A case of undifferentiated (embryonal) sarcoma of the liver: fine needle aspiration cytology diagnosis by one cell type

We present the first case in Bulgaria of cytologically verified undifferentiated (embryonal) sarcoma of the liver. A 10-year-old girl was admitted to the hospital with abdominal pain, extreme hepatomegaly, one-month fever, anemia and highly elevated erythrocyte sedimentation rate (95 mm). A large formation was found in the right liver lobe on ultrasound examination, which was polycystic on CT scan. Neither bone marrow abnormalities nor elevation of serum alpha-fetoprotein was found. In the scanty specimen obtained by fine-needle aspiration biopsy under ultrasound control a characteristic prevalence of polygonal cells within mesenchyme tissue was found. The cells had eccentric hyperchromic nuclei which were pushed to the periphery by large clusters of PAS-positive, diastase-resistant granules. Vimentin and alpha 1-antitrypsyn were expressed immunohistochemically. The cells are pathognomonic of undifferentiated (embryonal) sarcoma of the liver. After preoperative chemotherapy with ifosfamide, vincristine and actinomycin D (CEVAIE, CWS--96) resection of the tumor including IV, V, VI and VII liver segments was performed. A 30 x 30-cm soft gray-whitish tumor with fine capsule was found. The cytological diagnosis was histologically verified. The patient had a mechanical subileus episode after the 6th course of chemotherapy. One year after the surgery she is in good condition. The histogenesis of this rare tumor is discussed regarding its unique cytology, immunohistochemical constellation and ultrastructure. A literature review is presented. The case indicates that the unique cell appearance in embryonal sarcoma of the liver provides a correct diagnosis even in a scanty specimen.