[Sarcoidosis presenting as a splenic pseudotumour]. / Rate pseudotumorale révélatrice de sarcoïdose.

INTRODUCTION: Multinodular splenomegaly is a rare form of extrathoracic sarcoidosis. It may be the presenting feature of the disease. It poses problems of differential diagnosis, in particular with lymphoma, tuberculosis and other granulomatous diseases. In the absence of typical associated lesions, particularly thoracic, histological roof is essential. CASE REPORT: We report the case of a 55-year-old caucasian woman with multinodular pseudotumoural splenomegaly, associated with some small mediastinal lymph nodes and some non-specific parenchymatous pulmonary nodules. The diagnosis of sarcoidosis was made on the basis of splenectomy after eliminating other causes of granulomatosis. CONCLUSION: This case report describes a rare presentation of sarcoidosis and discusses the differential diagnosis of multinodular splenomegaly. It underlines the necessity of an exhaustive aetiological investigation of splenic granulomatosis as the diagnosis of sarcoidosis remains one of elimination.

[Perianal basal cell carcinoma]. / Carcinome basocellulaire périanal.

INTRODUCTION: The perianal localisation of a basal cell carcinoma is exceptional. Its long term progression and the modalities for its treatment should be known. OBSERVATION: A 66 Year-old man was referred for a non-regressive haemorrhoidal thrombosis. He presented with a painless but hemorrhagic lesion of the anal-cutaneous junction that had not progressed much over 5 Years but which did not heal. Following exeresis and biopsy, the anatomopathological exploration concluded in an ulcerous-nodular basal cell carcinoma. COMMENTS: The treatment of perianal basal cell carcinomas consists in the local exeresis of the tumour. Of good prognosis, the possibility of a local relapse however requires strict follow-up.

Intravascular large B-cell lymphoma with bone marrow involvement at presentation and haemophagocytic syndrome: two Western cases in favour of a specific variant.

AIMS: To report two cases of an unusual form of intravascular lymphoma, characterized by bone marrow involvement at presentation with haemophagocytic syndrome. METHODS AND RESULTS: We describe the clinicopathological features of two patients with intravascular lymphoma primarily involving bone marrow. Both patients complained only of fever with pancytopenia and reactive haemophagocytic syndrome. Diagnosis was made on bone marrow examination, which showed large tumour cells of B-cell lineage confined within the lumen of sinuses. CONCLUSION: These two cases and five previous reports could represent a variant of intravascular lymphoma, characterized by early involvement of bone marrow without dissemination to other organs. This form of intravascular lymphoma, called IVL-HS, seems to be an 'Asian' variant with a high prevalence in Asian people and a very low prevalence in Western countries. At a practical level, bone marrow biopsy may be useful in the diagnosis of intravascular lymphoma when the clinical presentation is restricted to fever of unknown origin with a reactive haemophagocytic syndrome.

[Non-Hodgkin lymphoma mimicking renal carcinoma: apropos of 1 case of follicular lymphoma]. / Lymphome non hodgkinien simulant un carcinome rénal. A propos d'un cas de lymphome folliculaire.

Primary renal non-Hodgkin's lymphoma (NHL) is unusual, in contrast to the frequent renal involvement in disseminated NHL. We report a case of follicular lymphoma presenting initially as a renal mass. In the literature, twenty-seven similar cases have been described since 1980. The median age at diagnosis is 64 years with a male predominance. Clinical and radiological findings generally evoke renal carcinoma. Histologically, tumors are usually large B-cell lymphomas. The existence of renal non-Hodgkin lymphoma mimicking renal carcinoma must be recognized. Such lymphomas can either be primitive or be nodal with a renal presentation. Nephrectomy followed by chemotherapy permits long disease free survival.

Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome.

AIMS: Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure. METHODS AND RESULTS: We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor VIII related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis. CONCLUSION: If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.

[Multiple trichodiscomas associated with colonic polyposis]. / Trichodiscomes multiples associés à une polypose colique.

BACKGROUND: Trichodiscoma is an uncommon benign tumor of the pilosebaceous system. CASE REPORT: An 83-year-old man had a past history of adenomatous polyps of the colon. The subject's brother had died from cancer of the colon. Over the past 4 years, the patient had developed approximately 30 small asymptomatic papulonodular tumors on the trunk. Skin biopsy demonstrated trichodiscomas. DISCUSSION: Together with fibrofolliculomas and perifollicular fibromas, trichodiscomas are benign tumors of the pilosebaceous system. These different tumors may occur simultaneously as in Birt-Hogg-Dubé syndrome which associates trichodiscoma and familial colonic polyposis. This combination has only been reported in Birt-Hogg-Dubé syndrome or with perifollicular fibromas. CONCLUSION: This case suggests that patients with hamartomas of the pilosebaceous system should undergo explorations in search for digestive tract polyposis.

[Lymphoepithelial tumor of the skin]. / Tumeur lympho-épithéliale cutanée.

The authors report an additional case of lympho-epithelial tumor. First described in 1987 by Santa-Cruz et al., this tumor, also called cutaneous lymphadenoma, is rare. The mean age is 44. The lesion is generally located on the face and the clinical diagnosis of basal cell carcinoma is most often evoked. The tumor has a peculiar histological feature with cyst-like cavities composed by an epithelial proliferation and intraepithelial lympho-histiocytic cells. It is considered as a benign tumor. Its origin is still discussed but a pilosebaceous nature seems to be accepted.

[Endobronchial granular cell tumor. A report of two cases in the course of staging of ORL cancers]. / Tumeur à cellules granuleuses endobronchique. A propos de deux cas de découverte systématique dans le cadre d'une surveillance de cancers ORL.

We report two new observations of endobronchial granular cell tumor discovering by bronchoscopy, during the staging of head and neck cancers. This site remains exceptional; the histologic features are well known. The ultrastructural and especially the immunohistological criteria trend to indicate a neurogenic histogenesis. Although the course is benign in this site and no malignant forms have been reported, local relapses are possible. However, some benign cases may have disturbing endoscopic and histologic features.

[Phrenic nerve malignant schwannoma: an exceptional diagnosis]. / Schwannome malin du phrénique: un diagnostic d'exception.

We report a case of malignant schwannoma of left phrenic nerve in a 65-year-old man. This uncommon diagnosis was suggested by radiological features and confirmed by histological findings on a needle biopsy and surgery specimens. We discuss management, follow-up and prognosis of malignant schwannomas.

[Retiform hemangioendothelioma]. / Hémangioendothéliome rétiforme.

Hemangioendotheliomas are vascular tumors of intermediate malignancy considered as low-grade angiosarcomas. The authors report two cases of a distinctive type, named retiform hemangioendothelioma because of its particular morphologic features, and recently delineated by Calonje and al. This tumor of the skin recurs frequently but has a very low metastatic rate.

[Efficacy of azidothymidine in thrombopenia associated with HIV infection]. / Efficacité de l'azidothymidine sur la thrombopénie associée à l'infection par le virus HIV.

We report an observation where a patient suffering HIV related thrombocytopenia was followed up for 23 months. Introduction or reintroduction of azidothymidine obtained a raise of platelet rate. When azidothymidine was stopped, platelet rate climbed down. This observation is in agreement with recent data suggesting efficiency of azidothymidine on HIV related thrombocytopenia.