Long-term experience with management of renal cell carcinoma involving the inferior vena cava.

OBJECTIVES: To evaluate the long-term outcome and prognosis and influence of patient and tumor characteristics and therapeutic interventions on patients with renal cell carcinoma (RCC) extending to the inferior vena cava (IVC). METHODS: The data of 75 patients (51 men and 24 women; age range 27 to 92 years) with RCC and involvement of the IVC, including 49 without and 26 with metastatic disease, treated between July 1973 and December 1998 were reviewed. The clinical presentation, laboratory and imaging investigations, extent and level of caval involvement, operative details, and estimated blood loss, as well as the postoperative course, morbidity, and actuarial and disease-free survival were analyzed. RESULTS: Seventy-five patients between 27 and 92 years old with RCC involving the IVC were studied. Of the 54 operative patients, 48 had no metastasis and 6 had metastatic disease; 32 had IVC tumor extension to the infrahepatic or low retrohepatic IVC, 7 had high intrahepatic IVC extension, and 15 had right atrial extension. In 7 patients, tumor had invaded the IVC wall. Partial IVC wall excision was done in 4 patients and resection of a complete segment of the IVC in 3 patients. Tube graft to replace a segment of the IVC was used in 2 patients. Patients with intracardiac extension were initially treated with cardiopulmonary bypass. Subsequently, profound hypothermia and circulatory arrest were also used. Three patients died in the postoperative period: two with and one without metastatic disease. The follow-up period ranged between 25 and 144 months. Of the 48 patients without evidence of metastasis at surgery, the perioperative mortality rate was 2%. Twenty-two patients (47%) were alive without evidence of metastases, 4% developed solitary metastasis, and 36% eventually developed multiple metastases. CONCLUSIONS: Our long-term experience confirms that of other investigators that nonmetastatic RCC with extension into the IVC is a potentially curable condition provided complete removal can be achieved. The level of extension of the tumor thrombus dictates the surgical techniques used for successful removal of the tumor thrombus. The treatment of patients with caval involvement and metastatic disease at presentation needs to be carefully individualized. Those with extensive multiorgan metastases continued to do poorly irrespective of the therapeutic approach chosen.

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.

Adrenal tumors associated with inadequately treated congenital adrenal hyperplasia.

We describe a case of salt-losing congenital adrenal hyperplasia due to 21-hydroxylase deficiency complicated by a right adrenal adenoma. The development of adrenal adenoma or carcinoma in-patients with congenital adrenal hyperplasia (CAH) is rare; the etiology is not clear but is thought to be related to inadequate glucocorticoid therapy. Tumor formation is postulated to be a consequence of ACTH hypersecretion, which results from the lack of glucocorticoid synthesis. Our patient underwent clitorectomy and multiple constructive procedures as a newborn baby; she was managed with hormone replacement for many years. However while she took adequate mineralocortocoid dosage, she chronically tended to take inadequate doses of glucocorticoid seeking to increase her muscle ability. She developed a 6.5 cm adrenal tumor. She was managed by a hand-assisted laparoscopic radical adrenalectomy. The tumor was histologically consistent with adrenal adenoma. The importance of compliance with her medications was emphasized.