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Uterine Corpus Smooth Muscle Tumor of Uncertain Malignant Potential

MedGen UID:
311428
•Concept ID:
C1519864
•
Neoplastic Process
Synonyms: Borderline Uterine Corpus Smooth Muscle Neoplasm; Uterine Corpus Smooth Muscle Neoplasm of Uncertain Malignant Potential

Definition

A smooth muscle neoplasm that arises from the uterine corpus and cannot be reliably diagnosed as benign or malignant, because of the presence of ambiguous morphologic findings. [from NCI]

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
Patterns of bone morphogenetic protein-2 expression in smooth muscle tumors of the uterine corpus and other uterine tissues.
Fadare O, Renshaw IL, Liang SX
Appl Immunohistochem Mol Morphol 2011 Jul;19(4):352-9. doi: 10.1097/PAI.0b013e3182013441. PMID: 21285872
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
Prognostically favorable "mitotically active" smooth-muscle tumors of the uterus. A clinicopathologic study of ten cases.
Perrone T, Dehner LP
Am J Surg Pathol 1988 Jan;12(1):1-8. doi: 10.1097/00000478-198801000-00001. PMID: 3337336

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
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
Uterine adenomyoma: a clinicopathologic review of 26 cases and a review of the literature.
Tahlan A, Nanda A, Mohan H
Int J Gynecol Pathol 2006 Oct;25(4):361-5. doi: 10.1097/01.pgp.0000209570.08716.b3. PMID: 16990713

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
Unexpected borderline malignant and malignant smooth muscle cell tumors of the uterine corpus in women treated with LH-RH analogues.
Wauters CC, Ter Harmsel BW, Hermans MP, Smedts F
Eur J Obstet Gynecol Reprod Biol 1995 Oct;62(2):257-9. doi: 10.1016/0301-2115(95)02192-a. PMID: 8582508

Prognosis

An unusual coincidence of giant cervical leiomyoma and incidental ovarian granulosa cell tumor: A case report.
Kotowski K, Małyszczak P, Towarek M, Jagasyk A, Murawski M, Sozański R
Medicine (Baltimore) 2023 Jul 28;102(30):e34387. doi: 10.1097/MD.0000000000034387. PMID: 37505129Free PMC Article
ALK Is a Specific Diagnostic Marker for Inflammatory Myofibroblastic Tumor of the Uterus.
Mohammad N, Haimes JD, Mishkin S, Kudlow BA, Leong MY, Chew SH, Koay E, Whitehouse A, Cope N, Ali RH, Köbel M, Stewart CJR, McCluggage WG, Lee CH
Am J Surg Pathol 2018 Oct;42(10):1353-1359. doi: 10.1097/PAS.0000000000001120. PMID: 30015720
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
Primary osteosarcoma of the uterine corpus: case report and review of the literature.
Su M, Tokairin T, Nishikawa Y, Yoshioka T, Takahashi O, Watanabe H, Doi Y, Omori Y, Yoshioka T, Sageshima M, Tanaka T, Enomoto K
Pathol Int 2002 Feb;52(2):158-63. doi: 10.1046/j.1440-1827.2002.01325.x. PMID: 11940222

Clinical prediction guides

An unusual coincidence of giant cervical leiomyoma and incidental ovarian granulosa cell tumor: A case report.
Kotowski K, Małyszczak P, Towarek M, Jagasyk A, Murawski M, Sozański R
Medicine (Baltimore) 2023 Jul 28;102(30):e34387. doi: 10.1097/MD.0000000000034387. PMID: 37505129Free PMC Article
ALK Is a Specific Diagnostic Marker for Inflammatory Myofibroblastic Tumor of the Uterus.
Mohammad N, Haimes JD, Mishkin S, Kudlow BA, Leong MY, Chew SH, Koay E, Whitehouse A, Cope N, Ali RH, Köbel M, Stewart CJR, McCluggage WG, Lee CH
Am J Surg Pathol 2018 Oct;42(10):1353-1359. doi: 10.1097/PAS.0000000000001120. PMID: 30015720
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
Endometrial stromal sarcoma: clinicopathological and immunophenotype study of 18 cases.
Vera AA, Guadarrama MB
Ann Diagn Pathol 2011 Oct;15(5):312-7. Epub 2011 Jun 8 doi: 10.1016/j.anndiagpath.2011.01.008. PMID: 21652246
Uterine adenomyoma: a clinicopathologic review of 26 cases and a review of the literature.
Tahlan A, Nanda A, Mohan H
Int J Gynecol Pathol 2006 Oct;25(4):361-5. doi: 10.1097/01.pgp.0000209570.08716.b3. PMID: 16990713

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