An indeterminate malignant soft-tissue tumour treated by exenteration

PURPOSE. To present a case of malignant soft-tissue tumour, which has yet to be categorised despite review by an international panel of experts, and which was treated by exenteration. PATIENT & METHODS. A 15-year-old Caucasian girl presented with progressive, non-axial proptosis and diplopia. CT scan showed an extraconal mass in the supero-medial part of the orbit suggestive of a vascular neoplasm. After initial incisional biopsy, the pathology of the tumour was felt to be malignant but so unique that further tissue was required for classification/categorisation and that exenteration was the best way to proceed. RESULTS. Despite there being an adequate amount of tissue and opinions being sought from international experts, including the Children(1)s Soft Tissue Panel (of the United Kingdom), this tumour has yet to be categorised. Reports have suggested it to be anything from a malignant desmoplastic round-cell tumour or a synovial sarcoma to an epithelioid haemangioendothelioma, which is our current working diagnosis. CONCLUSION. This case is unique in that it has not been possible to classify the tumour. Categorisation is important in order to give a reasonably accurate prognosis to the patient. Exenteration was required to offer complete tumour excision and will hopefully prevent local recurrence and metastatic spread.