Lymphangioleiomyomatosis - Retroperitoneum.

Lymphangioleiomyomatosis (LAM) is a progressive disease affecting women of childbearing age. It is microscopically characterized by abnormal smooth muscle proliferation in the walls of lymphatics and lymph nodes. A 52-year old female presented with pain in left leg and bilateral pedal oedema. USG and CT abdomen revealed a retroperitoneal cystic mass suspicious of malignancy. Retroperitoneal exploration was done and the mass was excised which revealed milky contents within. On gross examination of the specimen, a spongy mass was noted . Histopathological examination led to a diagnosis of lymphangioleiomyomatosis. LAM is a rare disease unfamiliar to many physicians and may pose a diagnostic dilemma to the physician, possibly resulting in delayed or missed diagnosis . Many treatment modalities including corticosteroids, cytotoxic drugs, chemotherapy, radiation and hormonal therapy are suggested. The prognosis of LAM varies with the individual, but many patients respond well to intramuscular medroxyprogesterone injections.

Cytological Diagnosis of Ewing Sarcoma – Clavicle.

Ewing sarcoma is a rare malignant round cell tumor of the bone. It is the second most common primary malignant tumor of bone found in children. It commonly affects long bones, pelvis and ribs. An 11 year old boy presented with painful swelling at the medial end of left clavicle. X-ray revealed a diaphyseal lesion of the medial end of the left clavicle with destruction of bone and mottled appearance. FNAC was done and reported as Malignant round cell tumor suggestive of Ewing sarcoma. Histopathologically the diagnosis of Ewing sarcoma was confirmed with special stains. Ewing sarcoma affecting clavicle is uncommon. Clavicle can be often resected with no need of reconstruction in young children.