Kobayashi Kenya, Yoshimoto Seiichi, Yamamura Koji, Kitayama Megumi, Kawakita Daisuke, Nibu Ken-Ichi, Kondo Kenji, Saito Yuki
Department of Otolaryngology, Head and Neck Surgery, The University of Tokyo, Tokyo, Japan.
The Japan Society for Head and Neck Cancer, Tokyo, Japan.
Laryngoscope. 2025 Jul;135(7):2403-2410. doi: 10.1002/lary.32027. Epub 2025 Jan 27.
The goal of this study was to better understand the epidemiology, clinical characteristics, and treatment outcomes of head and neck sarcomas using real-world data from Japan.
Using the Japanese Head and Neck Cancer Registry, we identified 438 patients who were pathologically diagnosed with head and neck sarcoma between 2011 and 2020. We compared epidemiological, clinical, and prognostic data for the different histological types of sarcoma.
The top five most common histopathological types were rhabdomyosarcoma, osteosarcoma, chondrosarcoma, leiomyosarcoma, and liposarcoma. The most common primary sites were the nasal cavity and paranasal sinuses. The overall cohort had clinical, pathologic, and occult lymph node positivity rate (OLR) of 18%, 15%, and 2%, respectively. Clinical lymph node positivity was highest in rhabdomyosarcoma (46%), followed by angiosarcoma (26%), with leiomyosarcoma, chondrosarcoma, and liposarcoma accounting for less than 10%. The 3-year overall survival rate was 74.8% among the 147 patients with available prognostic information. The histological type with the lowest 3-year overall survival was undifferentiated pleomorphic sarcoma (54%), followed by leiomyosarcoma (65%), and angiosarcoma (69%). In six of seven histological types, the 3-year locoregional-free survival rate was less than 70%.
Sarcomas of the head and neck most commonly occur in the nasal cavity and paranasal sinuses. The prevalent histological type depended on the primary site. Rhabdomyosarcoma and angiosarcoma are histological types with a high risk of cervical metastasis. In the head and neck region, there are numerous histological types many of which lack local control.
3 Laryngoscope, 135:2403-2410, 2025.
本研究的目的是利用来自日本的真实世界数据，更好地了解头颈部肉瘤的流行病学、临床特征和治疗结果。
利用日本头颈部癌症登记处的数据，我们确定了2011年至2020年间438例经病理诊断为头颈部肉瘤的患者。我们比较了不同组织学类型肉瘤的流行病学、临床和预后数据。
最常见的五种组织病理学类型为横纹肌肉瘤、骨肉瘤、软骨肉瘤、平滑肌肉瘤和脂肪肉瘤。最常见的原发部位是鼻腔和鼻窦。整个队列的临床、病理和隐匿性淋巴结阳性率（OLR）分别为18%、15%和2%。横纹肌肉瘤的临床淋巴结阳性率最高（46%），其次是血管肉瘤（26%），平滑肌肉瘤、软骨肉瘤和脂肪肉瘤的临床淋巴结阳性率低于10%。在147例有可用预后信息的患者中，3年总生存率为74.8%。3年总生存率最低的组织学类型是未分化多形性肉瘤（54%），其次是平滑肌肉瘤（65%）和血管肉瘤（69%）。在七种组织学类型中的六种中，3年局部区域无复发生存率低于70%。
头颈部肉瘤最常见于鼻腔和鼻窦。流行的组织学类型取决于原发部位。横纹肌肉瘤和血管肉瘤是颈部转移风险较高的组织学类型。在头颈部区域，有许多组织学类型，其中许多缺乏局部控制。
3《喉镜》，135:2403 - 2410，2025年。
