ORIGINAL ARTICLE

Non-hematological tumors of head and neck regionin the pediatric age group in a tertiary care cancer center

Kunal Das • Sandeep Jain • Akanksha Chichra •

Himesh Gupta • Gauri Kapoor

Accepted: 13 April 2011 / Published online: 5 May 2011

� Springer-Verlag 2011

Abstract

Purpose To analyze the clinical profile, management and

outcome of non-hematological tumors in head and neck

region in children less than 18 years of age.

Patients and methods Medical records of patients

(0–18 years) presenting with non-hematological tumors of

head and neck region from 2000 to 2010 were reviewed.

Result Out of the 78 patients identified, 50 were males

with median age of 14 years (3 months to 18 years). His-

tologically, 58 out of the 78 patients had malignant tumors

and 20 were metastatic at presentation. Common site of

tumor origin was noted as thyroid (23/78), salivary glands

(21/78) and nasopharynx (13/78). Out of 78 patients, 44

patients were treated by surgery, 26 patients received

radiation and 13 patients received chemotherapy, while 9

patients received radio-iodine (I-131) treatment. Median

follow up of all cases was 22 months (range 1 month to

8 years). The event free survival was 74.1% and overall

survival was 89.6%. Subset analysis revealed patients with

thyroid and salivary gland malignancies have excellent

survival, while those with squamous cell carcinoma and

high risk neuroblastoma had poor outcome.

Conclusion This study reveals the interesting profile of

head and neck tumors in children, and important role of

surgery for favorable outcome.

Keywords Non-hematological � Tumor � Head � Neck �Pediatric

Introduction

Masses in the head and neck region in children may be

congenital, inflammatory, or neoplastic. Majority of these

are non-malignant [1] but it is essential to have knowledge

of these neoplastic lesions to enable early diagnosis and

cure. The most common masses seen in the head and neck

region in the pediatric age group are lymph node masses,

which are usually inflammatory or neoplastic. Amongst the

neoplastic conditions, hematological malignancies, i.e.

lymphoma and leukemia are the most frequent, while

tumors arising from thyroid, salivary glands, nasopharynx,

bone and soft tissue are not uncommon. There is paucity of

data on the profile of non-hematological tumors of the head

and neck region in the pediatric age group. Hence, this

study was undertaken to analyze the spectrum of these

tumors at a tertiary care cancer center.

Materials and methods

This is a retrospective study of patients registered between

January 2000 and January 2010 at our Institute with non-

hematological head and neck masses. After obtaining

institutional review board approval, clinical charts of all

the patients in the 0–18 year age group presenting with

non-hematological head and neck masses were retrieved.

The medical records of these patients were reviewed and

analyzed in detail with regard to demographic profile,

clinical features, diagnosis, management and outcome. The

K. Das � S. Jain � A. Chichra � H. Gupta � G. Kapoor (&)

Rajiv Gandhi Cancer Institute and Research Center, Delhi, India

e-mail: gauri_kapoor2000@yahoo.com

123

Pediatr Surg Int (2011) 27:919–923

DOI 10.1007/s00383-011-2916-2

overall (OS) and event free survival (EFS) were plotted by

the Kaplan–Meier method.

Results

During the study period, 78 patients were identified with

non-hematological tumors in the head and neck region. The

median age of presentation was 14 years (range 3 months–

18 years) and male to female ratio was 1.7. The histolog-

ical spectrum of these 78 patients is displayed in Table 1.

The most common site involved was thyroid gland

(n = 23) and papillary carcinoma was the most common

pathology noted in these patients. Fifty-eight of the total 78

patients were diagnosed to have malignant disease. Twenty

out of these 58 patients with malignant tumors were met-

astatic at presentation. Fifty-eight out of 78 patients took

treatment at our institute which included surgery, radiation

therapy, chemotherapy or a combination of these, while 20

patients came for a second opinion and were treated at

other centers. Treatment details of these patients are shown

in Table 2. All the patients who took treatment for

pathology of thyroid gland, salivary glands and benign

lesions primarily required surgery for excision of the

tumor. All the neoplasms of the salivary gland and 13/23

cases of thyroid tumor were diagnosed with preoperative

FNAC (fine needle aspiration cytology), which was the

basis of the type of surgery performed. FNAC diagnosis of

thyroid neoplasms correlated with the final diagnosis. One

patient with parotid gland tumor had an FNAC diagnosis of

pleomorphic adenoma, which was later confirmed to be

mucoepidermoid carcinoma after superficial parotidectomy

and underwent total parotidectomy subsequently. There

were 13 patients with nasopharyngeal carcinomas and 5 of

these 13 were metastatic to cervical nodes at presentation.

The diagnosis was established on the basis of histopath-

logical finding of the cervical node in all these patients.

Chemoradiation was the main modality of treatment. Eight

patients presented with tumors arising from bone (n = 5,

mandible; n = 3, maxilla). The follow-up ranged from 1 to

99 months (median 22 months).

The OS and EFS curves of patients with tumors of

thyroid gland, salivary glands, nasopharyngeal carcinoma

are shown in Figs 1 and 2.

Discussion

Non-hematological neoplasms of head and neck region are

not seen very commonly in general pediatric surgery

practice as only 5% of childhood cancers arise in this

region [2]. The implications of missing diagnosis of a

malignant disease and consequences of delayed or inap-

propriate management may be severe. This review provides

the clinical profile and management of non-hematological

tumors of head and neck region in pediatric age group in a

tertiary care cancer center.

Out of 78 patients with head and neck masses, 74.3%

(58) were diagnosed with malignant disease, and this was

significantly higher when compared to that reported by

Sato et al. [3] (7%) from a general pediatric surgical

practice in Tokyo, which may be due to selective referral of

cancer patients to our tertiary center cancer care hospital.

Table 1 Histological profile of tumors in patients of age group

0–18 years with non-hematological head and neck malignancies

Tumor n = number

of cases (%)

Thyroid 23 (29.4)

Papillary carcinoma 19 (24.4)

Medullary carcinoma 3 (3.8)

Adenocarcinoma 1 (1.2)

Salivary glands 21 (26.9)

Parotid 19 (24.4)

Pleomorphic adenoma 13 (16.7)

Mucoepidermoid carcinoma 3 (3.8)

Acinic cell cancer 1 (1.2)

Hemangioma 1 (1.2)

Angiosarcoma 1 (1.2)

Submandibular gland—pleomorphic adenoma 1 (1.2)

Palatal gland—pleomorphic adenoma 1 (1.2)

Nasopharynx—carcinoma 13 (16.7)

Rhabdomyosarcoma 7 (8.9)

Squamous cell carcinoma 5 (6.4)

Neuroblastoma 2 (2.5)

Jaw bone 7 (8.9)

Mandible

Ameloblastoma 2 (2.5)

Ewings sarcoma 1 (1.2)

Non ossifying fibroma 1 (1.2)

Maxilla

Spindle cell tumor 1 (1.2)

Benign melanocytic tumor 1 (1.2)

Giant cell tumor 1 (1.2)

Table 2 Treatment modalities used for pediatric non-hematological

head and neck malignancies

Treatment

modality

n = number

of cases

Surgery 44

Radiotherapy 26

Chemotherapy 13

Radio-iodine 9

920 Pediatr Surg Int (2011) 27:919–923

123

In the present study, patients with thyroid neoplasm

comprised 29.4% of all patients. Gosepath et al. [4] noted a

higher incidence of thyroid tumor (34.8%) in children with

non-hematological head and neck malignancies in the data

based on German childhood cancer registry (1994–2003).

This may be attributed to the inclusion of patients with

benign tumors in our study. The median age of presentation

in our study was 17 years (range 7–18 years). Thirteen

patients were diagnosed with FNAC which correlated with

their final diagnosis. Clinically 11 (47.8%) patients pre-

sented with cervical lymph node metastasis which was

comparable to that reported by Danese et al. [5] (50%)

from Italy and Kumar et al. [6] (64%) from India. All the

cases were malignant and none were benign; papillary

carcinoma was the most common histological type noticed.

Papillary carcinoma was also noted as the most common

thyroid tumor in a study of European children and ado-

lescents by Steliasova-Foucher et al. [7] and by Kumar

et al. [6] in another study of thyroid neoplasms from India.

Of note, no patient was found to have a benign disease of

thyroid, although the incidence of malignancy in thyroid

nodule in several other studies is noted as 17–36% [8–11];

this may be due to selective referral to a tertiary care cancer

hospital. Surgery with or without I-131 remained the

mainstay of treatment. Six patients underwent total thy-

roidectomy while three were treated with near total thy-

roidectomy and one patient underwent hemithyroidectomy.

Patients with thyroid tumor showed good clinical outcome

and only 1 out of 23 patients showed recurrence while no

mortality was noted in a median follow-up of 45.5 months,

which is comparable to an analysis of cancer registries of

20 European countries by Gatta et al. [12], who also noted

5-year survival of 96.9% among pediatric (\15 years)

patients with thyroid cancer. An Indian study by Kumar

et al. [6] also noted 97.5% survival in pediatric thyroid

tumor.

Salivary gland tumors constituted the second common

group in our study and were also noted in (26.9%) patients.

This is higher than the incidence reported by Alam et al.

[13], who found that salivary gland neoplasm constituted

10% (10/99) of non-hematological head and neck tumors in

pediatric age group in a multispeciality teaching hospital in

India. The median age of patients was 13 years (range

2–18 years). Nineteen out of the 21 patients with salivary

gland tumors had involvement of parotid gland while

submandibular gland and minor salivary glands were

involved in one patient each. The most common histology

noted in our study was pleomorphic adenoma. In a study by

Guzzo et al. [14], parotid gland was involved in 41(79%)

patients out of total 57 patients attending a multi-speciality

hospital in Italy with salivary gland neoplasms, and 32

(78%) out of the 41 patients with parotid gland neoplasm

were pleomorphic adenoma Amongst all salivary gland

tumors, 16 patients had benign tumors while 5 had

malignant tumors. Out of 16 patients with benign tumor, 15

had pleomorphic adenoma ,while 1 had hemangioma. Out

of five patients with malignant tumor, three patients had

mucoepidermoid carcinoma while one patient each had

acinic tumor and angiosarcoma. In a review of 52 patients

in pediatric age group with salivary gland tumors, Ellies

et al. [15] also noted 40 patients with benign pathology and

pleomorphic adenoma as the most common histopathology

(60%), while mucoepidermoid carcinoma was the most

common histopathology, noted in 4 out of 12 patients with

malignant tumors (33%). However, Ribeiro et al. reported

71% (27/38) patients with malignant pathology in salivary

gland tumors in a study of pediatric age group from a

tertiary cancer center of Brazil. They also noted

Fig. 2 Kaplan–Meier curve showing event free survival

Fig. 1 Kaplan–Meier curve showing overall survival

Pediatr Surg Int (2011) 27:919–923 921

123

mucoepidermoid carcinoma as the most common malig-

nant tumor (17/27) and pleomorphic adenoma as the most

common benign tumor (7/11) [16]. All the patients were

treated with surgery and 1 patient with submandibular

gland neoplasm also required radiotherapy. No mortality

was noted on a median follow-up of 16.5 months. Ellies

et al. [15] also noted 92.3% survival, while Ribeiro et al.

[16] noted 81.6% survival in pediatric salivary gland

tumors.

In our study, 16.6% (13/78) patients were diagnosed as

nasopharyngeal carcinoma. This is comparable to a pedi-

atric (\19 years) study by Cunningham et al. [17], who

found 12 (12.2%) patients with nasopharyngeal carcinoma

out of 98 patients with non-hematological head and neck

malignancies. Median age at presentation was 15 years

(range 8–19 years). Five patients had metastatic disease at

presentation, with metastases limited to neck nodes. Higher

incidences of cervical node involvement in pediatric

nasopharyngeal carcinoma are reported from Pittsburg [17]

and from India [18]. Clinically the nodes were harder in

consistency than the common presentation of benign or

hematolymphoid malignancy. Seven patients were treated

with chemo-radiation and two were treated with radio-

therapy. Chemotherapy and radiation remained the treat-

ment of choice indicating higher incidence of metastasis

and loco regional spread of cancer. Out of eight patients

who completed treatment, six (75%) were alive without

disease with median follow-up of 17 months, which is

comparable to a study done by EUROCARE working

group (84%) [12]. One patient who was treated with che-

moradiotherapy developed a stricture in proximal esopha-

gus which was successfully managed with repeated

endoscopic dilations. There is increased concern of

development of secondary malignancies in pediatric

patients treated for nasopharyngeal carcinoma as compared

to adults [19, 20], although in our study no patient devel-

oped second malignancy. This may be attributed to short

follow-up of our study.

Seven out of 78 patients analyzed were diagnosed as

rhabdomyosarcoma. The median age at presentation was

4 years (range 2–18 years). Primary site was paramenin-

geal in 5/7 patients and non parameningeal in remaining 2.

SEER data analysis by Albright et al. [21] also revealed

comparable incidence of rhabdomyosarcoma of head and

neck region in pediatric patients, although they noted non-

parameningeal site as the more common one (72%). In our

study, all seven patients had embryonal histology which

was comparable to that reported by Cunningham et al.

(embryonal 27/31) in similar age group.

Squamous cell carcinoma was present in 5 patients out

of total 78 patients. Median age of presentation was

17.5 years. Surgery was the mainstay of treatment per-

formed in three patients. It is important to note that all the

patients of squamous cell carcinoma were in adolescent age

group (range 15–18 years) and carried worse prognosis.

Squamous cell carcinomas in pediatric patients are reported

to have worse prognosis in various case reports [22–24].

Out of 78 patients, 2 patients were diagnosed with Stage

4 neuroblastoma, which were stratified as high risk owing

to their age more than 18 months and metastases to bone at

presentation. Both these patients died due to progressive

disease. The survival of high risk neuroblastoma is notably

poor in the literature [25, 26].

Out of 78 patients, 7 patients had tumor involving bones

(mandible, n = 4; maxilla, n = 3). Out of the four patients

having mandibular tumor, three patients had benign tumors

(ameloblastoma, n = 2; non-ossifying fibroma, n = 1).

One patient had ESFT of mandible. Sato et al. [3] also

noted benign pathologies were more common (83.3%) in

head and neck bones and mandible being the predominant

site of origin (66.6%,72/108) with ameloblastoma as the

most common benign histopathological finding (37.5%) of

mandible. All four patients were treated with surgery and

showed good outcome. Out of seven patients with tumor

involving bone, three patients had maxilla involvement,

one case each with melanocytic tumor, giant cell tumor and

spindle cell tumor. All were treated with surgery and

showed good outcome.

Conclusion

Pediatric head and neck tumors have a varied clinical profile

and etiology. Thyroid gland, salivary glands and naso-

pharynx constituted the predominant sites of these tumors.

Surgery plays an important part in successful management

of these tumors. As these tumors involve varied organs, the

surgical management requires a team approach, which often

requires a plastic surgeon for reconstruction.

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