Non-islet-cell tumour hypoglycaemia (NICTH): About a series of 6 cases.

The purpose of this study was to analyse the characteristics of 6 patients managed in a university hospital between 1996 and 2016 for non-islet cell tumor hypoglycemia (NICTH), a form of hypoglycaemia due to the paraneoplastic secretion of IGF-2 or its related substances. RESULTS: Three of these 6 patients (50%), aged over 69 years, including 2 with acromegaloid phenotype, presented with a pleural solitary fibrous tumor (SFT), with median diameter 20 cm (interquartile range, 12.5-20.5) with a low median SUV (3.3 g/mL (QR, 2-7.5)) on 18F-FDG PET. The other 3 patients presented respectively neuroendocrine carcinoma (NEC) of the palate (70-year-old woman), retroperitoneal myxofibrosarcoma (66-year-old man) and meningeal hemangiopericytoma (36-year-old woman). All 3 were inoperable and did not respond to any therapy other than glucose solution. Corticosteroid therapy was effective in the 3 SFTs and the NEC. One of the SFTs recurred 10 years later with asymptomatic hypoglycemia, which resolved after reintervention. Median (IQR) blood glucose levels of the 6 patients was 0.4g/L (QR, 0.31-0.41), with hypoinsulinemia at 0.7mIU/L (QR 0.7-2.0), undetectable GH, low IGF-1, normal IGF-2 level in 5/6 cases, a high IGF-2:IGF-1 ratio at 26.9 (QR, 20.8-37.8), hypokalemia and hypomagnesemia. CONCLUSION: NICTH is a rare syndrome, which should be considered in the presence of hypoinsulinemic hypoglycemia with low GH and IGF-1, and a IGF-2:IGF-1 ratio>10. Corticosteroid therapy was effective in elderly subjects, particularly with solitary fibrous tumor, which was generally operable. Hemangiopericytoma and myxofibrosarcoma had poor prognosis in younger patients.

Serum ß-hCG as an Indicator of Recurrence After the Complete Resection of a Malignant Solitary Fibrous Tumor of the Pleura.

In solitary fibrous tumors (SFTs) of the pleura, malignant SFTs are uncommon. Although SFTs are known to cause paraneoplastic syndromes through the production of insulin-like growth factor, to the best of our knowledge, the production of beta-human chorionic gonadotropin (ß-hCG) has been reported only in 1 case involving a patient with a benign SFT. We herein report the first case of the elevation of ß-hCG serum levels associated with a malignant SFT in which the ß-hCG serum level became a useful indicator of recurrence after the complete resection of the primary mediastinal lesion.

A rare case of ß-hCG production by a solitary fibrous tumor of the pleura.

BACKGROUND: Solitary fibrous tumors are rare tumors of mesenchymal origins, most commonly seen arising from the pleural lining of the lungs. These are generally benign tumors, which in rare cases have been identified to be associated with multiple para-neoplastic syndromes. CASE REPORT: This is a case of a solitary fibrous tumor of the pleura in a 49 year old female which was found to be associated with elevated levels of serum beta human chorionic gonadotropin ß-hCG). Due to the lack of plausible causes for elevated ß-hCG in the patient, immune-histochemical staining of the tumor specimen for ß-hCG was obtained. This confirmed the patient's solitary fibrous tumor as the source of the ß-hCG. The patient was also found to have a possible paraneoplastic syndrome with irregular menstruation and hot flushes from the secreted ß-hCG. CONCLUSIONS: This is the first reported case of solitary fibrous tumors of the pleura producing {b-hCG. Multiple types of lung tumors have been associated with production of ß-subunit of human chorionic gonadotropin. Production of ß-hCG by these tumors has been associated with a poor prognosis. In this case, we find an aggressive form of solitary fibrous tumor associated with production of ß-hCG and associated paraneoplastic syndrome secondary to the ß-hCG. Further study is required to identify the frequency of this phenomenon and the implications of ß-hCG production in the prognosis of the solitary fibrous tumors.