Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chondrosarcoma of the skull base is a rare neoplasm. The most common presenting symptoms are hoarseness, dysphagia, and diplopia, which is associated with palsies of cranial nerves X, IX, and VI. The temporal bone is the most common site of tumor origin, followed by the sphenoid bone. These tumors must be differentiated from chordoma, chondroid chordoma, osteogenic sarcoma, enchondroma, and meningioma. Diagnosis is made by patient history, radiologic imaging, and biopsy. The treatment is surgical excision followed by radiation therapy. Five-year survival rates for grades I, II, and III are 90, 81, and 43%, respectively.
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PMID:Extracranial skull base chondrosarcoma. 268 2

A case of osteogenic sarcoma of the ethmoid sinus is reported in a 45 year old man. The tumour extended superiorly through the frontal sinus and involved the medial wall of the left orbit causing diplopia. Treatment comprised partial excision followed by radiotherapy and chemotherapy with symptomatic improvement, although 13 months later the patient died from progressive local and metastatic disease. A possible relationship to previous radiation exposure is discussed.
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PMID:Osteogenic sarcoma of the ethmoid sinus. 322 34

Primary extraskeletal osteosarcoma occurring in the brain parenchyma is distinctly uncommon, with only five cases having been reported. The authors describe the case of a 45-year-old man who presented with progressive headache and diplopia. Computerized tomography scanning and magnetic resonance imaging results revealed a pineal region tumor with obstructive hydrocephalus. The patient underwent partial resection of the tumor. The histological examination showed large pleomorphic tumor cells embedded in osteoid matrix. Immunohistochemical analysis was negative for various antibodies and thus excluded a glial, germ cell, epithelial, and lymphoid tumor origin. Only vimentin showed strong positivity in most of the tumor cells. Ultrastructurally, the tumor cells were rich in dilated rough endoplasmic reticula. Clear zones between tumor cells and osteoid matrix were observed. The osteoid matrix was made up of small collagen fibrils and hydroxyapatite deposits. The tumor was not attached to the bone structure of the skull. These findings are consistent with the features of extraskeletal osteosarcoma. Data from complete medical and radiological studies excluded a metastatic origin for this tumor. Partial resection and postoperative radiotherapy had provided tumor control at 11 months after the onset of symptoms. This is the first reported case of a primary extraskeletal osteosarcoma occurring in the pineal region.
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PMID:Primary extraskeletal osteosarcoma in the pineal region. Case report. 1559 71

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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PMID:Osteosarcoma of the skull base: case report and review of literature. 1798 Sep 19

Spontaneous degeneration of sarcomatosis of fibrous dysplasia is a rare phenomenon in adolescence. Fibrous dysplasia is often a deforming and devastating condition that begins in childhood and accounts for approximately 7.5% of the benign bone neoplasms. Approximately 50% to 100% of patients with polyostotic disease and 10% with monostotic disease have craniofacial involvement. This report is about a 16-year-old adolescent boy who had rapid facial mass growth and diplopia at the time of his referral to our center. The patient had been diagnosed with fibrous dysplasia a year earlier, and since the first diagnosis, he was submitted to only 1 surgical intervention, which had been followed by recurrence. The main clinical findings were a bilateral zygomatic mass, saddling of the nasal dorsum, diplopia, superior and inferior gaze, and numbness in the right inferior orbital nerve. The patient had a high level of serum alkaline phosphatase. The diagnosis of osteosarcoma was established by biopsy, and the patient underwent surgical treatment. His postsurgery results were good, and he did not experience a relapse until 1 year after surgery. Unfortunately, 6 months after the recurrence of his condition, he died of uncontrollable local spread.
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PMID:Spontaneous conversion of fibrous dysplasia into osteosarcoma. 2155 13

Primary epithelioid sarcoma (ES) of bone is extremely rare with only 2 reported cases in the English literature. A previously healthy 18-year-old man presented with a 6-month history of right facial numbness and tingling and right eye diplopia. A computerized tomography scan revealed an ill-defined mass with dense osseous matrix centered in the right zygomatic bone. An outside biopsy was read as osteosarcoma. The resection specimen revealed large epithelioid and spindle cells embedded in a prominent hyalinized matrix with focal metaplastic bone formation. The tumor cells were strongly and diffusely positive for AE1/AE3 and epithelial membrane antigen, but a definitive diagnosis of ES was not immediately reached due to the presence of dense hyalinized matrix and weak expression of SAT2B by tumor cells. Deficient INI1 protein expression by immunohistochemistry and homozygous loss of the SMARCB1 gene by chromosomal microarray analysis ultimately justified this tumor's designation as ES.
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PMID:Primary Epithelioid Sarcoma of the Zygomatic Bone. 2744 26

Plasmacytoma of the skull-base is a rare entity. Differential diagnosis includes chordoma, osteosarcoma, carcinoma nasopharynx, meningioma, metastatic carcinoma, lymphoma, and multiple myeloma. Accurate and precise diagnosis is extremely important for plasmacytoma of the skull-base as its treatment and prognosis is different from other skull-base lesions. A 41-year-old man presented with concerns of headache, diplopia, and left eye strabismus. A magnetic resonance image (MRI) of his brain showed a large expansile mass measuring 51 mm involving the clivus and central skull-base. Trans-sphenoidal tumor decompression was done. A biopsy confirmed the plasmacytoma. A positron emission tomography-computed tomography (PET-CT) scan showed a single 2-(18F) fluoro-D-glucose (FDG) avid lesion at the skull-base. The results of all other relevant investigations such as hemoglobin, renal function test, serum calcium, serum protein immunoelectrophoresis, serum quantitative immunoglobulin, bone marrow biopsy, serum lactate dehydrogenase, and beta-2 microglobulin levels were within normal limits. He was treated with radical radiotherapy. He developed complete clinical response after radiotherapy.
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PMID:Plasmacytoma of the Skull-base: A Rare Tumor. 2955 35