Long term results in adenoidcystic carcinoma
Long term results in adenoidcystic carcinoma
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DOI：
10.1055/s-2002-23108
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发表时间：
2002-02-01
影响因子：
1
通讯作者：
Lenarz, T
中科院分区：
文献类型：
--
作者：
Issing, PR;Hemmanouil, I;Lenarz, T
背景资料：腺样囊性癌（adenoidcystic carcinoma，ACC）由于其初始症状不明显、局部浸润性强、神经周围扩散，给诊断和治疗带来了特殊的挑战。患者：回顾性分析了54例患者的临床资料，平均年龄55.5（24 - 77）岁。大涎腺受累26例。腺样囊性癌的分布依次为：腮腺18例，颌下腺8例，口腔10例，鼻窦11例，鼻咽4例，喉3例。最常见的症状是肿瘤相关的肿胀和疼痛，在最终诊断后持续数月。面神经麻痹4例。组织学检查显示管状亚型4例，筛状亚型28例，实性亚型12例。在10个标本中没有明确的分化是可能的。16例患者颅底受侵。除1例患者外，所有54例患者均接受了手术治疗。术后25例加用放疗，6例加用化疗。然而，ACC在60%的患者中复发。13例患者在平均3.3年的潜伏期后观察到淋巴结转移，但18例患者在5.8年后显著晚于肺转移。2年生存率84.38%，5年生存率75.90%，10年生存率50.49%，20年生存率20.11%。男性、颅底浸润和神经周围和血管周围扩散的组织学证据被证明是预后不良的统计学显著因素。由于不常见的生物学行为，一方面是缓慢生长，另一方面是侵袭性局部侵袭，ACC可以被认为是临床医生的一种具有挑战性的恶性疾病，其充分的治疗不允许任何治疗。标准化制度。即使在临床上没有症状的一段时间后，复发的趋势也使得终身随访成为强制性的。
Background: Due to the discreet initial symptoms and the locally aggressive infiltration with perineural spread the adenoidcystic carcinoma (ACC) presents a special diagnostical and therapeutical challenge.Patients: in a retrospective study the forms of 54 patients were analysed, whose average age was 55.5 (24 - 77) years.Results: The sex ratio showed a slight female preponderance with 57% to 43%. The major salivary glands were affected in 26 cases. The exact distribution of the ACC was: parotid gland (n = 18), submandibular gland (n = 8), oral cavity (n = 10), paranasal sinuses (n = 11), nasopharynx (n = 4) and larynx (n = 3). The most common symptoms were a tumor-related swelling and pain which persisted for a duration of several months after final diagnosis could be established. Facial palsy was observed in 4 patients. Histological examination revealed a tubular subtype in 4 cases, in 28 cases a cribriforme and in 12 cases a solid subtype. No definitive differentiation was possible in 10 specimens. The skull base was infiltrated in 16 patients. Except one patient all 54 underwent surgical therapy. Postoperative radiotherapy was additionally given in 25 cases which was combined with a chemotherapy in 6 patients. Nevertheless ACC recurred in 60% of our patients. Lymphnode metastases were observed in 13 patients after a latency of 3.3 years in average, but predominantly pulmonary metastases as distant spread developed in 18 patients after 5.8 years significantly later. The overall survival ratewas 84.38% after 2 years, 75.90% after 5 years, 50.49% after 10 years and 20.11% after 20 years. Male sex, infiltration of the skull base and histological evidence of perineural and perivascular spread proved to be statistically significant factors for an unfavourable prognosis.Conclusions: Due to the uncommon biological behaviour with a slow growth on the one hand side and an aggressive local invasion on the other hand side the ACC can be regarded as a challenging malignant disease for the clinician whose adequate therapy does not allow any standardized regime. The tendency for recurrence even after a period clinically free of symptoms makes a life long follow-up mandatory.