,K.G.H.vanderWal S.A.H.Stolk-Liefferink ,A.G.Dumans ,E.H.vanderMeij *,P.P.Knegt Oralsquamouscellcarcinomainchildren;reviewofanunusualentity

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CASE REPORT

Oral squamous cell carcinoma in children;

review of an unusual entity

S.A.H. Stolk-Liefferink

^a

, A.G. Dumans

^a

, E.H. van der Meij

^a,

* , P.P. Knegt

^b

, K.G.H. van der Wal

^a

aDepartment of Oral and Maxillofacial Surgery, Erasmus University Medical Center, Rotterdam, The Netherlands

bDepartmant of Otorhinolaryngology and Head and Neck Surgery, Erasmus University Medical Center, Rotterdam, The Netherlands

Received 27 June 2007; received in revised form 28 August 2007; accepted 2 September 2007 Available online 29 October 2007

1. Introduction

Squamous cell carcinoma (SCC) of the oral cavity is rare in patients of age 40 and younger, being pri- marily a disease that occurs in males in their sixth or seventh decade. Younger patients (aged less than 40 years) account for approximately 4% of all oral cancers [9]. Only a small sample of this subgroup exists of pediatric patients (20 years), making oral SCC in the pediatric age group an extremely rare entity.

The purpose of the present report is to describe an 11-year-old boy who developed a SCC of the gingiva, and to review the literature of oral SCC in pediatric patients.

2. Case report

An 11-year-old boy was referred to the Department of Oral and Maxillofacial Surgery by his orthodontist because of a non-tender, progressive growing swelling of the gingiva of the upper front teeth, noticed by the patient since 6 weeks. The relevant medical history did not reveal any abnormalities. The patient did not use any medication at presentation.

Physical examination revealed a firm, non-tender, verrucous swelling with indurated borders and central ulceration on the buccal and palatal aspects

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KEYWORDS Oral;

Squamous cell carcimoma;

Maxilla

Summary Most commonly, squamous cell carcinoma (SCC) of the oral cavity presents during the fifth and sixth decades of life. Less than 4% of these cancers occur in patients younger than 40 years of age. Only a small sample of this subgroup exists of pediatric patients (20 years), making oral SCC in children an extremely rare entity.

An 11-year-old boy is presented who developed a SCC of the gingiva. The relevant literature of oral SCC in pediatric patients will be reviewed as well.

* Corresponding author at: Department of Oral and Maxillofa- cial Surgery, Erasmus University Medical Center, P.O. Box 2040, 3000 CA Rotterdam, The Netherlands. Tel.: +31 10 4634138;

fax: +31 10 4633098.

E-mail address:e.vandermeij@erasmusmc.nl (E.H. van der Meij).

doi:10.1016/j.ijporl.2007.09.006

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of the left upper front teeth measuring 2.5 cm by 2.0 cm (Fig. 1). The left upper incisors were mobile but tested positive on vitality tests. Multiple, non- tender, mobile submandibular lymph nodes were palpated bilaterally. On additional radiographic examination erosion, possibly infiltration of several millimeters of the alveolar bone between the two left upper incisors was observed. Histopathological examination of an incisional biopsy of the swelling showed a well-differentiated SCC. Because of the age of the patient there was some doubt about the diagnosis; a verruciform xanthoma with characteristics of pseudoepitheliomatous hyperplasia (PEH) was therefore considered as well (Fig. 2a).

A second biopsy specimen showed on microscopic examination again characteristics of a well-differentiated SCC (Fig. 2b). Based on fine needle aspira- tion cytology of the submandibular lymph nodes as well as a radiograph of the chest there were no signs of regional and/or distant metastasis. An additional computed tomogram revealed invasion of the buccal cortex of the left upper incisors (Fig. 3). Nineteen days after the initial presentation the tumor was diagnosed as a T4aN0M0 SCC of the gingiva of the left maxilla. The tumor was obviously grown in this short period (Fig. 4).

A partial maxillectomy was performed. The former diagnosis of a well-differentiated SCC was

confirmed on histopathological examination of the surgical specimen. The margins seemed to be free of tumor.

3. Discussion

Most commonly, SCC of the oral cavity presents during the fifth and sixth decades of life. Less than 4% of these cancers occur in patients younger than 40 years of age [9]. Only a small sample of this subgroup exists of pediatric patients (20 years), making oral SCC in children an extremely rare entity. The results of a literature review on oral SCC in pediatric patients from the period 1970—2005 have been summarized inTable 1 [1—5,7,8,10—18].

A total of 65 cases were found. There was no gender predilection. The majority of tumors occurred on the tongue, the lip or the gingiva. Patients’ ages ranged from 2 to 20 years with only four cases being

128 S.A.H. Stolk-Liefferink et al.

Fig. 2 (a) A biopsy specimen from the swelling shows histopathological features of a well differentiated SCC. A verruciform xanthoma with characteristics of pseudoepitheliomatous hyperplasia (PEH) was considered as well (haematoxylin—eosin, 10). (b) A second biopsy specimen confirming the former diagnosis; a well differentiated SCC (haematoxylin—eosin, 10).

Fig. 1 A firm, non-tender, verrucous swelling with indurated borders and central ulceration on the buccal and palatal aspects of the left upper front teeth measuring 2.5 cm by 2.0 cm (a + b).

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squamouscellcarcinomainchildren129

Table 1 Summary of studies and case reports reporting oral squamous cell carcinoma in pediatric patients (20 years) (1970—2005)

Authors Year Country No. of

patients

Gender (M/F)

Age (years) Site Histology (differentiation)

Byers[3] 1975 USA 4 Unknown 17, 19, 19, 19 Tongue, n = 4 Well, n = 1; moderate,

n = 1; poor, n = 1

Krolls and Hoffman[8] 1976 USA 19 Unknown 14, n = 3;

15—19, n = 16

Unknown Unknown

Harper and Copeman[5] 1981 UK 1 1/0 18 Tongue Poor

Yagi K et al.[18] 1981 Sudan 1 0/1 10 Tongue Well

McGregor et al.[11] 1983 Canada 1 0/1 18 Tongue Unknown

Newman et al.[12] 1983 USA 4 Unknown 14, 16, 18, 18 Tongue, n = 4 Unknown

Son and Kapp[15] 1985 USA 4 3/1 10, 17, 18, 19 Tongue, n = 2; cheek,

n = 1; gingiva, n = 1

Well, n = 4

Sacks et al.[13] 1985 USA 1 1/0 13 Gingival Well

Earle et al.[4] 1988 USA 1 1/0 7 Gingiva Moderate

Keukens et al.[7] 1989 The Netherlands 1 1/0 9 Tongue Well

Lund and Howard[10] 1990 UK 1 Unknown 20 Tongue Unknown

Tsukuda et al.[17] 1993 Japan 4 2/2 14—19, n = 4 Unknown Unknown

Sarkaria and Harari[14] 1994 USA 1 1/0 17 Tongue Unknown

Atula et al.[1] 1996 Finland 1 1/0 19 Tongue Unknown

Thompson et al.[16] 1999 USA 20 10/10 2—20 Tongue n = 9; lip n = 6; unknown n = 5 Well—poor

Bill et al.[2] 2001 USA 1 1/0 14 Gingiva Well

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younger than 12 years, making our case of an oral SCC in an 11-year-old boy unique.

Recently it has been observed that there is an increasing incidence of oral SCC in the younger population of several countries [6]. The role of traditional risk factors such as tobacco, alcohol, betel quid chewing, and low consumption of fruits and vegetables is unclear in this age group. Some studies have suggested that these patients may exhibit a predisposition to genetic instability.

There is a general trend in reported studies for SCC of the oral cavity in young patients to be particularly aggressive and carry a poorer prognosis than older patients[14]. A poorer prognosis in young patients could be due to (1) a differing disease etiology and tumor behavior or (2) delay in presentation and/or diagnosis (patients’ and/or doctors’

delay), the latter obviously playing a role in the currently presented case. The presentational and diagnostic delays may arise because of the reduced expectation of cancer in the young patient[6].

As inflammatory lesions in pediatric patients can become highly proliferative and assume neoplasm- like characteristics, the histopathologic diagnosis of SCC may present difficulties in this specific patient group. Pseudoepitheliomatous hyperplasia (PEH), a reactive process, may then be difficult to distinguish from SCC. Although differentiation between PEH and SCC in the adult patient group is generally not difficult for an experienced pathologist, PEH in pediatric patients may exhibit cellular atypia and irregular growth to such extend that this dis- tinction may be less obvious. The difficulties that were encountered in our patient in attempting to histopathologically distinguish between PEH and SCC are comparable with the difficulties seen in several previously reported case of SCC in pediatric patients[4,13].

Although rare, SCC does occur in pediatric patients and should therefore be included in the differential diagnosis of inflammatory-like, rapid growing lesions of the oral cavity. PEH may be difficult to histopathologically distinguish from SCC in this specific patient group. Close collabora- tion between clinician and pathologist is therefore from utmost importance to achieve the assessment of a proper diagnosis.

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