Soft Tissue Sarcomas with Special Reference to Molecular Aberration, Chemotherapy, and Recent Advances: A Review Article

Soft tissue sarcomas (STS) are a diverse group of rare solid tumors of mesenchymal cell origin with distinct clinical and pathological features. They account for less than 1% of all adult malignancies and 15% of pediatric neoplasms. They include over hundreds of different histological subtypes. Many of these subtypes can occur at any age and are not confined to a specific site. Each subtype displays variable clinical behavior. Low incidence, variable presentation, behavior, and long-term outcomes further make it challenging to treat. There are multiple ongoing trials that focus on the anatomic site and histologic subtype to tailor the treatment. Further rarity of each histotype is a major barrier to recruit patients to randomized controlled trials. A multidisciplinary approach is mandatory in all cases of soft tissue sarcomas. The purpose of this review is to thoroughly understand the existing literature on history, incidence, epidemiology, etiology, histology, pathogenesis, diagnostic modalities, prognosis, management, and post treatment surveillance of STS. Uterine sarcomas, gastrointestinal stromal tumors (GIST), and pediatric sarcomas are not included here. It briefly highlights various molecular aberrations, changes in staging as per the American Joint Committee on Cancer (AJCC) 8, drugs that are used off-label in specific subtypes of sarcoma along with the recent advances. The classification of STS is undergoing continuous evolution. A wide variety of subtypes can only be diagnosed accurately with sophisticated molecular diagnostic tests and with the involvement of expert geneticists and pathologists to interpret it. There is no clarity on tailoring the treatment of STS to date. There is always a question on how best we can incorporate chemotherapy and radiotherapy along with surgery as a part of multimodality treatment. The heterogeneity of STS has hindered the development of robust, evidence-based treatment strategies, and our therapeutic approach is neither histology-specific nor widely standardized. Increased knowledge about sarcoma biology could help to discover new and more effective treatment strategies and help overcome the therapeutic challenge imposed by this deadly disease. Continued collaboration among various sarcoma centers globally will be of prime importance to optimize STS management. This will allow studies to be both sufficiently large and reasonably focused to generate evidence that is clinically meaningful in specific STS patient populations.