Lipofibromatosis: A Rare Diagnosis on Fine Needle Aspiration Cytology

Lipofibromatosis is a recently recognized slow growing rare pediatric tumor. Paucity of its cytological description in the literature leads to its pre operative misdiagnosis and further incomplete management. A twelve-month-old female presented with a rapidly progressive mass in the right thigh and buttock region. On examination, the mass was huge and involved the medial, posterior and lateral aspects of the thigh. The cytological smears showed mature adipocytes with few spindled out cells. FNA was reported as a lipoma, corroborating with the radiological presumptive diagnosis. However, histopathological and immunohistochemical features favoured a diagnosis of Lipofibromatosis. The cytological smears were reviewed and a cyto-histo correlation was established. The diagnosis of Lipofibromatosis rests upon classical cytological features in a clinically and radiologically suggestive picture. An early and accurate diagnosis if established can help the surgeon plan excision with wider margins as incomplete excision is associated with a high rate of recurrence.