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Doege-Potter综合征1例
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作者 童玲玲 林燕妮 《承德医学院学报》 2023年第3期249-251,共3页
孤立性纤维性肿瘤(Solitary Fibrous Tumor,SFT)是一种间叶源性肿瘤,起源于表达CD34抗原的树突状间质细胞,可在任何年龄发病,但常见发病年龄为50~60岁,男女发病率相似,以胸膜肿瘤多见,胸膜外孤立性纤维性肿瘤较为少见[1]。由SFT导致的... 孤立性纤维性肿瘤(Solitary Fibrous Tumor,SFT)是一种间叶源性肿瘤,起源于表达CD34抗原的树突状间质细胞,可在任何年龄发病,但常见发病年龄为50~60岁,男女发病率相似,以胸膜肿瘤多见,胸膜外孤立性纤维性肿瘤较为少见[1]。由SFT导致的低血糖命名为Doege-Potter综合征(Doege-Potter Syndrome,DPS),DPS发病率低于5%[2]。 展开更多
关键词 孤立性纤维性肿瘤 低血糖 doege-potter综合征 病理
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Doege-Potter syndrome by malignant solitary fibrous tumor of the liver: A case report and review of literature 被引量:5
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作者 Delvecchio Antonella Duda Loren +8 位作者 Conticchio Maria Fiore Felicia Lafranceschina Stefano Riccelli Umberto Cristofano Antonella Pascazio Bianca Colagrande Anna Resta Leonardo Memeo Riccardo 《World Journal of Gastrointestinal Surgery》 SCIE CAS 2019年第8期348-357,共10页
BACKGROUND Solitary fibrous tumor of the liver (SFTL) is a rare occurrence with a low number of cases reported in literature. SFTL is usually benign but, 10%-20% cases are reported to be malignant with a tendency to m... BACKGROUND Solitary fibrous tumor of the liver (SFTL) is a rare occurrence with a low number of cases reported in literature. SFTL is usually benign but, 10%-20% cases are reported to be malignant with a tendency to metastasize. The majority of malignant SFTL cases are associated with a paraneoplastic hypoglycaemia defined as Doege-Potter syndrome. Surgery is the best therapeutic treatment, however, long- life follow-up is recommended. CASE SUMMARY A 74-year-old man, was admitted to the emergency department after a syncopal episode with detection of hypoglycaemia resistant to medical treatment. The computed tomography revealed a solid mass measuring 15 cm of the left liver. An open left hepatectomy was performed with complete resection of tumor. Histopathological analyses confirmed a malignant SFTL. CONCLUSION Large series with long-term follow-up have not been published neither have clinical trials been undertaken. Consequently, the methodical long-term followup of surgically treated SFTLs is strongly recommended. 展开更多
关键词 SOLITARY FIBROUS TUMOR Malignant SOLITARY FIBROUS TUMOR of the LIVER Mesenchymal TUMOR Hepatic TUMOR doege-potter SYNDROME
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Malignant solitary fibrous tumor of the pancreas with systemic metastasis: A case report and review of the literature 被引量:4
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作者 Hao Geng Yu Ye +4 位作者 Yun Jin Bai-Zhou Li Yuan-Quan Yu Yang-Yang Feng Jiang-Tao Li 《World Journal of Clinical Cases》 SCIE 2020年第2期343-352,共10页
BACKGROUND Pancreatic solitary fibrous tumor(SFT) is a rare neoplasm of intermediate biological potential. So far, only 22 cases have been reported since 1999. All the cases, except one, exhibited benign features. Her... BACKGROUND Pancreatic solitary fibrous tumor(SFT) is a rare neoplasm of intermediate biological potential. So far, only 22 cases have been reported since 1999. All the cases, except one, exhibited benign features. Here, we report the first case of malignant pancreatic SFT with typical Doege-Potter syndrome, along with the clinical and pathologic evidence of its systemic metastasis.CASE SUMMARY The patient was a 48-year-old man with a 1-year history of pancreatic and liver masses and refractory hypoglycemia. Increased uptake of the tracer fluorodeoxyglucose(FDG) was found in the liver and bones by fluorine-18 FDG positron emission tomography/computed tomography. After multidisciplinary discussion, a distal pancreatectomy procedure was performed, and histological examination showed a lesion composed of abundant heterogeneous spindle cells with localized necrosis. On immunohistochemistry evaluation, STAT6 was found to be diffusely expressed in the tumor. Based on the overall evidence, the patient was diagnosed with malignant pancreatic SFT with liver and bone metastases.CONCLUSION The diagnosis of malignant SFT requires comprehensive evidence including clinical, immunohistochemistry, and histological features. This case may be presented as a reference for diagnoses and management of malignant pancreatic SFTs with systemic metastasis. 展开更多
关键词 Solitary fibrous tumor PANCREAS MALIGNANT doege-potter syndrome Case report
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