CASE 10945 Published on 03.06.2013

Adult-type fibrosarcoma in a female patient of 17 years of age

Section

Abdominal imaging

Case Type

Clinical Cases

Authors

Sarti E, Rossi P, Tarantini G, Raffo L, Iodice V, Giusti S.

Diagnostic and Interventional Radiology,
University of Pisa, Via Roma 67,
56125 Pisa, Italy.
Patient

17 years, female

Categories
Area of Interest Pelvis, Oncology, Paediatric, Abdomen, Liver, Kidney ; Imaging Technique PET, CT, MR, Ultrasound
Clinical History
A 17-year-old woman came to our attention for the recent onset of abdominal pain, urinary disorders and change in bowel habit.
Imaging Findings
An MRI was performed showing the presence of a solid, inhomogeneous mass with defined margins located in the left pelvic region connected with the left ovary displacing uterus, rectum and bladder (Fig. 1).
A staging CT/PET confirmed MRI findings (Fig. 2).
The surgical excision of this mass confirmed the presence of a fibrosarcoma.
A follow-up CT after 3 months showed recurrence of disease in the left pelvic region extending along the iliac vessels. This mass involved the left ovary, ureter (Fig. 3), so the patient started adjuvant treatment with ifosfamide-epirubicin.
After 6 months of treatment a CT showed an increase in size of the lesion with involvement of the vagina, and left broad ligament (Fig. 4).
A follow-up MRI showed multiple lesions mostly in the right pelvic region, near the bladder, rectus muscles under the navel (Fig. 5).
A follow-up US (Fig. 6) revealed progression of disease with the onset of liver nodules and signs of a left hydronephrosis.
Discussion
Fibrosarcoma is a neoplasm of the group of soft tissue sarcomas. These types of tumour arise from mesenchymal tissues and they can grow in fat, muscles, tendons (bands of fibre that connect bones to muscle), nerves, joint tissue, blood vessels and other fibrous tissue. These tumours are very rare (1% of all cancers) and their incidence is approximately 2-3 new cases per year per 100,000 inhabitants. The frequency in males and females is the same. It is most common in people between 20 and 60 years old, but can occur at any age. The exact cause of fibrosarcoma and other soft tissue tumours is unknown; genetic alterations are probably involved. There are three forms of fibrosarcoma. The first is an infantile form present at birth or in early childhood that is identical to the adult form except for the clinical course, much more favourable. Lung metastases are rare (<10%) and can be cured by surgical excision without further treatment. Second is an adult form characterised by high degrees of malignancy and high frequency of recurrence that arise in adult or in older children and in adolescents. The third form is represented by the dermatofibrosarcoma protuberans. This is a rare variant that grows in the skin and subcutaneous tissues and is derived from the fibrous component of the dermis. Histologically the fibrosarcoma is composed of immature fibroblasts [1]. Symptoms are highly variable and depend on the location in which they grow: generally are characterised by the presence of a hard, fibrous mass grown in a few months which may cause pain and compression of adjacent organs [2]. Diagnosis is achieved with the diagnostic imaging and biopsy. The most important tests include MRI, CT and US in some case. The biopsy is essential to evaluate the type and histological grade of the lesion. The biopsy may be performed with thick needle (0.4-0.8 mm). The excisional biopsies that remove the mass without knowing the nature of the disease must be avoided [3]. The surgical therapy should be radical and the association with radiation therapy certainly improves local control. The importance of chemotherapy is still being evaluated. A careful follow-up is essential for the high incidence of recurrence of this injury [4].
Differential Diagnosis List
Adult-type fibrosarcoma
Malignant solitary fibrous tumour
Leiomyosarcoma
Myofibromatosis
Desmoplastic fibroma
Final Diagnosis
Adult-type fibrosarcoma
Case information
URL: https://www.eurorad.org/case/10945
DOI: 10.1594/EURORAD/CASE.10945
ISSN: 1563-4086