Solitary fibrous tumor

Davanzo, Brian; Emerson, Robert E.; Lisy, Megan; Koniaris, Leonidas G.; Kays, Joshua K.

doi:10.21037/tgh.2018.11.02

Cited by 141 publications

(242 citation statements)

References 69 publications

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“…Solitary fibrous tumors are the third most common histiotype in the retroperitoneum, although they remain an extremely rare histiotype, accounting for <2% of all soft‐tissue tumors 162 . This specific sarcoma histiotype more commonly has a low malignant potential, with a 5‐year local recurrence rate of 7% and a distant recurrence rate of 20%, resulting in a favorable survival outcome, with a 5‐year cumulative incidence of 19% for disease‐specific death 163 .…”

Section: Retroperitoneal Stsmentioning

confidence: 99%

Soft‐tissue sarcoma in adults: An update on the current state of histiotype‐specific management in an era of personalized medicine

Gamboa

Gronchi

Cardona

2020

CA A Cancer J Clinicians

349

340

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Soft-tissue sarcomas (STS) are rare tumors that account for 1% of all adult malignancies, with over 100 different histologic subtypes occurring predominately in the trunk, extremity, and retroperitoneum. This low incidence is further complicated by their variable presentation, behavior, and long-term outcomes, which emphasize the importance of centralized care in specialized centers with a multidisciplinary team approach. In the last decade, there has been an effort to improve the quality of care for patients with STS based on anatomic site and histology, and multiple ongoing clinical trials are focusing on tailoring therapy to histologic subtype. This report summarizes the latest evidence guiding the histiotype-specific management of extremity/truncal and retroperitoneal STS with regard to surgery, radiation, and chemotherapy.

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Section: Retroperitoneal Stsmentioning

confidence: 99%

Soft‐tissue sarcoma in adults: An update on the current state of histiotype‐specific management in an era of personalized medicine

Gamboa

Gronchi

Cardona

2020

CA A Cancer J Clinicians

349

340

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“…*Correspondence: jsakata2@med.niigata-u.ac.jp 1 Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-dori, Chuo-ku, Niigata, Niigata 951-8510, Japan Full list of author information is available at the end of the article tumor, which poses an increased risk of developing massive hemorrhage during resection, although surgical resection is the most effective treatment method for SFTs to achieve a potential cure. This tumor rarely develops with non-islet cell tumor hypoglycemia (NICTH), which is known as a paraneoplastic syndrome caused by extrapancreatic tumors that secrete insulin-like growth factor-II (IGF-II) [2].…”

Section: Open Accessmentioning

confidence: 99%

“…Solitary fibrous tumor (SFT) is a rare mesenchymal fibroblastic tumor with a hypervascular nature, which most commonly develops in the pleura followed by the retroperitoneal and abdominopelvic cavity [1]. SFT that develops in the pelvis is sometimes discovered as a giant…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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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.

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“…These findings eliminated pan NETs from differential diagnosis. IHC of signal transducer and activator of transcription 6 (STAT6), which has been used to differentiate solitary fibrous tumors from other soft tissue tumors [ 2 ], yielded negative immunoreactivity (Fig. 6 g).…”

Section: Case Presentationmentioning

confidence: 99%

Primary pancreatic glomus tumor invading into the superior mesenteric vein: a case report

et al. 2020

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Background Glomus tumors are subcutaneous tumors arising from glomus bodies, thermoregulatory components of the skin. These tumors could occur in visceral organs where glomus bodies are not normally present. Herein, we report a case of primary pancreatic glomus tumor with aggressive direct invasion into the superior mesenteric vein (SMV). To the best of our knowledge, this is the second case report of a glomus tumor arising in the pancreas. Case presentation A 46-year-old woman was referred to our hospital due to vomiting with epigastric and back pain. Dynamic-CT revealed a well-circumscribed hypervascular mass, measuring 37 mm in its maximal diameter involving the pancreatic head. Both CT and endoscopic ultrasonography (EUS) revealed direct invasion into the SMV and radiologically suspected tumor thrombus. Biopsy sample obtained by EUS-guided fine needle aspiration revealed proliferation of small cells, round-to-oval tumor cells with round nuclei and scant cytoplasm. A histological diagnosis of pancreatic neuroendocrine tumor, G1 was initially considered. Therefore, subtotal stomach-preserving pancreatoduodenectomy using Child-II reconstruction was subsequently performed. Her SMV was resected and reconstructed due to extensive tumor involvement. Subsequent histopathological analysis revealed solid tumor cells proliferation that comprised oval-shaped nuclei and scant cytoplasm around disorganized or slit-shaped vessels in hematoxylin–eosin-stained slides. Immunohistochemical analysis then demonstrated positive immunoreactivity for smooth muscle actin, vimentin, and CD34, but negative for chromogranin A, synaptophysin, CD56, and signal transducer and activator of transcription 6. Based on these histological findings of resected specimens, the lesion was subsequently diagnosed as a primary pancreatic glomus tumor harboring direct invasion into the SMV. Her postoperative course was uneventful and annual surveys for the following 4 years post-op detected no clinical signs of recurrence. Conclusions We report a very rare case of glomus tumor of the pancreas accompanied by venous invasion. Curative surgical resection is the best treatment option for pancreatic glomus tumors. Although pancreatic glomus tumor is rare, it should be taken into consideration in the differential diagnosis of a pancreatic solid tumor with hypervascularity.

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Solitary fibrous tumor

Cited by 141 publications

References 69 publications

Soft‐tissue sarcoma in adults: An update on the current state of histiotype‐specific management in an era of personalized medicine

Soft‐tissue sarcoma in adults: An update on the current state of histiotype‐specific management in an era of personalized medicine

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

Primary pancreatic glomus tumor invading into the superior mesenteric vein: a case report

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