Julie Sandruck1, Pedro Escobar, John Lurain, David Fishman. 1. Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, Prentice Women's Hospital, Northwestern University, Chicago, IL 60611, USA.
Abstract
BACKGROUND: Leiomyosarcoma (LMS) of the uterus is a rare neoplasm with an aggressive growth pattern. Although the majority of uterine LMS is diagnosed with disease confined to the uterus, the rate of recurrent disease is high. The most common sites of recurrent disease are lung, liver, and peritoneal cavity. The rate of lymph node involvement with disease confined to the uterus is less than 3%. Metastases to brain and skull are rare. CASE: A 39-year-old woman underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and paraaortic lymph node biopsies, omentectomy, and appendectomy for Grade 2 FIGO Stage 1 uterine leiomyosarcoma. She remained disease-free for 2 years until she presented with metastases to the sphenoid sinus. She underwent incomplete resection of the recurrence and was treated postoperatively with adjuvant MAID chemotherapy with poor response. She then underwent a second resection at an outside institution. Five months later, she was treated with radiation therapy to the base of the skull with no response. She expired from the disease 13 months after the diagnosis of the recurrence. CONCLUSION: The prognosis for patients who recur is dismal. The treatment options are limited. Surgical management should be considered as uterine LMS has a low response to chemotherapy and surgical resection of LMS to sites such as lung and abdomen has been suggested to offer a benefit. Radiation therapy may provide palliative benefit in the setting of metastatic disease.
BACKGROUND:Leiomyosarcoma (LMS) of the uterus is a rare neoplasm with an aggressive growth pattern. Although the majority of uterine LMS is diagnosed with disease confined to the uterus, the rate of recurrent disease is high. The most common sites of recurrent disease are lung, liver, and peritoneal cavity. The rate of lymph node involvement with disease confined to the uterus is less than 3%. Metastases to brain and skull are rare. CASE: A 39-year-old woman underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and paraaortic lymph node biopsies, omentectomy, and appendectomy for Grade 2 FIGO Stage 1 uterine leiomyosarcoma. She remained disease-free for 2 years until she presented with metastases to the sphenoid sinus. She underwent incomplete resection of the recurrence and was treated postoperatively with adjuvant MAID chemotherapy with poor response. She then underwent a second resection at an outside institution. Five months later, she was treated with radiation therapy to the base of the skull with no response. She expired from the disease 13 months after the diagnosis of the recurrence. CONCLUSION: The prognosis for patients who recur is dismal. The treatment options are limited. Surgical management should be considered as uterine LMS has a low response to chemotherapy and surgical resection of LMS to sites such as lung and abdomen has been suggested to offer a benefit. Radiation therapy may provide palliative benefit in the setting of metastatic disease.