An unusual cause of acromegaly

Lock, Karen; Lau, Ip Tim; Yeung, CK; Chan, C P

doi:10.12809/hkmj134044

Cited by 3 publications

(4 citation statements)

References 10 publications

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“…Single cases of neuroendocrine tumors causing acromegaly have been described with tumors deriving from duodenum [ 32 ], liver [ 33 ] and from the pancreas in the context of multiple endocrine neoplasia type 1 [ 9 , 34 , 35 ]. In addition, acromegaly due to GHRH hypersecretion in carcinoid tumors is the most common cause [ 27 , 36 , 37 ], whereas paraneoplastic GH secretion is extremely rare in NET [ 24 ].…”

Section: Discussionmentioning

confidence: 99%

Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature

et al. 2016

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BackgroundPulmonary neuroendocrine tumors (NET) form a heterogeneous group of rare diseases. In these tumors, paraneoplastic syndromes have been described to drive the course of the disease, among them acromegaly induced by paraneoplastic secretion of growth hormone-releasing hormone (GHRH).Case presentationWe report the case of a 43 years old patient initially diagnosed with acromegaly accompanied by weight gain and acral enlargement. Subsequently, further diagnostic work-up identified a solitary pulmonary neuroendocrine tumor (NET). Laboratory tests revealed markedly increased growth hormone (GH) and insulin-like growth factor 1 (IGF-1) without GHRH elevation in the absence of pituitary pathologies confirming the paraneoplastic origin of clinical presentation with acromegaly. Curative surgery was performed leading to normalization of the elevated hormone levels and improvement of the clinical symptoms. Immunohistochemically, a typical carcinoid (TC) was seen with low proliferation index and abundant IGF-1 expression.ConclusionsThe association of acromegaly and pulmonary NET has only rarely been reported. We present an individual case of paraneoplastic GH- and IGF-1 secretion in a patient with pulmonary NET. Based on their rarity, the knowledge of paraneoplastic syndromes occurring in patients with pulmonary NET such as acromegaly due to paraneoplastic GH- and IGF-1 secretion is mandatory to adequately diagnose and treat these patients.

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Section: Discussionmentioning

confidence: 99%

Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature

et al. 2016

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“…Moreover, better prognosis of acromegaly depends upon early recognition, diagnosis, and management. 5 A multidisciplinary management approach and close follow-up are thus necessary for acromegaly therapy. 14 …”

Section: Discussionmentioning

confidence: 99%

“…Most cases of acromegaly are because of a pituitary adenoma, which results in hypersecretion of GH and an elevated concentration of insulin-like growth factor 1 (IGF-1). 5 To the best of our knowledge, the simultaneous occurrence of both KS and acromegaly has never been reported in the literature. We herein report a rare case of a double syndrome involving both KS (47,XXY) and acromegaly and describe the clinical features, treatment, and final outcome.…”

Section: Introductionmentioning

confidence: 94%

Combination of Klinefelter Syndrome and Acromegaly

Fang

Wu³

et al. 2016

Medicine

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Klinefelter syndrome (KS) is the most common chromosomal aneuploidy in male population, which demonstrates an unusual association with acromegaly. We herein present a rare case involving the confirmation of KS 2 years after surgical treatment for acromegaly.A 27-year-old man presented with an acromegalic appearance. Endocrinological examination revealed a high growth hormone (GH) concentration, low testosterone concentration, and high follicle-stimulating hormone and luteinizing hormone concentration. Brain imaging revealed a 9 × 6 × 7− mm sellar low-density nodule suggestive of a microadenoma. Trans-sphenoidal surgery was undertaken, and immunohistochemistry revealed GH positivity. Two years after surgery, the patient underwent examination for infertility. He presented with diminished pubic hair, and small and firm testes. Hormonal assay revealed hypergonadotrophic hypogonadism on the basis of decreased serum total testosterone (<0.2 ng/mL), and elevated luteinizing hormone (14.71 mIU/mL) and follicle-stimulating hormone (21.8 mIU/mL). A chromosomal karyotype examination showed 47,XXY, confirming the diagnosis of KS. Replacement therapy with oral testosterone undecanoate was begun. Brain imaging showed no delayed enhancement in the saddle region of the pituitary gland, but the concentration of plasma insulin-like growth factor maintained a high level. The patient's GH concentration was not significantly suppressed by the GH glucose suppression test. In this consideration, he was referred for postoperative somatostatin analogue treatment to control GH hypersecretion.The misdiagnosis or delayed diagnosis of KS is mainly because of substantial variations in clinical presentation and insufficient professional awareness of the syndrome itself. As the simultaneous occurrence of KS and acromegaly is rare, and the association between them remains unclear, we suggest that complete pituitary hormonal screening and conventional pituitary MRI should be essential for patients with KS to screen for pituitary tumor.

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“…In a recent review, only 74 published cases were identified and except for a recent French series of 21 cases most were case reports . Tumours secreting GHRH are mainly NETs, usually welldifferentiated and of pancreatic or bronchial origin accounting for approximately 2/3 of cases (Fainstein et al 2007, Butler et al 2012, Lock et al 2014. The clinical presentation is variable with features similar to those caused from a somatotroph adenoma.…”

Section: Acromegalymentioning

confidence: 99%

Paraneoplastic endocrine syndromes

Dimitriadis

Angelousi

Weickert

et al. 2017

Endocrine-Related Cancer

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The majority of neoplasms are responsible for symptoms caused by mass effects to surrounding tissues and/or through the development of metastases. However, occasionally neoplasms, with or without endocrine differentiation, acquire the ability to secrete a variety of bioactive substances or induce immune cross-reactivity with the normal tissues that can lead to the development of characteristic clinical syndromes. These syndromes are named endocrine paraneoplastic syndromes when the specific secretory components (hormones, peptides or cytokines) are unrelated to the anticipated tissue or organ of origin. Endocrine paraneoplastic syndromes can complicate the patient's clinical course, response to treatment, impact prognosis and even be confused as metastatic spread. These syndromes can precede, occur concomitantly or present at a later stage of tumour development, and along with the secreted substances constitute the biological 'fingerprint' of the tumour. Their detection can facilitate early diagnosis of the underlying neoplasia, monitor response to treatment and/or detect early recurrences following successful initial management. Although when associated with tumours of low malignant potential they usually do not affect long-term outcome, in cases of highly malignant tumours, endocrine paraneoplastic syndromes are usually associated with poorer survival outcomes. Recent medical advances have not only improved our understanding of paraneoplastic syndrome pathogenesis in general but also enhanced their diagnosis and treatment. Yet, given the rarity of endocrine paraneoplastic syndromes, there is a paucity of prospective clinical trials to guide management. The development of well-designed prospective multicentre trials remains a priority in the field in order to fully characterise these syndromes and provide evidence-based diagnostic and therapeutic protocols.

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An unusual cause of acromegaly

Cited by 3 publications

References 10 publications

Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature

Acromegaly in a patient with a pulmonary neuroendocrine tumor: case report and review of current literature

Combination of Klinefelter Syndrome and Acromegaly

Paraneoplastic endocrine syndromes

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