scholarly journals Solitary fibrous tumor: molecular hallmarks and treatment for a rare sarcoma

2021 ◽  
Author(s):  
Alannah Smrke ◽  
Khin Thway ◽  
Paul H Huang ◽  
Robin L Jones ◽  
Andrew J Hayes

Solitary fibrous tumor (SFT) is a rare soft tissue sarcoma subtype which mainly affects adults in the fifth and sixth decades of life. Originally part of a spectrum of tumors called hemangiopericytomas, classification has been refined such that SFTs now represent a distinct subtype. The identification of NAB2-STAT6 fusion in virtually all SFTs has further aided to define this rare subgroup. SFTs have a spectrum of behavior from benign to malignant, with evidence suggesting risk of metastases related to age at diagnosis, extent of necrosis, mitotic rate and tumor size. The standard treatment for localized disease is surgical excision with or without radiotherapy. Retrospective and prospective evidence suggests antiangiogenic treatment is effective for unresectable disease. Further translational work is required to understand the biology driving the differential behavior and identify more effective treatments for patients with metastatic disease.

Sarcoma ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Nicolas Penel ◽  
Eric Yaovi Amela ◽  
Gauthier Decanter ◽  
Yves-Marie Robin ◽  
Perrine Marec-Berard

We have reviewed the literature data regarding the spectrum of tumors including solitary fibrous tumor and hemangiopericytoma with special focus on definition of the disease, discussion of the criteria for malignancy, and the key elements of standard treatment of localized disease. We have discussed the emerging concepts on the tumor biology and the different systemic treatments (chemotherapy and molecular-targeted therapies).


1993 ◽  
Vol 102 (1) ◽  
pp. 74-76 ◽  
Author(s):  
John G. Batsakis ◽  
Roger D. Hybels ◽  
Adel K. El-Naggar

First described as a pleural neoplasm, the solitary fibrous tumor has been reported in a number of extrapleural sites, including the upper respiratory tract. The neoplasm is of mesenchymal origin, exists in benign and malignant forms, and is a histopathologic diagnosis made after exclusion of other soft tissue neoplasms. None of the eight reported sinonasal and nasopharyngeal solitary fibrous tumors have been histologically or biologically malignant. Surgical excision appears to control the tumor at these sites.


2017 ◽  
Vol 07 (01) ◽  
pp. 20-24
Author(s):  
Javier Ortiz Rodríguez-Parets ◽  
Cristina González Velasco ◽  
Elisa Muñoz Torres ◽  
María Dolores Ludeña de la Cruz

Author(s):  
Kentaro Watanabe ◽  
Yuya Takabe ◽  
Shuhei Iizuka ◽  
Yoshiro Otsuki ◽  
Toru Nakamura

Reports ◽  
2021 ◽  
Vol 4 (4) ◽  
pp. 33
Author(s):  
Mattia Di Bartolomeo ◽  
Sara Negrello ◽  
Arrigo Pellacani ◽  
Anna Maria Cesinaro ◽  
Stefano Vallone ◽  
...  

A solitary fibrous tumor (SFT) is a benign neoplasm, firstly described as a mesenchymal tumor of the pleura. Its incidence range in the head and neck region is about 5–27%, but only rarely does it affect paranasal sinuses. The differential diagnosis is challenging, owing to its erosive growth pattern and immuno-histochemical features. SFTs have an aggressive behavior and an important recurrence potential. Therefore, a radical surgical excision is the gold standard therapeutic procedure. A rare SFT originating from the right maxillary sinus is reported here. The 37-year-old patient presented to the outpatient clinic with a painful expansive lesion in the whole right maxillary region. The overlying skin was inflamed and the patient had no epistaxis episodes. The 1.5 dentary element tested negative for vitality; however, a puncture of the lesion led to a hematic spill and no purulent discharge. An endoscopic-guided biopsy was suggestive either of SFT or hemangioperictoma, excluding a malignant neoplasm. A multi-equipe surgical team was activated. The lesion was embolized in order to achieve a good hemostatic control and, after 48 h, the neoplasm was radically excised with a combined open and endoscopic approach. The patient was disease-free at 12-month radiological and clinical follow-up. Given the rarity of this lesion and the delicacy required in addressing head and neck neoplasms, we believe that the present case report might be of help in further understanding how to approach cranio-facial SFTs.


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.


2015 ◽  
Vol 105 (6) ◽  
pp. 557-559
Author(s):  
Charles A. Kean ◽  
Bridget R. Moore ◽  
Ashley M. Nettles ◽  
Richard P. Bui

Solitary fibrous tumors are uncommon mesenchymal neoplasms that were initially described as pleural tumors, but in recent years, numerous extrapleural sites have been reported. A solitary fibrous tumor is characterized as a circumscribed, painless mass with a patternless growth pattern, positive CD34 spindle-shaped cells, bcl-2, and dense keloidal collagen with areas of hypercellularity and hypocellularity. This case report describes a 55-year-old male veteran who presented with a rare manifestation of an extrapleural solitary fibrous tumor of the medial right hallux. Microscopic and immunohistochemical findings were consistent with the previously documented characteristics of solitary fibrous tumors. Complete surgical excision of the tumor is the treatment of choice.


2010 ◽  
Vol 199 (6) ◽  
pp. e82-e83 ◽  
Author(s):  
Ali Ibrahim Sevinc ◽  
Aras Emre Canda ◽  
Tarkan Unek ◽  
Serafettin Canda ◽  
Mehmet Ali Kocdor ◽  
...  

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Everton Freitas de Morais ◽  
Deborah Gondim Lambert Moreira ◽  
Viviane Alves De Oliveira ◽  
Rodrigo Rodrigues Rodrigues ◽  
Adriano Rocha Germano ◽  
...  

Solitary fibrous tumor is a rare neoplasm of mesenchymal origin that usually affects the pleura. This rarity becomes more relevant in the oral cavity since the clinical features are nonspecific. A 66-year-old female patient presented with a 3-month history of a swelling in the floor of the mouth, measuring 2 cm in greatest diameter, and pain symptomatology. Occlusal and panoramic radiographs showed no bone involvement. Ultrasonography of the submandibular and parotid salivary glands revealed normal morphology, dimensions, and echogenicity. During this exam, a nodular image of low echogenicity measuring about 2.7 × 1.8 cm was detected. An excisional biopsy was performed and histopathological analysis revealed a well-defined tumor-like lesion with alternation between hypercellular areas without a defined pattern and hypocellular areas. On immunohistochemistry, the tumor was positive for CD34 and CD99 and negative forα-SMA, S-100, and bcl-2. Combining the histopathological and immunohistochemical features, the diagnosis was solitary fibrous tumor. The patient is under periodical clinical follow-up and shows no signs of recurrence 7 months after surgical excision of the tumor. The combination of clinical-pathological and immunohistochemical features is necessary for the diagnosis.


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