腎血管筋脂肪腫の4例

Four cases of renal angiomyolipoma are reported. All of them are female and two of them are with tuberous sclerosis.<br>Case 1: IVP revealed distortion of the left upper calyces and the aortogram showed extravasation of radiopaque dye in the upper region of the left kidney and neo-vascularization beyond renal contour, which were suggessive of renal cancer. In this case, correct diagnosis was not made preoperatively.<br>Case 2, Case 3: As there were signs of tuberous sclerosis, angiomyolipoma were highly considered preoperatively.<br>Case 4: The patient was with asymptomatic hematuria. Selective angiography demonstrated neo-vascularization protruding from the outline of the left kidney, which were highly suggestive of angiomyolipoma. On operation, frozen section diagnosis comfirmed angiomyolipoma. The all four patients are surviving in healthy condition.<br>In 1969, Nonaka et al. collected 16 cases from Japanese literatures and added 3 cases of his own. The authors collected additional 28 cases including these four cases reported in this paper. The clinical features of these 47 cases analyzed as following:<br>1) Of the 47 patients, 9 were male and 38 were female. The youngest was 17 years old male and the oldest was 58 years old female. The average age was 33.7 years (32 years for male and 27.4 years for female).<br>2) Eighteen patients (38%) were with tuberous sclerosis. Six of them were male and 12 were female. The rest of the 28 patients were without tuberous sclerosis. Three of them were male and 25 were female.<br>3) Sixteen patients had this tumor unilaterally in the right kidney, 27 patients in the left and 4 patients bilaterally. All the 4 patients with bilateral tumors were with tuberous sclerosis.<br>4) Nine tumors were seen in the upper pole, 12 were in the lowerepole, 7 were in the central or hilar region of the kidney. In three cases, the tumors occupied both upper pole and hilar region. The location of the 12 tumors were not given in their literature.<br>5) The majority of the patients had some clinical symptoms. Pain and tenderness was present in almost all the patients. Thirty two percent of the patient had hematuria and 30% of them had palpable mass. Fever, anemia and gastrointestinal symptoms were seen in some cases. Retroperitoneal hemorrhage due to spontaneous rupture occurred in 7 cases. Emergency operation was performed.<br>6) Renal angiography can be of diagnostic help in cases of angiomyolipoma. Careful interpretation of the angiogram and clinical manifestations enables us to differentiate this tumor from renal carcinoma.<br>7) Nephrectomy was done in 40 (85%) patients, partial nephrectomy in 2, and only biopsy was done in 3 patients.