Acromegaly Secondary to Ectopic Growth Hormone-Releasing Hormone-Secreting Bronchial Carcinoid Cured After Pneumectomy

Abstract

Acromegaly is caused by a primary growth hormonesecreting pituitary adenoma in more than 95% of the cases. In a small number of patients, it is the result of hypersecretion of growth hormone-releasing hormone (GHRH). Clinical and laboratory findings are similar in both conditions, and the diagnosis of a GHRHsecreting tumor is possible only by identification of GHRH in the tumor or circulation. We report the case of a 61-year-old woman referred to the Hospital Naval Marcílio Dias to investigate a pulmonary mass. The mass was large and calcified, suggesting a hamartoma. The patient also had acromegalic features, elevated serum insulin-like growth factor-1 (IGF-1) levels, and no GH suppression after an oral glucose tolerance test. Magnetic resonance imaging (MRI) showed a diffusely enlarged pituitary gland, suggestive of hyperplasia. The hypothesis of acromegaly secondary to ectopic GHRH secretion by the pulmonary mass was made. A left pneumectomy was performed and histopathologic analysis revealed a carcinoid tumor. A fragment of the tumor was sent for special stains and GHRH was identified by immunohistochemistry. The clinical and biochemical aspects of acromegaly improved 2 weeks after surgery. One year later, serum GH and IGF-1 levels were normal and MRI revealed a partial empty sella. The differential diagnosis of somatotroph adenoma and ectopic GHRH hypersecretion is difficult but is essential to appropriate therapy.A cromegaly is a rare disease caused by a primary growth hormone-secreting pituitary adenoma (classic acromegaly) in the large majority of the cases. 1 Less than 5% of cases are caused by eutopic tumors (hypothalamic gangliocytomas, hamartomas, and gliomas) or ectopic tumors (carcinoid tumors, pancreatic endocrine tumors, adrenal adenoma, smallcell lung cancer, and pheochromocytoma) secreting growth hormone-releasing hormone (GHRH). This condition is termed ectopic acromegaly. 2 In addition, acromegaly caused by ectopic 6H hypersecretion is extremely rare. 3 We report a patient with acromegaly resulting from ectopic GHRH secretion by a pulmonary carcinoid tumor. CASE REPORTA 61-year-old white woman was referred to the Hospital Naval Marcílio Dias for evaluation of a pulmonary mass in April 2000. The mass was extensively studied by bronchofibroscopy, helicoidal tomography, and magnetic resonance imaging (MRI). The mass was approximately 8 cm in diameter, calcified, and in close contact with mediastinal vessels, suggesting a hamartoma. No metastatic lesions were found. The patient exhibited clinical signs of acromegaly with typical facial features and acral changes. She reported progressive increase in hand and foot size. Hypertension had been present for 10 years. She reported arthralgia, hoarseness, and hyperhidrosis. Physical examination revealed a blood pressure of 140/90 mm Hg (taking ramipril 5 mg per day) and heart rate was 82 beats/min. No visual field defect was found and galactorrhea was not present.Endocrine evaluation confirmed acromegaly. An oral glucose tolerance test (OGTT) was perform...

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Acromegaly Secondary to Growth Hormone-releasing Hormone Secreted by an Incidentally Discovered Pheochromocytoma

“…Symptoms secondary to other secreted hormones (e.g., gastrin, cathecolamines, serotonin, insulin), tumor mass effects (at a site other than the sella turcica) and distant metastasis may suggest the presence of another tumor. Identification of a second tumor could suggest the possibility of acromegaly secondary to a GHRH-secreting tumor [6,7]. Also, the presence of somatotrophic hyperplasia on histopathologic examination and/or recurrence of acromegaly after a "successful" pituitary surgery strongly suggest GHRH hypersecretion.…”

Ectopic growth hormone-releasing hormone secretion by a metastatic bronchial carcinoid tumor: a case with a non hypophysial intracranial tumor that shrank during long acting octreotide treatment

“…IGF-1: 984 ng/ml (63-380), GH: 49.8 ng/ ml (<5), PRL: 23.3 ng/ml (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20), testosterone: 3.6 ng/ml (3)(4)(5)(6)(7)(8)(9)(10)(11)(12), LH: 2.0 mIU/ml (2)(3)(4)(5)(6)(7)(8), FSH: 3.9 mIU/ml (2)(3)(4)(5)(6)(7)(8), mid-molecule PTH: 31.2 pg/ml (£125), gastrin: 29 pg/ml (£90), 5-hydroxyindoleacetic acid: 9 mg/24 h (£6), serum chromogranin A: 20 nmol/l (<4).…”

“…Less frequently, pancreatic islet tumors, pheochromocytomas, medullary carcinomas of the thyroid, and small cell carcinomas of the lung and the thymus, among others, have been reported to cause acromegaly due to GHRH secretion [3][4][5]. Surgical resection of the primary tumor can cure the disease [6]; however, the diagnosis is frequently not established until the disease is metastatic, and therefore surgical cure is not possible. Although experience is still limited (only 19 patients to our knowledge) [5,7,8], somatostatin (SMS) analogs have been used in the treatment of ectopic acromegaly since they may not only control the endocrine hypersecretion but also tumor proliferation.…”

“…Although experience is still limited (only 19 patients to our knowledge) [5,7,8], somatostatin (SMS) analogs have been used in the treatment of ectopic acromegaly since they may not only control the endocrine hypersecretion but also tumor proliferation. Despite the favorable clinical improvement documented for most of the patients, the significant decrease in GH and insulin-like growth factor 1 (IGF-1) [9][10][11][12] and shrinkage of the previously hyperplastic pituitary gland [7,12], the response of the GHRH-producing tumor and its metastases is less predictable and tumor size can increase despite suppression of the GH axis [5][6][7][8][9].…”