Japan Society of Gynecologic Oncology 2018 guidelines for treatment of uterine body neoplasms / 부인종양

Mullerian adenosarcoma with sarcomatous overgrowth in a premenopausal patient / Philippine Journal of Obstetrics and Gynecology

@#We report a case of a 33-years old, nulligravid, diagnosed with mullerian adenosarcoma with sarcomatous overgrowth (MASO), who presented with vaginal bleeding and recurrent endometrial polyp. MASO is a rare type of uterine sarcomas, it is a variant of adenosarcomas with poor prognosis. The index patient underwent primary surgical management with lymphadenectomy with a final stage of IC. Histologic diagnosis was Mulllerian adenosarcoma with sarcomatous overgrowth. A panel of immunostaining for estrogen receptors, progesterone receptors and CD 10 showed diffused positivity for the hormones with loss of CD 10 which is consistent with MASO. The rarity of MASO has a distinctive histologic features which merits meticulous sectioning as the clinical course and management vary. It has a poor prognosis due to its short and fast course of the disease.

Imaging description of extragenital müllerian adenosarcoma: A case report / Descrição da imagem do adenosarcoma mülleriano extragenital: relato de caso

Abstract Müllerian adenosarcoma is a very rare gynecological disease, comprising 5% of uterine sarcomas. Extragenital localizations are even rarer.We report a very interesting case of a 27-year-old woman complaining of pelvic pain, with a subsequent diagnosis of extragenital Müllerian adenosarcoma. This is the first case reported in the literature with a complete and wide imaging description. Even if rare, Müllerian adenosarcoma should be hypothesized in case of young female patients presenting with suspicious pelvic mass.

Resumo O adenosarcoma Mülleriano é uma doença ginecológica muito rara, compreendendo 5% dos sarcomas uterinos. Localizações extragenitais são ainda mais raras. Relatamos um caso muito interessante de uma mulher de 27 anos queixando-se de dor pélvica com diagnóstico subsequente de adenosarcoma Mülleriano extragenital. Este é o primeiro caso relatado na literatura com uma descrição completa e ampla de imagem. Mesmo que raro, o adenosarcoma Mülleriano deve ser hipotetizado no caso de pacientes jovens do sexo feminino com massa pélvica suspeita.

Clinical and color Doppler ultrasound features of uterine sarcoma / 解放军医学杂志

Objective: To investigate the clinical characteristics of uterine sarcoma and clinical application of color Doppler ultrasonography in the sarcoma. Methods: We conducted a retrospective analysis on the clinical and ultrasonographic features in 128 surgically and pathologically confirmed uterine sarcoma patients from December 2011 to May 2018 in Tongji Hospital. The clinical features included age, clinical manifestation, serum tumor marker CA125 and clinical stage, and the features of ultrasonography included the size, boundary, echo type of the lesion and characteristics of blood flow signals. Results: The majority of uterine sarcoma in this study were endometrial stromal sarcoma and leiomyosarcoma. Endometrial stromal sarcoma occurred mostly in women in reproductive period, while leiomyosarcoma occurred mainly in perimenopausal and postmenopausal women. The predilection age of uterine sarcoma was 49.6 ± 13.4 years. The main clinical manifestations were abnormal uterine bleeding, including postmenopausal vaginal bleeding or irregular vaginal bleeding (47.7%), and abdominal pain (32.0%). About 20.3% of patients had no symptoms. Serum CA125 was detected before operation in all the patients, and it was in normal range (≤ 35 U/ml) in 59 patients, slightly higher than normal level (35-100 U/ml) in 51 women and significantly higher than normal range (>100 U/ml) in 18 women. Pelvic three-dimensional ultrasonography was usually characterized by large uterine tumors with solid, unclear boundary, heterogeneous echo structures with or without cystic degeneration and rich blood flow signals, which can be roughly classified as malignant tumors. Conclusion: Combined with clinical manifestations such as vaginal bleeding, abdominal pain, abdominal distension, ultrasonograms can help us identify and early predict large, ill-defined, hypoechoic or heterogeneous hypoechoic tumors with rich blood flow signals, and grasp the treatment opportunity reasonably and formulate treatment plans.

Preoperative differential diagnosis of uterine sarcoma and uterine myoma / 中国实用妇科与产科杂志

Uterine sarcoma is a rare uterine malignant tumor characterized by extremely aggressive behavior with a high recurrence rate and poor prognosis. It remains very challenging to distinguish uterine sarcomas from uterine fibroids prior to surgery because of the similar clinical manifestations and the lack of specific imaging features and tumor markers. An integrated analysis algorism including risk factors,symptoms,imaging analysis(pelvic ultrasound and MRI),and endometrial biopsy is helpful for the preoperative differential diagnosis between uterine sarcomas and uterine leiomyomas.

Sonographic and Clinical Characteristics of Uterine Sarcoma Initially Misdiagnosed as Uterine Fibroid in Women in the Late Reproductive Age

OBJECTIVES: Uterine sarcoma is a rare malignant tumor, which is usually diagnosed in postmenopausal women. These sarcomas are occasionally misdiagnosed as uterine fibroids, thereby leading to delayed diagnosis in the advanced stages. We analyzed the sonographic and clinical characteristics of unexpected uterine sarcomas detected after surgery in women in the late reproductive age.METHODS: The medical records of 61 patients preoperatively diagnosed with uterine leiomyomas through sonography but confirmed as uterine sarcomas after surgery from January 2005 to December 2018 at Asan Medical Center were retrospectively analyzed. We evaluated the clinical symptoms, sonographic findings, and Doppler indexes, and investigated whether there were any significant characteristics that could clearly differentiate uterine sarcoma from fibroids.RESULTS: The most common clinical finding was increased mass size (15 patients, 24.6%), while 9 patients (14.8%) showed no symptoms. Ultrasonography showed that the maximum diameter of most fibroids was > 5 cm (49 patients, 80.3%), and the average diameter was 75.6 ± 36.3 mm. All the patients showed heterogeneous echogenicity in sonographic imaging. Secondary degeneration of the myomas was reported in 36 patients (59%), and approximately 90% (32/36, 88.9%) showed cystic changes. Of the 40 patients who underwent the evaluation of vascularity, 35 showed increased vascularity of the mass.CONCLUSIONS: In this study, sarcomas misdiagnosed as leiomyomas were usually > 5 cm, and ultrasonography showed heterogeneous echogenicity and irregular cystic degeneration. No definite clinical symptoms were helpful; a thorough evaluation is necessary to rule out uterine sarcomas in women having uterine mass with these characteristics.

Leiomiosarcoma uterino de alto grado / High grade uterine leiomyosarcoma

Los sarcomas de útero son un grupo diverso de tumores que constituyen 1 por ciento de las neoplasias del aparato genital femenino. La incidencia mundial es de 0,5 a 3,3 casos por cada 100,000 mujeres por año. Se presenta un caso poco frecuente con diagnóstico de leiomiosarcoma uterino de alto grado y se hace una revisión de la literatura sobre este tema(AU)

Uterine sarcomas are a diverse group of tumors that make up 1 percent of the neoplasms of the female genital tract. The worldwide incidence is from 0.5 to 3.3 cases per 100,000 women per year. A rare case with a diagnosis of high-grade uterine leiomyosarcoma is presented and a review of the literature on this subject is made(AU)

A rare case of Epithelioid Trophoblastic tumor: An ultrasound dilemma / Philippine Journal of Obstetrics and Gynecology

@#Gestational trophoblastic neoplasia (GTN) represents the malignant end of the gestational trophoblastic disease spectrum and includes the more common types, invasive mole (IM) and choriocarcinoma (CC) and the rare types, placental site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT). This is a case of a 42-year-old, G2P2 (2002) patient who complained of left lower quadrant pain and a 1 year history of amenorrhea. Urine pregnancy test done just prior to the surgery revealed positive result. Pre-operative diagnosis was abdominopelvic mass mass probably Sarcoma, ovarian new growth probably benign, right. Patient underwent exploratory laparotomy, adhesiolysis, bilateral internal iliac artery ligation, total hysterectomy with bilateral salpingo-oophorectomy, targeted biopsy, appendectomy, JP drain insertion under epidural anesthesia. Final histopathologic and immunohistochemical diagnosis is Epithelioid Trophoblastic Tumor. Differential diagnoses, diagnostics, and therapeutic options are presented, with focus on the description of sonographic features.

Morcelamento elétrico de tecido uterino: qual o risco potencial de disseminação de neoplasias malignas? / Electrical uterine tissue morbidity: what is the potential risk of malignant neoplasm spread?

O Food and Drug Administration (FDA) fez um alerta de segurança em relação ao risco potencial de disseminação de neoplasias malignas pelo uso do morcelador elétrico durante procedimentos minimamente invasivos. Esse artigo visa avaliar as evidências científicas sobre morcelamento uterino em relação ao risco de disseminação de câncer, sendo abordados a incidência de sarcoma uterino entre as mulheres previamente diagnosticadas com doença uterina benigna aparente, a possibilidade de avaliação pré-operatória desse risco, o posicionamento das principais sociedades médicas em relação à utilização do morcelador elétrico e as repercussões na prática clínica após as recomendações do FDA.(AU)

The US Food and Drug Administration (FDA) released a safety communication advising the potential risk of inadvertent spread of cancer cellsafter the use of electrical morcellator in minimally invasive procedures. This article aims to evaluate the scientific evidence regarding the risk of spread of cancer after uterine morcellation, the incidence of uterine sarcoma among women diagnosed with benign uterine disease, the possibility of preoperative assessment of this risk, the main medical societies positions statements regarding theuse of electric morcellation and the FDA recommendation`s impact on clinical practice. A review of the literature and a research of the main medical societies positions statements regarding the use of electric morcellation in minimally invasive surgeries were done.(AU)

Uterine low grade endometrial stromal sarcoma: a case report with review of literature.

Endometrial stromal sarcomas are rare uterine tumours usually seen in perimenopausal females. We report here a case of low grade endometrial stromal sarcoma in a 45 year female with a clinical diagnosis of fibroid uterus and final histological diagnosis of low grade endometrial stromal sarcoma.

FIGO Staging for Uterine Sarcomas: Can the Revised 2008 Staging System Predict Survival Outcome Better?

PURPOSE: The aim of this study was to compare survival of patients with uterine sarcomas using the 1988 and 2008 International Federation of Gynecologists and Obstetricians (FIGO) staging systems to determine if revised 2008 staging accurately predicts patient survival. MATERIALS AND METHODS: A total of 83 patients with leiomyosarcoma and endometrial stromal sarcoma treated at Yonsei University Health System between March of 1989 and November of 2009 were reviewed. The prognostic validity of both FIGO staging systems, as well as other factors was analyzed. RESULTS: Leiomyosarcoma and endometrial stromal sarcoma comprised 47.0% and 53.0% of this study population, respectively. Using the new staging system, 43 (67.2%) of 64 eligible patients were reclassified. Among those 64 patients, 45 (70.3%) patients with limited uterine corpus involvement were divided into stage IA (n=14) and IB (n=31). Univariate analysis demonstrated a significant difference between stages I and II and the other stages in both staging systems (p<0.001) with respect to progression-free survival and overall survival (OS). Age, menopausal status, tumor size, and cell type were significantly associated with OS (p=0.011, p=0.031, p=0.044, p=0.009, respectively). In multivariate analysis, revised FIGO stage greater than III was an independent poor prognostic factor with a hazard ratio of 9.06 [95% confidence interval (CI) 2.49-33.0, p=0.001]. CONCLUSION: The 2008 FIGO staging system is more valid than the previous FIGO staging system for uterine sarcomas with respect to its ability to distinguish early-stage patients from advanced-stage patients.

A Case of Metastasis to the Right Ventricle from Uterine Stromal Sarcoma / 日本心臓血管外科学会雑誌

We report a case of metastasis to the right ventricle from uterine stromal sarcoma. A 61-year-old woman was admitted to our hospital because of abdominal pain due to gallbladder stones. Preoperative transthoracic echocardiography showed a tumor in the right ventricle and tricuspid regurgitation. The tumor was multilocular and had grown in the right atrium over the tricuspid valve. We performed tumor resection and tricuspid valve plasty. Postoperative transthoracic echocardiography showed the tricuspid regurgitation had resolved.

Rabdomiosarcoma embrionario uterino: aspectos morfológicos e inmunohistoquímicos / Embryonal rhabdomyosarcoma of the corpus uterine: morphological and immunohistochemical characteristics

Los sarcomas uterinos son relativamente raros. El tipo histológico más frecuente es el leiomiosarcoma, seguido por el sarcoma del estroma endometrial. Los rabdomiosarcomas (RMS) son neoplasias malignas con diferenciación muscular esquelética. El rabdomiosarcoma embrionario (RMSE) tipo botrioide es el sarcoma más común de la infancia; con escasos reportes en adultos, afectando la región de cabeza y cuello, tracto genitourinario y extremidades más frecuentemente. En el tracto genitourinario, la vagina es la localización mas frecuente, comprometiendo en raras ocasiones el cuello o fondo uterino. Se presentan las características clínicas y morfológicas de un caso de RMSE uterino tipo botrioide diagnosticado en una paciente de 58 años en la Unidad de Anatomía Patológica del Hospital Hernán Henríquez Aravena de Temuco.

Uterine sarcomas are relatively rare. The most common histological type is leiomyosarcoma, followed by endometrial stromal sarcoma. The rhabdomyosarcoma (RMS) are malignant neoplasms with skeletal muscle differentiation. Embryonal rhabdomyosarcoma (RMSE) type botryoides is the most common in childhood, with few reports in adults, affecting the head and neck region, genitourinary tract and extremities more frequently. In the genitourinary tract, the vagina is the most common location, rarely involving cervix and fundus uterine. Clinical and morphological characteristics are presented of a case of uterine RMSE diagnosed in a 58 year-old woman in the Pathology Unit of the Hernán Henríquez Aravena Hospital in Temuco.

Contemporary clinical trials in uterine corpus malignancies / 대한산부인과학회지

Uterine cancer is the most common gynecologic malignancy in Western countries. In Korea, its incidence has been rising, and the increased incidence is associated with a rising death rate. The first two decades of endometrial cancer study by the GOG began with a prospective, surgicopathologic staging trial that was the cornerstone for development of all subsequent trials. The therapeutic strategies were tailored to disease extent - low risk, intermediate risk, and high risk groups of patients. There were great advances in the area of chemotherapeutic agents through a number of extensive phase II trials. New trials of these agents have been opened for the patients with advanced or recurrent uterine cancers.

Uterine sarcomas in Siriraj Hospital (1991-2005).

Objective: To study the incidence, clinical characteristics, treatments and outcomes in patients with uterine sarcomas at Siriraj Hospital. Methods: A medical record search of patients treated at Siriraj Hospital from January 1991 to December 2005 was performed for clinical characteristics and treatments. Survival curves were generated using Kaplan-Meier method. Results: Sixty uterine sarcomas were diagnosed during a 15-year period. The mean age was 49.3 years (range 27-74; SD 10.6). Abnormal bleeding was the most common presenting symptom (40%). Only 11.7% of the cases could be diagnosed preoperatively. Of 60 patients, 37 (61.6%) had leiomyosarcoma (LMS), 9 (15%) had malignant mixed mullerian tumor (MMMT), and 14 (23.3%) had endometrial stromal sarcoma (ESS). The distribution by FIGO staging was as follows: stage I: 47.5%, stage II: 15%, stage III: 17.5%, and stage IV: 20%. The treatment was mainly hysterectomy with adjuvant chemotherapy. The median follow-up time was 25 months. The five-year survival rate was 55.4%. Conclusion: The incidence of uterine sarcoma at Siriraj Hospital was 4.4% of uterine malignancies. The most common histologic type was leiomyosarcoma (61.6%). The common presenting symptoms were uterine bleeding and pelvic mass. In most cases, the treatment modality was surgery combined with chemotherapy. The overall 5-year survival rate of the studied group was 55.4%.

Uterine Sarcoma: Analysis of Prognostic variables in 40 cases / 대한산부인과학회지

OBJECTIVE: Uterine sarcomas are rare tumors of mesodermal origin and constitute 2-6% of uterine malignancies. They are the most malignant group of uterine tumors and present difficult problems with regard to diagnosis and treatment. The aim of this study is to investigate the clinicopathologic finding and outcome of patients with uterine sarcoma. METHODS: From Jan. 1996 to Dec. 2004, 40 patients with histologically proven uterine sarcomas at Gospel Hospital were evaluated for their clinical profile and survival retrospectively. RESULTS: The age of patients with uterine sarcoma ranged 28 to 71 years, and the mean age was 48.0 years. The common presenting symptoms were abnormal uterine bleeding, abdominal pain and lower abdominal palpable mass. The overall 5-year survival rate of uterine sarcoma was 48.9% and the mean survival time was 73.6 months. The overall 5-year survival rate of 24 patients less than 50 years was significantly better than that of 16 patients more than 50 years, 69.1% vs 24.6% (P=0.0139). When classified according to histologic type, there were 21 cases (52.5%) of leiomyosarcoma (LMS), 8 cases (20.0%) of endometrial stromal sarcoma (ESS), 10 cases (25.0%) of malignant mixed mullerian tumors (MMMT) and 1 case (2.5%) of liposarcoma. The overall 5-year survival rate of MMMT was significantly worse than that of LMS and ESS, 26.7% vs 49.0% and 100.0%, respectively (P=0.0423). Classifying according to the FIGO surgical staging criteria, we found the following distribution; stage I of 26 cases (66.7%), stage II of 4 cases (10.3%), stage III of 5 cases (12.8%) and stage IV of 4 cases (10.3%). The overall 5-year survival rate of stage I and II was significantly better than stage III and IV, 61.8% vs 11.1% (P=0.0011). The overall 5-year survival rate of 14 patients less than 10 mitotic figures per 10 high-power microscopic fields had a tendency to good prognosis than that of 13 patients more than 10 mitotic figures per 10 high-power microscopic fields, 83.9% vs 38.5% (P=0.0568). The overall 5-year survival rate of 6 patients less than 35 U/mL of CA-125 had a tendency to good prognosis than that of 12 patients more than 35 U/mL of CA-125, 83.3% vs 20.8% (P=0.0580). However, menstrual status and treatment modality were not significant prognostic factors. CONCLUSIONS: Uterine sarcoma are aggressive tumors with a poor prognosis. Age, histologic type, and stage were statistically significant prognostic factors for overall survival in uterine sarcomas.

Clinical Analysis of 23 Cases of Endometrial Stromal Sarcoma / 대한산부인과학회지

OBJECTIVE: The aim of this retrospective study was to evaluate the survival, recurrence and prognostic factors of endometrial stromal sarcoma (ESS). METHODS: Twenty three patients who had diagnosed with ESS and managed between June 1989 and December 2004 were included. Clinicopathologic characteristics were evaluated including age, parity, menopause, stage, grade, type of surgery, adjuvant therapy, overall survival, disease free survival. RESULTS: The mean age of the group was 44 years. Eighty two percents of patients were premenopause and the most common symptom was vaginal bleeding. Thirteen patients were in stage I and four patients were in stage II and III. Six patients were not able to determine the stage. Eighteen patients were low grade ESS and five patients were high grade. The median overall survival was 179 months and the median disease free survival was 134 months. Disease had recurred in 6 patients and 2 patients died of the disease. Higher tumor grade was associated with lower overall survival (P=0.047) and disease free survival (P=0.035). CONCLUSION: The prognostic factor of ESS is tumor grade.

Multimodality therapy in uterine sarcoma / 대한산부인과학회지

Uterine sarcomas have been known for the poor prognosis and high mortality rate. In addition, since uterine sarcomas are a rare group and heterogenous group of tumors with many pathologic subtypes, it is difficult to define optimal management in patients with uterine sarcoma. Total abdominal hysterectomy and bilateral salpingo-oophorectomy have been considered as the cornerstone of treatment for patients with uterine sarcoma, although the efficacy of adnexectomy or lymphadenectomy is still controversial in several subtypes. Also, the role of adjuvant therapy including chemotherapy or radiotherapy remains unclear, although many studies support a possible benefit in terms of progression-free interval or pelvic control. In advanced uterine sarcoma, through the clinical trial, doxorubicin for leimyosarcoma and ifosfamide alone or with combination cisplatin for mixed mesodermal sarcoma represent reasonable therapeutic options. Recently gemcitabine combined with docetaxel has shown promising results for patients with leiomyosarcoma and there are currently some ongoing randomized clinical trials through the Gynecology Oncology Group. In conclusions, multi-institution, randomized clinical trials by histological stratification or accounting to the difference in natural history are more needed actively in patients with uterine sarcoma.

Multimodality therapy in uterine sarcoma / 대한산부인과학회지

Uterine sarcomas have been known for the poor prognosis and high mortality rate. In addition, since uterine sarcomas are a rare group and heterogenous group of tumors with many pathologic subtypes, it is difficult to define optimal management in patients with uterine sarcoma. Total abdominal hysterectomy and bilateral salpingo-oophorectomy have been considered as the cornerstone of treatment for patients with uterine sarcoma, although the efficacy of adnexectomy or lymphadenectomy is still controversial in several subtypes. Also, the role of adjuvant therapy including chemotherapy or radiotherapy remains unclear, although many studies support a possible benefit in terms of progression-free interval or pelvic control. In advanced uterine sarcoma, through the clinical trial, doxorubicin for leimyosarcoma and ifosfamide alone or with combination cisplatin for mixed mesodermal sarcoma represent reasonable therapeutic options. Recently gemcitabine combined with docetaxel has shown promising results for patients with leiomyosarcoma and there are currently some ongoing randomized clinical trials through the Gynecology Oncology Group. In conclusions, multi-institution, randomized clinical trials by histological stratification or accounting to the difference in natural history are more needed actively in patients with uterine sarcoma.

Sonographic Findings of Uterine Endometrial Stromal Sarcoma

OBJECTIVE: The study was performed to present the sonographic findings of uterine endometrial stromal sarcoma (ESS). MATERIALS AND METHODS: We conducted a retrospective review of sonographic findings of 10 cases that were diagnosed as uterine ESS. The patients' ages ranged from 25 to 51 years (mean age: 36.1 years). The reviews focused on the location, margin, size, number and echotexture of the lesions. Hysterectomy (n = 9) and myomectomy (n = 1) were performed and a pathologic diagnosis was obtained in all cases. RESULTS: The masses were located in the uterine wall (n = 6), or they presented as a polypoid mass protruding into the endometrial cavity from the myometrium (n = 3) or as a central cavity mass (n = 1). The lesion margins were smooth (n = 5), ill defined (n = 2), or smooth with partially nodular extensions (n = 3). The maximal mass length was 38 mm to 160 mm with a mean mass length of 83.5 mm. There were single lesions in eight cases and multiple lesions in two cases. The lesion echotextures were hypoechoic solid (n = 3), heterogeneously intermediate echoic (n = 5), diffuse myometrial thickening with heterogeneous echogenicity (n = 1) and septated cystic (n = 1). CONCLUSION: Endometrial stromal sarcoma presents with four patterns of its sonographic appearance; a polypoid mass with nodular myometrial extension, an intramural mass with an ill defined margin and heterogeneous echogenicity, an ill defined large central cavity mass or, diffuse myometrial thickening.