title: [Primary pleomorphic rhabdomyosarcoma of the kidney in adults: unusual tumor].
abstract: Primary rhabdomyosarcoma (RMS) of the kidney in an adult is a very rare and unusual tumor in this site. The clinical signs associated with the flank tumoral syndrome, the histologic appearance of cytoplasmic double striation in rhabdomyoblasts and the immunohistochemical expression of skeletal muscle differentiation (desmin, myoglobin, myogenin) are described in the context of a rapidly evolving renal RMS in a 77-year old man. The differential diagnosis are mainly represented by sarcomatoid renal cell carcinoma. According to the neoplastic extent, the treatment includes radical nephrectomy, chemotherapy and surgery. The prognosis of primary renal RMS is extremely poor, with lymph node, hepatic, bone marrow and pulmonary metastasis and a short survival rate.
authors.affiliation: Service de Pathologie, Centre Hospitalier Princesse Grace, BP 489, MC 98012 Monaco. path@chpg.mc
authors.forename: Claire
authors.initials: C
authors.lastname: Mainguené
authors.forename: Christian
authors.initials: C
authors.lastname: Choquenet
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Cucchi
authors.forename: Florence
authors.initials: F
authors.lastname: Dupré
authors.forename: Isabelle
authors.initials: I
authors.lastname: Monticelli
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Michiels
authors.forename: Gonzague
authors.initials: G
authors.lastname: de Pinieux
authors.forename: Annick
authors.initials: A
authors.lastname: Vieillefond
mesh_terms: D000368:Aged; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology*; D008297:Male; D012208:Rhabdomyosarcoma / Q000473:pathology*