Hyaline Globules In Ascitic Fluid In Primary Hepatic Undifferentiated Embryonal Sarcoma

摘要

Undifferentiated Embryonal Sarcoma (UES) of the liver is a rare predominantly pediatric malignancy. Only one brief report of cytologic findings in ascitic fluid of a recurrent tumor is available. The characteristic eosinophilic hyaline globules (HG) with immunohistochemistry on fluid cytology, has not previously been documented.A 20-year-old male presented with ascites. Cytology revealed a sparse number of pleomorphic malignant cells. Sections of the cell-block preparation revealed a loose myxomatous background and numerous atypical cells with marked pleomorphism, multinucleation and many intra- and extra- cytoplsmic eosinophilic HG that were periodic acid-Schiff (PAS) positive and diastase-resistant. They displayed a positive reaction to alpha-1-antitrypsin on immunoperoxidase staining. A needle biopsy of a liver mass detected by CT scan confirmed the diagnosis of UES.Detection of sarcoma cells on fluid cytology is not often difficult. But an accurate diagnosis can be achieved only by detailed immunohistochemistry. Preparation of cell-blocks is of value in the work-up and management of patients presenting with malignant effusion. Introduction Undifferentiated embryonal sarcoma (UES) of the liver is a rare neoplasm that occurs mainly in pediatric patients, but occasionally has been reported in adults1,2,3. The tumor has a myxomatous stroma with a markedly pleomorphic cellular component and many multinucleated and bizarre cells. Numerous eosinophilic hyaline globules (HG) appearing both intra- and extracellularly are a characteristic feature of UES. The HG are PAS- positive, diastase-resistant and stain positively for alpha-1-antitrypsin by immunocytochemistry. These features can be readily recognized on FNA cytology which has an important role to play in the pre-operative diagnosis and management of these patients4,5. Allen et al has described the cytologic findings from peritoneal washings in a recurrent UES6. HG were not present in that report. A detailed description of cytomorphology of this rare entity in serous fluid cytology has not appeared in the literature. Case Report In our case the patient, a 20-year-old man, presented with ascites. The cytologic smears were blood –stained and cellularity was scant. Isolated multinucleated, bizarre cells with clear abundant cytoplasm displayed indistinct borders. Cytoplasmic vacuoles were seen in large mononuclear cells. Nuclear chromatin was coarsely clumped and nucleoli were distinct. A few cells showed smudged nuclei. Of special interest were sparse multinucleate jelly-fish-like bizarre cells with transparent cytoplasmic tentacles (Fig 1 A,B).