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European Journal of Molecular & Clinical Medicine ISSN 2515-8260 Volume 07, Issue 5, 2020 Fibrosarcoma- A Malignant Tumour Of Origin E.Rajesh, Dr. G. NISHANTH, Dr. N.Anitha, Dr. K.M.K.Masthan

Department of Oral pathology and Microbiology Sree Balaji Dental College and Hospital and Research Bharath Institute of Higher Education

ABSTRACT:

Fibrosarcoma has been characterized as a malignant tumor of the that shows no other proof of cell separation and is equipped for repeat and metastasis. Fibrosarcomas are uncommon however may happen anyplace in the body, most ordinarily in the retroperitoneum, thigh, knee and distal limits. Fibrosarcoma is exceptional in the head and neck area and comprises about 1% of the relative multitude of influencing humankind. Of all the fibrosarcomas happening in people, just 0.05% happens in the head and neck region. Of this, practically 23% is found in the oral cavity. Fibosarcomas by and large have a helpless forecast and the general endurance rate is 20-35% over a time of 5 years.

Keywords: Fibroblasts, Fibrosarcoma, Connective tissue tumours,

INTRODUCTION:

Fibrosarcoma is a malignant tumor that emerges from the fibroblasts. This is a sort of that is dominatingly found in the territory around the bones or in delicate tissue. In prior investigations of delicate tissue , this tumor has been extraordinarily overdiagnosed, and this analysis has been often applied to for all intents and purposes any lavishly cell, -framing shaft cell tumor, including dangerous fibrous , Schwannoma and a large group of other sarcomatous and pseudosarcomatous lesions. [1] Malignancies of the fibroblasts are emphatically uncommon in the oral and oropharyngeal district, however fibrosarcoma is, by the by, the most well-known mesenchymal disease of the area, speaking to the greater part, everything being equal. Of all the fibrosarcomas happening in human, just 0.05% happens in the head and neck area. Of this, 23% of head and neck fibrosarcomas happen inside the oral cavity.[2],[3]

PATHOGENESIS:

The Specific reason for fibrosarcoma isn't altogether seen. Radiotherapy to the nearby site has been proposed as an inclining factor for expanded danger of Fibrosarcoma. Different components that can offer ascent to fibrosarcoma of the oral and para oral region incorporate tissues harmed by scarring and warmth. Disease processes like Paget's disease and osteomyelitis have likewise been ensnared in couple of cases in which the fibrosarcomas created in the bone. Fibrosarcoma somewhere else in the body creates in individuals between the ages of 25 and 79 years. The pinnacle frequency of event of this tumor is 55-69 years. In any case, fibrosarcomas in the head and neck locale create in the 3 rd and 5 th decade of life, however there is a wide age range and numerous patients are under 20 years old. Puerile or inherent fibrosarcoma is the most widely recognized delicate tissue sarcoma found in youngsters under 1 year old enough.[2,3,4,5,6] There is no sexual orientation inclination. By and large, the tumors create with equivalent recurrence in guys and females. In any case, in certain investigations, male inclination has been accounted for. Any submucosal site might be included albeit buccal mucosa, tongue and

1456 European Journal of Molecular & Clinical Medicine ISSN 2515-8260 Volume 07, Issue 5, 2020 alveolus represents the greater part of the cases. The side effects of fibrosarcoma change contingent upon size, area and spread of the tumor. Indications may incorporate agony, expanding or ulceration. Fibrosarcoma of the oral cavity regularly shows as a clinically harmless, lobulated, sessile, effortless and non-hemorrhagic submucosal mass of ordinary hue. Then again, forceful fibrosarcomas will in general be a quickly augmenting, hemorrhagic mass comparable in clinical appearance to a ulcerated pyogenic granuloma, fringe goliath cell granuloma or fringe hardening . Even lesions that don't exhibit surface ulceration or quick development may show obliteration of underlying muscle and bone. [6,7]

RADIOGRAPHIC FEATURES:

Radiographically, fibrosarcoma regularly shows up as an absolutely osteolytic sore without calcification and with inadequately characterized, unpredictable edges in the event that it has excite intraosseously. There is typically destruction of the cortical plates without expansion, and the lesion might be misdiagnosed as an odontogenic abscess or cyst. The underlying root of teeth might possibly show resorption [8].

HISTOPATHOLOGIC FINDINGS:

Well-differentiated forms have multiple plump fibroblasts with pale eosinophilic cytoplasm and deeply staining spindled nuclei with tapered ends. The malignant cells are dispersed in a rich collagen background. The lesion is typically scattered, histologically normal mitotic figures are seen in small numbers, but cells and nuclei are not pleomorphic. Intermediate grade tumors are cellular and have the typical herringbone pattern showing the diagnostic parallel sheets of cells arranged in intertwining whorls. Quite cellular with slight degree of cellular pleomorphism but moderate amounts of mature collagen may be produced, perhaps with areas of hyalinization. High-grade lesions are very cellular with marked cellular atypia and mitotic activity. The matrix is sparse. Multinucleated giant cells are rarely seen. No malignant osteoid formation should be present. Higher grades are extremely anaplastic and pleomorphic with bizarre nuclei that bring to mind the histologic features of malignant fibrous histiocytoma. In fact, some pathologists believe that the division between malignant fibrous histiocytoma, high-grade , and fibrosarcoma may be artificial. Immuno histochemical studies reveal positivity for , desmin, , and CD34. Sclerosing epithelioid fibrosarcoma is an uncommon tumor of deep soft tissues. Histologically, sclerosing epithelioid fibrosarcoma composed predominantly of small to moderate size, round to ovoid, relatively uniform cells, often with clear cytoplasm, embedded in a hyalinized fibrous stroma. The only consistent immunohistochemical finding was a strong, diffuse reactivity of tumor cells for .[10]

DIFFERENTIAL DIAGNOSIS:

In differential diagnosis, reactive fibromatosis, fibroblastic osteogenic sarcoma, pseudosarcomatous fasciitis, and cellular alveolar sarcoma must be excluded. The positive immunostaining for vimentin, together with negativity for muscular immunomarkers, will help establishing the diagnosis of the fibrosarcoma.

TREATMENT:

Tumors require radical surgery, including removal of potentially invaded muscle and bone. The use of is controversial, but is generally used in bone lesions. is used in conjunction with surgery for fibrosarcomas, with or without additional chemotherapy. Fibrosarcoma seldom metastasizes except late in its clinical course, but when this does occur the metastatic deposits are usually blood- born and carried to distant sites, especially the lungs, liver and bones. Radiotherapy may be used as salvage for recurrences. If all grades are included, primary fibrosarcoma of the bone has a worse prognosis than osteosarcoma, with a fiveyear survival rate of 65%. Specifically, in high-grade primary fibrosarcoma, the 10-year

1457 European Journal of Molecular & Clinical Medicine ISSN 2515-8260 Volume 07, Issue 5, 2020 survival rate is less than 30%. Secondary fibrosarcoma is associated with a very poor outcome, with a less than 10% survival rate at 10 years.[10]

CONCLUSION:

In conclusion, although are rare, meticulous patient histories, detailed clinical examination, and appropriate radiographic examination are very important in the evaluation of such cases. Oral physicians must always consider the possibility and be able to recognize the features of FS to propose appropriate investigations and help in treatment planning. This case report emphasizes the unique radiographic features of fibrosarcoma that have not been reported previously.

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2. 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.

3. Mark RJ, Sercarz JA, Tran L, Selch M, Calcaterra TC. Fibrosarcoma of the Head and Neck. The UCLA experience. Arch Otolaryngol Head Neck Surg 1991;117:396-401.

4. Oppenheimer RW, Friedman M. Fibrosarcoma of the maxillary sinus. Ear Nose Throat J 1988;67:193-8.

5. McKenna WG, Barnes MM, Kinsella TJ, Rosenberg SA, Lack EE, Glatstein E. Combined modality treatment of adult soft tissue sarcomas of the head and neck. Int J Radiat Oncol Biol Phys 1987;13:1127-33.

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7. K. Orhan, A. I. Orhan, U. Oz, F. N. Pekiner, and C. Delilbasi, “Misdiagnosed fibrosarcoma of the mandible mimicking temporomandibular disorder: a rare condition,” Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontology, vol. 104, no. 4, pp. e26–e29, 2007.

8. 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.

9. Wood RE. Malignant diseases of the jaw. In: White SC, Pharoah MJ, editors. Oral radiology principles and interpretation. St. Louis: Mosby; 2004; p. 474–5.

10. Soares AB, Lins LHS, Mazedo AP, Neto JSP, Vargas PA. Fibrosarcoma originating in the mandible. Med Oral Patol Oral Cir Bucal. 2006;11:e243–6.

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