Autopsy and Case Reports
https://app.periodikos.com.br/journal/autopsy/article/doi/10.4322/acr.2021.412
Autopsy and Case Reports
Clinical Case Report

Doege-Potter syndrome associated to metastatic solitary fibrous tumor

Matheus de Oliveira Andrade; Nathália da Cruz de Sousa; Paulo Siqueira do Amaral; Samantha Cabral Severino da Costa; Luiz Guilherme Cernaglia Aureliano de Lima; Delmar Muniz Lourenço Júnior; Olavo Feher

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Abstract

Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.

Keywords

Solitary Fibrous Tumors, Paraneoplastic Syndromes, Hypoglycemia

References

1 de Pinieux G, Karanian M, Le Loarer F, et al. Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network. PLoS One. 2021;16(2):e0246958. http://dx.doi.org/10.1371/journal.pone.0246958. PMid:33630918.

2 Olson NJ, Linos K. Dedifferentiated solitary fibrous tumor: a concise review. Arch Pathol Lab Med. 2018;142(6):761-6. http://dx.doi.org/10.5858/arpa.2016-0570-RS. PMid:29848035.

3 Robinson DR, Wu YM, Kalyana-Sundaram S, et al. Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. Nat Genet. 2013;45(2):180-5. http://dx.doi.org/10.1038/ng.2509. PMid:23313952.

4 Tariq MU, Din NU, Abdul-Ghafar J, Park YK. The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior. Diagn Pathol. 2021;16(1):32. http://dx.doi.org/10.1186/s13000-021-01095-2. PMid:33879215.

5 Khowaja A, Johnson-Rabbett B, Bantle J, Moheet A. Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor-2 from a malignant renal solitary fibrous tumor - clinical case and literature review. BMC Endocr Disord. 2014;14:49. http://dx.doi.org/10.1186/1472-6823-14-49. PMid:24934576.

6 Gold JS, Antonescu CR, Hajdu C, et al. Clinicopathologic correlates of solitary fibrous tumors. Cancer. 2002;94(4):1057-68. http://dx.doi.org/10.1002/cncr.10328. PMid:11920476.

7 Georgiesh T, Boye K, Bjerkehagen B. A novel risk score to predict early and late recurrence in solitary fibrous tumour. Histopathology. 2020;77(1):123-32. http://dx.doi.org/10.1111/his.14078. PMid:31991494.

8 Ishihara H, Omae K, Iizuka J, et al. Late recurrence of a malignant hypoglycemia-inducing pelvic solitary fibrous tumor secreting high-molecular-weight insulin-like growth factor-II: A case report with protein analysis. Oncol Lett. 2016;12(1):479-84. http://dx.doi.org/10.3892/ol.2016.4665. PMid:27347168.

9 Le Jeune S, Des Guetz G, Bihan H, Cohen R, Coindre JMMJ, Mourad JJ. Refractory hypoglycemia controlled by sorafenib in solitary fibrous tumor. J Clin Oncol. 2013;31(9):e118-21. http://dx.doi.org/10.1200/JCO.2011.40.7999. PMid:23341512.

10 Mohammed T, Ozcan G, Siddique AS, Araneta RN 3rd, Slater DE, Khan A. Doege-Potter syndrome with a benign solitary fibrous tumor: a case report and literature review. Case Rep Oncol. 2021;14(1):470-6. http://dx.doi.org/10.1159/000512823. PMid:33976622.

11 Smrke A, Thway K, H Huang P, Jones RL, Hayes AJ. Solitary fibrous tumor: molecular hallmarks and treatment for a rare sarcoma. Future Oncol. 2021;17(27):3627-36. http://dx.doi.org/10.2217/fon-2021-0030. PMid:34409859.

12 Kawai S, Ariyasu H, Uraki S, et al. Imbalanced expression of IGF2 and PCSK4 is associated with overproduction of big IGF2 in SFT with NICTH: a pilot study. J Clin Endocrinol Metab. 2018;103(7):2728-34. http://dx.doi.org/10.1210/jc.2018-00593. PMid:29897468.

13 Krishnan L, Clark J. Non-islet cell tumour hypoglycaemia. BMJ Case Rep. 2011;2011:bcr0220113914. http://dx.doi.org/10.1136/bcr.02.2011.3914. PMid:22693317.

14 Bodnar TW, Acevedo MJ, Pietropaolo M. Management of non-islet-cell tumor hypoglycemia: a clinical review. J Clin Endocrinol Metab. 2014;99(3):713-22. http://dx.doi.org/10.1210/jc.2013-3382. PMid:24423303.

15 Tsuro K, Kojima H, Okamoto S, et al. Glucocorticoid therapy ameliorated hypoglycemia in insulin-like growth factor-II-producing solitary fibrous tumor. Intern Med. 2006;45(8):525-9. http://dx.doi.org/10.2169/internalmedicine.45.1611. PMid:16702745.

16 de Boer J, Jager PL, Wiggers T, et al. The therapeutic challenge of a nonresectable solitary fibrous tumor in a hypoglycemic patient. Int J Clin Oncol. 2006;11(6):478-81. http://dx.doi.org/10.1007/s10147-006-0606-1. PMid:17180519.

17 Sawka A, Toh D. Biochemical challenge: difficult management of recurrent hypoglycaemia in the setting of a massive solitary fibrous tumour of the liver. Intern Med J. 2018;48(6):744-6. http://dx.doi.org/10.1111/imj.13819. PMid:29898268.

18 Bailly C, Bichali Alroumani M, Douane F, Ansquer C, Drui D. Metastatic solitary fibrous tumor with Doege-Potter Syndrome: hypoglycemia treated by 90Y radioembolization. Clin Nucl Med. 2018;43(3):e93-5. http://dx.doi.org/10.1097/RLU.0000000000001944. PMid:29293132.

19 Otake S, Kikkawa T, Takizawa M, et al. Hypoglycemia observed on continuous glucose monitoring associated with IGF-2-producing solitary fibrous tumor. J Clin Endocrinol Metab. 2015;100(7):2519-24. http://dx.doi.org/10.1210/jc.2014-4534. PMid:25915566.
 


Submitted date:
10/23/2022

Accepted date:
11/18/2022

Publication date:
12/13/2022

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