W. Marston Linehan

Laparoscopic adrenalectomy: a new standard of care.

OBJECTIVES Adrenalectomy is the mainstay of treatment for adrenal tumors. A variety of surgical approaches to the adrenal gland have been described. We studied the feasibility of laparoscopic adrenalectomy (LA), compared laparoscopic with open adrenalectomy (OA), and studied the hemodynamic changes in patients with pheochromocytoma. METHODS Our early experience with 20 consecutive LAs is compared with a contemporaneous, matched control cohort of 20 patients who underwent OA via a flank or subcostal incision. LA was performed via a transperitoneal approach, following a standardized surgical technique. RESULTS LA was successfully completed in 18 of 20 cases. Average operating time in the first 5 cases was 261 minutes, but, with further experience, a significant decrease in operative time was seen in the last cohort of 4 patients (155 minutes) (P = 0.0018). There was no significant difference in operative time or degree of blood loss between LA and OA groups. Patients who underwent LA required lower doses of postoperative parenteral narcotics (P = 0.0169), had a shorter hospital stay (mean 3.2 days) (P < 0.0001), and had a shorter convalescent period (mean 3.1 weeks) (P < 0.0001). Complications in the laparoscopic group (chronic port site pain in 1 patient, intra-abdominal fluid collection in another) occurred in the 2 patients who required open conversion. These 2 patients had large adrenal tumors (9 and 7 cm in diameter, respectively). LA resulted in similar hemodynamic changes as OA in patients with pheochromocytoma. CONCLUSIONS LA is a safe and effective approach in most patients with adrenal pathology. Benefits include excellent operative exposure and visualization, less postoperative pain, shorter hospital stay and convalescent period, and improved cosmetic result. Pheochromocytoma is not a contraindication to LA. Patients with large adrenal tumors (larger than 6 cm), evidence of venous involvement, or invasion into surrounding tissue should be approached cautiously.

Chapter 28 – The Genetic Basis of Cancer of the Kidney

Publisher Summary This chapter discusses the genetic basis of cancer of the kidney. The hereditary renal cancer syndromes include von Hippel-Lindau (VHL), hereditary papillary renal cancer (HPRC), Birt-Hogg-Dube (BHD), and hereditary leiomyomatosis and renal cell cancer (HLRCC). Von Hippel-Lindau is an autosomal dominantly inherited disorder that leads to development of hemangioblastomas of the central nervous system (CNS), endolymphatic sac tumors, pancreatic cysts and neoplasms, pheochromocytomas, renal cysts and cancers, and cystadenomas of the epididymis and broad ligament. Hemangioblastomas of the CNS are the most common manifestation of VHL. These are most commonly found in the spinal cord and cerebellum. These are benign tumors, but can cause much morbidity. Lesions are resected when they become symptomatic. HPRC is an autosomal dominant syndrome with high penetrance marked by the predisposition of patients to form multifocal, bilateral renal tumors of papillary type I pathology. The tumors often appear later than in other hereditary renal cancer syndromes, with onset in the 4th, 5th, and 6th decades of life. Patients with HPRC have mutations in the tyrosine kinase domain of the METgene, located on chromosome 7q31, which is postulated to be an activating mutation, unlike VHL, a tumor suppressor gene. METencodes a tyrosine kinase activated by hepatocyte growth factor (HGF). The Birt-Hogg-Dube syndrome is an autosomal dominant disorder in which affected individuals develop benign cutaneous tumors, pulmonary air-filled cysts and spontaneous pneumothoraces, and renal neoplasms. The hallmark lesion of BHD is the fibrofolliculoma, a benign tumor of the hair follicle, which appears in the third to fourth decade of life.