2017 Sec 1 Green Book

C.C. Cockerill et al. / International Journal of Pediatric Otorhinolaryngology 88 (2016) 1 e 6

were almost all after initial enucleation (10/14). Median time to recurrence in this group was 9.5 months (range 3 e 540 months). Two had a recurrence in the cervical lymph nodes that was treated with selective neck dissection. Neither of these patients had a neck dissection as part of their initial surgery. One patient with rhab- domyosarcoma developed lung metastases that were treated curatively with chemotherapy and radiation but ultimately died of treatment associated acute myeloid leukemia. Mean length of follow up for patients with major salivary gland malignancies was 13.5 years (range: 0.2 e 62.3). Two patients died of major salivary malignancy making the overall disease speci fi c survival 96%. Two patients died of acute myeloid leukemia thought to be secondary to chemoradiation treatment received for their salivary malignancy and two patients died of other causes. Information on factors associated with recurrence is presented in Table 2 . There was evidence that adverse pathologic factors (extrac- apsular spread, vascular invasion and or perineural spread), enucle- ation or super fi cial versus total parotidectomy, and no neck dissection increased the risk of recurrence. However, statistical signi fi cance was only seen in patients who underwent enucleation versus total paro- tidectomy (p value ¼ 0.005) ( Fig. 1 ). There was insuf fi cient evidence that low versus high grade pathologic types, T stage, and addition of adjuvant radiation was related to risk of recurrence. Based on retrospective chart review, the most common complication involved the facial nerve with 4 patients having complete facial paralysis, and another 4 patients with limited branch facial paralysis. Gustatory sweating (Frey's syndrome) was recorded in 5 patients. Other complications related to surgery included hypertrophic scar and major depression associated with appearance from facial paralysis. Complications associated with radiation included facial lymphedema, xerostomia, paresthesias, external auditory canal stenosis and arrested mandibular growth requiring reconstructive surgery. Two patients developed treat- ment related acute myeloid leukemia. 4.4. Factors associated with recurrence 4.5. Complications

4.6. Long term follow up survey data

An attempt was made to contact all 52 patients still alive at last follow up. Ultimately, 13 patients or parents of patients could be reached for a phone survey. Average follow up time for this cohort was 28.7 years (range 2.1 e 62.3 years). One hundred percent re- ported normal facial movement with no eye problems. One patient reported facial twitching or spasm despite not having any facial weakness after initial treatment. Over half (54%) described symp- toms of Frey's syndrome. All of these patients reported that their gustatory sweating symptoms never resolved and stated that the effect on their quality of life was a “ 1 ” on a scale of 1 e 10. Other reported long term side effects of treatment included facial numbness, change in ear position, speech impairment, dif fi - culty eating, chronic facial pain, need for long term feeding tube, dif fi culty whistling/blowing, excessive scarring and drooling (n ¼ 1 for all). Four patients that were treated with surgery alone reported excessively dry mouth. One patient reported a recurrence 45 years after being treated for mucoepidermoid carcinoma with surgery, radiation and chemotherapy.

5. Minor gland

There were 4 cases of minor salivary gland cancer (2 low grade mucoepidermoid, 1 high grade mucoepidermoid, 1 low grade adenocarcinoma). Three out of 4 patients suffered a local recur- rence and one of these had a cervical lymph node recurrence 3.5 years later. Two patients (high grade mucoepidermoid and adenocarcinoma) developed metastases and both died of their disease, which made an overall disease speci fi c survival of 50% for this group. Average length of follow up in this cohort was 6.9 years.

6. Discussion

We present our single institution experience treating pediatric salivary gland malignancies over a 62 year time period. Our series con fi rms that mucoepidermoid carcinoma is the most common histologic type in pediatric patients followed by acinic cell and adenoid cystic carcinoma [1,4,7] . An average age at presentation of 14 e 15 years also appears to be consistent across studies [1,7] .

Table 2 Factors associated with locoregional recurrence of parotid and submandibular gland tumors. Locoregional recurrence n/total n (%)

p-value

Low grade pathology a High grade pathology b

8/32 (25%) 5/18 (28%) 10/35 (29%) 3/10 (30%)

T1/T2 T3/T4

Adverse pathologic factors No adverse pathologic factors Positive intraparotid LN No positive intraparotid LN

2/5 (40%)

0.6

13/46 (28%)

0/4 (0%)

14/45 (31%) 7/11 (64%) 2/5 (40%) 5/32 (16%)

Enucleation

Super fi cial parotidectomy (SP)

Total parotidectomy (TP)

De fi nitive surgery

Enucleation vs. TP Enucleation vs. SP

0.005

0.15 0.10 0.09

SP vs. TP

Neck dissection

3/22 (14%) 12/30 (40%)

No neck dissection Adjuvant radiation No adjuvant radiation

1/6 (17%)

0.57

14/46 (30%)

LN: lymph nodes. a Low grade mucoepidermoid, acinic cell, lymphoma. b Intermediate and high grade mucoepidermoid, adenoid cystic, rhabdomyosarcoma, high grade synovial cell sarcoma.

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