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ABSTRACT: The role of radiotherapy in the management of perianal Paget’s disease (PPD) is not well defined in clinical practice or within the medical literature. We present 6 cases, document the radiotherapy details and review our results. A comprehensive literature search has been undertaken attempting to identify all published cases of PPD and survey the number receiving radiotherapy. To further define the role for radiotherapy in PPD these cases have been reviewed. Published results are sporadic and often poorly documented with respect to technical radiotherapy details. Two main roles for radiotherapy in PPD were found. One is as primary treatment for in situ or invasive disease and the other is following surgical relapse ofin-situ or invasive disease. Other possible uses of radiotherapy in PPD such as neoadjuvant or adjuvant treatment or chemo-radiotherapy are discussed. Results of radiotherapy treatment for case of in situ and invasive disease are presented. We disagree with the view in some areas of the surgical literature that radiotherapy has no place in the management of the disease. Despite a thorough surveying of the literature however, precise recommendations on several areas of the technical radiotherapy treatment such as dose-fractionation schedules and field margins are difficult because of the small number of cases and poor general documentation. Our practice recommendations are presented. Radiotherapists should be encouraged to publish their experience in this disease to help define further a role for this treatment. [Copyright &y& Elsevier]

A 9-year-old boy was seen to have embryonal rhabdomyosarcoma of his right hand. He was treated with surgery, radiotherapy and adjuvant combination chemotherapy and remained free of disease for 103 months. He then developed signs of upper respiratory tract obstruction and a mass in the superior mediastinum was found. Histopathological and immunohistochemical studies confirmed metastatic embryonal rhabdomyosarcoma. This very unusual, long, relapse-free interval is discussed.