Malignant chondroid syringoma (MCS; malignant mixed tumour) is a rare neoplasm typically arising on the extremities and trunk. We are report 2 unique cases of MCS, one occurring on the scalp of a 78-year-old man and the other on the trunk of a 72-year-old woman. Both tumours harboured malignant epithelial and malignant mesenchymal components. The latter was represented by liposarcoma in the first case. The malignant components of the second tumour comprised spindle cell squamous cell carcinoma (SCC) and osteosarcoma. Origin from a pre-existing benign chondroid syringoma was clearly evident in both neoplasms. The presence of heterologous malignant mesenchymal components, however, is hitherto unreported in the context of MCS, while a spindle cell SCC component is exceptionally rare. The 2 cases presented herein highlight an expanded morphological spectrum of MCS, with resultant blurring of the boundaries between MCS and cutaneous carcinosarcoma.
Adrenal cortical carcinomas (ACC) are rare tumours, most commonly reported in adult patients. However, an important peak in incidence occurs in paediatric patients. ACC is a rare cause of paediatric endocrinopathy which may masquerade as a non-neoplastic disease process. Herein we present ACC in a five-year-old female patient. Histopathological features associated with poor outcome included tumour weight >500 g, tumour size >10.5 cm, invasive properties, confluent tumour necrosis, high nuclear grade and high proliferation index assessed by Ki67 immunohistochemistry. This article focuses on clinical features, treatment, pathological characteristics, evolving classification and genetic significance of ACC in paediatric patients.
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