[Isolated leiomyomatosis of the right renal vein associated with hepatic myelolipoma: diagnostic and therapeutic problems]. / Léiomyomatose isolée d'une veine rénale droite associée à un myélolipome hépatique: problèmes diagnostiques et thérapeutiques.

The authors report a case of isolated intravenous leiomyomatosis involving a vein of the right renal hilum associated with operated hepatic myelolipoma with a diagnosis of metastatic renal cancer. These tumours are very rare and are usually diagnosed on histological examination of the resection specimen. Although they have a benign histology, they have a very different prognosis and therapeutic management. Although the association of these two exceptional tumours may appear to be purely incidental, a common aetiological factor may nevertheless exist.

Systemic mast cell disease associated with hairy cell leukaemia.

Systemic mast cell disease (SMCD) can be regarded as a tumorous proliferation of tissue mast cells involving various organs. The frequency with which SMCD is found in patients with haematological disorders suggests that the association is non-random. The association includes primarily, myeloid disorders such as myelodysplastic syndromes and acute or chronic myeloproliferative disorders. Lymphoproliferative disorders may also occur but more rarely, mostly non-Hodgkin's low grade B cell lymphomas. In this report a case is described in which SMCD occurred in a patient with hairy cell leukaemia.

Implication of the Epstein-Barr virus in the progression of chronic lymphocytic leukaemia/small lymphocytic lymphoma to Hodgkin-like lymphomas.

Low grade CLL/SLL can evolve to a spectrum of various morphologic higher grade malignancies showing Reed-Sternberg like cells. The evolution towards Hodgkin's disease is rare but frequently associated with the presence of scattered RSL cells within the small lymphocyte proliferation of the CLL/SLL. The evolution towards a Richter's syndrome is more frequent and it can exhibit CD30 positive Reed-Sternberg like cells. In these Richter's syndrome cases, regarding the morphology and the phenotype, it seems likely that there is a spectrum of lesions between true HD and large cell NHL. In the present study, the authors report two cases of transformation of CLL/SLL in non immuno-suppressed patients; one evolved to a morphological and immunohistochemical Hodgkin's disease and the second to a NHL (Richter's syndrome) with numerous Reed-Sternberg like cells. In both cases, EBV has been detected within RSL cells by immunohistochemistry and in-situ hybridization (ISH). So, the role of EBV is suggested in that kind of transformation.