Retroperitoneal Malignant Solitary Fibrous Tumor of the Small Pelvis Causing Recurrent Hypoglycemia by Secretion of Insulin-like Growth Factor 2

Department of Urology and Kidney Transplantation, Martin Luther University, Ernst Grube Str. 40, 06120 Halle, Saale, Germany.
European Urology (Impact Factor: 13.94). 11/2008; 55(3):739-42. DOI: 10.1016/j.eururo.2008.09.050
Source: PubMed


A 28-yr-old man presented with recurrent reduced consciousness, generalized seizures of unknown etiology, recurrent hypoglycemia, psychomotor retardation, and grade 2 ectasia of the left kidney. Abdominal computed tomography (CT) and positron emission tomography (PET) scans demonstrated a well-circumscribed suprapubic pelvic mass, measuring 18 x 15 x 11 cm, with involvement of para-aortic lymph nodes and dilatation of the left ureter suggestive of an extragonadal testicular tumor. We excised the tumor by laparotomy, and it was confirmed to be a solitary fibrous tumor (SFT). After surgery and R0 tumor resection, the patient had no further evidence of hypoglycemia or of recurrence.

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    • "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. "
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    ABSTRACT: Background Hypoglycemic episodes are infrequent in individuals without a history of diabetes mellitus or bariatric surgery. When hypoglycemia does occur in such individuals, an uncommon but important diagnosis to consider is non-islet cell tumor hypoglycemia (NICTH). We report a case of NICTH associated with paraneoplastic insulin-like growth factor-2 (IGF-2) production and review current relevant medical literature. Case presentation A 60 year old male with no relevant past medical history was referred to the endocrinology clinic with 18 month history of episodic hypoglycemic symptoms and, on one occasion was noted to have a fingerstick glucose of 36 mg/dL while having symptoms of hypoglycemia. Basic laboratory evaluation was unrevealing. Further evaluation however showed an elevated serum IGF-2 level at 2215 ng/mL (reference range 411–1248 ng/mL). Imaging demonstrated a large right suprarenal mass. A right nephrectomy with resection of the mass demonstrated a malignant solitary fibrous tumor. Post resection, the patient’s IGF-2 levels normalized and hypoglycemic symptoms resolved. Conclusion Due to the structural and biochemical homology between IGF-2 and insulin, elevated levels of IGF-2 can result in hypoglycemia. A posttranslational precursor to IGF-2 known as “big IGF” also possesses biologic activity. Review of recent reported cases of NICTH identified widespread anatomic locations and varied pathologic diagnoses of tumors associated with paraneoplastic production of IGF-2 causing hypoglycemia. Definitive management of hypoglycemia associated with paraneoplastic production of IGF-2 consists of resection of the tumor responsible for IGF-2 production. Accumulating literature provides a firm basis for routine IGF-2 laboratory evaluation in patients presenting with spontaneous hypoglycemia with no readily apparent cause.
    BMC Endocrine Disorders 06/2014; 14(1):49. DOI:10.1186/1472-6823-14-49 · 1.71 Impact Factor
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    • "Extrapleural SFTs account for 0.6% of all soft tissue tumours [1]. Malignant extrapleural SFT is an even more rare neoplasm and has been described only in limited series [2, 3] and case reports [4–6]. In a large case series of SFTs, Gold et al. [1] reported that no difference exists between intrathoracic SFT and extrapleural SFT regarding rates of malignant pathological features but extrapleural SFTs had a significantly higher rate of locoregional recurrence suggesting a more aggressive clinical behavior. "
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    ABSTRACT: Solitary fibrous tumour (SFT) is a rare oncological entity that most often arises in the pleura. Over the past 10 years, the tumour has been described at numerous extrapleural locations. We present the case of a 42-year-old female Caucasian patient with an extrapleural SFT located at the anterior thoracic wall for 22 years, with atypical histological characteristics and clinical features of malignancy. Management consisted of a wide surgical resection, plastic reconstruction, and postoperative radiotherapy. Although extrapleural SFT usually behaves as a benign soft tissue tumour, it can also present with a more aggressive local behavior, including locoregional recurrence or metastasis. In that case, a multidisciplinary approach is required for accurate diagnosis and proper management.
    Case Reports in Medicine 06/2010; 2010:257167. DOI:10.1155/2010/257167
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    ABSTRACT: A rare case of Solitary fibrous tumor (SFT) of the pelvis is reported. A 76-years-old man presented with a low abdominal pain, acute urine retention and constipation. Imaging studies (US, CT MR) showed an 17 x 10 x 9 ovoid mass in the pelvis, dislocating bladder and rectum. Finally, trans-rectal needle biopsy suggested the diagnosis of SFT. En bloc excision of tumor and rectum (because of strong adhesions) was performed. Histological examination showed spindle and fibroblastic-like cells dispersed in collagenous areas with positive stains for CD34, bcl-2, CD99 and it confirmed diagnosis of SFT. No postoperative complications occurred, only vesico-sphincter dyssynergia was found by urodynamics. After 5 years, patient is disease-free. SFT is, usually, benign tumor with slow growth and excellent prognosis. Complete surgical resection is the only curative treatment. However, 10-15% of SFT are malignant and histological findings cannot always predict clinical behaviour. For this reason, careful and long term follow-up is necessary after surgery.
    Annali italiani di chirurgia 11/2010; 81(6):457-60. · 0.60 Impact Factor
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