Adult fibrosarcomas are rare, malignant and highly aggressive fibroblastic soft tissue tumors. They constitute a diagnosis of exclusion.
Adult fibrosarcomas by more recent definition are rare and make up for around 1% of soft tissue sarcomas. They are mostly seen in the middle-aged people within the 4th and 7th decade of life and with a minor male predilection 1-3.
Adult fibrosarcomas have been associated with radiation therapy and foreign implants 1.
The typical complaint is a soft tissue mass, which can present with or without pain 1.
Adult fibrosarcomas have become a diagnosis of exclusion. They are characterized by relatively monomorphic spindle cells growing in a fascicular pattern with variable collagen production also referred to as herringbone architecture 1.
The etiology is unclear some adult fibrosarcomas have been found in the field of previous radiation therapy.
Locations, where adult fibrosarcomas are mostly found, include the deep soft tissues underneath the deep fascia of the extremities, the trunk and the head and neck region 1-3.
Macroscopically adult fibrosarcoma usually appears as a circumscribed tumor of tannish white color and firm consistency sometimes featuring areas of necrosis and/or hemorrhage 1,2.
Microscopic features of adult fibrosarcoma include the following 1:
- relatively monomorphic spindle cells (no high degree of pleomorphism)
- fascicular growth in a ‘herringbone’ architecture
- variable collagen production
- variable mitotic activity
- areas of necroses
Immunohistochemistry stains can show restricted expression of SMA or calponin 1.
Imaging features of adult fibrosarcomas have been described as lobulated, well-defined lesions with fibrous bands and slightly irregular margins as well as relations to the deep fasciae with nodular lumps around the fascia edge 2,3. They displace the surrounding tissues 2.
The CT appearance of adult fibrosarcomas has been described as a soft tissue density mass usually isodense to muscle 2.
On MRI adult fibrosarcomas have been described to look like a heterogeneous soft tissue mass with low signal intensity band-like fibrous streaks in all sequences. Changes of the deep fascia and associated muscle edema have been described in a higher number of cases 3.
Signal characteristics are usually as follows:
- T1: hypo- to isointense to muscle
- T2: mixed heterogeneous signal intensity
- DWI: diffusion restriction
- T1 C+ (Gd): heterogeneous peripheral or spoke wheel enhancement
The radiological report should include a description of the following 3:
- form, location and size
- tumor margins
- relation to and alterations of the muscular fascia
- relationship to local nerves and vessels
- subcutaneous tissue or bony involvement
Treatment and prognosis
Treatment options include surgery complemented by adjuvant radiotherapy and neoadjuvant chemotherapy. Local recurrence depends on tumor residues upon surgical removal. Distant metastases occur in the lung liver and bones, in particular, the axial skeleton. Lymph node metastases are rare. The 5-year survival is around 50% 1-3.
History and etymology
Adult fibrosarcoma was once regarded as the most common soft tissue sarcomas in adults 1. Due to advances in testing methods and changes in diagnostic criteria they have become rare entities and a diagnosis of exclusion 1.
Conditions which can mimic the presentation and/or the appearance of adult fibrosarcoma include 1-3:
- 1. W.H.O. Classification WHO Classification of Tumours Editorial Board, Who Classification of Tumours Editorial. Soft Tissue and Bone Tumours. (2020) ISBN: 9789283245025
- 2. Augsburger D, Nelson PJ, Kalinski T, Udelnow A, Knösel T, Hofstetter M, Qin JW, Wang Y, Gupta AS, Bonifatius S, Li M, Bruns CJ, Zhao Y. Current diagnostics and treatment of fibrosarcoma -perspectives for future therapeutic targets and strategies. (2017) Oncotarget. 8 (61): 104638-104653. doi:10.18632/oncotarget.20136 - Pubmed
- 3. Wang H, Nie P, Dong C, Li J, Huang Y, Hao D, Xu W. CT and MRI Findings of Soft Tissue Adult Fibrosarcoma in Extremities. (2018) BioMed research international. 2018: 6075705. doi:10.1155/2018/6075705 - Pubmed