腺樣囊狀癌(adenoid cystic carcinoma)爲常見的唾液腺腫瘤之一,好發於腭部,腮腺及顎下腺次之。發生年齡以中年成人最爲常見,甚少發生於20歲以下的未成年人,其發生並無明顯的性別差異。臨床上通常以生長速度緩慢的腫塊出現,在病變肉眼明顯可見前,病人常抱怨患部的疼痛。組織學型態以篩狀最爲典型及最容易辨識,但通常伴隨著管狀或固狀的型態。手術切除是主要的治療方式,術後合併放射線治療可以提高病人的存活率。腺樣囊狀癌不僅容易局部復發也易發生遠處轉移,遠處轉移常發生於肺臟及骨頭。本報告提出一16歲年輕男性病例,主訴爲張口時在右側喉嚨會有不舒服感,已持續有兩年之久,而由母親帶來求診,切片檢查結果爲軟腭右側腺樣囊狀癌,於是安排住院手術治療。由於腹部超音波掃描發現疑似肝腫瘤,於是會診腸胃科及一般外科,腹部電腦斷層及正子掃描檢查後,高度懷疑肝臟左葉轉移。遂安排患者接受協同手術一併切除右軟腭及肝腫瘤,術後病理報告爲軟腭右側腺樣囊狀癌合併肝轉移。由於發生於年輕男性及合併肝轉移的腺樣囊狀癌十分罕見,故就此病例與腺樣囊狀癌的診斷治療及預後,作相關文獻的回顧與討論。
Adenoid cystic carcinoma is a common salivary gland tumor. The palate is the most common site and the remaining is found mostly in the parotid and submandibular glands. The lesion is most in middle-aged adults and is rare in people younger than age 20, no gender distribution predilection. It usually appears as a slowly growing mass, pain is a common finding before there is a noticeable swelling. The cribriform pattern is the most classic and best recognized appearance in histopathologic features, but usually a combination of tubular and solid pattern is seen. Surgical excision is usually the treatment of choice, and adjuvant radiation therapy may slightly improve survival in some cases. It is prone to local recurrence and distant metastasis. Metastatic spread most commonly occurs to the lungs and bones. In this case, a 16 years old male patient who suffered from discomfort over right throat area while mouth opening for about 2 years. He came to our OPD for help and incisional biopsy was performed and the result revealed adenoid cystic carcinoma of right palate. Then he was admitted for surgical intervention. Due to abdominal sonography impressed liver tumor, GI and GS doctors were consulted and both the abdominal CT and PET-CT scan impressed liver metastasis in the left lobe. After combined surgery with wide excision and bisegmentectomy, the pathological report revealed adenoid cystic carcinoma of soft palate with liver metastasis. Summery, this case was the rare adenoid cystic carcinoma occurring on soft palate with liver metastasis in a juvenile. We reviewed the articles and discussed the diagnosis, treatment and prognosis about this case and adenoid cystic carcinoma.