Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review

Celia Green, Dominic V. Spagnolo, Peter D. Robbins, Soraya Fermoyle, Daniel D. Wong

Research output: Contribution to journalReview article

3 Citations (Scopus)

Abstract

Clear cell sarcoma is an uncommon sarcoma which rarely occurs as a primary tumour in the gastrointestinal tract (CCS-GIT). It shares common molecular genetic abnormalities with the more recently described entity, malignant gastrointestinal neuroectodermal tumour (GNET) but is distinguished by its morphological and immunohistochemical findings. The exact nosological relationship between these tumours continues to be debated. In this review, we present two cases of these rare neoplasms from our files and perform a statistical comparison of all published cases to determine if significant differences exist in their clinicopathological features and biological behaviour. Thirteen cases of CCS-GIT and 58 of GNET were included. CCS-GIT occurred more commonly in males (84.6% vs 46.6%, p = 0.01) and in an older age group (median 57 vs 33 years, p <0.01). There was no significant difference in their location in the gastrointestinal tract, median tumour size and proportion of cases with an EWSR1-ATF1 vs EWSR1-CREB1 fusion. Median survival for CCS-GIT was 13.5 months and for GNET, 9.5 months (p = 0.78). There was no significant difference in the Kaplan-Meier survival curves for either time to first metastasis (p = 0.88) or overall survival (p = 0.18), including after controlling for tumour size using regression models. Our analysis confirms that aside from morphological variations between these tumours, they also exhibit epidemiological and clinical differences. Despite the prevalent perception that GNET is associated with a more aggressive clinical course, our findings indicate that there is no significant difference in their biological behaviour, although both clearly share a bleak prognosis. Further experience is awaited to determine optimal treatment strategies and whether CCS-GIT and GNET would differ in their response to various therapies.

Original languageEnglish
Pages (from-to)490-498
Number of pages9
JournalPathology
Volume50
Issue number5
DOIs
Publication statusPublished - Aug 2018

Cite this

Green, Celia ; Spagnolo, Dominic V. ; Robbins, Peter D. ; Fermoyle, Soraya ; Wong, Daniel D. / Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour : distinct or related entities? A review. In: Pathology. 2018 ; Vol. 50, No. 5. pp. 490-498.
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title = "Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review",
abstract = "Clear cell sarcoma is an uncommon sarcoma which rarely occurs as a primary tumour in the gastrointestinal tract (CCS-GIT). It shares common molecular genetic abnormalities with the more recently described entity, malignant gastrointestinal neuroectodermal tumour (GNET) but is distinguished by its morphological and immunohistochemical findings. The exact nosological relationship between these tumours continues to be debated. In this review, we present two cases of these rare neoplasms from our files and perform a statistical comparison of all published cases to determine if significant differences exist in their clinicopathological features and biological behaviour. Thirteen cases of CCS-GIT and 58 of GNET were included. CCS-GIT occurred more commonly in males (84.6{\%} vs 46.6{\%}, p = 0.01) and in an older age group (median 57 vs 33 years, p <0.01). There was no significant difference in their location in the gastrointestinal tract, median tumour size and proportion of cases with an EWSR1-ATF1 vs EWSR1-CREB1 fusion. Median survival for CCS-GIT was 13.5 months and for GNET, 9.5 months (p = 0.78). There was no significant difference in the Kaplan-Meier survival curves for either time to first metastasis (p = 0.88) or overall survival (p = 0.18), including after controlling for tumour size using regression models. Our analysis confirms that aside from morphological variations between these tumours, they also exhibit epidemiological and clinical differences. Despite the prevalent perception that GNET is associated with a more aggressive clinical course, our findings indicate that there is no significant difference in their biological behaviour, although both clearly share a bleak prognosis. Further experience is awaited to determine optimal treatment strategies and whether CCS-GIT and GNET would differ in their response to various therapies.",
keywords = "Clear cell sarcoma, clear cell sarcoma of gastrointestinal tract, clear cell sarcoma-like tumour of gastrointestinal tract, gastrointestinal neuroectodermal tumour, EWSR1, ATF1, CREB1, SOFT PARTS, MOLECULAR ANALYSIS, MELANOMA, FUSION, DIAGNOSIS, VARIANT, APONEUROSES, ASSOCIATION, TRANSCRIPTS, EWSR1-CREB1",
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Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour : distinct or related entities? A review. / Green, Celia; Spagnolo, Dominic V.; Robbins, Peter D.; Fermoyle, Soraya; Wong, Daniel D.

In: Pathology, Vol. 50, No. 5, 08.2018, p. 490-498.

Research output: Contribution to journalReview article

TY - JOUR

T1 - Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour

T2 - distinct or related entities? A review

AU - Green, Celia

AU - Spagnolo, Dominic V.

AU - Robbins, Peter D.

AU - Fermoyle, Soraya

AU - Wong, Daniel D.

PY - 2018/8

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N2 - Clear cell sarcoma is an uncommon sarcoma which rarely occurs as a primary tumour in the gastrointestinal tract (CCS-GIT). It shares common molecular genetic abnormalities with the more recently described entity, malignant gastrointestinal neuroectodermal tumour (GNET) but is distinguished by its morphological and immunohistochemical findings. The exact nosological relationship between these tumours continues to be debated. In this review, we present two cases of these rare neoplasms from our files and perform a statistical comparison of all published cases to determine if significant differences exist in their clinicopathological features and biological behaviour. Thirteen cases of CCS-GIT and 58 of GNET were included. CCS-GIT occurred more commonly in males (84.6% vs 46.6%, p = 0.01) and in an older age group (median 57 vs 33 years, p <0.01). There was no significant difference in their location in the gastrointestinal tract, median tumour size and proportion of cases with an EWSR1-ATF1 vs EWSR1-CREB1 fusion. Median survival for CCS-GIT was 13.5 months and for GNET, 9.5 months (p = 0.78). There was no significant difference in the Kaplan-Meier survival curves for either time to first metastasis (p = 0.88) or overall survival (p = 0.18), including after controlling for tumour size using regression models. Our analysis confirms that aside from morphological variations between these tumours, they also exhibit epidemiological and clinical differences. Despite the prevalent perception that GNET is associated with a more aggressive clinical course, our findings indicate that there is no significant difference in their biological behaviour, although both clearly share a bleak prognosis. Further experience is awaited to determine optimal treatment strategies and whether CCS-GIT and GNET would differ in their response to various therapies.

AB - Clear cell sarcoma is an uncommon sarcoma which rarely occurs as a primary tumour in the gastrointestinal tract (CCS-GIT). It shares common molecular genetic abnormalities with the more recently described entity, malignant gastrointestinal neuroectodermal tumour (GNET) but is distinguished by its morphological and immunohistochemical findings. The exact nosological relationship between these tumours continues to be debated. In this review, we present two cases of these rare neoplasms from our files and perform a statistical comparison of all published cases to determine if significant differences exist in their clinicopathological features and biological behaviour. Thirteen cases of CCS-GIT and 58 of GNET were included. CCS-GIT occurred more commonly in males (84.6% vs 46.6%, p = 0.01) and in an older age group (median 57 vs 33 years, p <0.01). There was no significant difference in their location in the gastrointestinal tract, median tumour size and proportion of cases with an EWSR1-ATF1 vs EWSR1-CREB1 fusion. Median survival for CCS-GIT was 13.5 months and for GNET, 9.5 months (p = 0.78). There was no significant difference in the Kaplan-Meier survival curves for either time to first metastasis (p = 0.88) or overall survival (p = 0.18), including after controlling for tumour size using regression models. Our analysis confirms that aside from morphological variations between these tumours, they also exhibit epidemiological and clinical differences. Despite the prevalent perception that GNET is associated with a more aggressive clinical course, our findings indicate that there is no significant difference in their biological behaviour, although both clearly share a bleak prognosis. Further experience is awaited to determine optimal treatment strategies and whether CCS-GIT and GNET would differ in their response to various therapies.

KW - Clear cell sarcoma

KW - clear cell sarcoma of gastrointestinal tract

KW - clear cell sarcoma-like tumour of gastrointestinal tract

KW - gastrointestinal neuroectodermal tumour

KW - EWSR1

KW - ATF1

KW - CREB1

KW - SOFT PARTS

KW - MOLECULAR ANALYSIS

KW - MELANOMA

KW - FUSION

KW - DIAGNOSIS

KW - VARIANT

KW - APONEUROSES

KW - ASSOCIATION

KW - TRANSCRIPTS

KW - EWSR1-CREB1

U2 - 10.1016/j.pathol.2018.05.001

DO - 10.1016/j.pathol.2018.05.001

M3 - Review article

VL - 50

SP - 490

EP - 498

JO - Pathology

JF - Pathology

SN - 0031-3025

IS - 5

ER -