[Primary osseous leiomyosarcoma of the jaw. Apropos of a case]. / Léiomyosarcome osseux primitif des maxillaires. A propos d'un cas.

Primary leiomyosarcoma of bone is a malignant smooth muscle tumour uncommonly found in the jaws. We report a case of primary leiomyosarcoma of the mandible in a 31-year-old man treated with wide resection and radiotherapy then secondary reconstruction with a fibula free flap. No recurrence or distant metastasis has developed four years after the initial surgery. We review the literature on primary leiomyosarcoma of the jaws.

[Solid pseudopapillary tumors of the pancreas. Pathology report of 13 cases]. / Tumeurs pseudo-papillaires et solides du pancréas. Etude anatomoclinique de 13 cas.

OBJECTIVES: Solid pseudopapillary tumors of the pancreas are exceptional. The aims of our study were to reevaluate the mode of presentation of these tumors and to analyze the role of pathological examination in diagnostic assessment and prognostic evaluation. PATIENTS: We report the clinical, radiological and pathological findings in a retrospective series of 13 patients in whom a diagnosis of solid pseudopapillary tumor of the pancreas was made between 1983 and 1997. There were 12 females (median age: 22.5 years) and one male, aged 73. RESULTS: The tumor was discovered incidentally (3 cases) or because of nonspecific digestive symptoms (10 cases). Biological data were uninformative. The tumor was pancreatic in 12 cases and duodenal in 1. In all cases, imaging techniques showed an heterogeneous lesion with no or poor vascularization. A cystic component was identified in 4 cases. Surgical resection was performed in all cases. Pathological examination showed an encapsulated tumor in 8 cases, a non-encapsulated but well-limited lesion in 3 cases and an infiltrative tumor in 2 cases. At the time of diagnosis, multiple liver metastases were present in 1 case. Mean duration of follow-up was 24 months (range: 3-168). At last follow-up, all patients, including the patient with synchronous metastatic disease, were alive, without local recurrence. CONCLUSION: Our study confirms that most cases of solid pseudopapillary tumors of the pancreas present with a suggestive clinical picture, including their occurrence in young women and their good prognosis after surgical resection. However, our results also underline the occurrence of cases presenting with unusual features, including old age, male sex, extra-pancreatic localization and malignant evolution. Histopathological examination is essential for the establishment of the diagnosis but morphological data are of little prognostic value.

[Autochthonal leprosy]. / Lèpre autochtone.

INTRODUCTION: In continental France, Hansen's disease is now a strictly imported disease. Native contamination is rare since the foyers described early in the century have totally disappeared. CASE REPORT: A case of locally acquired diffuse lepromatous Hansen's disease was observed in a patient who developed acrocyanosis with distal necrosis over a period of several years. Late cutaneomucal signs led to the diagnosis of Hansen's disease after eliminating sarcoidosis and tuberculosis. Skin biopsy with Ziehl stain confirmed the lepromatous nature of the disease. In addition the patient had undergone splenectomy following trauma and also developed babesiasis (a rare protozoan disease in Europe--15 cases reported--similar to Texas fever). DISCUSSION: The source of infection in this case of Hansen's disease was not found. As in the other cases reported in the literature, late diagnosis due to lack of any suggestive context allowed the disease to develop into an advanced stage.

[Necrolytic migrating erythema without glucagonoma]. / Erytheme necrolytique migrateur sans glucagonome.

INTRODUCTION: The glucagonoma syndrome is an uncommon but well-known entity associating erythema necroticans migrans (ENM) with glucagonoma. CASE REPORT: A 43-year-old man with a past history of alcoholic cirrhosis and ascitis was hospitalized for skin disorders which had developed over the past 4 months. Centrifugal erythematous skin lesions were observed, some with non-turgid bullae and marginal desquamation, others with an erosive center. Lesions first appeared on the hands then diffused widely without involving the periorifical areas, folds and lower limbs. Laboratory results revealed an anemia, hypovitaminosis K, cholestatic liver failure, a beta-gamma block and low zinc levels. Histology study of the skin biopsy demonstrated a << Neapolitan trench >> image suggestive of ENM. A paraneoplasic syndrome and pancreas tumor were not found. Despite supplementation with zinc, amino acids and vitamins, the patient died from his liver disease. DISCUSSION: There have been 4 cases of ENM reported in the literature, all in cirrhosis patients, two of which had low zinc levels. Our case is thus the third with cirrhosis and low zinc. Bazex's syndrome, acrodermatitis enteropathica, annular chronic lupus erythematosis and annular superficial pemphigus were eliminated as possible diagnoses. The failure of zinc and amino acid supplementation would favor the secondary nature of the zinc deficiency and the predominant role of cirrhosis in this skin disease. The possible role of essential fatty acids in ENM is raised.

[Disseminated xanthosiderohistiocytosis with cardiac involvement and monoclonal gammapathy]. / Xanthosidérohistiocytose disséminée avec atteinte cardiaque et gammapathie monoclonale.

Xanthosiderohistiocytosis is a rare non-Langerhans histiocytosis (4 reported cases). The clinical characteristics include diffuse, sometimes deep, dark-brown infiltrations of the skin. Histological examination reveals abundant deposits of haemosiderin within the histiocyte proliferation. This entity is a clinical form of Montgomery's disease (xanthoma disseminatum) which has been reported in over 100 cases. Our case of disseminated xanthosiderohistiocytosis was particular because it involved the heart and was associated with a monoclonal gammapathy. Five cases have been reported associating xanthoma disseminatum and monoclonal gammapathy, including one case of xanthosiderohistiocytosis. In our case, rapidly increasing levels of monoclonal immunoglobulin suggested an evolution towards a myeloma. A monoclonal gammapathy should be looked for and monitored in cases of xanthosiderohistiocytosis, and more generally xanthoma disseminatum because of the risk of developing lymphoma or myeloma.

[Gold therapy in pemphigus]. / Chrysothérapie du pemphigus.

According to works of Penneys and Eaglstein on the management of pemphigus with gold compounds, the authors report one observation of chronic pemphigus, in which steroid therapy caused severe psychiatric disorders. The use of gold compounds permitted reduction of the dose of steroids and control of the pemphigus.