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.

Role of 18F-FDG PET/CT in restaging and follow-up of patients with uterine sarcomas. / 18F-FDG PET/TC en reestadificación y seguimiento de pacientes con sarcomas uterinos.

PURPOSE: Uterine sarcomas are rare tumors with poor prognosis due to the high recurrence rates. The current role of 18F-FDG PET/CT in the post-therapy surveillance is not established yet. MATERIALS AND METHODS: Forty-one women with uterine sarcoma underwent 73 18F-FDG PET/CT for restaging in suspected recurrence or during follow-up in asymptomatic patients. Histopathology results and/or clinical/imaging follow-up for at least 12 months were considered the reference standard. The diagnostic accuracy and clinical impact of 18F-FDG PET/CT was calculated. RESULTS: Thirty-three 18F-FDG PET/CT were positive, while the remaining 40 studies were negative. The overall sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy of 18F-FDG PET/CT were 88%, 98%, 97%, 91% and 93%, respectively. Considering patients with clinical or radiological suspicion of recurrence (n=47) and those during follow-up (n=26), sensitivity, specificity, PPV, NPV and accuracy of 18F-FDG PET/CT were 89%, 100%, 100%, 86% and 94%, and 80%, 95%, 80%, 95% and 92%, respectively. 18F-FDG PET/CT had a positive clinical impact in 9/73 (12%) studies and changed the clinical management in 8/41 (20%) patients. CONCLUSIONS: 18F-FDG PET/CT seems to be an accurate method for detection and localization of local and distant recurrence in patients with uterine sarcoma with good sensitivity and specificity and significant impact on clinical decision making.

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)

[Mullerian adenosarcoma of the cervix: case report with conservative management]. / Adenosarcoma mulleriano del cérvix: reporte de caso con manejo conservador.

BACKGROUND: Mullerian adenosarcoma is a rare gynecological malignancy with a low malignant potential, with biphasic growth, consisting of a benign epithelial element and a malignant mesenchymal element. It occurs in all ages predominating in postmenopausal women. Cervical localization of Mullerian adenosarcomas is rare; however, it is associated with a presentation in young women. The diagnosis is made by anatomopathological study of the lesion and immunohistochemistry. The prognosis is generally good although the recurrence rate is high. CLINICAL CASE: We present the case of a 27-year-old patient who attended a gynecological consultation with bleeding and transvaginal flow. During the gynecological examination, a polypoid lesion originating in the cervix was identified, which was removed by torsion and was diagnosed as Mullerian cervical adenosarcoma. Subsequently, a cervical cone was performed because the patient refused hysterectomy. CONCLUSIONS: Mullerian cervical adenosarcoma is a rare neoplasm with a recurrence rate that can reach up to 50% of cases, so close follow-up is necessary. A local excision can be considered in patients without poor prognosis factors and who wish to preserve their fertility.

ANTECEDENTES: El adenosarcoma mulleriano (AM) es una neoplasia ginecológica rara, de bajo potencial maligno, con crecimiento bifásico, constituida por un elemento epitelial benigno y otro mesenquimatoso maligno. Se presenta en todas las edades, pero predomina en mujeres posmenopáusicas. La localización cervical de los AM es poco frecuente; sin embargo, se asocia a una presentación en mujeres jóvenes. El diagnóstico se realiza mediante estudio anatomopatológico de la lesión e inmunohistoquímica. El pronóstico es generalmente bueno, aunque la tasa de recidiva es alta. CASO CLÍNICO: Presentamos el caso de una paciente de 27 años que acudió a consulta ginecológica con sangrado y flujo transvaginal. En la exploración ginecológica se identificó una lesión polipoide originada en el cérvix, la cual se extirpó por torsión y fue diagnosticada como AM cervical. Posteriormente se realizó conización cervical debido a que la paciente rechazó la histerectomía. CONCLUSIÓN: El AM cervical es una neoplasia poco frecuente que tiene una tasa de recidiva que puede llegar hasta al 50% de los casos, por lo que es necesario un seguimiento estrecho. La escisión local puede ser considerada en pacientes sin factores de mal pronóstico y que deseen conservar su fertilidad.

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)

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.