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Review
. 2016 Jun 27:9:326.
doi: 10.1186/s13104-016-2132-1.

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

Sebastian Krug  1   2 , Michael Boch  1 , Peter Rexin  3 , Andreas Pfestroff  4 , Thomas Gress  1 , Patrick Michl  5 , Anja Rinke  1
Affiliations
Review

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

Sebastian Krug et al. BMC Res Notes. .

Abstract

Background: Pulmonary 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 presentation: We 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.

Conclusions: The 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.

Keywords: Acromegaly; GH; IGF-1; Paraneoplastic syndromes; Pulmonary neuroendocrine tumor.

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Figures

Fig. 1
Fig. 1
Representative image of the patient’s hand (left side) before surgery compared to a hand of one of our doctors
Fig. 2
Fig. 2
68Gallium-DOTATOC PET-CT before surgery revealed a large tumor mass located in the right lung with high somatostatin receptor expression. Isolated CT- and PET component (a left side and b) is presented as well as combined images (a right side). Metastatic spread was excluded as seen on the planar reconstruction (b right side)
Fig. 3
Fig. 3
Neuroendocrine tumor with typical growth pattern. Tumor cells show intermediate cytological pleomorphy and a distinct ‘‘pepper and salt’’ chromatin of the nucleolus as well as details of the so called “Zellballen” or nesting pattern (a). Antibodies directed against chromogranin a show a strong staining pattern (b) while synaptophysin is intermediately expressed (c). Immunohiostochemical staining with antibodies against Ki-67 indicate a low proliferation rate <2 % (d)
Fig. 4
Fig. 4
Abundant cytoplasmic protein expression of IGF-1 in the resected tumor specimen as detected by immunohistochemistry
See this image and copyright information in PMC

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