2017 Sec 1 Green Book

6.1. Major gland

Major salivary gland tumors appear to behave substantially

different than minor salivary gland tumors and therefore these

ndings are reported separately. For major gland malignancies, we

found that the majority of these tumors are low grade (66%) and

present at an early stage (80%). This may explainwhy the patients in

our series had an excellent prognosis with an overall disease spe-

c survival of 96%. Comparatively, the adult literature reports a

65% ten year overall survival

[1,8,9]

. Sultan et al. reviewed the

SEER database from 1974 to 2006 and likewise demonstrated that

pediatric salivary gland malignancies are generally less advanced

and have a better outcome than their adult counterparts

[1]

. Due to

the good overall survival in our series we were unable to identify

statistically signi

cant factors associated with poor prognosis.

However, Kupferman et al. found that age greater than 14 years,

non-Caucasian ethnicity, high-grade histopathology and perineural

invasion predicted for adverse survival

[7]

Based on the outcomes in our series, we believe that the ma-

jority of these patients can be managed with surgery alone. In fact,

only 11% of patients received adjuvant radiation. Complications

from radiation in our series included facial lymphedema, xero-

stomia, paresthesias, external auditory canal stenosis and arrested

mandibular growth requiring reconstructive surgery. Additionally,

two patients developed and died from acute myeloid leukemia

thought to be secondary to chemoradiation treatment received for

their salivary malignancy. Risk versus bene

t of radiation should be

carefully weighed in this population given the potential for

morbidity. We believe radiation therapy should be considered in

patients with positive margins, high grade tumors, advanced stage,

adverse pathologic factors (perineural spread, extracapsular

extension, vascular invasion) and bone or soft tissue invasion.

Over half (65%) of the patients with parotid tumors in our series

underwent total parotidectomy (TP). The most common indications

for TP in our series were deep lobe tumors or intermediate grade

mucoepidermoid carcinoma. Patients who underwent TP were

much less likely to recur than those who underwent enucleation or

super

cial parotidectomy (

Table 2

; 16% vs 64% and 40% respec-

tively). Based on our experience we recommend TP for deep lobe

tumors, high grade, positive intraparotid lymph nodes or positive

cervical lymph nodes. Enucleation of parotid tumors is strongly

discouraged. Patients that present to our institution who have

undergone enucleation alone at an outside facility are recom-

mended to undergo completion super

cial parotidectomy at a

minimum. We recommend resection of the facial nerve only if it is

grossly involved by tumor.

Locoregional recurrence in our series was 28%, similar to other

published series reporting 25

31%

[10,11]

. Recurrence was more

likely in patients with adverse pathologic features (vascular/peri-

neural invasion or extracapsular spread), who underwent enucle-

ation or super

cial parotidectomy as opposed to total parotidectomy

and patients with no neck dissection (

Table 2

). However, given the

limited sample size, the only factor that reached statistical signi

cance was enucleation versus total parotidectomy. Recurrence

occurred at a median time of 9.5 months and at a maximum time of

45 years. Therefore, we recommend at least yearly surveillance for a

prolonged period in this patient population.

Nodal metastasis is rare in pediatric salivary malignancies. The

majority of patients who underwent neck dissection in our series

had intermediate grade mucoepidermoid carcinoma and no pa-

tients were found to have positive lymph nodes. In the series by

Kupferman et al., only 17% of neck dissections specimens were

found to harbor positive nodes

[7]

. The two patients in our series

who developed cervical recurrences did not originally undergo

neck dissection. Therefore, despite the low occurrence of cervical

metastasis, we recommend neck dissection for patients with pos-

itive intraparotid lymph nodes, high grade histology, clinical or

radiographically suspicious lymph nodes, submandibular gland

pathology and T3/T4 tumors. Neck dissection should include levels

II and III for parotid tumors and level Ib for submandibular tumors

unless there is clinical or radiologic evidence of suspicious

lymphadenopathy outside of those regions.

There is scarce literature regarding long term outcomes of pa-

tients with pediatric parotid malignancies. In order to determine

Fig. 1.

Kaplan-Meier curve for recurrence free survival (Major gland). Total parotidectomy vs super

cial parotidectomy vs. enucleation of tumor.

C.C. Cockerill et al. / International Journal of Pediatric Otorhinolaryngology 88 (2016) 1

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