Luteinized Thecomas ('Thecomatosis') with Sclerosing Peritonitis (LTSP): Report of 2 Cases and Review of an Enigmatic Syndrome Associated with a Peritoneal Proliferation of Specialized (vimentin+/keratin+/CD34+) Submesothelial Fibroblasts
| Autor: | Shawn K. Murray, Alon D. Altman, James Bentley, Paula V.C. Rittenberg |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
Adult
0301 basic medicine Abdominal pain Pathology medicine.medical_specialty Ovariectomy Antigens CD34 Antineoplastic Agents Vimentin 03 medical and health sciences Peritoneal Neoplasm 0302 clinical medicine Thecoma Ascites medicine Humans Meigs Syndrome Meigs' syndrome Peritoneal Neoplasms Ovarian Neoplasms biology business.industry Carcinoma Ovarian Luteinized Thecoma Disease Management Obstetrics and Gynecology Peritoneal Fibrosis Fibroblasts Middle Aged medicine.disease Bowel obstruction Treatment Outcome 030104 developmental biology 030220 oncology & carcinogenesis biology.protein Keratins Female medicine.symptom business Intestinal Obstruction |
| Zdroj: | Journal of Obstetrics and Gynaecology Canada. 38:41-50 |
| ISSN: | 1701-2163 |
| DOI: | 10.1016/j.jogc.2015.10.010 |
| Popis: | Objective To present the clinicopathologic features of two cases of luteinized thecomas with sclerosing peritonitis (LTSP), characterize the cellular proliferation in the sclerosing peritonitis (SP), and review the literature. Methods The clinical, laboratory, and imaging data, operative findings, and pathology materials were reviewed and summarized. Samples of the SP were stained with keratin AE1/AE3, vimentin, CD34, calretinin, smooth muscle actin, ER/PR, CD10 and desmin. A literature search was performed to identify cases of LTSP for comparison. Results A total of 43 cases of LTSP syndrome were identified. Frequent clinical features included ascites (74%), abdominal pain (35%), bowel obstruction (42%), and bilateral masses (84%). We isolated a distinct form of ovarian luteinized thecoma (thecomatosis) and peculiar sclerosing peritonitis (SP). IHC analysis shows a proliferation of specialized (vimentin+/keratin+/CD34+) submesothelial fibroblasts (SMF) with patchy expression of calretinin and hormone receptors. Conclusion LTSP syndrome is a rare entity presenting with abdominal pain, bowel obstruction, ascites, ovarian masses, and SP containing specialized (vimentin+/keratin+/CD34+) SMF. LTSP must be distinguished from abdominal cocoon, isolated SP, Meigs' syndrome, and peritoneal carcinomatosis. The importance of recognizing the diagnosis is stressed, as failure to manage this disease conservatively leads to significant morbidity and mortality. The SP and bowel obstruction may persist for months, even after resection of the tumours, resulting in extended medical therapy. Based on the immunophenotype of the peritoneal lesions, strategies to elucidate ‘targeted' pharmacologic agents that could inhibit the proliferation of specialized (vimentin+/keratin+/CD34+) SMF may be of benefit. |
| Databáze: | OpenAIRE |
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