Solitary Fibrous Tumor/Hemangiopericytoma: A Companion Case Discussion of a Spectrum of Rare CNS Mesenchymal Tumors and Their Evolving WHO Classification

Neurographics ◽  
2018 ◽  
Vol 8 (3) ◽  
pp. 188-192
Author(s):  
K.T. Golden ◽  
Y. Sun ◽  
T. Li ◽  
S. Bobra ◽  
H. Mehta
2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Moyosore Awobajo ◽  
Stefanie Hettwer ◽  
Sarah Hackman

Solitary fibrous tumors (SFTs) are rare mesenchymal tumors, originally identified in the pleura. Even though they have subsequently been described in several extrapleural sites, the incidence of SFTs in the spermatic cord is particularly rare. Here, we report a case of a 27-year-old male that presented with a 3-year history of left scrotal swelling. Computed tomography (CT) and ultrasound demonstrated multiple solid, hypoechoic well-circumscribed masses that were separate from the testis. Surgical excision of the mass led to pathologic diagnosis of a solitary fibrous tumor involving the spermatic cord. Solitary fibrous tumors, although rare, are an important differential diagnosis for urogenital tumors.


2018 ◽  
Vol 129 (6) ◽  
pp. 1384-1396 ◽  
Author(s):  
Byung Sup Kim ◽  
Yuil Kim ◽  
Doo-Sik Kong ◽  
Do-Hyun Nam ◽  
Jung-Il Lee ◽  
...  

OBJECTIVEThe authors conducted this retrospective study to investigate the clinical outcomes of intracranial solitary fibrous tumor (SFT) and hemangiopericytoma (HPC), defined according to the 2016 WHO classification of central nervous system (CNS) tumors.METHODSHistopathologically proven intracranial SFT and HPC cases treated in the period from June 1996 to September 2014 were retrospectively reviewed and analyzed. Two neuropathologists reviewed pathological slides and regraded the specimens according to the 2016 WHO classification. Factors associated with progression-free survival (PFS) and overall survival (OS) were statistically evaluated with uni- and multivariate analyses.RESULTSThe records of 47 patients—10 with SFT, 33 with HPC, and 4 with anaplastic HPC—were reviewed. A malignant transition from conventional SFT to WHO grade III SFT/HPC was observed in 2 cases, and 13 HPC cases were assigned grade III SFT/HPC. Mean and median follow-ups were 114.6 and 94.7 months, respectively (range 7.1–366.7 months). Gross-total resection (GTR) was significantly associated with longer PFS and OS (p = 0.012 for both), and adjuvant radiation therapy versus no such therapy led to significantly longer PFS (p = 0.018). Extracranial metastases to the liver, bone, lung, spine, and kidney occurred in 10 patients (21.3%). Grade III SFT/HPC was strongly correlated with the development of extracranial metastases (p = 0.031).CONCLUSIONSThe 2016 WHO classification of CNS tumors reflected the different types of pathological malignant progression and clinical outcomes better than prior classifications. Gross-total resection should be the primary treatment goal in patients with SFT/HPC, regardless of the pathological grade, and radiation can be administered as adjuvant therapy for patients with SFT/HPC that shows an aggressive phenotype or that is not treated with GTR.


2017 ◽  
Vol 19 (suppl_3) ◽  
pp. iii115-iii115
Author(s):  
K. Sung ◽  
J. Moon ◽  
E. Kim ◽  
W. Chang ◽  
S. Kang ◽  
...  

2015 ◽  
Vol 100 (5) ◽  
pp. 836-840 ◽  
Author(s):  
Masayuki Urabe ◽  
Yukinori Yamagata ◽  
Susumu Aikou ◽  
Kazuhiko Mori ◽  
Hiroharu Yamashita ◽  
...  

Solitary fibrous tumor (SFT) is one of the mesenchymal tumors, which rarely arises in the abdominal space. We report a very rare case of abdominal SFT, mimicking another mesenchymal tumor. A 52-year-old Japanese man was referred to our hospital for further evaluation and treatment of gallbladder polyp. Contrast-enhanced computed tomography (CT) showed an enhanced nodule within the gallbladder, and incidentally, also showed a well-circumscribed mass adjacent to the small intestine. The mass was depicted as slightly high density in plain CT, and with contrast-enhancement, the mass was partially stained in early phase and the stained area spread heterogeneously in delayed phase. Magnetic resonance imaging showed that the abdominal mass was depicted as slightly high intensity on T2-weighted imaging and low intensity on T1-weighted imaging. With double-balloon endoscopy and capsule endoscopy, we did not find any tumor inside the small intestine. These visual findings lead us to diagnose it as gastrointestinal stromal tumor of the small intestine with extraluminal growth. We planned to resect both the gallbladder polyp and the intraperitoneal tumor at the same time for pathologic diagnosis and treatment. When the operation was performed, we found a milk-white lobulated tumor on the greater omentum and the tumor was entirely resected. Microscopically, the gallbladder polyp was diagnosed as tubular adenoma, and the omental tumor was diagnosed as SFT. It is important to bear in mind that omental SFTs sometimes mimic other mesenchymal tumors and should be included in the differential diagnosis of abdominal tumor not revealed by endoscopy.


2019 ◽  
Vol 130 (2) ◽  
pp. 418-425 ◽  
Author(s):  
Kyoung Su Sung ◽  
Ju Hyung Moon ◽  
Eui Hyun Kim ◽  
Seok-Gu Kang ◽  
Se Hoon Kim ◽  
...  

OBJECTIVESolitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a new combined entity for which a soft-tissue–type grading system, ranging from grades I to III, has been introduced in the 2016 WHO classification of tumors of the CNS. The results of the treatment of this new disease entity require evaluation.METHODSThe authors retrospectively reevaluated the pathological findings and medical records of patients with SFT/HPC. This study included 60 patients (27 men and 33 women, median age 42.5 years, range 13–69 years) treated at Severance Hospital between February 1981 and February 2016. Four, 40, and 16 patients were categorized as having SFT/HPC grades I, II, and III, respectively. Among these patients, SFTs diagnosed in 7 patients were regraded as grades I (n = 4), II (n = 2), and III (n = 1).RESULTSThe median overall survival (OS) was 73.2 months (range 1.4–275.7 months), and the progression-free survival (PFS) after the first operation was 53.8 months (range 1.4–217.7 months). Six patients (10%) showed extracranial metastasis during a median period of 103.7 months (range 31.9–182.3 months). Nineteen patients (31.7%) presented with tumor recurrences. The patients in the grade III group had shorter PFS and OS, as well as a shorter period to extracranial metastasis, compared with patients in the grade II group. In the grade II group, patients who underwent gross-total resection showed longer PFS than those who underwent subtotal resection; however, there was no difference in OS. Patients who underwent adjuvant radiation therapy (RT) after surgery had longer PFS compared with that of patients who did not undergo adjuvant RT.CONCLUSIONSThe SFT/HPC grade I group showed a relatively benign course compared with those of the other groups. The grade III group presented a course with a more aggressive nature than that of the grade II group. In the grade II group, the extent of resection and adjuvant RT was significantly associated with longer PFS. The long-term follow-up and periodic systemic evaluation are mandatory to detect systemic metastasis.


2017 ◽  
pp. 96-104
Author(s):  
V. Yu. Pletneva ◽  
N. Yu. Kashtanova ◽  
G. G. Karmazanovsky ◽  
Yu. A. Stepanova ◽  
D. V. Kalinin

Solitary fibrous tumor (SFT) is an extremely rare tumor of the pancreas. The content of stromal tissue in the pancreas is insignificant, this fact explains the extremely rare occurrence of mesenchymal tumors (less than 1% of pancreatic neoplasms). It has been described only 16 such cases in the world literature.A clinical case of solitary fibrous tumor of the pancreatic head of a man 43 years old, illustrating the difficulty of diagnosis is presents in the article/ In order to emphasize difficulties in diagnostics we introduce you another clinical case of extrapleural solitary fibrous tumors of the retroperitoneal space of a woman 63 years old.Pancreatic SFT and NEN cannot be reliably differentiated on the basis of lesion structure and contrast medium uptake. However, in most cases SFT will progradiently enhance due to abundant collagen stroma, leading to marked difference between delayed-phase images of SFT and NEN with cystic degeneration. SFT is a rare tumour and radiologist should be aware of its characteristics, particularly in the setting of NEN with cystic degeneration or atypical contrast uptake. 


2019 ◽  
Vol 7 ◽  
pp. 2050313X1882346 ◽  
Author(s):  
Céline Forster ◽  
Aurélien Roumy ◽  
Michel Gonzalez

Solitary fibrous tumors are rare mesenchymal tumors most commonly arising from the pleura. When associated with paraneoplastic syndrome of hypoglycemia caused by the secretion of a high-molecular-weight form of insulin-like growth factor II, it is referred to as the Doege–Potter syndrome. Surgery with complete tumor resection is the only curative treatment. We present here the case of a 93-year-old female diagnosed with a Doege–Potter syndrome who underwent three repetitive surgical resections of recurrent solitary fibrous tumor.


2019 ◽  
Vol 78 (8) ◽  
pp. 685-693
Author(s):  
Zhi-Gang Yao ◽  
Hai-Bo Wu ◽  
Ying-Hua Hao ◽  
Xing-Fu Wang ◽  
Guang-Zhen Ma ◽  
...  

Abstract Solitary fibrous tumor/hemangiopericytomas (SFT/HPCs) are mesenchymal tumors characterized by “staghorn” blood vessels and collagen deposition. Little is known about SFT/HPCs with papillary architecture. We summarized the clinicopathologic features of 12 patients with papillary SFT/HPCs (8 males and 4 females; median age: 59 years), including 8 previously reported cases. Tumors were present in the meninges (75%, 9/12), adrenal gland (8%, 1/12), orbit (8%, 1/12), or spinal canal (8%, 1/12). Six tumors (50%) had a true papillary architecture with fibrovascular cores and 6 tumors (50%) had a pseudopapillary architecture with vascular cores. Nuclear staining for STAT6 was present in all tested tumors (10/10). RT-PCR indicated NAB2 ex6-STAT6 ex17 fusion in 4 tumors (80%, 4/5) and NAB2 ex4-STAT6 ex2 fusion in 1 tumor (20%, 1/5). Five patients (42%, 5/12), all with tumors in the meninges, developed local recurrence at a median of 61 months after surgery (range: 56–165 months; mean: 88.6 months). These results indicated that the papillary architecture is a morphological form of SFT/HPCs. The recognition of this pattern, with appropriate immunohistochemical analysis and assessment of NAB2-STAT6 fusion, should facilitate the distinction of these rare neoplasms from morphologically similar tumors in the meninges, lung, pleura, and soft tissue.


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