Endometrial stromal tumors: A clinico-histomorphological spectrum of endometrial stromal tumors with review of literature.

BACKGROUND: Endometrial stromal tumors (ESTs) are rare subset of mesenchymal uterine neoplasms. There are heterogeneous morphological, immunohistochemical, and genetic features. Approximately 50% of ESTs occur in perimenopausal women. In 2020, WHO sub-categorized ESTs into four groups: endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LGESS), high-grade endometrial stromal sarcoma (HGESS), and undifferentiated uterine sarcoma (UUS). OBJECTIVE: To review the morphological spectrum of endometrial stromal tumors. METHOD: This retrospective study reviewed the histomorphological features of 15 endometrial stromal tumors with respect to atypia, necrosis, mitosis, collagen bands, whorling around vessels, myometrial invasion, and inflammatory cells. Immunohistochemistry markers (CD10, SMA, and ER) along with special stains (Masson's trichrome, toluidine blue) were also studied. RESULTS: The age of the patients ranged from 32 to 60 years. Three patients were postmenopausal. The most common presenting symptom was vaginal bleeding. Five patients were operated with a clinical diagnosis of uterine fibroid. One patient presented with prolapse with no other complaint. All the 15 patients had total abdominal hysterectomy and salpingo-oophorectomy. One case showed necrosis, eight cases showed collagen bands, all the 15 cases showed whorling around vessels, one case showed vascular emboli, and seven cases showed inflammatory cells. In low-grade cases, one case showed focal atypia and one case showed focal coagulative necrosis indicating infarction. Thirteen cases were LGESS, and one case of ESN and HGESS. All cases were positive for ER and CD10. CONCLUSION: Endometrial stromal tumors demonstrate extensive permeation of the myometrium as irregular islands with frequent vascular invasion, whorling around vessels, collagen bands, and inflammatory cells. All these features should be observed thoroughly on microscopy by pathologists to clearly differentiate the low-grade and high-grade endometrial stromal tumors, and to understand the overlapping gray areas morphologically as it affects the prognosis of the patient.

Refractory Pneumothorax Due to Cystic Pulmonary Metastasis of a Lowgrade Endometrial Stromal Sarcoma: A Case Report.

Low-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine neoplasm. Computed tomography (CT) revealed the presence of multiple small bilateral pulmonary nodules in a 58-year-old woman 1 year after surgery for LG-ESS; the clinical diagnosis was pulmonary metastasis. Hormone therapy with progesterone was initiated, after which most of the solid nodules disappeared and some transformed into cystic lesions. Seven years after hormone therapy, the patient experienced repeated pneumothorax. The cause of the pneumothorax was perforation of a metastatic focus within the wall of a small subpleural cyst that was not evident on CT images.

The value of surgery and the prognostic factors for patients with recurrent low-grade endometrial stromal sarcoma: a retrospective study of 38 patients.

OBJECTIVE: As an indolent malignant tumor, the long-term management of low-grade endometrial stromal sarcoma (LGESS) patients required awareness, especially the management of recurrences. Unfortunately, few studies focused on the treatment of recurrent LGESS. Our study aimed to investigate the prognostic factors and the value of recurrent surgery on recurrent LGESS. METHODS: This retrospective study consecutively recruited patients with pathologically diagnosed recurrent LGESS at our center from April 1, 2004 to April 1, 2020. RESULTS: After a median follow-up of 137.0 months (95% confidence interval=85.4-188.6), the 5-year cumulative survival rate of the cohort of 38 patients with recurrent LGESS was 71.1%. The median overall survival (OS) and post-recurrence survival (PRS) was 156 and 89.0 months. Survival analysis showed that patients with younger age, positive estrogen receptor (ER) and optimal abdominopelvic debulking in the first recurrent surgery had better prognosis (p<0.05). Multivariate analysis showed that optimal abdominopelvic debulking in the first recurrent surgery was the only independent prognostic factor for OS and PRS (OS=216.0/35.0 months, hazard ratio [HR]=5.319, p=0.034; PRS=not reached/4.0 months, HR=10.900, p=0.006). There was no significant difference in OS and PRS between patients recurred only once and those recurred at least twice (p>0.05). CONCLUSIONS: The prognosis of recurrent LGESS was favorable. Optimal debulking of no residual tumor in abdominal and pelvic cavity should be the first choice of treatment for recurrent patients, while preservation of ovary or fertility should not be recommended.

Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study.

OBJECTIVE: Little is known about the survival differences between uterine and extrauterine low-grade endometrial stromal sarcoma (LGESS). Survival outcomes, consisting of disease-free survivals and overall survivals (OS), were compared in these two entities. METHODS: From February 2012 to June 2019, all primary LGESS cases and LGESS cases with first recurrence in the study center were reviewed. The clinicopathological characteristics and survival outcomes of extrauterine and uterine LGESS patients were compared for both primary and recurrent diseases. RESULTS: During the study period, 143 patients with primary LGESS and 56 patients with recurrent LGESS were included and followed up to 1 June 2020, among whom 8 (5.6%) and 10 (17.8%) patients were identified as having extrauterine LGESS. Patients with primary and recurrent extrauterine LGESS had similar clinicopathological characteristics to those of patients with uterine LGESS. In primary or in recurrent LGESS cases, in univariate analysis, patients with uterine and extrauterine LGESS had similar disease-free intervals after the last treatment, and they also had similar OSs after the diagnosis. Ovarian preservation led to significantly increased recurrence for primary LGESS [hazard ratio (HR) 4.9, 95% CI: 2.3-10.1, P <0.001) and repeated recurrence for recurrent LGESS (HR 3.1, 95% CI: 1.3-7.3, P =0.009). Surgical treatment for recurrent LGESS decreased repeated recurrence after the first recurrence (HR 0.2, 95% CI: 0.1-0.7, P =0.006). No factors were found to be associated with the OS of primary or recurrent LGESS. CONCLUSION: The clinical characteristics and survival outcomes of extrauterine LGESS are similar to those of uterine LGESS. Surgery is the treatment of choice for recurrent LGESS. Ovarian preservation is detrimental to disease-free survival but not to OS in both uterine and extrauterine LGESS.

Sarcoma del estroma endometrial mixoide, una rara variante histopatológica. Dos casos clinicopatológicos / Myxoid endometrial stromal sarcoma: A rare histopathologic variant. Two clinicopathological cases

La variante mixoide del sarcoma del estroma endometrial es una neoplasia uterina extremadamente rara caracterizada por la presencia de predominante material mixoide y células fusiformes atípicas que muestran características histopatológicas e inmunohistoquímicas de sarcoma del estroma endometrial. Presentamos 2 casos clinicopatológicos de mujeres de 54 y 76 años de edad, ambas con metrorragia posmenopáusica, útero aumentado de tamaño e imagen de tumoración dependiente de la pared miometrial, sometidas a histerectomía total con doble anexectomía con diagnóstico anatomopatológico de sarcoma del estroma endometrial variante mixoide

Myxoid endometrial stromal sarcoma is an extremely rare uterine neoplasm characterised by the presence of atypical predominantly myxoid material fusiform cells showing histopathological and immunohistochemical characteristics of endometrial stromal sarcoma. We present two clinicopathological cases of a 54-year-old woman and 76-year-old woman, both with postmenopausal vaginal bleeding, enlarged uterus and myometrial wall-dependent tumour imaging, undergoing total hysterectomy with double oophorectomy with pathological diagnosis of myxoid endometrial stromal sarcoma

Sarcoma de estroma prostático gigante / Giant prostatic stromal sarcoma

Los tumores de próstata representan la neoplasia maligna más frecuente en el varón. Mientras que las lesiones epiteliales son frecuentes, las neoplasias de origen mesenquimal representan entre 1 y 2 porciento de las neoplasias malignas prostáticas. Son poco conocidas, sobre todo las de origen estromal. Se presenta el caso de un varón de 77 años de edad, con un sarcoma del estroma prostático al que se le realiza una exéresis parcial. El tumor ocupa prácticamente la totalidad de la cavidad pélvico-abdominal, con un diámetro de 35 cm y un peso de 3 800 g. Histológicamente se concluyó, como sarcoma prostático aunque en el transoperatorio no se encontró un nexo evidente con este órgano. El comportamiento clínico de estos tumores, basado en los escasos casos descritos en la literatura, es prácticamente desconocido y su diagnóstico preoperatorio difícil. Este paciente se presentó con pérdida de peso, sin dolor, hematuria, ni síntomas obstructivos(AU)

Prostate tumors represent the most frequent malignancy in men. Epithelial lesions are frequent, whereas malignancies of mesenchymal origin represent only 1-2 percent of all prostatic malignacies. They are little known, mostly those of stromal origin. A case of a 77 year- old man with prostatic stromal sarcoma is presented to whom partial exeresis is performed. The tumor practically occupies the totality of the pelvic abdominal cavity, and it had a diameter of 35 cm and a weight of 3 800 g. Histologically, it was recognized as prostatic sarcoma although an evident link with this organ was not found during the transoperatory. The clinical behavior of these tumors, based on the little cases described in the literature, is practically unknown and its preoperative diagnosis becomes difficult. The patient presented with weight loss, he had no pain, hematuria, or obstructive symptoms(AU)

Endometrial stromal sarcoma. Is there a place for radiotherapy?

OBJECTIVES: The optimal approach in the management of endometrial stromal sarcoma (ESS) remains unclear. The aim of the present study was to retrospectively report the outcome of patients treated for ESS in our hospital over a 27-year period in order to evaluate the treatment results and the role of radiotherapy. PATIENTS AND METHODS: From 1979 to 2006, 13 patients with ESS were treated at the Hospital Clínic of Barcelona. Patients underwent abdominal hysterectomy and bilateral salpingo-oophorectomy. The 1989 FIGO classification for endometrial carcinoma was used in this retrospective study. Seven patients presented stage I (6 IB and 1 IC), 1 stage II, 3 stage III and 2 stage IV. Nine patients had high-grade tumours with an infiltration of the outer 50% of the myometrium. Postoperative radiotherapy was administered in 10 patients. RESULTS: The mean follow-up of the patients was 54.6 months (range between 3 and 190). Patients with stage IB had a better outcome in comparison to more advanced stages. Five of the six patients with stage IB received adjuvant radiotherapy and none developed local recurrence, while one patient who received no treatment with radiotherapy had a relapse. Seven of the 13 patients had stages over IB: 5 who had received radiotherapy after surgery had locoregional control and 2 who did not receive radiotherapy had local relapse. Nine patients had high-grade tumours, 6 received radiotherapy after surgery and only one had local relapse. Of the three who did not receive radiotherapy, 2 relapsed locally. Local control rate of the patients who received adjuvant radiotherapy was higher than in the patients who did not (88.9% vs. 50%). CONCLUSIONS: Our data reveal that deep myometrial invasion and stage over IB are significantly associated with poor overall survival and this finding is similar to those of studies in patients with endometrial cancer. The present study showed that the local control was higher in patients receiving radiotherapy (AU)

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Sarcoma del estroma endometrial con patrón tipo cordones sexuales / Endometrial stromal sarcoma with sex cord-like differentiation

Los sarcomas del estroma endometrial son neoplasias malignas poco frecuentes originadas en el estroma endometrial, que pueden presentar varias formas de diferenciación, incluidos diferenciación muscular lisa y tipo cordones sexuales. Describimos un caso de sarcoma del estroma endometrial con diferenciación tipo cordones sexuales en una paciente posmenopáusica, y se discute su diagnóstico diferencial y tratamiento. Los sarcomas del estroma endometrial son neoplasias heterogéneas con diferenciación multipotencial y su diferenciación tipo cordones sexuales puede plantear dificultades diagnósticas (AU)

Endometrial stromal sarcomas are rare malignant neoplasms arising from the endometrial stroma. These tumors may exhibit several forms of differentiation, including smooth muscle and sex cord differentiation. We report a case of endometrial stromal sarcoma with sex cord-like differentiation in a postmenopausal woman and discuss the differential diagnosis and treatment of this entity. Endometrial stromal sarcomas are heterogeneous neoplasms with multipotential differentiation. A uterine tumor resembling an ovarian sex cord tumor may pose diagnostic difficulties

Sarcoma estromal de bajo grado. A propósito de un caso / Low grade stromal sarcoma. a case study

We present the case of a 26-year-old woman who on two occasions underwent transcervical resection through resectoscopy of intracavity formations compatible with endometrial and myometrial fragments with stromal proliferation. These findings guided the diagnosis of low-grade stromal tumor. Because of the diagnostic finding and the aggressiveness of the tumor, hysterectomy was performed. Histological diagnosis was low-grade stromal tumor. Laparoscopic bilateral annexectomy was subsequently performed (AU)

Sarcoma uterino como causa de inversión uterina / Uterine sarcoma as a cause of uterine inversion

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