A non-acromegalic case of multiple endocrine neoplasia type 1 accompanied by a growth hormone-releasing hormone-producing pancreatic tumor View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2007-05

AUTHORS

H. Sugihara, T. Shibasaki, A. Tatsuguchi, F. Okajima, S. Wakita, Y. Nakajima, K. Tanimura, H. Tamura, S. Ishii, J. Kamegai, H. Akasu, W. Kitagawa, K. Shimizu, Y. Nakamura, E. Uchida, T. Tajiri, Z. Naito, H. Katakami, S. Oikawa

ABSTRACT

Cases of acromegaly due to GHRH-producing pancreatic endocrine tumors have been reported. Here we present a case of a 31-yr-old nonacromegalic man with hyperparathyroidism and elevated serum IGF-I with normal serum GH levels. Serum GH was not suppressed below 1 ng/ml by the glucose tolerance test and increased in response to TRH and GHRH administration. Magnetic resonance imaging (MRI) revealed pituitary hyperplasia and an abdominal computed tomography (CT) scan showed a tumor in the pancreatic tail. Plasma concentration of GHRH was elevated. Based on these clinical data, multiple endocrine neoplasia (MEN) type 1 was suspected. Three enlarged parathyroid glands were removed and a distal pancreatectomy was performed. Pathological examination of the parathyroid glands and pancreatic tumor showed nodular hyperplasia and a well-differentiated endocrine tumor, respectively, both compatible with MEN features. Immuno-histochemistry revealed positive immunoreactivity for GHRH, SS, insulin, glucagon, chromogranin A, and pancreatic polypeptide in the pancreatic tumor. After pancreatic surgery, elevated levels of GHRH and IGF-I were normalized and pituitary hyperplasia definitely decreased in size. In cases of pituitary hyperplasia with elevated IGF-I, ectopic GHRH syndrome must be considered even if physical features of acromegaly are absent. It is also important to measure plasma GHRH concentrations in order to give a diagnosis. More... »

PAGES

421-427

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/bf03346321

DOI

http://dx.doi.org/10.1007/bf03346321

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1006885568

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/17598976


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21 schema:description Cases of acromegaly due to GHRH-producing pancreatic endocrine tumors have been reported. Here we present a case of a 31-yr-old nonacromegalic man with hyperparathyroidism and elevated serum IGF-I with normal serum GH levels. Serum GH was not suppressed below 1 ng/ml by the glucose tolerance test and increased in response to TRH and GHRH administration. Magnetic resonance imaging (MRI) revealed pituitary hyperplasia and an abdominal computed tomography (CT) scan showed a tumor in the pancreatic tail. Plasma concentration of GHRH was elevated. Based on these clinical data, multiple endocrine neoplasia (MEN) type 1 was suspected. Three enlarged parathyroid glands were removed and a distal pancreatectomy was performed. Pathological examination of the parathyroid glands and pancreatic tumor showed nodular hyperplasia and a well-differentiated endocrine tumor, respectively, both compatible with MEN features. Immuno-histochemistry revealed positive immunoreactivity for GHRH, SS, insulin, glucagon, chromogranin A, and pancreatic polypeptide in the pancreatic tumor. After pancreatic surgery, elevated levels of GHRH and IGF-I were normalized and pituitary hyperplasia definitely decreased in size. In cases of pituitary hyperplasia with elevated IGF-I, ectopic GHRH syndrome must be considered even if physical features of acromegaly are absent. It is also important to measure plasma GHRH concentrations in order to give a diagnosis.
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28 GH levels
29 GHRH
30 GHRH administration
31 GHRH concentrations
32 IGF
33 Ss
34 TRH
35 abdominal computed tomography scan
36 acromegaly
37 administration
38 case of acromegaly
39 cases
40 clinical data
41 computed tomography scan
42 concentration
43 data
44 diagnosis
45 distal pancreatectomy
46 ectopic GHRH syndrome
47 elevated IGF
48 elevated levels
49 elevated serum IGF
50 endocrine tumors
51 examination
52 features
53 gland
54 glucagon
55 glucose tolerance test
56 hyperparathyroidism
57 hyperplasia
58 imaging
59 immuno-histochemistry
60 immunoreactivity
61 insulin
62 levels
63 magnetic resonance imaging
64 man’s features
65 men
66 multiple endocrine neoplasia type 1
67 nodular hyperplasia
68 order
69 pancreatectomy
70 pancreatic endocrine tumors
71 pancreatic polypeptide
72 pancreatic surgery
73 pancreatic tail
74 pancreatic tumors
75 parathyroid glands
76 pathological examination
77 physical features
78 pituitary hyperplasia
79 plasma GHRH concentration
80 plasma concentrations
81 polypeptide
82 positive immunoreactivity
83 resonance imaging
84 response
85 scans
86 serum GH
87 serum GH levels
88 serum IGF
89 size
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91 syndrome
92 tail
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95 tomography scan
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