Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases

被引：35

作者：

Al-Ibraheemi, Alyaa ^{[1
]}

Broehm, Cory ^{[2
]}

Tanas, Munir R. ^{[3
]}

Horvai, Andrew E. ^{[4
]}

Rubin, Brian P. ^{[5
]}

Cheah, Alison L. ^{[6
]}

Thway, Khin ^{[7
]}

Fisher, Cyril ^{[7
]}

Bahrami, Armita ^{[8
]}

Folpe, Andrew L. ^{[2
]}

Fritchie, Karen J. ^{[2
]}

机构：

[1] Boston Childrens Hosp, Boston, MA USA

[2] Mayo Clin, Rochester, MN USA

[3] Univ Iowa, Iowa City, IA USA

[4] Univ Calif San Francisco, San Francisco, CA 94143 USA

[5] Cleveland Clin, Robert J Tomsich Pathol & Lab Med Inst, Cleveland, OH 44106 USA

[6] Douglass Hanly Moir Pathol, Sydney, NSW, Australia

[7] Royal Marsden Hosp, Sarcoma Unit, London, England

[8] St Jude Childrens Res Hosp, 332 N Lauderdale St, Memphis, TN 38105 USA

来源：

INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY | 2019年 / 27卷 / 03期

关键词：

DSRCT; sarcoma; EWSR1-WT1; desmoplastic small round cell tumor; EWS-WT1 GENE FUSION; TRANSLOCATION; EXPRESSION; DIVERSITY; EMPHASIS; SPECTRUM; FEATURES; SARCOMAS; 22)(P13; ADULTS;

D O I：

10.1177/1066896918817140

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Objectives. Desmoplastic small round cell tumor (DSRCT) is an aggressive round cell sarcoma that arises in the abdominal cavity/pelvis of young males. We sought to expand its clinicopathologic spectrum. Methods. Cases of DSRCT presenting in patients >30 years of age or tumors arising outside of the abdominal cavity/pelvis were retrieved. Results. Thirty-four cases were identified. Sixteen tumors arose at atypical sites (head/neck, intracranial, thigh, axilla/shoulder, inguinal/paratesticular, intraosseous, and uterine corpus). The remaining 18 patients were older than 30 years, and their tumors involved the abdomen or pelvis. The majority of cases showed areas with classic histology, while 6 cases exhibited solid growth and 5 showed macronodular architecture. Cytologic appearance included round cell, rhabdoid, epithelioid, and small cell. Conclusion. DSRCT may arise at nonabdominal locations in both pediatric and adult populations, as well as intra-abdominally in older adults, and these tumors exhibit high rates of metastasis and morbidity.

引用

页码：236 / 243

页数：8

共 32 条

[1] Primary desmoplastic small cell tumor of soft tissues and bone of the hand [J].