Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms.

OBJECTIVES: To evaluate the changing management of sporadic renal angiomyolipoma and renal angiomyolipoma associated with the tuberous sclerosis complex (TSC) during the past 16 years. METHODS: We retrospectively reviewed the charts of 60 patients with angiomyolipoma seen at our institutions. RESULTS: The median age at presentation was 45 years (range 7-78). The presentation was pain in 30 patients and hematuria in 13; it was incidentally discovered in 17 patients. Of the 60 patients, 43 were females. TSC was present in 14 patients. The median tumor size was 4 cm (range 0.3-40, mean 6.5 +/- 1.1). Of the 60 patients, 31 were followed up expectantly. Surgery or intervention was needed for 29 patients to control hemorrhage or relieve pain or because of the suspicion of malignancy. Of these 29 patients, 12 underwent nephrectomy, 11 partial nephrectomy, and 6 embolization. The patients treated for hemorrhage had a median tumor diameter of 11 cm (range 2-21). Patients were followed up for a mean of 39.3 +/- 5.4 months. The lesions grew an average of 4.7 +/- 3.4 cm for TSC tumors and 0.6 +/- 0.2 cm for sporadic angiomyolipoma tumors. None of the patients developed renal impairment. Patients with TSC presented at a younger age, had larger and bilateral lesions, and were more symptomatic during follow-up. In the past 6 years, a significant trend was seen toward finding tumors in asymptomatic patients and toward the use of conservative or interventional (embolization) treatment. CONCLUSIONS: Renal angiomyolipoma has a slow growth rate. The preservation of renal function was noted in all our patients. A recent shift was noted toward finding smaller tumors in asymptomatic patients and the use of conservative and interventional treatment.

Multi-institutional long-term experience with conservative surgery for invasive penile carcinoma.

PURPOSE: Invasive squamous cell carcinoma of the penis occurs on the glans, prepuce, glans and prepuce, coronal sulcus and shaft. Penile squamous cell carcinoma subsequently invades local structures, corpora cavernosa and the urethra, and metastasizes to the inguinal lymph nodes. Invasive squamous cell carcinoma of the penis usually requires total or partial penectomy. We studied the effect of primary tumor resections tailored to the anatomical extent of the cancer with preservation of uninvolved structures in select patients with invasive penile squamous cell carcinoma. MATERIALS AND METHODS: A total of 30 patients between 39 and 82 years old were treated with unconventional conservative surgical excision of the primary penile lesion. More than 130 patients were excluded from the study because they were treated with partial or total penectomy, Mohs' surgery or more extensive surgery. The 30 patients underwent preoperative biopsy with careful mapping of the extent of the disease. Patient age, tumor extent and grade, operative details, outcome and length of followup were analyzed. RESULTS: Tumor size ranged from 1.5 to 8 cm. in diameter. Tumors were well differentiated in 19 patients, moderately differentiated in 5 and poorly differentiated in 6. A total of 17 patients underwent ilioinguinal lymphadenectomy, 12 of whom had pathologically positive lymph nodes. Inguinal radiation was used in 2 patients. Chemotherapy was given to 7 patients with extensive inguinal lymphadenopathy and to 2 of 5 with pathologically positive lymph nodes. Followup ranged from 12 to 360 months. A total of 21 patients had no evidence of disease at last followup. Tumor resection with no sacrifice of function was performed in 2 patients in whom 3 small recurrences developed. One patient with numerous tumors had 2 small recurrences, which were completely excised with no further recurrence. Of the 7 patients with advanced lymphadenopathy 5 and of 5 patients with pathologically positive lymph nodes at presentation 1 died of the cancer but had no local recurrence in the penis. CONCLUSIONS: In a minority of patients with anatomically suitable penile cancer conservative surgical techniques are safe and provide equal tumor control compared to conventional resections. The anatomical situation and tumor characteristics should dictate the choice of treatment for the primary penile lesion. Inguinal lymph nodes should be managed by appropriately established guidelines but should not influence the extent of primary penile lesion resection.

Experience with coapted gastric tube outlet and composite gastrointestinal reservoir for continent cutaneous urinary diversion.

PURPOSE: Established techniques for urinary diversion are not ideal for certain patients such as those with extensive pelvic irradiation, metabolic acidosis, short bowel syndrome or renal insufficiency. In a multi-institutional study a gastrointestinal reservoir was previously found to provide metabolic balance in such patients. We used a coapted gastric tube as the continent outlet in patients undergoing gastrointestinal urinary diversion. We evaluate the long-term functional results of the gastric tube to provide continence and report our long-term followup results. MATERIALS AND METHODS: A composite reservoir was constructed from gastric and transverse colon or ileal segments. In addition, a gastric strip, in continuity with the gastric segment of the composite reservoir, was tubularized and coapted to provide the continence mechanism. RESULTS: A total of 19 patients with a gastrointestinal reservoir and coapted gastric tube outlet were followed for 24 to 101 months, of whom 18 are continent day and night on intermittent catheterization every 3 to 8 hours. Four of 34 ureters (12.5%) became obstructed. One patient had significant preoperative renal insufficiency, which progressed to end stage renal disease. Another patient had slow progression of renal insufficiency. In the remaining 17 patients mean serum creatinine did not change significantly while serum chloride and bicarbonate improved or remained normal. CONCLUSIONS: A coapted gastric tube functions well as the continence mechanism in patients with a gastrointestinal urinary reservoir. We also confirm the metabolic advantages of a composite gastrointestinal urinary reservoir. Gastrointestinal cutaneous urinary diversion can be an alternative to incontinent diversion in select patients who are not suitable for other forms of diversion.