Pugliese G N, Saetti R, Furlanetto A
Divisione Otorinolaringoiatria, Ospedale Civile di Belluno.
Acta Otorhinolaryngol Ital. 1992 Jul-Aug;12(4):389-97.
Synovial sarcoma is a clinically and morphologically well defined entity that has been described extensively in Literature. It occurs primarily in the para-articular regions, usually in close association with tendon sheaths, bursae and joint capsules. On rare occasions it is also encountered in areas without any apparent relationship to synovial structures, as in the parapharyngeal region or the abdominal wall. It is considered the fourth most common type of sarcoma (7-10%) after malignant fibrous histiocytoma, liposarcoma and rhabdomyosarcoma. There are three histological variants: the classical biphasic, the monophasic fibrous type and the monophasic epithelial type (the biphasic and monophasic fibrous type are equally common). Clinical sign complaints are subtle and at times noted 20 years before diagnosis. The course of the disease is slow and insidious. The most typical presentation is that of a palpable deep-seated swelling or mass associated with pain or tenderness. Patients with synovial sarcoma in the head and neck (10%) tend to have difficulties in swallowing and breathing and not infrequently have alteration or loss of voice. Head and neck synovial sarcoma seem to originate from the paravertebral connective tissue spaces and manifest themselves as solitary retropharyngeal or parapharyngeal masses near the forking of the carotid. Additional cases in this general area have been reported in the soft palate, tongue, maxillofacial region, mandible corner, sternoclavicular region, scapular region and the cervical oesophagus. As in other types of sarcoma, the principal sites of metastases are the lung, but many make their appearance many years after the initial diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)
滑膜肉瘤是一种在临床和形态学上定义明确的实体瘤,在文献中已有广泛描述。它主要发生在关节旁区域,通常与腱鞘、滑囊和关节囊密切相关。极少数情况下,它也出现在与滑膜结构无明显关联的部位,如咽旁区域或腹壁。它被认为是继恶性纤维组织细胞瘤、脂肪肉瘤和横纹肌肉瘤之后第四常见的肉瘤类型(占7 - 10%)。有三种组织学变体:经典的双相型、单相纤维型和单相上皮型(双相型和单相纤维型同样常见)。临床症状不明显,有时在诊断前20年才被注意到。疾病进展缓慢且隐匿。最典型的表现是可触及的深部肿胀或肿块,伴有疼痛或压痛。头颈部滑膜肉瘤患者(占10%)往往有吞咽和呼吸困难,且经常出现声音改变或丧失。头颈部滑膜肉瘤似乎起源于椎旁结缔组织间隙,表现为颈动脉分叉处附近的孤立性咽后或咽旁肿块。该区域其他部位的病例还报道于软腭、舌、颌面区域、下颌角、胸锁区域、肩胛区域和颈段食管。与其他类型的肉瘤一样,转移的主要部位是肺,但许多转移灶在初次诊断多年后才出现。(摘要截断于250字)