Desmoplastic fibroma of the mandible - review of the literature and presentation of a rare case

Desmoplastic fibroma (DF) is a rare, benign but locally aggressive, intraosseous lesion with a high tendency of local recurrence. In this report the actual literature is reviewed regarding epidemiological data, pathology, clinical diagnostic criterias, therapy and prognosis. Moreover, a report of an interesting case is included localized in the mandibular corpus

Evaluation of a clinically applicable post-surgical classification system for primary retroperitoneal soft-tissue sarcoma.

Item does not contain fulltextBACKGROUND: The present AJCC/TNM staging system is of limited value for prediction of prognosis for patients with retroperitoneal sarcoma. The objective of the present study was to develop a postsurgical classification system that would enable comparison of outcomes for patients with primary retroperitoneal soft-tissue sarcoma. METHODS: Four classes were defined: I, low-grade/complete resection/no metastasis; II, high-grade/complete resection/no metastasis; III, any-grade/incomplete resection/no metastasis; and IV, any-grade/any resection/distant metastasis. The prognostic value of this classification system was analyzed in a population-based multicenter group(MCG) of patients with primary retroperitoneal soft-tissue sarcoma (n = 124) and in a cohort of patients treated in a single tertiary referral center (SCG; n = 107). RESULTS: Overall 5-year survival rates were 55% in the SCG and 43% in the MCG (P = 0.02). Class III (incomplete resection) was more frequent in the MCG than in the SCG (33% vs. 16%; P = 0.02). In the SCG, stage-specific 5-year survival rates were 89%, 40%, 26%, and 17% for classes I, II, III, and IV, respectively (P < 0.001), in comparison with 68%, 46%, 24%, and 0% in the MCG (P < 0.001). In a comparison of class-specific survival between the groups, only class I patients in the SCG had significantly better survival than class I patients in the MCG (P = 0.048). CONCLUSIONS: Classification based on grade, completeness of resection, and distant metastasis offers a reproducible prognostic tool that can be used to evaluate treatment strategies for primary retroperitoneal soft-tissue sarcoma. The probability of complete resection was significantly higher in the SCG than in the MCG. In patients with low-grade, completely resected sarcoma, there is a significant survival benefit with treatment in a high-volume tertiary center of excellence

Ameloblastic Carcinoma

Ameloblastic carcinoma (AC) is a rare aggressive malignant epithelial odontogenic tumor of the maxillofacial skeleton with a distinct predilection in the mandible. It may appear de novo or originate from a pre-existing ameloblastoma or odontogenic cyst. It exhibits cytological features of ameloblastoma and carcinoma. It may present as a cystic lesion with benign clinical features or as a large tissue mass with ulceration, significant bone resorption and tooth mobility. The clinical course of ameloblastic carcinoma is typically aggressive, with extensive local destruction. Direct extension of the tumour, lymph node involvement and metastasis to various sites has been reported. Wide local excision is the treatment of choice. Regional lymph node dissection should be considered and performed selectively. Radiotherapy and chemotherapy have limited role in the treatment of ameloblastic carcinomas. Close periodic reassessment of the patient is mandatory