Síndrome de Doege – Potter: Un reporte de caso  :  Doege-Potter Syndrome: A case report     |     Doege-Potter 综合征：病例报告

Patricia Segura Nuñez; Fernando Monge Espinoza; Mayra  Orihuela Baldeon; Jose  Laca Forero; Gianella  Mejia Reyes

doi:10.25176/RFMH.v24i4.6515

Authors

Patricia Segura Nuñez Department of Pulmonology, Hipólito Unanue National Hospital. Lima, Peru
Fernando Monge Espinoza Department of Pulmonology, Hipólito Unanue National Hospital. Lima, Peru
Mayra Orihuela Baldeon Department of Pulmonology, Hipólito Unanue National Hospital. Lima, Peru
Jose Laca Forero SANNA / Clínica San Borja
Gianella Mejia Reyes Department of Pulmonology, Hipólito Unanue National Hospital

DOI:

https://doi.org/10.25176/RFMH.v24i4.6515

Keywords:

Doege-Potter Syndrome, Solitary Fibrous Tumor, Hypoglycemia

Abstract

Doege-Potter syndrome is a rare paraneoplastic syndrome characterized by a benign or malignant mesenchymal tumor associated with persistent hypoglycemia. We present the case of a 70-year-old woman with a 4-year illness characterized by productive cough, oppressive pain in the left hemithorax, and progressively increasing dyspnea. The chest tomography revealed an extensive solid tumor in the lower 2/3 of the left hemithorax. An ultrasound-guided transthoracic biopsy of the tumor was performed. The histopathological study reports a solitary fibrous tumor and immunohistochemistry: STAT6 positive. Therefore, she underwent a thoracotomy with excision of the tumor. After surgery, glucose values normalized. The patient met the criteria for Doege-Potter syndrome, a rare entity.

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Author Biographies

Patricia Segura Nuñez, Department of Pulmonology, Hipólito Unanue National Hospital. Lima, Peru

Pulmonologist doctor

Fernando Monge Espinoza, Department of Pulmonology, Hipólito Unanue National Hospital. Lima, Peru

Pulmonologist doctor

Mayra Orihuela Baldeon, Department of Pulmonology, Hipólito Unanue National Hospital. Lima, Peru

Pulmonologist doctor

Jose Laca Forero, SANNA / Clínica San Borja

pulmonologist doctor

Gianella Mejia Reyes, Department of Pulmonology, Hipólito Unanue National Hospital

Pulmonology resident doctor

References

Davanzo B, Emerson R, Lisy M, Koniaris L, Kays J. Solitary fibrous tumor. Transl Gastroenterol Hepatol 2018; 3:94. DOI: 10.21037/tgh.2018.11.02.

Fernandez-Trujillo L, Bolaños J, Alvarez C, Giraldo J, Velasquez M, et al. Doege–Potter Syndrome and Hypoglycemia associated with Solitary Fibrous Tumor of the Pleura: Two Case Reports. Clin Med Insights Circ Respir Pulm Med. 2020; 14: 1-7. DOI: 10.1177/1179548420964759

Lopez-Hinostroza M, Moya - Salazar J, Dávila J, Absencio A, Contreras-Pulache H. Doege–Potter syndrome due to endothoracic solitary hypoglycemic fibrous tumor. Clin Case Rep. 2022;10:e05611. DOI: https://doi.org/10.1002/ccr3.5611

Gohir Q, Ghosh S, Bosher O, Crawford E, Srinivasan K, et al. Pleural-based giant solitary fibrous tumour with associated hypoglycaemia: unusual presentation with pulmonary hypertension in a patient with Doege–Potter syndrome. Clinical Medicine. 2023; 5:518-20. DOI: 10.7861/clinmed.2023-0274

Mindaye E, Tesfaye G, Aboye A. Intrathoracic giant solitary fibrous tumor of the pleura: Case report. Int J Surg Case Rep. 2021;85: 106224. DOI: 10.1016/j.ijscr.2021.106224

Donato B, Sewell M, Sen A, Beamer S. Doege Potter syndrome presenting as multiple fibrous tumours of the chest. Interact Cardiovasc Thorac Surg. 2022;35(2):ivac089. DOI: 10.1093/icvts/ivac089

Guo W, Ji Y, Guo L, Che S, Huai Q, et al. Severe hypoglycemia and finger clubbing in a patient with a BRCA1 mutation in a solitary fibrous tumor: a case report. Ann Transl Med. 2021;9(13):1093. DOI: 10.21037/atm-21-914

Solsi A, Pho K, Shojaie S, Findakly D, Noori T. Doege-Potter syndrome and Pierre-Marie-Bamberger syndrome in a patient with pleural solitary fibrous tumor: a rare case with literature review. Cureus. 2020;12(5): e7919. DOI: 10.7759/cureus.7919

Castaldo V, Domenici D, Biscosi M, Ubiali P, Miranda C, et al. Doege-Potter Syndrome; A Case of Solitary Fibrous Pleura Tumor Associated with Severe Hypoglycemia: A Case Report in Internal Medicine. Endocrine, Metabolic & Immune Disorders-Drug Targets, 2023;23,1562-1569. DOI: 10.2174/1871530323666230623112047

Mohammed T, Ozcan G, Siddique A, Araneta R, Slater D, et al. Doege-Potter Syndrome with a Benign Solitary Fibrous Tumor: A Case Report and Literature Review. Case Rep Oncol. 2021;14:470–476. DOI: 10.1159/000512823

Berrebi D, Symczyk O, Cater T, Adelanwa A, Bacaj P, et al. Outside the Thorax: Doege–Potter Syndrome Presenting as a Retroperitoneal Abdominal Mass. Case Rep Endocrinol. 2021; 9919321. DOI: 10.1155/2021/9919321

Guo Y, Liao X, Li Z, Chen Y, Wang S, et al. Ultrasound-guided percutaneous needle biopsy for peripheral pulmonary lesions: diagnostic accuracy and influencing factors. Ultrasound in Med. & Biol. 2018; Vol. 44, No. 5, pp. 1003–1011. DOI: https://doi.org/10.1016/j.ultrasmedbio.2018.01.016

Mohamed M, Laz N, Kamel K, Mahmoud R. The role of ultrasound-guided transthoracic Tru-Cut core biopsy in the diagnosis peripheral thorax lesion. Egypt J Bronchol. 2021;15,31. DOI: https://doi.org/10.1186/s43168-021-00080-z

Deguchi Y, Komuta W, Watanabe T, Saiga K, Kuruhashi K, et al. Successful Surgical Treatment of a Recurrent Pelvic Solitary Fibrous Tumor of Uterine Origin Accompanied by Doege-Potter Syndrome: A Case Report. Am J Case Rep. 2022; 23: e936806. DOI: 10.12659/AJCR.936806

Chen S, Zheng Y, Chen L, Yi Q. A broad ligament solitary fibrous tumor with Doege–Potter syndrome. Medicine. 2018. 97:39. DOI: 10.1097/MD.0000000000012564