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Vulvar Smooth Muscle Tumor of Uncertain Malignant Potential

MedGen UID:
1793821
•Concept ID:
C5557380
•
Neoplastic Process
Synonym: Vulvar Smooth Muscle Neoplasm of Uncertain Malignant Potential

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVVulvar Smooth Muscle Tumor of Uncertain Malignant Potential

Professional guidelines

PubMed

Criteria for risk stratification of vulvar and vaginal smooth muscle tumors: a follow-up study with application to leiomyoma variants, smooth muscle tumors of uncertain malignant potential, and leiomyosarcomas.
Swanson AA, Howitt BE, Schoolmeester JK
Hum Pathol 2020 Sep;103:83-94. Epub 2020 Jul 17 doi: 10.1016/j.humpath.2020.06.008. PMID: 32687944
Criteria for Risk Stratification of Vulvar and Vaginal Smooth Muscle Tumors: An Evaluation of 71 Cases Comparing Proposed Classification Systems.
Sayeed S, Xing D, Jenkins SM, Weisman PS, Buehler D, Warmke L, Uram-Tuculescu C, Bakkum-Gamez JN, Howitt BE, Cortese C, Park KJ, Schoolmeester JK
Am J Surg Pathol 2018 Jan;42(1):84-94. doi: 10.1097/PAS.0000000000000920. PMID: 28786880Free PMC Article
Leiomyoma cutis: a focused review on presentation, management, and association with malignancy.
Malik K, Patel P, Chen J, Khachemoune A
Am J Clin Dermatol 2015 Feb;16(1):35-46. doi: 10.1007/s40257-015-0112-1. PMID: 25605645

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
Update on Mesenchymal Lesions of the Lower Female Genital Tract.
Croce S, Perret R, Le Loarer F
Surg Pathol Clin 2022 Jun;15(2):341-367. Epub 2022 May 20 doi: 10.1016/j.path.2022.02.009. PMID: 35715165
Vulvar carcinosarcoma secondary to radiotherapy: a case report and review of the literature.
Fiset PO, Wou K, Arseneau J, Gilbert L
J Low Genit Tract Dis 2014 Jul;18(3):e74-9. doi: 10.1097/LGT.0b013e3182a82115. PMID: 24556613
Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities.
Nielsen GP, Young RH
Int J Gynecol Pathol 2001 Apr;20(2):105-27. doi: 10.1097/00004347-200104000-00002. PMID: 11293156
Smooth-muscle tumors of the vulva. A clinicopathological study of 25 cases and review of the literature.
Nielsen GP, Rosenberg AE, Koerner FC, Young RH, Scully RE
Am J Surg Pathol 1996 Jul;20(7):779-93. doi: 10.1097/00000478-199607000-00001. PMID: 8669526

Diagnosis

Surgical excision of giant vulvar angiofibroma: A case report and a review of literature.
Altal OF, Rawashdeh S, Al Sharie S, Al Zu'bi YO, Al Sharie AH, Daoud MN, Alkhawaldeh KM
Medicine (Baltimore) 2022 Sep 9;101(36):e30125. doi: 10.1097/MD.0000000000030125. PMID: 36086676Free PMC Article
Update on Mesenchymal Lesions of the Lower Female Genital Tract.
Croce S, Perret R, Le Loarer F
Surg Pathol Clin 2022 Jun;15(2):341-367. Epub 2022 May 20 doi: 10.1016/j.path.2022.02.009. PMID: 35715165
Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy.
Libertini M, Hallin M, Thway K, Noujaim J, Benson C, van der Graaf W, Jones RL
Int J Surg Pathol 2021 Feb;29(1):4-20. Epub 2020 Sep 10 doi: 10.1177/1066896920958120. PMID: 32909482
Smooth Muscle Tumors of the Female Genital Tract.
Devereaux KA, Schoolmeester JK
Surg Pathol Clin 2019 Jun;12(2):397-455. doi: 10.1016/j.path.2019.02.004. PMID: 31097110
Review of the pathophysiology, diagnosis, and therapy of vulvar leiomyoma, a rare gynecological tumor.
Sun C, Zou J, Wang Q, Wang Q, Han L, Batchu N, Ulain Q, Du J, Lv S, Song Q, Li Q
J Int Med Res 2018 Feb;46(2):663-674. Epub 2017 Sep 6 doi: 10.1177/0300060517721796. PMID: 28875758Free PMC Article

Therapy

Vulvar carcinosarcoma secondary to radiotherapy: a case report and review of the literature.
Fiset PO, Wou K, Arseneau J, Gilbert L
J Low Genit Tract Dis 2014 Jul;18(3):e74-9. doi: 10.1097/LGT.0b013e3182a82115. PMID: 24556613
Extragastrointestinal stromal tumors presenting as vulvovaginal/rectovaginal septal masses: a diagnostic pitfall.
Lam MM, Corless CL, Goldblum JR, Heinrich MC, Downs-Kelly E, Rubin BP
Int J Gynecol Pathol 2006 Jul;25(3):288-92. doi: 10.1097/01.pgp.0000215291.22867.18. PMID: 16810068
Clear cell tumors of the female genital tract.
Matias-Guiu X, Lerma E, Prat J
Semin Diagn Pathol 1997 Nov;14(4):233-9. PMID: 9383823

Prognosis

Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy.
Libertini M, Hallin M, Thway K, Noujaim J, Benson C, van der Graaf W, Jones RL
Int J Surg Pathol 2021 Feb;29(1):4-20. Epub 2020 Sep 10 doi: 10.1177/1066896920958120. PMID: 32909482
Smooth Muscle Tumors of the Female Genital Tract.
Devereaux KA, Schoolmeester JK
Surg Pathol Clin 2019 Jun;12(2):397-455. doi: 10.1016/j.path.2019.02.004. PMID: 31097110
Primary myoepithelial carcinoma of the vulva and review of the literature.
Miyata M, Hasegawa K, Ishikawa K, Kato R, Udagawa Y, Kuroda M
J Obstet Gynaecol Res 2011 Jun;37(6):617-22. Epub 2011 Feb 23 doi: 10.1111/j.1447-0756.2010.01392.x. PMID: 21349127
Mesenchymal tumors and tumor-like lesions of the female genital tract: a selective review with emphasis on recently described entities.
Nielsen GP, Young RH
Int J Gynecol Pathol 2001 Apr;20(2):105-27. doi: 10.1097/00004347-200104000-00002. PMID: 11293156
Smooth-muscle tumors of the vulva. A clinicopathological study of 25 cases and review of the literature.
Nielsen GP, Rosenberg AE, Koerner FC, Young RH, Scully RE
Am J Surg Pathol 1996 Jul;20(7):779-93. doi: 10.1097/00000478-199607000-00001. PMID: 8669526

Clinical prediction guides

Gynecological Sarcomas: Molecular Characteristics, Behavior, and Histology-Driven Therapy.
Libertini M, Hallin M, Thway K, Noujaim J, Benson C, van der Graaf W, Jones RL
Int J Surg Pathol 2021 Feb;29(1):4-20. Epub 2020 Sep 10 doi: 10.1177/1066896920958120. PMID: 32909482
HMGA2 Is a Useful Marker of Vulvovaginal Aggressive Angiomyxoma But May Be Positive in Other Mesenchymal Lesions at This Site.
Harkness R, McCluggage WG
Int J Gynecol Pathol 2021 Mar 1;40(2):185-189. doi: 10.1097/PGP.0000000000000689. PMID: 32897956
Alveolar Soft Part Sarcoma of the Female Genital Tract: A Morphologic, Immunohistochemical, and Molecular Cytogenetic Study of 10 Cases With Emphasis on its Distinction From Morphologic Mimics.
Schoolmeester JK, Carlson J, Keeney GL, Fritchie KJ, Oliva E, Young RH, Nucci MR
Am J Surg Pathol 2017 May;41(5):622-632. doi: 10.1097/PAS.0000000000000796. PMID: 28009610
Multifocal vulvar smooth muscle tumor with an unusual intravascular growth pattern and multiple local recurrences in a 10-year-old child: a diagnostic dilemma.
Harrington A, Bell E, Suchi M, Behmaram B, Uyar D
Pediatr Dev Pathol 2011 May-Jun;14(3):235-9. Epub 2010 Nov 19 doi: 10.2350/09-10-0730-CR.1. PMID: 21090988
Giant superficial angiomyxoma of the vulva: a case report and review of the literature.
Kim HS, Kim GY, Lim SJ, Ki KD, Kim HC
J Cutan Pathol 2010 Jun;37(6):672-7. Epub 2009 Jul 13 doi: 10.1111/j.1600-0560.2009.01333.x. PMID: 19615026

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