Papillary Solitary Fibrous Tumor/Hemangiopericytoma: An Uncommon Morphological Form With NAB2-STAT6 Gene Fusion

Author:

Yao Zhi-Gang12ORCID,Wu Hai-Bo3,Hao Ying-Hua4,Wang Xing-Fu5,Ma Guang-Zhen6,Li Jia12,Li Ji-Feng27,Lin Chun-Hu12,Zhong Xiu-Ming12,Wang Zhou12,Wang Dai-Zhong4

Affiliation:

1. Department of Pathology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China

2. Shandong Provincial Hospital Affiliated to Shandong First Medical University, Tai’an, China

3. Department of Pathology, The First Affiliated Hospital of University of Science and Technology of China (USTC), Division of Life Sciences and Medicine of USTC, Hefei, Anhui, China

4. Department of Pathology, Taihe Hospital, Hubei University of Medicine, Hubei, China

5. Department of Pathology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China

6. Department of Pathology, The Second People’s Hospital of Liaocheng, Linqing, China

7. Department of Neurology, Shandong Provincial Hospital Affiliated to Shandong University, Jinan, China

Abstract

Abstract Solitary fibrous tumor/hemangiopericytomas (SFT/HPCs) are mesenchymal tumors characterized by “staghorn” blood vessels and collagen deposition. Little is known about SFT/HPCs with papillary architecture. We summarized the clinicopathologic features of 12 patients with papillary SFT/HPCs (8 males and 4 females; median age: 59 years), including 8 previously reported cases. Tumors were present in the meninges (75%, 9/12), adrenal gland (8%, 1/12), orbit (8%, 1/12), or spinal canal (8%, 1/12). Six tumors (50%) had a true papillary architecture with fibrovascular cores and 6 tumors (50%) had a pseudopapillary architecture with vascular cores. Nuclear staining for STAT6 was present in all tested tumors (10/10). RT-PCR indicated NAB2 ex6-STAT6 ex17 fusion in 4 tumors (80%, 4/5) and NAB2 ex4-STAT6 ex2 fusion in 1 tumor (20%, 1/5). Five patients (42%, 5/12), all with tumors in the meninges, developed local recurrence at a median of 61 months after surgery (range: 56–165 months; mean: 88.6 months). These results indicated that the papillary architecture is a morphological form of SFT/HPCs. The recognition of this pattern, with appropriate immunohistochemical analysis and assessment of NAB2-STAT6 fusion, should facilitate the distinction of these rare neoplasms from morphologically similar tumors in the meninges, lung, pleura, and soft tissue.

Funder

Key Research and Development Project of Shandong Province

Shandong Medical and Health Science Technology Development Program

Natural Science Foundation of China

Publisher

Oxford University Press (OUP)

Subject

Cellular and Molecular Neuroscience,Clinical Neurology,Neurology,General Medicine,Pathology and Forensic Medicine

Reference31 articles.

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