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Primary fibrosarcoma of maxilla in an 8-year-old child: A rare entity.

Swain N, Kumar SV, Dhariwal R, Routray S - J Oral Maxillofac Pathol (2013)

Bottom Line: This condition primarily affects long bones, and its occurrence in the cranium is rare (15%), with the mandible being the most commonly involved cranial site.Here a case of primary FS in anterior maxilla of an 8-year-old male child is reported.This article is presented to document the rarity of FSs in the jaws of children with review of literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Pathology, Mahatma Gandhi Mission Dental College and Hospital, Kamothe, Navi, Mumbai, Maharashtra, India.

ABSTRACT
Fibrosarcoma (FS) is a malignant mesenchymal neoplasm of the fibroblasts that is uncommon in the head and neck and constitutes less than 1% of malignancies and approximately 6% of the soft tissue sarcomas. FSs rarely occur before the third decade except infantile type. This condition primarily affects long bones, and its occurrence in the cranium is rare (15%), with the mandible being the most commonly involved cranial site. Here a case of primary FS in anterior maxilla of an 8-year-old male child is reported. This article is presented to document the rarity of FSs in the jaws of children with review of literature.

No MeSH data available.


Related in: MedlinePlus

Tumor cells showing intense immunoreactivity for vimentin (IHC stain, ×200)
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Figure 5: Tumor cells showing intense immunoreactivity for vimentin (IHC stain, ×200)

Mentions: An 8-year-old male child reported with the complaint of fast growing swelling over middle of face. The patient did not give any history of systemic illness or trauma to the head and neck region. There was no significant contributing family history. He gave a history of painless but rapidly growing swelling in the anterior maxilla since 20 days, which attained to the present size of 7×10 cm. There was no associated history of difficulty in speech and mastication with incomplete closure of mouth. The patient had also given the history of previous incisional biopsy, which was conducted by a general physician with histopathological report of benign fibrous lesion. Intraorally, a globular sessile mass with an area of ulceration due to previous biopsy procedure was present in the anterior hard palate extending from 55 to 65 [Figure 1]. The buccal and palatal cortical plates were completely destroyed. Missing regional teeth was also noticed, which had the history of spontaneous exfoliation. The labial vestibule was completely obliterated. The orthopantomograph (OPG) revealed a purely radiolucent destructive lesion in the anterior maxilla [Figure 2]. A chest radiograph was advised to rule out the metastasis, which was found to be clear without any significant and relevant finding. With a clinical provisional diagnosis of bone malignancy, the intraoral mass was subjected to incisional biopsy. The Hematoxylin and Eosin (H and E) stained section showed nonencapsulated tumor tissue made up of highly cellular stroma arranged in interlacing fascicles [Figure 3]. The tumor tissue showed the presence of spindle-shaped cells with large nuclei and scanty cytoplasm. Mitotic figures (6-7/10 HPF) were observed [Figure 4]. In few areas, Herring bone pattern characteristic of FS was also noticed. Immunohistochemically, vimentin positive cells were abundant [Figure 5], whereas other immunomarkers like desmin, smooth muscle actin (SMA), S-100 were negative. A histopathological diagnosis of primary intraosseous FS (grade-II) was made. Patient had undergone surgical excision. The excisional biopsy was also consistent with the diagnosis of incisional biopsy. Finally the patient succumbed to the tumor within time period of 6 months of diagnosis.


Primary fibrosarcoma of maxilla in an 8-year-old child: A rare entity.

Swain N, Kumar SV, Dhariwal R, Routray S - J Oral Maxillofac Pathol (2013)

Tumor cells showing intense immunoreactivity for vimentin (IHC stain, ×200)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3927362&req=5

Figure 5: Tumor cells showing intense immunoreactivity for vimentin (IHC stain, ×200)
Mentions: An 8-year-old male child reported with the complaint of fast growing swelling over middle of face. The patient did not give any history of systemic illness or trauma to the head and neck region. There was no significant contributing family history. He gave a history of painless but rapidly growing swelling in the anterior maxilla since 20 days, which attained to the present size of 7×10 cm. There was no associated history of difficulty in speech and mastication with incomplete closure of mouth. The patient had also given the history of previous incisional biopsy, which was conducted by a general physician with histopathological report of benign fibrous lesion. Intraorally, a globular sessile mass with an area of ulceration due to previous biopsy procedure was present in the anterior hard palate extending from 55 to 65 [Figure 1]. The buccal and palatal cortical plates were completely destroyed. Missing regional teeth was also noticed, which had the history of spontaneous exfoliation. The labial vestibule was completely obliterated. The orthopantomograph (OPG) revealed a purely radiolucent destructive lesion in the anterior maxilla [Figure 2]. A chest radiograph was advised to rule out the metastasis, which was found to be clear without any significant and relevant finding. With a clinical provisional diagnosis of bone malignancy, the intraoral mass was subjected to incisional biopsy. The Hematoxylin and Eosin (H and E) stained section showed nonencapsulated tumor tissue made up of highly cellular stroma arranged in interlacing fascicles [Figure 3]. The tumor tissue showed the presence of spindle-shaped cells with large nuclei and scanty cytoplasm. Mitotic figures (6-7/10 HPF) were observed [Figure 4]. In few areas, Herring bone pattern characteristic of FS was also noticed. Immunohistochemically, vimentin positive cells were abundant [Figure 5], whereas other immunomarkers like desmin, smooth muscle actin (SMA), S-100 were negative. A histopathological diagnosis of primary intraosseous FS (grade-II) was made. Patient had undergone surgical excision. The excisional biopsy was also consistent with the diagnosis of incisional biopsy. Finally the patient succumbed to the tumor within time period of 6 months of diagnosis.

Bottom Line: This condition primarily affects long bones, and its occurrence in the cranium is rare (15%), with the mandible being the most commonly involved cranial site.Here a case of primary FS in anterior maxilla of an 8-year-old male child is reported.This article is presented to document the rarity of FSs in the jaws of children with review of literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Pathology, Mahatma Gandhi Mission Dental College and Hospital, Kamothe, Navi, Mumbai, Maharashtra, India.

ABSTRACT
Fibrosarcoma (FS) is a malignant mesenchymal neoplasm of the fibroblasts that is uncommon in the head and neck and constitutes less than 1% of malignancies and approximately 6% of the soft tissue sarcomas. FSs rarely occur before the third decade except infantile type. This condition primarily affects long bones, and its occurrence in the cranium is rare (15%), with the mandible being the most commonly involved cranial site. Here a case of primary FS in anterior maxilla of an 8-year-old male child is reported. This article is presented to document the rarity of FSs in the jaws of children with review of literature.

No MeSH data available.


Related in: MedlinePlus