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Adrenocortical carcinoma

Adrenocortical carcinoma is a carcinoma of the cortex (outer layer) of the adrenal gland. While most tumors of the adrenal cortex are benign (adenomas) and only occasionally cause Cushing's syndrome, the malignant form makes up about 3% of all cortical tumors and requires surgery and sometimes chemotherapy. Excess cortisol production may require suppression with ketoconazole or metyrapone. Production of aldosterone or androgens by carcinomas is extremely rare. Adrenocortical carcinomas are also sometimes referred to as adrenal cortical carcinomas, ACC, or adrenal cortex cancers. more...

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Complete surgical excision (if possible) is the primary treatment used for these neoplasms.

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Primary adrenocortical carcinoma with endobronchial metastasis
From CHEST, 10/1/05 by Himanshu Desai

INTRODUCTION: Pulmonary metastases to the adrenal gland are common. Malignant endocrine tumors infrequently metastasize to the lungs and treatment options are limited. Primary adrenal tumors with metastases to the lung are even more uncommon. We present a rare case of endobronchial metastasis from a primary adrenocortical cancer.

CASE PRESENTATION: A 61 year-old white female with known adrenocortical carcinoma diagnosed in August 1998 presented to our pulmonary clinic for abnormal radiographs. The patient initially presented in August 1998 with signs and symptoms of virilization. She was subsequently diagnosed with stage IV adrenocortical carcinoma. She underwent a surgical resection of the right adrenal gland and right kidney that same year. She received no further therapy and has been followed with DHEA-S levels and serial thoraco-abdominal CT scans every 3-5 months. Serial thoracic CT scans were normal until January 2005 (Figure 1). The patient was asymptomatic. Physical examination was normal and did not reveal lymphadenopathy. Patient has a 20 pack year history of smoking which stopped 6 years ago. Pertinent laboratory include a pre-operative DHEA-S level of 797 micrograms/deciliter (normal: 60-255 [micro]g/dl), postoperative levels of 12[micro]g/dl (8/98), 57[micro]g/dl (6/01) and 151[micro]g/dl (1/05) respectively. Patient underwent a fiberoptic bronchoscopy which revealed a smooth, well rounded endobronchial lesion in the right lower lobe (Figure 2). This area was washed, brushed and biopsied. The biopsy results revealed metastatic adrenocortical carcinoma.

DISCUSSIONS: Adrenal tumors are usually suspected when the patient presents with signs and symptoms of excessive cortisol secretion. Often, tumors are found incidentally on abdominal CT scans. When symptoms are present they include oligomenorrhea, hirsutism, acne, purple striae, osteoporosis, and muscle wasting. Though no clear epidemiological data exists, adrenal tumors with metastases to the lung are uncommon. Furthermore, endobronchial metastases from nonpulmonary tumors are uncommon. (1) CT scans are effective in localizing metastatic disease to the lung. The diagnosis can be confirmed by serum assays for excess hormone secretion. Survival in patients with adrenocortical confined to the adrenal gland at resection is 30% at 5 years, but the median survival of patients with metastatic disease is six months. (2) Mitomycin is used when metastatic disease is present with mixed results.

CONCLUSION: Thoracic manifestations of adrenocortical carcinoma are infrequent and not well documented. We cared for a patient with metastatic disease at diagnosis in 1998 that initially had a favorable response to surgery alone. Over six years later the patient presented with endobronchial metastasis from an intra-abdominal adrenocortical carcinoma. There were no abnormalities of the serial hormone assays. Though there has been a case report of endobronchial pheochromocytoma, A MEDLINE search in the English language revealed no cases of endobronchial involvement from a primary adrenocortical carcinoma. (3) The patient is currently undergoing chemotherapy.

[FIGURE 1 OMITTED]

REFERENCES:

(1) Kilyu T, Hoshi H, Matsui E, Iwata H, Kokubo M, Shimokawa K, Kawaguchi S. Endotracheal/Endobronchial Metastases. Chest 2001; 119: 768-775.

(2) Khan JH, McElhinney DB, Rahman SB, George TI, Clark OH, Merrick SH. Pulmonary Metastases of Endocrine Origin. Chest 1998; 114: 526-534.

(3) Sorensen JB. Endobronchial Metastases from Extrapulmonary Solid Tumors. Acta Oncologica 2004; 43: 73-79.

Himanshu Desai MD * Neelima Chintapalli MD Jonathan Glass MD Shawn Milligan MD Louisiana State University Health Sciences Center, Shreveport, LA

COPYRIGHT 2005 American College of Chest Physicians
COPYRIGHT 2005 Gale Group

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