[Parapharyngeal chondrosarcoma and Ollier's disease: a case report and review of the literature]. / Chondrosarcome para-pharyngé et maladie d'Ollier.

OBJECTIVES: Ollier's disease is a constitutional pathology of unknown etiology. It is characterized by bone dysplasia generating numerous enchondromas. The malignant degeneration of this dysplasia is well known. The aim of this article is to study the diagnostic, therapeutic and prognostic characteristics of these lesions. MATERIAL AND METHODS: We report a case of parapharyngeal chondrosarcoma extended to the base of the skull in a patient with Ollier's disease. The treatment was a surgical removal by a cervicotransoral incision combined with a preauricular incision and with a mastoidectomy. It was therefore possible to control the skull base, the parapharyngeal space, the infratemporal fossa and the major neurovascular structures. The removal of the lesion was completed at the level of the clivus and sphenoid with optics (30 and 70 degrees ). We discuss this treatment and the follow up on the bases of literature data. RESULTS: The neoplastic degeneration of enchondromas is estimated between 25 to 50% of cases. The most frequent location is the pelvic bones. Chondrosarcomas are slow growing tumors and their metastatic potential is less significant as we note it in our case report. Their diagnostic is essentially based on histological criteria's and their treatment is surgical. CONCLUSION: Chondrosarcomas of the ENT area occurring with Ollier's disease is rare. Their prognostic is good if the surgical treatment is well done.

Histological, immunohistochemical, ultrastructural and biochemical study of human gastric composite tumor: expression of the serotonin-2B receptor by the neuroendocrine component.

We report a case of a human gastric composite tumor occurring seven years after a partial gastrectomy for a low grade B cell MALT lymphoma. Histological examination of the tumor revealed two intimately intermingled components: 1. A moderately to poorly differentiated tubulo-acinar adenocarcinoma with signet-ring cells; and 2. Isolated or clustered small neuroendocrine cells without atypia expressing chromogranin A, somatostatin and/or glucagon, serotonin (5-HT) and, the 5-HT2B receptors. In addition to immunohistochemical detection, the presence of 5-HT2B receptors was shown pharmacologically through [125I]-DOI binding. Since 5-HT2B receptors have been demonstrated to have autocrine functions and, mitogenic and transforming properties, these results suggest a role of 5-HT in neuroendocrine malignant transformation. On the other hand, the expression of somatostatin and the detection by reverse transcriptase polymerase chain reaction (RT-PCR) of somatostatin receptor subtypes 2, 3, and 5, which have been shown to be involved in tumor regression, might account for the long evolution of this case (> 5 yr). This case illustrates the importance of local humoral modulation in tumor growth. Moreover, ultrastructural results favor a unique origin of the tumor cells from one amphicrine cell type.

[Rectal malignant stromal tumor (leiomyosarcoma): echoendoscopic, computed tomographic aspects and anatomopathologic correlations]. / Tumeur stromale maligne rectale (léiomyosarcome): aspects échoendoscopique, tomodensitométrique et corrélation anatomopathologique.

We report the CT and endorectal us features of a malignant stromal tumor of the rectum (leiomyosarcoma). On CT scan, rectal leiomyosarcoma appeared as a large soft tissue mass, arising from the anterior rectal wall with exorectal extension. On endorectal US, the rectal leiomyosarcoma presented as a well-defined hypoechogenic and heterogeneous mass arising from the rectal muscularis propria. The uncommon location of leiomyosarcoma to the rectum may simulate other rectal tumors such as adenocarcinoma on CT. As suggested by this report, endorectal us demonstrated the muscular origin of the tumor.

Carcinoid tumors of the abdomen: CT features.

Carcinoid tumors are rare neuroendocrine neoplasms that belong to a more general category of tumor called the APUDomas. Ninety percent of carcinoid tumors are located in the gastrointestinal tract. Abdominal carcinoid tumors are categorized according to the division of the primitive gut from which they arise. Carcinoid tumors originating from the foregut develop in the gastric wall, duodenum, and pancreas; those originating from the midgut develop from the small bowel, appendix, and right colon; and those originating from the hindgut develop from the transverse or left colon or from the rectum. This report illustrates the computed tomographic appearance of primary and metastatic carcinoid tumors of the abdomen. Among the different organs that may be involved by metastases from carcinoid tumor, special emphasis is placed on the liver.

[Sinusoidal hemangioma]. / Hémangiome sinusoïdal.

INTRODUCTION: Sinusoidal hemangioma (SH) was described by two pathologists, Calonje and Fletcher, in 1991. This uncommon cutaneous benign vascular lesions with distinctive histologic features was considered a subset of lesions known as "cavernous hemangiomas". It is made of dilated interconnecting ("sinusoidal"), thin-walled channels with a single-layered lining endothelium and a lobular architecture. PATIENTS AND METHODS: Clinical, radiological (CT, MRI and arteriography), evolutive, and histologic data of 4 patients, whose lesions appeared very similar clinically and histologically, were reviewed and compared to the patterns of the so-called SH. RESULTS: There were 2 males and 2 females. Lesions had an infantile onset in 3 (congenital in 2). They exhibited a protracted course over years in the 4, and they all invaded the right forehead, eyelids and orbit areas. Clinically, they appeared as multilobulated, building, firm tumors under a normal shiny skin. Radiologically, lesions were strikingly nodular, with criteria of slow-flow vascular lesions on MRI and arteriograms. Histologically, all lesions were comprised of well-circumscribed nodules with a lobulated architecture, made of greatly dilated, blood-filled, thin-walled vascular channels, with scanty fibrous stroma between them. Interconnecting channels had the distinctive sinusoidal pattern. There were some elongated pseudopapillary structures. Islands of spindle cells were noted in 2 lesions. DISCUSSION: On the one hand histologic features of the lesions in our 4 patients are identical to those described by Calonje and Fletcher as SH, a lesion not to be confused with classic, involuting-type, hemangioma of infancy and childhood, and with venous or lymphatic malformations of the same location. SH fits into the group of lesions defined by pathologists as "cavenous hemangiomas". On the other hand the lesions in our 4 patients differed from the clinical description of SH as a solitary acquired small (less than 3 cm) nodule in adults. Otherwise the four lesions described herein constitute a distinctive clinicopathologic entity with a striking aggressive local growth although there is a histologic malformative pattern. The presence in 2 cases of islands of spindle cells led us to discuss the differences with spindle cell hemangioendothelioma.