Paraneoplastic syndromes and other systemic disorders associated with neuroendocrine neoplasms

Guilmette, Julie; Nosé, Vânia

doi:10.1053/j.semdp.2019.03.002

Cited by 14 publications

(9 citation statements)

References 175 publications

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“…NME is a complex lesion which is typically associated with glucagonoma and it is part of the paraneoplasic syndrome as seen in other pNETs widely ranging from tumour-related hypercalcemia or hypoglycemia, ectopic Cushing's syndrome to carcinoid syndrome, etc. (49). Lesions that are similar to NME have been reported in non-GS associated liver morbidities (for instance, cirrhosis), malnutrition and inflammatory bowel disease (50,51).…”

Section: Discussionmentioning

confidence: 91%

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

et al. 2020

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Glucagonoma is a hormonally active rare pancreatic neuroendocrine tumour causing an excess of glucagon. This is a narrative review based on a multidisciplinary approach of the tumour. Typically associated dermatosis is necrolytic migratory erythema (NME) which is most frequently seen at disease onset. Insulin-dependent diabetes mellitus, depression, diarrhoea, deep vein thrombosis are also identified, as parts of so-called 'D' syndrome. Early diagnosis is life saving due to potential aggressive profile and high risk of liver metastasis. NME as paraneoplastic syndrome may be present for months and even years until adequate recognition and therapy; it is remitted after successful pancreatic surgery. Thus the level of practitioners' awareness is essential. If surgery is not curative, debulking techniques may improve the clinical aspects and even the outcome in association with other procedures such as embolization of hepatic metastasis; ablation of radiofrequency type; medical therapy including chemotherapy, targeted therapy with mTOR inhibitors such as everolimus, PRRT (peptide receptor radiotherapy), and somatostatin analogues (including combinations of medical treatments). Increased awareness of the condition involves multidisciplinary practitioners.

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Section: Discussionmentioning

confidence: 91%

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

et al. 2020

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“…In this type of tumours the functional aspects are frequently positive thus the endocrine profile might help the early detection (63,64). Opposite to non-neuroendocrine tumours, the neuroendocrine paraneoplastic syndromes are associated with both benign and malignant neoplasia and they do not necessarily represents a poor prognostic marker (64,65). Somatostatinoma through the above mentioned presentation displays this scenario.…”

Section: Discussionmentioning

confidence: 98%

“…Duodenal NENs underline this pattern as well as a potential curable approach based on surgery especially in early stages and in well differentiated NENs; if possible, less aggressive surgical approach decreases the risk of complications (63). The level of awareness improves the overall survival due to early recognition and therapy (63,64). In this type of tumours the functional aspects are frequently positive thus the endocrine profile might help the early detection (63,64).…”

Section: Discussionmentioning

confidence: 99%

“…The level of awareness improves the overall survival due to early recognition and therapy (63,64). In this type of tumours the functional aspects are frequently positive thus the endocrine profile might help the early detection (63,64). Opposite to non-neuroendocrine tumours, the neuroendocrine paraneoplastic syndromes are associated with both benign and malignant neoplasia and they do not necessarily represents a poor prognostic marker (64,65).…”

Section: Discussionmentioning

confidence: 99%

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Somatostatinoma: Beyond neurofibromatosis type 1 (Review)

Şandru

Cârșote

Valea³

et al. 2020

Exp Ther Med

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Somatostatinoma is a tumour mainly originating from pancreas or duodenum; overall with an incidence of 1/40 million persons. We introduce a narrative review of literature of somatostatinoma including the relationship with neurofibromatosis type 1. Clinical presentation includes: Diabetes mellitus, cholelithiasis, steatorrhea, abdominal pain, and obstructive jaundice while papillary tumour may cause acute pancreatitis. The neoplasia may develop completely asymptomatic or it is detected as an incidental finding during an imaging or a surgical procedure. It may be sporadic or associated to genetic backgrounds especially for duodenal localisation as neurofibromatosis type 1 (NF1 gene with malfunction of RAS/MAPK pathway) or Pacak-Zhuang syndrome (EPAS1 gene encoding HIF). Surgery represents the central approach if feasible but the prognostic depends on location, and grading as indicated by WHO 2017 classification of neuroendocrine tumours. Previously known as Von Recklinghausen disease, neurofibromatosis type 1, the most frequent neurocutaneous syndrome, is an autosomal dominant disorder including: Café-au-lait spot, skin fold freckling on flexural zones, and neurofibromas as well as tumours such as gliomas of optic nerve, gastrointestinal stromal tumours (GISTs), iris hamartomas and brain tumours. Duodenal somatostatinoma is associated with the syndrome which actually involves more often a duodenal tumour of GIST type than a somatostatin secreting neoplasia. Other neuroendocrine tumours are reported: Gastrointestinal NENs at the level of rectum or jejunum and pheocromocytoma. Overall, one quarter of subjects have gastrointestinal tumours of different types. Somatostatinoma, when not located on pancreas but in duodenoum, may be registered in subjects with neurofibromatosis type 1 most probably in addition to other tumours. Overall, this type of neuroendocrine tumour with a challenging presentation has a poor prognosis unless adequate radical surgery is promptly offered to the patient.

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“…His height was −0.87 SDS, weight +0.54 SDS and body mass index (BMI) SDS was +1. 21. During the physical examination we observed facial plethora and fullness, lower limb edema, dorso-cervical fat pad ("buffalo hump"), hirsutism, obesity and hypertension (BP 135/80 mmHg, > 99 • pct).…”

Section: Case Reportmentioning

confidence: 99%

Ectopic ACTH Secretion in a Child With Metastatic Ewing's Sarcoma: A Case Report

et al. 2020

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Ectopic ACTH syndrome is rare in pediatric age. Sarcomas that cause Ectopic ACTH Syndrome (EAS) are even more uncommon. Currently, only three cases of EAS caused by Ewing' sarcoma have been reported. We detail a 10-year-old boy with Cushing's syndrome symptoms caused by ectopic ACTH production by a metastatic Ewing's sarcoma of the right ischio-pubic and ileo-pubic branches. The rapid appearance of cushingoid symptoms, with significant weight gain, acne, hirsutism, and hypercortisolism were implications of ectopic ACTH production as paraneoplastic Cushing's Syndrome. The very high levels of ACTH and non-suppression at the high dose dexamethasone test confirmed the clinical suspicion. We underline the possibility EAS was caused by an ACTH-secreting tumor, including soft tissue sarcomas.

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Paraneoplastic syndromes and other systemic disorders associated with neuroendocrine neoplasms

Cited by 14 publications

References 175 publications

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

Glucagonoma: From skin lesions to the neuroendocrine component (Review)

Somatostatinoma: Beyond neurofibromatosis type 1 (Review)

Ectopic ACTH Secretion in a Child With Metastatic Ewing's Sarcoma: A Case Report

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