Abstract
Carcinoma cuniculatum is a locally aggressive, low-grade variant of squamous cell carcinoma. Though primarily a tumour of the skin, it has also been found to be arising from various mucosal surfaces including oral mucosa. When it occurs in the mandible it starts as a mucosal lesion which progressively invades the jaw and destroys the underlying bone, often presenting as a cystic lesion. With only around 30 cases reported in the mandible, this entity remains an elusive one for the pathologist and all except a few cases reported so far, had been misdiagnosed initially. The delay in the diagnosis is a major concern as it results in further progression of the disease with extensive involvement of bone and associated soft tissue structures. The aim of this article is to discuss the histopathological challenges faced in arriving at the diagnosis of a case of carcinoma cuniculatum involving the mandible. Owing to its overlapping clinico-pathological characteristics with odontogenic keratocyst the lesion has been misdiagnosed initially resulting in inadequate management, leading to multiple recurrences and unexpected complications. As a few cases like the present one had been erroneously diagnosed as odontogenic keratocyst previously, it becomes important to identify the varied clinical presentation and unique histopathological characteristic of carcinoma cuniculatum of mandible to help us in recognizing this often-missed entity at an earlier stage, so that it can be managed effectively.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Aird I, Johnson HD, Lennox B, Stansfeld AG. Epithelioma cuniculatum: a variety of squamous carcinoma peculiar to the foot. Br J Surg. 1954;42(173):245–50. https://doi.org/10.1002/bjs.18004217304.
Goh GH, Venkateswaran K, Leow PC, Loh KS, Thamboo TP, Petersson F. Carcinoma cuniculatum of the esophagus and tongue: report of two cases, including TP53 mutational analysis. Head Neck Pathol. 2014;8:261–8.
Barreto JE, Velazquez EF, Ayala E, Torres J, Cubilla AL. Carcinoma cuniculatum: a distinctive variant of penile squamous cell carcinoma: report of 7 cases. Am J Surg Pathol. 2007;31:71–5.
Puxeddu R, Cocco D, Parodo G, Mallarini G, Medda M, Brennan PA. Carcinoma cuniculatum of the larynx: a rare clinicopathological entity. J Laryngol Otol. 2008;122:1118–23.
Sun Y, Kuyama K, Burkhardt A, Yamamoto H. Clinicopathological evaluation of carcinoma cuniculatum: a variant of oral squamous cell carcinoma. J Oral Pathol Med. 2012;41:303–8.
Sloan P, Gale N, Hunter K, et al. Tumours of the oral cavity and mobile tongue. In: EI-Naggar AK, Chan JKC, Grandis JR, Takata T, Slootweg P, editors., et al., WHO classification of head and neck tumours. Lyon: International Agency for Research on Cancer; 2017. p. 109–11.
Fliegar S, Owinski T. Epithelioma cuniculatum an unusual form of mouth and jaw neoplasm [in Polish]. Czas Stomatol. 1977;30:395–401.
Farag AF, Abou-Alnour DA, Abu-Taleb NS. Oral carcinoma cuniculatum, an unacquainted variant of oral squamous cell carcinoma: a systematic review. Imaging Sci Dent. 2018;48(4):233–44. https://doi.org/10.5624/isd.2018.48.4.233.
Ajith A, Subramaniam N, Balasubramanian D, Thankappan K, Iyer S. Carcinoma cuniculatum of the oral cavity: a diagnostic dilemma. J Head Neck Physicians Surg. 2018;6:54–6.
Pons Y, Kerrary S, Cox A, Guerre A, Bertolus C, Gruffaz F, Capron F, Goudot P, Ruhin-Poncet B. Mandibular cuniculatum carcinoma: apropos of 3 cases and literature review. Head Neck. 2012;34(2):291–5. https://doi.org/10.1002/hed.21493.
Shay S, Choy W, Christensen RE, St John MA. Extensive carcinoma cuniculatum of the mandible. Am J Otolaryngol. 2015;36(3):446. https://doi.org/10.1016/j.amjoto.2015.01.002.
Fonseca FP, Pontes HA, Pontes FS, de Carvalho PL, Sena-Filho M, Jorge J, Santos-Silva AR, de Almeida OP. Oral carcinoma cuniculatum: two cases illustrative of a diagnostic challenge. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;116(4):457–63. https://doi.org/10.1016/j.oooo.2013.06.006.
Raguse JD, Menneking H, Scholmann HJ, Bier J. Manifestation of carcinoma cuniculatum in the mandible. Oral Oncol Extra. 2006;42:173–5.
Suzuki J, Hashimoto S, Watanabe K, Takahashi K, Usubuchi H, Suzuki H. Carcinoma cuniculatum mimicking leukoplakia of the mandibular gingiva. Auris Nasus Larynx. 2012;39(3):321–5. https://doi.org/10.1016/j.anl.2011.06.004.
Padilla RJ, Murrah VA. Carcinoma cuniculatum of the oral mucosa: a potentially underdiagnosed entity in the absence of clinical correlation. Oral Surg Oral Med Oral Pathol Oral Radiol. 2014;118(6):684–93. https://doi.org/10.1016/j.oooo.2014.08.011.
Shapiro MC, Wong B, O’Brien MJ, Salama A. Mandibular destruction secondary to invasion by carcinoma cuniculatum. J Oral Maxillofac Surg. 2015;73(12):2343–51. https://doi.org/10.1016/j.joms.2015.05.034.
Datar UV, Kale A, Mane D. Oral carcinoma cuniculatum: a new entity in the clinicopathological spectrum of oral squamous cell carcinoma. J Clin Diagn Res. 2017;11(1):ZD37–9. https://doi.org/10.7860/JCDR/2017/23437.9226.
Zhang C, Hu Y, Tian Z, Zhu L, Zhang C, Li J. Oral carcinoma cuniculatum presenting with moth-eaten destruction of the mandible. Oral Surg Oral Med Oral Pathol Oral Radiol. 2018;125(4):e86–93. https://doi.org/10.1016/j.oooo.2018.01.008.
Sivapathasundharam B, Kavitha B, Padmapriya VM. Carcinoma cuniculatum of the alveolar mucosa: a rare variant of squamous cell carcinoma. Head Neck Pathol. 2019;13(4):652–5. https://doi.org/10.1007/s12105-018-0938-y.
Broly E, Barthélémy P, Ciftci S, Borel C, Broly M, Gros CI, Marcellin L, Bornert F. Hidden intra-mandibular carcinoma cuniculatum appearing in a patient with metastatic prostate cancer: a case report. BMC Oral Health. 2019;19(1):54. https://doi.org/10.1186/s12903-019-0745-1.
Barrett AW, Garg M, Armstrong D, Bisase BS, Newman L, Norris PM, Shelley M, Tighe JV, Hyde NC, Chaston NJ, Gulati A. Cystic squamous cell carcinomas of the jaws: twelve cases highlighting histopathological pitfalls. Int J Surg Pathol. 2020;28(6):624–30. https://doi.org/10.1177/1066896920903388.
Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. 3rd ed. Philadelphia, PA: Saunders; 2008. p. 678–740.
El-Naggar AK, Chan JK, Grandis JR, Takata T, Slootweg PJ. World Health Organization classification of head and neck tumours. 4th ed. Lyon: IARC Press; 2017.
de Morais EF, Carlan LM, de Farias Morais HG, et al. Primary intraosseous squamous cell carcinoma involving the jaw bones: a systematic review and update. Head and Neck Pathol. 2020. https://doi.org/10.1007/s12105-020-01234-z.
Allon I, Vered M, Kaplan I, Nahlieli O, Yahalom R, Shalmon B, Srouji S, Livoff A. Rare variants of head and neck squamous cell carcinoma -differential immunohistochemical profiles. Acta Histochem. 2019;121(8):151444. https://doi.org/10.1016/j.acthis.2019.151444.
Funding
No funding obtained.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declares that they have no conflict of interest.
Ethical Approval
The manuscript is complying with the institutional ethical requirements. Written informed consent for publication of the clinical details and/or clinical images was obtained from the patient.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Janardhanan, M., Rakesh, S., Savithri, V. et al. Carcinoma Cuniculatum of Mandible Masquerading as Odontogenic Keratocyst: Challenges in the Histopathological Diagnosis. Head and Neck Pathol 15, 1313–1321 (2021). https://doi.org/10.1007/s12105-020-01265-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12105-020-01265-6