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branchial cleft cyst carcinoma

Tuesday 20 September 2016

carcinoma arising in a branchial cyst.

Definition: squamous cell carcinoma arising in a branchial cleft cyst .

BCCC is currently regarded as an uncommon clinicopathological entity.

In 1950, Martin et al. reviewed 250 cases of BCCC, of which the majority represented metastatic head and neck primary cancers resulting from an absence of long-term follow-up.

Strict diagnostic criteria for BCCC were subsequently established, as follows:
- a) tumor located along the anterior border of the sternocleidomastoid muscle;
- b) histological findings consistent with tissue originating from a branchial cleft;
- c) histological evidence of carcinoma arising in the wall of an epithelial-lined cyst;
- d) no evidence of a primary source during a minimum 5-year follow-up period.

In 1989, Khafif et al. modified the above criteria as follows:
- a) tumor located in the anatomic region of a branchial cleft cyst;
- b) histological appearance consistent with a branchial vestige origin;
- c) for a squamous cell carcinoma, presence of the carcinoma within the lining of an identifiable epithelial cyst;
- d) evidence of transition from a normal squamous epithelium of the cyst to carcinoma;
- e) absence of any identifiable primary malignant tumor following exhaustive evaluation of the patient.

According to these criteria, fewer than 40 cases were reported.

Differential diagnosis

A solitary cystic metastatic cervical lymph node, from a small occult primary tumor, is easily misdiagnosed as BCCC, especially during initial presentation.

Hardee et al. reported two cases of solitary cystic metastatic cervical lymph nodes initially presenting as branchial cysts, one of which was not located at the primary site.

In the other case, primary carcinoma was discovered in the right tonsil.

Solitary cystic masses located in level II of the neck are problematic for clinicians.

Briggs et al. suggested the following reasons for this diagnostic difficulty:
- a) the two diseases always manifest in similar locations;
- b) cervical cystic metastases might represent the initial presentation of an occult primary carcinoma in the upper aerodigestive tract;
- c) histological features usually confer difficulty with respect to differentiation of a branchial cleft carcinoma from solitary cystic degeneration of a metastatic cervical lymph node.

In the present case, the patient also presented with a painless solitary cystic mass in the left side of level II of the neck. FNAB suggested a branchial cleft cyst. A primary lesion was not discovered during thorough work-up investigations.

Subsequently, the mass was completely excised. The postoperative histological results revealed that the lesion was a BCCC, and should therefore not be considered an occult primary carcinoma according to the criteria of Martin et al. and Khafif et al. .

Differential diagnosis

- metastatic cystic squamous cell carcinoma (25400796)


- Report of a rare case of carcinoma arising in a branchial cyst.
Anantharajan N, Ravindranathan N.
Ear Nose Throat J. 2014 Sep;93(9):E4-6.4
PMID: 25255359

- Branchial cleft cyst carcinoma: fact or fiction?
Bradley PT, Bradley PJ.
Curr Opin Otolaryngol Head Neck Surg. 2013 Apr;21(2):118-23.
doi : 10.1097/MOO.0b013e32835cebde
PMID: 23328541