Doege-Potter syndrome associated to metastatic solitary fibrous tumor

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

doi:10.4322/acr.2021.412

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Sumário

Clinical Case Report • Autops. Case Rep. 12 • 2022 • https://doi.org/10.4322/acr.2021.412 copy

Doege-Potter syndrome associated to metastatic solitary fibrous tumor

Authorship SCIMAGO INSTITUTIONS RANKINGS

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

Test	Values	Reference range
Glucose^* * Venipuncture during hypoglycemia (6-hour fasting period). (mg/dL)	13	70 - 99 mg/dL
Insulin*(μU/mL)	< 0.6	2.6 – 24.9 μU/mL
C-peptide* (ng/mL)	0.05	1.1 – 4.4 ng/mL
IGF-1* (ng/mL)	23	74.9 – 216.4 ng/mL
HbA1c (%)	5.0	4.1 – 6.0%
TSH (μIU/mL)	2.2	0.27 – 4.20 μIU/mL
Serum cortisol (μg/dL)	25.7	5.0 – 25 ug/dL
AST (U/L)	19	<37 U/L
ALT (U/L)	18	<41 U/L
INR	1.13	0.95 – 1.2
Albumin (g/dL)	3.4	3.4 – 3.9 g/dL
Bilirubin (mg/dL)	0.61	0.2 – 1.0 mg/dL
Creatinine (mg/dL)	0.39	0.7 – 1.2 mg/dL

Hospital Universitário da Universidade de São Paulo Hospital Universitário da Universidade de São Paulo, Av. Prof. Lineu Prestes, 2565 - Cidade Universitária, 05508-000 - São Paulo - SP - Brasil, (16) 3307-2068, (16) 3307-2068 - São Paulo - SP - Brazil
E-mail: autopsy.hu@gmail.com

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[1] Authors’ contributions: Matheus de Oliveira Andrade was responsible for the case review and manuscript writing. Nathália da Cruz de Sousa was responsible for the case review and manuscript writing. Paulo Siqueira do Amaral contributed to case review and manuscript review. Samantha Cabral Severino da Costa contributed to case review and manuscript review. Luiz Guilherme Cernaglia Aureliano de Lima was responsible for the pathology data collection. Delmar Muniz Lourenço Júnior wrote and reviewed the manuscript. Olavo Feher contributed to manuscript review.