Ectopic Acromegaly Due to a Pancreatic Neuroendocrine Tumor Producing Growth Hormone-Releasing Hormone

2011 ◽  
Vol 17 (1) ◽  
pp. 79-84 ◽  
Author(s):  
Danielle Weiss ◽  
Hannes Vogel ◽  
M. Lopes ◽  
Steven Chang ◽  
Laurence Katznelson
Keyword(s):  
Growth Hormone ◽  
Neuroendocrine Tumor ◽  
Pancreatic Neuroendocrine Tumor ◽  
Growth Hormone Releasing Hormone ◽  
Releasing Hormone ◽  
Ectopic Acromegaly

Ectopic Growth Hormone-Releasing Hormone Secretion by a Neuroendocrine Tumor Causing Acromegaly: Long-Term Follow-Up Results

2009 ◽  
Vol 20 (2) ◽  
pp. 127-132 ◽  
Author(s):  
Neşe Colak Ozbey ◽  
Yersu Kapran ◽  
Alp Bozbora ◽  
Yeşim Erbil ◽  
Cemil Tascioglu ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Neuroendocrine Tumor ◽  
Hormone Secretion ◽  
Growth Hormone Releasing Hormone ◽  
Releasing Hormone ◽  
Long Term Follow Up

Subcutaneous octreotide treatment of a growth hormone-releasing hormone-secreting bronchial carcinoid: superiority of continuous versus intermittent administration to control hormonal secretion

1995 ◽  
Vol 133 (3) ◽  
pp. 320-324 ◽  
Author(s):  
Sophie Lefebvre ◽  
Lutgarde De Paepe ◽  
Roger Abs ◽  
Jacques Rahier ◽  
Philippe Selvais ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Bronchial Carcinoid ◽  
Growth Hormone Releasing Hormone ◽  
Releasing Hormone ◽  
Intermittent Administration ◽  
Hormonal Secretion ◽  
Igf I ◽  
Octreotide Treatment ◽  
Serum Gh ◽  
Ectopic Acromegaly

Lefebvre S, De Paepe L, Abs R, Rahier J, Selvais P, Maiter D. Subcutaneous octreotide treatment of a growth hormone-releasing hormone-secreting bronchial carcinoid: superiority of continuous versus intermittent administration to control hormonal secretion. Eur J Endocrinol 1995;133:320–4. ISSN 0804–4643 Diagnosis of ectopic acromegaly was made in a 21-year-old female patient who 3 years before had undergone a right pneumectomy for a disseminated bronchial carcinoid. Plasma growth hormonereleasing hormone (GHRH) concentrations were markedly elevated (6440 ng/l; normal value <100 ng/l), as were serum GH (187 μg/l; normal <5 μg/l) and plasma insulin-like growth factor I (IGF-I) levels (6.7 U/ml; normal <2 U/ml). Retrospective immunohistochemical examination of the carcinoid tumor was positive for GHRH and the tumoral content of GHRH was 2130 ng/g wet weight. Subcutaneous treatment with octreotide was begun and first resulted in a profound inhibition of GH hypersecretion, normalization of plasma IGF-I and only partial reduction of GHRH concentrations. However, the initial dose of 3 × 100 μg had to be increased gradually to 4 × 750 μg because of a progressive deterioration of the hormonal control. After 15 months of intermittent therapy, octreotide was administered by continuous sc infusion. This treatment improved compliance, allowed the daily dose of octreotide to be reduced to 1500 μg and normalized serum GH levels. A near-normalization of the plasma IGF-I concentrations was also obtained, whereas the suppression of plasma GHRH concentrations remained incomplete. Despite favorable evolution of the endocrine parameters, intramedullar metastases were diagnosed and required radiation therapy. This observation emphasizes the superiority of continuous over intermittent administration of octreotide in the treatment of ectopic acromegaly. It also shows that the somatostatin analog acts more at the pituitary level to inhibit GH secretion than at the site of the neuroendocrine tumor. S Lefebvre, Division of Rheumatology, Clinique du Refuge, Rue du Couvent 39, B-7700 Mouscron, Belgium

scholarly journals Diagnostic challenges and management of a patient with acromegaly due to ectopic growth hormone-releasing hormone secretion from a bronchial carcinoid tumour

2017 ◽  
Vol 2017 ◽  
Author(s):  
Nikolaos Kyriakakis ◽  
Jacqueline Trouillas ◽  
Mary N Dang ◽  
Julie Lynch ◽  
Paul Belchetz ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Carcinoid Tumour ◽  
Common Finding ◽  
Diagnostic Tools ◽  
Oral Glucose ◽  
List Type ◽  
Bronchial Carcinoid ◽  
Growth Hormone Releasing Hormone ◽  
Releasing Hormone ◽  
Ectopic Acromegaly

Summary A male patient presented at the age of 30 with classic clinical features of acromegaly and was found to have elevated growth hormone levels, not suppressing during an oral glucose tolerance test. His acromegaly was originally considered to be of pituitary origin, based on a CT scan, which was interpreted as showing a pituitary macroadenoma. Despite two trans-sphenoidal surgeries, cranial radiotherapy and periods of treatment with bromocriptine and octreotide, his acromegaly remained active clinically and biochemically. A lung mass was discovered incidentally on a chest X-ray performed as part of a routine pre-assessment for spinal surgery 5 years following the initial presentation. This was confirmed to be a bronchial carcinoid tumour, which was strongly positive for growth hormone-releasing hormone (GHRH) and somatostatin receptor type 2 by immunohistochemistry. The re-examination of the pituitary specimens asserted the diagnosis of pituitary GH hyperplasia. Complete resolution of the patient’s acromegaly was achieved following right lower and middle lobectomy. Seventeen years following the successful resection of the bronchial carcinoid tumour the patient remains under annual endocrine follow-up for monitoring of the hypopituitarism he developed after the original interventions to his pituitary gland, while there has been no evidence of active acromegaly or recurrence of the carcinoid tumour. Ectopic acromegaly is extremely rare, accounting for <1% of all cases of acromegaly. Our case highlights the diagnostic challenges differentiating between ectopic acromegaly and acromegaly of pituitary origin and emphasises the importance of avoiding unnecessary pituitary surgery and radiotherapy. The role of laboratory investigations, imaging and histology as diagnostic tools is discussed. Learning points: Ectopic acromegaly is rare, accounting for less than 1% of all cases of acromegaly. Ectopic acromegaly is almost always due to extra-pituitary GHRH secretion, mainly from neuroendocrine tumours of pancreatic or bronchial origin. Differentiating between acromegaly of pituitary origin and ectopic acromegaly can cause diagnostic challenges due to similarities in clinical presentation and biochemistry. Serum GHRH can be a useful diagnostic tool to diagnose ectopic acromegaly. Pituitary imaging is crucial to differentiate between a pituitary adenoma and pituitary hyperplasia, which is a common finding in ectopic acromegaly. Diagnosing ectopic acromegaly is pivotal to avoid unnecessary interventions to the pituitary and preserve normal pituitary function.

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