Congenital fibrosarcoma

Abstract

Congenital fibrosarcoma is a rare fibrous tissue malignancy occurring most commonly in the extremities (71%), but presenting in axial locations as well (29%). Two of our five patients had lower extremity tumors and three had axial lesions located in the thoracolumbar region and anterior neck. Amputation was considered necessary to treat the extremity tumors. Two of the patients with axial lesions had incomplete resection, which raised the question of adjunctive therapy and prompted this review. Forty-seven additional cases of fibrosarcoma, present at birth or diagnosed within the first 3 months of life, were adequately described and documented in the literature giving a total of 52 cases. There was no sex predominance (male 26, female 20, unknown 6). Thirty-seven (71%) of the patients had extremity lesions. The local recurrence rate for extremity tumors was 32% with metastatic and mortality rates of 8% and 5%, respectively. Axial tumors (15 cases, 29%) had a local recurrence rate of 33%, which is similar to that for extremity lesions, but the metatstatic and mortality rates were 26% each indicating a more aggressive behavior for axial lesions. The overall mortality rate of 11.5% and metastatic rate of 13.5% for congenital fibrosarcoma, are not dissimilar to the figures quoted for older children. This pattern of tumor behavior, therefore, does not seem to warrant a different attitude because of the "congenital" occurrence of the tumor. Surgery must be considered the mainstay of therapy for fibrosarcoma, but there is a need for adjunctive therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Orthopaedic Case of the Month: Rapidly Progressive Shoulder Soft Tissue Mass in an 8-week-old Girl

“…Moreover, unlike its adult counterpart, congenital fibrosarcoma metastasizes in less than 10% of patients and carries a good prognosis, despite its ominous histologic appearance and locally aggressive behavior [3,4,14,36]. Five-and 10-year survival rates are approximately 80% to 90% [6,27,30,32], and amputation rates appear to be declining [3,8,30] MRI findings are generally nonspecific, and show a well-circumscribed, heterogeneous mass, with vascular elements, areas of necrosis, and hemorrhage. The mass is typically isointense to muscle on T1-weighted images and heterogeneously hyperintense on T2-weighted images; gadolinium enhancement is variable [5,24].…”

“…The mass is typically isointense to muscle on T1-weighted images and heterogeneously hyperintense on T2-weighted images; gadolinium enhancement is variable [5,24]. Although the lung is the most common site of metastases, congenital fibrosarcoma can metastasize to bone [3,36], with technetium-99 bone scan being useful for detection of these metastases.…”

“…Although the adoption of limb-salvage techniques has been proposed, the overall amputation rate for congenital fibrosarcoma still approaches 50% [3,8,30], and it is not always clear which patients will have postsurgical local recurrence. Chemoresponsiveness of congenital fibrosarcoma is unclear [6,30], however neoadjuvant chemotherapy may be used, in an attempt to decrease tumor size and reduce the surgical morbidity.…”

A newborn with infantile fibrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery

“…Its clinical course is more favorable with a rare metastatic spread. 2 The local recurrence rate is high, up to 43%, and recurrence may occur as late as 15 to 31 years after the initial operation. 4 Wide local excision is the primary form of treatment, but in some cases, as in our case, it is impossible to remove lesion because of the anatomic extent of these tumors without disrupting peripheral vessels and nerves.…”

“…It is locally aggressive, with a rare metastatic spread and a good rate of survival. 2 Hemangiopericytoma is also very rare in childhood and comprises two different clinical entities: the adult type and the infantile type occurring in the first year of age. The infantile type is locally invasive with rare metastasis, and prognosis is better than that of the adult type.…”