Ectopic insulin production by a primary ovarian carcinoid

Morgello, Susan; Schwartz, Ernest; Horwith, Melvin; King, Mary E.; Görden, Phillip; Alonso, Daniel R.

doi:10.1002/1097-0142(19880215)61:4<800::aid-cncr2820610426>3.0.co;2-3

Cited by 35 publications

(19 citation statements)

References 17 publications

(1 reference statement)

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“…A further case of ectopic insulin production from an apparently primary ovarian carcinoid tumour associated with episodic hyperinsulinaemic hypoglycaemia has also been described (Morgello et al 1988). 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 Insulinsupporting

confidence: 54%

“…The hepatic origin of hyperinsulinism was demonstrated by selective arterial calcium stimulation and insulin and C-peptide immunoreactivity by the tumour cell (Furrer et al 2001). A further case of ectopic insulin production from an apparently primary ovarian carcinoid tumour associated with episodic hyperinsulinaemic hypoglycaemia has also been described (Morgello et al 1988). 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).…”

Section: Ectopic Insulinmentioning

confidence: 99%

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Paraneoplastic syndromes secondary to neuroendocrine tumours

Kaltsas

Androulakis²,

Herder³

et al. 2010

Endocrine-Related Cancer

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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.

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Section: Ectopic Insulinsupporting

confidence: 54%

Section: Ectopic Insulinmentioning

confidence: 99%

Paraneoplastic syndromes secondary to neuroendocrine tumours

Kaltsas

Androulakis²,

Herder³

et al. 2010

Endocrine-Related Cancer

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“…report on "ectopic" neuroendocrine tumor in a patient with possible Men1 [18]. therefore, we examined if MEN1 gene had involved in tumor development of our patient, and found that both MEN1 alleles are functionally lost by germline-and somatic mutations.…”

Section: Discussionmentioning

confidence: 99%

“…As far as we are aware, there has been only one article reporting Men1 case with "carcinoid tumor" occurred outside of common organs (e.g. thymus, bronchus, stomach and duodenum) [18]. Morgello et al reported a 63-year-old woman who had presented insulin overproduction from an ovarian carcinoid.…”

Section: Genetic Analysismentioning

confidence: 99%

Unusual Clinical and Pathological Presentation of a Neuroendocrine Tumor in a Patient with Multiple Endocrine Neoplasia Type 1

et al. 2009

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Abstract. neuroendocrine tumors develop in various organs in patients with multiple endocrine neoplasia type 1 (Men1). Among those, tumors developed in upper gastrointestinal tract, thymus and bronchus have historically been called "carcinoid tumor". occurrence of "carcinoid tumor" in other region is very rare and molecular pathogenesis of such tumors is unknown. We have experienced a patient with Men1 who have developed an "ectopic" retroperitoneal neuroendocrine tumor. Genetic analysis of the MEN1 gene in tumor cells revealed a somatic mutation in exon 9 as well as a germline mutation in exon 10. Allele-specific amplification followed by sequence analysis revealed these two mutations exist on the different allele, indicating both alleles are functionally inactivated. immunohistochemical staining with an anti-menin antibody revealed that wild-type menin is not expressed in tumor cells. expression of p27 kip1 protein is not observed in tumor cells, either. These results confirmed the inactivation of the MEN1 gene as a genetic cause of an ectopically developed neuroendocrine tumor in a patient with Men1. [4,5] and inactivation of rat Men1 gene causes reduction of p27 kip1 expression, which may partly explains molecular mechanism of tumorigenesis due to MEN1 inactivation [6,7]. Subsequently, homozygous germline mutation of the Cdkn1b, encoding p27 kip1 , was identified in the rat with MenX, a recessive Men-like syndrome [8]. rats with MenX manifest phenotypic features overlapping both Men1 and Men2 including parathyroid adenoma, C cell hyperplasia of thyroid and pheochroReceived Apr. 30, 2009; Accepted Jun. 10, 2009 as K09E-126 Released online in J-STAGE as advance publication Jun. 30, 2009 Correspondence to: Akihiro SAkUrAi M.d., Ph.d., division of Medical Genetics, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621 Japan. e-mail: aksakura@shinshu-u.ac.jp *1 Alternative splicing at exon 2 of the MEN1 gene produces two isoforms of menin, 610 and 615 amino acids in length, respectively. numbering of amino acid and nucleotide in this manuscript is based on that for 615 amino acid protein.

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“…in duodenum or small intestine) are extremly rare (Service et al, 1991(Service et al, , Škrha, 2001. One case report describes the insulin producing carcinoid of ovary (Morgello et al, 1988). The most of insulinoma cases are benign whereas malignant forms have been described in 5 to 10 % of patients (Perry & Vinik, 1995, Service et al 1991.…”

Section: Epidemiology and Basic Characteristicsmentioning

confidence: 99%