Ruptured desmoid tumor imitating acute appendicitis – a rare reason for an emergency surgery

BMC Surgery

Jeni Panaiotova¹  Valeria Hadjiiska²  Tihtchev Veselin³  Theophil Sedloev⁴  Yavor Asenov⁴  Alexander Timev⁵  Stoyan Genadiev⁶ 

[1] Department of Gynecology, Multi-profile Hospital for Active Treatment “Nadezhda”;Department of Nuclear Medicine, Medical University – Sofia, University Hospital “Alexandrovska”;Department of Pathology, Medical University – Sofia, University Hospital “Queen Joanna”;Department of Surgery, Medical University – Sofia, University Hospital “Queen Joanna”;Department of Urology, Medical University – Sofia, University Hospital “Alexandrovska”;Department of Vascular Surgery, Multi-profile Hospital for Active Treatment “Uni Hospital”;

关键词: Desmoid tumor;    Aggressive fibromatosis;    Fibromatosis;    Intestinal mesentery;    Soft-tissue sarcomas;    Case report;   

DOI  :  10.1186/s12893-019-0662-x

来源: DOAJ

【 摘 要 】

Abstract Background Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications. Case presentation We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis. Conclusions Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation.

【 授权许可】

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