Primary peritoneal clear cell carcinoma versus ovarian carcinoma versus malignant transformation of endometriosis: a vexing issue

Our plain-English summary of Primary peritoneal clear cell carcinoma versus ovarian carcinoma versus malignant transformation of endometriosis: a vexing issue, by L. Insabato, V. Natella, A. Somma, et al (International Journal of Surgical Pathology, 2015).

The study

• Authors: L. Insabato, V. Natella, A. Somma, M. Persico, L. Camera, N.S. Losito, S. Masone
• Journal: International Journal of Surgical Pathology
• Published: 2015;23(3):211-6
• Type of article: Case report
• Cited by: 7 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Peritoneum is a site for both primary and secondary tumors. Primary peritoneal tumors are fairly rare. The most common primary tumors of the peritoneum are malignant mesothelioma and serous papillary adenocarcinoma. Clear cell carcinoma of the peritoneum is extremely rare and often misdiagnosed as mesothelioma, serous carcinoma, or metastatic adenocarcinoma, so it represents a diagnostic challenge for both clinicians and pathologists. Up to date, to the best of our knowledge, only 11 cases of primary peritoneal clear cell carcinoma have been reported in the English literature. Distinguishing this tumor of the peritoneum versus ovarian carcinoma can be problematic. Herein, we report a rare case of primary peritoneal clear cell carcinoma occurring in a 49-year-old woman, along with a review of the literature.

© The Author(s) 2015.

In plain English

This article was a case report with a review of the literature on primary peritoneal clear cell carcinoma, a rare cancer of the abdominal lining that the authors said must be told apart from mesothelioma and other tumors. The article did not discuss asbestos. In February 2013 a 49-year-old woman was referred to the authors' hospital in Naples, Italy, and had surgery for a 9.5 by 9 by 7 cm mass attached to the outside of the ileum (small bowel). It was a clear cell carcinoma. Its cells were negative for thrombomodulin and Ck5/6, markers the authors linked to mesothelioma. No tumor was found in the left ovary or the omentum, extensive sampling found no endometriosis, and slides from an earlier removal of the right ovary confirmed a benign cyst. Six months later cancer was found in a groin lymph node. After more surgery and chemotherapy, the patient was alive and disease-free in July 2014. Across this case and 11 earlier reported cases, the authors counted endometriosis in 4 cases (33.3%) and 3 deaths (25%) within 6 months. The authors concluded that new treatment strategies are needed.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report of one patient, with a review of the 11 previously reported cases in the English literature
• Who was studied: One 49-year-old woman referred in February 2013 to the authors' hospital (University Federico II, Naples, Italy) for an abdominal mass. The literature review covered 11 earlier reported cases.
• Main results:
  • In the patient, surgery removed a 9.5 by 9 by 7 cm mass attached to the outer surface of the ileum.
  • The patient's tumor cells were strongly positive for Ck7 and focally positive for PR and vimentin.
  • The patient's tumor cells were negative for a list of markers that included thrombomodulin, Ck5/6, WT-1 and CA125.
  • In the patient, the tumor infiltrated the serosa of the ileum. No tumor was found in the left ovary or the omentum, and extensive sampling found no endometriosis.
  • Slides from the patient's earlier right oophorectomy were reviewed and the benign ovarian cyst diagnosis was confirmed, leading to a definitive diagnosis of primary peritoneal clear cell carcinoma.
  • Six months later the patient had a left inguinal lymph node that showed metastasis from clear cell carcinoma.
  • After 6 cycles of carboplatin and taxol chemotherapy, the patient was alive and disease-free at the last follow-up in July 2014.
  • Across the 12 tabulated cases (11 earlier cases plus this one), the average age was 54.41 years (range 37 to 67), and endometriosis was present in 4 cases (33.3%).
  • In the reported cases, the recurrence and metastatic rate was about 33%, and 3 patients (25%) died of disease within 6 months.
• The authors' conclusion: The authors concluded that a diagnosis of primary peritoneal clear cell carcinoma should be kept in mind when dealing with a primary peritoneal tumor, and that new treatment strategies are needed to improve local control and reduce deaths.
• Limitations the authors state:
  • The authors noted that few data on the outcome of this tumor are available because it is rare.

Topics

Medical terms in this study, explained in our Lexicon: Carcinoma, Malignant Transformation, Peritoneum, Malignant Mesothelioma, Adenocarcinoma, Mesothelioma.

Related studies

• Cytomorphology of ovarian clear cell carcinomas in peritoneal effusions — D. Damiani, V. Suciu, et al. (2016)
• Are oestrogens and genetic predisposition etiologic factors in the development of clear cell carcinoma of the peritoneum? — R. Wuntakal, A. Lawrence (2013)
• Hepatocyte nuclear factor-1β is not a specific marker of clear cell carcinoma in serous effusions — B. Davidson (2014)
• Clear cell colorectal carcinoma: Time to clarify diagnosis — A. Remo, F. Grillo, et al. (2017)
• Malignant peritoneal mesothelioma, clear cell variant, in a female and its differentiation from clear cell carcinoma — H. Hayashi, T. Kawata, et al. (2017)
• Potentiation of cisplatin and carboplatin cytotoxicity by amphotericin B in different human ovarian carcinoma and malignant peritoneal mesothelioma cells — L. Poulain, F. Sichel, et al. (1997)

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