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: 12.48). 11/2008; 55(3):739-42. DOI: 10.1016/j.eururo.2008.09.050
Source: PubMed

ABSTRACT 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.


Available from: M. Raschid Hoda, Jun 03, 2015
  • [Show abstract] [Hide abstract]
    ABSTRACT: Until recently, most solid renal neoplasms without macroscopic fat were presumed to represent renal cell carcinoma and were indiscriminately treated with nephrectomy. Expanding surgical options and ablative technologies, a growing acceptance of renal mass biopsy, the advent of targeted molecular agents, and advances in our understanding of tumor biology have challenged the wisdom of this approach and are ushering in a potential new era in which therapy is linked to histologic subtype and cytogenetics. This approach mandates evolution of our diagnostic algorithm beyond the distinction between solid and cystic and enhancing and nonenhancing. Computed tomography (CT) has traditionally been the imaging technique of choice for evaluating potential solid renal tumors, in large part due to its widespread availability, high spatial resolution, calcium discrimination, and multiphase, enhanced imaging capabilities. For the most part, however, CT is limited to characterization based upon the attenuation and enhancement characteristics of a lesion and necessitates exposure of patients to ionizing radiation. For these latter reasons, multiparametric magnetic resonance imaging (MRI) is being increasingly used to characterize solid renal masses. The purpose of this manuscript is to review our imaging approach to solid renal masses in adults utilizing MRI with an emphasis on a multiparametric approach augmented by clinical data.
    Abdominal Imaging 01/2014; 39(2). DOI:10.1007/s00261-014-0074-4 · 1.73 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Context: Non-islet cell tumor hypoglycemia (NICTH) is a rare but serious paraneoplastic syndrome in which a tumor secretes high-molecular weight insulin-like growth factor II (IGF-II), causing hypoglycemia. Complete tumor resection is curative, but is often delayed or unfeasible. There is no clear "standard of care" for managing these patients. Evidence Acquisition: PubMed searches were conducted for: "non-islet-cell tumor hypoglycemia", "NICTH", "Doege-Potter", "Doege-Potter syndrome", "high molecular weight IGF-II", and "big IGF-II". Relevant articles were reviewed in detail. We limited our review to English-language articles, focusing on 1988-2013 (corresponding with the elucidation of the pathophysiology of NICTH). Evidence Synthesis: The available literature exists as case reports or small case series, with a void of higher-order treatment studies. Thus, an evidence-based approach to data synthesis was difficult. Nevertheless, the available literature is presented objectively with an attempt to describe clinically useful trends and findings in the management of NICTH. Conclusions: Appropriate identification of NICTH, and prompt and complete tumor resection represents ideal management. However, when prompt resection is not feasible, intravenous glucose or dextrose often do not suffice to prevent hypoglycemia. In such cases, we suggest consideration of local anti-tumor therapies for disease control, and trial of glucocorticoids alone or in combination with growth hormone. Continuous glucagon infusion can be successful if the patient has a positive response to a glucagon stimulation test, and parenteral nutrition may allow higher glucose delivery, but both are limited by the need for continuous intravenous infusion. Diazoxide and octreotide have no role in NICTH.
    The Journal of Clinical Endocrinology and Metabolism 12/2013; 99(3):jc20133382. DOI:10.1210/jc.2013-3382 · 6.31 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: BackgroundHypoglycemic 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 presentationA 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.ConclusionDue 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 · 1.67 Impact Factor