Endometrial Stromal Sarcoma With Hyalinizing Giant Rosettes, Mimicking Low-Grade Fibromyxoid Sarcoma.

We highlight a rare variant pattern of low-grade endometrial stromal sarcoma showing extensive collagenous rosette formation, closely mimicking low-grade fibromyxoid sarcoma. Additionally, this neoplasm showed diffuse and strong expression of muscle markers, favoring an initial diagnosis of leiomyosarcoma. Reverse transcription-polymerase chain reaction showed the presence of JAZF1-SUZ12 fusion transcripts, and this highlights the broad morphologic and immunophenotypic spectrum of endometrial stromal sarcoma.

Long-Term Outcome of Aromatase Inhibitor Therapy With Letrozole in Patients With Advanced Low-Grade Endometrial Stromal Sarcoma.

BACKGROUND: There has been no consensus on the indications for the treatment of advanced low-grade endometrial stromal sarcoma (LGESS), and the possible effects of hormonal treatment including progestins and aromatase inhibitors have been reported. The aim of this study was to investigate the efficacy of aromatase inhibitor therapy with letrozole for patients with residual or recurrent LGESS. METHODS: We retrospectively reviewed the clinical response of patients with advanced LGESS who had been treated with letrozole. We also analyzed the adverse effects after the administration of letrozole. The expression levels of estrogen receptor and aromatase in the tumors were immunohistochemically examined. RESULTS: In 5 patients who had been treated for unresectable LGESS lesions after initial or repeat surgical procedures, residual lesions in 3 patients and recurrence lesions in 2 patients were the indications for hormonal therapy with letrozole. The median duration of letrozole exposure at retrospective analysis was 53 (10-96) months. The clinical outcomes were classified as complete response in 2 patients, partial response in 1 patient, and stable disease in 2 patients. Myalgias, hot flashes, and arthralgias were not observed during the follow-up period in any patients. The median serum levels of estradiol were <5.0 (cutoff value, <0.5-11.8) pg/mL. The median age-matched bone mineral densities were 92% (79%-123%). The LGESS tissues in all 5 patients were positive for estrogen receptor and aromatase expression. CONCLUSIONS: Letrozole as well as progestins could be the first choice of treatment for patients with recurrent or residual LGESS, which is difficult to resect surgically because of its efficacy and minimal adverse effects.

Clinical characteristics and outcomes of uterine tumors resembling ovarian sex-cord tumors (UTROSCT): a systematic review of literature.

Uterine tumor resembling ovarian sex-cord tumors (UTROSCT) is an extremely rare type of uterine tumor, and its clinical characteristics are not fully understood. A systematic literature search was conducted in PubMed and MEDLINE using the keywords, "uterine tumors resembling ovarian sex cord tumors", limited to case reports. Clinico-pathological characteristics and survival data were abstracted and evaluated for the analysis. Among 43 cases reporting UTROSCT, Type I (endometrial stromal tumors with sex cord-like elements, ESTSCLE) and Type II (classic UTROSCT) were reported in 5 (11.6%) and 17 (39.5%), respectively, and nearly half of reported UTROSCT did not subcategorize the histology pattern into Type I or II (unspecified, n=21, 48.8%). Mean age was 52.2. The two most common symptoms were postmenopausal vaginal bleeding (44.2%) and abnormal menstruation (39.5%). The majority underwent total hysterectomy with adnexectomy (65.1%) followed by hysterectomy alone (18.6%) and tumor resection alone (14.0%). Mean tumor size was 6.2cm, and extra-uterine spread was seen in 7.0%. By immunohistochemistry, calretinin expression was significantly correlated with CAM5.2, inhibin, and progesterone receptor expression (all, p<0.05). In survival analysis, disease-free survival (DFS) rates for all 43 cases at 1, 2, and 5 years for all cases were 97.0%, 92.7%, and 69.7%, respectively. Among recurrent cases, median time to recur was 24 months (range 9-48). Decreased DFS was significantly associated with pelvic pain (2-year rate, 81.8% versus 94.7%, p=0.006), histology subcategory (Type I versus II, 23.8% versus 100%, p=0.006), tumor size ≥10cm (75.0% versus 100%, p=0.046), cervical/extra-uterine metastasis (46.7% versus 100%, p=0.024), and lymphovascular space involvement (50% versus 100%, p=0.002). Treatment patterns were not statistically associated with DFS (hysterectomy, p=0.28; and adnexectomy, p=0.38). When histology patterns were examined, Type II disease was associated with less aggressive tumor behavior when compared to Type I disease: extra-uterine spread (Type I versus II, 40% versus 5.9%, p=0.007) and lymphovascular space invasion (50% versus 6.7%, p=0.012). Among 17 cases of Type II disease, disease recurrence was reported in 1 (5.9%) case at 3 years after the initial treatment. In conclusion, our study showed that UTROSCT was often not subcategorized. Because classic UTROSCT has a distinct clinical outcome and characteristic histological patterns when compared to ESTSCLE, distinguishing UTROSCT from ESTSCLE is an integral component of the diagnosis. While classic UTROSCT typically has a favorable prognosis, it has been known to develop a late recurrence. If risk factors for recurrence are absent, both hysterectomy and mass resection alone are possible options for management.

Primary endometrioid stromal sarcoma of the ovary: a clinicopathologic study of 27 cases with morphologic and behavioral features similar to those of uterine low-grade endometrial stromal sarcoma.

Twenty-seven endometrioid stromal sarcomas of the ovary from patients 38 to 76 (mean 56) years of age are reported. The tumors were unilateral in 20 cases and bilateral in 7. They were solid (9), solid and cystic (9), or predominantly cystic (6) when this information was known and ranged from 1 to 20 (mean 9.5) cm. The solid areas typically had a tan-yellow cut surface, with areas of hemorrhage and/or necrosis noted in 6; however, in addition, blood was often present in the cyst lumens. On microscopic examination, the predominant and frequently exclusive pattern was a diffuse growth of small cells with interspersed arterioles, the latter appearing round to elongated. A fibromatous pattern was present in 14 of the tumors but was extensive in only 3. A vague nodular growth was observed in 10 tumors but was never striking; a storiform growth was seen in 2 tumors, being conspicuous in 1. Hyaline plaques were present in 10 tumors but were striking in only 2. Sex cord-like or smooth muscle differentiation was seen in 7 and 6 tumors, respectively, being striking in 2 and 3 of them. Foam cells were present in 6 tumors. The tumors showed minimal cytologic atypia. The mitotic index ranged from <1 to 17/10 high-power fields (HPF), being <1/10 HPF in 12, 1 to 5/10 HPF in 9, 6 to 10/10 HPF in 2, and >10/10 HPF in 4 tumors. Infarct-type necrosis was noted in 12 tumors. Hemorrhage, typically recent, was seen in 20 cases, being conspicuous in 5. Ovarian endometriosis was intimately associated with the tumor in 16 cases. Seven patients had stage I tumors, 5 stage II, 13 stage III, and 2 stage IV. Follow-up information was available for 21 patients; 10 were alive and free of disease from 4 to 21 years postoperatively (follow-up being ≥ 11 y in 5); 6 were alive with disease from 1 to 22 years postoperatively; 5 patients are known to have died of disease, with the interval being unknown in 1, and 2, 4, 13, and 17 years in the others. Follow-up information was unavailable in the remaining 6 patients. These findings indicate that these tumors, as in the uterus, often have an indolent course with a better prognosis than other ovarian sarcomas, indicating the importance of correct diagnosis. The differential diagnosis of these neoplasms is in the first instance with a metastasis from the uterus; knowledge of the status of the uterus is paramount in this distinction. Associated ovarian endometriosis suggests a primary tumor. When a primary ovarian origin is determined, the differential diagnosis is most often with a sex cord-stromal tumor, particularly a granulosa cell tumor because of a diffuse growth of cells with scant cytoplasm.

Metastatic endometrial sarcoma with inferior vena caval and cardiac involvement: a combined surgical approach.

Low-grade endometrial stromal sarcoma with intracaval and intracardiac extension represents a complex and often lethal condition. A case of a 40-year-old woman, who underwent hysterectomy for endometrial stromal sarcoma 4 years previously and developed recurrence with intracaval and intracardiac metastases, is presented. Operative management of resection of the intracaval and intracardiac tumor is described. The literature is reviewed in order to highlight the data on diagnosis and management of recurrent endometrial stromal sarcoma with vascular involvement, including the multimodality approach required in treating this disease.

Study of the sentinel node in endometrial cancer at early stages: preliminary results.

AIM: To investigate the applicability of the sentinel lymph node biopsy technique in early stages of endometrial cancer. MATERIAL AND METHODS: A prospective study that included consecutive patients with a histological diagnosis of clinical state I endometrial carcinoma was performed. Two doses of 2 mCi (74 MBq) of (99m)Tc-albumin nanocolloid were injected in the uterine cervix, and planar and SPECT-CT images were obtained at one hour, and at 24 hours if no migration of the tracer was observed. Methylene blue dye was also injected into the cervix immediately prior to the surgery. A gamma probe was used during the surgical procedure for sentinel lymph node identification. In all cases, a hysterectomy, double adnexectomy and pelvic lymphadenectomy were performed, carrying out a histological analysis (hematoxylin-eosin) of the sentinel lymph nodes and the lymphadenectomy specimen. RESULTS: We included 19 patients, with a final diagnoses of endometrioid carcinoma (18 cases) and endometrial stromal sarcoma (1 case). At least one sentinel lymph node was identified in 17 of them (89.5% detection rate). Twenty-nine sentinel lymph nodes were identified during surgery, all of them negative for neoplastic infiltration. No metastatic invasion was found in the pelvic lymphadenectomy specimens as well. CONCLUSIONS: The sentinel lymph node biopsy technique seems to be a reliable tool in nodal staging of endometrial cancer at early stages, with an acceptable detection rate and high histological correlation. The low prevalence of lymphatic spread in this group of patients and the encouraging results obtained could make the sentinel lymph node an alternative to routine complete lymphadenectomy.

Endometrial stromal sarcoma invading the abdominal aorta treated with aortic replacement.

Endometrial stromal sarcomas are extremely rare tumors with few reports describing invasion of major blood vessels. Tumors that primarily involve the abdominal aorta or invade the vessel secondarily are uncommon. Outcomes from radical tumor resection and aortic reconstruction are thus poorly understood. We present a rare case of a symptomatic recurrent endometrial stromal sarcoma with encasement of the infrarenal abdominal aorta requiring en bloc tumor resection and in situ aortic replacement.

[Postoperative radiotherapy of uterine sarcoma: a multicentric retrospective study]. / Radiothérapie postopératoire dans les sarcomes utérins: étude rétrospective multicentrique.

PURPOSE: Surgery is the treatment of choice for localized uterine sarcomas. We conducted a retrospective study to define prognostic factors. PATIENTS AND METHODS: We studied 111 cases of patients treated by adjuvant radiotherapy for uterine sarcoma in seven French centers. The median decline was 31 months. We conducted a univariate analysis to identify factors correlated with local recurrence. The statistically significant factors were studied in multivariate analysis by Cox model. RESULTS: The median dose of external beam radiotherapy was 45 Gy. Forty-three percent of patients had vaginal vault brachytherapy and 21 % chemotherapy. Only 6.3 % of patients had complications of acute grade III and 8.1 % of long-term sequelae of radiotherapy. The survival rate at 5 years was 74.6 %. They noted 12.6 % of isolated locoregional recurrences, against 29.7 % for distant recurrences, 80 % were pulmonary. Factors correlated with the risk of locoregional relapse were menopausal status (P = 0.045) and surgical margins suspicious or not healthy (P = 0.0095). The chemotherapy did not improve overall survival or disease free survival but the numbers were low. CONCLUSION: The postoperative radiotherapy provides good local control in this disease. Brachytherapy is sometimes done, but it does not improve local control. Chemotherapy is not a standard localized stage but the rate of metastatic recurrence calls for the development of strategies involving systemic treatment with radiotherapy.

Gynecologic cancers: factors affecting survival after pulmonary metastasectomy.

BACKGROUND: Little information is available regarding long-term survival after pulmonary metastasectomy for gynecologic malignancies. METHODS: All patients who underwent pulmonary resection for gynecologic malignancies at our institution between January 1985 and June 2001 were reviewed. Factors affecting long-term survival were analyzed. RESULTS: There were 103 patients, 70 of whom had metastatic disease limited to the lungs. Median age of these 70 patients was 59.4 years (range, 31 to 80 years). The primary tumor originated in the uterine corpus in 37 patients, endometrium in 23, cervix in 7, ovaries in 2, and vagina in 1. Histopathology was leiomyosarcoma in 29 patients, adenocarcinoma in 23, other sarcoma in 11, squamous cell carcinoma in 5, and choriocarcinoma and endolymphatic stromal myosis in 1 each. The median time interval between the first gynecologic procedure and pulmonary resection was 24 months (range, 0 to 237 months). A wedge excision was performed in 44 patients, lobectomy in 14, bilobectomy in 2, pneumonectomy in 1, and a combination in 9. Five patients (7%) had an incomplete resection. Eighteen patients (25.7%) developed at least one complication and 1 died (operative mortality, 1.4%). At last follow-up, 35 had died, and the median follow-up among those who were still alive was 36 months (range, 6 months to 13 years). Five-year and 10-year survival was 46.8% (95% confidence interval, 34.2% to 63.0%) and 34.3% (95% confidence interval, 19.7% to 52.5%), respectively. Factors that adversely affected survival include a disease-free interval between the first gynecologic procedure and pulmonary resection of less than 24 months (p = 0.004) and a primary site located in the cervix (p < 0.001). CONCLUSIONS: Pulmonary resection for metastatic gynecologic cancer in selected patients is safe and effective. Both a short disease-free interval between the primary gynecologic procedure and pulmonary metastasectomy, and a primary cervical tumor had an adverse effect on survival.