Introduction: Doege-Potter syndrome is a rare syndrome characterized by hypo-insulinemic hypoglycemia. It is caused by excessive ectopic secretion of insulin-like growth factor II from a solitary fibrous tumors of intrapleural or extrapleural origin. Laboratory tests reveal low levels of C-peptide and insulin, on the contrary insulin-like growth factor II level is elevated, which is characteristic for Doege-Potter syndrome. Majority of solitary fibrous tumors present no symptomatology, recurrent hypoglycemia is relatively rare, but it may be the only clinical manifestation. The therapy is surgical, consisting of radical en-bloc tumor resection.
Case Report: Authors present a case report of a patient with recurrent hypoglycemia caused solely by solitary fibrous tumor. Hypoglycemia resolved immediately after surgical resection and there were no recurrences.
Conclusion: Doege-Potter syndrome should be considered as the differential diagnosis in a patient with suspicion on thoracic malignancy if accompanied by features suggestive of hypoglycemia. Prolonged follow up is strongly advised because of the risk of disease recurrence, even in patients with benign solitary fibrous tumors of the pleura (SFTP).
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http://dx.doi.org/10.33699/PIS.2020.99.2.95-98 | DOI Listing |
Ear Nose Throat J
January 2025
Department of Otolaryngology Head and Neck Surgery, Peking University First Hospital, Beijing, China.
Solitary fibrous tumor (SFT) is an uncommon spindle cell neoplasm, which generally arises from the pleura. There have been documented a number of extrapleural origins including the head and neck in the literature. It is emphasized to make a diagnosis in a rare location such as the retropharyngeal space.
View Article and Find Full Text PDFJ Cardiothorac Surg
January 2025
Division of Thoracic Surgery, Department of Surgery, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, 602-8566, Kyoto, Japan.
Background: Solitary fibrous tumors (SFTs) of the pleura are usually benign. We present a case of SFT of the pleura which grew rapidly after slow long-term progression.
Case Presentation: A 78-year-old man was referred to our hospital for left-sided back pain and shortness of breath.
Front Oncol
January 2025
Department of Radiology, Daping Hospital, Army Medical University, Chongqing, China.
Background: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant disorder characterized by excessive proliferation of histiocytes, the cause of which remains unknown. Although the lymph nodes are the most commonly affected site, some patients may present with extranodal involvement, particularly in the skin, nasal cavity, eyes, and bones. In this report, we aim to present a unique case of RDD with pleural involvement in a 61-year-old patient.
View Article and Find Full Text PDFCureus
December 2024
Neurosurgery, Npistanbul Brain Hospital, Istanbul, TUR.
Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare, aggressive tumors typically found along the dural sinuses. Despite their aggressive nature, complete surgical resection remains the most significant factor in reducing recurrence and improving survival. Here, we present the case of a 32-year-old male patient who presented with a new-onset headache and vertigo.
View Article and Find Full Text PDFHead Neck Pathol
January 2025
Department of Pathology, University Medical Center Utrecht, Utrecht, 3508 GA, The Netherlands.
Purpose: The NAB2::STAT6 fusion is predominantly associated with solitary fibrous tumors (SFTs) and is utilized in diagnosing SFTs through nuclear STAT6 protein overexpression. Recent studies expanded the phenotypic spectrum of NAB2::STAT6 rearranged neoplasms, including adamantinoma-like and teratocarcinosarcoma-like phenotypes. We report a case of a NAB2::STAT6 rearranged epithelial tumor exhibiting sebaceous differentiation in the parotid gland.
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