[Kidney cancer and angiomyolipoma in lymphangiomyomatosis].
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.
Conrad
S
S
Urologische Universitätsklinik Hamburg-Eppendorf.
Wagner
B
B
Hamper
K
K
Henke
R P
RP
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
Nierenkarzinom und Angiomyolipom bei Lymphangiomyomatose.