RESUMEN
Liposarcoma (LPS) is a rare malignancy of adipocytic differentiation. According to World Health Organization classification, LPS comprises of four principle subtypes Atypical lipomatous tumor/Well-differentiated liposarcoma (ATL/WDLPS), Dedifferentiated liposarcoma (WDLPS), Myxoid liposarcoma (MLPS), and Pleomorphic liposarcoma (PLPS). Each subtype can develop at any location and shows distinct clinical behavior and treatment sensitivity. ATL/ WDLPS subtype has a higher incidence rate, low recurrence, and is insensitive to radiation and chemotherapy. DDLPS is the focal progression of WDLPS, which is aggressive and highly metastasizing. MLPS is sensitive to radiation and chemotherapy, with a higher recurrence rate and metastasis. PLPS subtype is highly metastasizing, has a poor prognosis, and exhibiting higher recurrence rate. Initial histological analysis provides information for the characterization of LPS subtypes', further molecular and genetic analysis provides certain subtype specifications, such as gene amplifications and gene fusions. Such molecular genetic alterations will be useful as therapeutic targets in various cancers, including the LPS subtypes. A wide range of novel therapeutic agents based on genetic alterations that aim to target LPS subtypes specifically are under investigation. This review summarizes the LPS subtype classification, their molecular genetic characteristics, and the implications of genetic alterations in therapeutics.
Asunto(s)
Liposarcoma , Humanos , Liposarcoma/genética , Liposarcoma/terapia , Liposarcoma/patología , Liposarcoma/diagnóstico , Liposarcoma/clasificaciónRESUMEN
Primary sacral tumors are uncommon and sacrectomy is a complex surgical procedure with substantial risk of morbidity. We conducted a retrospective study of patients who had undergone sacral resections for primary sacral tumors between 2010 and 2020. Ten sacral resections including five type 1 sacrectomy (S1 resected), four type 2 (S1 spared), and one type 3 (S3 spared) were performed during the above period. The median age was 47 years and the most common histologic diagnosis was chordoma (50%). The median operating time was 705 min (range 180-960 min) with a median blood loss of 3400 ml (range 500-7000 ml) and a median duration of hospital stay of 13.5 days (range 7-68 days). All patients who underwent type 1 sacrectomy experienced major complications (Clavien-Dindo grade 3 or above) including one death in the immediate perioperative period. Microscopically positive margins (R1) were noted in two patients (20%). All patients with type 1 sacrectomy had R0 resection. The median follow-up period was 31 months. The median MSTS score was 12 (range 4-27). A total of seven patients (70%) had a minimum follow-up of 2 years without disease recurrence. Sacral resection for primary tumors of the sacrum with oncologically safe margins is feasible. Although associated with substantial perioperative morbidity, a detailed preoperative planning and execution of the surgery by a team of orthopedic oncosurgeon, surgical oncologist, and plastic surgeon offer a hope for survival in patients with acceptable functional outcome.