Abstract
Squamous cell carcinomas (SCCs) are amongst the commonest malignancies in adults but in paediatric patients are exceptionally rare, particularly those involving the oral mucosa. The aim of the present report is to describe the features of a gingival well-differentiated SCC in a 7-year-old Brazilian boy. Immunostaining for p53, Ki-67 and Mcm2 showed increased cellular proliferation compared with normal epithelium. In situ hybridization failed to identify human papilloma virus infection. Correct diagnosis of well-differentiated squamous carcinoma can be difficult in children and differentiation from pseudoepitheliomatous hyperplasia is essential to establish proper treatment.
Oral squamous cell carcinomas (OSCCs) are amongst the commonest malignancies in adults and are considered to be age-related . Patients younger than 40 years account for less than 4% of oral malignancies and only a small subset of this tumour occurs in paediatric patients . OSCC in children may be considered a distinct entity possibly with a unique aetiology and clinical progression. Paediatric OSCC can be related to immunosuppressive therapy, viral infection, malnourishment, passive smoking and late complications of chemo/radiotherapy . Genetic conditions such as KID syndrome (keratitis, ichthyosis, and deafness), xeroderma pigmentosum, Fanconi’s anaemia and Li Fraumeni syndrome have also been associated with the occurrence of carcinoma in young individuals . The aetiology of paediatric OSCC should not ignore the competitive trauma .
Clinical features of paediatric OSCC are less suggestive of the disease compared with the presentation in adults, and eventually can mimic proliferative inflammatory lesions, which sometimes assume neoplasm-like characteristics . The tongue and gingiva have been suggested to be the main sites of occurrence, whilst in adults the tongue and floor of the mouth are the regions most affected. Metastasis is not regular in this subset of patients, possibly supported by the lower mitotic index observed in the tumours of younger patients . The aim of this paper is to report a case of gingival SCC in a male child treated with surgery.
Case report
A 7-year-old boy was referred by his paediatric dentist for treatment of a gingival hyperplasia with approximately 3 months of duration. The patient was otherwise healthy. Extraoral examination was unremarkable and no palpable lymph nodes were observed. Intraoral examination revealed a nodular ulcerated lesion on the left maxilla, extending from the lateral incisor to the second deciduous upper molar, which presented grade 3 mobility ( Fig. 1 A) . An orthopantomogram revealed a slightly radiolucent lesion, causing alveolar bone destruction and occlusal radiography showed a small multilocular area on the palate ( Fig. 1 B). The clinical diagnosis included a reactive lesion (pyogenic granuloma), Langerhans histiocytosis, lymphoma and SCC. An incisional biopsy was performed under local anaesthesia and the specimen was sent for microscopic evaluation.
After tissue processing, the slides were stained routinely and histopathological analysis revealed an intense epithelial hyperplasia associated with abundant keratinization ( Fig. 2 A) . Islands of neoplastic epithelial cells infiltrating the subjacent connective tissue with scarce mitotic figures were observed. A diagnosis of well-differentiated OSCC was made and additional image studies were requested for staging the disease. Nasofibroscopy, gastric endoscopic examination, plain radiography of the thorax, CT and magnetic resonance imaging failed to show evidence of regional or distant metastases. The tumour was clinically staged as T3N0M0 and a partial maxillectomy was the treatment of choice. A surgical specimen revealed similar aspects to the biopsy, confirming the diagnosis of OSCC. Considering the unknown clinical behaviour of the tumour, immunohistochemical reactions for p53 (DO7, 1:200, Dako A/S, Denmark) and proliferation markers Ki-67 (MIB-1, 1:200, Dako S/A, Denmark) and Mcm2 (NCL-MCM2, 1:50, Novocastra, UK) were performed. p53 was slightly positive on few basal cells ( Fig. 2 B). Ki-67 and Mcm2 were positive on the basal and suprabasal cells, with the last also being positive in the central portion of the tumoural islands ( Fig. 2 C and D). In situ hybridization for human papilloma virus (HPV) was negative (Wide Spectrum, HPV Probe, Dako S/A, Denmark). The patient has been followed up for 18 months and no signs of recurrence have been observed. A removable palatal obturator was manufactured and subsequent substitutions will be made at the end of the growth phase, when an implant-based oral rehabilitation will be performed.
Discussion
OSCC is extremely rare in children . To the best of the authors’ knowledge, only 73 and 9 cases have been reported in patients younger than 20 and 12 years, respectively ( Table 1 ). Six of these patients were male and 3 were female, yielding a male:female ratio of 2:1. Site distribution presented a unique pattern; gingival lesions accounted for six and tongue lesions for two cases. Gingival OSCC in adults is uncommon, but it is the predominant site in children under 12 years.
| Authors | Patients | Gender | Age | Site |
|---|---|---|---|---|
| Present case | 1 | M | 7 | Maxillary gingiva |
| M ehanna et al. | 1 | M | 10 | Mandibular gingiva |
| S tolk -L iefferink et al. | 1 | M | 11 | Maxillary gingiva |
| A jayi et al. | 1 | M | Unknown | Mandible |
| B inahmed et al. | 1 | F | 10 | Maxillary alveolus |
| T hompson et al. | 20 | M (10) F (10) |
2–20 | Tongue = 9 Lip = 6 Unknown = 5 |
| C arvalho et al. | 1 | F | 6 | Maxillary gingiva |
| K eukens et al. | 1 | M | 9 | Tongue |
| E arle et al. | 1 | M | 7 | Gingiva |
| Y agi et al. | 1 | F | 10 | Tongue |
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