Solitary Fibrous Tumor with Rapid Progression after 16 Years' Follow Up

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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Rapid progression of solitary fibrous tumor after induction of hemodialysis

Oxford Medical Case Reports ◽

10.1093/omcr/omx037 ◽

2017 ◽

Vol 2017 (7) ◽

Author(s):

Motoaki Yasukawa ◽

Chiho Ohbayashi ◽

Tomoko Uchiyama ◽

Takeshi Kawaguchi ◽

Norikazu Kawai ◽

...

Keyword(s):

Solitary Fibrous Tumor ◽

Rapid Progression ◽

Fibrous Tumor

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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 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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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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Malignant solitary fibrous tumor of the thyroid: a case-report and review of the literature

Arquivos Brasileiros de Endocrinologia & Metabologia ◽

10.1590/0004-2730000003230 ◽

2014 ◽

Vol 58 (4) ◽

pp. 402-406 ◽

Cited By ~ 5

Author(s):

Wellington Alves Filho ◽

Renata Regina da Graça Lorencetti Mahmoud ◽

Daniel Marin Ramos ◽

Vergilius José Furtado de Araujo-Filho ◽

Patricia Picciarelli de Lima ◽

...

Keyword(s):

Thyroid Gland ◽

Solitary Fibrous Tumor ◽

Spindle Cell ◽

Spindle Cell Neoplasm ◽

Cellular Atypia ◽

Long Term Follow Up ◽

History Of ◽

Fibrous Tumor

Solitary fibrous tumor (SFT) is an uncommon spindle-cell neoplasm that most often involves the pleura, rarely occurring in extra-thoracic locations. Twenty-six cases of SFT arising in the thyroid gland have been described. We report a case of a 60-year-old woman presenting an 8-month history of enlargement of the neck associated with dysphagia. The patient underwent a right hemithyroidectomy and SFT of the thyroid was diagnosed. Immunohistochemistry showed positivity for CD34 marker, and the high number of mitoses and the presence of cellular atypia suggested that the tumor was malignant. To our knowledge, this is the second case of malignant SFT of the thyroid gland ever reported. Due to the rarity of these tumors, the indication of adjuvant therapy and prognosis are uncertain. Long-term follow-up after surgical resection seems to be advisable.

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