scholarly journals Dedifferentiated Solitary Fibrous Tumor: A Concise Review

2018 ◽  
Vol 142 (6) ◽  
pp. 761-766 ◽  
Author(s):  
Nicholas J. Olson ◽  
Konstantinos Linos

Solitary fibrous tumor (SFT) is a unique mesenchymal neoplasm that was originally believed to be of submesothelial origin. Eventually, SFT expanded to include what was previously called hemangiopericytoma in other regions of the body that had similar immunohistochemical and morphologic features. Although most are benign, many studies have tried to identify histologic features that predict which tumors will behave in an aggressive manner. Recently, dedifferentiation has been described in rare cases of SFT and does appear to correlate with a more aggressive clinical course. Dedifferentiated SFT occurs in a similar age range and location as conventional SFT and can resemble multiple different malignant entities. Utilization of ancillary studies and thorough tissue sampling is important to reach the correct diagnosis. The morphologic features, immunohistochemistry, molecular alterations, and prognosis will be discussed.

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Brian Cervenka ◽  
Brenda Villegas ◽  
Uttam Sinha

Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm that can present essentially anywhere in the body. Presentations in the hypopharynx are extremely rare with only two previous cases reported. We report the first case of postcricoid SFT occurring in a 58-year-old male requiring a microsuspension laryngoscopy excision following an unsuccessful transoral robotic attempt. The excision was uneventful, and the patient is currently without recurrence. Current management strategies of the hypopharyngeal SFT, the unique differential diagnosis, and challenges in surgical approaches in the postcricoid region are discussed.


2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Erin A. Kaya ◽  
Jonathan D. Carlson ◽  
Cheddhi J. Thomas ◽  
Aaron E. Wagner ◽  
Robert K. Fairbanks ◽  
...  

We present a unique case of a patient simultaneously diagnosed with solitary fibrous tumor (SFT) and unrelated adenocarcinoma of the lung, both proven with separate pathology. It was subsequently found that the SFT had metastasized to the brain by additional pathology, and not the predicted adenocarcinoma. SFTs are a rare mesenchymal neoplasm that accounts for less than 2% of all reported soft tissue tumors. SFTs most commonly arise in the thoracic cavity, but are frequently found in various locations throughout the body, and rarely metastasize to the brain. This case highlights that rare neoplasms, such as SFT, should not be ruled out as a potential cause of metastasis. Due to the rarity of this clinical situation, we also provide a review and discussion of previously reported SFT cases and the use of postoperative radiation therapy. The optimal treatment for individual patients remains unclear in this unique situation. Surgical resection followed by adjuvant Gamma Knife radiation therapy to the surgical bed appears to be a safe option for local treatment of SFT in select patients. Further studies are needed of this rare clinical situation in order to better understand and optimize future treatments for patients with SFT and metastasis to the brain.


2021 ◽  
pp. 17-18
Author(s):  
Shaila Shaila ◽  
Aparna. C ◽  
Sowmiya. J ◽  
Geetha Sree. A

Solitary Fibrous Tumor (SFT) of prostate is an unusual mesenchymal neoplasm. We report SFT in a 62 year-old man which was clinically misdiagnosed as benign prostatic hyperplasia (BPH). The specimen of surgically resected two prostate mass, we received were solid grey-white nodular on cut surface. Based on histopathological ndings and IHC assessment, a diagnosis of SFT was made. In addition, we review the literature and discuss the challenging issues of misdiagnosis. The case is presented in view of its rarity.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Maitane I. Orue-Echebarria ◽  
Laura Garciafília ◽  
Luis Rodriguez-Bachiller ◽  
Benjamín Díaz-Zorita ◽  
Enrique Velasco ◽  
...  

Abstract Background Solitary fibrous tumor is an unusual fibroblastic mesenchymal neoplasm typically described in the pleura. It may appear anywhere with a varied anatomic distribution and essentially it can develop from any soft tissue or visceral location. Its course is usually indolent and it rarely causes distant metastases, so it has a prolonged survival rate. It sometimes presents itself as a disseminate disease being the liver the most frequently involved location. In these occasions, the management should be discussed in a multidisciplinary tumor committee formed by surgeons, oncologists and radiologists. Surgery remains the gold standard for treatment. Case representation We present the case of a woman with a tumor in the left abdominal wall and bilobar massive liver metastases, both locations histologically diagnosed as solitary fibrous tumor. She receives biological treatment for a severe case of Crohn´s disease. Evaluated in a multidisciplinary committee, surgery was recommended for both the primary lesion and the liver metastases. The hepatobiliary surgeons considered a two-stage hepatectomy with portal vein embolization (PVE) as the best strategy. After the first procedure consisting in cleaning the left hepatic lobe followed by PVE the future liver remnant volume (FLRV) was considered inadequate, so the patient was also treated with right transarterial radioembolizacion with yttrium 90 (TARE-Y90) intending a double goal: to treat the tumor and to increased the FLRV. Furthermore, a severe flare of Crohn´s disease forced us to intensify the patient’s treatment with the addition of biological agents (infliximab and adalimumab) until complete remission of the symptoms. The second stage of the liver surgery had to be postponed for more than 6 months and could finally be carried out without complications, achieving an R0 resection. The postoperative course was uneventful and the follow up has showed no recurrence to date. Conclusion Solitary fibrous tumours with extensive liver metastases are infrequent but when they appear modern surgical strategies like two stage hepatectomy are the treatment of choice and must be carried out by specialised units. The therapeutic decisions should be guided by a multidisciplinary committee.


2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 11068-11068
Author(s):  
Marcelle Goldner Cesca ◽  
Celso Lopes Mello ◽  
Tiago Felismino ◽  
Maria Nirvana Formiga ◽  
Ulisses Ribaldo Nicolau ◽  
...  

11068 Background: Solitary fibrous tumor (SFT) is a rare mesenchymal tumor that account for less than 2% of all soft tissue sarcomas. SFT has been identified in multiple anatomic locations and can arise anywhere in the body. Surgical management is the mainstay of treatment for localized disease. However, about 20% will develop locoregional recurrences or distant metastasis with a role for systemic treatment. Methods: A retrospective analysis was carried out in a large cancer center in Brazil. Our primary objective was to evaluate clinical and treatment aspects of metastatic/ locally advanced (Mtx/LA) SFT cohort and secondary to describe clinical characteristics of entire population diagnosed with SFT. Descriptive statistics was used for main results. Survival curves were estimated using Kaplan-Meier. Data were retrieved from electronic patient medical records. Results: From April, 1971 to October, 2017, 82 patients with SFT were treated. Median follow-up was 45.5 months. 67 (81.7%) were alive on the cut-off date. Median age at diagnosis was 51 (14-78). 40.2% men. Most common primary sites (PS) were pleura (19.8%), central nervous system (CNS - 11%) and pelvis (11%). 18 (21.9%) underwent chemotherapy for Mtx/LA disease. In this subgroup, 61.1% were men; PS retroperitoneal (22.2%), extremities (16.7%), CNS (16.7%). 66.7% had pulmonary, 44.4% hepatic, 27.8% bone metastasis and one (5.5%) local recurrence. All patients had at least one adverse prognostic factor (tumor size ≥ 10cm, positive margins, necrosis, ≥ 4/10 mitosis). One (5,5%) had Doege-Potter syndrome. 7 (38.9%) did one, 5 (27.8%) two and 6 (33.3%) ≥ 3 lines of treatment. First line was temozolomide/bevacizumab (TMZ/Bev) in 55.6%, followed by chemotherapy (Ch) in 27.8% and tyrosine kinase inhibitors (TKI) in 16.7%. Median progression-free survival was 3.5 months (95% IC: 0.0-7.4) and overall survival 27.3 months (95% IC: 18.7-36.0). Response rate using RECIST criteria was 12.5% for TMZ/Bev and 62.5% had stable disease. TKI and Ch had no response. Conclusions: SFT is rare and with heterogeneous clinical presentation. In our analysis, patients received a wide range of therapy, reflecting the lack of well-established systemic treatment option. TMZ/Bev showed consistent activity in Mtx/LA scenario.


2007 ◽  
Vol 36 (9) ◽  
pp. 538-542 ◽  
Author(s):  
Lorenzo Lo Muzio ◽  
Massimo Mascolo ◽  
Saverio Capodiferro ◽  
Gianfranco Favia ◽  
Eugenio Maiorano

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.


2020 ◽  
Vol 11 ◽  
pp. 238
Author(s):  
Hammad Ghanchi ◽  
Tye Patchana ◽  
Eisha Christian ◽  
Chao Li ◽  
Mark Calayag

Background: Solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is a rare tumor which originates from the walls of capillaries and has historically been thought to be able to occur anywhere in the body that blood vessels are found. It is rarely found in the sellar region. Case Description: InS this report, we present the first case of this tumor occurring in the sellar region of a pediatric patient. This 12-year-old male presented with progressive vision loss which prompted surgical resection after a sellar lesion was discovered on imaging. The initial transsphenoidal approach resulted in subtotal resection and the patient experienced reoccurrence within 3 months. He underwent an orbitozygomatic craniotomy to achieve gross total tumor resection. Conclusion: We conducted a literature review of intracranial SFT/HPC in the pediatric population and found it to be an extremely rare occurrence, with <30 cases reported. The incidence of SFT/HPC occurring in the sellar region for any age group was also found to be a rare entity. Treatment recommendations for this tumor are also scarce, based on retrospective chart reviews from the adult population. The role for adjuvant radiation has mixed results.


2016 ◽  
Vol 2016 ◽  
pp. 1-4
Author(s):  
Gaby N. Moawad ◽  
Elias D. Abi Khalil ◽  
Cheryl Silverbrook ◽  
Stephanie Barak ◽  
Alice Semerjian ◽  
...  

Solitary fibrous tumors (SFTs) may occur at any site in the body. SFTs can only be conclusively diagnosed based on histopathologic and immunohistochemical characteristics of the tumor. The presence of SFTs in the abdomen and pelvis is extremely rare. To our knowledge no cases of urethral solitary fibrous tumor in the literature have been reported so far. We present a case of a solitary fibrous tumor arising from the urethra in a twenty-three-year-old female presenting with vaginal mass.


2018 ◽  
Vol 27 (3) ◽  
pp. 322-327 ◽  
Author(s):  
Nicholas J. Olson ◽  
Julianna M. Czum ◽  
Francine B. de Abreu ◽  
Konstantinos Linos ◽  
Candice C. Black

Pulmonary adenofibroma (PAF) is a rare neoplasm that may be related to solitary fibrous tumor (SFT). A subset of PAFs harbor the NAB2-STAT6 fusion that is typical of SFT, but a significant proportion do not. Their distinction is clinically important as SFTs can potentially have an aggressive clinical course, while there has been no report of a PAF behaving in a malignant fashion. We report a case of a 60-year-old male who developed a SFT and PAF in the same lung. The SFT harbored a NAB2-STAT6 fusion, while the PAF did not have any identifiable fusion. This case represents the first instance of a single patient with both of these tumors occurring simultaneously in the same lung.


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