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A 43-year-old man, who is an identical twin, presented with a 2-year history of lethargy, weakness, and headaches and a several-year history of gradual loss of body hair and declining libido. He had undergone left orchidopexy at 6 years of age. A physical examination revealed features of hypopituitarism and hypogonadism, including central adiposity, nipple pallor, proximal muscle wasting, preservation of scalp hair, loss of body hair, gynecomastia, and right and left testicular volumes of 12 ml and 5 ml, respectively (normal volume, >15). A remarkable feature was the contrast in appearance between the hypogonadal patient (Panel A, right side) and his unaffected identical twin (left side). Laboratory tests confirmed secondary hypogonadism, with a testosterone level of 1.5 nmol per liter (0.4 ng per milliliter) (normal range, 9.9 to 27.8 [2.9 to 8.0]), a follicle-stimulating hormone level of 2.8 IU per liter (normal range, 1.5 to 12.4), and a luteinizing hormone level of 1.5 IU per liter (normal range, 1.7 to 8.6). Serum prolactin levels, thyroid function (measured by free thyroxine and thyroid-stimulating hormone), and cortisol levels were normal; the insulin-like growth factor I level was mildly decreased. Magnetic resonance imaging of the pituitary showed a lobulated, contrast-enhancing suprasellar mass (16 by 29 mm; seen in the coronal view in Panel B [arrow] and in the sagittal view in Panel C [arrow]). (An image of the unaffected twin is shown for comparison in the Supplementary Appendix, available with the full text of this article at www.nejm.org.) After needle biopsy and subsequent excision by craniotomy, pathological analysis confirmed the diagnosis of pituicytoma. Apart from a seizure, the patient's postoperative recovery on pituitary-replacement therapy has been uneventful, with resolution of his symptoms.
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