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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 of in-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.

Malignant pilomatrixoma is a rare malignant tumour of the hair matrix, which was first described in 1980. Only five patients with distant metastases have been reported. We report a sixth case with metastatic disease in a 52-year-old male, who also developed multiple local recurrences. We also review the previous five cases.

Nasal Natural Killer-Cell Lymphoma is an aggressive subtype of NHL even when it presents with localized disease. It is more common in Oriental than Western population. We report the case history of a white Caucasian male patient with this disease who died 5 months from diagnosis despite aggressive treatment with 2 different chemotherapy regimes and radiotherapy. We discuss the diagnosis, presentation, treatment and prognosis of this rare disease.

Angiosarcoma of the head and neck is a rare malignant vascular tumour that predominantly affects elderly men and generally has a poor prognosis. Complete responses to treatment for larger tumours are uncommon. A patient with an extensive tumour that showed a complete response to combination therapy with liposomal daunorubicin and radiotherapy is presented. The rationale for using liposomal chemotherapy agents in the treatment of vascular tumours such as angiosarcoma is discussed.

A patient with human immunodeficiency virus-related non-Hodgkin's lymphoma arising in the oral cavity is reported. The tumour had an unusual immunohistochemical profile that was negative for leucocyte common antigen and the B-cell antigen CD20 but positive for the plasma cell-reactive antibody VS38c. The features of this type of tumour, which has recently been categorized, are presented and discussed.

Classical Kaposi's sarcoma and angiosarcoma are rare malignant tumours arising from vascular tissue. However, they have distinct histological features and appear also to differ in their epidemiology and pathogenesis. We present the case history of a patient in whom both these tumours occurred simultaneously. The relationship between these events is discussed in the light of the new virus, human herpes virus 8.

Lymphangioma circumscriptum is a rare non-malignant skin tumour, conventionally treated by surgical resection, but with variable results. We report two cases, which, although unsuitable for excision, were treated successfully by radiotherapy and consider the place of this modality in managing this disorder. We conclude that radiotherapy is an effective treatment for unresectable lesions, or for patients who are unwilling to consider surgery.

We retrospectively analysed serial pulmonary function tests in 14 HIV infected patients receiving either bleomycin and vincristine or liposomal doxorubicin therapy (Doxil) for AIDS related Kaposi's sarcoma. There was a significant reduction in the carbon monoxide transfer coefficient in bleomycin treated patients compared with patients treated with Doxil. No other significant changes in pulmonary function, including the carbon monoxide diffusing capacity, were observed. These preliminary findings suggest that HIV infected patients with Kaposi's sarcoma, who are receiving bleomycin, may be at risk of accelerated pulmonary dysfunction. A larger prospective study should be performed to enable further investigation.

This paper describes the results of a national survey on the non-surgical treatment of early breast cancer in the UK relating to the management of ductal carcinoma in-situ (DCIS), and the rationale for the current status of the UK DCIS trial. The survey shows that there is considerable clinical uncertainty, which can only be resolved by the trial.

A patient with a seminoma, squamous cell carcinomas of the skin and anus, Bowen's disease and lymphopenia is reported. The underlying aetiology is thought to be impaired cell mediated immunity and susceptibility to viral infections.

A case of Stage 1 nodular lymphocytic predominant Hodgkin's Disease relapsing after 32 years is described. Recent immunophenotyping suggests that this is a low grade B-cell lymphoma.

Between July 1985 and December 1987, 87 patients with advanced breast carcinoma were randomized to receive single agent doxorubicin (70 mg/m2), epirubicin (70 mg/m2) or mitozantrone (14 mg/m2) at 3-weekly intervals. The patients had received no previous chemotherapy for their advanced disease but 91% had received prior hormonal therapy. The response rates were 36% with doxorubicin, 32% with epirubicin and 26% with mitozantrone, but these differences did not reach statistical significance. The median survival of all patients was 8.3 months. There was no significant difference in response rates or survival according to menopausal status. The toxicities of the three agents are compared. Nausea, vomiting and alopecia were more severe in patients treated with doxorubicin or epirubicin than those treated with mitozantrone. Myelosuppression and infective episodes occurred more frequently with mitozantrone. Two cardiac complications were reported. This study shows that the toxicity and low efficacy of all three agents limit their use as single agents in advanced breast carcinoma. The role of single agent chemotherapy and the relative toxicities of these drugs are discussed.

High grade non-Hodgkin's lymphoma accounts for only 5%-10% of all non-Hodgkin's lymphoma. Infection with the human immunodeficiency virus increases the risk of developing high grade, usually B-cell, lymphoma, which has been noted to occur more commonly in homosexual men. These lymphomas often have unusual clinical presentations. We report five cases of high grade non-Hodgkin's lymphoma in HIV negative homosexual men who presented to our hospital in a 13-month period. As a major centre for the treatment of the acquired immune deficiency syndrome, there may be a self-selected homosexual population attending for medical care, and thus a bias in the relative incidence of lymphoma seen in this group.

Sixteen patients with advanced AIDS-related Kaposi's sarcoma received liposomal doxorubicin (Doxil, Liposome Technology, Menlo Park, California) at 20 mg/m2 every 2 or 3 weeks in an open label study, and were evaluated for efficacy and toxicity. Eleven patients achieved a partial remission and five had stable disease. The median time to achieve a maximum response was two cycles (range 1-3) and the median duration of response was 14 weeks (range 6-30). Myelosuppression was the commonest adverse event; one patient was withdrawn because of neutropenia. Other adverse events were uncommon and mild. Liposomal doxorubicin is an effective and safe single agent treatment for advanced AIDs-related Kaposi's sarcoma.

The axillae of 30 patients with primary breast cancer (Stage I and II) were prospectively examined in this pilot study using ultrasound. No patient had palpable axillary lymph nodes on clinical examination. Treatment had involved wide local excision, but no prior form of surgical dissection had been performed on the axilla. Using the contralateral axilla as an internal control, lymph nodes were observed in the ipsilateral axilla alone on ultrasound in 8/30 patients (27%). Following radical irradiation of the breast and local lymph drainage areas, 2/8 patients of the group with observed lymph nodes have relapsed, one with systemic disease and the other with local recurrence in the breast, after a minimum follow-up of 12 months. No patient without observed nodes has recurred. This difference does not reach statistical significance. This technique merits further investigation as an adjunct to current staging procedures for early breast cancer.