scholarly journals A giant pelvic solitary fibrous tumor with Doege–Potter syndrome successfully treated with transcatheter arterial embolization followed by surgical resection: a case report

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Kizuki Yuza ◽  
Jun Sakata ◽  
Hiroki Nagaro ◽  
Takuya Ando ◽  
Yuki Hirose ◽  
...  

Abstract Background Solitary fibrous tumor (SFT), a mesenchymal fibroblastic tumor with a hypervascular nature, rarely develops in the pelvis. Resection of a giant SFT occupying the pelvic cavity poses an increased risk of developing massive hemorrhage during resection, although surgical resection is the most effective treatment method for this tumor to achieve a potential cure. SFT rarely develops with Doege–Potter syndrome, which is known as a paraneoplastic syndrome characterized by non-islet cell tumor hypoglycemia (NICTH) secondary to SFT that secretes insulin-like growth factor-II (IGF-II). We present a case of a giant pelvic SFT with Doege–Potter syndrome, which was successfully treated with transcatheter arterial embolization (TAE) followed by surgical resection. Case presentation A 46-year-old woman presented with a disorder of consciousness due to refractory hypoglycemia. Images of the pelvis showed a giant and heterogeneously hypervascular mass displacing and compressing the rectum. Endocrinological evaluation revealed low serum levels of insulin and C-peptide consistent with NICTH. Angiography identified both the inferior mesenteric artery and the bilateral internal iliac artery as the main feeders of the tumor. To avoid intraoperative massive bleeding, super-selective TAE was performed for the tumor 2 days prior to surgery. Hypoglycemia disappeared after TAE. The tumor was resected completely, with no massive hemorrhage during resection. Histologically, it was diagnosed as IGF-II-secreting SFT. Partial necrosis of the rectum in the specimen was observed due to TAE. The patient was followed up for 2 years and no evidence of disease has been reported. Conclusions Preoperative angiography followed by TAE is an exceedingly helpful method to reduce intraoperative hemorrhage when planning to resect SFT occupying the pelvic cavity. Complications related to ischemia should be kept in mind after TAE, which needs to be planned within 1 or 2 days before surgery. TAE for tumors may be an option in addition to medical and surgical treatment for persistent hypoglycemia in Doege–Potter syndrome.

2020 ◽  
Author(s):  
Wenjing Wang ◽  
Banghe Bao ◽  
Anbin Hu ◽  
Xiaofeng Zhu ◽  
Qing Chen

Abstract Background Solitary fibrous tumor (SFT) is a rare soft tissue tumor originating from mesenchyme. Two cases of SFT we report right now occurred in the splenic vein and liver respectively, this primary splenic vein SFT may be the first report case, and also the first report of liver recurrence SFT cured by orthotopic liver transplantation (OLT). Case presentation One case was a 37-year-old female patient whose primary tumor site was located in the splenic vein, which resulted in splenomegaly and hypersplenism; its recurrence again and again after surgical resection and eventually transferred to the liver, during 10 years of follow-up, 4 operations were performed, and he is in a good condition right now. The second case was a 54-year-old male patient whose primary tumor site was located in the liver, spleen and left side of the chest wall; however, he had no uncomfortable symptoms. Surgeons performed two operations to remove these tumors, totally. 6 years later, SFT recurrence in the liver, and given that the tumor was so large that it could not be completely surgical resected, we chose orthotopic liver transplantation (OLT), and no tumor recurrence during 12-month follow-up. Conclusion The reports of these two cases of SFT are very rare, especially the splenic vein SFT, which expand the understanding of SFT. The main treatment of SFT is still surgical resection, right now, and liver transplantation may be a new option treatment for the huge liver SFT.


2007 ◽  
Vol 84 (1) ◽  
pp. 292-294 ◽  
Author(s):  
Kotaro Kameyama ◽  
Norihito Okumura ◽  
Yujiro Kokado ◽  
Kentaroh Miyoshi ◽  
Tomoaki Matsuoka ◽  
...  

2015 ◽  
Vol 13 (1) ◽  
Author(s):  
Yuichiro Yokoyama ◽  
Keisuke Hata ◽  
Takamitsu Kanazawa ◽  
Hironori Yamaguchi ◽  
Soichiro Ishihara ◽  
...  

2020 ◽  
Author(s):  
Yan He ◽  
Guan Huang ◽  
Wensong Lin ◽  
Zhaohui Zheng ◽  
Haiyan Zhao

Abstract Background: Solitary fibrous tumor is an uncommon mesenchymal neoplasm that originates from fibroblasts and occurs predominantly in the visceral pleura. Pelvic cavity small sized malignant solitary fibrous tumor is very rare; it is easily misjudged by surgeons as a benign lesion and resected by laparoscopic surgery. When accompanied by dedifferentiation, malignant solitary fibrous tumor is difficult to diagnose by pathologists. Here, we describe a challenging case.Case presentation: A 47-year-old man was accidentally found to have a pelvic mass for three months, with pain and distension for seven days. The mass was adhered and compressed to the ureter and bladder. The right side of the pelvic cavity had a palpable and substantial mass, with no obvious associated pain. With an assumed diagnosis of a benign tumor, the patient underwent laparoscopic surgery to resect the tumor. Histologically, spindle cell areas and dedifferentiated areas were observed. Immunohistochemical analysis of dedifferentiated regions revealed cytokeratin showed multifocal expression. Vimentin and CD34 were abnormal negative. This case was diagnosed as a MSFT with dedifferentiation. The patient was well after the operation, but, unfortunately, he had a recurrence one year later.Conclusion: Pelvic cavity malignant solitary fibrous tumor is a rare tumor that is in frequently dedifferentiated and exhibiting cytokeratin expression. The diagnosis of this type of tumor can be confusing, and it should be distinguished from Synovial Sarcoma, Liposarcoma, and other malignant tumors. The expression of cytokeratin and the absence of vimentin and CD34 are pitfalls to diagnosis. In this case, there was still a high degree of malignancy despite the small size of the tumor. Clinical-image-pathological multidisciplinary analysis has a great effect on diagnosis and therapy of diseases. This case is a cautionary tale for surgeons and pathologists.


1989 ◽  
Vol 9 (6) ◽  
pp. 513-515
Author(s):  
Yuji Makita ◽  
Kei Hori ◽  
Shinichiro Osato ◽  
Hideki Hirakata ◽  
Kaoru Onoyama ◽  
...  

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