Adenoid Cystic Carcinoma of the Parotid and Submandibular Glands in Children and Young Adults: A Population-based Study

Overview

Journal Pediatr Blood Cancer

Specialties Hematology
Oncology
Pediatrics

Date 2024 Feb 28

PMID 38418934

Authors

Alisa L Phillips

Cai Li

Jia Liang

Anthony Sheyn

Jeffrey C Rastatter

Daniel C Chelius Jr

Daniel Orbach

Celine Richard

Affiliations

Soon will be listed here.

Abstract

Objectives: This study aims to analyze the behavior and treatment of adenoid cystic carcinoma (AdCC) in the pediatric and young adult population and to identify factors affecting overall survival (OS).

Materials And Methods: The study analyzed salivary gland malignancies in patients aged 0-21 with AdCC histology using the National Cancer Database from 2004 to 2018.

Results: A total of 72 patients (59.7% parotid, 36.1% submandibular, 1.4% sublingual, 2.8% unspecified) met criteria. Median age was 18 years [range: 0-21]. High-grade dysplasia was present in 67% of cases. Therapy consisted of primary surgery for all cases, regional lymph node dissection (LND) (74%), radiotherapy (71%), chemotherapy (8%), and chemoradiation (7%). The 5-year OS rate was 93.2% [95% confidence interval (CI): 86.9%-99.9%], respectively. Patients who underwent associated LND had improved OS (p = .0083, log-rank test) with a 5-year OS at 82.4% [95% CI: 66.1%-100%] versus 97.6% [95% CI: 93.0%-100%]. A significant difference in OS was found with unfavorable outcomes after positive marginal status: 5-year OS 84.1% [95% CI: 71.0%-99.7%] versus 100% [95% CI: 100%]; p < .001. Adjuvant therapy did not seem to impact the outcome.

Conclusion: This study confirms that AdCC in children and young adults has an overall good prognosis despite frequent high grade. It suggests that cervical LND may be of importance, but the value of systematic adjuvant therapy is not confirmed. These findings emphasize the importance and relevance of population-based studies in shaping clinical practice and informing the design of future prospective investigations.

References

Hussain T, Savariar E, Diaz-Perez J, Messer K, Pu M, Tsien R . Surgical molecular navigation with ratiometric activatable cell penetrating peptide for intraoperative identification and resection of small salivary gland cancers. Head Neck. 2014; 38(5):715-23. PMC: 4472578. DOI: 10.1002/hed.23946. View

Richard C, White S, Williams R, Zaghloul T, Helmig S, Sheyn A . Indocyanine green near infrared-guided surgery in children, adolescents, and young adults with otolaryngologic malignancies. Auris Nasus Larynx. 2022; 50(4):576-585. DOI: 10.1016/j.anl.2022.11.007. View

Coca-Pelaz A, Rodrigo J, Bradley P, Vander Poorten V, Triantafyllou A, Hunt J . Adenoid cystic carcinoma of the head and neck--An update. Oral Oncol. 2015; 51(7):652-61. DOI: 10.1016/j.oraloncology.2015.04.005. View

Oplatek A, Ozer E, Agrawal A, Bapna S, Schuller D . Patterns of recurrence and survival of head and neck adenoid cystic carcinoma after definitive resection. Laryngoscope. 2009; 120(1):65-70. DOI: 10.1002/lary.20684. View

SPIRO R . Salivary neoplasms: overview of a 35-year experience with 2,807 patients. Head Neck Surg. 1986; 8(3):177-84. DOI: 10.1002/hed.2890080309. View

Hofmann E, Priebe J, Rieke D, Doll C, Coordes A, Olze H . Prognostic factors in surgically treated malignant salivary gland tumors. Oral Oncol. 2023; 144:106484. DOI: 10.1016/j.oraloncology.2023.106484. View

Ferrarotto R, Heymach J . Taking it up a NOTCH: a novel subgroup of ACC is identified. Oncotarget. 2017; 8(47):81725-81726. PMC: 5669839. DOI: 10.18632/oncotarget.20879. View

Merkow R, Rademaker A, Bilimoria K . Practical Guide to Surgical Data Sets: National Cancer Database (NCDB). JAMA Surg. 2018; 153(9):850-851. DOI: 10.1001/jamasurg.2018.0492. View

Eveson J, CAWSON R . Salivary gland tumours. A review of 2410 cases with particular reference to histological types, site, age and sex distribution. J Pathol. 1985; 146(1):51-8. DOI: 10.1002/path.1711460106. View

10.

Perez D, de Abreu Alves F, Nishimoto I, de Almeida O, Kowalski L . Prognostic factors in head and neck adenoid cystic carcinoma. Oral Oncol. 2005; 42(2):139-46. DOI: 10.1016/j.oraloncology.2005.06.024. View

11.

Geiger J, Ismaila N, Beadle B, Caudell J, Chau N, Deschler D . Management of Salivary Gland Malignancy: ASCO Guideline. J Clin Oncol. 2021; 39(17):1909-1941. DOI: 10.1200/JCO.21.00449. View

12.

Scarpa A, Viola P, Ralli M, Gioacchini F, Salzano G, Stadio A . Post-operative radiotherapy in adenoid cystic carcinoma of salivary glands versus surgery alone: what is the evidence about survival and local control? A systematic review and meta-analysis. Eur Arch Otorhinolaryngol. 2023; 281(2):563-571. DOI: 10.1007/s00405-023-08252-x. View

13.

Morlandt A, Moore L, Johnson A, Smith C, Stevens T, Warram J . Fluorescently Labeled Cetuximab-IRDye800 for Guided Surgical Excision of Ameloblastoma: A Proof of Principle Study. J Oral Maxillofac Surg. 2020; 78(10):1736-1747. PMC: 7541684. DOI: 10.1016/j.joms.2020.05.022. View

14.

Chen S, Liao C, Lin C, Chang J, Lai Y . Distress and care needs in newly diagnosed oral cavity cancer patients receiving surgery. Oral Oncol. 2009; 45(9):815-20. DOI: 10.1016/j.oraloncology.2009.01.001. View

15.

Chen A, Bucci M, Weinberg V, Garcia J, Quivey J, Schechter N . Adenoid cystic carcinoma of the head and neck treated by surgery with or without postoperative radiation therapy: prognostic features of recurrence. Int J Radiat Oncol Biol Phys. 2006; 66(1):152-9. DOI: 10.1016/j.ijrobp.2006.04.014. View

16.

Ferrand F, Even C, Chabrillac E, Thariat J, Fakhry N, Vergez S . Systemic therapies for salivary gland cancer: Adenoid cystic carcinoma. REFCOR recommendations by the formal consensus method. Eur Ann Otorhinolaryngol Head Neck Dis. 2023; 141(5):286-291. DOI: 10.1016/j.anorl.2023.11.009. View

17.

Tasoulas J, Divaris K, Theocharis S, Farquhar D, Shen C, Hackman T . Impact of Tumor Site and Adjuvant Radiotherapy on Survival of Patients with Adenoid Cystic Carcinoma: A SEER Database Analysis. Cancers (Basel). 2021; 13(4). PMC: 7913220. DOI: 10.3390/cancers13040589. View

18.

Amit M, Binenbaum Y, Sharma K, Ramer N, Ramer I, Agbetoba A . Analysis of failure in patients with adenoid cystic carcinoma of the head and neck. An international collaborative study. Head Neck. 2013; 36(7):998-1004. DOI: 10.1002/hed.23405. View

19.

Ettl T, Schwarz-Furlan S, Gosau M, Reichert T . Salivary gland carcinomas. Oral Maxillofac Surg. 2012; 16(3):267-83. DOI: 10.1007/s10006-012-0350-9. View

20.

Martins-Andrade B, Costa S, SantAna M, Altemani A, Vargas P, Fregnani E . Prognostic importance of the lymphovascular invasion in head and neck adenoid cystic carcinoma: A systematic review and meta-analysis. Oral Oncol. 2019; 93:52-58. DOI: 10.1016/j.oraloncology.2019.04.014. View