Hemangiopericytomas in the Central Nervous System: A Multicenter Study of Korean Cases with Validation of the Usage of STAT6 Immunohistochemistry for Diagnosis of Disease

Junjeong Choi, Sung Hye Park, Shin Kwang Khang, Yeon Lim Suh, Sang Pyo Kim, Youn Soo Lee, Hyun Seok Kwon, Seok Gu Kang, Se Hoon Kim

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Background: Hemangiopericytoma (HPC) in the central nervous system (CNS) is a rare disease with distinctive biological/clinical characteristics compared with meningioma. Methods: Cases of HPCs of the CNS were collected, and clinicopathological records were retrospectively reviewed and analyzed. Immunohistochemistry (IHC) for proliferative markers (Ki-67, PHH3) and STAT6 were performed. Results: A total of 140 cases were collected, with mean follow-up of 77 months (median 58.8 months; range 0.53–540.5 months). 1-, 5-, 10-, and 20-year survival rates were 99.1, 94.0, 74.4, and 61.9 %, respectively. Thirty-nine patients (27.9 %) had recurrent disease. Mean and median times to recurrence were 62.9 and 47.3 months with 1-, 5-, 10-, and 20-year recurrence-free survival rates of 98.3, 78.3, 50.1, and 11.0 %, respectively. Thirteen patients (9.3 %) developed extracranial metastases. No adjuvant radiation therapy, higher histologic grades, failure of gross-total resection, and cases with gamma-knife surgery (GKS) were factors associated with shorter disease-free survival (log-rank test, p = 0.02, 0.00, 0.02, 0.00), among which high histologic grade and cases with GKS were significant in multivariable analysis. Strong nuclear STAT6 expression was noted in HPCs in 62 cases of HPCs (60/62, 96.8 %), whereas diffuse weak positivity was demonstrated in all meningioma cases. Conclusions: The survival rate in patients with HPC of the CNS is comparable to that of previously reported series. Recurrence remains a critical clinical issue of the disease. Identification of NAB2-STAT6 fusion transcript with surrogate IHC marker is a valuable diagnostic tool in the differential diagnosis of the disease.

Original languageEnglish
Pages (from-to)954-961
Number of pages8
JournalAnnals of Surgical Oncology
Volume23
DOIs
Publication statusPublished - 2016 Dec 1

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Hemangiopericytoma
Multicenter Studies
Survival Rate
Central Nervous System
Immunohistochemistry
Meningioma
Recurrence
Rare Diseases
Disease-Free Survival
Differential Diagnosis
Radiotherapy
Biomarkers
Neoplasm Metastasis

All Science Journal Classification (ASJC) codes

  • Surgery
  • Oncology

Cite this

@article{93470262b7084214833c86e27bc12a2e,
title = "Hemangiopericytomas in the Central Nervous System: A Multicenter Study of Korean Cases with Validation of the Usage of STAT6 Immunohistochemistry for Diagnosis of Disease",
abstract = "Background: Hemangiopericytoma (HPC) in the central nervous system (CNS) is a rare disease with distinctive biological/clinical characteristics compared with meningioma. Methods: Cases of HPCs of the CNS were collected, and clinicopathological records were retrospectively reviewed and analyzed. Immunohistochemistry (IHC) for proliferative markers (Ki-67, PHH3) and STAT6 were performed. Results: A total of 140 cases were collected, with mean follow-up of 77 months (median 58.8 months; range 0.53–540.5 months). 1-, 5-, 10-, and 20-year survival rates were 99.1, 94.0, 74.4, and 61.9 {\%}, respectively. Thirty-nine patients (27.9 {\%}) had recurrent disease. Mean and median times to recurrence were 62.9 and 47.3 months with 1-, 5-, 10-, and 20-year recurrence-free survival rates of 98.3, 78.3, 50.1, and 11.0 {\%}, respectively. Thirteen patients (9.3 {\%}) developed extracranial metastases. No adjuvant radiation therapy, higher histologic grades, failure of gross-total resection, and cases with gamma-knife surgery (GKS) were factors associated with shorter disease-free survival (log-rank test, p = 0.02, 0.00, 0.02, 0.00), among which high histologic grade and cases with GKS were significant in multivariable analysis. Strong nuclear STAT6 expression was noted in HPCs in 62 cases of HPCs (60/62, 96.8 {\%}), whereas diffuse weak positivity was demonstrated in all meningioma cases. Conclusions: The survival rate in patients with HPC of the CNS is comparable to that of previously reported series. Recurrence remains a critical clinical issue of the disease. Identification of NAB2-STAT6 fusion transcript with surrogate IHC marker is a valuable diagnostic tool in the differential diagnosis of the disease.",
author = "Junjeong Choi and Park, {Sung Hye} and Khang, {Shin Kwang} and Suh, {Yeon Lim} and Kim, {Sang Pyo} and Lee, {Youn Soo} and Kwon, {Hyun Seok} and Kang, {Seok Gu} and Kim, {Se Hoon}",
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doi = "10.1245/s10434-016-5414-x",
language = "English",
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pages = "954--961",
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Hemangiopericytomas in the Central Nervous System : A Multicenter Study of Korean Cases with Validation of the Usage of STAT6 Immunohistochemistry for Diagnosis of Disease. / Choi, Junjeong; Park, Sung Hye; Khang, Shin Kwang; Suh, Yeon Lim; Kim, Sang Pyo; Lee, Youn Soo; Kwon, Hyun Seok; Kang, Seok Gu; Kim, Se Hoon.

In: Annals of Surgical Oncology, Vol. 23, 01.12.2016, p. 954-961.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Hemangiopericytomas in the Central Nervous System

T2 - A Multicenter Study of Korean Cases with Validation of the Usage of STAT6 Immunohistochemistry for Diagnosis of Disease

AU - Choi, Junjeong

AU - Park, Sung Hye

AU - Khang, Shin Kwang

AU - Suh, Yeon Lim

AU - Kim, Sang Pyo

AU - Lee, Youn Soo

AU - Kwon, Hyun Seok

AU - Kang, Seok Gu

AU - Kim, Se Hoon

PY - 2016/12/1

Y1 - 2016/12/1

N2 - Background: Hemangiopericytoma (HPC) in the central nervous system (CNS) is a rare disease with distinctive biological/clinical characteristics compared with meningioma. Methods: Cases of HPCs of the CNS were collected, and clinicopathological records were retrospectively reviewed and analyzed. Immunohistochemistry (IHC) for proliferative markers (Ki-67, PHH3) and STAT6 were performed. Results: A total of 140 cases were collected, with mean follow-up of 77 months (median 58.8 months; range 0.53–540.5 months). 1-, 5-, 10-, and 20-year survival rates were 99.1, 94.0, 74.4, and 61.9 %, respectively. Thirty-nine patients (27.9 %) had recurrent disease. Mean and median times to recurrence were 62.9 and 47.3 months with 1-, 5-, 10-, and 20-year recurrence-free survival rates of 98.3, 78.3, 50.1, and 11.0 %, respectively. Thirteen patients (9.3 %) developed extracranial metastases. No adjuvant radiation therapy, higher histologic grades, failure of gross-total resection, and cases with gamma-knife surgery (GKS) were factors associated with shorter disease-free survival (log-rank test, p = 0.02, 0.00, 0.02, 0.00), among which high histologic grade and cases with GKS were significant in multivariable analysis. Strong nuclear STAT6 expression was noted in HPCs in 62 cases of HPCs (60/62, 96.8 %), whereas diffuse weak positivity was demonstrated in all meningioma cases. Conclusions: The survival rate in patients with HPC of the CNS is comparable to that of previously reported series. Recurrence remains a critical clinical issue of the disease. Identification of NAB2-STAT6 fusion transcript with surrogate IHC marker is a valuable diagnostic tool in the differential diagnosis of the disease.

AB - Background: Hemangiopericytoma (HPC) in the central nervous system (CNS) is a rare disease with distinctive biological/clinical characteristics compared with meningioma. Methods: Cases of HPCs of the CNS were collected, and clinicopathological records were retrospectively reviewed and analyzed. Immunohistochemistry (IHC) for proliferative markers (Ki-67, PHH3) and STAT6 were performed. Results: A total of 140 cases were collected, with mean follow-up of 77 months (median 58.8 months; range 0.53–540.5 months). 1-, 5-, 10-, and 20-year survival rates were 99.1, 94.0, 74.4, and 61.9 %, respectively. Thirty-nine patients (27.9 %) had recurrent disease. Mean and median times to recurrence were 62.9 and 47.3 months with 1-, 5-, 10-, and 20-year recurrence-free survival rates of 98.3, 78.3, 50.1, and 11.0 %, respectively. Thirteen patients (9.3 %) developed extracranial metastases. No adjuvant radiation therapy, higher histologic grades, failure of gross-total resection, and cases with gamma-knife surgery (GKS) were factors associated with shorter disease-free survival (log-rank test, p = 0.02, 0.00, 0.02, 0.00), among which high histologic grade and cases with GKS were significant in multivariable analysis. Strong nuclear STAT6 expression was noted in HPCs in 62 cases of HPCs (60/62, 96.8 %), whereas diffuse weak positivity was demonstrated in all meningioma cases. Conclusions: The survival rate in patients with HPC of the CNS is comparable to that of previously reported series. Recurrence remains a critical clinical issue of the disease. Identification of NAB2-STAT6 fusion transcript with surrogate IHC marker is a valuable diagnostic tool in the differential diagnosis of the disease.

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