Orbital tumor revealing a systemic sarcoidosis.

Ocular involvement is seen in approximately 25% of patients with sarcoidosis. Uveitis is the most common ocular manifestation, but sarcoidosis may involve any part of the eye. Orbital manifestations of sarcoidosis are uncommon with few series in the literature. A 65-year-old woman presented with redness of the right eye and painless, unilateral eyelid swelling. Orbital scanning revealed mass infiltrating the soft tissue of the inferior right orbital quadrant. Biopsy results showed nodular, noncaseating granulomas consistent with sarcoidosis. The complete systemic workup revealed systemic manifestations of sarcoidosis at the time of examination with hilar and mediastinal lymphadenopathies noted on CT scan. The orbital surgical treatment was followed by systemic prednisone therapy with good response. Although rare, orbital sarcoidosis must be considered in the evaluation of orbital tumors in elderly patients. A search for systemic findings should be undertaken and appropriate therapy should be instituted.

[Cholestasis caused by a choledochal botryoid rhabdomyosarcoma in a 22-month-old boy]. / Une choléstase causée par un rhabdomyosarcome botryoïde du cholédoque chez un nourrisson de 22 mois.

Botrioid rhabdomyosarcoma of the extrahepatic bile ducts is a rare cause of jaundice in children. It has a very poor prognosis and is rarely diagnosed preoperatively. We report a choledochal botrioid rhabdomyosarcoma in a 22-month-old boy who developed an obstructive jaundice. Radiographic explorations suggested cystic lymphangioma. The gallbladder, the cystic duct, the common bile duct and the pancreatic head were resected. The diagnosis was made on pathological examination; adjuvant chemotherapy followed. The patient was disease free 20 months following treatment.

[Extra-nodal Rosai-Dorfman disease: a case report with thyroid involvement]. / La maladie de Rosai-Dorfman extra-ganglionnaire: à propos d'une localisation thyroïdienne.

Extra nodal involvement by Rosaï-Dorfman disease (RDD) is not rare but remains poorly described clinically and microscopically. We report a case of RDD involving the thyroid and revealed by a 15 mm cold nodule developed on an ancient goiter. The patient was a 53 year old North African woman. She also presented an involvement of cervical lymph nodes, respiratory tract and right kidney. Microscopic analysis of thyroid revealed a diffuse and dense infiltrate of large pale histiocytes with few features of lymphophagocytosis. To the best of our knowledge, this is the fifth case reported in the literature. In previous reported cases, thyroid RDD was associated to constant lymph node involvement (massive lymphadenopathy in 3 cases and occult in one case) and no other extra nodal localization except the respiratory tract in one case. The characteristic features of lymphophagocytosis must be searched with great care.