Oral medicine
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A mucoepidermoid carcinoma in a young man with intellectual disability: review of oral cancer in people with intellectual disability

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Oral tumors in patients with intellectual disabilities (ID) remain poorly documented, despite cancer incidence suggesting that malignancies are globally as frequent in this group as in the general population. A clinical case of a 36-year-old man with severe ID presenting with a mucoepidermoid carcinoma of intermediate grade in the right mandible is reported. Delayed diagnosis and problems managing complementary chemotherapy and radiotherapy are described. The literature review reported only 27 cases of malignant tumors in patients with ID. This finding indicates that oral tumors in patients with ID may be less frequent than in the general population, are usually diagnosed at an advanced stage, and may occur in patients who are younger than the general population. Diagnosis and treatment are difficult, implying a comprehensive knowledge of the underlying condition of each individual and the need for good communication skills to obtain patient cooperation, including an understanding of how the patient expresses pain.

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Case Report

A 36-year-old man with severe ID, living in an institution, was referred for a lump on the right posterior region of the mandible. He was the issue of a normal pregnancy and was in good health during infancy. At 18 months of age, he developed varicella encephalitis, which led to ID. At 6 years of age, he developed herpetic encephalitis, resulting in epilepsy and a regressive left hemiplegia. A lumbar scoliosis was surgically corrected during childhood. His parents were in good physical and

Review of the Literature

The search of the literature was conducted on PubMed using the words “cancer,” “carcinoma,” “sarcoma,” “lymphoma,” “malignant tumor,” “neoplasm,” “malignancy” and “intellectual disability,” “mental retardation,” “learning disability” and “oral region,” “mouth,” “lip,” and “tongue.” Additionally, one of us (MN) searched articles on oral cancer in PWIDs in the Japanese medical literature. These searches were conducted on the basis of a long-standing survey of the literature on cancer in PWIDs for

Frequency and risk factors

Oral cancer accounts for nearly 3% of all malignancies.26 In PWIDs, who correspond to 2% of the global population, we could find only 27 cases after an extensive search and long-standing follow-up of the literature. These cases are probably an underestimation because in some epidemiologic studies on cancer incidence and cancer mortality, oral tumors are pooled with pharyngeal tumors. Nonetheless, given that other cancers, particularly those of the digestive tract and those of the central

Conclusions

Oral tumors are rare in PWIDs and usually are found at an advanced stage and, in our review, in younger patients compared with the general population. The treatment of oral cancer in PWIDs is difficult, requiring good knowledge of the underlying condition and special skills to communicate with these persons and understand their symptoms, evaluate their pain, and obtain their consent to treatment.

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    Competing interests: The authors declare no conflict of interest.

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