*Department of Dermatopathology, St John's Institute of Dermatology, St Thomas' Hospital, London †Department of Pathology, Manchester Royal Infirmary, Manchester #Department of Pathology, Western General Hospital and The University of Edinburgh, Edinburgh, UK ‡Nelson R. Mandela School of Medicine and Central Hospital, Durban, South Africa §Sections of Dermatopathology and Soft Tissue/Sarcoma Pathology, MD Anderson Cancer Center, University of Texas, Houston, TX ∥Department of Pathology, Dublin Hospital, Dublin, Ireland ¶Charles University Medical Faculty Hospital, Pilsen, Czech Republic.
Am J Surg Pathol. 2013 Oct;37(10):1603-11. doi: 10.1097/PAS.0b013e318299fcac.
This study examines clinical and pathologic features of primary cutaneous adenoid cystic carcinoma (ACC), with emphasis on biological behavior of these tumors. A total of 27 cases of primary cutaneous ACC with detailed follow-up information were evaluated. Clinically, these were solitary, slow-growing lesions, half of which were in the head and neck area. The median age was 62 years with a male predilection. Surgical excision was the treatment of choice. Histologically, the lesions were similar to those seen in the salivary glands. Tumors were classified as grade 1 (17), grade 2 (3), and grade 3 (7). The mitotic count was generally low (mean=1.9/mm), except in 2 high-grade tumors (>10 mitotic figures/mm). Sixteen cases showed perineural invasion. Immunohistochemically, cytokeratin positivity was noted in 13/13 cases, and CD117 was observed in 10/10 cases, with luminal/cytoplasmic staining for epithelial membrane antigen (14/16) and at least focal luminal expression for carcinoembryonic antigen (11/16), smooth muscle actin (10/13), and S100 staining (9/13). Eighteen cases had follow-up data (median 54 mo), 9 of which had local recurrences (50%). Three cases showed metastatic disease. No statistical difference was noted between tumor grade and local recurrence (P=0.77). Primary cutaneous ACC is a distinct entity with a more indolent behavior compared with its salivary counterpart. The cutaneous lesions tend to recur locally but have a low metastatic potential.
本研究探讨了原发性皮肤腺样囊性癌（ACC）的临床和病理特征，重点关注这些肿瘤的生物学行为。共评估了 27 例具有详细随访信息的原发性皮肤 ACC 病例。临床上，这些肿瘤为单发、生长缓慢的病变，其中一半位于头颈部。中位年龄为 62 岁，男性多见。手术切除是首选治疗方法。组织学上，这些病变与唾液腺中的病变相似。肿瘤分为 1 级（17 例）、2 级（3 例）和 3 级（7 例）。有丝分裂计数通常较低（平均为 1.9/毫米），但在 2 例高级别肿瘤中（>10 个有丝分裂/毫米）较高。16 例有神经周围侵犯。免疫组化染色显示，13/13 例细胞角蛋白阳性，10/10 例 CD117 阳性，上皮膜抗原的腔/细胞质染色阳性（14/16），癌胚抗原的至少局灶性腔表达阳性（11/16）、平滑肌肌动蛋白（10/13）和 S100 染色阳性（9/13）。18 例有随访资料（中位随访时间为 54 个月），其中 9 例出现局部复发（50%）。3 例出现转移性疾病。肿瘤分级与局部复发之间无统计学差异（P=0.77）。原发性皮肤 ACC 是一种独特的实体瘤，与唾液腺相比，其行为更为惰性。皮肤病变倾向于局部复发，但转移潜能较低。
