Recurrent Solitary Fibrous Tumor of the Pleura with Malignant Transformation and Non-islet Cell Tumor-induced Hypoglycemia due to Paraneoplastic Overexpression and Secretion of High-molecular-weight Insulin-like Growth Factor II

Abstract

A 41-year-old man was diagnosed with a solitary fibrous tumor (SFT) of the pleura in the posterior mediastinum. Despite two surgeries for excision, the SFT recurred and progressed with direct invasion of the chest wall and bone metastases. He was hospitalized because of cerebral infarction and presented with recurrent severe hypoglycemia fourteen years later. High-molecular-weight (HMW) insulin-like growth factor II (IGF-II) was identified in the serum and tumor using Western blotting and immunohistochemistry. These findings suggested that the cause of the recurrent severe hypoglycemia was SFT production of HMW IGF-II, a mediator of non-islet cell tumor-induced hypoglycemia (NICTH).Key words: solitary fibrous tumor of the pleura, non-islet cell tumor hypoglycemia, high-molecular-weight insulin-like growth factor II, platelet-derived growth factor receptor tyrosine kinase

Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor–2 from a malignant renal solitary fibrous tumor – clinical case and literature review

“…We reviewed case reports of NICTH associated with paraneoplastic production of IGF-2 from 2008 to 2012 (Table 3) [17-38]. On our review of literature, we identified 22 published reports of NICTH with elevated IGF-2 levels causing hypoglycemia.…”

Late recurrence of a malignant hypoglycemia-inducing pelvic solitary fibrous tumor secreting high-molecular-weight insulin-like growth factor-II: A case report with protein analysis

“…Low IGF-I levels in patients with NICTH are attributed to chronic attenuation of GH secretion, due to the negative feedback of IGF-II ( 9 ). Several studies have shown that NICTH due to HMW-IGF-II can be diagnosed using western blot analysis ( 10 – 12 ). Hata et al ( 10 ) examined HMW-IGF-II by using preoperative and postoperative blots and demonstrated that HMW-IGF-II disappeared postoperatively, which is considered to be a useful therapeutic index.…”

Retroperitoneal solitary fibrous tumor: surgery as first line therapy

“…Different criteria have been developed to assign malignant or benign classification to SFTs based on histologic features, age, location, size, sessile/pedunculated nature [ 5 , 18 ]. Nevertheless, SFTs classified as benign have been observed to undergo malignant transformation and recur at local and distant sites [ 8 , 9 , 12 , 17 , 27 ]. In view of this unpredictable behavior and the increased risk of retroperitoneal SFTs to recur than other SFTs, it is judicious to follow-up all cases of retroperitoneal SFTs irrespective of satisfying the criteria for benign tumors.…”

“…Retroperitoneal SFTs are a distinct sub-group of extra-pleural SFTs, often incidentally discovered during imaging for an unrelated pathology and present with non-specific symptoms, mainly abdominal pain, hip pain and urinary symptoms [ 6 ]. Extra-thoracic SFTs have been classically described as benign tumors but recent studies with larger numbers of cases and longer follow up have demonstrated the existence of a subgroup of tumors that exhibit a malignant clinical course with local and distant recurrence even after surgical excision [ 4 , 7 , 8 ]. Adverse outcomes are usually associated with atypical histological features (nuclear pleomorphism, increased cellularity, necrosis or mitoses greater than 4/10 HPF) and size greater than 10 cm [ 9 , 10 ].…”