Solitary Fibrous Tumor of the Parapharyngeal Space

Solitary fibrous tumors are benign neoplasms of mesenchymal origin. They usually arise from the visceral or parietal pleura and peritoneum, although they have been found in many areas throughout the body. We report a case of solitary fibrous tumor of the parapharyngeal space. Microscopically, the tumor contained spindle cells with areas of marked hypercellularity without a definite pattern. Consistent with a benign lesion, there were few mitoses and no necrosis. The tumor cells stained strongly positive for CD34 and vimentin. At the 2-year follow-up, the patient was well and free of local and/or distant disease.

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Solitary fibrous tumor of the pleura: 3 case reports

Revista da Associação Médica Brasileira ◽

10.1590/1806-9282.61.03.207 ◽

2015 ◽

Vol 61 (3) ◽

pp. 207-208 ◽

Cited By ~ 1

Author(s):

Elias Amorim

Keyword(s):

Solitary Fibrous Tumor ◽

Case Reports ◽

Benign Lesion ◽

Complete Resection ◽

The Other ◽

Posterolateral Thoracotomy ◽

Pertinent Literature ◽

Complete Surgical Resection ◽

Fibrous Tumor

Summary Introduction: solitary fibrous tumor of the pleura (SFTP) is a rare tumor arising from mesenchymatous cells in submesothelial pleural tissue which, unlike mesothelioma, is not related to asbestos or smoking. Methods: report of four patients who underwent surgical treatment for giant SFTP and review of the pertinent literature. Results: of the four patients operated, two presented symptoms including cough, chest pain and feeling of compression, whereas the other two subjects were asymptomatic. All patients underwent complete surgical resection by wide posterolateral thoracotomy, and surgical specimens removed with minimum bleeding. None of the cases required complementary lobectomy or segmentectomy. All tumors were histologically benign. Conclusion: complete resection of the lesion is the treatment of choice in all SFTP cases. Prognosis of the benign lesion is excellent, although close follow-up is necessary. In the rarer, more aggressive forms, treatment may be complemented by adjunctive chemotherapy or radiotherapy, the benefits of which have yet to be confirmed.

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Solitary fibrous tumor of the post-styloid parapharyngeal space

Acta Radiologica Short Reports ◽

10.1177/2047981614536158 ◽

2014 ◽

Vol 3 (6) ◽

pp. 204798161453615

Author(s):

Ji Eun Lee ◽

Hyun Sook Hong ◽

Kee-Hyun Chang ◽

Hee Kyung Kim ◽

Jisang Park

Keyword(s):

Differential Diagnosis ◽

Head And Neck ◽

Solitary Fibrous Tumor ◽

Parapharyngeal Space ◽

Rare Occurrence ◽

Radiographic Findings ◽

Spindle Cells ◽

Neck Area ◽

Rare Location ◽

Fibrous Tumor

Solitary fibrous tumor (SFT) is a well-known tumor composed of spindle cells found most commonly in the pleura. Recently, accounts of their rare occurrence at other sites, including the head and neck area, have been reported. The parapharyngeal space is a rare location even for head and neck SFTs, and thus, could be confused with a variety of other tumors that can originate in this area. Here, we report a case of SFT originating from the post-styloid parapharyngeal space and discuss the possible differential diagnosis on radiographic findings.

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Pazopanib-associated secondary adrenal insufficiency in a patient with malignant solitary fibrous tumor

Journal of Oncology Pharmacy Practice ◽

10.1177/10781552211016081 ◽

2021 ◽

pp. 107815522110160

Author(s):

Muhammed Muhiddin Er ◽

Murat Araz ◽

Meryem Karabacak ◽

Muzaffer Uğraklı ◽

Melek Karakurt Eryılmaz ◽

...

Keyword(s):

Adrenal Insufficiency ◽

Solitary Fibrous Tumor ◽

Single Case ◽

Soft Tissue Sarcomas ◽

Pituitary Hormones ◽

Secondary Adrenal Insufficiency ◽

Primary Adrenal Insufficiency ◽

Hypoglycemic Attack ◽

Fibrous Tumor

Introduction Pazopanib is an agent that is being successfully used in soft tissue sarcomas. Some endocrine side effects may develop during pazopanib treatment. Here, we presented a case diagnosed with secondary adrenal insufficiency while being investigated for etiology of hypoglycemia which developed after pazopanib. Case report A 69-year-old male patient was operated in June 2019 due to a lung mass 26 × 18 × 10 cm in size. Pathological diagnosis revealed a solitary fibrous tumor with malignant behavior. The patient received three lines of chemotherapy. After pazopanib treatment, a hypoglycemic attack was reported. Management and outcome: Blood cortisol and ACTH (Adrenocorticotropic hormone) levels were not increased at the time of the hypoglycemic attack, and levels of other pituitary hormones were found to be normal. Electrolyte levels were in normal range. Since the counteracting hormone did not reach a sufficient level, it was considered secondary adrenal insufficiency. Hypoglycemic attacks did not occur during follow-up while taking steroid therapy and pazopanib. Discussion A single case of primary adrenal insufficiency has been reported in the literature. We here present a case who developed hypoglycemia after pazopanib and was diagnosed with drug-associated secondary adrenal insufficiency. When hypoglycemia develops during pazopanib treatment, we must be aware of adrenal insufficiency.

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Solitary Fibrous Tumors of the Chest: An Analysis of Fifty Patients

Frontiers in Oncology ◽

10.3389/fonc.2021.697156 ◽

2021 ◽

Vol 11 ◽

Author(s):

Jingwen Zhang ◽

Jumin Liu ◽

Zhihao Zhang ◽

Beizong Tian

Keyword(s):

Solitary Fibrous Tumor ◽

Chest Tightness ◽

Ki 67 ◽

Imaging Characteristics ◽

Enhanced Ct ◽

The Common ◽

The Difference ◽

Low Incidence ◽

Fibrous Tumor

BackgroundA solitary fibrous tumor of the chest (SFTC) is a subtype of solitary fibrous tumor (SFT) with a low incidence rate. The purpose of this study is to analyze the diagnosis and treatment of SFTC and the difference between benign and malignant solitary fibrous tumor of the pleura (SFTP) to improve the understanding of this rare disease.MethodsA retrospective analysis of fifty patients with SFTC (33 cases in the pleura and 17 in the lung) was performed. Clinical and imaging characteristics, pathological features, and treatment follow-up outcomes were analyzed.ResultsThe common symptoms of the 50 patients included a cough, expectoration, chest tightness, fever, and chest pain. Space occupying lesions were found via plain computed tomography (CT) and enhanced CT was used for enhancement of the tumors. It was also found that 18 cases had necrosis, and 5 cases had calcification. The histopathology results showed that frequent nuclear division, obvious morphological variation, necrosis, and the high expression of Ki-67 cells are markers of malignant SFTC. There were significant differences in age, chest tightness, necrotic foci in CT, and expression of Ki-67 between the benign and malignant SFTP cases. All the patients who received treatment were given an excellent prognosis.ConclusionA combination of enhanced CT, histopathology, and immunohistochemistry can be used for the accurate diagnosis of SFTC. Advanced age, chest tightness, necrotic foci in CT, and a high Ki-67 index were more likely to be malignant SFTP. Operation and radiofrequency ablation can provide favorable outcomes for both benign and malignant SFTC.

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Clinical presentation, natural history and therapeutic approach in patients with solitary fibrous tumor: A retrospective analysis.

Journal of Clinical Oncology ◽

10.1200/jco.2019.37.15_suppl.e22522 ◽

2019 ◽

Vol 37 (15_suppl) ◽

pp. e22522-e22522

Author(s):

Patrick Schoffski ◽

Iris Timmermans ◽

Daphne Hompes ◽

Marguerite Stas ◽

Veerle Boecxstaens ◽

...

Keyword(s):

Retrospective Analysis ◽

Metastatic Disease ◽

Solitary Fibrous Tumor ◽

Systemic Therapy ◽

Single Agent ◽

Disease Free Survival ◽

Metachronous Metastasis ◽

Palliative Intent ◽

Fibrous Tumor

e22522 Background: Solitary fibrous tumor (SFT), a rare variant of soft tissue sarcoma (STS), is characterized by the presence of a NAB2-STAT6 fusion. Given the orphan character of SFT we performed a retrospective analysis. Methods: We retrospectively reviewed all patients (pts) with SFT treated in our institution between 12/1990 - 09/2017. Results: We identified 94 SFT pts (incl. hemangiopericytoma) with a med. follow-up for 4.7 yrs. Common anatomic sites were chest (33%), abdomen (21.3%), brain (12.8%) and extremities (9.6%). The symptomatology at diagnosis was variable. Only 6.4% presented with synchronous metastasis. Hypoglycemia (Doege-Potter syndrome) was seen in 2.1% of cases. A resection of the primary SFT was done in 86 pts (91.5%), their disease-free survival was 35.5 mo and 43% stayed SFT-free during follow-up. Local recurrence occurred in 26.7% of cases, associated with a med. overall survival (OS) of 45.1 mo. Metachronous metastasis was seen in 30.2% of pts, occurring after a med. follow-up of 36 mo. Med. OS after diagnosis of metastasis was 19.0 mo. Systemic therapy was given to 92.9% of pts with inoperable/metastatic disease. The most common 1st line therapy was doxorubicin single agent (57.7% of pts), achieving responses in 13.3% of pts. 2nd line therapies included ifosfamide and pazopanib (31.3% of pts each), 3rd line treatment was very heterogeneous. Conclusions: SFT is an orphan malignancy with a variable clinical course, low incidence of distant spread at first diagnosis but considerable risk of local failure and metachronous metastasis. Surgery is the only curative option at diagnosis, time of relapse and in case of resectable metastasis. Palliative systemic therapy is considered in pts with inoperable/metastatic disease but achieves low response rates. The natural course and survival outcomes of SFT cases treated with palliative intent tend to be better than in non-selected STS pts.

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Recurrent Solitary Fibrous Tumor of the Pleura With Malignant Transformation

Archives of Pathology & Laboratory Medicine ◽

10.5858/2004-128-460-rsftot ◽

2004 ◽

Vol 128 (4) ◽

pp. 460-462

Author(s):

Rani Kanthan ◽

Bahman Torkian

Keyword(s):

Malignant Transformation ◽

Solitary Fibrous Tumor ◽

Immunohistochemical Staining ◽

White Woman ◽

Accurate Diagnosis ◽

Malignant Progression ◽

Biological Behavior ◽

Fibrous Tumor

Abstract Recurrent solitary fibrous tumor of the pleura with malignant progression occurs rarely. We report a case of solitary fibrous tumor of the pleura in an 85-year-old white woman that recurred 4 times during a span of 10 years and subsequently underwent malignant transformation. The accurate diagnosis of solitary fibrous tumor is aided by ancillary techniques, such as immunohistochemical staining; however, with malignant transformation, such tools may be of limited value. Long-term clinical follow-up is recommended for all patients with solitary fibrous tumor because of the potential adverse biological behavior of this tumor, which may lead to repeated recurrences and/or malignant transformation.

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Two case reports of rare diseases occurring in rare parts: Splenic vein solitary fibrous tumor and Liver solitary fibrous tumor

10.21203/rs.3.rs-32710/v1 ◽

2020 ◽

Author(s):

Wenjing Wang ◽

Banghe Bao ◽

Anbin Hu ◽

Xiaofeng Zhu ◽

Qing Chen

Keyword(s):

Liver Transplantation ◽

Surgical Resection ◽

Orthotopic Liver Transplantation ◽

Solitary Fibrous Tumor ◽

Primary Tumor ◽

Splenic Vein ◽

Tumor Site ◽

Primary Tumor Site ◽

Fibrous Tumor

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.

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Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis

Sarcoma ◽

10.1155/2020/1385978 ◽

2020 ◽

Vol 2020 ◽

pp. 1-9 ◽

Cited By ~ 2

Author(s):

P. Schöffski ◽

I. Timmermans ◽

D. Hompes ◽

M. Stas ◽

F. Sinnaeve ◽

...

Keyword(s):

Solitary Fibrous Tumor ◽

Systemic Therapy ◽

Median Overall Survival ◽

Progression Free Survival ◽

Response Rates ◽

Free Survival ◽

Metachronous Metastasis ◽

Third Line ◽

Fibrous Tumor

Background. Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). Materials and Methods. We reviewed SFT patients (pts) treated at our institution between 12/1990 and 09/2017. Results. We identified 94 pts with a median follow-up (mFU) of 4.7 years (range: 0.1–21.53). Primary sites were the chest (33%), abdomen (21.3%), brain (12.8%), and extremities (9.6%); 6.4% of pts presented with synchronous metastasis. Median overall survival (mOS) from the first diagnosis was 56.0 months (m) (0.3–258.3). Doege–Potter syndrome was seen in 2.1% of pts. Primary resection was performed in 86 pts (91.5%). Median progression-free survival was 34.1 m (1.0–157.1), and 43% of pts stayed SFT-free during FU. Local recurrence occurred in 26.7% after a mFU of 35.5 m (1.0–153.8), associated with an OS of 45.1 m (4.7–118.2). Metachronous metastasis occurred in 30.2% after a mFU of 36.0 m (0.1–157.1). OS in metastatic pts was 19.0 m (0.3–149.0). Systemic therapy was given to 26 pts (27.7%) with inoperable/metastatic disease. The most common (57.7%) upfront therapy was doxorubicin, achieving responses in 13.3% of pts with a PFS of 4.8 m (0.4–23.8). In second line, pts were treated with ifosfamide or pazopanib, the latter achieving the highest response rates. Third-line treatment was heterogeneous. Conclusion. SFT is an orphan malignancy with a highly variable clinical course and a considerable risk of local failure and metachronous metastasis. Surgery is the only curative option; palliative systemic therapy is used in inoperable/metastatic cases but achieves low response rates. The highest response rates are seen with pazopanib in second/third line.

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Solitary Fibrous Tumor of the Parapharyngeal Space: A Case Report and Review of the Literature

Ear Nose & Throat Journal ◽

10.1177/014556139607501011 ◽

1996 ◽

Vol 75 (10) ◽

pp. 681-684 ◽

Cited By ~ 15

Author(s):

Kunal Gangopadhyay ◽

Khalid Taibah ◽

M. Babu Manohar ◽

Hala Kfoury

Keyword(s):

Case Report ◽

Head And Neck ◽

Solitary Fibrous Tumor ◽

Spindle Cell ◽

Parapharyngeal Space ◽

English Literature ◽

Review Of The Literature ◽

Solitary Fibrous Tumors ◽

Rare Location ◽

Fibrous Tumor

Solitary fibrous tumors are uncommon spindle cell neoplasms generally associated with serosal surfaces, especially the pleura. Recently, these tumors have been documented in a number of extrapleural sites including the head and neck. So far only two cases of parapharyngeal solitary fibrous tumor have been reported in the English literature. Rare location of an uncommon lesion often gives rise to difficulty in diagnosis or to misdiagnosis. In both the previously reported cases, as well as in our case, the diagnosis of solitary fibrous tumor was not made until the excised tumor was subjected to histopathology and immunohistochemistry.

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Dedifferentiated Solitary Fibrous Tumor: A Concise Review

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2016-0570-rs ◽

2018 ◽

Vol 142 (6) ◽

pp. 761-766 ◽

Cited By ~ 11

Author(s):

Nicholas J. Olson ◽

Konstantinos Linos

Keyword(s):

Solitary Fibrous Tumor ◽

Correct Diagnosis ◽

The Body ◽

Molecular Alterations ◽

Concise Review ◽

Mesenchymal Neoplasm ◽

Fibrous Tumor ◽

Aggressive Clinical Course ◽

Age Range ◽

Ancillary Studies

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.

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