Primary branchiogenic carcinoma: malignant degeneration of a branchial cyst, a case report

Abstract

Background Primary branchiogenic carcinoma (PBC) is an extremely rare and poorly documented disease developed from a brachial cleft cyst. Case 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. Conclusions 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.

Non-syndromic Bilateral Branchial Cyst: A Case Report

“…Even though it is extremely rare, it must be excluded prior to surgical intervention as it entails a different course of treatment. 20 Al-Mufarrej et al, 14 found malignant degeneration in four of 421 (1%) cases in older patients (> 45 years) with branchial anomalies.…”

Malignancy and “Violated Neck” Rates in Consecutive Cohort of 79 Adult Patients With Solitary Cystic Neck Mass—Lessons Learned and Recommendations for Clinical Practice Guidelines

“…14,21 Other primary cancers within SCNM (eg, primary branchiogenic carcinoma, dermoid cyst carcinoma) are typically found in case reports or review articles in the literature. 2,22,23 In contrast, SCNMets are frequently encountered, with a slight increase in their prevalence over the past few decades. 6,10 The most critical part of the diagnostic evaluation in these cases is identifying the primary tumor.…”

“…Primary cancers arising from the cyst wall are exceedingly rare, and the most common malignancy within this group is primary TDR carcinoma (1–3% of all TDRs) 14,21 . Other primary cancers within SCNM (eg, primary branchiogenic carcinoma, dermoid cyst carcinoma) are typically found in case reports or review articles in the literature 2,22,23 . In contrast, SCNMets are frequently encountered, with a slight increase in their prevalence over the past few decades 6,10 .…”

Current approach to branchial remnants in the neck

“…Between 3–24% of cystic tumor masses are malignant, especially in adults over 40 years. In 1988, Khaffif reformulated the diagnostic criteria for primary branchiogenic carcinoma (PBC) and all criteria must be met to confirm the diagnosis: (i) the tumor is located in the anatomical region of the gill cysts; (ii) the histology of the tumor must be in concordance with the origin in the branchial vestiges, so it has to be squamous cell carcinoma; (iii) the presence of carcinoma at the level of the shell of an identifiable epithelial cyst; (iv) to be able to identify the transition from normal squamous epithelium to enveloped carcinoma; (v) comprehensive evaluation of the patient should not reveal another primary malignant tumor [ 33 ]. There were situations in which the diagnosis of malignant branchial cyst delayed the true diagnosis of a primary occult palatine tonsil carcinoma with associated cystic lymph node metastasis [ 34 ].…”