Pathological features of congenital fibrosarcoma and haemangiopericytoma with long term followup after local surgical resection

Abstract

The treatment of congenital neoplasms has been the subject of debate due to their infrequent occurrence, and difference in biological behaviour compared to adult tumours of similar histological appearance. We report 2 cases of congenital fibrosarcoma and haemangiopericytoma, respectively with long term followup, in which the sole method of treatment was local surgical resection. Case I: A term male infant underwent excision of a 10 cm. diameter mass that was overlying the left scapula. Histologically the tumour was a fibrosarcoma which abutted the resection margin, and had up to 3 mitoses/HPF, with moderate nuclear pleomorphism. The patient is alive and well with no evidence of recurrence or of metastasic disease at 18 yr followup. Case II: A term female infant underwent excision of a 5 cm diameter facial mass. Histologically the tumour was a haemangiopericytoma which abutted the resection margin, and had 1 mitosis/3 HPF, with focal areas of necrosis. The patient is alive and well with no evidence of recurrence or of metastasic disease at 15 yr followup. Histologic features of frequent mitoses, cytologic pleomorphism and necrosis did not signify a poor prognosis in these congenital tumours. Subsequent ploidy analysis of both tumours revealed predominantly diploid cell populations, and so it is possible that this may be of use as an alternative prognostic parameter. As these cases both had reasonable cosmetic and functional results, local surgical resection alone with careful followup may be regarded as a viable treatment option. The added advantage of extensive surgery, or of adjuvant therapy with chemo- or radiotherapy would have to be questioned, particularly in view of the apparently incomplete nature of the original excisions. Given reports in the literature of recurrence and metastases in similar neoplasms and the lack of reliable histological features predicting outcome in individual cases, close and prolonged clinical followup is, however, essential.