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Uterine Corpus Malignant PEComa

MedGen UID:
926306
Concept ID:
C4288774
Neoplastic Process
Synonyms: Malignant Uterine Corpus PEComa; Malignant Uterine Corpus Perivascular Epithelioid Cell Tumor; Uterine Corpus Malignant Perivascular Epithelioid Cell Tumor

Definition

A malignant neoplasm with perivascular epithelioid cell differentiation arising from the uterine corpus. The neoplasm is usually a large size, and charcaterized by the presence of marked nuclear atypia, pleomorphism, increased mitotic activity, necrosis, and infiltrative margins. The most common metastatic sites are lungs, lymph nodes, and bone. Patients present with a pelvic mass or abnormal bleeding. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUterine Corpus Malignant PEComa

Professional guidelines

PubMed

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

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
Acosta AM, Adley BP
Arch Pathol Lab Med 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS. PMID: 28234575
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Fadare O
Adv Anat Pathol 2008 Mar;15(2):63-75. doi: 10.1097/PAP.0b013e31816613b0. PMID: 18418088
Vang R, Kempson RL
Am J Surg Pathol 2002 Jan;26(1):1-13. doi: 10.1097/00000478-200201000-00001. PMID: 11756764

Diagnosis

Valencia-Guerrero A, Pinto A, Anderson WJ, Trevisan G, Nucci MR, Hirsch MS
Int J Gynecol Pathol 2020 Nov;39(6):529-536. doi: 10.1097/PGP.0000000000000653. PMID: 31851063
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
Conlon N, Soslow RA, Murali R
J Clin Pathol 2015 Jun;68(6):418-26. Epub 2015 Mar 6 doi: 10.1136/jclinpath-2015-202945. PMID: 25750268Free PMC Article
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Cil AP, Haberal A, Hucumenoglu S, Kovalak EE, Gunes M
Gynecol Oncol 2004 Aug;94(2):593-6. doi: 10.1016/j.ygyno.2004.05.015. PMID: 15297212

Prognosis

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
Acosta AM, Adley BP
Arch Pathol Lab Med 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS. PMID: 28234575
Conlon N, Soslow RA, Murali R
J Clin Pathol 2015 Jun;68(6):418-26. Epub 2015 Mar 6 doi: 10.1136/jclinpath-2015-202945. PMID: 25750268Free PMC Article
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739
Fadare O
Adv Anat Pathol 2008 Mar;15(2):63-75. doi: 10.1097/PAP.0b013e31816613b0. PMID: 18418088

Clinical prediction guides

Tang X, Feng M, Shen Y, Chen Q
Diagn Pathol 2023 Jan 16;18(1):7. doi: 10.1186/s13000-023-01290-3. PMID: 36647157Free PMC Article
Ando H, Ogawa M, Watanabe Y, Tsurunaga K, Nakamura C, Tamura H, Ikeda K, Takahashi R, Nakajima R, Yoshida S, Tsujie T, Doi R, Wakimoto A, Adachi S
Int J Gynecol Pathol 2020 May;39(3):227-232. doi: 10.1097/PGP.0000000000000589. PMID: 30789500
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
Acosta AM, Adley BP
Arch Pathol Lab Med 2017 Mar;141(3):463-469. doi: 10.5858/arpa.2016-0092-RS. PMID: 28234575
Fadare O
Adv Anat Pathol 2011 Jan;18(1):60-74. doi: 10.1097/PAP.0b013e3182026be7. PMID: 21169739

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