Department of Multidisciplinary Medical, Surgical and Dental Specialties, University of Campania "Luigi Vanvitelli", Naples, Italy.
Department of Neurosciences, Reproductive and Odontostomatological Sciences, University of Naples "Federico II", Naples, Italy.
Cancer Rep (Hoboken). 2021 Apr;4(2):e1315. doi: 10.1002/cnr2.1315. Epub 2020 Dec 9.
Primary branchiogenic carcinoma (PBC) is an extremely rare and poorly documented disease developed from a brachial cleft cyst.
A 51-year-old patient was referred to our unit for an upper neck mass. PBC was confirmed in accordance with Kahfif's diagnostic criteria. Prophylactic selective neck dissection was performed in a second-stage surgery to ensure the complete removal of the neoplasm. Branchiogenic origin with lymphoid tissue was confirmed in the "host cyst" after histological examination and no other tumors were found elsewhere. Regular follow up documented no relapse 12 months after surgery.
Although rare, PBC must be suspected in presence of cervical masses, especially in patients older than 40 years. A standardized treatment algorithm still lacks, but prophylactic selective neck dissection could be considered as the first line choice after the diagnosis has been confirmed.
原发鳃源性癌（PBC）是一种极为罕见且记录甚少的疾病，由鳃裂囊肿发展而来。
一名 51 岁患者因颈上部肿块就诊于我院。根据 Kahfif 的诊断标准，确诊为 PBC。为确保肿瘤完全切除，在二期手术中进行预防性选择性颈淋巴结清扫术。组织学检查证实“宿主囊肿”来源于鳃源性，伴淋巴组织，且未在其他部位发现其他肿瘤。术后 12 个月定期随访未发现复发。
尽管罕见，但对于存在颈上部肿块的患者，尤其是年龄大于 40 岁的患者，应怀疑存在 PBC。目前仍缺乏标准化的治疗方案，但在确诊后，预防性选择性颈淋巴结清扫术可作为一线选择。
