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Its mechanism remains poorly defined, with proposed origins from Müllerian metaplasia or malignant transformation of endometriosis. A 48-year-old woman presented with acute lower abdominal pain and imaging showed pelvic and pancreatic tail lesions. Surgery included hysterectomy, bilateral salpingo-oophorectomy, and distal pancreatectomy with splenectomy, confirming metastatic clear cell carcinoma by immunoprofiles and sequencing.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/primary-peritoneal-clear-cell-carcinoma-with-metastasis-mimicking-ovarian-carcinoma-a-case-report-and-literature-review/359152/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/primary-peritoneal-clear-cell-carcinoma-with-metastasis-mimicking-ovarian-carcinoma-a-case-report-and-literature-review/359152.png","ImageObject",300,407,{"name":92,"@type":93},"Mia  ","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-27","2026-09-23",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",11,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"Why is PPCCC difficult to diagnose clinically?","Question",{"text":113,"@type":114},"PPCCC closely resembles common gynecologic epithelial cancers both clinically and histologically, and its overlapping morphology can mislead diagnosis toward ovarian, endometrial, or renal tumors.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"What findings supported PPCCC with metastasis to the pancreas and spleen?",{"text":118,"@type":114},"The pelvic, pancreatic, and splenic lesions showed identical clear cell carcinoma features, with immunohistochemistry positive for PAX8, CK7, and HNF1β, loss of MSH2 and MSH6, and sequencing showing ARID1A loss and PIK3CA mutations; no primary ovarian, endometrial, or renal tumor was detected.",{"name":120,"@type":111,"acceptedAnswer":121},"What were the key clinical symptoms and initial imaging impressions?",{"text":122,"@type":114},"A 48-year-old woman presented with acute lower abdominal pain. CT suggested a pancreatic lesion suspicious for pancreatic adenocarcinoma or an islet cell tumor and also identified a large solid pelvic mass suspicious for malignant tumor or metastasis.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},359152,1790193067,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":34,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145},687207024478,"https://ap-avatar.wpscdn.com/davatar_a8503ba1806abce46bf441b54a3ca4cd","Kim etal. Journal of Ovarian Research (2026) 19:111  \n[https://doi.org/10.1186/s13048-026-02009-w](https://doi.org/10.1186/s13048-026-02009-w)  \nJournal of Ovarian Research  \nCASE REPORT Open Access  \nPrimary peritoneal clear cell carcinoma  with metastasis mimicking ovarian carcinoma: a case report and literature review  \nNah Ihm Kim 1,2, Ji Shin Lee3,4, Kyung Hwa Lee3,4 and Sung Sun Kim 1,2*  \nAbstract  \nBackground Primary peritoneal clear cell carcinoma (PPCCC) is an exceptionally rare malignancy that closely resembles gynecologic epithelial cancers clinically and histologically. Its pathogenesis is poorly understood, with possible origins from Müllerian metaplasia or malignant transformation of endometriosis.  \nCase presentation A 48-year-old woman with no history of endometriosis or hormone therapy presented with acute lower abdominal pain. Imaging revealed a pelvic mass and a lesion in the pancreatic tail. The patient underwent hysterectomy with bilateral salpingo-oophorectomy and distal pancreatectomy with splenectomy. Intraoperatively, the pelvic mass was located in the peritoneum, while the gynecologic organs appeared grossly normal. Histopathologic examination of the pelvic, pancreatic, and splenic lesions revealed identical features of clear cell carcinoma. Immunohistochemistry demonstrated positivity for PAX8, CK7, and HNF1β, with loss of MSH2 and MSH6 . Next-generation sequencing revealed ARID1A loss and somatic PIK3CA mutations. No primary ovarian, endometrial, or renal tumor was detected, supporting a diagnosis of primary peritoneal clear cell carcinoma with metastases to the pancreas and spleen.  \nConclusions This case highlights the diagnostic challenges of PPCCC and offers valuable insights into the clinical and pathological spectrum of this underrecognized malignancy.  \nKeywords Primary peritoneum carcinoma, Clear cell carcinoma, Endometriosis  \n*Correspondence:  \nSung Sun Kim  \n[succeedsoon@hanmail.net](succeedsoon@hanmail.net)  \n1Department of Pathology, Chonnam National University Hospital, Gwangju, Korea  \n2Department of Pathology, Chonnam National University Medical School and Hospital , Gwangju, Korea  \n3Department of Pathology, Chonnam National University Hwasun Hospital, Jeollanamdo, Hwasuneup, Korea  \n4Department of Pathology, Chonnam National University Medical School and Hwasun Hospital, Gwangju, Korea  \nBackground  \nPrimary peritoneal clear cell carcinoma (PPCCC) is an exceptionally rare malignancy that closely resembles gynecologic epithelial cancers both clinically and histologically. While clear cell carcinoma (CCC) most commonly arises in the ovary or endometrium, its occurrence as a primary tumor in the peritoneum is exceedingly uncommon. Since its first description in 1990 [1], only a limited number of cases have been reported in the English literature [2–15]. Diagnosing PPCCC is challenging due to its overlapping morphological features with more common neoplasms originating from the ovary, endometrium and kidney. The pathogenesis of PPCCC remains  \n© The Author(s) 2026. Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To ","cbCaikkv9IGvhasC","https://ap.wps.com/l/cbCaikkv9IGvhasC","pdf",1352896,"English","# Case presentation\n## Imaging and surgical management\n## Pathology and molecular findings\n# Background and diagnostic challenges\n# Conclusions","[{\"question\":\"Why is PPCCC difficult to diagnose clinically?\",\"answer\":\"PPCCC closely resembles common gynecologic epithelial cancers both clinically and histologically, and its overlapping morphology can mislead diagnosis toward ovarian, endometrial, or renal tumors.\"},{\"question\":\"What findings supported PPCCC with metastasis to the pancreas and spleen?\",\"answer\":\"The pelvic, pancreatic, and splenic lesions showed identical clear cell carcinoma features, with immunohistochemistry positive for PAX8, CK7, and HNF1β, loss of MSH2 and MSH6, and sequencing showing ARID1A loss and PIK3CA mutations; no primary ovarian, endometrial, or renal tumor was detected.\"},{\"question\":\"What were the key clinical symptoms and initial imaging impressions?\",\"answer\":\"A 48-year-old woman presented with acute lower abdominal pain. CT suggested a pancreatic lesion suspicious for pancreatic adenocarcinoma or an islet cell tumor and also identified a large solid pelvic mass suspicious for malignant tumor or metastasis.\"}]","Primary peritoneal clear cell carcinoma with metastasis mimicking ovarian carcinoma - a case report and literature review | PDF",1790136259,18]