Clear cell sarcoma of the soft parts arising in the rectus abdominis in a child – aspiration cytology of a rare case

BACKGROUND: Clear cell sarcoma of soft parts is most commonly found associated with the tendons and aponeuroses of distal extremities in young adults with a peak incidence in the third decade. Location in the abdominal wall and in a child is very rare. CASE PRESENTATION: A nine-year-old female child presented with a swelling in the anterior abdominal wall in the suprapubic region. Fine needle aspiration revealed predominantly discrete cells with loose clustering at places. The cytoplasm was abundant, finely granular, and eosinophilic with some cells exhibiting clear vacuolated zones. No pigment was seen. The nuclei were rounded and eccentrically placed with a striking single eosinophilic macro nucleolus present in all the cells. Taking into consideration, the history, age of the patient, location of the tumor and absence of melanin pigment, a diagnosis of soft tissue sarcoma was made, the differential including Clear cell sarcoma. This was subsequently confirmed on histopathological examination and immunohistochemistry CONCLUSION: The atypical presentation of the case made the cytological diagnosis rather challenging. Clear cell sarcoma should be considered when cytology of a soft-tissue tumor shows uncharacteristically high cellularity and relatively uniform cells with macronucleoli.