A 68 year old lady was referred to oncology outpatients in January 2010, with a month history of severe abdominal pain. Her past clinical history included no tubal ligation or hormone replacement therapy. She had previously given birth to a female child at her age of 21. The patient was fit and well with no significant past medical history apart from hypertension and diabetes. There was no family history of breast or ovarian carcinoma.
Physical examination revealed an abdominal pelvic mass with ascites and omental deposits. Blood analysis showed haemoglobin concentration of 11.3 g/dl while the rest of the analysis were normal including the carcinoembryonic antigen (CEA), alphafetoprotein and Cancer Antigen-125 (CA-125) (1.25 U/ml). The cytological examination of the ascitic fluid showed cellular smear composing of mixed inflammatory cells admixed with papillary and acinar clusters of eosinophilic cells with pleomorphic hyperchromatic nuclei thus suggesting a metastatic carcinoma.
Pelvic sonogram revealed a large tumour mass with solid and cystic components. Minimal ascites was noted. A subsequent computerized tomography (CT) scan of the abdomen and pelvis revealed heterogeneously enhancing mass lesion measuring 9 × 7.1 cm with solid and cystic areas and calcification in the retrovesical region (Figure 1A). The mass was found to be compressing on the right lower ureter leading to right hydrodeuteronephrosis. Multiple enlarged peritoneal nodules with a largest one measuring 10 × 6.3 cm were observed. Moderate free fluid in abdomen and pelvis with moderate right pleural effusion was observed. The diagnosis of the malignant transformation was suggested by the invasive growth of soft tissue components through the teratoma wall by CT scan images. Finally based on the clinical manifestations she was diagnosed as having FIGO stage IIIC of immature teratoma.
Due to unresectable bulky tumours and poor performance status the patient underwent neoadjuvant chemotherapy (NAC) followed by Interval Cytoreductive Surgery (ICS). Four cycles of combination of paclitaxel and carboplatin were administered every 3 weeks. ICS was performed in the 5th week after administration of the 4th cycle of NAC. Standard procedures of ICS consisting of total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy and maximal debulking of metastatic tumour was adopted. Following the procedure there was no residual macroscopic disease and she was transferred to high dependency.
The surface of the tumour appeared rough and congested. On cutting, the ovarian mass was full of sebaceous material and hair densely adherent to surrounding structures. Histopathological examination confirmed bilateral teratoma complicated with carcinosarcoma (Figure 1B) with heterogeneous rhabdoid elements (Figure 1C) Microscopically the left ovarian tumour displayed variable size cyst lined by multilayered malignant squamous cells (Figure 1D) with rhabdoid spindle cells, cytoplasmic clearing, mature atypical cartilage (Figure 1E), malignant tubules, small round cells with rosettes, bone marrow and neural bundle. In addition the focal area showed atypical giant bizarre cells. The observation of the right ovarian tumour displayed admixture of malignant, epithelial and mesenchymal elements. The epithelial layers showed variable sized islands of squamoid and polygonal spindle cells and rarely showed tubular papillary structure. The stroma appeared to be a mixture of rhabdoid spindle cells, primitive mesenchymal cells, neural elements, adipocytic elements. Focal area showed pigmented cells.
The patient recovered well from surgery and was referred for oncological follow up and post- surgical chemotherapy (same regime as NAC). Given her age and performance status a surveillance approach was taken with regular clinical examinations, serial tumour markers and routine CT scans. The follow up studies showed no evidence of recurrence, regional or distant metastasis.
