Ann Thorac Surg 2008;85:330-332. doi:10.1016/j.athoracsur.2007.06.072
© 2008 The Society of Thoracic Surgeons
Case Reports
Acromegaly Caused by Ectopic Growth Hormone: A Rare Manifestation of a Bronchial Carcinoid
Suvakanta Biswal, MCha,
Balamurali Srinivasan, MCha,*,
Pinaki Dutta, DMb,
Prabat Ranjan, MDc,
Kim Vaiphei, MDc,
Rana Sandeep Singh, MCha,
Shyamkumar Singh Thingnam, MCha
a Department of Cardiothoracic and Vascular Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
b Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
c Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
Accepted for publication June 25, 2007.
* Address correspondence to Dr Srinivasan, Department of CVTS, PGIMER, Chandigarh, 160012, India (Email: eazzyryder{at}yahoo.com).
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Abstract
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Ectopic acromegaly due to growth hormone-releasing hormone secretion by a bronchial carcinoid is rare. We report a case of bronchial carcinoid presenting with acromegaly due to ectopic growth hormone production. The patient was treated successfully with right pneumonectomy.
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Introduction
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Acromegaly is caused in the vast majority of cases by a pituitary adenoma. Ectopic acromegaly is rare (<1% of cases), and it is caused by ectopic growth hormone-releasing hormone (GHRH) or growth hormone (GH) secreting tumors. Ectopic GHRH production is a more common cause for ectopic acromegaly than ectopic GH [1]. The most common tumors secreting ectopic GHRH or GH are bronchial carcinoids and pancreatic islet cell tumor [2].
A 53-year-old woman presented with complaints of cough with hemoptysis for the past 1 year. She had noticed progressive enlargement of her hands and feet and coarsening of facial features during the last 6 months. Physical examinations revealed features of acromegaly (Fig 1), and decreased breath sounds on the left side of the chest. There was no evidence of cardiovascular disease. Examination of the eye showed no visual field defects.
A chest roentgenogram showed a mass lesion in the left lower lobe of the lung. Computed tomography (CT) showed large lobulated, soft tissue density in the left lower zone lung with contrast enhancement and patchy calcification (Fig 2). Fiberoptic bronchoscopic examination revealed a left lower lobe main bronchial growth extending up to 1 cm of carina. Bronchoscopic biopsy and cytologic study confirmed a carcinoid tumor. Immunostaining for chromogranins showed focal positivity. Magnetic resonance imaging scans of the head demonstrated no enlargement of the pituitary (Fig 3). A preoperative growth hormone suppression test using glucose load showed elevated growth hormone levels (41.60 ng/mL [normal, 0.5 to 5 ng/mL]).
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Fig 2. Computed tomographic scan of the chest showing a large lobulated mass in the left lower zone lung.
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The patient subsequently underwent a left pneumonectomy. The postoperative course was uneventful. Four weeks after surgery, the GH levels had normalized (0.8 ng/mL). Histopathologic examination of the resected specimen revealed a tumor showing classic features for a typical carcinoid (Fig 4). Immunostaining for GH was strongly positive within the tumor cells (Fig 5). Three months after surgery her GH levels remained normal (2.56 ng/mL) and the acromegalic features were still regressing.
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Comment
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Most patients with bronchial carcinoids present with pulmonary symptoms like cough, hemoptysis, and recurrent chest infections. Paraneoplastic syndromes are uncommon and occur in about 2% of cases [3]. Bronchial carcinoids are the most common cause for ectopic GHRH production [2, 3]. Apart from bronchial carcinoids and pancreatic islet cell tumors, other tumors such as small-cell lung cancers, adrenal adenomas, and pheochromocytomas have also been reported to secrete GHRH [4]. However, many carcinoids and other tumors producing ectopic GHRH do not cause overt acromegaly because of the low levels of the ectopic hormones produced and their short half lives [5]. Ectopic GH secretion is a less common cause than ectopic GHRH for ectopic acromegaly. Reports of ectopic GH-producing pancreatic islet cell tumors and ectopic pituitary adenomas exist, but we could not find any report in the literature of a bronchial carcinoid causing acromegaly due to ectopic GH production [6, 7]. Commonly, bronchial carcinoids with ectopic GH and GHRH production are generally small (ie, approximately 2 to 4 cm in diameter) [5]. However, in our patient the mass lesion in the chest was large and readily revealed the probable source of ectopic GH.
The dynamics of ectopic GH secretion in ectopic acromegaly is characterized by high basal GH levels, which is not suppressed by glucose load, and by low or undetectable plasma GHRH levels. This hormonal profile is similar to that of acromegaly due to a pituitary adenoma. However, ectopic acromegaly due to ectopic GHRH is characterized by increased plasma GHRH levels (ie, 100 to 300 fold) [8]. Plasma GHRH estimation was not done in this patient as the assay was not available at our institute. However, a normal pituitary gland on magnetic resonance imaging, combined with laboratory evidence of GH hypersecretion, and positive immunostaining for GH in tumor tissue will constitute sufficient evidence for diagnosing an ectopic acromegaly caused by ectopic GH production. We believe that we are the first to report such a case from a bronchial carcinoid. Normal magnetic resonance imaging also ruled out a coexisting pituitary adenoma as carcinoids can present as a part of a more widespread pluriglandular syndrome [3]. Complete surgical excision is the treatment of choice, and it usually leads to normalization of GH levels and regression of acromegalic features, which was also the case with our patient. Residual, recurrent, and inoperable lesions have been successfully treated with octreotide and its analogues [8].
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References
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- Faglia G, Arosio M, Bazzoni N. Ectopic acromegaly Endocrinol Metab Clin North Am 1992;21:575-595.[Medline]
- Melmed S. Extrapituitary acromegaly Endocrinol Metab Clin North Am 1991;20:507-518.[Medline]
- Hage R, de la Riviere AB, Seldenrijk CA, van den Bosch JM. Update in pulmonary carcinoid tumors: a review article Ann Surg Oncol 2003;10:697-704.[Medline]
- Doga M, Bonadonna S, Burattin A, Giustina A. Ectopic secretion of growth hormone-releasing hormone (GHRH) in neuroendocrine tumors: relevant clinical aspects Ann Oncol 2001;12(Suppl 2):S89-S94.[Abstract]
- Huber RM, Schopohl J, Losa M, et al. Growth-hormone releasing hormone in a bronchial carcinoid Cancer 1991;67:2538-2542.[Medline]
- Melmed S, Ezrin C, Kovacs K, Goodman RS, Frohman LA. Acromegaly due to secretion of growth hormone by an ectopic pancreatic islet-cell tumor N Engl J Med 1985;312:9-17.[Medline]
- Hori E, Akai T, Kurimoto M, Hirashima Y, Endo S. Growth hormone-secreting pituitary adenoma confined to the sphenoid sinus associated with a normal-sized empty sella J Clin Neurosci 2002;9:196-199.[Medline]
- Drange MR, Melmed S. Long-acting lanreotide induces clinical and biochemical remission of acromegaly caused by disseminated growth hormone-releasing hormone-secreting carcinoid J Clin Endocrinol Metab 1998;83:3104-3109.[Abstract/Free Full Text]
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Abstract
Introduction
