Article
Oncology
Wen-Tong Ji, Yu Hu, Yao Wang
Summary: This article reports a typical case of kidney solitary fibrous tumor (SFT), which contributes to the existing diagnostic methods and emphasizes the importance of STAT6 mutations in SFT.
FRONTIERS IN ONCOLOGY
(2022)
Article
Pathology
Tatiana Georgiesh, Heidi Maria Namlos, Nitin Sharma, Susanne Loren, Ola Myklebost, Bodil Bjerkehagen, Leonardo A. Meza-Zepeda, Kjetil Boye
Summary: This study found that NAB2-STAT6 fusion variants are associated with distinct clinicopathological and molecular characteristics in solitary fibrous tumour patients, and have prognostic significance in extrameningeal SFT.
Article
Oncology
Haruna Nonaka, Shuya Kandori, Satoshi Nitta, Masanobu Shiga, Yoshiyuki Nagumo, Tomokazu Kimura, Takashi Kawahara, Hiromitsu Negoro, Akio Hoshi, Takahiro Kojima, Koji Kawai, Bryan J. Mathis, Takuro Tamura, Taka-Aki Sato, Mariko Yamato, Masayuki Noguchi, Hiroyuki Nishiyama
Summary: Solitary fibrous tumors (SFT) are usually originated from the visceral pleura with a favorable prognosis, but they can also occur at various extrapleural sites. This study reports a rare case of retroperitoneal SFT with malignant behavior and dedifferentiation. The downregulation of NAB2-STAT6 fusion gene at the transcriptional level is associated with malignant SFT for the first time, supporting the idea that TP53 mutations promote malignancy in SFTs.
FRONTIERS IN ONCOLOGY
(2021)
Article
Pathology
Matthias Bieg, Evgeny A. Moskalev, Rainer Will, Simone Hebele, Matthias Schwarzbach, Sanja Schmeck, Peter Hohenberger, Jens Jakob, Bernd Kasper, Timo Gaiser, Philip Stroebel, Eva Wardelmann, Udo Kontny, Till Braunschweig, Horia Sirbu, Robert Gruetzmann, Norbert Meidenbauer, Naveed Ishaque, Roland Eils, Stefan Wiemann, Arndt Hartmann, Abbas Agaimy, Karen Fritchie, Caterina Giannini, Florian Haller
Summary: This study utilized next-generation sequencing-based gene expression profiling to identify significant differences in gene expression associated with anatomic localization and NAB2-STAT6 gene fusion variants in solitary fibrous tumors (SFTs). The research found that different fusion variants of NAB2-STAT6 were associated with distinct gene expression profiles, cellular morphology, and anatomical locations in SFTs. The study established a potential molecular biologic basis for clinicopathologic differences in SFTs with different NAB2-STAT6 gene fusion variants.
AMERICAN JOURNAL OF PATHOLOGY
(2021)
Article
Medicine, General & Internal
Boluwatito T. Abraham, Prithvi Balaji, Jae Woo Lee, Wendy Verola, John T. Williams
Summary: Solitary fibrous tumors (SFTs) are rare spindle cell neoplasms that most commonly occur in the pleura. While generally benign and asymptomatic, they can cause symptoms if they grow large enough to exert pressure on other organs. Diagnosis requires biopsy, and recent advances in immunohistochemistry and molecular diagnostics have been utilized to confirm SFTs. Treatment typically involves surgical resection and post-operative surveillance, with radiation therapy and chemotherapy not currently recommended.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Review
Oncology
Alannah Smrke, Khin Thway, Paul H Huang, Robin L. Jones, Andrew J. Hayes
Summary: Solitary fibrous tumor is a rare soft tissue sarcoma that mainly affects adults in their fifth and sixth decades of life, now recognized as a distinct subtype with the identification of NAB2-STAT6 fusion. Standard treatment includes surgical excision, with antiangiogenic therapy being effective for unresectable cases. Further translational research is needed to understand the underlying biology and develop more effective treatments for metastatic disease.
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Ziyu Guo, Wei Liu, Ziqian Dong, Lan Yang, Peng Xie
Summary: This is a rare case of malignant peripheral nerve sheath tumor involving the solitary lumbar vertebra. The patient presented with growing lumbocrural pain for 2 months. CT scan revealed a solitary vertebral lesion, highly suggestive of metastatic malignancy. F-18-FDG PET/CT showed heterogeneous intense FDG accumulation in the vertebral lesion with an SUVmax of 16.4. Pathological examination confirmed the diagnosis of malignant peripheral nerve sheath tumor. This case highlights the importance of considering MPNST when there is solitary vertebra invasion with increased FDG uptake.
CLINICAL NUCLEAR MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
David S. Moura, Juan Diaz-Martin, Silvia Bague, Ruth Orellana-Fernandez, Ana Sebio, Jose L. Mondaza-Hernandez, Carmen Salguero-Aranda, Federico Rojo, Nadia Hindi, Christopher D. M. Fletcher, Javier Martin-Broto
Summary: Solitary fibrous tumor is a rare subtype of soft-tissue sarcoma with NAB2-STAT6 gene fusion as the most common genetic driver alteration. This study identified a new NFIX-STAT6 gene fusion using targeted RNA-Seq in a patient, opening up new hypotheses for the role of STAT6 in the sarcomatogenesis of solitary fibrous tumor.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Pathology
Takahiro Shirakura, Yuichi Yamada, Satoshi Nakata, Bunsho Asayama, Yoshinobu Seo, Satoshi Tanikawa, Takayuki Kato, Nobukazu Komoribayashi, Naohiko Kubo, Nobuhiro Monma, Naoki Okura, Shinya Tanaka, Yoshinao Oda, Junko Hirato, Hideaki Yokoo, Sumihito Nobusawa
Summary: Solitary fibrous tumors (SFTs) are rare mesenchymal tumors that can occur at any location. This study analyzed 3 cases of meningeal SFT with ectopic salivary glands (ESGs) and found that ESGs may be associated with SFTs, especially in the cerebellopontine angle (CPA). Molecular analysis also revealed that meningeal SFTs with ESGs may be linked to the minor fusion variant of NAB2-STAT6.
Article
Clinical Neurology
Megan C. Everson, Courtney Pendleton, Megan M. Jack, Brandon W. Smith, Jodi M. Carter, Robert J. Spinner
Summary: Malignant perineurioma is a rare subset of MPNST with distinct clinical features. Patients in the study had uncomplicated clinical courses post-diagnosis, but further research is needed to fully understand the clinical course of these rare tumors.
WORLD NEUROSURGERY
(2021)
Review
Oncology
Giuseppe Bianchi, Debora Lana, Marco Gambarotti, Cristina Ferrari, Marta Sbaraglia, Elena Pedrini, Laura Pazzaglia, Luca Sangiorgi, Isabella Bartolotti, Angelo Paolo Dei Tos, Katia Scotlandi, Alberto Righi
Summary: In contrast to SFT of soft tissues, aggressive behavior of primary SFT of the bone appears to be independent from mitotic count or any other clinicopathological and molecular features.
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Liu Xiao, Yuhao Li, Lin Li
Summary: This article reports a rare case of MPNST in the first thoracic vertebra with lymph node metastasis in the left axillary region. It emphasizes the importance of considering MPNST as a differential diagnosis for solitary lytic thoracic vertebral lesions with intense FDG uptake. Furthermore, FDG PET/CT plays a crucial role in the initial staging of MPNST.
CLINICAL NUCLEAR MEDICINE
(2022)
Article
Oncology
Caroline Apra, Delphine Guillemot, Eleonore Frouin, Corinne Bouvier, Karima Mokhtari, Michel Kalamarides, Gaelle Pierron
Summary: The study revealed that meningeal solitary fibrous tumors (SFT) have a unique molecular signature distinct from other tumor tissues. Gene expression analysis showed significant over-expression of certain genes in CNS-SFT, and a novel pathogenic TP53 variant was identified in one sample of meningeal SFT.
JOURNAL OF NEURO-ONCOLOGY
(2021)
Review
Oncology
Giulio Bonomo, Alessandro Gans, Elio Mazzapicchi, Emanuele Rubiu, Paolo Alimonti, Marica Eoli, Rosina Paterra, Bianca Pollo, Guglielmo Iess, Francesco Restelli, Jacopo Falco, Francesco Acerbi, Marco Paolo Schiariti, Paolo Ferroli, Morgan Broggi
Summary: In this paper, the clinical and diagnostic features of Sporadic Spinal Psammomatous Malignant Melanotic Nerve Sheath Tumor (SSP-MMNST) are reviewed, and possible pathogenetic mechanisms are investigated to improve therapeutic strategies. A case of a young female with SSP-MMNST arising from C5-6 right spinal roots is also reported. The study found that SSP-MMNST has a high recurrence rate and residual disease is associated with a higher risk of systemic disease spreading, suggesting the importance of adjuvant radiation therapy.
FRONTIERS IN ONCOLOGY
(2023)
Article
Medicine, General & Internal
Katsuya Yanagisawa, Kenichi Nishie, Hidekazu Takahashi, Kenji Sano, Kazuyoshi Takei, Hiroshi Yamamoto, Tomonobu Koizumi, Masayuki Hanaoka
Summary: This case report describes a rare case of primary lung MPNST and suggests that radiotherapy is an effective treatment option for inoperable cases.
