Otolaryngology–Head and Neck Surgery (2010) 143, 167-168
CASE REPORT
Branchiogenic carcinoma of a first branchial cleft cyst Joseph P. Roche, MD, Maher N. Younes, MD, William K. Funkhouser, MD, PhD, and Mark C. Weissler, MD, Chapel Hill, NC Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article.
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irst branchial cleft cysts are much less common than other branchial cleft cysts. Type I first branchial cleft cysts arise from an ectodermal duplication anomaly of the external auditory canal.1,2 Type II first branchial cysts are localized in the anterior neck, superior to the hyoid bone.1 The existence of carcinoma arising from within branchial cleft cysts (branchial cleft cyst carcinoma, branchiogenic carcinoma) has been controversial since its proposal in 1882. Martin et al defined strict criteria for diagnosis in 1950 that were later revised.1,3 In essence, there are two major diagnostic criteria: 1) absence of an identifiable primary carcinoma elsewhere, which can be confirmed by panendoscopy, computed tomography/positron emission tomography (CT/PET) imaging, and/or directed biopsies; and 2) histological identification, including a cystic structure lined by normal squamous epithelium with a transition to in situ and invasive squamous cell carcinoma.4 We present a case of first branchial cleft cyst carcinoma found abutting the external auditory canal (EAC). To the best of our knowledge, this represents the second reported branchiogenic carcinoma at this particular location and fifth report of first branchial cyst carcinoma overall in the literature.1,2 A 62-year-old male initially presented to The University of North Carolina at Chapel Hill (UNC) Hospitals in 2002 with a left parotid mass that was diagnosed as sialoadenitis and treated medically. In 2005, he underwent fine-needle aspiration (FNA) of the persistent mass, which demonstrated cystic fluid containing macrophages, multinucleated giant cells, and an amylase of 99 U/L. The mass was thought to represent a parotid cyst. CT imaging, however, revealed features suggestive of a pleomorphic adenoma. Surgical excision was recommended, but the patient declined. In 2008, he presented with the same mass, which, during the interval period, had grown into his left ear canal. This was accompanied by purulent, foul-smelling drainage from the skin overlying the mass. He had a 30 pack per year history of tobacco usage but no significant medical or surgical history, nor a history of any neoplasms. FNA of the
Figure 1 Soft-tissue window CT image demonstrating a large, cystic lesion measuring 4 ⫻ 3.5 cm in its largest dimension. An eccentrically located nodule (asterisk) is present within the walls of the cyst.
mass, as well as biopsies from the left ear canal, were negative for malignancy. CT imaging (Figs 1 and A1, available online at www.otojournal.org) demonstrated a large, partially cystic/solid mass of the left superficial parotid gland. On examination, the mass appeared to be compressing his left ear canal, but the canal was free of any lesions. Palpation revealed a left-sided, cystic, mobile parotid mass measuring 5 cm in diameter. No cervical adenopathy was palpable, and he had no weakness in his left facial musculature. Indirect mirror and flexible fiberoptic examination of the upper aerodigestive tracts did not reveal any lesions or irregularities. In April of 2009, he underwent a left superficial parotidectomy including the posterior aspect of the external auditory canal and in-continuity excision of the cystic mass.
Received November 20, 2009; revised January 15, 2010; accepted January 27, 2010.
0194-5998/$36.00 © 2010 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved. doi:10.1016/j.otohns.2010.01.031
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Otolaryngology–Head and Neck Surgery, Vol 143, No 1, July 2010 previous cases of first branchial cleft carcinoma have been reported in the literature, with three of these cases likely representing lesions of type II anomalies.1,2 Our patient represents a case of branchiogenic carcinoma of a first branchial cleft cyst, as defined by the criteria outlined above.1,3 Whether this patient’s carcinoma arose in a type I or II first branchial cleft cyst is unknown. If degenerating from a type II duplication cyst, this case would represent the first reported carcinoma of this type at the EAC. This case highlights that, though rare, branchial carcinoma exists, should be included within the differential diagnosis for a lateral neck mass, and may require open biopsy of the branchial vestigia for definitive diagnosis.
Acknowledgment The authors thank Benjamin Y. Huang, MD, Department of Radiology, The University of North Carolina at Chapel Hill, for assistance with imaging data.
Author Information From the Department of Otolaryngology–Head and Neck Surgery (Drs. Roche, Younes, and Weissler) and Department of Pathology & Lab Medicine (Dr. Funkhouser), The University of North Carolina at Chapel Hill, Chapel Hill, NC.
Figure 2 Hematoxylin-eosin section showing the excised cystic mass, with squamous cell carcinoma in situ (arrows), invasive squamous cell carcinoma arising from the in situ carcinoma (asterisk), and benign squamous epithelium (arrowhead). (Original magnification: ⫻40.)
Pathologic analysis revealed invasive squamous cell carcinoma arising within squamous cell carcinoma in situ (Fig 2) inside a squamous-lined cyst. The patient completed 50 Gy of postoperative adjuvant radiation therapy to the ipsilateral neck and parotid bed. At the time of this report, the patient has been seen in follow-up and remains free of oncologic recurrence, with intact neurologic function. This case report received Institutional Review Board exemption by the Biomedical Institutional Review Board at UNC.
Discussion Anomalies of the first branchial cleft are rare and can be of several types, including duplications of the membranous EAC.2 Branchiogenic carcinoma is thought to develop from carcinomatous degeneration of the epidermal lining from cystic remnants of the embryonal branchial apparatus. Controversy has surrounded the existence and diagnosis of cancer arising in branchial cleft vestigial remnants.1,4 Four
Corresponding author: Mark C. Weissler, MD, Department of Otolaryngology–Head and Neck Surgery, G106, Physician’s Office Building, CB 7070, 170 Manning Drive, Chapel Hill, NC 27599-7070. E-mail address:
[email protected].
Author Contributions Joseph P. Roche, design of the project, acquisition and interpretation of data, creation of figures, and manuscript preparation and revision; Maher N. Younes, manuscript preparation and revision; William K. Funkhouser, acquisition and interpretation of pathology images and manuscript revision; Mark C. Weissler, design of the project, interpretation of data, and manuscript preparation and revision.
Disclosures Competing interests: None. Sponsorships: This study was supported in part by NIDCD T32DC005360-05.
References 1. 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. 2. Park SS, Karmody CS. The first branchial cleft carcinoma. Arch Otolaryngol Head Neck Surg 1992;118:969 –71. 3. Martin H, Morfit HM, Ehrlich H. The case for branchiogenic cancer (malignant branchioma). Ann Surg 1950;132:867– 87. 4. Lin YC, Fang SY, Huang RH. Branchiogenic squamous cell carcinoma: a case report. Int J Oral Maxillofac Surg 2004;33:209 –12.
Roche et al
Branchiogenic carcinoma of a first branchial . . .
Figure A1 Coronal, soft-tissue window reconstructed CT image demonstrating a large, cystic lesion measuring 4 ⫻ 3.5 cm in its largest dimension. An eccentrically located nodule (asterisk) is present within the walls of the cyst.
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