ArticleA rare case of fibrosarcoma of the jaws in a 4-year-old male
Introduction
Infantile fibrosarcoma is an uncommon tumour in children, representing about 5% of all malignancies in the head and neck region[1]. Neoplasms of the head and neck region represent 2.5% of all tumours in children[2]and malignant tumours of the oral cavity account for less than 2% of them. About 200 infantile fibrosarcomas have been published1, 2, 3, 4, 5, 6, 7, 8; only five cases involving the soft tissue of the oral cavity have been described4, 9, 10, 11, 12. Consequently, most clinicians and pathologists have very limited experience with these tumours.
The histopathology varies from that occurring in adults and it has a markedly different clinical behaviour. Fibrosarcoma is a malignant tumour of mesenchymal origin; it may arise in soft tissues or within bone[13]. Primary fibrosarcoma arising in normal bone has been reported to comprise 10.9% of all osteogenic sarcomas[14]. Localization in the maxilla is rare with the incidence ranging from 0 to 6.1% of all primary fibrosarcomas of bone[15].
Fibrosarcomas of bone may be sited centrally or peripherally, the latter affecting bone by secondary spread from adjacent soft tissue[13]. The tumour may resemble a simple fibrous overgrowth16, 17, 18: the major clinical symptoms are pain, swelling, and sometimes loosening of teeth[19], paraesthesia and occasionally ulceration of the overlying mucosa[20].
Radiographically, an osteolytic lesion is usually present, with ill-defined borders; however, fibrosarcoma of the jaws cannot be distinguished from other destructive lesions of bone[21].
The tumour is not encapsulated; microscopically there is proliferation of fibroblasts with collagen and reticulin fibre formation. The amount of collagen and reticulin fibres is variable[18]. Sometimes fibrosarcoma contains hyalinised collagen that cannot be differentiated from osteoid substance.
Characteristically, the cells are usually giant spindle-shaped (spindle cell sarcoma), multipolar, with elongated oval or round hyperchromatic nuclei. These cells are arranged in interlocking bands that run in different directions, or in fascicles. Tumour cells may be arranged in a herring-bone pattern with areas of mixomatous, pseudomyxomatous and cartilaginous changes[22].
The degree of differentiation is extremely variable and it has been observed that the histological appearance of the tumours is related to grade of differentation. The accepted treatment is radical surgery.
Section snippets
Case report
A 4-year-old male child was referred to our Institute of Dental Discipline of the University of Naples, in January 1994, with a painless swelling that arose within a few weeks in the right mandibular region. Clinical examinations revealed a not well-circumscribed swelling (Fig. 1). The overlying mucosa showed an ulceration associated with a high mobility of the lower residual incisors. No regional lymphnodes were palpable. Radiographs showed a large multilocular radiolucency located between the
Materials and methods
Sections from formalin-fixed and paraffin-embedded blocks were stained with haematoxylin and eosin for routine histopathological examination. For the immunohistochemical techniques, 5-μm standard sections from paraffin-embedded tissue from each case were stained using the streptavidin–biotin complex immunoperoxidase. The antibody anti-vimentin (Dako-Italia) was used to stain neoplastic cells. Briefly, the tissue sections were incubated in citric acid, 10 mM, at 90°C for 60 min to enhance
Histological findings
The neoplasm was characterized by a proliferation of spindle-shaped cells surrounded by collagen fibres arranged in interlacing bundles (Fig. 3). In some fields a vascular proliferation with a haemangiopericytoma-like architectural pattern was present. The cellularity was high (Fig. 4) with a variable deposition of collagen. The nuclei appeared highly hyperchromatic. Rare mitotic figures were present. The tumour showed a tendency to infiltrate adjacent tissue. Immunohistochemically there was a
Discussion
Fibrosarcoma is a rare malignancy in the oral cavity; local recurrence is frequent, but metastases are rare. It is more frequent in the fifth and sixth decades of age but cases in children and congenital cases were described in the literature[24]. Fibrosarcoma spreads by both local invasion and haematogenous dissemination. A latent period of 10 years before metastases appear is quite possible, the commonest site for metastases being the lungs[22]. Infantile fibrosarcoma occurring in the first
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