Pheochromocytoma: clinical observations from a Brooklyn tertiary hospital.

OBJECTIVE: To report eight cases of pheochromocytoma, diagnosed and treated at our tertiary hospital during a 42-month period. METHODS: We review clinical manifestations as well as historical and family data. Biochemical and radiologic methods for diagnosis and tumor localization at our institution were compared with methods at other teaching centers. RESULTS: Among 4,180 patients with essential hypertension encountered during a 42-month interval, 8 had pheochromocytoma. The most common clinical findings were diaphoresis, chest or abdominal pain, palpitations, headaches, and nausea. Clinical features were remarkable only for the significant correlation between urinary excretion of vanillylmandelic acid and tumor volume (r = 0.925; P<0.01). Only one patient, with a history of medullary thyroid carcinoma, had multiple endocrine neoplasia. No unusual familial concentration of pheochromocytomas was found in our community, and our diagnostic techniques were similar to those reported from other institutions. We calculated an average annual incidence rate of 0.5 per 100,000 person-years, a figure comparable to that reported at the Mayo Clinic in Rochester, Minnesota. CONCLUSION: Because our institution does not see the volume of referral patients as does the Mayo Clinic, we suggest that the frequency of pheochromocytoma may be underestimated in the patient population that we serve.

Ketoconazole reverses hyperandrogenism in a patient with insulin resistance and acanthosis nigricans.

We administered ketoconazole to a young woman with ovarian hyperandrogenism, insulin resistance, and acanthosis nigricans. While taking ketoconazole, her serum testosterone, androstenedione, dehydroepiandrosterone, and cortisol levels declined, while serum progesterone, 17-hydroxyprogesterone, and 11-deoxycortisol rose. Serum LH, FSH, and estradiol levels were intermittently higher during ketoconazole treatment, although LH and FSH responsiveness to GnRH did not change. Basal and stimulated serum insulin concentrations were high before and during ketoconazole therapy, while fasting glucose levels and glucose disappearance rate constants were normal throughout the study. A dramatic improvement in hirsutism occurred, and menses resumed after a 6-yr hiatus. Adverse drug effects or clinical evidence of adrenal insufficiency were not encountered. These results support a role for ketoconazole in the therapy of ovarian hyperandrogenism.