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      Cloud-like pattern of mineralization in skull base osteosarcoma

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          Abstract

          Osteosarcoma derives from primitive bone-forming mesenchymal cells, although it is the most common primary bone malignancy,[1–2] osteosarcoma of the skull base is very rare.[1 3–6] A 40-year-old woman, a known case of left temporal bone osteosarcoma operated 3 years back, now presented with progressively increasing swelling over the previously operated site for the previous 6 months and foul smelling discharge for 3 days. Not able to take food, breathing difficulty, and was in altered sensorium. At the time of initial presentation (3 years back) there was pinkish, firm to hard, moderately vascular, nonsuckable tumor, infiltrating into the temporal muscle and fascia with ill-defined plane of cleavage. The tumor was adherent to the dura but there was no dural invasion. Histologically the tumor comprised highly pleomorphic tumor cells, findings suggestive of anaplastic variant of osteosarcoma and a poor prognosis was predicted. The patient received a course of chemotherapy but did not receive radiotherapy. Plain axial CT images of the head soft tissue and bone showed an aggressive, densely ossified bone-forming tumor with extensive involvement of the soft tissue and bony destruction of the anterior, middle, and part of the posterior cranial fossa on left side [Figure 1]. The patient was managed conservatively but did not do well and expired after 3 days of admission. Figure 1 Plain axial CT images of the head soft tissue and bone showing an aggressive, densely ossifi ed bone-forming tumor with extensive involvement of the soft tissue and bony destruction of the anterior, middle, and part of the posterior cranial fossa on left side producing cloud-like pattern of mineralization Fibro-osseous lesions are characteristically centered within bone and surface fibro-osseous lesions prompt consideration of parosteal osteosarcoma, which is rare but well documented in the skull.[7] Because of the superimposed bony structures, conventional radiographs are of limited worth in evaluating head and neck osteosarcomas.[8] However, CT and plain films are superior to MR in detecting the matrix calcifications and bone destruction or reaction.[8] CT provides excellent detection of tumor calcification, cortical involvement, and, in most instances, soft tissue as well as intramedullary extension[8] as in the present case, on axial CT poorly defined destructive osteosarcoma that neoplasm extends into the soft tissues produces cloud-like pattern of mineralization.[2] The key to disease-free survival in treating primary osteosarcoma of the calvarium is complete surgical resection with immediate reconstruction followed by adjuvant chemotherapy.[4] In skull base osteosarcoma, prognosis is poor even in patients who have received adequate therapy.[6]

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          Update on bone forming tumors of the head and neck.

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            Craniofacial osteosarcomas: plain film, CT, and MR findings in 46 cases.

            Forty-six osteosarcomas of the cranial and facial bones were reviewed radiographically by using the conventional parameters for long bone tumors. There were 32 de novo osteosarcomas (11 maxillary, 13 mandibular, and eight cranial) and 14 postradiation osteosarcomas. All the maxillary tumors originated from the alveolar ridge, and the majority of mandibular lesions began in the body of the mandible. The postradiation osteosarcomas occurred in portions of bones at the borders of the radiation field; the latent period ranged from 4 years, 2 months to 50 years (mean, 14 years). The majority of de novo or postradiation craniofacial osteosarcomas were osteolytic with a long transition zone and no periosteal reaction; the exception was in the mandible, where nearly half the cases were osteoblastic and periosteal reaction was occasionally present. Tumor matrix mineralization occurred in more than 75% of the cases, and osteoid matrix calcification was most frequent, even though most tumors were chondroblastic. Soft-tissue extension of tumor was present in all cases and contained calcifications in more than half. Conventional radiographs are of limited value in evaluating head and neck osteosarcomas because of the superimposed bony structures. CT provides excellent detection of tumor calcification, cortical involvement, and, in most instances, soft-tissue and intramedullary extension. MR is even more effective in demonstrating the intramedullary and extraosseous tumor components on both T1- and T2-weighted images. However, CT and plain films are superior to MR in detecting the matrix calcifications and bone destruction or reaction.
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              Osteosarcoma of the skull base: case report and review of literature.

              Osteosarcoma is the most common primary malignancy of bone in children and adolescents. Osteosarcomas are an aggressive neoplasm composed of spindle cells producing osteoid. They primarily affect the long bones, particularly after radiation or chemotherapy for other neoplasms; however, 6-7% present in the head and neck. Primary head and neck osteosarcomas in children are rare. There are few case reports and limited-sized case series in the literature. A case report presentation of a skull base osteosarcoma in a teenage female. A 14-year-old African American female presented with dysphagia, voice changes, and neck pain. On examination, she had right-sided palsies in cranial nerves X, XI, and XII. Imaging revealed partial enhancement of the clivus without bony erosion and expansion of the hypoglossal canal. There were also findings consistent with chronic denervation of her right tongue and pharynx. During the evaluation process, she developed diplopia from a right cranial nerve VI palsy. Repeat imaging revealed progression of the skull base lesion with extension into the right sphenoid sinus. An endoscopic sphenoidotomy was performed to obtain tissue. The diagnosis of high-grade osteosarcoma was made by histologic morphology and immunohistochemistry. The child was treated primarily with chemotherapy. Other adjunctive therapies are being considered. Osteosarcoma of the skull base is a rare entity. We describe a case of a high-grade clival osteosarcoma presenting primarily with lower cranial nerve palsies and pain. The rapid progression, treatment options, and prognosis are discussed.
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                Author and article information

                Journal
                J Neurosci Rural Pract
                JNRP
                Journal of Neurosciences in Rural Practice
                Medknow Publications (India )
                0976-3147
                0976-3155
                Jan-Jun 2011
                : 2
                : 1
                : 103-104
                Affiliations
                [1] Department of ENT, MM Institute of Medical Sciences & Research, Mullana (Ambala), India
                [1 ] Department of Neurosurgery, MM Institute of Medical Sciences & Research, Mullana (Ambala), India
                [2 ] Department of Radiology, MM Institute of Medical Sciences & Research, Mullana (Ambala), India
                [3 ] Department of Surgery, MM Institute of Medical Sciences & Research, Mullana (Ambala), India
                Author notes
                Address for correspondence: Dr. Lakshmi Narayan Garg, Department of ENT, MM Institute of Medical Sciences & Research Maharishi Markandeshwar University, Mullana- Ambala, 133-203 (Haryana), India. E-mail: dr.lngarg@ 123456gmail.com
                Article
                JNRP-2-103
                10.4103/0976-3147.80088
                3122992
                21716873
                c5e500fd-c4ef-4ed7-81e1-a70600805774
                © Journal of Neurosciences in Rural Practice

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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                Neurosciences
                Neurosciences

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