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Year : 2013  |  Volume : 9  |  Issue : 1  |  Page : 135-137

Primary branchiogenic carcinoma: Report of a case and a review of the literature

1 Department of Surgical Oncology, Command Hospital, Lucknow, Uttar Pradesh, India
2 Department of Pathology, Command Hospital, Lucknow, Uttar Pradesh, India

Date of Web Publication10-Apr-2013

Correspondence Address:
Ashutosh Chauhan
Department of Oncology, Command Hospital, Lucknow, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.110380

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 > Abstract 

Branchiogenic carcinoma, which is squamous cell carcinoma arising in a branchial cyst, is extremely rare and a highly contentious clinicopathologic entity. A 56-year-old male presented with a well-defined, fluctuant, painless mass on the right side of the neck which was excised with a diagnosis of branchial cyst. The pathological diagnosis was branchial cleft squamous cell carcinoma, which was characterized microscopically by a branchial cleft cyst with a tumour and a non-tumour transitional zone. The report highlights the controversy surrounding this entity and establishes the diagnosis based ona set of histopathologic criteria proposed by Martin et al. and later modified by Khafif et al.

Keywords: Branchial cyst, squamous cell carcinoma, primary

How to cite this article:
Chauhan A, Tiwari S, Pathak N. Primary branchiogenic carcinoma: Report of a case and a review of the literature. J Can Res Ther 2013;9:135-7

How to cite this URL:
Chauhan A, Tiwari S, Pathak N. Primary branchiogenic carcinoma: Report of a case and a review of the literature. J Can Res Ther [serial online] 2013 [cited 2021 May 7];9:135-7. Available from: https://www.cancerjournal.net/text.asp?2013/9/1/135/110380

 > Introduction Top

Primary branchiogenic carcinoma -that is, carcinoma arising in a pre-existing branchial cleft cyst-is an extremely controversial clinic-pathologic entity. Initially described by Volkmann in 1882, it was better defined by Martin et al.[1] in 1950 and the definition further refined by Khafif et al.[2] in 1989. In spite of this, the scepticism about this entity persists. We present a case wherein the patient underwent excision of a branchial cyst on the right side of the neck which revealed squamous cell carcinoma in its lining.

 > Case Report Top

A 56-year-old male patient, non-smoker, presented to our center in 2008 with a right upper neck mass of 4-year duration. Clinical examination revealed a well-defined, painless, fluctuant mass anterior to the anterior border of sternocleidomastoid and at the level of junction of upper and middle one-third of the muscle. Ultrasonogram of the neck showed a well-defined cystic mass, 3cm in diameter, which was anterolateral to the ipsilateral carotid sheath. There was no ipsilateral lymphadenopathy. It was diagnosed as a branchial cyst and underwent a surgical excision in toto.

The resected specimen was a cystic structure with a dark tan smooth surface and measured 3cm in diameter. Multiple hematoxylin and eosin (H&E)-stained sections examined showed partially collapsed cyst lumen surrounded by squamous cell epithelium, which at places revealed progression from benign epithelium to dysplasia, carcinoma in situ (CIS), and invasive moderately differentiated squamous cell carcinoma. Progression into invasive carcinoma was exhibited by infiltration of the highly atypical squamous cells in the form of nests and broad tongues [Figure 1] and [Figure 2]. Dysplastic features in the form of loss of polarity, anisochromasia, nuclear pleomorphism mitotic figures, and prominence of nucleoli were also evident [Figure 3] and [Figure 4]. The cyst wall was conspicuously absent in any organized lymphoid tissue structure like lymph sinuses and lymphoid follicles. The above pathologic findings identified a squamous cell carcinoma arising in an epithelial lined cyst with a gradual transition of benign epithelium to squamous cell carcinoma.

The patient then underwent a computed tomography scan of the head and neck followed by a 18 FDG PET-CT scan. He also underwent panendocopy under general anesthesia followed by bilateral tonsillectomy. The ibid diagnostic workup did not reveal any occult primary. A diagnosis of primary branchiogenic carcinoma was made and the patient underwent adjuvant radiotherapy 60Gy/30#/35 days. The patient has been on regular follow-up since last 3 years. On every three monthly visit, he has undergone a head and neck clinical evaluation in the OPD.He has also undergone a panendoscopy and a whole-body PET-CT scan on two more occasions. There has been no evidence of any occult primary manifesting itself.
Figure 1: Photomicrograph shows the normal squamous epithelium with two dysplastic areas and progression to frank carcinoma in the lower part of the figure. (H and E stain, 4×). (a) Normal squamous epithelium. (b)&(c) Dysplastic area. (d) Invasive squamous cell carcinoma. in situ (e) Lumen of cyst

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Figure 2: Photomicrograph from another area shows benign squamous epithelium progressing to carcinoma in situ and frank carcinoma in the right part of the figure. (H and E stain, 10×). (a) Normal squamous epithelium. (b) Dysplastic area. (c) Carcinoma. (d) Invasive squamous cell carcinoma. (e) Lumen of cyst

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Figure 3: Photomicrograph (H and E stain, 40×) corresponds to Label 'A' in Figure 1: the benign squamous epithelium progressing into dysplasia in the lower part

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Figure 4: Photomicrograph (H and E stain, 40×) corresponds to Label 'B' in Figure 1: progression from dysplasia to carcimoma in situ

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 > Discussion Top

Branchial cleft cysts arise from the incomplete obliteration of any branchial tract, which results in a sinus or cyst. Second branchial cleft cysts present clinically as smooth, soft, ovoid, cystic masses in the lateral part of the neck, behind the angle of the mandible, and anterior to the sternocleidomastoid muscle. [3] As the cyst is usually lined with stratified squamous epithelium, there is a possibility for the development of carcinomatous transformation in the cyst lining. However, there has never been unanimity about the existence of true branchiogenic carcinoma. Many authors argue that this malignancy is more conceptual than a true clinicopathologic entity. [4] This argument is based on the recognition that a variety of head and neck sites - including in particular the tonsil - have a propensity to give rise to cervical metastases while the primary tumours themselves remain undetected. Virtually all cystic carcinomas of the neck represent metastatic deposits, whether or not their primary sites are found. [5],[6] Thus, the authors argue that majority of branchiogenic carcinomas are in fact cystic metastases from oropharyngeal carcinoma, most commonly originating in the tonsils, and not true carcinomas arising in a branchial cleft cyst. [7],[8] As against this, other authors opine that primary branchiogenic carcinoma is indeed a distinct, albeit ill-understood, entity. [9] Martin et al. published a review of 250 cases, including 15 of their own, which had been reported in the English literature from 1882 till 1950. They proposed a set of conditions which required to be satisfied for a diagnosis of branchiogenic carcinoma [Table 1]. The fourth criterion, which was considered the most important, was not satisfied in any of the 250 cases they reviewed, including the 15 cases reported by themselves. Based on a retrospective review of 67 more cases reported from 1950 to 1988, Khafif et al. proposed some more criteria in addition to Martins [Table 2]. Since then very few case reports [8],[10],[11] have been reported which have managed to satisfy the strict criteria mentioned by Martin and Khafif. We believe our case satisfies these criteria. The most important criteria emphasized by both the authors of demonstrating presence of normal lining epithelium, zone of transistion, and frank malignancy is indeed demonstrated in our case.
Table 1: Criteria established by Martin et al. *for the diagnosis of branchiogenic carcinoma

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Table 2: Criteria proposed by Khafif et al *for the diagnosis of branchiogenic carcinoma

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In cases of cervical metastases from occult primary, a diagnostic workup including PET-CT, alongside panendoscopy with directed biopsies including bilateral tonsillectomy, offers the greatest likelihood, up to 69%, of identifying the primary. [12] Our patient has been evaluated on multiple occasions for occult primary during the 3-year follow-up period and no occult primary has been found as yet thus lending credence to the fact that it is indeed a case of primary branchiogenic carcinoma.

Complete surgical excision followed by adjuvant radiotherapy appears to be appropriate treatment. [11],[13] The data on the prognosis of branchiogenic carcinoma are scarce, though long-term survival has been reported. Our patient has been disease free since 3 years.

 > References Top

1.Martin H, Morfit HM, Ehrlich H. The case for branchiogenic carcinoma (malignant branchioma). Ann Surg 1950;132:867-87.  Back to cited text no. 1
2.Khafif RA, Prichep R, Minkowitz S. Primary branchiogenic carcinoma. Head Neck 1989;11:153-63.  Back to cited text no. 2
3.Choo MJ, Kim YJ, Jin HR. A case of second branchial cleft cyst with oropharyngeal presentation. J Korean Med Sci 2002;17:564-65.  Back to cited text no. 3
4.Jereczek-Fossa BA, Casadio C, Jassem J, Luzzatto F, Viale G, Bruschini R, et al. Branchiogenic carcinoma--conceptual or true clinico-pathological entity? Cancer Treat Rev 2005;31:106-14.  Back to cited text no. 4
5.Mallet Y, Lallemant B, RobinY M, Lefebvre JL. Cystic lymph node metastases of head and neck squamous cell carcinoma: Pitfalls and controversies.Oral Oncol 2005;41:429-34.  Back to cited text no. 5
6.Goldenberg D, Sciubba J, Koch WM. Cystic metastasis from head and neck squamous cell cancer: A distinct disease variant? Head Neck 2006;28:633-8.  Back to cited text no. 6
7.Devaney KO, Rinaldo A, Ferlito A, Silver CE, Fagan JJ, Bradley PJ, et al. Squamous carcinoma arising in a branchial cleft cyst: Have you ever treated one? Will you? J Laryngol Otol 2008;122:547-50.  Back to cited text no. 7
8.Maturo SC, Michaelson PG, Faulkner JA. Primary branchiogenic carcinoma: The confusion continues. Am J Otolaryngol 2007;28:25-7.  Back to cited text no. 8
9.Roche JP, Younes MN, Funkhouser WK, Weissler MC.Branchiogenic carcinoma of a first branchial cleft cyst.Otolaryngol Head Neck Surg 2010;143:167-8.  Back to cited text no. 9
10.Girvigian MR, Rechdouni AK, Zeger GD, Segall H, Rice DH, Petrovich Z. Squamous cell carcinoma arising in a second branchial cleft cyst. Am J Clin Oncol 2004;27:96-100.  Back to cited text no. 10
11.Katori H, Nozawa A, Tsukuda M. Post-operative adjuvant chemoradiotherapy with carboplatin and 5-fluorouracil for primary branchiogenic carcinoma. J Laryngol Otol 2005;119:467-69.  Back to cited text no. 11
12.Waltonen JD, Ozer E, Hall NC, Schuller DE, Agrawal A. Metastatic carcinoma of the neck of unknown primary origin: Evolution and efficacy of the modern workup. Arch Otolaryngol Head Neck Surg 2009;135:1024-9.  Back to cited text no. 12
13.Bhanote M, Yang GC. Malignant first branchial cleft cysts presented as submandibular abscesses in fine-needle aspiration: Report of three cases and review of literature. Diagn Cytopathol 2008;36:876-81.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1], [Table 2]

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