Doege-Potter syndrome associated to metastatic solitary fibrous tumor.
Matheus de Oliveira AndradeNathália da Cruz de SousaPaulo Siqueira do AmaralSamantha Cabral Severino da CostaLuiz Guilherme Cernaglia Aureliano de LimaDelmar Muniz LourençoOlavo FeherPublished in: Autopsy & case reports (2022)
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.
Keyphrases
- type diabetes
- small cell lung cancer
- squamous cell carcinoma
- emergency department
- case report
- stem cells
- multiple sclerosis
- acute lymphoblastic leukemia
- acute myeloid leukemia
- risk factors
- palliative care
- diffuse large b cell lymphoma
- cell therapy
- mesenchymal stem cells
- skeletal muscle
- weight loss
- insulin resistance
- minimally invasive
- combination therapy
- temporal lobe epilepsy