Giant Epignathus Teratoma Discovered at Birth: A Case Report and 7-Year Follow-Up.

Teratomas are tumors composed by tissues derived from the three germ cell layers, and they are relatively uncommon in head and neck. The term epignathus has been applied to teratomas from the oropharynx. This paper reports the case of a giant epignathus teratoma discovered at birth, which was successfully managed and followed up for 7 years. A newborn boy presented a polypoid tumor mass exteriorizing through the mouth over a length of 9 cm, with some surface areas resembling skin and others exhibiting hair. Computed tomography showed that the mass arose deep from the left hemiface. Alpha-fetoprotein (AFP) levels were high (316,000 ng/mL). Surgery was performed and microscopic analysis confirmed the diagnosis of mature teratoma. Because of residual tumor and high AFP levels, the patient was submitted to chemotherapy, resulting in complete regression of the lesion and normalization of AFP levels. Surgical repair of a cleft palate was performed at 5 years of age. At 7 years of age, the patient was in good general health and showed no clinical signs of recurrence. Although epignathus is a rare condition, it should be diagnosed in the fetus as early as possible. Prenatal care provides unquestionable benefits, providing the early diagnosis of anomalies that can jeopardize the life of the fetus and contributing to the indication of cases that require treatment before birth.

Giant Epignathus Teratoma Discovered at Birth: A Case Report and 7-Year Follow-Up

Abstract Teratomas are tumors composed by tissues derived from the three germ cell layers, and they are relatively uncommon in head and neck. The term epignathus has been applied to teratomas from the oropharynx. This paper reports the case of a giant epignathus teratoma discovered at birth, which was successfully managed and followed up for 7 years. A newborn boy presented a polypoid tumor mass exteriorizing through the mouth over a length of 9 cm, with some surface areas resembling skin and others exhibiting hair. Computed tomography showed that the mass arose deep from the left hemiface. Alpha-fetoprotein (AFP) levels were high (316,000 ng/mL). Surgery was performed and microscopic analysis confirmed the diagnosis of mature teratoma. Because of residual tumor and high AFP levels, the patient was submitted to chemotherapy, resulting in complete regression of the lesion and normalization of AFP levels. Surgical repair of a cleft palate was performed at 5 years of age. At 7 years of age, the patient was in good general health and showed no clinical signs of recurrence. Although epignathus is a rare condition, it should be diagnosed in the fetus as early as possible. Prenatal care provides unquestionable benefits, providing the early diagnosis of anomalies that can jeopardize the life of the fetus and contributing to the indication of cases that require treatment before birth.

Resumo Teratomas são tumores constituídos por tecidos derivados das três camadas de células germinativas e são relativamente incomuns em cabeça e pescoço. O termo epignathus tem sido utilizado para designar teratomas que se originam na orofaringe. Este artigo relata o caso de um teratoma epignathus gigante descoberto ao nascimento, o qual foi tratado com sucesso e proservado por 7 anos. Um menino recém-nascido apresentou uma massa tumoral polipoide que se exteriorizava através da boca por uma extensão de 9 cm, com regiões da superfície semelhantes à pele e outras exibindo pelos. Exame de tomografia computadorizada revelou que a massa se originava profundamente na hemiface esquerda. Os níveis de alfa-fetoproteína (AFP) se apresentavam elevados (316.000 ng/mL). Foi realizada cirurgia e a análise microscópica confirmou o diagnóstico de teratoma maduro. Por apresentar lesão residual e altos níveis de AFP, o paciente foi submetido à quimioterapia, resultando em regressão completa da lesão e normalização dos níveis de AFP. Correção cirúrgica de uma fenda palatina foi realizada aos 5 anos de idade. Aos 7 anos de idade, o paciente apresentava um bom estado de saúde geral, sem sinais clínicos de recorrência da lesão. Embora o epignathus seja uma condição rara, seu diagnóstico no feto deve ser realizado o mais precocemente possível. O cuidado pré-natal proporciona benefícios inquestionáveis, permitindo o diagnóstico precoce de anomalias que podem comprometer a vida do feto e contribuindo para a indicação de casos que requerem tratamento antes do nascimento.

Oral manifestations resulting from chemotherapy in children with acute lymphoblastic leukemia.

INTRODUCTION: Acute lymphocytic leukemia is a type of cancer most common in children and it is characterized by excessive and disordered immature leukocytes in the bone marrow. AIM: Identify most frequent oral manifestations in children with acute lymphocytic leukemia under chemotherapy treatment. METHODOLOGY: The research was conducted on the electronic database PubMed/Medline, Science Direct, Scielo and Scopus. It has been sought papers with full presentation, wrote in Portuguese, English and Spanish, published between January 1992 and April 2013. RESULTS: From studies primarily selected, only eight met the criteria of inclusion. All studies performed intraoral examinations to diagnose oral lesions. According to results, the most frequent lesions were mucositis, candidiasis, periodontitis and gingivitis. The oral health condition from acute lymphocytic leukemia carriers varied according oral hygiene of the patient. CONCLUSION: The results of studies identified such a great part of patients with ALL presented some lesion in oral cavity during or after chemotherapy treatment. The dentist surgeon needs to recognize oral manifestations and intervene in the oral health of patients with ALL, contributing and helping with treatment.

Manifestacoes orais decorrentes da quimioterapia em criancas portadoras de leucemia linfocitica aguda / Oral manifestations resulting from chemotherapy in children with acute lymphoblastic leukemia

Introdução: A leucemia linfocítica aguda é um dos tipos de câncer mais comuns em crianças e é caracterizada pela produção excessiva e desordenada de leucócitos imaturos na medula óssea. Objetivo: Identificar as manifestações orais mais frequentes em crianças portadoras de leucemia linfocítica aguda sob o tratamento quimioterápico. Metodologia: A pesquisa foi realizada nas bases de dados eletrônicas PubMed/Medline, Science Direct, Scielo e Scopus. Procurou-se por artigos apresentados na íntegra, escritos em português, inglês e espanhol, publicados entre janeiro de 1992 e abril de 2013. Resultados: Dos estudos selecionados primariamente, apenas oito atenderam aos critérios de inclusão. A população avaliada foi um grupo de crianças portadoras de leucemia linfócitica aguda. Todos os estudos realizaram exames intraorais para o diagnóstico das lesões bucais. De acordo com os resultados, as lesões mais frequentes foram mucosite, candidíase, periodontite e gengivite. A condição de saúde bucal dos portadores de leucemia linfócitica aguda variou de acordo com a higiene bucal do paciente. Conclusão: Pacientes com LLA podem apresentar alguma lesão na cavidade oral durante ou após o início da quimioterapia. O cirurgião dentista necessita reconhecer as manifestações orais e intervir na saúde bucal do paciente com LLA, contribuindo e auxiliando no seu tratamento. .

Introduction: Acute lymphocytic leukemia is a type of cancer most common in children and it is characterized by excessive and disordered immature leukocytes in the bone marrow. Aim: Identify most frequent oral manifestations in children with acute lymphocytic leukemia under chemotherapy treatment. Methodology: The research was conducted on the eletronic database PubMed/Medline, Science Direct, Scielo and Scopus. It has been sought papers with full presentation, wrote in Portuguese, English and Spanish, published between January 1992 and April 2013. Results: From studies primarily selected, only eight met the criteria of inclusion. All studies performed intraoral examinations to diagnose oral lesions. According to results, the most frequent lesions were mucositis, candidiasis, periodontitis and gingivitis. The oral health condition from acute lymphocytic leukemia carriers varied according oral hygiene of the patient. Conclusion: The results of studies identified such a great part of patients with ALL presented some lesion in oral cavity during or after chemotherapy treatment. The dentist surgeon needs to recognize oral manifestations and intervene in the oral health of patients with ALL, contributing and helping with treatment. .