Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumour. To date, 60 well-documented cases have been published in the English-language literature. Two additional cases located in the posterior region of the mandible are reported. The relevant clinicopathological features of these cases, as well as those previously reported are discussed. Approximately two-thirds of AFS are malignant tumors de novo, with an average age of the affected patients being 22.9 years. This age is lower than the one observed in AFS developing from a pre-existent benign lesion (mean 33 years). Although regional and distant metastases are very infrequent, AFS is a locally aggressive lesion, with 23 (37%) of the reported cases having at least one recurrence; 12 patients (19.3%) died of the disease. Wide surgical excision with long-term follow-up remains the treatment of choice for this neoplasm.
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PMID:Ameloblastic fibrosarcoma of the mandible: report of two cases and review of the literature. 1133 69

Ameloblastic fibrosarcoma is a rare malignant odontogenic tumour characterized by a benign epithelial component within a malignant fibrous stroma. Its behaviour is relatively benign, with absence of metastatic disease, and the prognosis is reported to be good. It is a paradoxical neoplasm with "sarcomatous" morphological and immunohistochemical patterns but with a favourable clinical course. We report a new case of this tumour in a mandibular ramus of a 31-years-old male patient, that was surgically excised and treated with adjuvant chemotherapy and radiotherapy. Five years later the patient is free of disease. The growth potential of ameloblastic fibrosarcoma is evaluated and compared with a related lesion, the ameloblastic fibroma. The sarcomatous mesenchymal component of ameloblastic fibrosarcoma is positive to Ki67, PCNA and p53, in front of the negativity of ameloblastic fibroma.
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PMID:Ameloblastic fibrosarcoma: report of a case. Immunohistochemical study and review of the literature. 1150 Jun 34

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumour regarded as the malignant counterpart of ameloblastic fibroma. It is characterized by a benign epithelial component within a malignant fibrous stroma. AFS is a locally aggressive neoplasm with extremely low potential for metastasis. We report an extremely rare, rapidly progressive, and fatal case originating in the posterior mandible of a 20-year old female patient. Initially histopathologically diagnosed as a benign lesion, it rapidly recurred with apparent transformation into a high-grade sarcoma over a period of 6 months. Subsequent intracranial and pulmonary metastases were noted, and the patient died within 15 months of initial consultation. This case emphasizes the need for a high element of suspicion about clinically ambiguous lesions. We recommend more extensive or radical, primary excisions in lesions that have a known potential for recurrence or malignancy.
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PMID:Rapid sarcomatous transformation of an ameloblastic fibroma of the mandible: case report and literature review. 1971 96