Metastatic Pancreatic Neuroendocrine Tumor with Ectopic Adrenocorticotropic Hormone Production

Vaduganathan, Muthiah; Nagarur, Amulya; Kerr, Darcy A.; Lauter, Kelly; Padmanabhan, Arun; Raghavan, Srivatsan; Pallais, J. Carl; Fenves, Andrew Z.

doi:10.1080/08998280.2015.11929184

Cited by 4 publications

(2 citation statements)

References 21 publications

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“…In the second case [22] a 71-year old man got the same treatment but it was ineffective and adrenalectomy was performed, after which the patient died because of complications.…”

Section: динамическое изменение объема селезенки при актг-эктопированmentioning

confidence: 99%

Spleen Volume Evolution in Course of Acth-Ectopic Syndrome Due to Pancreatic Neuroendocrine Tumor Liver Metastases. A Case Report and Literature Review

Шориков¹,

Сергеева²,

Виршке³

et al. 2016

Rejr

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urpose. The paper is based on a seven-year surveillance of the patient showing ectopic adrenocorticotropic hormone (ACTH) syndrome. Materials and methods. A female 49-year old patient underwent pancreaticoduodenectomy for pancreatic head neuroendocrine tumor (NET) with synchronous liver metastases in 2009. Over time the metastatic lesions were gradually enlarging and conventional transarterial chemoembolization (cTACE) was performed twice: on December 2012 and on March 2014 in "on demand" regimen. Since 2013 the patient developed clinical signs suspicious for hypercortisolism but the elevated serum cortisol and ACTH levels were registered only on May 2015. ACTH production was proven to be ectopic by dexamethasone suppression test and it was a quite rare finding in a pancreatic NET. At the same time the spleen volume on MRI was evaluated as 126 cm3 which was almost two times lower than in 2009 (231 cm3).Results. With regard to occasional papers which mentioned cTACE as a method for hypercortisolism abatement the third cTACE was performed on June 2015. It resulted in tumor destruction, normalization of cortisol and ACTH levels and restoration of the spleen size, ongoing up to the date (12 months after the procedure). The retrospective MRI-based spleen volumetry demonstrated reduction of the spleen volume overall, and its increase in a period 1.5-12.0 months after each cTACE (r2=0.5, p<0.006). The spleen volume also correlated with ACTH levels (p<0.0001). Thought a remarkable spleen weight decrease after prolonged dexamethasone treatment has been shown on rodent models, PubMed search revealed no previous clinical reports focusing on splenic volume evolution depending on glucocorticoid levels.Conclusions. The presented case makes us hypothesize that the consistent pattern similar to that in the animal studies is also present in humans and, thus, microsplenia in cancer patients may serve as an indirect visualization sign of the ectopic ACTH production.Keywords: spleen, neuroendocrine tumors, chemoembolization, ACTH-ectopic syndrome, MRI, volumetry.

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“…In the second case [22] a 71-year old man got the same treatment but it was ineffective and adrenalectomy was performed, after which the patient died because of complications.…”

Section: динамическое изменение объема селезенки при актг-эктопированmentioning

confidence: 99%

Spleen Volume Evolution in Course of Acth-Ectopic Syndrome Due to Pancreatic Neuroendocrine Tumor Liver Metastases. A Case Report and Literature Review

Шориков¹,

Сергеева²,

Виршке³

et al. 2016

Rejr

View full text Add to dashboard Cite

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“…Comprehensive treatment, consisting of surgery, somatostatin analogues, chemotherapy, and targeted therapy are helpful to control hypercortisolemia in EAS-PNET patients, and reduce tumor burden, prolonging survival. 3 Individually tailored treatment by a multidisciplinary approach impacts on EAS-PNET patient care and should be considered for such patients.…”

mentioning

confidence: 99%

A Rare Cause of Severe Hypokalemia and Back Pain

Yang

Jin

2017

Gastroenterology

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Question: A 63-year-old man with a past medical history of hypertension presented with increasing lower extremity edema, persistent hypokalemia, and back pain for >2 months. Despite administration of spironolactone together with intravenous and oral potassium supplement, his clinical symptoms progressed, prompting admission to our institution. Physical examination showed prominent bilateral pitting lower extremity edema up to his knees. Laboratory results showed potassium 2.9 mmol/L (normal, 3.5-5.3), Na þ 150 mmol/L (normal, 137-147), Ca 2þ 1.89 mmol/L (normal, 2.1-2.6),

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Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances

Guilmette

Nosé

2019

Advances in Anatomic Pathology

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This review focuses on discussing the main modifications of the recently published 2017 WHO Classification of Neoplasms of the Neuroendocrine Pancreas (panNEN). Recent updates separate pancreatic neuroendocrine tumors into 2 broad categories: well-differentiated pancreatic neuroendocrine tumors (panNET) and poorly differentiated pancreatic neuroendocrine carcinoma (panNEC), and incorporates a new subcategory of "well-differentiated high-grade NET (G3)" to the well-differentiated NET category. This new classification algorithm aims to improve the prediction of clinical outcomes and survival and help clinicians select better therapeutic strategies for patient care and management. In addition, these neuroendocrine neoplasms are capable of producing large quantity of hormones leading to clinical hormone hypersecretion syndromes. These functioning tumors include, insulinomas, glucagonomas, somatostatinomas, gastrinomas, VIPomas, serotonin-producing tumors, and ACTH-producing tumors. Although most panNENs arise as sporadic diseases, a subset of these heterogeneous tumors present as parts on inherited genetic syndromes, such as multiple endocrine neoplasia type 1, von Hippel-Lindau, neurofibromatosis type 1, tuberous sclerosis, and glucagon cell hyperplasia and neoplasia syndromes. Characteristic clinical and morphologic findings for certain functioning and syndromic panNENs should alert both pathologists and clinicians as appropriate patient management and possible genetic counseling may be necessary.

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Metastatic Pancreatic Neuroendocrine Tumor with Ectopic Adrenocorticotropic Hormone Production

Cited by 4 publications

References 21 publications

Spleen Volume Evolution in Course of Acth-Ectopic Syndrome Due to Pancreatic Neuroendocrine Tumor Liver Metastases. A Case Report and Literature Review

Spleen Volume Evolution in Course of Acth-Ectopic Syndrome Due to Pancreatic Neuroendocrine Tumor Liver Metastases. A Case Report and Literature Review

A Rare Cause of Severe Hypokalemia and Back Pain

Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances

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