Ovarian tumors secreting insulin

Abstract

Combined ovarian germ cell and neuroendocrine tumors are rare. Only few cases of hyperinsulinism due to ovarian ectopic secretion have been hypothesized in the literature. An ovarian tumor was diagnosed in a 76-year-old woman, referred to our department for recurrent hypoglycemia with hyperinsulinism. In vivo tests, in particular fasting test, rapid calcium infusion test, and Octreotide test were performed. Ectopic hyperinsulinemic hypoglycemia was demonstrated in vivo and hypoglycemia disappeared after hysteroadnexectomy. Histological exam revealed an ovarian germ cell tumor with neuroendocrine and Yolk sac differentiation, while immunostaining showed insulin positivity in neuroendocrine cells. A cell culture was obtained by tumoral cells, testing Everolimus, and Pasireotide. Insulin was detected in cell culture medium and Everolimus and Pasireotide demonstrated their potentiality in reducing insulin secretion, more than controlling cell viability. Nine cases of hyperinsulinism due to ovarian ectopic secretion reported in literature have been reviewed. These data confirm the ovarian tissue potentiality to induce hyperinsulinemic hypoglycemic syndrome after neoplastic transformation.

Yolk sac tumours of the female genital tract in older adults derive commonly from somatic epithelial neoplasms: somatically derived yolk sac tumours

“…11,12 However, it should also be noted that there have been rare reports of a carcinoid tumour arising in a YST in younger patients. 13,14 A B C D Apart from the two cases associated with immature teratoma, one of the pure YSTs and the two with a hepatoid appearance, in all cases in our series the YST exhibited a predominantly glandular morphology (often also with solid, papillary and cystic elements) which resulted in close mimicry of a variety of primary ovarian adenocarcinomas, especially endometrioid, clear cell and high-grade serous. Glandular variants of YST are well described where there is close mimicry to an endometrioid adenocarcinoma (sometimes with cytoplasmic supranuclear or subnuclear vacuolation mimicking a secretory endometrioid carcinoma) or an intestinal type adenocarcinoma.…”

“…This raises the possibility that, rarely, YSTs in older patients may derive from a neuroendocrine carcinoma; the latter, when occurring as primary ovarian neoplasms, often arise in association with an ovarian carcinoma . However, it should also be noted that there have been rare reports of a carcinoid tumour arising in a YST in younger patients …”

Ectopic insulinoma: case report

“…However, the case report caused controversy. Third, other extrapancreatic tumors have been reported to secrete insulin and cause hyperinsulinemic hypoglycemia, including ovarian tumors, cervical carcinoids, kidney carcinoids, paragangliomas and liver neuroendocrine tumors [14, 15]. In this study, these tumors were not classified as an ectopic insulinoma.…”

Phaeochromocytomas overexpress insulin transcript and produce insulin

“…Generally, tumours with ectopic hormone production often lack normal regulatory mechanism of hormone secretion (39). This is also shown for insulin producing tumours causing hypoglycaemia (10,35). Therefore, if a…”