|Year : 2015 | Volume
| Issue : 2 | Page : 294-297
Myxofibrosarcoma of maxilla: A case report of rare entity
Venkateswarlu Nallapu, Bhavya Balasankulu, Sailaja Sambhana, Hima Bindu Vuppalapati
Department of Oral Medicine and Radiology, Government Dental College and Hospital, Hyderabad, Telangana, India
|Date of Submission||10-Jan-2015|
|Date of Acceptance||22-Sep-2015|
|Date of Web Publication||21-Nov-2015|
House No. 108, Block No. 8A, APIIC Colony, Opposite Radhika Theatre, ECIL, Hyderabad - 500 062, Telangana
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients that rarely affect the oral cavity. In this article we describe a case of primary myxofibrosarcoma of maxilla in a 19-year-old boy. Microscopically the tumor showed loosely arranged spindle-shaped tumor cells with mild pleomorphic spindle-shaped nuclei, some with minute nucleoli in a fibromyxoid stroma. The cells were strongly immunoreactive for vimentin and negative for CD-68, S-100 protein and desmin. Based on clinical, histological and immunohistochemical findings, a final diagnosis of myxofibrosarcoma was established. It is an unusual aggressive variant that frequently recurs and metastasizes, reinforcing the importance of its correct diagnosis. We report a rare case of myxofibrosarcoma in maxilla, with emphasis on immunohistochemical findings.
Keywords: Fibrosarcoma, immunohistochemistry, maxilla, myxofibrosarcoma
|How to cite this article:|
Nallapu V, Balasankulu B, Sambhana S, Vuppalapati HB. Myxofibrosarcoma of maxilla: A case report of rare entity. J Indian Acad Oral Med Radiol 2015;27:294-7
|How to cite this URL:|
Nallapu V, Balasankulu B, Sambhana S, Vuppalapati HB. Myxofibrosarcoma of maxilla: A case report of rare entity. J Indian Acad Oral Med Radiol [serial online] 2015 [cited 2019 May 22];27:294-7. Available from: http://www.jiaomr.in/text.asp?2015/27/2/294/170163
| Introduction|| |
In the days before the term "high-grade undifferentiated pleomorphic sarcoma" came into use, one of the most common sarcoma diagnoses was "malignant fibrous histiocytoma," and before that, in an era before immunohistochemistry, "fibrosarcoma" was used to describe most sarcomas.  The terms "myxofibrosarcoma" and "myxosarcoma" refer to a connective tissue neoplasm of fibroblastic origin set in myxoid matrix which has been classified by O'Brien and Stout as a myxoid variant of malignant fibrous histiocytoma. In 2002, the World Health Organization declassified the malignant fibrous histiocytoma (MFH) as a diagnostic entity and determined that myxoid MFH without myogenic, lipoblastic and chondrogenic features be diagnosed as myxofibrosarcoma.  However, myxofibrosarcoma is now believed by many authors to be a clinically distinct entity.  They advocated naming it myxofibrosaroma instead of myxoid malignant fibrous histiocytoma. It is the most common soft tissue sarcoma that occurs in late adult life, with peak incidence in the seventh decade. It grows commonly in the subcutaneous region of the extremities of elderly people, with rare occurrences in the head and neck (2.7%). 
| Case Report|| |
A 19-year-old male patient presented with a complaint of swelling on left side of his face and pain in his upper left back teeth since 3 months. Initially he gives history of a small swelling in front of his left ear and pain in his upper left back teeth. Pain was spontaneous in onset, intermittent, severe throbbing type, radiating to left ear, aggravates on chewing food, lying down and relieves on taking pain killers. He underwent removal of the painful tooth in a local dental clinic, followed by which the swelling gradually increased and reached the present size. He gives history of placement of smokeless form of tobacco over the painful tooth.
On examination, gross facial asymmetry was detected due to the swelling on left side of his face and the patient had difficulty in mouth opening. On intraoral examination, a single swelling was seen on the left alveolar ridge extending anteriorly from 24 to the maxillary tuberosity posteriorly with missing 27 and 28, laterally obliterating the buccal vestibule and medially onto the palate. The surface was smooth, except for an ulcerative defect distal to 26 with a fleshy black exophytic mass arising from it. On palpation it was tender, soft to firm in consistency and associated with grade-II mobile 26 [Figure 1]. Based on the above findings, a clinical diagnosis of carcinoma of left maxilla was made.
|Figure 1: Clinical photograph showing swelling with fleshy black exophytic mass on left posterior maxillary alveolar ridge|
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Waters (PNS/Para nasal sinus) view showed opacification of left maxillary sinus, destruction of its lateral wall, loss of continuity of innominate line with diffuse, irregular, ill-defined radiolucency involving the left zygomatic bone and its processes [Figure 2]. An orthopantomograph (OPG) showed diffuse, irregular, ill-defined radiolucency in the left maxilla extending from 26 anteriorly to involvement of the posterior wall of maxilla and destruction of zygomatic bone, arch, part of coronoid, condyle and ramus of mandible, posteriorly [Figure 3]. Computed tomographic (CT) scan with contrast in coronal and axial sections showed a well-defined lobulated mass in the left maxillary region with intranasal, intraorbital, intracranial and intratemporal extension with destruction suggestive of a maxillary sinus tumor or sarcoma.
|Figure 2: Waters (Para-nasal sinus) view shows opacification of left maxillary sinus, destruction of its lateral wall, loss of continuity of innominate line with diffuse, irregular, ill-defi ned radiolucency involving the left zygomatic bone and its processes|
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|Figure 3: OPG shows diffuse, irregular, ill-defined radiolucency in the left maxilla extending from 26 anteriorly to involve the posterior wall of maxilla and destruction of zygomatic bone, arch, part of coronoid, condyle and ramus of mandible, posteriorly|
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Fine needle aspiration cytology (FNAC) was performed and the cytosmear showed numerous cohesive bundles of spindle cells with oval to elongated hyper chromatic nuclei and few bare nuclei of tumor cells dispersed in a hemorrhagic background suggestive of low grade mesenchymal tumor. An intraoral incisional biopsy was performed. Hematoxylin and eosin-stained biopsy section under higher magnification (100X) showed loosely arranged spindle-shaped tumor cells with mild pleomorphic spindle-shaped nuclei, some with minute nucleoli in a fibromyxoid stroma [Figure 4], features suggestive of spindle cell sarcoma.
|Figure 4: Hematoxylin and eosin-stained section at high power (100× magnification) view showing loosely arranged spindle-shaped tumor cells with mild pleomorphic spindle-shaped nuclei in a fibromyxoid stroma|
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"Spindle cell" is a descriptive phrase that denotes the cellular shape of many of the sarcomas encountered in the adult population. Differential diagnosis includes monophasic fibrous synovial sarcoma, malignant fibrous histiocytoma, malignant nerve sheath tumor, liposarcoma, spindle cell carcinoma as well as benign tumors, i.e., benign fibrous histiocytoma, nodular fasciitis, fibroma and fibromatosis.  In order to confirm the diagnosis, immunohistochemistry (IHC) was performed using polymer IHC detection system. In our case, immunohistochemistry included Ki-67 antigen which is useful in determining the proliferation index; S-100, a protein widely distributed in peripheral and central nervous system, enabling a distinction to be made from malignant peripheral nerve sheath tumor; CD68, a fibrohistiocytic marker; vimentin a mesenchymal marker and muscle marker, i.e., desmin. And the tumor cells displayed intense immunoreactivity (positive) for vimentin, focal positivity for Ki-67, negativity for S-100, CD-68 and desmin [Figure 5] [Figure 6] [Figure 7] [Figure 8].
|Figure 7: Neoplastic cells showing intense immunoreactivity with vimentin|
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Based on the above features, a final diagnosis of myxofibrosarcoma was established. Considering the advanced stage of the disease, patient was given palliative therapy. Thereafter, health of the patient rapidly declined and he died 6 months after the initial diagnosis.
| Discussion|| |
Fibrosarcoma was once considered to be one of the most common of the malignant soft tissue neoplasms. However, the gradual separation of other tumors from this group has reduced the apparent frequency of the disease, so that today fibrosarcoma, particularly of the head and neck area, is a quite uncommon neoplasm.  Different histological types of fibrosarcoma exist, one of which is myxofibrosarcoma, which was initially described by Angerval in 1977.  Myxofibrosarcoma has been defined as a malignant fibroblastic lesion in which at least 50% of the entire tumor displays a highly vascularized and myxoid stroma with distinctive curvilinear vessels. It often grows slowly and painlessly with almost equal incidence in men and women. Clinically it can cause pain, swelling, paresthesia, occasionally loosening of teeth and ulceration of the overlying mucosa. Radiographically it presents as an osteolytic lesion with ill-defined borders which cannot be distinguished from other destructive lesions of the bone. Although magnetic resonance imaging and computed tomography scans evidently make a great contribution to the visualization of malignant features, such as local tissue invasion, histopathological examination is recognized as the gold standard, for its capability to provide a definitive diagnosis. It is characterized by low cellularity composed of spindle-shaped cells with minimal cytological atypia, and with cells deposited in a variably fibrous and myxoid stroma, usually appearing more myxoid than fibrous.
The diagnosis of a myxofibrosarcoma based solely on morphologic features is unacceptable and only those with immunohistochemical evidence should be regarded as myxofibrosarcoma. It stains strongly positive for the intermediate filament vimentin. Markers for muscle (desmin, smooth muscle actin), human osteoblasts (osteocalcin), macrophages (CD-68), leukocyte common antigen (LCA), neural tissue (S-100, neuron-specific enolase), melanoma (S-100, HMB-45), neutrophils (CD-31), hematopoietic cells (CD-34), epithelial tissue (cytokeratin, epithelial membrane antigen) and CD-99 will be absent. ,
The local recurrence rate of the low-grade type is as high (50-60%) as that of the high-grade type, with an overall risk of approximately 20-25% of metastasis;  thus, it is obvious that wide surgical treatment is necessary to suppress the risk of local recurrence and the risk of histological progression. The need for adjuvant radiotherapy and/or chemotherapy is still unclear, and is normally indicated in high-grade tumors because these may present subclinical or microscopic metastases at the time of diagnosis. Prophylactic neck dissection is also controversial.
| Conclusion|| |
Thus, to summarize, diagnosis of myxofibrosarcoma in the head and neck region is challenging because of it's overlapping histopathological features with other spindle cell tumors. Understanding their clinical, morphological and immunohistochemical features with a systematic approach is critical for their accurate diagnosis which aids in correct patient management.
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Conflicts of interest
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| References|| |
Collini P, Sorensen PH, Patel S, Blay JY, Issels RD, Maki RG, et al
. Sarcomas with spindle cell morphology. Semin Oncol 2009;36:324-37.
Fletcher CD, Mehrtens F. World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. France: IARC Press; 2002. p. 102-3.
Huang HY, Lal P, Qin J, Brennan MF, Antonescu CR. Low-grade myxofibrosarcoma: A clinicopathologic analysis of 49 cases treated at a single institution with simultaneous assessment of the efficacy of 3-tier and 4-tier grading systems. Hum Pathol 2004;35:612-21.
Mentzel T, Calonje E, Wadden C, Camplejohn RS, Beham A, Smith MA, et al
. Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol 1996;20:391-405.
Lo Muzio L, Mignogna MD, Pannone G, Staibano S, Testa NF. A rare case of fibrosarcoma of the jaws in a 4-year-old male. Oral Oncol 1998;34:383-6.
Rajendran R. Benign and malignant tumors of the oral cavity. In: Rajendran R, Sivapathasundharam B, editors. Shafer's Textbook of Oral Pathology, 5 th
ed. New Delhi: Elsevier; 2007. p. 113-307.
Angervall L, Kindblom LG, Merck C. Myxofibrosarcoma: A study of 30 cases. Acta Pathol Microbiol Scand 1997;85A:127-40.
Hattinger CM, Tarkkanen M, Benini S, Pasello M, Stoico G, Bacchini P, et al
. Genetic analysis of fibrosarcoma of bone, a rare tumor entity closely related to osteosarcoma and malignant fibrous histiocytoma of bone. Eur J Cell Biol 2004;83:483-91.
Antonescu CR, Errandson RA, Huvos AG. Primary fibrosarcoma and malignant fibrous histiocytoma of bone- a comparative ultrastructural study: Evidence of a spectrum of fibroblastic differentiation. Ultrastruct Pathol 2000;24:83-91.
Huang HY, Lal P, Qin J, Bennan MF, Antonescu CR. Low grade myxofibrosarcoma: A clinicopathologic analysis of 49 cases treated at a single institution with simultaneous assessment of the efficacy of 3-tier and 4-tier grading systems. Hum Pathol 2004;35:612-21.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]