Virilizing adrenal ganglioneuroma containing Leydig cells.

An adrenal medullary ganglioneuroma containing Leydig cells and revealed by a virilizing syndrome in a 56-year old woman is presented. The syndrome, associating with masculinization an elevated serum testosterone level and a normal urinary 17 ketosteroids, is uncommon in adrenal tumours. The tumour was located by computed tomographic (CT) scan and treated by right adrenalectomy. Microscopic examination showed typical features of an adrenal medullary ganglioneuroma containing Leydig cells with Reinke crystalloids. Positive immunohistochemical study confirmed the testosterone secreting nature of the cells. The presence of Leydig cells in the adrenal gland is discussed according embryogenic studies: 1. the common origin of the gonad and the adrenal cortex from the coelomic epithelium, 2. the vicinity of adrenocortical gland and gonad during embryogenesis, 3. the thecal metaplasia of mesenchymal cells, 4. the development of Leydig cells from Schwann cells. Fourth case published in the world literature.

[EC-cell pancreatic endocrine carcinoma responsible for chronic diarrhea]. / Carcinome endocrine pancréatique à cellules EC (carcinoïde) responsable d'une diarrhée chronique.

Ec cell or carcinoid enterochromaffinomas are very rare among the endocrine tumors, and more specifically orthocrine tumors, of the pancreas. We report one case of primary malignant carcinoid tumor of the pancreas, with no apparent metastasis, discovered during exploration for chronic diarrhea associated with hypercalcemia, disorders of carbohydrate metabolism and a serotonin secretion. The treatment consisted in a left splenopancreatectomy associated with a left adrenectomy. The clinical and biological signs immediately disappeared and, after a time lapse of almost five years, the general condition of this patient is quite satisfactory.

[A malignant schwannoma of the jejunum associated with multiple neurofibromas and a primary adenoma of the parathyroid]. / Schwannome malin du jéjunum associé à un neurofibrome pleural et à un adénome primitif de la parathyroïde.

A case of schwannosarcoma associated with a pleural neurofibroma and a parathyroid adenoma is presented. The neuroectodermal origin and the relationship with neurofibromatosis are discussed. The sarcomatous relapse of a schwannoma previously classified as benign confirms the difficulty of microscopic analysis and the malignancy power of the tumor. A long survival is obtained by a large intestinal and mesenteric resection as long as hepatic metastases are not present or can be resected. The pleural neurofibroma needs symptomatic treatment while parathyroid adenoma must be early removed as soon as malignant hypercalcemia is detected.

[Non-functional or silent endocrine tumors of the pancreas. Apropos of a case of cystic form]. / Les tumeurs endocrines "non fonctionnelles" ou silencieuses du pancréas. A propos d'une observation à forme kystique.

The authors report a new case of silent cystic pancreatic endocrine tumour discovered by chance on abdominal ultrasonography. This tumour was treated surgically by simple enucleation-resection. On the basis of the histological appearance and the absence of metastases, this tumour was considered to be benign, but only the long-term course will confirm the diagnosis.