title: [Kidney cancer and angiomyolipoma in lymphangiomyomatosis].
abstract: Lymphangiomyomatosis is an extremely rare benign disease characterized by extensive proliferation of smooth muscle cells within lymphatic vessels and lymph nodes. While 10-15% of all patients show concomitant renal angiomyolipomas, no association so far has been reported with renal cell carcinoma. We present a case with coincident lymphangiomyomatosis, renal cell carcinoma and renal angiomyolipoma. The possible underlying pathogenesis is discussed. Classifying lymphangiomyomatosis as a "forme fruste" of tuberous sclerosis allows postulation of a common pathogenesis for all three entities.
authors.affiliation: Urologische Universitätsklinik Hamburg-Eppendorf.
authors.forename: S
authors.initials: S
authors.lastname: Conrad
authors.forename: B
authors.initials: B
authors.lastname: Wagner
authors.forename: K
authors.initials: K
authors.lastname: Hamper
authors.forename: R P
authors.initials: RP
authors.lastname: Henke
mesh_terms: D000315:Adrenalectomy; D018207:Angiomyolipoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002292:Carcinoma, Renal Cell / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D008197:Lymph Node Excision; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D009392:Nephrectomy; D014057:Tomography, X-Ray Computed
vernacular_title: Nierenkarzinom und Angiomyolipom bei Lymphangiomyomatose.