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Uterine Corpus Spindle Smooth Muscle Tumor of Uncertain Malignant Potential

MedGen UID:
1792894
•Concept ID:
C5556453
•
Neoplastic Process
Synonym: Uterine Corpus Spindle STUMP

Definition

A smooth muscle tumor of uncertain malignant potential that arises from the uterine corpus and is composed of neoplastic spindle cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUterine Corpus Spindle Smooth Muscle Tumor of Uncertain Malignant Potential

Professional guidelines

PubMed

TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis.
Schoolmeester JK, Dao LN, Sukov WR, Wang L, Park KJ, Murali R, Hameed MR, Soslow RA
Am J Surg Pathol 2015 Mar;39(3):394-404. doi: 10.1097/PAS.0000000000000349. PMID: 25517951Free PMC Article

Recent clinical studies

Etiology

ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms.
Bowman CJ, Medeiros F, Fadare O, Sangoi AR, Horvai AE, Devine WP, McCluggage WG, Rabban JT
Int J Gynecol Pathol 2023 Jan 1;42(1):1-10. Epub 2022 Feb 18 doi: 10.1097/PGP.0000000000000858. PMID: 35180768
Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases.
Fadare O
Adv Anat Pathol 2008 Mar;15(2):63-75. doi: 10.1097/PAP.0b013e31816613b0. PMID: 18418088

Diagnosis

Pan-TRK immunohistochemistry in gynaecological mesenchymal tumours: diagnostic implications and pitfalls.
Moura MS, Costa J, Velasco V, Kommoss F, Oliva E, Le Loarer F, McCluggage WG, Razack R, Treilleux I, Mills A, Longacre T, Devouassoux-Shisheboran M, Hostein I, Azmani R, Blanchard L, Hartog C, Soubeyran I, Khalifa E, Croce S
Histopathology 2024 Feb;84(3):451-462. Epub 2023 Nov 21 doi: 10.1111/his.15082. PMID: 37988282
ALK Immunoexpression is Specific for Inflammatory Myofibroblastic Tumor Among Vulvovaginal Mesenchymal Neoplasms.
Bowman CJ, Medeiros F, Fadare O, Sangoi AR, Horvai AE, Devine WP, McCluggage WG, Rabban JT
Int J Gynecol Pathol 2023 Jan 1;42(1):1-10. Epub 2022 Feb 18 doi: 10.1097/PGP.0000000000000858. PMID: 35180768
NTRK-Fusion Sarcoma of the Uterine Cervix: Report of 2 Cases With Comparative Clinicopathologic Features.
Nilforoushan N, Wethington SL, Nonogaki H, Gross J, Vang R, Xing D
Int J Gynecol Pathol 2022 Nov 1;41(6):642-648. Epub 2021 Oct 29 doi: 10.1097/PGP.0000000000000834. PMID: 34723848Free PMC Article
Spindle Cell Sarcoma of the Uterine Corpus With Adipose Metaplasia: Expanding the Morphologic Spectrum of Neoplasms With MEIS1-NCOA2 Gene Fusion.
Kommoss FKF, Kölsche C, Mentzel T, Schmidt D, von Deimling A, McCluggage WG, Kommoss F
Int J Gynecol Pathol 2022 Jul 1;41(4):417-422. Epub 2021 Jun 23 doi: 10.1097/PGP.0000000000000803. PMID: 34166279
Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article

Therapy

Large cell neuroendocrine carcinoma with sarcomatous changes of the endometrium: a case report with immunohistochemical studies and molecular genetic study of KIT and PDGFRA.
Terada T
Pathol Res Pract 2010 Jun 15;206(6):420-5. Epub 2010 Feb 26 doi: 10.1016/j.prp.2009.12.008. PMID: 20189318

Prognosis

Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis.
Schoolmeester JK, Dao LN, Sukov WR, Wang L, Park KJ, Murali R, Hameed MR, Soslow RA
Am J Surg Pathol 2015 Mar;39(3):394-404. doi: 10.1097/PAS.0000000000000349. PMID: 25517951Free PMC Article
Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases.
Fadare O
Adv Anat Pathol 2008 Mar;15(2):63-75. doi: 10.1097/PAP.0b013e31816613b0. PMID: 18418088
Endometrioid carcinomas of the uterine corpus with sex cord-like formations, hyalinization, and other unusual morphologic features: a report of 31 cases of a neoplasm that may be confused with carcinosarcoma and other uterine neoplasms.
Murray SK, Clement PB, Young RH
Am J Surg Pathol 2005 Feb;29(2):157-66. doi: 10.1097/01.pas.0000149704.89463.05. PMID: 15644772

Clinical prediction guides

Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors.
Bennett JA, Braga AC, Pinto A, Van de Vijver K, Cornejo K, Pesci A, Zhang L, Morales-Oyarvide V, Kiyokawa T, Zannoni GF, Carlson J, Slavik T, Tornos C, Antonescu CR, Oliva E
Am J Surg Pathol 2018 Oct;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119. PMID: 30001237Free PMC Article
TFE3 translocation-associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis.
Schoolmeester JK, Dao LN, Sukov WR, Wang L, Park KJ, Murali R, Hameed MR, Soslow RA
Am J Surg Pathol 2015 Mar;39(3):394-404. doi: 10.1097/PAS.0000000000000349. PMID: 25517951Free PMC Article
Extrauterine endometrial stromal sarcoma with JAZF1/JJAZ1 fusion confirmed by RT-PCR and interphase FISH presenting as an inguinal tumor.
Sato K, Ueda Y, Sugaya J, Ozaki M, Hisaoka M, Katsuda S
Virchows Arch 2007 Mar;450(3):349-53. Epub 2007 Jan 18 doi: 10.1007/s00428-006-0345-8. PMID: 17235569
Inflammatory myofibroblastic tumor of the uterus with prominent myxoid change.
Shintaku M, Fukushima A
Pathol Int 2006 Oct;56(10):625-8. doi: 10.1111/j.1440-1827.2006.02018.x. PMID: 16984620

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