RESUMO
Xanthoma disseminatum (XD) is a rare and potentially progressive non-Langerhans-cell histiocytosis. To date, a few cases of XD with spontaneous complete resolution have been described. The present report describes a 16-year-old girl who presented with yellow to red-brown papules and nodules on her eyelids, cheeks, axillae, back and buttocks. Indirect laryngoscopy showed multiple xanthomatous plaques on the larynx, posterior pharynx, epiglottis, and vocal cords. Additional findings were polyuria, polydipsia, and amenorrhea. Skin biopsy and electron microscopy results confirmed the diagnosis of XD. The patient was treated with fenofibrate, simvastatin, desmopressin, and sex-hormone replacement therapy. Her skin lesions began to slowly fade 6 years after disease onset, eventually resolving spontaneously and completely, but leaving an atrophic scar, frank anetoderma, and persisting diabetes insipidus. This case report together with a review of the English-language literature on the long-term follow-up of XD patients provides additional information on the natural history of this disease.
Assuntos
Histiocitose de Células não Langerhans/diagnóstico , Adolescente , Amenorreia/diagnóstico , Amenorreia/tratamento farmacológico , Anetodermia/diagnóstico , Anetodermia/tratamento farmacológico , Antidiuréticos/uso terapêutico , Biópsia , Cicatriz/patologia , Desamino Arginina Vasopressina/uso terapêutico , Fármacos Dermatológicos/uso terapêutico , Diabetes Insípido/diagnóstico , Diabetes Insípido/tratamento farmacológico , Feminino , Fenofibrato/uso terapêutico , Gadolínio DTPA , Histiocitose de Células não Langerhans/diagnóstico por imagem , Histiocitose de Células não Langerhans/tratamento farmacológico , Histiocitose de Células não Langerhans/patologia , Terapia de Reposição Hormonal , Humanos , Cintilografia , Remissão Espontânea , Sinvastatina/uso terapêuticoRESUMO
Leiomyosarcoma is a rare tumor that originates in the smooth muscle, usually in the gastrointestinal tract, the retroperitoneum and the genitourinary tract. The omentum has been rarely reported as a primary site of occurrence with leiomyosarcoma. A 72 year-old woman, with palpable mass on the left upper quadrant of abdomen and weight loss, was admitted to St. Paul's Hospital. Abdominal CT scan showed a 12 x 8 x 8 cm-sized cystic mass in the left upper quadrant of the abdomen. Endoscopic ultrasonography showed a large cystic mass between the stomach and the liver, which was 1.6 cm length in wall thickness. Laparotomy and resection of the mass was performed. A 12 x 8 x 8 cm-sized mass, originated from the lesser omentum, was discovered near the lesser curvature of the stomach. Microscopic examination revealed spindle-shaped cells with 7-8 mitoses per high power field. She was diagnosed as primary leiomyosarcoma originated from the lesser omentum.
