Sharma Nivedita, George Nebu Abraham, Singh Rajesh, Iype Elizabeth Mathew, Varghese Bipin T, Thomas Shaji
Surgical Oncology Division, Regional Cancer Centre, Trivandrum, 695011 India.
Indian J Surg Oncol. 2018 Jun;9(2):187-191. doi: 10.1007/s13193-018-0755-5. Epub 2018 Apr 20.
Head and neck soft tissue sarcoma (HNSTS) is a rare neoplasm accounting for 1% of all head and neck tumours. Because of rarity and varied biological behaviour among various subtypes, knowledge about these tumours is limited. This study aimed at analysing clinicopathological, recurrence and survival pattern of surgically treated HNSTS. Case records of 28 patients of HNSTS who underwent surgery at the Regional Cancer Centre (RCC), Trivandrum (India) between 2002 and 2012 were analysed retrospectively for demographic profile, clinical features, treatment given, recurrence pattern and outcome. The median age of patients was 37 years (range, 3-79) with male:female ratio of 3:2. Majority of patients presented with painless lump in the neck as the most common subsite affected followed by scalp and face. One patient had nodal disease, while none had distant metastasis at presentation. The most frequent histological subtypes were synovial sarcoma and fibrosarcoma followed by malignant fibrous histiocytoma, angiosarcoma and rhabdomyosarcoma. Majority (78.5%) of patients received adjuvant therapy in the form of radiation, chemotherapy or chemo-radiation. After mean follow-up of 49 months, four patients had died, and six developed local recurrence and four distant metastasis. The overall 5-year survival was 82.7% while 5-year disease-free survival was 55.3%. HNSTS is a rare entity that requires multimodality treatment to achieve optimum locoregional control and survival.
头颈部软组织肉瘤(HNSTS)是一种罕见的肿瘤,占所有头颈部肿瘤的1%。由于其罕见性以及各亚型之间生物学行为的差异,关于这些肿瘤的知识有限。本研究旨在分析手术治疗的HNSTS的临床病理、复发及生存模式。回顾性分析了2002年至2012年间在印度特里凡得琅地区癌症中心(RCC)接受手术的28例HNSTS患者的病例记录,以了解其人口统计学特征、临床特征、所接受的治疗、复发模式及预后。患者的中位年龄为37岁(范围3 - 79岁),男女比例为3:2。大多数患者以颈部无痛性肿块为最常见的受累部位,其次是头皮和面部。1例患者有淋巴结疾病,而就诊时无远处转移。最常见的组织学亚型是滑膜肉瘤和纤维肉瘤,其次是恶性纤维组织细胞瘤、血管肉瘤和横纹肌肉瘤。大多数(78.5%)患者接受了放疗、化疗或放化疗形式的辅助治疗。平均随访49个月后,4例患者死亡,6例出现局部复发,4例出现远处转移。总体5年生存率为82.7%,而5年无病生存率为55.3%。HNSTS是一种罕见的疾病,需要多模式治疗以实现最佳的局部区域控制和生存。