleftovary showed
in a biphasic pattern. The epithelial component of a
a metastatic deposit of Adenocarcinoma.
carcinosarcoma may be any type of mullerian
The Final Diagnosis given was Malignant Mixed
carcinoma: mucinous, squamous, serous, endom-
Mullerian Tumor with Heterologous Elements with
etroid,
Metastatic Deposit in the Left Ovary.
undifferentiated, or a mixture of these types. The
The Stage of the tumor was ΙΙΙA.
appearance of the sarcomatous component is the
Discussion
basis for division of these neoplasms into
Malignant mixed mullarian tumors of the uterus are
homologous (leiomyosarcoma, stromal sarcoma,
rare neoplasms that are practically always seen in
fibrosarcoma)
postmenopausal patients.
2
The symptom triad
masses.Traditionally
findings
high
3
diagnosis
of
Radiological investigations
6
In our case similar
were noted.The
grade
and
papillary,
characteristic
clear
heterologous
cells,
varieties
(chondrosarcoma, rhabdomyosarcoma, osteogenic liposarcoma).6In
indicative of MMMT includes pain, severe vaginal
sarcoma,
bleeding and passage of necrotic tissue per
histopathological diagnosis given was malignant
vaginum.
3
Very little is known about the
aetiopathogenesis
of
MMMTs.
Exposure
to
our
case
the
mixed mullerian tumor (Adenocarcinoma) with heterologous elements (Chondro-sarcoma).
radiation, excessive estrogen exposure, obesity, and
MMMTs express epithelial (EMA, Pancytokeratin)
nulliparity are belived to be associated with
& stromal lineage marker in relation to their
4
MMMT development. In our case the patient was
histological appearances such as desmin in
70yr old postmenopausal women presenting with
muscular differentiation or S100 in areas with
abnormal vaginal bleeding, with no known
chondroid or lipomatous differentiation.3 However
predisposing factor.
IHC studies are not mandatory for diagnosis of
The usual location is the uterine body, particularly
MMMT.
the posterior wall of the fundus but a few cases
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Indian Journal of Basic & Applied Medical Research; December 2013: Issue-1, Vol.-3, P.33-36
3
Abdominal hysterectomy with bilateral salpingo –
glands or bone, heart, & brain.
oophorectomy and pelvic lymphadenectomy is the
tumor had produced metastatic deposit to the left
treatment of choice, usually followed by adjuvant
ovary.
2
In our case the
therapy. MMMTs are highly aggressive neoplasms.
Although uterine MMMT account for less than 5%
Reoccurrence occur in over half of patients after
of uterine malignancies, they are responsible for
primary surgical and adjuvant therapy. Specific
over 15% of uterine cancer-related deaths. Over the
factors that increase the risk of recurrence include
past 30 years despite evolving and advancing
patient’s age, adnexal spread, and metastasis to the
therapeutic regimes, prognosis remain poor, with
lymphnodes,
no
tumor
size,
lymphovascular
significant
improvement
in
survival
or
involvement, histologic grade, peritoneal cytologic
recurrence rate The most important prognostic
findings, & the depth of invasion of the primary
features are the stage, the size of the tumor, and the
Extension to the pelvis, lymphatic and
depth of myometrial invasion.3 The patient, in our
vascular permeation, distant lymph-borne and
case, had stage IIIA disease. The patient was
tumor.
3
2
blood – borne metastasis are all common. The
referred to Tata Memorial Hospital for further
most common site of metastatic deposit include
adjuvant therapy.
lung, peritoneum, pelvic or para-aortic, adrenal
Fig 1:Specimen of total abdominal hysterectomy
Fig 3:Photomicrograph showing
showing polypoidal tumor mass in the uterine
adenocarcinomatous&chondrosarcomatous
cavity with areas ofhaemorrhage& necrosis.
elements in MMMT (H&E 4X).
Fig 2:Specimen of total abdominal hysterectomy showing polypoidal tumor mass arising from the postero-lateral uterine wall along with tiny polypoidal masses.
Fig 4:Photomicrograph showing a papilla lined by tumor cells (H&E 40X).
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Indian Journal of Basic & Applied Medical Research; December 2013: Issue-1, Vol.-3, P.33-36
Fig 5:Photomicrograph showing foci of chondrosarcoma in MMMT(H&E 4X ).
References : 1.
Mixed Mullarian Tumor – Wikipedia, the free encyclopedia (online) Available from http:// en.wikipedia.org/wiki/mixed_mullarian_tumor (Last modified on 1st Sept 2011).
2.
Rosai J. Female Reproductive System, Uterus-Corpus Chapter 19 In: Rosai J, editor. Ackerman’s Surgical Pathology. 9th Edition. St. Louis: Mosby; 2003.1569-1635.
3.
Rani Kanthan and Jenna – Lynn Senger. Uterine Carcinosarcomas (Malignant Mixed Mullarian Tumors): A review with special emphasis on the controversies in Management. Hindawi Publishing Corporation, Obstetrics & Gynaecology International, Volume 2011, Article id 470795; 13 pages doi: 10.1155/2011/470795.
4.
Rani Kanthan and Jenna – Lynn Senger, Dana Diudea. Malignant mixed mullarian tumors of the uterus: Histopathological evaluation of cell cycle and apoptotic regulatory proteins. World Journal of Surgical Oncology 2010 , 8:60 doi:10.1186/1477-7819-8-60.
5.
Ribeiro-Silva A, Novello-Vilar A, Cunha-Mercante AM, Angelo Andrade LA. Malignant mixed mullarian tumors of the utererian cervix with neuroendocrine differentiation. Int J Gynecol Cancer 2002; 12(2): 223-227.
6.
Michael R. Hendrickson, Teri A Longacre, Richard L Kempson The uterine Corpus. Chapter 53 In: Sternberg’s Diagnostic Surgical Pathology. 4thed; 2004; 2435-2542. Date of submission: 12 September 2013
Date of Provisional acceptance: 18 September 2013
Date of Final acceptance: 24 October 2013
Date of Publication: 04 December 2013
Source of support: Nil; Conflict of Interest: Nil
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