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

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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Primary endodermal hemangiopericytoma/solitary fibrous tumor of the cervical spine: a case report and literature review

BMC Surgery ◽

10.1186/s12893-021-01399-6 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Liyun Wang ◽

Jianbo Yu ◽

Dongping Shu ◽

Bin Huang ◽

Yumin Wang ◽

...

Keyword(s):

Cervical Spine ◽

Magnetic Resonance ◽

Solitary Fibrous Tumor ◽

Postoperative Radiotherapy ◽

Immunohistochemical Analysis ◽

Resonance Spectroscopy ◽

Common Location ◽

Imaging Characteristics ◽

Fibrous Tumor

Abstract Background Hemangiopericytoma (HPC), also known as solitary fibrous tumor (SFT), is a type of soft tissue sarcoma with a special aggressive behavior. The HPC/SFT is locally aggressive with possibility of late recurrence locally or distant extraneural metastasis. The most common location of this HPC/SFT is the lower extremities. The HPC/SFT in the central nervous system (CNS) is very rare, and compared with the brain, it is rarer in the spinal region. However, clinicians also lack an overall understanding of the diagnosis of HPC/SFT in the spinal cord. Case presentation In this study, we report a rare case of primary cervical spine HPC/SFT in a 53-year-old woman. Two to three weeks before admission, she experienced pain and numbness in her left upper extremity. After computerized tomography (CT) and magnetic resonance imaging (MRI), a gross total resection was performed. Obvious neurological improvement was observed postoperatively. The pain and numbness in the patient's left upper limb were relieved subsequently. We then reviewed the literature on HPC/SFT, such as its clinical presentation, imaging characteristics, treatment, and follow-up. Conclusions Diagnosis of HPC/SFT relies on magnetic resonance spectroscopy, enhanced CT, and MRI. Postoperative radiotherapy is strongly recommended to reduce the HPC/SFT recurrence. Immunohistochemical analysis can also help in the differential diagnosis. However; early and long-term follow-up is necessary for patients.

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The Diagnosis and Treatment of Orbital Solitary Fibrous Tumor

10.21203/rs.3.rs-109816/v1 ◽

2020 ◽

Author(s):

Mingyu Ren ◽

Yixiang Wu ◽

Ruimiao Li ◽

Jing Li ◽

Jingjing Wang ◽

...

Keyword(s):

Solitary Fibrous Tumor ◽

Immunohistochemical Analysis ◽

Integrated Approach ◽

Demographic Information ◽

Immunohistochemical Examination ◽

Ki 67 ◽

The Mean ◽

Fibrous Tumor ◽

Irregular Mass

Abstract BackgroundSolitary fibrous tumor (SFT) is an uncommon spindle-cell tumor of mesenchymal origin, it commonly occurs within pleura, the occurrence of SFT in the orbit is very rare. The diagnosis of orbital SFT is challenging in clinical, and requires an integrated approach that includes specific clinical, histological, immunohistochemical, and even molecular findings.MethodsIn our retrospective study, we collected 13 patients with orbital SFT. All patients were diagnosed with orbital SFT by postoperative histopathological and immunohistochemical examination. Patient charts and medical records were reviewed for demographic information, relevant medical and family history, clinical presentation, radiological examination, histopathological and immunohistochemical examination, the treatment and prognosis.ResultsThis study included 7 males and 6 females. The age ranged from 11 to 78 years. 7 patients presented with predominant features of exophthalmos. 7 patients showed eyeball dislocation. 6 patients with motility disturbances. 9 patients had the palpable masses. 6 patients had the secondary lesions. On CT scan, there were 6 patients were located in the superomedial quadrant of the orbit, 3 patients were located in the inferomedial quadrant of the orbit. The morphology of lesions was solitary ovoid mass in 10 patients, and irregular mass in 3 patients. The CT value of the tumor was 22.8-64.4Hu, with the median of 45.9Hu. On MRI, 3 patients showed hypointense mixed signals on T1WI, 10 patients showed isointense mixed signal on T1WI. 3 patients showed hypointense mixed signals, 4 patients showed isointense mixed signals, 6 patients showed hyperintense signals on T2WI. There were 12 patients showed obviously enhancement, and we found patchy lesions which were no enhancement in the tumor. All patients were treated by surgery. Immunohistochemical analysis showed that the tumor cells exhibited diffuse immunoreactivity for CD34 and CD99 in all the patients. The lesions were positive for Bcl-2 in 11 patients. The lesions were positivity for Ki-67 <5% in one patient, 5%-10% in 10 patients, >10% in 2 patients. ConclusionsThe lesions are often outside the muscular cone, most commonly located at superomedial quadrant and inferomeddial quadrant of the orbit. The density of the lesions is uneven in most cases, the mean CT values of the tumors on CT scans are variable. The signal of lesions on orbital MRI is uncertainty, it is inheterogeneous enhancement, and the lamellar unenhanced regions can also be found in the lesion. The complete gross resection, and even more aggressive wide excision is preferred. Although the Ki-67 labelling index is very low, malignant forms with an increased propensity for local recurrence have been found. A longer follow-up is needed to determine the recurrence rate of the disease, and to identify the causes of recurrence.

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Solitary Fibrous Tumor of the Kidney Developing Local Recurrence

Case Reports in Urology ◽

10.1155/2016/2426874 ◽

2016 ◽

Vol 2016 ◽

pp. 1-8

Author(s):

Wataru Usuba ◽

Hideo Sasaki ◽

Hidekazu Yoshie ◽

Kazuki Kitajima ◽

Hiroya Kudo ◽

...

Keyword(s):

Local Recurrence ◽

Solitary Fibrous Tumor ◽

Abdominal Ct ◽

Nuclear Pleomorphism ◽

Ki 67 ◽

Tumor Bed ◽

Collagenous Tissue ◽

Resected Tumor ◽

Fibrous Tumor

Solitary fibrous tumor (SFT) of the kidney is a rare entity and usually displays a favorable prognosis. We herein report a second case of renal SFT developing local recurrence. A 50-year-old man was referred to our hospital because of a left renal mass. An abdominal CT detected a large renal tumor and radical nephrectomy was performed with a possible diagnosis of renal cell carcinoma. The resected tumor size was measured at 17 × 11 × 8 cm. Grossly, necrosis was observed in central lesion of the tumor but hemorrhage was not observed. Microscopically, the tumor consisted of spindle-shaped cells with scant cytoplasm accompanied by hyalinized collagenous tissue, which displayed hemangiopericytomatous patterns. The cellularity was normal and nuclear pleomorphism was not observed. Ki-67 labeling index was less than 3%. The pathological diagnosis of SFT was made without obvious malignant findings. Three years after the surgery, a follow-up CT scan detected a mass lesion in the tumor bed. Surgical resection was performed and the resected tumor was compatible with local recurrence of the SFT without obvious malignant findings. Renal SFT should be carefully monitored even in the absence of obvious malignant 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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Basic FGF and Ki-67 proteins useful for immunohistological diagnostic evaluations in malignant solitary fibrous tumor

Pathology International ◽

10.1046/j.1440-1827.2003.01474.x ◽

2003 ◽

Vol 53 (5) ◽

pp. 284-290 ◽

Cited By ~ 48

Author(s):

Yuliang Sun ◽

Zenya Naito ◽

Toshiyuki Ishiwata ◽

Shotaro Maeda ◽

Yuichi Sugisaki ◽

...

Keyword(s):

Solitary Fibrous Tumor ◽

Ki 67 ◽

Basic Fgf ◽

Fibrous Tumor

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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 Kidney: A Case Report

Case Reports in Urology ◽

10.1155/2013/147496 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4

Author(s):

Abdullah Demirtaş ◽

Volkan Sabur ◽

Hülya Akgün ◽

Emre Can Akınsal ◽

Deniz Demirci

Keyword(s):

Solitary Fibrous Tumor ◽

Cystic Lesion ◽

Spindle Cell ◽

Left Kidney ◽

Ki 67 ◽

Spindle Cell Neoplasm ◽

Cell Neoplasm ◽

Fibrous Tumor ◽

Immunohistochemical Studies ◽

Hmb 45

Solitary fibrous tumor is a spindle cell neoplasm mostly originating from pleura; however, it has also recently been reported to be extrapleural. A 57-year-old man presented with left lumbal pain. Ultrasonography and computed tomography showed a cystic lesion of 14 × 11 cm with solid areas and septations in middle and lower poles of the left kidney. Radical nephrectomy was performed. Immunohistochemical studies showed strong reactions with CD34 and CD99. A nuclear positivity with Ki-67 was observed in less than 1% of cells. Despite repeated stainings with vimentin, no clear tumor evaluation could be made due to artifacts. The tumor was negative with Bcl-2, desmin, HMB-45, S100, FVIII, and CD31. Histopathological and molecular studies made the diagnosis of a solitary fibrous tumor. The patient is now currently free of disease at the 26th month of followup.

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