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Inicio Revista Española de Patología Low-grade extrauterine endometrial stromal sarcoma of the peritoneum: A case rep...
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Vol. 54. Issue 3.
Pages 201-205 (July - September 2021)
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Vol. 54. Issue 3.
Pages 201-205 (July - September 2021)
Brief report
Low-grade extrauterine endometrial stromal sarcoma of the peritoneum: A case report and literature review
Sarcoma del estroma endometrial de bajo grado extrauterino del peritoneo: presentación de un caso y revisión de la literatura
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Iñigo Gorostiagaa,
Corresponding author
igorostiagaltuna@gmail.com

Corresponding author.
, Alvaro Perez-Rodrigueza, María Carmen Gómez-Mateob, María Cuadra-Cestafec, Amaia Sagastaa
a Bioaraba Research Health Institute, Vitoria-Gasteiz, Spain; Osakidetza Basque Health Service, Araba University Hospital, Pathology Department, Alava, Vitoria-Gasteiz, Spain
b Pathology Department, Miguel Servet University Hospital, Zaragoza, Spain
c Bioaraba Research Health Institute, Vitoria-Gasteiz, Spain; Osakidetza Basque Health Service, Araba University Hospital, Gynecology Department, Vitoria-Gasteiz, Spain
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Abstract

Endometrial stromal sarcoma (ESS) is an uncommon mesenchymal tumor that accounts for less than 1% of all primary uterine malignancies and extrauterine endometrial stromal sarcoma (EESS) is even rarer.

We report the case of a 75-year-old woman with an abdominal tumor and multiple peritoneal implants. Histological analysis of the surgical specimens showed bland cellularity resembling normal endometrial stroma. The diagnosis of a low-grade EESS was confirmed by immunophenotypic findings and demonstration of JAZF1 translocation. After extensive sampling, no evidence of endometriosis was found.

Our case showed atypical aggressive behavior and we discuss the possible influence of the high mitotic count (8/10 HPFs) in some areas of the tumor, the multifocality of the abdominal implants and the postmenopausal status of the patient.

The unusual clinical presentation and extrauterine location of such a rare tumor were challenging implying a wide range of differential diagnosis. The correlation of morphological, immunohistochemical and molecular findings was necessary to arrive at the correct diagnosis.

Keywords:
Endometrial stromal sarcoma
Extrauterine endometrial stromal sarcoma
Low grade
JAZF1 (7p15) translocation
Resumen

El sarcoma del estroma endometrial (SEE) es una neoplasia mesenquimal que representa <1% de todas las malignidades primarias uterinas. Su localización extrauterina (SEEE) es aún más infrecuente.

Reportamos el caso de una mujer de 75 años con una masa abdominal y múltiples implantes peritoneales. El estudio histológico de las muestras quirúrgicas mostró una celularidad similar al estroma endometrial usual. El diagnóstico de SEEE de bajo grado fue apoyado por el inmunofenotipo y la presencia de la translocación JAZF1 (7p15). Tras un muestreo exhaustivo, no se hallaron signos de endometriosis.

Nuestro caso mostró un comportamiento agresivo inusual. Discutimos la posible influencia del alto contaje mitótico (8/10HPF) en las áreas más proliferativas, la multifocalidad de los implantes abdominales y la edad posmenopaúsica de la paciente a este respecto.

La localización extrauterina y la presentación clínica son inesperadas, con un amplio diagnóstico diferencial. La correlación morfológica, inmunofenotípica y molecular son claves para un diagnóstico correcto.

Palabras clave:
Sarcoma del estroma endometrial
Sarcoma del estroma endometrial extrauterino
Bajo grado
Translocación JAZF1 (7p15)

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