Primary leiomyosarcomas of the gastrointestinal tract in the post-gastrointestinal stromal tumor era

Gitika Aggarwal, Suash Sharma, Mei Zheng, Michelle D. Reid, John H Crosby, Sherman M. Chamberlain, Asha Nayak, Jeffrey R. Lee

Research output: Contribution to journalReview article

28 Citations (Scopus)

Abstract

Most mesenchymal neoplasms of the gastrointestinal tract are currently classified as gastrointestinal stromal tumors (GIST). Gastrointestinal stromal tumors are diagnosed by immunopositivity for CD117, CD34, and DOG1.1, with or without molecular analyses. According to the World Health Organization classification, the diagnosis of primary leiomyosarcomas of the gastrointestinal tract is so rare that there are no significant data on demographic, clinical, or gross features of this tumor. A comprehensive literature search was performed to identify gastrointestinal leiomyosarcomas. Searches were limited to the past 12 years because definitive tools to differentiate leiomyosarcomas from GIST were introduced in the late 1990s. Cases were included only if convincing data were presented. Six cases of esophageal leiomyosarcoma and 5 cases of gastric leiomyosarcoma were confirmed. Furthermore, 26 cases of leiomyosarcoma of the small bowel, 11 cases of the colon, and 8 cases arising in the rectum were identified. Finally, 28 cases of infantile and adolescent leiomyosarcoma were reviewed. Although survival analysis is precluded by small case numbers and limited survival data availability, the trend identifies that increased size and mitotic activity portends to a worse prognosis in small bowel leiomyosarcomas. Colonic leiomyosarcomas appear to be aggressive tumors, regardless of tumor size and mitotic activity. Rectal leiomyosarcomas present as smaller tumors with favorable prognosis. Leiomyosarcomas in post-GIST era are rare tumors of the gastrointestinal tract with distinctive clinicopathologic characteristics. Owing to different treatment options, it is necessary to accurately differentiate these from GIST, using a combination of histologic appearance, presence of smooth muscle antigens, and absence of specific GIST immunomarkers.

Original languageEnglish (US)
Pages (from-to)532-540
Number of pages9
JournalAnnals of Diagnostic Pathology
Volume16
Issue number6
DOIs
StatePublished - Dec 1 2012

Fingerprint

Gastrointestinal Stromal Tumors
Leiomyosarcoma
Gastrointestinal Tract
Neoplasms
Survival Analysis
Rectum
Smooth Muscle
Stomach
Colon
Demography

Keywords

  • DOG1.1
  • Gastrointestinal stromal tumor
  • KIT
  • Leiomyosarcoma
  • PDGFRA

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Primary leiomyosarcomas of the gastrointestinal tract in the post-gastrointestinal stromal tumor era. / Aggarwal, Gitika; Sharma, Suash; Zheng, Mei; Reid, Michelle D.; Crosby, John H; Chamberlain, Sherman M.; Nayak, Asha; Lee, Jeffrey R.

In: Annals of Diagnostic Pathology, Vol. 16, No. 6, 01.12.2012, p. 532-540.

Research output: Contribution to journalReview article

Aggarwal, Gitika ; Sharma, Suash ; Zheng, Mei ; Reid, Michelle D. ; Crosby, John H ; Chamberlain, Sherman M. ; Nayak, Asha ; Lee, Jeffrey R. / Primary leiomyosarcomas of the gastrointestinal tract in the post-gastrointestinal stromal tumor era. In: Annals of Diagnostic Pathology. 2012 ; Vol. 16, No. 6. pp. 532-540.
@article{18034834125a4d42a7751e8e8bb26d2b,
title = "Primary leiomyosarcomas of the gastrointestinal tract in the post-gastrointestinal stromal tumor era",
abstract = "Most mesenchymal neoplasms of the gastrointestinal tract are currently classified as gastrointestinal stromal tumors (GIST). Gastrointestinal stromal tumors are diagnosed by immunopositivity for CD117, CD34, and DOG1.1, with or without molecular analyses. According to the World Health Organization classification, the diagnosis of primary leiomyosarcomas of the gastrointestinal tract is so rare that there are no significant data on demographic, clinical, or gross features of this tumor. A comprehensive literature search was performed to identify gastrointestinal leiomyosarcomas. Searches were limited to the past 12 years because definitive tools to differentiate leiomyosarcomas from GIST were introduced in the late 1990s. Cases were included only if convincing data were presented. Six cases of esophageal leiomyosarcoma and 5 cases of gastric leiomyosarcoma were confirmed. Furthermore, 26 cases of leiomyosarcoma of the small bowel, 11 cases of the colon, and 8 cases arising in the rectum were identified. Finally, 28 cases of infantile and adolescent leiomyosarcoma were reviewed. Although survival analysis is precluded by small case numbers and limited survival data availability, the trend identifies that increased size and mitotic activity portends to a worse prognosis in small bowel leiomyosarcomas. Colonic leiomyosarcomas appear to be aggressive tumors, regardless of tumor size and mitotic activity. Rectal leiomyosarcomas present as smaller tumors with favorable prognosis. Leiomyosarcomas in post-GIST era are rare tumors of the gastrointestinal tract with distinctive clinicopathologic characteristics. Owing to different treatment options, it is necessary to accurately differentiate these from GIST, using a combination of histologic appearance, presence of smooth muscle antigens, and absence of specific GIST immunomarkers.",
keywords = "DOG1.1, Gastrointestinal stromal tumor, KIT, Leiomyosarcoma, PDGFRA",
author = "Gitika Aggarwal and Suash Sharma and Mei Zheng and Reid, {Michelle D.} and Crosby, {John H} and Chamberlain, {Sherman M.} and Asha Nayak and Lee, {Jeffrey R.}",
year = "2012",
month = "12",
day = "1",
doi = "10.1016/j.anndiagpath.2012.07.005",
language = "English (US)",
volume = "16",
pages = "532--540",
journal = "Annals of Diagnostic Pathology",
issn = "1092-9134",
publisher = "W.B. Saunders Ltd",
number = "6",

}

TY - JOUR

T1 - Primary leiomyosarcomas of the gastrointestinal tract in the post-gastrointestinal stromal tumor era

AU - Aggarwal, Gitika

AU - Sharma, Suash

AU - Zheng, Mei

AU - Reid, Michelle D.

AU - Crosby, John H

AU - Chamberlain, Sherman M.

AU - Nayak, Asha

AU - Lee, Jeffrey R.

PY - 2012/12/1

Y1 - 2012/12/1

N2 - Most mesenchymal neoplasms of the gastrointestinal tract are currently classified as gastrointestinal stromal tumors (GIST). Gastrointestinal stromal tumors are diagnosed by immunopositivity for CD117, CD34, and DOG1.1, with or without molecular analyses. According to the World Health Organization classification, the diagnosis of primary leiomyosarcomas of the gastrointestinal tract is so rare that there are no significant data on demographic, clinical, or gross features of this tumor. A comprehensive literature search was performed to identify gastrointestinal leiomyosarcomas. Searches were limited to the past 12 years because definitive tools to differentiate leiomyosarcomas from GIST were introduced in the late 1990s. Cases were included only if convincing data were presented. Six cases of esophageal leiomyosarcoma and 5 cases of gastric leiomyosarcoma were confirmed. Furthermore, 26 cases of leiomyosarcoma of the small bowel, 11 cases of the colon, and 8 cases arising in the rectum were identified. Finally, 28 cases of infantile and adolescent leiomyosarcoma were reviewed. Although survival analysis is precluded by small case numbers and limited survival data availability, the trend identifies that increased size and mitotic activity portends to a worse prognosis in small bowel leiomyosarcomas. Colonic leiomyosarcomas appear to be aggressive tumors, regardless of tumor size and mitotic activity. Rectal leiomyosarcomas present as smaller tumors with favorable prognosis. Leiomyosarcomas in post-GIST era are rare tumors of the gastrointestinal tract with distinctive clinicopathologic characteristics. Owing to different treatment options, it is necessary to accurately differentiate these from GIST, using a combination of histologic appearance, presence of smooth muscle antigens, and absence of specific GIST immunomarkers.

AB - Most mesenchymal neoplasms of the gastrointestinal tract are currently classified as gastrointestinal stromal tumors (GIST). Gastrointestinal stromal tumors are diagnosed by immunopositivity for CD117, CD34, and DOG1.1, with or without molecular analyses. According to the World Health Organization classification, the diagnosis of primary leiomyosarcomas of the gastrointestinal tract is so rare that there are no significant data on demographic, clinical, or gross features of this tumor. A comprehensive literature search was performed to identify gastrointestinal leiomyosarcomas. Searches were limited to the past 12 years because definitive tools to differentiate leiomyosarcomas from GIST were introduced in the late 1990s. Cases were included only if convincing data were presented. Six cases of esophageal leiomyosarcoma and 5 cases of gastric leiomyosarcoma were confirmed. Furthermore, 26 cases of leiomyosarcoma of the small bowel, 11 cases of the colon, and 8 cases arising in the rectum were identified. Finally, 28 cases of infantile and adolescent leiomyosarcoma were reviewed. Although survival analysis is precluded by small case numbers and limited survival data availability, the trend identifies that increased size and mitotic activity portends to a worse prognosis in small bowel leiomyosarcomas. Colonic leiomyosarcomas appear to be aggressive tumors, regardless of tumor size and mitotic activity. Rectal leiomyosarcomas present as smaller tumors with favorable prognosis. Leiomyosarcomas in post-GIST era are rare tumors of the gastrointestinal tract with distinctive clinicopathologic characteristics. Owing to different treatment options, it is necessary to accurately differentiate these from GIST, using a combination of histologic appearance, presence of smooth muscle antigens, and absence of specific GIST immunomarkers.

KW - DOG1.1

KW - Gastrointestinal stromal tumor

KW - KIT

KW - Leiomyosarcoma

KW - PDGFRA

UR - http://www.scopus.com/inward/record.url?scp=84868544506&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84868544506&partnerID=8YFLogxK

U2 - 10.1016/j.anndiagpath.2012.07.005

DO - 10.1016/j.anndiagpath.2012.07.005

M3 - Review article

VL - 16

SP - 532

EP - 540

JO - Annals of Diagnostic Pathology

JF - Annals of Diagnostic Pathology

SN - 1092-9134

IS - 6

ER -