Ovarian Carcinosarcoma with Malignant Neuroectodermal Components (Teratoid Carcinosarcoma) - A Rare Entity

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Abstract Introduction/Objective Ovarian carcinosarcoma is an aggressive tumor accounting for 2% of all ovarian malignancies. Ovarian carcinosarcoma with malignant neuroectodermal components is exceedingly rare and only four cases have been previously reported. Here we report a series of three cases in 65(case 1), 61 (case 2) and 72 (case 3) year old females. Methods All patients presented with pelvic mass and elevated CA125. They underwent hysterectomy, bilateral salpingo-oophorectomy, omentectomy and tumor-debulking, along with lymph node dissection (2,3) and appendectomy (3). The FIGO stages were IIIC (1,3) or IIA (2). Case #2 experienced pelvic recurrence three months after initiating platinum based chemotherapy and was treated with BEP. She died of metastatic disease ten months after initial surgery. Case #3 is a current case and is being treated with chemotherapy. Case #1 was an outside consult and was lost to follow–up. Results Grossly the dominant ovarian mass measured 25 (left), 20 (left) and 21 (right) cm. The cut surfaces were hetergenous tan, yellow, partially cystic and solid with hemorrhage and necrosis. Microscopically, they showed mixed morphology of carcinomatous, sarcomatous, and undifferentiated neuroectodermal component. The carcinomatous element showed squamous cell carcinoma and adenocarcinoma. The sarcomatous component included rhabdomyosarcoma, chondrosarcoma and liposarcoma. The undifferentiated neuroectodermal component consisted of nested or solid primitive cells. All three cases showed fetal-appearing teratoid squamous cell epithelium with clear cytoplasm. The contralateral ovary and extraovarian metastatic implants were either carcinosarcoma, carcinoma or contained all three components. Conclusion Case #1 and 3 also showed a yolk sac component. All lymph nodes (2,3) were negative for metastatic tumor at the time of initial surgery. Case #2 had first recurrence in retroperitoneal lymph node showing mature neural tissue. Her second recurrence in abdomen was undifferentiated neuroblastoma. Immunohistochemical studies included CK7, CK20, CDX2, NapsinA, S100, GFAP, PAX-8, CK5/6, P63, AE1/AE3, desmin, Myf-4, CD56, glypican3, AFB, CD99, chromogranin, synaptophysin, NF, NeuN, calretinin, and inhibin. The staining pattern was consistent with histologic interpretation. These cases histologically resemble to so-called “teratoid carcinosarcoma” of paranasal sinuses. The histogenesis remains unknown.