[Multilocular, giant angiomyolipoma of the kidney, adrenal gland and para-aortic lymph nodes. Case report of a 9-year-old boy with tuberous sclerosis].
The symptoms and therapy of a multicentric angiomyolipoma of the kidney, adrenal gland, paraaortic lymph nodes and renal fat tissue in a 9-year-old boy with tuberous sclerosis are reported. Angiomyolipomas are benign mesenchymal tumours that often occur together with tuberous sclerosis. Renal cell carcinoma in an angiomyolipoma is rare. Multicentricity and lymph node involvement is not a sign of malignancy or metastatic disease. Radiological characteristics in CT and plain X-ray may help in the diagnosis. Characteristically, the renal lesions are asymptomatic. Patients with incidental symptom-free angiomyolipoma should be followed. In other patients with pain in the loin, or when a solid tumour cannot be confidently excluded, conservative surgery or nephrectomy should be performed.
Urologische Universitätsklinik, Hamburg-Eppendorf.
J
J
Noldus
U
U
Ancker
H
H
Schäfer
S
S
Conrad
H
H
Huland
D000310:Adrenal Gland Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D018207:Angiomyolipoma / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D002648:Child; D006801:Humans; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis; D008297:Male; D016609:Neoplasms, Second Primary / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery; D009392:Nephrectomy; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000000981:diagnostic imaging* / Q000473:pathology / Q000601:surgery
Multilokuläres, riesiges Angiomyolipom der Niere, Nebenniere und der paraaortalen Lymphknoten. Fallbericht eines 9jährigen Jungen mit tuberöser Sklerose.