|Year : 2014 | Volume
| Issue : 5 | Page : 47-50
Malignant fibrous histiocytoma: A rare case report
Thathekalva Sridhar1, Sreeram Vsnkata Suman2, Jayam Raviraj2, Dirasantchu Suresh2
1 Department of Oral Medicine and Radiology, Priyadarshini Dental College, Tamil Nadu, India
2 Department of Oral Medicine and Radiology, CKS Theja Dental College, Hospital and Research Centre, Tirupati, Andhra Pradesh, India
|Date of Web Publication||10-Mar-2014|
Sreeram Vsnkata Suman
Department of Oral Medicine and Radiology, CKS Theja Dental College, Hospital and Research Centre, Tirupati, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Malignant fibrous histiocytoma (MFH) is the most common soft-tissue sarcoma, but is relatively uncommon in the head and neck area. This tumor is difficult to distinguish histologically from other sarcomas and carcinomas. The most reliable treatment for MFH is surgery. Prognosis is poor and recurrence and local metastasis are common. In comparison with MFH of the extremities and trunk, the 5-year survival rate for cases of this tumor in the head and neck region is low. It is important to consider MFH in the differential diagnosis of head and neck tumors because of its poor prognosis. We report such a rare case in the rare site of maxillofacial region.
Keywords: Fibrous histiocytoma, malignant, mandible
|How to cite this article:|
Sridhar T, Suman SV, Raviraj J, Suresh D. Malignant fibrous histiocytoma: A rare case report. J NTR Univ Health Sci 2014;3, Suppl S1:47-50
|How to cite this URL:|
Sridhar T, Suman SV, Raviraj J, Suresh D. Malignant fibrous histiocytoma: A rare case report. J NTR Univ Health Sci [serial online] 2014 [cited 2022 Nov 30];3, Suppl S1:47-50. Available from: https://www.jdrntruhs.org/text.asp?2014/3/5/47/128490
| Introduction|| |
Malignant fibrous histiocytoma (MFH), the most common soft-tissue sarcoma of adults, was first described by O'Brien and Stout in the 1960s.  It was first recognized as a distinct clinicopathologic entity in the early 1960s as a pleomorphic sarcoma that contains both fibroblastic and histiocytic cells in varying proportions, arranged in a storiform pattern. In the 1970s, for the first time, Feldman and Norman described primary malignant tumor of bone that satisfied the histopathologic criteria of MFH.  It is now more convenient, for the purpose of description and comparison of behavior, to divide MFH into several subgroups proposed by Weiss,  including storiform-pleomorphic, myxoid, giant cell, inflammatory and angiomatoid types.  MFH is more common in males than in females and mostly occurs in the 5 th and 6 th decades of life.  Commonly it presents as a slowly enlarging mass in the affected area. Head and neck MFH can be confused with a variety of fibrous tumors and inflammatory conditions, including neurilemmoma, fibromatosis, premature sarcoma, hemangiopericytoma, granuloma, nodular fasciitis, malignant mesenchymal tumor, myoblastoma, fat necrosis, Langerhans cell histiocytosis, pleomorphic rhabdomyosarcoma and fibrosarcoma. 
| Case Report|| |
A 50-year-old male patient came with a complaint of swelling on the left side of the face since 4 months. History revealed that the swelling started as a small one on the left side of the face in the posterior tooth region of lower jaw 4 months back and since then, it had been gradually increasing to reach the present size. The swelling was not associated with pain or secondary changes.
His medical history and family history were non-contributory. Patient had history of smoking beedies 3-4 per day for 20 years.
On clinical examination, a diffuse swelling measuring about 5 cm anteroposteriorly and 4 cm superoinferiorly was seen on the left side of the face [Figure 1] involving ramus, lower border of the mandible and anteriorly about 2 cm short of the angle of mouth. Skin over the swelling was normal. On palpation, the swelling was bony hard in consistency, not movable, non-tender, lobulated and did not yield to pressure. Skin over the swelling was pinchable and edges of the swelling were well defined. No palpitations or discharge was elicited on palpation.
|Figure 1: Extraorally, a solitary swelling was seen in the left submandibular region extending from parasymphysis to the angle of mandible|
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Corresponding to the extraoral swelling, intraorally, a diffuse bony hard swelling was present [Figure 2]. The swelling was non-tender with bilateral expansion obliterating buccal sulcus from 35-38 region. Mucosa over the swelling was normal and aspiration was negative.
|Figure 2: Intraorally, a 1 × 1 cm swelling was seen in the left retromolar area with slight erythematous surface|
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As the swelling was slow growing, non-tender, not movable and hard in consistency, it was a bony swelling. As there was bilateral expansion, with negative aspiration, the swelling under investigation was a benign bony neoplasm, either odontogenic (ameloblastoma, odontogenic myxoma) or non-odontogenic (ossifying fibroma).
Hemogram showed blood parameters within normal limits.
Panoramic [Figure 3] radiograph showed irregular multilocular radiolucency in the posterior part of the mandible involving left ramus and body. The lower border of ramus was thinned out, but did not exhibit any fracture.
|Figure 3: Cropped panoramic radiograph reveals multilocular radiolucency in the left body and ramus of mandible|
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Computed tomography (CT) [Figure 4] showed an osteolytic expansile lesion occupying left parasymphysis and body of the mandible.
|Figure 4: Axial CT showing destructive lesion of the left mandible extending from parasymphysis to retromolar area|
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Upon incision biopsy, histopathology [Figure 5] showed proliferating fibroblasts and macrophages which showed cellular and nuclear pleomorphism. There was abnormal mitosis and presence of giant cells. There was also presence of abnormal proliferating spindle cells.
|Figure 5: Histopathology shows proliferating fibroblasts, macrophages and proliferating spindle cells|
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Based on clinical, radiographic and histopathologic features, a final clinical diagnosis of MFH was made.
The patient was referred to oral surgery and surgical excision of the lesion was done.
| Discussion|| |
MFH is the most common soft-tissue sarcoma in adults, accounting for 20-30% of all soft-tissue sarcomas. ,,, It remains relatively uncommon in the head and neck region, accounting for 3-10% of all cases. In addition to soft tissue, the tumor has been reported to occur in virtually every part of the body, including bone, viscera and skin.  In the head and neck area, the more common sites involved are the craniofacial bones (15-25%), larynx (10-15%), soft tissue of the neck (10-15%), major salivary glands (5-15%) and oral cavity (5-15%).  In our case, it was located in the left ramus and the body of mandible.
The etiology of this tumor is unknown, but seems to be multifactorial. Genetic background, environmental factors such as trauma, radiotherapy and malignant transformation from benign lesions can be involved in the etiology of this malignant tumor. 
Clinically, patients with MFH usually present with an enlarging, painless, solid soft-tissue mass. Most patients are between 50 and 70 years of age and men are affected 2-3 times more commonly than women.  Our case was a 50-year-old male and presented with similar features. Reported radiographic findings were irregular bone margin, a moth-eaten appearance, erosion of cortex, pathological fracture and tooth root resorption.  Kanazawa et al. reported that lesions of the mandible presented as an extensive, ill-defined osteolytic lesion radiographically and neither marginal sclerosis nor periosteal reaction was observed. In our case, it presented as a multilocular radiolucency. However, these radiographic findings are not specific to MFH and may be observed in squamous cell carcinoma, fibromyxoma, lymphoma, plasmacytoma and other malignant bone tumors.
Abdul-Karim et al. concluded that MFH of the jaws has a distinct clinical behavior exclusive to this location, being very aggressive with a high rate of local recurrences and frequent metastases. The behavior of MFH is more aggressive than that of fibrosarcoma and osteosarcoma in this site. Histopathologically, MFH can be divided into the following four morphologic subtypes in a decreasing order, depending on the predominant cellular components: storiform-pleomorphic (50-60%), myxoid (25%), giant cell (5-10%) and inflammatory (5%), some of which have prognostic significance. The myxoid variant has better prognosis compared with the storiform-pleomorphic type. It is not unusual for several morphologic patterns to be observed within one lesion. High-grade fibrosarcoma may resemble MFH, but the absence of giant cells in fibrosarcoma differentiates them.  In our case, histopathologically, proliferating fibroblasts, macrophages and proliferating spindle cells were observed.
According to Huvos, metastatic spread in patients with primary MFH in bone occurs via hematogenous dissemination, predominantly to the lungs, rather than to regional lymph nodes.
The prognosis of this tumor is often unfavourable and recurrence rate is approximately 44-48%. Metastases more commonly occur in the lung (90%) followed by lymph nodes (12%), bone (8%) and liver (1%). About 5% of cases already reveal metastasis when the primary tumor is diagnosed. 
To conclude, MFH is relatively uncommon in the head and neck region, which manifested in our case as an asymptomatic swelling in the posterior mandible and radiographically showed a multilocular radiolucent destructive lesion. The most reliable treatment for MFH is surgery. Prognosis is poor and recurrence and local metastasis are common.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]