Langerhans cell histiocytosis: an uncommon disease commonly manifesting in the craniofacial skeleton.

Langerhans cell histiocytosis is an infrequent and enigmatic proliferative disorder that commonly presents in the head and neck region. This is an analysis of 77 patients with Langerhans cell histiocytosis treated at Children's Hospital and Dana Farber Cancer Institute from 1974 through 1993. The study focused on clinical findings, anatomic location and extent of disease, therapy, and outcome. The patients were, on average, under 5 years of age at initial presentation. Over 62 percent of the patients had signs and symptoms referred to the craniofacial skeleton. Osteolytic lesions of the cranium were the most common, followed, in frequency, by scalp rash, osteolytic mandibular tumor(s), enlarged nodes, and gingival swelling or ulceration. Single bony lesions usually were treated with curettage or radiotherapy. Chemotherapy was used commonly for advanced disease with multifocal or disseminated presentation. Initial therapy included moderate doses of single agents; other agents were added if no response was achieved. The natural history of Langerhans cell histiocytosis varied from an acute fulminant course, a waxing and waning chronic disease, to spontaneous regression. Young age at presentation and organ dysfunction predicted a poor prognosis. Statistical analysis showed that there was no significant relationship between outcome and extent of skeletal involvement when controlling for age or organ dysfunction.

Pediatric oral lesions: a 15-year review from São Paulo, Brazil.

There are few studies on pediatric oral pathologies in the literature. This study presents data from a review of 2,356 biopsies of young patients (birth to 14 years) received over 15 years (1985-2000) in the Oral Pathology Service at the University of Sao Paulo, Brazil. Information about patients (sex, age, race) and histopathological diagnosis was retrieved. Diagnosis data of 2,356 biopsies were classified into 20 groups. There was no significant difference between male (50.0%) and female (49.0%) patients. White is the predominant race (69.0%), and patients ages were concentrated between 9 and 14 years old (70%). Mucocele was the most frequent (13.5%), followed by dentigerous cyst (6.5%) and fibrous hyperplasia (5.4%). Papilloma and Langerhans cells histiocytosis were the most common non-odontogenic benign and malignant tumors, respectively. In the group of odontogenic tumors, odontoma was the most frequent, and ameloblastoma had a significant incidence (27 cases). These data are important in order to detect differences in geographic areas, diagnosis line tendencies and for clinicians to perform judgment to evaluate of the pediatric patients before the biopsy and management of pediatric oral lesions.

Enfermedad de células de Langerhans crónica localizada: reporte de caso con lesiones periodontales. Revisión de la literatura / Chronic localized Langerhans cells disease. Report of a case with periodontal lesions. Review of the literature

La enfermedad de células de Langerhans es una enfermedad de etiología desconocida, la cual sucede con mayor frecuencia en la edad infantil. El presente es un reporte de un caso de enfermedad de células de Langerhans en un adulto. El paciente presentó lesiones destructivas a nivel periodontal como única manifestaciónb de la enfermedad. Los pacientes con enfermedades periodontales producto de la enfermedad de células de Langerhans son frecuentemente diagnosticados como otras patologías. Este caso constituye una muestra del diagnóstico precoz de esta enfermedad por un abordaje multidisciplinario. Las manifestaciones bucales de esta enfermedad, sus métodos de diagnóstico y tratamiento son discutidos

Lesöes pseudotumorais de maior prevalência na infância / Pseudotumoral lesions of more prevalence at childhood

O autor apresenta uma revisäo bibliográfica referente aos principais aspectos no estudo das lesöes pseudotumorais que acometem a populaçäo infantil com maior freqüência. Säo enfocados aspectos clínicos, radiográficos e histopatológicos, bem como fatores relacionados à etilogia, prevalência e conduta terapêutica frente a tais lesöes

Estudo epidemiológico da doença da célula de langerhans: análise de 33 casos em Natal/RN / Epidemiologic estudy of the langerhans cell disease: analysis of 33 cases in Natal/RN

A doença da célula da Langerhans é uma desordem de etiologia incerta, caracterizada por um amplo aspecto clínico e comportamento bastante variado. Neste estudo foram analisados 33 casos de pacientes com diagnóstico de Doenca de célula de Langerhans no Hospital Infantil Varela Santiago em Natal, RN-Brasil, entre 1997 e 1999. Deste total, 42,4 por cento dos pacientes manifestaram o Granuloma Eosinofílico Solitário, 33 por cento dos pacientes tinham a Doença de Hand-Schüller- Christian e 24,3 por cento destes pertenciam a forma clínica da Doença de Letterer-Siwe. Do total, 54,5 por cento dos pacientes eram do sexo masculino e 45,5 por cento do feminino, com uma média de idade de 3 anos e 7 meses (variando de 06 meses a 15 anos). Setenta por cento dos pacientes apresentavam envolvimento extra-oral, sendo os ossos do crânio e extremidades inferiores, os sítios mais comuns da doença. O tratamento consistiu primariamente da excisão cirúrgica para pacientes com lesões localizadas e terapia com drogas para aquelas com a Doença de Letterer-Siwe. Nosso estudo demonstrou que a forma clínica mais freqüente foi o Granuloma Eosinofílico Solitário e que pacientes mais jovens com disfunção orgânica têm prognóstico pouco favóravel