Angiosarcoma originating from an ovarian mature teratoma, a rare disease with complex treatment modalities
A 64 year-old female with a history of type 2 diabetes mellitus, hypertension, and vaginal hysterectomy for uterine leiomyomas 30 years ago, presented to the emergency room with acute abdominal pain and fever. She had lower abdominal pain and bloating for the last 2 months with decreased appetite, nausea, and a 22-pound weight loss.\nOn examination, the patient had a palpable abdominal mass with diffuse rebound tenderness and rigidity. Computed tomography (CT) scan showed a complex pelvic mass measuring 19.8 × 17.8 × 14.5 cm invading and perforating the sigmoid colon ().\nExploratory laparotomy showed infected ascites from a sigmoid colon perforation. This mass also extended to the right ovary and invaded the terminal ileum and the cecum. Optimal debulking to no visible disease was performed with bilateral salpingo-oophorectomy, resection of the terminal ileum and cecum with side-to-side anastomosis, resection of sigmoid and upper rectum, and a formation of a permanent descending end colostomy. The patient was discharged home after four days of inpatient hospitalization.\nPathologic evaluation of the mass showed angiosarcoma involving bilateral ovaries with extensive necrosis associated with the cystic teratoma of the left ovary (). Microscopic examination revealed atypical cells, varying from spindled to an epithelioid character with abundant cytoplasm, and large hyperchromatic, pleomorphic nuclei. The tumor showed solid, sheet-like areas and vascular spaces. Immunohistochemisty of the tumor showed intense staining for CD31 and vimentin, reacted weakly for FLI-1, but had lost expression of CD34 and factor VIII related antigen. Additional staining for CD117, DOG-1, S100, desmin, muscle-specific actin, myogenin, melanoma cocktail, and cytokeratin AE1/AE3 were negative. The tumor additionally involved the left peri-fallopian soft tissue, pelvic soft tissue, and right peri-adnexal tissue. Metastatic foci involved full thickness of the sigmoid colon wall, terminal ileum, and one regional lymph node.\nAfter extensive counseling and given the extent of the disease, the decision was made to start adjuvant chemotherapy to be followed by radiotherapy (RT). Three weeks after recovering from her surgery, the patient received intravenous paclitaxel (175 mg/m2 = 362 mg) to be scheduled every 3 weeks. More studies have looked in to the use of paclitaxel as a single agent in sarcoma literature rather than combination chemotherapy. Considering this and the patient's medical co-morbidities and performance status, single agent paclitaxel was used as an adjuvant treatment. However, she was readmitted 2 weeks later with a recurrence of her original symptoms and CT scan showing recurrence of her cancer to the pelvis and upper abdomen. With a rapid clinical deterioration, the patient developed multi-organ failure and elected to be at home with hospice care. She passed away 2 weeks later—8 weeks after initial diagnosis and 4 months after her symptoms began.