|Year : 2014 | Volume
| Issue : 2 | Page : 114-116
A case of primary branchial carcinoma
Abid AK Parambil, Mohammed Suaib, Sarvotaman Nedungadi, Asiq S Nalakath
Department of General Surgery, MES Medical College, Palachode, Perintalmanna, Kerala, India
|Date of Web Publication||16-Oct-2014|
Abid AK Parambil
Department of General Surgery, MES Medical College, Palachode, Perintalmanna, Kerala
Source of Support: None, Conflict of Interest: None
Branchiogenic carcinoma, which is squamous cell carcinoma arising in a branchial cyst, is extremely rare and a highly contentious clinicopathologic entity. Initially described by von Volkmann in 1882, it was better defined by Martin et al. in 1950 and the definition further refined by Khafif et al. in 1989. In spite of this, the skepticism about this entity persists. The existence of primary branchiogenic carcinoma is controversial according to some authors this malignancy is more conceptual than a real clinic pathological entity. It's very difficult to distinguish a primary branchiogenic carcinoma from a cystic nodal metastatic one. There are different diagnostic criteria for primary branchiogenic carcinoma, but its efficacy is still challenging. We present a case, wherein the patient underwent excision of a branchial cyst on the left side of the neck, which revealed squamous cell carcinoma in its lining.
Keywords: Branchial cyst carcinoma, cystic neck mass, squamous cell carcinoma, tonsilar malignancy
|How to cite this article:|
Parambil AA, Suaib M, Nedungadi S, Nalakath AS. A case of primary branchial carcinoma
. Arch Int Surg 2014;4:114-6
| Introduction|| |
Branchial cysts are congenital and occur due to the failure of obliteration of cervical sinus of his. It is considered as one of the most common cause of congenital swelling and occurs in the upper part of anterior triangle of neck. Branchial cyst carcinoma is a rare entity and occurs in the fifth and sixth decades of life, more commonly in males. Incidence ranges from 11% to 21%, respectively.  Lateral cystic masses usually occur as secondary squamous cell carcinoma arising from tonsil, tongue base and nasopharynx. Therefore, it is difficult to differentiate primary branchial cyst malignancy from secondary cystic mass. Initially described by von Volkmann in 1882, it was better defined by Martin et al. in 1950 and the definition further refined by Khafif et al. in 1989. , In spite of this, the existence of primary branchiogenic carcinoma is still controversial. According to some authors, this malignancy is more conceptual than a real clinic pathological entity. There are different diagnostic criteria for primary branchiogenic carcinoma, but their efficacy is still challenging. We present a case wherein the patient underwent excision of a branchial cyst on the left side of the neck, which histologically revealed squamous cell carcinoma in its lining.
| Case Report|| |
A 44-year-old male presented with the complaints of swelling at left upper part of neck for the past 6 years and rapid increase in size for the past 3 months. There were no other symptoms. Examination revealed a 6 cm × 4 cm cystic mobile swelling in the upper anterior triangle of neck deep to left sternocleidomastoid muscle, with well-defined borders, without transilluminency. There was no other swelling in the neck. Ultrasound revealed a cystic mass and cytology showed scattered mature squamous epithelial cells. Few metaplastic cells and anucleate forms were noted on a background of amorphous debris. A diagnosis of brachial cyst was made.
Intra operatively, there was a well-circumscribed cystic structure with dense adhesions in the upper part of neck. The lesion was excised completely. Grossly, it was pedunculated and pale brown measuring 5 cm × 2.5 cm. Cut-section showed pale white to dark brown areas [Figure 1]. Microscopy revealed cyst wall composed of infiltrating neoplasm in sheet and cords. Pleomorphic cells with moderate amount of eosinophilic cytoplasm mildly pleomorphic vesicular to hyper chromatic nuclei. The stroma was fibrocollagenous with inflammatory infiltrate and reported as well differentiated squamous cell carcinoma arising from branchial cyst [Figure 2] and [Figure 3].
|Figure 2: Cyst wall with infiltrating neoplasm arising from epithelium composing of cells in nests and sheaths and inferiorly tumor cells infiltrating into cyst wall|
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After getting the result, we did a thorough search for primary focus by doing pan endoscopy, tonsillectomy and biopsies from nasopharynx. All were negative and postoperative computed tomography (CT) picture showed no other specific lesion.
| Discussion|| |
Branchial cyst usually develops because of incomplete obliteration of branchial cleft. Second branchial cleft cyst usually occurs in the upper part of neck medial to sternocleidomastoid as a smooth, fluctuant, and ovoid structure.  The cyst wall is lined by squamous epithelium hence carcinomatous transformation is possible even though it is very rare. The existence of true branchial cyst carcinoma is still debated. von Volkman described this entity first in 1882.  At the moment, controversies still exist regarding the existence of this primary tumor. This is mainly because of most of the cervical masses are secondaries and these are undetectable also especially from tonsillar area. Probably, all cystic malignancies of the neck are metastatic deposit whether there is a detectable primary or not. [3.4] That's why many authors argue that branchiogenic carcinomas are not primary they are the secondary cystic metastasis from oropharyngeal carcinoma especially from tonsil. , Dating back to the original description by von Volkman, until the landmark article of Martin et al. in 1950 there were frequent questioning about the existence of this tumor. ,, The main reason behind this is to differentiate branchial carcinoma from cystic degeneration of secondary lymph node from an occult primary. Martin et al. provided a set of criteria to differentiate this tumor by reviewing about 250 cases.  However, the criterion put forward by him is not fully satisfied by any of these 250 cases. In 1989 Khafif et al. by reviewing 67 more cases postulated additional information to Martins criterion.  Even then only few cases have been reported which have satisfied the strict criteria mentioned by Martin et al. and Khafif et al. ,, We believe our case satisfies these criteria the most important being demonstrating a normal lining epithelium and zone of transition. The occult primary work-up include contrast enhanced CT, positron emission tomography scan, pan endoscopy and biopsies. We investigated our patient with all these to detect a primary but we could not find any. The treatment of branchial cyst malignancy is wide surgical excision, preferably with ipsilateral radical neck dissection. Postoperative irradiation to the ipsilateral neck is recommended. 
Data on the prognosis of branchiogenic carcinoma are scarce. Nevertheless, a proportion of patients seem to enjoy long-term survival.  Cystic metastases from squamous cell carcinoma appear to have a better prognosis compared to other squamous cell carcinomas of the head and neck. Some authors suggest that these lesions constitute a separate category, distinct from other head and neck malignancies. In one study, the 5 years survival was 77%.  The survival rates in patients with cystic metastases and an overt primary or with an unknown primary site seem to be similar.
| Conclusion|| |
Primary branchial carcinoma is a rare entity. It's difficult to clinically diagnose it; even on histopathology, where all the criteria need not to be present. Hence, a primary source for a secondary lymph node with cystic degeneration should be definitely looked for.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]