Fibrosarcoma in pediatric patients: Results of the Italian Cooperative Group studies (1979–1995)

Abstract

Background and ObjectivesFibrosarcoma is a rare soft tissue sarcoma (STS) that has two peaks of incidence in pediatric patients: one in infants and young childern (infantile fibrosarcoma), another in older children (“adult type” fibrosarcoma). The purpose of this study was to describe the clinical features and the treatment results in patients affected by fibrosarcoma enrolled in two consecutive studies run by the STS‐Italian Cooperative Group (ICG) between 1979 and 1995.Patients and MethodsTwenty‐five evaluable patients were grouped according the intergroup rhabdomyosarcoma staging (IRS) system: 12 Gr I, 5 Gr II, 8 Gr III. The cut‐off point between the two forms was considered the age of 2 years: 14 patients were affected by infantile and 11 by adult type of fibrosarcoma. The therapeutic guidelines were not strict and not different for the two forms: patients with initial macroscopic excision (Gr I–II) were given IVA or VAC; Gr III had VAC or VAIA and subsequent excision, if feasible. Radiation therapy (RT) was delivered in patients > 3 years with microscopic (42 Gy) and macroscopic (54 Gy) residuals.ResultsThirteen patients are alive without evidence of disease: 10 in first complete remission (CR), 7 Gr I, 1 Gr II, 2 Gr III, 3 in second CR after local relapse (LR) (2 Gr I, 1 Gr II). Two Gr I patients are alive with metastatic relapse. Nine patients died: six of progressive disease (PD) (three Gr II, three Gr III), two because of a second tumor (one Gr I, one Gr III) and one because of other causes (Gr III). One Gr III patient was lost at follow up, 2 years from diagnosis. The 10 years overall survival (OS) is 67.4% and the progression‐free survival (PFS) is 52.2%. The OS for patients < 2 years is 78.6% (11 of 14), versus 51% for patients > 2 years (5 of 11).ConclusionsThe complete excision at diagnosis was the treatment of choice and was related to the best outcome. Microscopical residuals were difficult to treat with chemo‐radiotherapy in both forms of fibrosarcoma. Neoadjuvant chemotherapy (CT) obtained a partial remission (PR) only in three of eight cases, while no conclusions concerning the efficacy of CT for infantile forms are possible. Patients < 2 years had a better outcome than the older ones: most of them had a tumor on extremities which was excised at diagnosis. J. Surg. Oncol. 2001;78:225–231. © 2001 Wiley‐Liss, Inc.

Lumps and Bumps on the Head in Children: Use of CT and MR Imaging in Solving the Clinical Diagnostic Dilemma

“…Approximately 5% occur in the head and neck region (50 -52), and 25%-40% occur in children during the first 5 years of life, with up to 92% detected in the 1st year of life (53,54). The tumor is slightly more common in boys (60% of cases) (55).…”

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].…”

“…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. Chemotherapy also has been used for unresectable lesions [3,16,17,22,27,37].…”

“…Chemotherapy also has been used for unresectable lesions [3,16,17,22,27,37]. The most common regimen used combines vincristine, actinomycin D, and cyclophosphamide (VAC) [6,30]; its side effect is well established and is tolerable in infants [10,22,23].…”

Right foot congenital infantile fibrosarcoma treated only with chemotherapy

“…Approximately 50% of these tumors are present at birth, the remainder developing during the first year of life [4]. A male predilection is noted in most series [4], but a study by Cecchetto et al [5] reported that 15/25 cases were female.…”