{"paper_id":"1511017b-4897-499e-8f86-f9b22469d06a","body_text":"Abstract\nWe present a case of clear cell carcinoma arising in endometriosis of the rectum that provides insights to the biological behavior of this extremely rare tumor. The patient presented with a large rectal mass, and the biopsy was suggestive of clear cell carcinoma of Müllerian origin based on the morphology and immunoprofile. The patient underwent a proctosigmoidectomy and hysterectomy with bilateral salpingo-oophorectomy with clear cell carcinoma involving the colon. Five pericolonic lymph nodes were positive for carcinoma. Endometriosis was identified in the colonic wall adjacent to the carcinoma. No tumor was identified in the endometrium, fallopian tubes, or ovaries. ARID1A mutations were identified by next-generation sequencing. The patient underwent adjuvant chemotherapy and is disease-free 4 years after the diagnosis. To our knowledge, this is the first case of clear cell carcinoma arising in colon endometriosis with pericolonic lymph node metastasis.\nData availability\nNot applicable to this article.\nChange history\n10 December 2024\nA Correction to this paper has been published: https://doi.org/10.1007/s00428-024-03990-7\nReferences\nHeaps JM, Nieberg RK, Berek JS (1990) Malignant neoplasms arising in endometriosis. Obstet Gynecol 75(6):1023–1028\nSlavin RE, Krum R, Van Dinh T (2000) Endometriosis-associated intestinal tumors: a clinical and pathological study of 6 cases with a review of the literature. Hum Pathol 31(4):456–463. https://doi.org/10.1053/hp.2000.6712\nHitti I, Glasberg S, Lubicz S (1990) Clear cell carcinoma arising from extraovarian endometriosis: report of three cases and review of literature. Gynecol Oncol 39:314–320\nMcCluggage WG (2001) Clear cell adenocarcinoma of the colon arising in endometriosis: a rare variant of primary colonic adenocarcinoma. 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Am J Surg Pathol 35(6):816–826. https://doi.org/10.1097/PAS.0b013e318216c112\nWiegand KC, Shah SP, Al-Agha OM et al (2010) ARID1A mutations in endometriosis-associated ovarian carcinomas. N Engl J Med 363(16):1532–1543. https://doi.org/10.1056/NEJMoa1008433\nCancer Genome Atlas Network (2012) Comprehensive molecular characterization of human colon and rectal cancer. Nature 487(7407):330–7. https://doi.org/10.1038/nature11252\nNational Comprehensive Cancer Network. Ovarian cancer/fallopian tube cancer/primary peritoneal cancer. (version 1.2024). https://www.nccn.org/professionals/physician_gls/pdf/ovarian.pdf. Accessed 24 Mar 2024\nNational Comprehensive Cancer Network. Rectal cancer. (version 1.2024). https://www.nccn.org/professionals/physician_gls/pdf/rectal.pdf. Accessed 24 Mar 2024\nAuthor information\nAuthors and Affiliations\nContributions\nDr. Riley Lochner participated in the manuscript writing and review. Dr. John Nakayama was involved in the review and revision of the manuscript. Dr. Emily Steinhagen was involved in the review and revision of the manuscript. Dr. Christina Bagby was involved in slide review, the review and revision of the manuscript. Dr. Min Cui was responsible for conceptual design, slide review, the review and revision of the manuscript.\nCorresponding author\nEthics declarations\nEthics approval\nThis study was approved by the authors’ institutional review board.\nConflict of interest\nThe authors declare no competing interests.\nAdditional information\nPublisher's Note\nSpringer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.\nThe original version of this article was revised: This article was originally published with an omitted abstract and keywords as a result of a system error.\nSupplementary information\nBelow is the link to the electronic supplementary material.\nRights and permissions\nAbout this article\nCite this article\nLochner, R., Nakayama, J., Steinhagen, E. et al. Müllerian clear cell carcinoma arising from rectal endometriosis with pericolonic lymph node metastasis. Virchows Arch 486, 1073–1077 (2025). https://doi.org/10.1007/s00428-024-03864-y\nReceived:\nRevised:\nAccepted:\nPublished:\nVersion of record:\nIssue date:\nDOI: https://doi.org/10.1007/s00428-024-03864-y","source_license":"CC0","license_restricted":false}