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Metastatic tumors to the hands and feet (acrometastases) are rare. We report a case in which the primary presentation of a lung carcinoma was a metastatic lesion to the distal phalanx of the hallux. A 60-year-old woman was evaluated for pain in her big toe. Radiographs and computed tomography suggested a benign lesion in the distal phalanx of the hallux, but curettage and biopsy revealed metastatic adenocarcinoma. A chest x-ray revealed a mass in the right perihilar region, which was confirmed by CT, bronchoscopy, and biopsy as carcinoma of the lung. A review of the literature reveals that there is a tendency toward delayed diagnosis in similar cases, especially when the primary lesion is asymptomatic. A high index of suspicion is needed for early diagnosis in such cases.

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BACKGROUND: Gut involvement in inflammatory myositis is rare but causes significant morbidity and mortality. CASE REPORT: A case of eosinophilic gastroenteritis and polymyositis occurring in the same patient is described. The interface of visceral and striated muscle involvement is discussed. The pathophysiology of eosinophilic gastroenteritis and the spectrum of gastrointestinal involvement in inflammatory myositis are also discussed. RESULTS: Both gastrointestinal and skeletal muscle symptoms improved with immunosuppression, suggesting a possible common underlying mechanism.

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BACKGROUND: Animal models of carcinogenesis have been produced in transgenic mice by directing the expression of oncogenes such as SV40 T antigen and myc to different tissues by creating fusions with promoter/enhancer elements of various mammalian or viral genes. RESULTS: A transgenic mouse line was created in which SV40 T antigen is under the control of the mouse nerve growth factor (NGF) promoter. While the oncogene is expressed in a wide range of NGF producing tissues, it specifically causes the development of either neurofibromas or neurofibrosarcomas similar to those found in the human disease neurofibromatosis type 1 (NF1). These tumours are completely penetrant and appear after a mean latency of about 8 months. In contrast to the previously reported neurofibromatosis mouse model HTLV-1 tax, the tumours in these transgenic mice arise in Schwann cells rather than perineural fibroblasts and have a very restricted tissue distribution. In a cell line cloned from a neurofibroma from these mice, NGF was detected in the culture medium at levels similar to those produced by cultured primary Schwann cells. CONCLUSION: As all animal model for a heritable neurocristopathy resembling NF1, this mouse should allow study of the pathology and treatment of this disease.

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Primary primitive neuroectodermal tumours of the cauda equina are rare. We report a case and review the literature.

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A total of 68 men (mean age 66.2 years) were treated for a primary carcinoma of the breast at Mount Vernon Hospital between January 1968 and June 1988. The mean duration of the symptoms before diagnosis was 13.3 months. The most common sign of presentation (60% of the patients) was a mass in the breast (mean size 3.9 cm). Thirty-three tumours (48.5%) were fixed to the skin and in nine the skin was ulcerated. Sixty-nine per cent of the patients had a T4 tumour and 51.5% had a palpable axillary node. Thirteen patients (19%) were treated by radiotherapy alone, 46 (67%) by surgery and postoperative radiotherapy, and nine (13.3%) by surgery alone. Sixty patients (88%) were in complete remission after the initial treatment and seven of these (12%) developed a local recurrence. The median follow-up was 54 months. The overall survival was 23% at 10 years and the corrected survival 42% at 10 years. Patients with no nodal disease at presentation had a better survival than those with N1 or N2 disease (55% versus 22% 10 years corrected survival, P < 0.002). The risk of nodal disease was strongly correlated by univariate analysis with the size of the tumour (P = 0.002) and the skin fixation (P = 0.005). The risk of metastatic dissemination was correlated with the nodal clinical involvement (P = 0.02) and the existence of a local recurrence (P = 0.003). In multivariate analysis, T and N stages were significantly associated with an increasing risk of treatment failure (P = 0.01). Forty-seven per cent of the patients with T4 tumours developed metastatic disease. This emphasizes the need for adjuvant systemic treatment for these patients.

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DNA ploidy was assessed retrospectively, using flow cytometry, in 13 nodular fasciitis (NF) lesions, three proliferative myositis (PM) lesions, one proliferative fasciitis lesion, and 12 other benign fibrous lesions (BFLs). All were diploid. In view of the large amounts of cellular debris, cell cycle analysis was possible in only seven NF lesions, three PM lesions, and six BFLs. The mean percentage of S phase did not differ markedly between the combination of NF and PM lesions (6.6%) and BFLs (7.1%); the mean percentage of G2 + M phase (5.4%) of the NF/PM lesion combination was twice as large as that of the BFLs (2.5%). No correlation was detected between the cell cycle analysis and the mitotic count, the predominant histologic type in NF lesions, or the predominant stroma in PM lesions.

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We studied four cases of proliferative myositis by the avidin-biotin-peroxidase complex technique, using a panel of 12 antibodies, and by electron microscopy. The aim was to clarify the nature of their constituent cells, specifically the giant ganglion-like cells and spindle cells, and to discuss the implications for histogenesis. In all cases, both cell types showed positive cytoplasmic staining with antibodies to vimentin, actin (C4), and alpha-smooth muscle actin-1, but in only one was there positive staining with desmin. No staining was obtained with factor XIIIa, muramidase, alpha-1-antitrypsin, myoglobin, S-100 protein, CAM 5.2, factor VIII-related antigen, or neuron-specific enolase. By electron microscopy, both types of cells were seen to contain numerous thin filaments, dense bodies, coated and pinocytotic vesicles, active and dilated rough endoplasmic reticulum, few microvilli, and incomplete desmosomal junctions. Our findings imply a myofibroblastic nature for the giant ganglion-like cells and spindle cells. Our observations also support the hypothesis that they are derived from a pericytic cell.

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Between 1975 and 1984, 125 cases of histologically confirmed soft tissue sarcomata (STS) were registered in the Department of Clinical Oncology in Edinburgh. Of these, 100 were eligible for analysis of prognostic factors. The overall 5-year survival rate was 21.5%. Univariate analysis demonstrated that extent of surgery, radical versus palliative or no radiotherapy, mass as a presenting symptom, metastases at presentation, site, histological type, mitotic activity, grade and UICC stage all had a statistically significant effect on survival. Analysis using the proportional hazard regression model was performed on the 87 patients for whom all variables were recorded. When all histological and clinical features and treatment modalities were included in the model then radiotherapy, surgery, necrosis, sex and mitoses were identified as independent prognostic variables. When symptoms and treatment were excluded then the multivariate analysis identified sex and mitotic activity as independent parameters. For the 33 superficial STS with tumour size recorded multivariate analysis revealed size, necrosis and cellularity as independent prognostic variables. For the 31 deep STS histological type, sex, surgery and radiotherapy were identified as independent prognostic parameters.

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A recent paper has again drawn attention to the occurrence of intraplacental choriocarcinoma. We present a similar case in which a small intraplacental choriocarcinoma was identified after delivery of a full-term stillborn fetus. Interpretation of this lesion was influenced by the presence of normal villi and its full significance appreciated only after diagnosis of choriocarcinoma in the mother. This case presents histological proof that choriocarcinoma may arise from an otherwise normal placenta.

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A synchronous occurrence of large bowel adenocarcinoma and extragenital malignant mixed mesodermal tumour (MMMT) is reported. This case represents the sixth extragenital MMMT reported in the literature.

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This paper reports the pathological features of a diaphragmatic haemangiopericytoma with a discussion of diagnostic difficulties it may present in relation to other localised mesenchymal tumours of the pleura. The published reports of diaphragmatic haemangiopericytoma in the world english language literature are reviewed.

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