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      Doege-Potter Syndrome: A Presumptive Case of Metastatic Hemangiopericytoma with Persistent Hypoglycemia in a 27-Year-Old Male

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          Abstract

          Doege-Potter syndrome (DPS) is a rare paraneoplastic condition characterized by hypoinsulinemic hypoglycemia from a solitary fibrous tumor. The underlying mechanism is the secretion of a prohormone form of insulin-like growth factor II (IGF-II) by the tumor, which causes decreased release of glucose into the circulation. We report the case of a 27-year-old Filipino male with presumptive DPS from a recurrent right temporo-zygomatic hemangiopericytoma (HPC). The complexity of DPS requires a multidisciplinary approach. Early screening for metastases from HPC may prevent the undesirable sequelae of the disease process.

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          Localized benign and malignant fibrous tumors of the pleura. A clinicopathologic review of 223 cases.

          D M England, L Hochholzer, M J McCarthy (1989)
          We reviewed 223 localized fibrous tumors of the pleura and divided them histologically into 141 benign and 82 malignant neoplasms. The criteria used for a judgement of malignancy were high cellularity and mitotic activity (more than four mitotic figures per 10 high-power fields), pleomorphism, hemorrhage, and necrosis. The tumors occurred equally in both sexes, most commonly in the sixth to seventh decades of life. Presenting symptoms included chest pain, dyspnea, and cough; they were observed in three-fourths of patients with a malignant tumor. One in every four of these patients had hypoglycemia, clubbed digits, or pleural effusion. Two-thirds of the tumors were attached to visceral pleura, often by a pedicle. The rest arose from the parietal pleura of the chest wall, diaphragm, or mediastinum. Neoplasms in these atypical sites, together with fissural lesions and tumors "inverted" into peripheral lung, were more often malignant. Most neoplasms measured 5-10 cm and weighed 100-400 g. Microscopically, the "patternless pattern," or hemangiopericytic type, was seen in the majority of cases, and mixed patterns were seen in nearly 40% of tumors. Of the 169 tumors where follow-up was available, all of the benign and 45% of the malignant tumors were cured by simple excision. Patients surgically cured of a malignant neoplasm had pedunculated or well-circumscribed lesions. However, 55% of patients with malignant tumors succumbed to their disease secondary to invasion, recurrence, or metastasis. Resectability is the single most important indicator of clinical outcome. No tumor expressed epithelial differentiation, either immunohistochemically or ultrastructurally; therefore, we favor the term "localized fibrous tumor" of pleura instead of "localized mesothelioma."
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            Evaluation and management of adult hypoglycemic disorders: an Endocrine Society Clinical Practice Guideline.

            Philip Cryer, Lloyd Axelrod, Ashley B. Grossman … (2009)
            The aim is to provide guidelines for the evaluation and management of adults with hypoglycemic disorders, including those with diabetes mellitus. Using the recommendations of the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system, the quality of evidence is graded very low (plus sign in circle ooo), low (plus sign in circle plus sign in circle oo), moderate (plus sign in circle plus sign in circle plus sign in circle o), or high (plus sign in circle plus sign in circle plus sign in circle plus sign in circle). We recommend evaluation and management of hypoglycemia only in patients in whom Whipple's triad--symptoms, signs, or both consistent with hypoglycemia, a low plasma glucose concentration, and resolution of those symptoms or signs after the plasma glucose concentration is raised--is documented. In patients with hypoglycemia without diabetes mellitus, we recommend the following strategy. First, pursue clinical clues to potential hypoglycemic etiologies--drugs, critical illnesses, hormone deficiencies, nonislet cell tumors. In the absence of these causes, the differential diagnosis narrows to accidental, surreptitious, or even malicious hypoglycemia or endogenous hyperinsulinism. In patients suspected of having endogenous hyperinsulinism, measure plasma glucose, insulin, C-peptide, proinsulin, beta-hydroxybutyrate, and circulating oral hypoglycemic agents during an episode of hypoglycemia and measure insulin antibodies. Insulin or insulin secretagogue treatment of diabetes mellitus is the most common cause of hypoglycemia. We recommend the practice of hypoglycemia risk factor reduction--addressing the issue of hypoglycemia, applying the principles of intensive glycemic therapy, and considering both the conventional risk factors and those indicative of compromised defenses against falling plasma glucose concentrations--in persons with diabetes.
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              HEMANGIOPERICYTOMA: A VASCULAR TUMOR FEATURING ZIMMERMANN'S PERICYTES.

              Tom Stout, M. Murray (1942)
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                Author and article information

                Journal
                Journal ID (nlm-ta): J ASEAN Fed Endocr Soc
                Journal ID (iso-abbrev): J ASEAN Fed Endocr Soc
                Journal ID (publisher-id): JAFES
                Title: Journal of the ASEAN Federation of Endocrine Societies
                Publisher: Journal of the ASEAN Federation of Endocrine Societies
                ISSN (Print): 0857-1074
                ISSN (Electronic): 2308-118X
                Publication date (Electronic): 12 May 2021
                Publication date (Print): 2021
                Volume: 36
                Issue: 1
                Pages: 90-94
                Affiliations
                [1 ]Department of Internal Medicine, Southern Philippines Medical Center, Davao City, Philippines
                [2 ]Section of Oncology, Department of Internal Medicine, Southern Philippines Medical Center, Davao City, Philippines
                [3 ]Section of Endocrinology, Department of Internal Medicine, Southern Philippines Medical Center, Davao City, Philippines
                Author notes
                Corresponding author: Von Lovel D. Zarra, MD, Medical Officer III, Department of Internal Medicine, Teresita Perez Hall, 3rd floor ICU Building, Southern Philippines Medical Center, JP Laurel Street, Bajada, Davao City, Davao del Sur 8000. E-mail: von_lobes@ 123456yahoo.com , ORCiD: https://orcid.org/0000-0002-4551-9434
                Article
                Publisher ID: JAFES-36-1-090
                DOI: 10.15605/jafes.036.01.16
                PMC ID: 8214353
                PubMed ID: 34177094
                SO-VID: e546659e-6870-4789-8888-420039a98389
                Copyright © © 2021 Journal of the ASEAN Federation of Endocrine Societies
                License:

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International.

                History
                Date received : 07 September 2020
                Date accepted : 28 February 2021
                Categories
                Subject: Case Report

                Keywords: doege-potter syndrome,hemangiopericytoma,hypoglycemia
                Data availability:
                Keywords: doege-potter syndrome, hemangiopericytoma, hypoglycemia

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