Massive Pregnanetriol Excretion Due to Virilizing Adrenal Tumour

Abstract

Urinary 17-oxosteroid values in excess of 100 mg./24 hr. are not uncommonly associated with virilizing tumours of adrenal origin. However, the secretion of pregnanetriol in amounts greater than 50 mg./24 hr. is unusual. The patient reported here exhibited the highest level of pregnanetriol excretion due to adrenal tumour without metastases ever recorded in the English literature.A 47-year-old Negro woman presented with a 10-month history of hirsutism, darkening of the facial skin, periorbital oedema, abdominal swelling, and right flank pain, progressively severe and debilitating. Twenty years previously, total abdominal hysterectomy and bilateral salpingo-oophorectomy had been accomplished for pelvic inflammatory disease.Physical examination revealed a round-faced hirsute Negress in no distress with blood pressure of 150/105 mm. Hg, atrophic breasts, masculine abdominal and pubic hair distribution and patchy hyperpigmentation of the face and arms. The liver was palpable 4 cm. below the right costal margin and was only slightly tender. The clitoris was 3 cm. in length and the vaginal cuff and adnexal regions were unremarkable.Haematocrit, serum electrolytes, blood urea nitrogen, glucose tolerance tests, and liver function studies were normal. Persistent leukocytosis ranging from 13,000 to 17,000 white blood cells/mm.3 with a normal differential count was present. X-rays of the chest and bones were normal, disclosing no evidence of métastases. Intravenous urography disclosed normal collecting systems with depression and lateral displace¬ ment of the right kidney (PI. 1, fig. 1) and bilateral perirenal C02 insufflation revealed a large right suprarenal mass, consistent with an adrenal tumour (PI. 1, fig. 2).Endocrine assays included urinary 17-hydroxysteroids ranging from 13 to 37 mg./ 24 hr. and urinary 17-oxosteroids of 270-503 mg./24 hr. ; neither of these steroids was suppressed by dexamethasone nor were they altered by metyrapone administration. Pregnanetriol excretion was 107 mg./24 hr. in a single determination.On 30 October 1967, the patient underwent removal of the encapsulated vascular adrenal tumour of 16 cm. diam. through an extrapleural llth-rib approach. The peri¬ toneal cavity was free of metastatic involvement and liver biopsy was unremarkable. Histological examination disclosed a well-differentiated benign cellular structure without vascular or capsular invasion (PL 2, figs. 3 and 4). Six weeks after operation, urinary steroid values were within normal limits.Although the excretion of large quantities of 17-oxosteroids is not uncommon in patients with virilizing adrenal tumours, as observed