Non-islet Cell Tumor Hypoglycemia Research Articles

Imatinib use in the management of a patient with Doege-Potter syndrome.

Doege-Potter syndromeis a paraneoplastic syndrome characterized by nonislet cell tumor hypoglycemia due to a solitary fibrous tumor, which produces insulin-like growth factor II. In this report, we present the case of a 67-year-old male with recurrent and refractory hypoglycemia due to DPS successfully treated with imatinib. He initially presented with neuroglycopenic symptoms and dyspnea secondary to a giant tumor in the left hemithorax, which was totally resected. During follow-up, 7 years later, he presented with thoracoabdominal tumor recurrence associated with severe hypoglycemia and underwent subtotal tumor resection, with a subsequent improvement of symptoms. The following year, he had a recurrence of his intra-abdominal tumor, which was unresectable, associated with severe hypoglycemia refractory to dextrose infusion and corticosteroids, thus receiving imatinib with a favorable response. The clinical presentation, diagnostic approach, progression of the disease, and response to treatment with imatinib in the management of a patient with large, recurrent, and unresectable mesenchymal tumors with insulin-like growth factor-2 secretion causing hypoglycemiahighlight the importance of this case report. Doege-Potter syndrome (DPS) is a rare cause of tumoral hypoglycemia of non-pancreatic origin. Some malignant or benignant neoplasms have ectopic secretion of insulin-like growth factor-2. Total surgical removal is the principal treatment in patients with DPS. Tyrosine kinase inhibitors management in DPS may contribute to improved tumor control in patients with unresectable tumors and severe hypoglycemia.

Surgical resection of a retroperitoneal liposarcoma producing insulin-like growth factor II: a case report

BackgroundTumor-produced high molecular weight insulin-like growth factor-II (big insulin-like growth factor-II) is considered to cause non-islet cell tumor hypoglycemia. This paper presents a case of surgically resected retroperitoneal liposarcoma that produced big insulin-like growth factor-II.Case presentationHere, we report the case of a 62-year-old woman who presented with an abdominal mass and hypoglycemia. Non-islet cell tumor hypoglycemia due to retroperitoneal liposarcoma was suspected. After complete resection of the tumor, the patient’s hypoglycemia improved and big insulin-like growth factor-II disappeared in the molecular weight analysis of serum insulin-like growth factor-II by western blotting. The patient had no tumor recurrence or reappearance of hypoglycemia 16 months after the operation without any adjuvant therapy.ConclusionsAlthough insulin-like growth factor-II-producing tumors are generally large and difficult to operate on, surgical resection is currently the most effective and only treatment; thus, it is essential to attempt resection aggressively.

PSUN329 Non-islet Cell Tumor Hypoglycemia Secondary to Advanced Hepatocellular Carcinoma in a 29-Year-Old Male

Abstract Summary: Non-islet cell tumor hypoglycemia (NICTH) is a rare, but serious complication that can be seen in most all tumors. It is typically mediated by high levels of an incompletely processed form of insulin-growth factor 2 ("big IGF-2") that is produced by the tumor. We describe a case of a 29-year-old male with history of chronic hepatitis B and advanced stage hepatocellular carcinoma (HCC) who had failed prior chemotherapies. The patient was admitted to the hospital for a liver biopsy, following which he developed severe hypoglycemia, refractory to high dose, continuous dextrose infusion. Evaluation was remarkable for low levels of insulin, proinsulin, C-peptide, and IGF-1, normal cortisol, and an IGF-2: IGF-1 ratio of 8, suggestive of NICTH due to IGF-2, although the patient's hypoglycemia may additionally have been due to worsening, and strikingly extensive tumor invasion of the liver. This patient was unfortunately not considered a surgical candidate; hypoglycemia was ultimately controlled with initiation of high dose corticosteroids and total parenteral nutrition with high amounts of dextrose. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

ODP187 Doedge-Potter syndrome and papillary thyroid carcinoma: An unusual association

Abstract Hypoglycemia can occur in non-diabetic individuals. Doege-Potter is a rare paraneoplastic syndrome presented as a hypoinsulinemic hypoglycemia caused by ectopic secretion of insulin-like growth factor-2 (IGF-2) from a solitary fibrous tumor. There is recent evidence of the involvement of the IGF system in different malignancies as papillary thyroid carcinoma, being exceptional. A 69-year-old female was brought to Emergency after a routine analysis uncovered severe hypoglycemia. She mentioned a history of morning sickness, dizziness, and confusion for the last week that reverted after breakfast, and dry cough for three months. She had a 25-pack-year history of smoking, no history of alcohol use, no other antecedent of interest, no history ofdiabetes mellitus or weight loss surgery, nor chronic treatments. Physical examinations revealed diminished breath sounds in the left middle lung. A thorax radiography showed a submassive left pleural effusion and toracocentesis revealed a hemorrhagic exudate. Laboratory data showed normal GH, and cortisol levels. Despite marked hypoglycemia, insulin level was 0.4 μIU/mL (2,6-24,9), C-peptide 0.11ng/ml (1,1-4,4) and hydroxy butyrate <0.1 mg/dl. The serum IGF-2 level was 428μg/dL (350-1000), and the IGF-1 level was 55.4 μg/L(161-384). Anti-insulin antibodies and sulfonylureas concentration were negative. CT showed a solid mass concerning parietal pleura with areas of necrosis and calcifications, measuring 14.6×15.4×20cm. Low blood glucose with suppressed insulin, C-peptide and IGF-1 levels, elevated IGF-2/IGF-1 ratio (7,7, normal<3) and CT findings suggested the possibility of non-islet cell tumor hypoglycemia (NICTH). Corticoid treatment was initiated to manage the hypoglycemia. Biopsy of the mass showed a malignant solitary fibrous tumor, positive for CD34 and BCL2. After surgery, glucose and IGF-2 levels returned to normal, the patient didn't experience further episodes of hypoglycemia, and corticoid treatment was gradually suspended. A FDG-PET scan, part of extension study, found a PET-positive thyroid nodule. A thyroid ultrasound confirmed a solid, taller than wide thyroid nodule, measuring 6.7×6.4×9,5mm and fine-needle aspiration biopsy was suggestive of papillary thyroid carcinoma. Hemithyroidectomy was performed. The patient is with no evidence of disease. Doege-Potter syndrome is a rare cause of hypoglycemia, resulting from a tumor producing elevated levels of IGF-2. Diagnosis is suggested by low insulin, C-peptide and IGF-1 levels, normal or elevated IGF-2, and elevated IGF2/IGF1 ratio. High levels of ''big-IGF-2'' are associated with high risk papillary thyroid carcinoma, suggesting the relation between the IGF-system and tumor growth, nevertheless there are few cases reported describing this association. Surgical resection of the tumor is the treatment of choice. Presentation: No date and time listed

PSAT277 Glucocorticoid Therapy for Management of Hypoglycemia in the context of Non-Islet Cell Tumor-Induced Hypoglycemia

Abstract Background Non-islet cell tumor-induced hypoglycemia (NICTH) is a rare paraneoplastic syndrome which can cause recurrent hypoglycemia. There is no clear standard of care for management of hypoglycemia. Often, these patients have high morbidity and therefore surgical tumor resection is not always possible. Clinical case: This is the case of a 69 year-old male presenting with altered mental status in the context of hypoglycemia without clear etiology with an initial serum glucose level of 32 mg/dL (normal range 60-100 mg/dL) which quickly corrected after administration of intravenous glucose. He was found to have a large bowel obstruction with CT abdomen revealing an underlying neoplasm. Biopsy of suspected lesion on colonoscopy revealed colorectal adenocarcinoma. Repeated episodes of hypoglycemia complicated by seizures required inpatient management. Workup included serum insulin level which resulted in suppressed level (<1. 0 uU/mL, normal range 1.9-23. 0 uU/mL), low c-peptide level (0.1 ng/mL, normal range 1.1-4.4 ng/mL), low beta hydroxybutyrate level (0. 07 mmol/L, normal level <0.3 mmol/L), cortisol level of 43.7 mcg/dL, undetectable insulin like growth factor-1 (IGF-1) level (<10 ng/mL, normal range 59-230 ng/mL) and low insulin like growth factor-2 (IGF-2) level (66 ng/mL, normal range 333 - 967 ng/mL). IGF2: IGF1 ratio was 66 (ratio >10 is indicative for diagnosis of non-islet cell tumor hypoglycemia). Due to extremely poor oral intake and recurrent episodes of hypoglycemia despite continuous dextrose infusion, the patient was started on 20 mg prednisone daily. Eventually, 40 mg of intravenous methylprednisolone in addition to dextrose infusion was needed as maintenance therapy effectively preventing hypoglycemia. Given his poor functional status, the surgical and oncology team decided he was no longer a surgical or chemotherapy candidate. Eventually, palliative medicine was consulted and the patient was transitioned to comfort care with a plan for outpatient hospice. Conclusion NICTH should be suspected in any patient with hypoglycemia without clear etiology, especially if there are suggestions to NICTH such as known malignancy or newly diagnosed mass. Once NICTH is identified and a primary tumor is found, complete tumor resection represents ideal management, however, not always attainable. In such cases, dextrose infusion might be insufficient to prevent hypoglycemia and is not always the preferred option given the required long-term venous access. In these circumstances, early high dose glucocorticoids are safe and appear to successfully prevent hypoglycemic events. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Glucoregulatory factors in canine hepatocellular carcinoma and leiomyosarcoma with non-islet cell tumour hypoglycaemia

IntroductionHypoglycaemia caused by malignant tumours other than insulinoma is referred to as non-islet cell tumour hypoglycaemia (NICTH), which may be caused by hepatocellular carcinoma (HCC) and leiomyosarcoma (LMS) in veterinary medicine. However, the pathogenetic mechanism of NICTH remains unclear. Therefore, this study aimed to evaluate the gene-expression levels of glucoregulatory factors in canine HCC and LMS accompanied by hypoglycaemia. Materials & methodsFour patients (three with HCC and one with LMS) exhibiting hypoglycemia were included in the hypoglycemic (H) group, whereas ten patients not exhibiting hypoglycemia were in the non-hypoglycaemia (NH) group. The preoperative and postoperative blood glucose and serum insulin-like growth factor-2 (IGF-2) levels, as well as the expression of genes involved regulating blood glucose levels were analysed. ResultsCompared with the NH group, the H group exhibited significantly decreased blood-glucose levels, which increased to normal values after surgery. Compared with the NH group, the H group exhibited significantly increased gene expression of insulin-like growth factor 1, IGF–2, and insulin-like growth factor binding protein 3 in the tumours. Conversely, expression of genes encoding glucoregulatory factors including insulin, gastric inhibitory polypeptide and glucagon was not observed. Serum IGF–2 levels were significantly higher in the H group compared with that in the control group (healthy dogs) and NH group. In two cases in the H group, serum IGF-2 levels decreased after tumour resection. ConclusionThese results suggest that NICTH development in dogs with HCC and LMS is mechanistically associated with IGF–2 overexpression and elevated serum IGF–2 levels.

A Rare Cause of Severe Hypoglycemia in Intracranial Hemangiopericytoma Associated With Non-Islet Cell Tumor Hypoglycemia

A 60-year-old man with a history of metastatic intracranial hemangiopericytoma and type 2 diabetes mellitus presented with acute episodes of confusion and dizziness. A magnetic resonance imaging scan of the brain showed progressing calvarial metastasis without intracranial abnormality. He was found to have severe persistent hypoglycemia. The initial hypoglycemia work-up revealed low serum insulin and C-peptide level and negative panel for oral hypoglycemic agents. Further laboratory testing revealed an elevated insulin-like growth factor II (IGF II):IGF-I ratio and low β-hydroxybutyrate, consistent with nonislet cell tumor hypoglycemia. The patient received prednisone and his hypoglycemia improved.

S3663 Refractory Hypoglycemia Induced by a Gastrointestinal Stromal Tumor Secreting Insulin-Like Growth Factor-2

Introduction: Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome that leads to the release of insulin-like growth factor-2 (IGF-2). It is commonly associated with tumors of mesenchymal origin like gastrointestinal stromal tumors (GIST). Case Description/Methods: A 62-year-old male, with a past medical history of recently diagnosed GIST was started on imatinib but stopped taking it one month ago pending surgical evaluation, presents with altered mental status due to severe hypoglycemia. The patient had no prior history of diabetes or adrenal insufficiency. The patient's repetitive finger sticks glucose remained persistently low even after good oral intake and two D50 pushes. Urine toxicology and drug screen for sulfonylureas were negative. Laboratory investigations showed reduced levels of insulin, C-peptide, and insulin-like growth factor-1 (IGF-1), but his IGF-2 was increased. Endocrinology was consulted for refractory hypoglycemia; recommended to start D10 fluids in the setting of resistant low blood glucose and start octreotide and steroids. Insulinoma was unlikely as insulin and C-peptides were low. CT chest/abdomen/pelvis shows grossly unchanged gastric GIST, measuring 25 × 19.6 × 11.4 cm with no evidence of metastatic disease in the abdomen or pelvis (Figure 1A). Esophagogastroduodenoscopy (EGD) demonstrated large, bilobed subepithelial mass measuring 10-15 cm with an area of ulceration within the mass (Figure 1B) followed up with Endoscopic ultrasound (EUS) demonstrating subepithelial lesion was found likely arising from the fundus, lesser curve and body of the stomach, layer of origin could not be determined on due to the large size of the mass (Figure 1C). Oncology was consulted for further management of malignancy; imatinib was resumed and referred for surgical evaluation for tumor removal. Discussion: IGF-II acts as an autocrine growth factor in tumor cells therefore, it is highly likely that IGF-II will be high in case of high tumor bulk and making them at high risk of developing NICTH. In patients diagnosed with GIST with hypoglycemic symptoms, we should consider IGF-II secreting GIST in addition to insulinoma especially in bulky tumors.Figure 1.: A: CT scan abdomen/pelvis with contrast showing large soft tissue density mass inseparable from the lesser curvature of the stomach marked with asterisk. B: EGD findings consistent with a large, bilobed subepithelial mass measuring 10-15 cm with an area of ulceration within the mass (seen best on retroflexion) was found on the lesser curvature of the stomach along the anterior wall of the stomach. C: Endoscopic US findings showed A 43.0 mm × 77.8 mm subepithelial lesion was found likely arising from the fundus, lesser curve and body of the stomach s/p FNB × 4. The lesion was hypoechoic and had some cystic spaces within it in some areas. Sonographically, the lesion appeared to originate from the stomach, but the wall layers could not be determined due to the large size of the mass.

Late-Onset Non-islet Cell Tumor Hypoglycemia Associated with a Pleural Solitary Fibrous Tumor

Late-Onset Non-islet Cell Tumor Hypoglycemia Associated with a Pleural Solitary Fibrous Tumor

Severe refractory nonislet cell tumor hypoglycemia and paraneoplastic hypercalcemia in a patient with pancreatic adenocarcinoma with neuroendocrine differentiation: a case report.

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Extremely Severe Hypoglycemia in a Patient Who Experienced Spontaneous Rupture of a Hepatocellular Carcinoma

An 85-year-old woman was brought to the emergency room because of shock and coma. She was found to have extreme hypoglycemia (blood glucose <10 mg/dL), associated with the spontaneous rupture of an endstage Hepatocellular Carcinoma (HCC). Because the patient had declined any treatment for her HCC during the preceding year, no specific treatment was instituted for the ruptured HCC. Intravenous infusion of high-glucose fluid normalized her blood glucose concentration within 4 hours, but she died further 6 hours later. At autopsy, neither insulinoma nor a malignancy other than the HCC was identified. Her liver weighed 1,290 g and was filled with tumor masses. The HCC cells were negative for insulin-like growth factor 2 expression on immunohistochemistry, indicating that the extreme hypoglycemia was not a “Non-Islet Cell Tumor Hypoglycemia (NICHT)”.

Solitary Fibrous Tumour of Liver Presenting as Doege–Potter Syndrome – A Case Report

Background: Doege–Potter syndrome is a rare syndrome which is defined as Solitary fibrous tumour (SFT) that is associated with NICTH (non-islet cell tumour hypoglycaemia). Limited case reports are available on Solitary fibrous tumour of liver presenting as Doege–Potter syndrome in published medical literature. Case report: A 60-year-old female presented with episodic weakness and incoherent behaviour since 3 months and during that episode her serum glucose were low which corrected on glucose administration and abdominal distension for 1 month. Abdominal lump was noted in right hypochondrium arising from liver. On investigation- fasting serum glucose levels, fasting insulin levels and fasting C peptide levels were low and serum IGFII levels were elevated. CT abdomen revealed heterogeneously enhancing solitary lesion of 23 x 18 x 14 cm arising from segment 5 ,6, 7, 8 of liver. AFP, CA19-9 levels were within normal range. Liver biopsy shows spindle cell tumour of fibroblastic origin. Anatomical right hepatectomy with MHV preservation was done. Postoperative course was uneventful with symptomatic improvement, serum glucose levels normalised, and patient was discharged on 8th POD. Discussion: Solitary fibrous tumours are uncommon benign or malignant mesenchymal cell tumours which originate from visceral pleura, pelvis, retroperitoneum and liver. Doege–Potter syndrome which is a rare paraneoplastic manifestation of Solitary fibrous tumour, when there is secretion of prohormone - insulin-like growth factor II (IGF-II), causing hypo insulinemic hypoglycaemia and suppressed serum insulin, C peptide and elevated IGFII/IGFI ratio . Definitive treatment is complete tumour resection.

Malignant mixed Müllerian tumour of the uterus associated with non-islet cell tumour hypoglycaemia.

Malignant mixed Müllerian tumour of the uterus associated with non-islet cell tumour hypoglycaemia.

Abstract #999342: Non-Islet Cell Tumor Hypoglycemia Secondary to Metastatic Bladder Urothelial Carcinoma: A Case Report

Non-islet cell tumor hypoglycemia (NICTH) is a rare but life-threatening paraneoplastic syndrome which is marked by the autonomous secretion of incompletely processed precursors of insulin-like growth factor-II (IGF-II), known as “big” IGF-II . Many types of tumors have been associated with NICTH in the literature; Of the tumors from epithelial origin that cause NICTH, less than 1% are of bladder origin. Our literature search only revealed one case of NICTH caused by urothelial carcinoma of the bladder.

Refractory hypoglycaemia in a localised gastrointestinal stromal tumour: Case report.

IntroductionGIST and NICTH are mesenchymal in origin however there are very few reports of GIST associated with NICTH which is a para neoplastic syndrome, generally diagnosed when a tumour induced hypoglycaemia is noted.Case presentationA 46 years old female with prime complain of awareness of a mass in the upper abdomen was admitted for evaluation and further management. Detailed investigation revealed the mass to be gastrointestinal stromal tumour. On the day of admission patient was found to be hypoglycaemic which didn't resolve even after 10% glucose infusion. A growth hormone releasing peptide-2 (GHRP-2) assay was carried out which showed an excessive reaction of basal growth hormone however corticotropin releasing hormone (CRH) tests were within normal limits. She was suspected to be Non Islet cell tumour hypoglycaemia (NICTH) and hypoglycaemia resolved upon administering dexamethasone. Later she underwent chemotherapy and surgical resection after which her blood sugar levels were within normal limits.DiscussionExpression of big IGF-II on the surface of GIST be it metastatic or nonmetastatic can cause refractory hypoglycaemia and can be fatal if left untreated.ConclusionClinicians should be aware of refractory hypoglycaemia in patients with large GIST's as glucocorticoid therapy may prove to be extremely useful and lifesaving even before considering any forms of definitive management of the tumour.

A Case of Non-Islet Cell Tumor Hypoglycemia in Metastatic Solitary Fibrous Tumor

Abstract Introduction: Insulin like growth factor (IGF-2) mediated hypoglycemia secondary to solitary fibrous tumor (SFT), also known as Doege-Potter syndrome is a rare paraneoplastic syndrome. The tumor cells produce large amounts of high molecular weight IGF 2 precursor protein called “big IGF-2” which binds to insulin and IGF receptors in liver, muscle and other peripheral tissues. This causes reduced gluconeogenesis and increased uptake of glucose by the muscle and other tissues leading to hypoglycemia. Big IGF-2 also exerts central negative feedback of growth hormone causing reduction of IGF-I production. Most SFTs are benign and localized (approximately 78-88%). As a result, tumor excision alone would often lead to resolution of the hypoglycemia. We present a case of metastatic SFT with multiple metastasis managed with oral prednisone. Clinical Case: A 44-year-old man with metastatic SFT presented with bilateral humeral fractures. He has known metastatic disease to the brain, lung, liver, bony lytic lesions over a course of eleven years. It has progressed despite multiple chemotherapy and radiation therapies. Prior to admission, he had multiple syncopal episodes associated with fasting hypoglycemia. He reported capillary blood glucose values ranging between 30-50 mg/dl during these episodes which would improve after drinking juice or eating candy. There was no history of diabetes mellitus or use of oral hypoglycemic agents or insulin. On admission, he had a capillary blood glucose value of less than 20 mg/dl, which was confirmed by a serum glucose value of 18 mg/dl on basic metabolic panel. His renal, liver and thyroid function tests were normal. Significant labs include: serum glucose 17 mg/dl, C-peptide <0.10 ng/ml (n: 1-4 ng/ml), serum insulin <1.6 Uu/ml (n: <20 Uu/ml), beta-hydroxybutyrate <0.2 mmol/L (n: <0.3), cortisol 10.8 ug/dl(n: 5-15 ug/dl) glucagon 6 pg/ml(ref 8-57 pg/ml), insulin-like growth factor-1 (IGF 1) 20 ng/ml (n: 52-328), and IGF-2 level 380 ng/ml (267-616 ng/ml), improvement in blood glucose from 46 to 111 mg/dl after 1-gram glucagon administration. The IGF-2/IGF-1 ratio of 19 confirmed our clinical suspicion of non-islet cell tumor hypoglycemia (NICTH). He was started on prednisone 20 mg twice daily with marked improvement in hypoglycemia. Conclusion: NICTH is a rare cause of hypoglycemia and should be considered in the differential while evaluating hypoglycemia in malignancy. For diagnosing NICTH, assays for big IGF-II are not commercially available. However, the IGF-II:IGF-I ratio is considered to be a surrogate marker of big IGFII concentration. The normal ratio is 3 and ratio >10 is diagnostic of NICTH. In cases like ours where tumor resection is not possible, glucocorticoids are most effective in management of hypoglycemia by inhibiting big IGF2 production and stimulating gluconeogenesis.

A Case of Paraneoplastic Hypoglycemia From Squamous Cell Carcinoma of Undetermined Primary

Case: A 57 year old man with squamous cell carcinoma (SCC) of the tongue with complete response to chemoradiation was found unresponsive with a reading of “low” by a POC glucometer. He was treated with an IV dextrose bolus but had recurrent hypoglycemia requiring a continuous dextrose infusion. He was diagnosed with COVID-19 pneumonia, acute hepatitis (elevated liver enzymes), and acute kidney injury (elevated serum creatinine 1.2 mg/dL). Other labs: elevated TSH 8.44 uIU/mL, normal AM cortisol 16.4 ug/dl. A 5.1 cm mass was discovered in the left lung with bilateral nodules, biopsi revealed SCC of unclear origin (either lung or metastatic disease from prior tongue cancer). He was malnourished from prior cancer related dysphagia and nutritional supplements were added. Despite this and improvement in liver and kidney function, he had persistent hypoglycemia. He became hypoglycemic within 4-hrs while performing a 72-hr fast with labs: serum glucose 45 mg/dL, insulin < 2 uU/mL, c-peptide < 0.1 ng/mL, proinsulin < 4 pmol/L, beta hydroxybutyrate 0.17 mmol/L, IGF1 < 16 ng/mL (ref: 50 - 317), IGF2 147 ng/mL (ref: 267-616), negative hypoglycemia panel and insulin antibody. This was consistent with a paraneoplastic hypoglycemia known as non-islet cell tumor hypoglycemia (NICTH). To discontinue the dextrose infusion, he was started on prednisone 20 mg daily titrated up to 60 mg daily, intermittent tube feeds and palliative chemotherapy. With this, hypoglycemia improved, and the dextrose infusion was discontinued. Unfortunately, he had ischemic bowel perforation leading to cardiac arrest and death. Discussion: Our patient had NICTH as suggested by the 72-hr fast (non-insulin mediated hypoglycemia, IGF2/IG1 ratio > 10) and the presence of a tumor. It is mediated by tumor-produced IGF-2 causing increased glucose utilization, decreased gluconeogenesis, glycogenolysis and ketogenesis. Curiously, IGF-2 may not be elevated if the tumor produces a partially processed “big IGF-2” for which there is no commercial assay. Instead, an IGF2/IGF1 ratio close to or more 10 is indicative of NICTH. Mesenchymal and hepatic tumors are the most common cause of this rare entity with an incidence of one per million people years. A literature search showed very few reports of SCC-mediated-NICTH, with one case of esophageal SCC. Our patients’ primary tumor was undetermined (lung vs tongue) - but in either case this could be a novel association. A multidisciplinary approach is required centered around the tumor (surgery, chemotherapy, or radiation). High dose prednisone 30 to 60 mg daily can be used in the interim as it decreases IGF-2 but is not always successful. Recombinant hGH and glucagon are alternatives or can be combined with steroids. In summary IGF2/IGF1 ratio should be calculated, palliative tumor directed therapy should be initiated with prednisone and supplemental nutrition as adjuncts.

Think Outside the Thorax: Doege-Potter Syndrome Presenting as a Retroperitoneal Mass

Introduction: Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome, occurring less commonly than insulinoma. These tumors produce high molecular weight insulin-like growth factor-2 or “big IGF-2”, resulting in hypoglycemia: big IGF-2 decreases glycogenolysis, gluconeogenesis, and glucagon release as well as increases glucose uptake by adipocytes and skeletal muscle. NICTH specifically associated with a solitary fibrous tumor (SFT) is known as Doege-Potter syndrome. Doege-Potter syndrome is seen most with thoracic SFT’s. This case study details the diagnosis and management of Doege-Potter syndrome due to retroperitoneal SFT.Case Description: Our patient is a 68-year-old, white female who initially presented to an outside facility with a 3-week history of episodic neuroglycopenic symptoms temporarily relieved with eating. She reported night sweats but no weight change or fever. Medical history was notable only for hypertension and GERD. She had no personal or family history of diabetes. She was transferred to our facility for further evaluation of an 18.6 cm multilobular mass seen just inferior to the liver on abdominal CT. Whipple’s triad was confirmed with venous glucose sampling while inpatient. C-peptide, free/total insulin, and pro-insulin were collected during permissive hypoglycemia and were low. Morning cortisol and TSH were unremarkable. Additionally, sulfonylurea screen and insulin antibodies were negative. Ultrasound-guided biopsy of the lesion was positive for CD34 and STAT6, consistent with SFT. Refractory hypoglycemia was treated with D5W infusion until surgical resection of the retroperitoneal mass, after which, she had complete resolution of her hypoglycemia.Discussion: This patient’s serum IGF-2 was within normal limits, consistent with reports of feedback inhibition exerted by increased levels of big-IGF-2. Therefore, the IGF-2:IGF-1 ratio is used to determine increased serum levels of big IGF-2, but this test is not widely available. SFT’s can recur within months to years of resection, necessitating surveillance. Recurrence is also more common with extrathoracic tumors. Surgical pathology noted tumor dimensions of 22 cm and 2270 g; mitotic rate was 1 per 10 hpf. Features typical of malignancy include large size (>15 cm) and mitotic rate >4 mitoses per hpf. This patient’s follow-up CT abdomen at 6 weeks did not show any findings to suggest recurrent or metastatic disease.

Doege-Potter syndrome: a case report

Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as hypoinsulinemic hypoglycemia due to ectopic secretion of prohormone: insulin-like growth factor-2 (IGF-2) from a solitary fibrous tumor (SFT). It is essentially a non-islet cell tumor hypoglycemia (NICTH) or in more recent terms, an ‘IGF-2oma’. In case of delay in diagnosis, tumor-induced hypoglycemia could cause hypoxic cerebral injury or death. We hereby present a case of 45 years old male who presented with hypoglycemia due to pleural SFT, which is a very rare and unique presentation. Upon biochemical and radiological investigations, Doege-Potter syndrome was diagnosed and the patient was planned for surgical resection of the tumor. This case stresses upon thorough evaluation of patients presenting with non-diabetic hypoglycemia to rule out underlying malignancy.

A giant pelvic solitary fibrous tumor with Doege\u2013Potter syndrome successfully treated with transcatheter arterial embolization followed by surgical resection: a case report

BackgroundSolitary fibrous tumor (SFT), a mesenchymal fibroblastic tumor with a hypervascular nature, rarely develops in the pelvis. Resection of a giant SFT occupying the pelvic cavity poses an increased risk of developing massive hemorrhage during resection, although surgical resection is the most effective treatment method for this tumor to achieve a potential cure. SFT rarely develops with Doege–Potter syndrome, which is known as a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia (NICTH) secondary to SFT that secretes insulin-like growth factor-II (IGF-II). We present a case of a giant pelvic SFT with Doege–Potter syndrome, which was successfully treated with transcatheter arterial embolization (TAE) followed by surgical resection.Case presentationA 46-year-old woman presented with a disorder of consciousness due to refractory hypoglycemia. Images of the pelvis showed a giant and heterogeneously hypervascular mass displacing and compressing the rectum. Endocrinological evaluation revealed low serum levels of insulin and C-peptide consistent with NICTH. Angiography identified both the inferior mesenteric artery and the bilateral internal iliac artery as the main feeders of the tumor. To avoid intraoperative massive bleeding, super-selective TAE was performed for the tumor 2 days prior to surgery. Hypoglycemia disappeared after TAE. The tumor was resected completely, with no massive hemorrhage during resection. Histologically, it was diagnosed as IGF-II-secreting SFT. Partial necrosis of the rectum in the specimen was observed due to TAE. The patient was followed up for 2 years and no evidence of disease has been reported.ConclusionsPreoperative angiography followed by TAE is an exceedingly helpful method to reduce intraoperative hemorrhage when planning to resect SFT occupying the pelvic cavity. Complications related to ischemia should be kept in mind after TAE, which needs to be planned within 1 or 2 days before surgery. TAE for tumors may be an option in addition to medical and surgical treatment for persistent hypoglycemia in Doege–Potter syndrome.