Capodiferro S, Scully C, Macaita M G, Lo Muzio L, Favia G, Mairoano E
Department of Dental Sciences and Surgery, University of Bari - piazza Giulio Cesare, 11, 70124 Bari, Italy.
Oral Dis. 2005 Mar;11(2):109-12. doi: 10.1111/j.1601-0825.2004.01060.x.
Malignant epithelial tumours arising in the jaws are very rare. Adenoid cystic carcinoma (ACC) represents approximately 7.5% of all carcinomas and only a few cases of intraosseous (central) ACC have been reported in the literature.
The salient clinico-pathological features of a case of ACC, bilaterally occurring in the mandible of a young caucasian woman who also had lung metastases are reported to appropriately characterize such unusual lesions and discriminate them from other tumours that more commonly affect the mandible.
The patient presented with a painful swelling of the right retro-molar area and paraesthesia of the ipsilateral lower lip and radiological investigations disclosed bilateral radiolucent lesions of the mandible with unequivocal signs of malignancy but without intra-lesional calcifications or association with teeth roots or cystic component. Conventional histological examination disclosed typical ACC with solid and cribriform growth patterns and extensive infiltration of the adjacent tissues.
The diagnosis of intraosseous malignant salivary gland type neoplasms is very difficult in view of their rarity and lack of specific signs and mainly achieved after histological examination and complete clinico-radiological work up. As surgical treatment of the patient was not indicated, due to extensive neoplastic disease, the patient is being controlled with multimodal treatment, including chemo- and radiotherapy and is alive with persistent disease 3 years after the original diagnosis.
颌骨发生的恶性上皮性肿瘤非常罕见。腺样囊性癌(ACC)约占所有癌症的7.5%,而文献中仅报道了少数几例骨内(中央型)ACC。
报告了一例ACC的显著临床病理特征,该病例双侧发生于一名年轻白人女性的下颌骨,且伴有肺转移,以恰当描述此类罕见病变,并将其与其他更常见累及下颌骨的肿瘤相鉴别。
患者表现为右磨牙后区疼痛性肿胀及同侧下唇感觉异常,影像学检查显示双侧下颌骨透光性病变,有明确的恶性征象,但病变内无钙化,与牙根或囊性成分无关。常规组织学检查显示为典型的ACC,具有实性和筛状生长模式,并广泛浸润相邻组织。
鉴于骨内恶性涎腺型肿瘤罕见且缺乏特异性体征,其诊断非常困难,主要依靠组织学检查及全面的临床影像学检查来确诊。由于肿瘤病变广泛,该患者不适合手术治疗,目前正在接受包括化疗和放疗在内的多模式治疗,自最初诊断后3年仍存活,疾病持续存在。