Loin Mass, Back Pain and Gynecomastia

by Glenn Matfin, M.D., Ch.B., F.A.C.E., Safak Guven, M.D. and Andy P. Cohen, F.R.C.S. Ed. (Special Contributor)

Case History: A 51 year old male sign-writer presented to the Orthopedic Department complaining of pain in his thoracolumbar spine. He dated this to an accident in which he was thrown violently on a bus during an emergency stop. The pain failed to settle, and he consulted a chiropractor who noted kyphotic deformity, global stiffness, and treated the patient for facet joint pain. Following a poor response to this therapy, an X-Ray was obtained showing wedge-type collapse of T11 to L1 vertebral bodies. Accordingly, an orthopedic opinion was requested. Of note in the patient's medical history revealed recent investigation for gynecomastia in which a raised estradiol level of 323 pmol/L (normal 25-180), with reduced FSH and LH to < 0.2 U/L and normal prolactin were detected. Referral to an endocrinologist was made, but he had yet to be seen at the time of his acute presentation.

On examination, he displayed predominantly left-sided facial acne with patchy hair loss and generalized edema, particularly evident in the ankles. The abdomen was distended with a palpable left-loin mass. Examination of the endocrine system showed gynecomastia with testicular atrophy. Orthopedic examination revealed a tender thoracolumbar junction with no neurological deficit in the perineum or lower limbs.

Investigations: Plain X-Rays were obtained of the thoracolumbar spine, and showed collapse of the vertebral bodies of T9 - L1 consistent with metastatic disease. CBC, Ca, BUN, creatinine, K, TFTs, and serum electrophoresis were normal. Alkaline phosphatase and ESR were slightly elevated. A 24-hour urinary cortisol was markedly elevated on two occasions at 2010 and 1265 compared to the normal range of 140 - 305 nmol/24h.

In order to show the extent of bony metastases, in particular to assess the possibility of spinal stabilization, a bone-scan was performed. This showed abnormal activity at T6 - L2, as well as multiple "hot spots" in the rib cage (Figure1). Subsequent abdominal CT scanning revealed a well encapsulated mass of mixed density containing calcification, and blending intimately with the upper pole of the left kidney. It was adherent to the spleen, and displaced the pancreas anteriorly. The lesion was consistent with an adrenal neoplasm (Figure 2). A biopsy was performed which showed a vascular neoplasm containing pleomorphic cells with granular pink and occasionally clear cytoplasm and marked nuclear pleomorphism. Special staining characteristics favored a diagnosis of adrenocortical carcinoma.

Clinical course: The patient was subsequently treated by local radiotherapy for the spinal metastases as the extent of involvement precluded surgical stabilization. Management of metastatic adrenal carcinoma will be discussed.

Editorial discussion: Adrenocortical carcinomas are generally quite large (over 100g), and are commonly palpable as abdominal masses. These tumors are encapsulated, and calcification is not uncommon. Spread is typically by local invasion of the retroperitoneum, kidney or liver, or hematogeneously to the liver and lungs. Adrenocortical carcinomas frequently secrete multiple steroids in an unpredictable pattern. Patients usually have severe and rapidly progressive clinical manifestations of cortisol excess; in females, androgen excess is prominent. Therapy for adrenocortical carcinomas is less than satisfactory as the majority of patients have metastases at presentation. Surgery is the first step in therapy. Although surgical cure is rare, debulking of tumor decreases steroid hypersecretion. When metastatic or residual disease is present, mitotane is the drug of choice. Ketoconazole, metyrapone, and/or aminoglutethamide may also be used.1 Radiotherapy and conventional chemotherapy are not generally useful in treating this condition.

REFERENCES
1. Shimon I and Melmed S. Management of pituitary tumors. Ann Intern Med 1998 (15 Sept), 129:472-483.