k Pediatric Salivary Gland Pathology 401 (a) (b) k k (c) (d) Figure 15.9. An 8-year-old girl (a) with left submandibular swelling that had been present for 6 months. Imaging with CT identified an ill-defined mass of the submandibular gland region (b) as well as hydrocephalus (c). Oral examination (d)showed fullness in the left sublingual space. With a differential diagnosis of neurofibroma the patient underwent a debulking of this lesion (e and f). Histopathology identified a plexiform neurofibroma (g), indicative of neurofibromatosis. The patient did well postoperatively as noted at her 2-year visit (h and i). No reaccumulation of the tumor was noted on physical examination. k k 402 Chapter 15 (e) (f) k k (g) (h) (i) Figure 15.9. (Continued) k k Pediatric Salivary Gland Pathology 403 PAROTID TUMORS of which occurred in children. Fifty-seven (71%) of these tumors developed in the parotid gland. In their review of the Salivary Gland Register from 1965–1984 at the University of Hamburg, Seifert, Pleomorphic adenoma accounts for virtually all of et al. (1986) reported on 9883 cases of salivary the benign pediatric neoplasms occurring in the gland pathology, including 3326 neoplasms, 80 parotid gland with the Warthin tumor representing k k (a) (b) (c) (d) Figure 15.10. A 9-year-old boy (a) with a mass of the left hard-soft palate junction (b). Incisional biopsy identified low-grade mucoepidermoid carcinoma. Imaging with CT (c) showed no bone erosion of the hard palate such that the patient underwent wide local excision of his cancer (d). Final histopathology (e) identified intermediate grade mucoepidermoid carcinoma. (Hematoxylin and eosin, original magnification ×100.) k k 404 Chapter 15 SUBMANDIBULAR GLAND TUMORS As in adults, submandibular gland tumors are very uncommon in pediatric patients. Of 168 sali- vary gland tumors in the series of Krolls, et al. (1972), submandibular gland tumors included 3 lymphomas, 4 mucoepidermoid carcinomas, and 10 benign tumors. While there were 12 acinic cell carcinomas in this series, none occurred in the submandibular gland. Lack and Upton (1988) similarly identified their five cases of acinic cell carcinoma exclusively present in the parotid gland, but they identified no cases of mucoepidermoid carcinoma in the submandibular gland. These authors identified 4 of 10 cases of pleomorphic (e) adenoma occurring in the submandibular gland. In Figure 15.10. (Continued) Castro’s series of 38 major salivary gland tumors, 5 were noted to occur in the submandibular gland. a minor contribution at this anatomic site. In 166 These included four cases of pleomorphic ade- children with epithelial parotid tumors, 93 (55%) noma and one case of malignant mixed tumor. were benign and 73 (45%) were malignant (Ord The treatment of submandibular gland tumors in 2004). Of the 73 malignancies, 47 (64%) were children includes excision of the submandibular mucoepidermoid carcinomas and 17 (23%) were gland and tumor en bloc. acinic cell carcinomas. This relative incidence of mucoepidermoid carcinomas and acinic cell MINOR SALIVARY GLAND TUMORS k carcinomas of the parotid gland in children is k distinguished from the near equal incidence of Minor salivary gland tumors in children are rare. these malignancies in the parotid gland in adult Population studies have demonstrated that only 5% patients in the AFIP data (Ellis, et al. 1991). Finally, of minor salivary gland tumors occur in children, although the parotid gland is a less common site with a near equal distribution of benign and malig- for salivary gland tumors in children compared to nant tumors (Galer, et al. 2012). In their study of adults, there is a greater chance for malignancy in 35 minor salivary gland tumors in children, Galer, children. et al. (2012) identified 22 cases of mucoepidermoid The workup of a child with a parotid swelling carcinoma, 9 cases of adenoid cystic carcinoma, does not differ significantly from that of an adult. and 4 cases of adenocarcinoma. Thirty-one of the Once inflammatory disease has been ruled out, a malignancies were low-intermediate grade. Twenty fine needle aspiration biopsy of a discrete parotid of the tumors were classified as occurring in the mass in a child is useful to establish its cytologic oral cavity and 13 were classified as occurring in character. Structural imaging is also valuable so the hard palate. These authors found an excellent as to establish the anatomic extent of the tumor. prognosis for their patients with an overall survival Superficial parotidectomy or partial superficial of 88.4% and disease free survival of 89.3% at parotidectomy with facial nerve identification and 5 years. Preferred treatment is identical to that preservation are the procedures of choice for pedi- of adult’s diagnosis for diagnosis. In the author’s atric parotid tumors. Facial nerve sacrifice is only unpublished series of 275 minor salivary gland performed when preoperative palsy is noted to tumors, 12 (4.3%) were pediatric cases (age 9–19). exist or when nerve invasion is appreciated intra- Ten cases were malignant (83.3%), of which 7 operatively. The facial nerve is more superficial in (70%) were mucoepidermoid carcinomas and 9 of infants younger than 4 months of age compared the 12 tumors was located in the palate (75%). to older children due to the lack of development A mucoepidermoid carcinoma of the palate of the mastoid process (Ord 2004). Neck dissec- in a child should undergo structural imaging and tion is performed for high-grade malignancies excision of the tumor with appropriate anatomic or when evidence of metastatic adenopathy is barrier inclusion on the deep aspect of the tumor present. (Figure 15.10). Unless the bone is clinically or k k Pediatric Salivary Gland Pathology 405 (a) (b) k k (c) Figure 15.11. A 12-year-old girl (a) with a long history of a slowly growing mass of the palate (b). Axial T2 weighted MRI (c) identifies a hyperintense multilobulated mass consistent with a pleomorphic adenoma that was confirmed by incisional biopsy. Source: Reproduced with permission of John Caccamese. k k 406 Chapter 15 radiologically involved the bone does not require in infant and its review. Case Reports in Dentistry,article resection for low grade mucoepidermoid carci- ID 723130. noma in children (Caccamese and Ord 2002). The Bradley PJ, Hartley B. 2011. Salivary Gland Neoplasms. In: periosteum appears to be an effective oncologic Bradley PJ, Guntinas-Lichius O (eds), Salivary Gland barrier and its inclusion on the deep surface of the Disorders and Diseases: Diagnosis and Management. tumor permits a margin-free cancer surgery with Stuttgart, Thieme, p. 94. Caccamese JF, Ord RA. 2002. Pediatric mucoepidermoid high rates of cure. carcinoma of the palate. Int J Oral Maxillofac Surg The surgical management of benign palatal 31(2):136–139. salivary gland tumors, like that of malignant sali- Canzi P, Occhini A, Pagella F, Marchal F, Benazzo M, vary gland tumors is identical to those surgeries et al. 2013. Sialendoscopy in juvenile recurrent parotitis; performed in adult patients (Figure 15.11). Specifi- a review of the literature Acta Otorhinolaryngol Ital cally, the inclusion of the periosteum on the deep 33(6):367–373. surface of the tumor provides effective cure of Castro EB, Huvos AG, Strong EW, Foote FW. 1972. these benign tumors. The superiorly located max- Tumors of the major salivary glands in children. Cancer illary bone need not be included with the tumor 29:312–317. specimen. Chiaravali S, Guzzo M , Bisogno G, De Pasquale MD, Migliorati R, De Leonardis F, Collini P, Casanova M, Cecchetto G, Ferrari A. 2014. Salivary gland carcinomas in children and adolescents: The Italian TREP project Summary experience. Pediatr Blood Cancer 61(11):1961–1968. Chitre VV, Premchandra DJ. 1997. Recurrent parotitis. Arch • Salivary gland lesions are rare in the pediatric Dis Child 77:359–363. population that is variably defined in terms of Chung MK, Jeong HS, Ko MH, Cho HJ, Ryu NG, Cho DY, Son age. YI, Baek CH. 2007. Pediatric sialolithiasis: What is differ- • The mucocele is the most common salivary ent from adult sialolithiasis? Int J Pediatr Otorhinolaryngol k gland lesion encountered in pediatric patients. 71(5):787–791. k • Mumps has historically been the most com- Craver RD, Carr R. 2012. Paediatric salivary gland pathology. mon form of sialadenitis diagnosed in children. Diagn Histo 18:373–380. • Chronic recurrent parotitis is the second most Drolet BA, Esterly NB, Frieden IJ. 1999. Hemangiomas in common form of sialadenitis in children. children. NEnglJMed341:173–181. • The pleomorphic adenoma is the most com- Ellies M, Laskawi R. 2010. Diseases of the salivary glands in infants and adolescents. Head & Face Medicine 6:1–7. mon pediatric salivary gland tumor and the Ellis GL, Auclair PL, Gnepp DR. 1991. Surgical Pathology most common benign tumor in children. of the Salivary Glands. Philadelphia, WB Saunders Co., Together with the hemangioma, these benign Ch. 9. tumors account for nearly 90% of all benign Enjolras O, Rich MC, Merland JJ, Escarde JP. 1990. Manage- salivary gland tumors in children. ment of alarming hemangiomas in infants: a review of 25 • The mucoepidermoid carcinoma is the most cases. Pediatrics 25:491–498. common malignant pediatric salivary gland Ezekowitz RAB, Mulliken JB, Folkman J. 1992. Interferon tumor. Together with the acinic cell carci- alfa-2a therapy for life-threatening hemangiomas of noma, these two malignant tumors account infancy. NEnglJMed326:1456–1463. for approximately 60% of malignant salivary Fang QG, Shi S, Li ZN, Zhang X, Liu FY, Sun CE. 2013. gland tumors in children. Epithelial salivary gland tumors in children; a twenty-five year experience of 122 patients. Int J Pediatr Otorhino- laryngol 77:1252–1254. Galer C, Santillan AA, Chelius D, et al.