Hypoglycemia due to Ectopic Release of Insulin from a Paraganglioma

Uysal, M.; Temiz, Süleyman; Gül, Nurdan; Yarman, Sema; Tanakol, Refik; Kapran, Y

doi:10.1159/000099291

Cited by 15 publications

(13 citation statements)

References 42 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The diagnosis was biochemically proven, and at autopsy, the ovarian tumour exhibited secretory granules on electron microscopy and insulin immunoreactivity on immunohistochemistry, while there was a suggestion that this developed in the context of MEN-1 syndrome (Morgello et al 1988). Following that original report, a further case of an insulin-secreting NET of the cervix, and two paragangliomas, has also been described (Seckl et al 1999, Uysal et al 2007). …”

Section: Ectopic Insulinmentioning

confidence: 96%

Paraneoplastic syndromes secondary to neuroendocrine tumours

Kaltsas

Androulakis²,

Herder³

et al. 2010

Endocrine-Related Cancer

100

View full text Add to dashboard Cite

Neuroendocrine tumours may be either benign or malignant tumours, and have the ability to synthesise and secrete biologically active substances characteristic of the cell of origin that can cause distinct clinical syndromes. The term 'paraneoplastic syndromes' (PNSs) is used to denote syndromes secondary to substances secreted from tumours not related to their specific organ or tissue of origin and/or production of autoantibodies against tumour cells; such syndromes are mainly associated with hormonal and neurological symptoms. Appreciation of the presence of such syndromes is important as clinical presentation, if not identified, may delay the diagnosis of the underlying neoplasia. Conversely, early recognition can allow for more rapid diagnosis, particularly as the coexistence of a neoplasm with a clinical or biochemical marker offers an additional determinant of tumour status/progression. PNSs can complicate the patient's clinical course, response to treatment, impact prognosis and even be confused as metastatic spread. Their diagnosis involves a multidisciplinary approach, and detailed endocrinological, neurological, radiological and histological studies are required. Correct diagnosis is essential as the treatment of choice will be different for each disorder, particularly in the case of malignant tumours; it is therefore important to develop appropriate means to correctly identify and localise these tumours. Clinical awareness and the incorporation into clinical practise of 111 In-octreotide scintigraphy, chromogranin A and other evolving biochemical marker measurement techniques have substantially contributed to the identification of patients harbouring such syndromes. Diseasespecific medical therapies are mandatory in order to prevent recurrence and/or further tumour growth. Owing to their rarity, central registration of these syndromes is very helpful in order to be able to provide evidence-based diagnostic and therapeutic approaches.

show abstract

Section: Ectopic Insulinmentioning

confidence: 96%

Paraneoplastic syndromes secondary to neuroendocrine tumours

Kaltsas

Androulakis²,

Herder³

et al. 2010

Endocrine-Related Cancer

100

View full text Add to dashboard Cite

show abstract

“…Other mechanisms for NICTH are IGF1 tumor secretion (12), the production of autoantibodies to insulin (13) or its receptor (14), or more rarely, the secretion of glucagon-like peptide 1 (GLP1) (15,16), and massive tumor burden (17). Lastly, ectopic insulin secretion by a non-islet-cell tumor has also been exceptionally reported (18,19,20,21,22,23).…”

Section: Pathophysiology Of Tihmentioning

confidence: 99%

“…A small number of non-islet-cell tumors have been associated with ectopic insulin secretion so far (Table 1) (18,19,20,21,22,23). Among them are bronchial carcinoid tumor (18), squamous-cell carcinoma of the cervix (19), neurofibrosarcoma (65), schwannoma (20), paraganglioma (21,23), small-cell carcinoma of the cervix (22), and gastrointestinal stromal tumor (66).…”

Section: Ectopic Tumor Insulin Secretion: Non-islet-cell Tumorsmentioning

confidence: 99%

“…Among them are bronchial carcinoid tumor (18), squamous-cell carcinoma of the cervix (19), neurofibrosarcoma (65), schwannoma (20), paraganglioma (21,23), small-cell carcinoma of the cervix (22), and gastrointestinal stromal tumor (66). Although in some of these cases there were alternative mechanisms for hypoglycemia and the possibility of pancreatic insulinoma was not definitely excluded, other more recent clinical reports (22,23) have clearly shown that non-isletcell tumors were the origin of the hyperinsulinemia on the basis of the detection of proinsulin mRNA and insulin protein within the tumor cells.…”

Section: Ectopic Tumor Insulin Secretion: Non-islet-cell Tumorsmentioning

confidence: 99%

See 1 more Smart Citation

MANAGEMENT OF ENDOCRINE DISEASE: A clinical update on tumor-induced hypoglycemia

Iglesias

Díez

2014

European Journal of Endocrinology

140

144

View full text Add to dashboard Cite

Tumor-induced hypoglycemia (TIH) is a rare clinical entity that may occur in patients with diverse kinds of tumor lineages and that may be caused by different mechanisms. These pathogenic mechanisms include the eutopic insulin secretion by a pancreatic islet b-cell tumor, and also the ectopic tumor insulin secretion by non-islet-cell tumor, such as bronchial carcinoids and gastrointestinal stromal tumors. Insulinoma is, by far, the most common tumor associated with clinical and biochemical hypoglycemia. Insulinomas are usually single, small, sporadic, and intrapancreatic benign tumors. Only 5-10% of insulinomas are malignant. Insulinoma may be associated with the multiple endocrine neoplasia type 1 in 4-6% of patients. Medical therapy with diazoxide or somatostatin analogs has been used to control hypoglycemic symptoms in patients with insulinoma, but only surgical excision by enucleation or partial pancreatectomy is curative. Other mechanisms that may, more uncommonly, account for tumor-associated hypoglycemia without excess insulin secretion are the tumor secretion of peptides capable of causing glucose consumption by different mechanisms. These are the cases of tumors producing IGF2 precursors, IGF1, somatostatin, and glucagon-like peptide 1. Tumor autoimmune hypoglycemia occurs due to the production of insulin by tumor cells or insulin receptor autoantibodies. Lastly, massive tumor burden with glucose consumption, massive tumor liver infiltration, and pituitary or adrenal glands destruction by tumor are other mechanisms for TIH in cases of large and aggressive neoplasias.

show abstract

“…Uysel and colleagues described the case of a patient presenting with an insulin-secreting paraganglioma, while Innerman and colleagues reported a patient presenting with hypoglycaemia and a phaeochromocytoma metastatic to the liver. 8,9 The mechanism of hypoglycaemia is clear in the former case, while in the latter several explanations proposed by the authors included secretion of insulin or a substance with insulin-like activity by the tumour, decreased gluconeogenesis, disruption of glucagon metabolism, increased utilisation of glucose by the tumour and/or a local effect of the tumour on the hepatic parenchyma. Finally, the phaeochromocytoma in our patient was large, increasing the chance of malignancy, a diagnosis which can rarely be made…”

Section: Pm 252/06mentioning

confidence: 99%

Phaeochromocytoma crisis presenting with profound hypoglycaemia and subsequent hypertension

Frankton¹,

Baithun²,

Husain³

et al. 2009

View full text Add to dashboard Cite

A patient was presented with four days of vomiting, abdominal pain and sweating. At presentation the Capillary Blood Glucose (CBG) was 1.7 mmol/L, the Blood Pressure (BP) was 182/102 mmHg, and the pulse 100 bpm. on examination, he was sweaty, pale and cold. The initial differential diagnosis was hypoglycaemia secondary to insulin abuse, hypoadrenalism or insulinoma, the transient hypertension being considered a consequence of sympathetic stimulation. He remained clinically well overnight with a CBG of 10-14 mmol/L following intravenous glucose. The next morning he complained of nausea and abdominal pain. The BP had risen to 203/127 mmHg when he was later reviewed, having been given 10mg intramuscular metoclopramide. Shortly afterwards, he developed acute pulmonary oedema and had become hypoglycaemic again; a phaeochromocytoma crisis was suspected. Treatment with α-adrenoceptor blockade with intravenous phenoxybenzamine was advised. However, the patient deteriorated and died in the Intensive Care Unit within two hours. Autopsy examination confirmed a phaeochromocytoma in the left adrenal, with haemorrhage within the head of pancreas, but no evidence of a pancreatic tumour.

show abstract

Hypoglycemia due to Ectopic Release of Insulin from a Paraganglioma

Cited by 15 publications

References 42 publications

Paraneoplastic syndromes secondary to neuroendocrine tumours

Paraneoplastic syndromes secondary to neuroendocrine tumours

MANAGEMENT OF ENDOCRINE DISEASE: A clinical update on tumor-induced hypoglycemia

Phaeochromocytoma crisis presenting with profound hypoglycaemia and subsequent hypertension

Contact Info

Product

Resources

About