Analysis of Characteristics and Survival of Primary Cutaneous Adenoid Cystic Carcinoma of the Head and Neck

Ann Otol Rhinol Laryngol. 2021 Jan;130(1):12-17. doi: 10.1177/0003489420936719. Epub 2020 Jun 21.


Background/objectives: Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare skin tumor. The head and neck (H&N) is the most common anatomical location. Due to limited published cases, its clinical course and management are not well understood.

Methods: The National Cancer Database (NCDB) was queried for all cases of H&N PCACC diagnosed from 2004 to 2016. Kaplan-Meier (KM) and Cox proportional hazards models were used to determine clinicopathological and treatment factors associated with survival outcomes.

Results: A total of 201 cases were analyzed. The average age of diagnosis was 57.7 years (± SD 15.8). There was a female predilection (57.7%; P < .05) with the most common primary site being the ear (58.2%). The average tumor size was 15.9 mm in diameter. The most common treatment was surgery alone (51.7%) with wide local excision being the common surgery performed (36.3%). 5-year and 10-year OS were 87.0% and 76.0%, respectively. A total of 65.8% of cases were localized (Stage I and II). KM analysis indicated that gender, age, insurance status, Charlson-Deyo Comobordity Score, and stage were significant predictors of OS (P < .05). Cox proportional hazards analysis revealed that patients with both private (HR 0.11, 95%CI [0.019-0.670]; P = .02) and government (HR 0.12, 95%CI [0.019-0.972]; P = .03) health insurance had a significantly decreased hazard of death than patients who were uninsured. Increasing age was associated with an increased hazard of death (HR 1.06, 95%CI [1.016-1.110]; P = .01).

Conclusion: This study represents the largest cohort of H&N PCACC studied to date and provides important clinicopathologic information for this rare tumor. Additionally, our results emphasize the importance of health insurance as an independent predictor of survival in PCACC.

Keywords: NCDB; National Cancer Database; cancer; primary cutaneous adenoid cystic carcinoma; survival.

MeSH terms

  • Age Factors
  • Aged
  • Carcinoma, Adenoid Cystic / mortality*
  • Carcinoma, Adenoid Cystic / pathology
  • Carcinoma, Adenoid Cystic / surgery*
  • Comorbidity
  • Databases, Factual
  • Female
  • Head and Neck Neoplasms / mortality*
  • Head and Neck Neoplasms / pathology
  • Head and Neck Neoplasms / surgery*
  • Humans
  • Insurance Coverage
  • Insurance, Health
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Proportional Hazards Models
  • Sex Factors
  • Skin Neoplasms / mortality*
  • Skin Neoplasms / pathology
  • Skin Neoplasms / surgery*
  • United States / epidemiology