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.

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.

Stage II endometrial carcinoma: limiting post-operative radiotherapy to the vaginal vault in node-negative tumors.

OBJECTIVE: To evaluate the outcomes of patients with node-negative stage II endometrial cancer who received vault brachytherapy without external beam pelvic radiotherapy (EBRT). METHODS: A retrospective review of all stage II endometrioid type endometrial cancer patients referred to Cancer Care Manitoba was undertaken between October 1995 and March 2001. Forty-nine patients were identified with disease confined to the uterus, but not all patients received extended surgical staging (ESS) with pelvic lymphadenectomy. These patients were evaluated for recurrence and morbidity data. RESULTS: Twenty node-negative stage II cancers were identified. Three were treated without adjuvant treatment, 12 received vault brachytherapy and 5 received more conventional treatment with EBRT and vault brachytherapy. No recurrences or deaths occurred in these patients. Mean follow-up was 40 months. No surgical complications were encountered in this group and no morbidity from radiotherapy was observed. CONCLUSIONS: Limiting adjuvant treatment to vault brachytherapy for node-negative stage II endometrial cancer results in less morbidity and excellent survival and is worthy of further investigation.

High-risk surgical stage 1 endometrial cancer: outcomes with vault brachytherapy alone.

OBJECTIVE: Prior to 1995, in our center, patients with surgically staged endometrial cancer with greater than 50% myoinvasion (FIGO 1C) were treated with vault brachytherapy and whole pelvis (WP) radiotherapy despite negative nodes. After October 1, 1995, these patients were treated with vault brachytherapy alone. The aim of this study was to ensure that the survival and recurrence rate had not changed. METHODS: A retrospective review of Cancer Care Manitoba charts was undertaken. All patients diagnosed with endometrioid adenocarcinoma between October 1, 1995, and March 1, 2001, were reviewed. Data for all FIGO surgical stage 1 patients, and a subset of stage 1C patients, were analyzed and compared with those of a historical control group, composed of patient data previously collected in our center (1978 to 1990) [Gynecol. Oncol. 55 (1994), 51]. RESULTS: A total of 172 patients had negative selective pelvic lymphadenectomy and FIGO stage 1 disease. Fifty-three stage 1C patients were spared WP radiotherapy. Median follow-up was 32 months. Recurrence rate in FIGO stage 1 disease was 2.3% (4/172) and for the subset 1C was 5.7% (3/53). The recurrence rate was not statistically significantly different from that of the historical control group, 3.6% for stage 1 (P = 0.562) and 7.2% for stage 1C (P = 0.51). Two- and five-year survival rates for stage 1 patients in this study were 97 and 95%, respectively. In the historical group, 2- and 5-year survival rates were 97 and 94%. CONCLUSION: Whole pelvis radiotherapy can be safely omitted in patients with FIGO stage 1C endometrial cancer if nodal status is known.