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By Barbara Ann Burtness, MD
A 52-year-old man who lives in the Midwest presents with a lesion in his nose. He has an extensive history of sun exposure and a 35 pack-year smoking history. The patient sought attention from an otolaryngologist, who cultured the lesion in the right upper medial nasal vestibule but noted “no lesion of the nose.” He was treated with Bactroban ointment. During a follow-up visit, the cultures were negative and the lesion had not improved.
Six months later, the patient returned to his otolaryngologist because of reddening of the exterior tip of the nose. Cultures were again negative, and a course of sulfamethoxazole and trimethoprim was prescribed. When the redness and swelling of the nose persisted, a tentative diagnosis of relapsing polychondritis was rendered, and a trial of Medrol Dosepak was undertaken. Within several weeks, the patient noted progressive reddening of the nose and upper lip, which became tender.
The patient sought a second opinion. The second otolaryngologist noted that the entire nasal tip was swollen, with crusting in the bilateral nasal vestibules. The patient was asked to continue Bactroban. Four subsequent trials of oral antibiotics were completed without improvement. A chest CT scan was normal.
A further 6 months elapsed. The nose was reddened and uncomfortable for the entire duration, and the patient was maintained on hydrocodone and acetominophen. Changes in the skin over the right nose were noted, consistent with a blister. The surface of the nose occasionally bled slightly. The patient was referred to a dermatologist. At this time, exophytic plaque with distinct borders was present on the skin of the nose, extending to the nasal sidewall/cheek junction, nodular purpuric plaque on the columella, philtrum, and upper lip, and a crusting lesion was visible in the right nasal vestibule, eroding the septum and contralateral nares.
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A differential diagnosis of sarcoid vs. lupus pernio vs. tuberculosis was entertained. Smears were negative for fungal and AFB forms. Skin biopsy demonstrated keratinizing, invasive squamous cell carcinoma.
Nasal endoscopy demonstrated tumor mass extending into the right nasal cavity. CT scan showed an irregular enhancing soft tissue mass involving the nasal tip and extending into the right ventral nasal cavity with narrowing of the nasal cavity. No lymphadenopathy was present. Total rhinectomy was undertaken. Pathology showed a 3.8 cm, poorly differentiated squamous cell carcinoma invading the reticular dermis and extending into the hyaline cartilage of the nose. Perineural invasion was present. All margins were negative for invasive and in situ carcinoma, and the cancer was staged as T2Nx.
This is a rare case of squamous cell carcinoma arising in the nasal vestibule. Both squamous cell carcinomas and keratoacanthomas of the nasal vestibule are well described1. Most nasal vestibule cancers are keratoacanthomata, which have a low risk of recurrence or dissemination. Initial manifestations of nasal vestibule carcinoma may be subtler than in keratoacanthomata. Squamous cell carcinoma of the nasal vestibule may manifest with chronic vestibulitis and pseudo-lupus pernio. Of the diagnostic options listed, the metastatic lesions are extremely unlikely, given the long natural history. Tuberculosis would be expected to be associated with constitutional symptoms, and sarcoid with hilar adenopathy.
A retrospective Danish study is the largest study of nasal vestibule cancer reported, including data on 174 patients treated with surgery and/or radiation, including 109 with T1 cancers and 30 with T2 cancers. For patients with T1 cancers, the 5-year locoregional control rate was 79%, 5-year disease-specific survival was 83%, and overall survival (OS) was 61%. For patients with T2 cancers, 5-year loco-regional control was 54%, 5-year disease-specific survival was 63%, and OS at 5 years was 43%. The median age was 69 years, and age was a significant predictor of OS.
A series of 27 patients from the UK has also been reported.2 In this series, 3 of 5 patients with T2 or T3 cancer succumbed to their disease, compared with an OS at 8 years of 68% for the group as a whole. A review of an experience with primary radiation treatment for squamous cell carcinoma of the nasal vestibule has been reported from the Netherlands, with a 5-year OS rate for patients with T1 and T2 cancer of 66%; however, 75% of deaths were due to causes other than nasal vestibule cancer.3 The experience from the University of Florida suggests 95% local control, albeit in a population with a high rate of competing causes of mortality.4
Comparison of these data to a series including cutaneous squamous cell carcinoma from all sites leads to the conclusion that nasal vestibule cancer has a worse prognosis than other cutaneous squamous cell cancers. A 20-year review from the Netherlands5 reports a 5-year relative survival for cutaneous squamous cell cancer of 93% for all patients with squamous cell cancer of the face, and 95% for all patients with stage I cancer; however, 5-year relative survival falls to 76% for patients with stage II cancer.
Ironically, the first association between a habitual exposure and cancer was the recognition by John Hill in 1761 that the use of tobacco snuff caused nasal cancer. However, as nasal vestibule cancers are no longer common, it is worth emphasizing that any nonhealing lesion of the nasal vestibule merits biopsy. In addition to squamous cell carcinoma, the differential diagnosis of a nonhealing lesion in the nasal vestibule can include: metastasis from another malignancy such as non-small cell lung cancer or melanoma; submucosal extension from a nasopharyngeal or sinus primary; primary mucosal melanoma; pleomorophic adenoma; intramuscular myxoma; angiofibroma; leiomyoma; lipoma; and T-cell lymphoma.
Generally, management is with primary radiation therapy for T1-T2 disease, and rhinectomy followed by radiation or chemoradiation for more advanced disease. The roles of induction chemotherapy, concurrent cetuximab, and brachytherapy are all poorly defined.
References
- Agger A, von Buchwald C, Madsen AR, et al. Squamous cell carcinoma of the nasal vestibule 1993-2002: a nationwide retrospective study from DAHANCA. Head Neck. 2009;31(12):1593-1599.
- Dowley A, Hoskison E, Allibone R, Jones NS. Squamous cell carcinoma of the nasal vestibule: a 20-year case series and literature review. J Laryngol Otol. 2008;122(10):1019-1023.
- Langendijk JA, Poorter R, Leemans CR. Radiotherapy of squamous cell carcinoma of the nasal vestibule. Int J Radiat Oncol Biol Phys. 2004;59(5):1319-1325.
- Wallace A, Morris CG, Kirwan J, et al. Radiotherapy for squamous cell carcinoma of the nasal vestibule. Am J Clin Oncol. 2007;30(6):612-616.
- Hollestein LM, de Vries E, Nijsten T. Trends of cutaneous squamous cell carcinoma in the Netherlands: Increased incidence rates, but stable relative survival and mortality 1989-2008. Eur J Cancer. 2012;48(13):2046-2053.
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