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Fibroblastic variant of osteosarcoma: a challenge in diagnosis & management.

Desai D, Pandith S, Jeergal PA, Arathi K, Saini R - Open Dent J (2010)

Bottom Line: Osteosarcoma of the jaws is a relative rare malignant bone tumor.Its diagnosis is a challenge to histopathologists and is especially important in early stages to improve its prognosis.In the initial phase, it may present as nondescript swelling with an indolent growth rate, only to become overtly aggressive and malignant towards the later phase of the disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral and Maxillofacial Pathology, A.J. Institute of Dental Sciences, Mangalore 575004, Karnataka, India.

ABSTRACT
Osteosarcoma of the jaws is a relative rare malignant bone tumor. Like, its counterpart in the long bones, osteosarcoma affecting the head and neck region shows distinct yet diverse clinical, histologic and prognostic characteristics. Its diagnosis is a challenge to histopathologists and is especially important in early stages to improve its prognosis. In the initial phase, it may present as nondescript swelling with an indolent growth rate, only to become overtly aggressive and malignant towards the later phase of the disease. This article reports on a case of an advanced osteosarcoma of the maxilla in a 42 year old woman who came for the evaluation of a swelling. The case was submitted for surgical intervention and was followed by one recurrence till the time of reporting. This case illustrates that immunohistochemical staining of Vimentin, S-100 and CD 68 markers are useful to confirm the histologic diagnosis of osteosarcoma, along with radiographic evaluation using CT scan and 3D imaging.

No MeSH data available.


Related in: MedlinePlus

A. Further increase in the size of the swelling causing inability in closing the mouth. B. Immediate partial maxillectomy being carried out. C. Complete resected mass measured 14x8 cms.
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Figure 3: A. Further increase in the size of the swelling causing inability in closing the mouth. B. Immediate partial maxillectomy being carried out. C. Complete resected mass measured 14x8 cms.

Mentions: An incisional biopsy was performed and histopathological findings showed a nonspecific infection suggesting an inaccurate biopsy site (Fig. 2C). A repeat biopsy was requested in relation to deeper areas. However, patient reported back within two days of incisional biopsy with a further increase in the size of the swelling with the lesion protruding out of the oral cavity causing inability to close the mouth (Fig. 3A). An immediate partial maxillectomy was done based on the clinical features, computed tomography findings, to avoid further encroachment of the base of the skull (Fig. 3B). The resected mass weighed about 250gms, measuring 14x8 cms (Fig. 3C). Histopathology showed the lesion to be composed of proliferating fibroblasts along with bundles of collagen fibers. A markedly cellular lesion comprising of mature proliferating fibroblasts with infrequent mitosis and occasional atypia was seen (Fig. 4A). There was a presence of abundant inflammatory cells along with few reactionary giant cells, interspersed with a myxoid background (Fig. 4B). Storiform pattern of arrangement of fibroblasts was also seen, with the fibroblasts showing atypia in relation to size and shape. Invasion into the adjacent adipose tissue was also noted. Immunohistochemical staining was performed with vimentin markers and S-100 protein. Vimentin positivity proved the myofibroblastic nature of the spindle cell lesion (Fig. 4C). S-100 was negative, thus ruling out neural tumours (Fig. 4D). Based on the clinical picture, rate of growth of the lesion and histopathological findings, a final diagnosis of fibrosarcoma was given. Patient reported back with an uneventful healing following which functional and esthetic reconstruction was done by giving an obturator. After hemimaxillectomy, chemotherapy was administered which included Cysplatin 80 mg/m2 on day 1 along with 5 Fluorouracil - 500mg/m2 which was administered on day 1 to 4. This resulted in partial response of the tumour with stoppage of bleeding. Patient also underwent a course of radiotherapy (60 Gy over 6 weeks). The decision to give radiotherapy was made taking into consideration the large size of the lesion along with a history of recurrence. After surgery, an acrylic plate obturator was placed which was to be replaced by a cast partial hollow bulb closed obturator.


Fibroblastic variant of osteosarcoma: a challenge in diagnosis & management.

Desai D, Pandith S, Jeergal PA, Arathi K, Saini R - Open Dent J (2010)

A. Further increase in the size of the swelling causing inability in closing the mouth. B. Immediate partial maxillectomy being carried out. C. Complete resected mass measured 14x8 cms.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3020523&req=5

Figure 3: A. Further increase in the size of the swelling causing inability in closing the mouth. B. Immediate partial maxillectomy being carried out. C. Complete resected mass measured 14x8 cms.
Mentions: An incisional biopsy was performed and histopathological findings showed a nonspecific infection suggesting an inaccurate biopsy site (Fig. 2C). A repeat biopsy was requested in relation to deeper areas. However, patient reported back within two days of incisional biopsy with a further increase in the size of the swelling with the lesion protruding out of the oral cavity causing inability to close the mouth (Fig. 3A). An immediate partial maxillectomy was done based on the clinical features, computed tomography findings, to avoid further encroachment of the base of the skull (Fig. 3B). The resected mass weighed about 250gms, measuring 14x8 cms (Fig. 3C). Histopathology showed the lesion to be composed of proliferating fibroblasts along with bundles of collagen fibers. A markedly cellular lesion comprising of mature proliferating fibroblasts with infrequent mitosis and occasional atypia was seen (Fig. 4A). There was a presence of abundant inflammatory cells along with few reactionary giant cells, interspersed with a myxoid background (Fig. 4B). Storiform pattern of arrangement of fibroblasts was also seen, with the fibroblasts showing atypia in relation to size and shape. Invasion into the adjacent adipose tissue was also noted. Immunohistochemical staining was performed with vimentin markers and S-100 protein. Vimentin positivity proved the myofibroblastic nature of the spindle cell lesion (Fig. 4C). S-100 was negative, thus ruling out neural tumours (Fig. 4D). Based on the clinical picture, rate of growth of the lesion and histopathological findings, a final diagnosis of fibrosarcoma was given. Patient reported back with an uneventful healing following which functional and esthetic reconstruction was done by giving an obturator. After hemimaxillectomy, chemotherapy was administered which included Cysplatin 80 mg/m2 on day 1 along with 5 Fluorouracil - 500mg/m2 which was administered on day 1 to 4. This resulted in partial response of the tumour with stoppage of bleeding. Patient also underwent a course of radiotherapy (60 Gy over 6 weeks). The decision to give radiotherapy was made taking into consideration the large size of the lesion along with a history of recurrence. After surgery, an acrylic plate obturator was placed which was to be replaced by a cast partial hollow bulb closed obturator.

Bottom Line: Osteosarcoma of the jaws is a relative rare malignant bone tumor.Its diagnosis is a challenge to histopathologists and is especially important in early stages to improve its prognosis.In the initial phase, it may present as nondescript swelling with an indolent growth rate, only to become overtly aggressive and malignant towards the later phase of the disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral and Maxillofacial Pathology, A.J. Institute of Dental Sciences, Mangalore 575004, Karnataka, India.

ABSTRACT
Osteosarcoma of the jaws is a relative rare malignant bone tumor. Like, its counterpart in the long bones, osteosarcoma affecting the head and neck region shows distinct yet diverse clinical, histologic and prognostic characteristics. Its diagnosis is a challenge to histopathologists and is especially important in early stages to improve its prognosis. In the initial phase, it may present as nondescript swelling with an indolent growth rate, only to become overtly aggressive and malignant towards the later phase of the disease. This article reports on a case of an advanced osteosarcoma of the maxilla in a 42 year old woman who came for the evaluation of a swelling. The case was submitted for surgical intervention and was followed by one recurrence till the time of reporting. This case illustrates that immunohistochemical staining of Vimentin, S-100 and CD 68 markers are useful to confirm the histologic diagnosis of osteosarcoma, along with radiographic evaluation using CT scan and 3D imaging.

No MeSH data available.


Related in: MedlinePlus