RESUMO
INTRODUCTION: Langerhans' cell histiocytosis is a collective term used to describe a group of enigmatic proliferative disorders. The natural history of the disease varies from a slow, benign, localized symptomatic bony or soft tissue lesion, to a rapidly progressive widespread multiple organ disorder which is often fatal. Eosinophilic granuloma accounts for 60-70% of all cases of Langerhans' cell histiocytosis and can present as solitary (50-75%) or multifocal defects in bone. It occasionally presents as a localized soft tissue lesion. There are multiple treatment options but the response is unpredictable. AIMS: We present three separate cases, of the maxillofacial skeleton where the lesions of eosinophilic granuloma resolved following incisional biopsy only. PATIENTS AND RESULTS: Three patients presented with solitary lesions of the maxillofacial skeleton. All were diagnosed as Langerhans' cell histiocytosis following open curettage, which also resulted in resolution of the lesions. Follow-up has thus far been disease free. CONCLUSION: For some solitary Langerhans' cell histiocytosis lesions, simple curettage is the only treatment required. The paper discusses the need to confirm the solitary nature of the disease and the need for follow-up. Reviewing the literature on the disease, the authors suggest that perhaps cellular immaturity holds the cells of the lesion in a disease state until pushed to maturity by the trauma of open curettage surgery, resulting in a complete resolution of the disease.
Assuntos
Doenças Ósseas/patologia , Granuloma Eosinófilo/patologia , Ossos Faciais/patologia , Biópsia , Criança , Pré-Escolar , Curetagem , Feminino , Seguimentos , Histiocitose de Células de Langerhans/patologia , Humanos , Masculino , Doenças Mandibulares/patologia , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Minimamente Invasivos , Doenças Orbitárias/patologia , Indução de Remissão , Transtornos da Articulação Temporomandibular/patologiaRESUMO
The All Wales Lymphoma Panel (AWLP) was established in January 1998 to provide a central expert pathological review service for district general hospital pathologists. A discordance rate of 20% between the submitted and reviewed diagnosis has previously been identified. It has not been known whether this change in diagnosis affects clinical management. Ninety-nine patients whose diagnosis was changed as a result of central pathological review are presented. Between January 1998 and August 2000, 125 of 745 (17%) specimens submitted for AWLP review had a consequent change in pathological diagnosis. Of these 125 specimens, 99 (79%) complete case notes were recovered. In all 99 cases, a hypothetical management plan was generated using collected data, clinical protocols and the submitted pathological diagnosis. These plans were compared with the actual management patients received based on the reviewed diagnosis proffered by the AWLP. Forty-six of 99 (46%) cases had a change in management as a result of central pathological review. Overall, management was changed in 8% of cases referred for central pathological review. In conclusion, expert central pathological review has a direct effect on patient management.