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Malaysian Journal of Medical Sciences
School of Medical Sciences, Universiti Sains Malaysia
ISSN: 1394-195X
Vol. 10, Num. 2, 2003, pp. 93-95
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Malaysian Journal of Medical Sciences, Vol. 10, No. 2, July 2003, pp. 93-95
CASE REPORT
Malignant Mixed Mullerian Tumour, Heterologous in a 66-year-old Malay Lady
- a case report.
Md.Tahminur Rahman, Venkatesh R. Naik, Shah Reza Johan Noor*,Nik Mohamed Zaki
Nik Mahmud*, Mohd. Isa *
Department of Pathology and Gynecology*, School of Medical Sciences,
Universiti Sains Malaysia, Health Campus
16150 Kubang Kerian, Kelantan, Malaysia
Correspondence : Dr. Md.Tahminur Rahman MBBS (Dhaka), M.Phil. Path
(Dhaka), Department of Pathology, School of Medical Sciences, Universiti Sains
Malaysia, Health Campus, 16150 Kubang Kerian, Kelantan, Malaysia, Email : tahminur@kb.usm.my
Submitted-30.7.2002,
Revised-11.6.2003,
Accepted-15.6.2003
Code Number: mj03030
A 66-year-old Malay woman, known hypertensive, presented
with post menopausal bleeding associated with clot for three months. She
was postmenopausal for last ten years. She also complaints of developing
a mass in the abdomen which was growing in size also for last three months.
Abdominal examination revealed a twenty week size mass, movable from side
to side but unable to get below the mass. Vaginal examination revealed a
fleshy fungating mass arising from the uterus coming out through the vagina.
Cervix could not be visualized properly. Subsequent histopathology of the
removed mass was reported as a Malignant Mixed Mullerian Tumour - Heterologous.
Key words : Malignant Mixed Mullerian Tumour,
Heterologous.
INTRODUCTION
Malignant Mixed Mullaerian Tumour is a rare uterine neoplasm
that is seen always in postmenopausal woman although exceptions occur (1).
They present with uterine bleeding and enlargement and the usual location is
uterine body. Grossly they present as large, soft, polypoid growth involving
the endomyometrium and some times protruding through the cervix. Here we present
an interesting case of Malignant Mixed Mullerian Tumor in a 66 year old Malayu
Lady.
Case History
The patient was a 66 year old Malay Woman who was a known
hypertensive and was on Nifedipine 15mg TDS. She was admitted in the USM hospital
on 22nd June 2002 with the complaints of postmenopausal bleeding associated
with clot for last 3 months. She also complained of an abdominal mass increasing
in size for the same three months. She had foul smelling vaginal discharge
and also had history of loss of appetite and loss of weight.
Menstrual history
She attained menarche at the age of 13 years and had regular
cycle for 7 days flow with 30 days cycle. She never had Pap's smear test. She
never used any contraceptive device or pill before. She is still sexually active.
Obstetric history
Para 8. All of the deliveries were full term spontaneous vaginal
deliveries except the last childbirth, which was a Caesarian section with bilateral
tubal ligation. Physical examination revealed she was moderate built, had mild
pallor and not cachectic. Her blood pressure was 200/100 mm Hg., temperature
38º C, CVS and RS were normal. Per abdomen examination showed a mass 20
weeks in size, firm, mobile from side to side, unable to get below the mass.
There was no ascitis. Per vaginal examination revealed atrophic vagina and
no other gross abnormality was detected in the vulvovagina area. An irregular
fleshy fungating mass, arising from the uterus was seen. There was contact
bleeding and foul smelling discharge. The Cervix could not be visualized. The
adnexae and
pouch of Douglus were normal. Trans
abdominal scan showed uterus grossly enlarged with
discrete mass and areas of calcification. The
clinical impression was an infected degenerative fibroid
and to rule out endometrial carcinoma. She was
treated with antihypertensive, I.V antibiotics (Zinacef+Gentamicin+Flagyl). Biopsy
was done and the report came as infarcted malignant tumour. Subsequently she
was planned for total
hysterectomy with bilateral salpingo- oophorectomy
(TAHBSO) and the operation was done on 26/06/02.
The specimen was sent for histopathological examination.
Pathological Examination:
Gross examination:
Specimen consists of a total abdominal hysterectomy with bilateral
salpingo- oophorectomy specimen (TAHBSO) which was sent already cut open and
completely distorted measuring 110X120X60mm. Right fallopian tube measured
50X10mm, Right ovary measured 25X15X12mm. Left fallopian tube measured 50X10mm
and the left ovary measured 20X15X5mm. The whole specimen weighed 900gm. The
endometrial cavity was filled with grey white necrotic friable tumour. Myometrium
measured 15mm in thickness. The tumor appeared to be confined to the endometrial
cavity grossly. The cervix looks normal grossly. Seven blocks one each from
the anterior and posterior lip of cervix, right ovary and tube, left ovary
and tube and three blocks from the tumour with myometrium, one from the tumour
proper were embedded.
Microscopic examination:
Multiples sections show a tumour in the uterine cavity. The
tumour is an admixture of both carcinomatous and sarcomatous elements.(Fig-1).
The carcinomatous element is composed of moderate to poorly differentiated
endometrial glands of endometroid type. In few foci squamous metaplasia are
also seen. The sarcomatous component is made up of anaplastic spindle shaped
cells and large plump cells having large hyperchromatic nuclei with prominent
nucleoli and moderate amount of pink cytoplasm. Some large myoblasts exhibiting
cross striations were seen. In few areas the stroma is myxoid. Mitotic figures
are
more than 15/ HPF in some foci.(Fig2)The
tumour has involved 3/4th of the myometrium wall. Extensive areas of necrosis
are seen. Sections from the cervix show chronic cervicitis. Sections
from both ovaries and tubes are within normal
limit. Special stains (IHC) for Cytokeratin,
Vimentin, Desmin and Myoglobulin (Fig-3) were positive.
Dx: Malignant Mixed Mullaerian Tumour, heterologous.
DISCUSSION
Our patient presented with postmenopausal bleeding with passage
of clot for last three months and also gradual abdominal swelling for the same
periods. She also complained of foul smelling discharge from vagina. These
all coincides with the usual presentation of Malignant Mixed Mullerian Tumour.
Sarcomas collectively made up about 5% or less of uterine tumours, the commonest
variant being mixed mesodermal tumors, leiomyosarcomas, endometrial stromal
sarcomas (2). Malignant mixed Mullerian tumor is called carcinosarcomas because
they consists of both glandular and stromal elements. The present case also
showed carcinosarcoma in histopathology. The stromal components may differentiate
into a variety of malignant mesodermal components like muscle, cartilage and
bone ; malignant mixed Mullerian tumors are highly aggressive. The case we
report here also showed rhabdomyoblasts in light microscopy where cross striation
was easily identifiable and Immunohistochemistry(IHC) showed positivity for
myoglobulin and also for Desmin and Vimentin. In recent years convincing evidence
suggested that most not all are monoclonal in origin rather than true collision
tumors (3). Various data confirms that the sarcomatous component is derived
from the carcinoma or from a stem cell that undergoes divergent differentiation.
Thus uterine carcinosarcomas are best regarded as metaplastic carcinomas. Although
Malignant Mixed Mullerian tumours are rare neoplasm's occurring mostly in uterus,
rare cases of malignant mixed mullerian tumours have also been noted in the
cervix (4,5,6), and the omentum (7). The lesion in the cervix was a poorly
differentiated lesion composed of poorly differentiated epithelial component
(cytokeratin positive), and a spindle cell component (vimentin positive) with
heterologous (myeoblastic) differentiation. Also there was a report of primary
peritoneal Mullerian adenosarcoma with sarcomatous overgrowth associated with
endometriosis in a 50-year-old female (8).
Histologically the tumor was composed of benign mullerian
glands and a sarcomatous stroma. Multiple foci of endometriosis were associated
with pelvic mass. Both estrogen and progesterone receptors differ in function
and expression with different neoplastic states. Recent studies has shown that
Malignant Mixed Mullerian Tumours and Endometrial adenocarcinomas exhibit decreased
ER alpha expression and significant loss of PR protein (9). We also did the
ER & PR staining on the histological slides and these were negative. A
recent study which studied clinical and pathological analysis of 106 cases
(10) of uterine sarcoma showed that the malignant mixed mullerian tumour presented
in 15.1% of the total cases, while the predominant sarcoma was leiomyosarcoma
(63.2%), malignant endometrial interstitial sarcomas (21.7%). They noticed
that the patients with leiomyosarcoma and malignant endometrial stromal sarcoma
were younger and less than 50 years of age (70.1% and 60.9% respectively),
while most of the malignant mixed mullerian tumour patient were above 50 years
of age. The presenting complaints of these patients were abnormal vaginal bleeding
(67.0%), palpable lower abdominal mass (32.1%), vaginal discharge (27.4%),
and discomfort feeling (28.3%). They concluded that the clinical symptoms of
uterine sarcoma are non specific (mostly abnormal vaginal bleeding) and the
prognosis was poor. The prognosis of uterine sarcoma is related to histopathological
sub types, clinical stages and age of the patient. The mainstay of treatment
is surgery with
adjuvant chemotherapy and radiotherapy.
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Copyright 2003 - Malaysian Journal of Medical Science
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