CLINICAL HISTORY:
A 52-year-old postmenopausal and nulliparous woman with a familial past
medical history of gynecological cancer, presented with a four-month
history of progressive diffuse abdominal pain, and distension. Physical
examination revealed an abdominal swelling with perception of pelvic
masses. Contrast-enhanced computed tomography scan of the abdomen
revealed bilateral variegated ovarian masses with heterogeneous
enhancement (Figure 1A). The right ovarian mass measured 43x53x60 mm and
the left ovarian mass measured 75x76x89 mm. The preoperative serum level
of cancer antigen 125 (CA125) was elevated to 67 U/ml (normal range:
< 35.0), whereas the carcinoembryonic antigen (CEA) and cancer
antigen 19-9 (CA19-9) values were within the respective normal ranges.
At laparotomy, the surgeon found bilateral ovarian masses and massive
ascites. The left ovarian mass was adherent to the uterus and to the
sigmoid colon. Intraoperative frozen section analysis of both ovarian
masses concluded to a malignant neoplasm. The patient underwent a total
abdominal hysterectomy, bilateral salpingo-oophorectomy, bilateral
pelvic lymph node dissection, para-aortic lymph nodal sampling,
peritoneal biopsies, and total omentectomy.
Grossly, both ovarian masses were ill defined, lobulated and friable
with capsular rupture. The external surface showed numerous fragmented
pieces, which were soft, encephaloid, grey, glistening with areas of
hemorrhage (Figure 1B). On cut section, the tumors were solid, grey
white showing variegated appearance with areas of hemorrhage and
necrosis (Figure 1C). We did not notice residual ovarian tissue.
Moreover, there was tumor invasion on the left side of the uterine
corpus (Figure 1D). Histological examination of both ovarian masses
showed a malignant biphasic tumor proliferation. The major part of the
tumor was sarcomatous (Figure 2A) with the carcinomatous part well
differentiated serous adenocarcinoma (Figure 2C). Heterologous elements
including cartilage (Figure 2B) and adipose tissue (Figure 2 D) were
found in the tumor. Mitotic activity was frequent (8-9/10 high power
field). We also noted the presence of tumor necrosis.
Immunohistochemical study showed positive immunostaining of the
sarcomatous component with Demin, Smooth Muscle Actin and Calponin.
Cytokeratin highlighted the epithelial component. Omentum, lymph nodes
and peritoneal biopsy were negative for malignant cells. Cytological
examination of the ascitic fluid did not reveal the presence of
malignant cells. Based on the histopathological and immunohistochemical
findings, the final diagnosis was that of bilateral ovarian
carcinosarcoma classified as stage IIA according to the International
Federation of Gynecology and Obstetrics (FIGO) 2014. The postoperative
course was uneventful. The patient was planned for adjuvant
chemotherapy: six cycles of combination with paclitaxel and carboplatin.
At present, she is still being followed-up.