ABSTRACT
Myometrial smooth muscle neoplasms are the most common gynecologic tumors with a prevalence of 70-80% at age 50. Among women undergoing hysterectomy or myomectomy for a suspected diagnosis of leiomyoma, 0.01% receive a diagnosis of STUMP. Clinically, the average age of presentation is between 41-48 years. Tumors ranging from 3 to 30 cm have been described. Signs and symptoms are similar to leiomyomas, such as abnormal uterine bleeding, anemia, dysmenorrhea, pelvic pain, pelvic mass, infertility or other types of pain secondary to compression of adjacent organs. Due to the limited literature available, there is no definite management consensus, and treatment and follow-up options are limited to observational studies. The standard treatment is total hysterectomy with or without bilateral salpingo-oophorectomy; if fertility has already been completed, there is no role for adjuvant hormonal therapy or chemotherapy. The overall 5-year survival is 92-100%. A case of a 31-year-old woman with STUMP is presented.
Subject(s)
Female , Adult , Uterine Neoplasms , LeiomyosarcomaABSTRACT
El PEComa es un tumor de células epitelioides, perivascular, de origen mesenquimatoso. Estos tumores corresponden a un grupo raro de neoplasias. Hay cerca de 100 casos reportados de los que solo el 30% son de origen ginecológico y el cuerpo uterino es el sitio más frecuente. Debido a su baja frecuencia de aparición no hay una conducta médica y tratamiento establecidos, por lo cual es un reto el manejo de esta entidad. La presentación en población pediátrica es extremadamente rara, con datos limitados de su frecuencia de presentación en este grupo etario. Presentamos el caso de una paciente de 13 años que consultó por dolor pélvico; se realizaron imágenes diagnósticas que documentaron una masa paraanexial derecha sólida; es llevada a cirugía obteniendo como hallazgo un tumor dependiente del cuerpo uterino cuya histología correspondía a un PEComa con características de comportamiento maligno
PEComa is a perivascular epithelioid cell tumor of mesenchymal origin. These tumors correspond to a rare group of neoplasms. About 100 cases have been reported, of which only 30% are of gynecological origin, and the uterine body is the most frequent site. Due to its low frequency of appearance, there is no established medical conduct and treatment, which is why the management of this entity is a challenge. Presentation in the pediatric population is extremely rare, with limited data on its frequency in this age group. We present the case of a 13-year-old patient who consulted for pelvic pain. Diagnostic images were performed that documented a solid right para-adnexal mass. She was taken to surgery, which evidenced a tumor dependent on the uterine body with histology corresponding to a PEComa with characteristics of malignant behavior