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Original Articles
- Meningeal Solitary Fibrous Tumors with Delayed Extracranial Metastasis
- Nayoung Han, Hannah Kim, Soo Kee Min, Sun-Ha Paek, Chul-Kee Park, Seung-Hong Choi, U-Ri Chae, Sung-Hye Park
- J Pathol Transl Med. 2016;50(2):113-121. Published online December 14, 2015
- DOI: https://doi.org/10.4132/jptm.2015.10.30
- 10,355 View
- 114 Download
- 21 Web of Science
- 18 Crossref
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Abstract
PDF - Background
The term solitary fibrous tumor (SFT) is preferred over meningeal hemangiopericytoma (HPC), because NAB2-STAT6 gene fusion has been observed in both intracranial and extracranial HPCs. HPCs are now considered cellular variants of SFTs. Methods: This study analyzes 19 patients with STAT6-confirmed SFTs, who were followed for over 11 years in a single institution. Ten patients (10/19, 56.2%) had extracranial metastases (metastatic group), while the remainder (9/19) did not (non-metastatic group). These two groups were compared clinicopathologically. Results: In the metastatic group, the primary metastatic sites were the lungs (n = 6), bone (n = 4), and liver (n = 3). There was a mean lag time of 14.2 years between the diagnosis of the initial meningeal tumor to that of systemic metastasis. The median age at initial tumor onset was 37.1 years in the metastatic group and 52.5 in the non-metastatic group. The 10-year survival rates of the metastatic- and non-metastatic groups were 100% and 33%, respectively. The significant prognostic factors for poor outcomes on univariate analysis included advanced age (≥45 years) and large initial tumor size (≥5 cm). In contrast, the patients with higher tumor grade, high mitotic rate (≥5/10 high-power fields), high Ki-67 index (≥5%), and the presence of necrosis or CD34 positivity showed tendency of poor prognosis but these parameters were not statistically significant poor prognostic markers. Conclusions: Among patients with SFTs, younger patients (<45 years) experienced longer survival times and paradoxically had more frequent extracranial metastases after long latent periods than did older patients. Therefore, young patients with SFTs require careful surveillance and follow-up for early detection of systemic metastases. -
Citations
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- Meningeal Solitary Fibrous Tumor: A Single-Center Retrospective Cohort Study
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International Journal of Neuroscience.2022; 132(8): 843. CrossRef - Long-term extracranial metastatic relapse of an intraventricular solitary fibrous tumor: a case report
Tarek Assi, Elie Samaha, Hussein Nassereddine
Anti-Cancer Drugs.2022; 33(1): e764. CrossRef - Multidisciplinary Treatment of Liver Metastases from Intracranial SFTs/HPCs: A Report of Three Consecutive Cases
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Kyoung Su Sung, Ju Hyung Moon, Eui Hyun Kim, Seok-Gu Kang, Se Hoon Kim, Chang-Ok Suh, Sun Ho Kim, Kyu-Sung Lee, Won Seok Chang, Jong Hee Chang
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Nicolas Macagno, Rob Vogels, Romain Appay, Carole Colin, Karima Mokhtari, Benno Küsters, Pieter Wesseling, Dominique Figarella‐Branger, Uta Flucke, Corinne Bouvier
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Yongjie Wang, Jingying Zhang, Qichang Liu, Fuyi Liu, Xiangdong Zhu, Jianmin Zhang
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AiJun Peng, LiBing Zhang, Hai Zhao, LiangXue Zhou
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Tarini Ratneswaren, Florence Rosie Avila Hogg, Mathew Joseph Gallagher, Keyoumars Ashkan
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Eveline Claus, Patrick Seynaeve, Jeroen Ceuppens, Alain Vanneste, Koenraad Verstraete
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Ji Min Kim, Yoon-La Choi, Yu Jin Kim, Hyung Kyu Park
Pathology - Research and Practice.2017; 213(6): 619. CrossRef - Molecular Testing of Brain Tumor
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Vittoria Colia, Salvatore Provenzano, Carlo Morosi, Paola Collini, Salvatore Lorenzo Renne, Paolo G. Dagrada, Claudia Sangalli, Angelo Paolo Dei Tos, Andrea Marrari, Paolo G. Casali, Silvia Stacchiotti
Clinical Sarcoma Research.2016;[Epub] CrossRef
- Meningeal Solitary Fibrous Tumor: A Single-Center Retrospective Cohort Study
- Diagnostic Utility of the JAZF1/JJAZ1 Gene Fusion in Endometrial Stromal Sarcomas and Their Histologic Variants.
- Sang Ryung Lee, Joon Seon Song, Ga Hye Kim, Jene Choi, Hyung Kyoung Kim, Yonghee Lee, Kyu Rae Kim
- Korean J Pathol. 2011;45(5):498-505.
- DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.5.498
- 3,031 View
- 30 Download
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Abstract
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- BACKGROUND
The diagnosis of endometrial stromal sarcoma (ESS) is often difficult in cases showing diverse histological differentiation or in undifferentiated endometrial sarcoma (UES). Recently, JAZF1/JJAZ1 gene fusion has been described as a defining feature of low-grade ESS (LGESS). However, its prevalence is variably reported, and the diagnostic utility has rarely been examined for cases showing various histological differentiation.
METHODS
To test the diagnostic utility of JAZF1/JJAZ1 gene fusion in difficult cases, we compared the prevalence of the JAZF1/JJAZ1 fusion gene in LGESS with and without histological differentiation.
RESULTS
The JAZF1/JJAZ1 fusion transcript was detected in 18 of 21 LGESS (85.7%), including 14 classical LGESS (93%), four LGESS with diverse histological differentiation (67%), and two with UES (28.6%). Positive cases included two LGESS with sex cord-like differentiation, one with osseous differentiation, and two UES. LGESS showing smooth muscle differentiation revealed the fusion transcript only in the classic area. Direct sequencing analysis of two LGESS revealed a previously reported breakpoint at t(7;17)(p15;q21).
CONCLUSIONS
The JAZF1/JJAZ1 fusion gene was identified in a significant proportion of LGESS showing secondary histological differentiation except in cases with smooth muscle differentiation. Thus, this fusion gene may be useful to confirm the diagnosis in difficult cases of LGESS.
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