دورية أكاديمية
Sarcomatous transformation in the McCune-Albright syndrome.
| العنوان: | Sarcomatous transformation in the McCune-Albright syndrome. |
|---|---|
| المؤلفون: | de Araújo PI; Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital of Brasília, Ambulatório II, SGAN, Via L2 Norte, Quadra 604/605, Asa Norte, CEP 70840-050, Brasilia, Federal District, Brazil., Soares VY, Queiroz AL, dos Santos AM, Nascimento LA |
| المصدر: | Oral and maxillofacial surgery [Oral Maxillofac Surg] 2012 Jun; Vol. 16 (2), pp. 217-20. Date of Electronic Publication: 2011 Sep 03. |
| نوع المنشور: | Case Reports; Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Springer-Verlag Country of Publication: Germany NLM ID: 101319632 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1865-1569 (Electronic) Linking ISSN: 18651550 NLM ISO Abbreviation: Oral Maxillofac Surg Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Berlin : Springer-Verlag |
| مواضيع طبية MeSH: | Cell Transformation, Neoplastic/*pathology , Fibrous Dysplasia, Polyostotic/*pathology , Mandibular Neoplasms/*pathology , Osteosarcoma/*pathology, Adult ; Biopsy ; Chemotherapy, Adjuvant ; Combined Modality Therapy ; Female ; Fibrous Dysplasia, Polyostotic/diagnostic imaging ; Fibrous Dysplasia, Polyostotic/drug therapy ; Fibrous Dysplasia, Polyostotic/surgery ; Humans ; Mandible/diagnostic imaging ; Mandible/pathology ; Mandible/surgery ; Mandibular Neoplasms/diagnostic imaging ; Mandibular Neoplasms/drug therapy ; Mandibular Neoplasms/surgery ; Mouth Floor/diagnostic imaging ; Mouth Floor/pathology ; Osteosarcoma/diagnostic imaging ; Osteosarcoma/drug therapy ; Osteosarcoma/surgery ; Tomography, X-Ray Computed |
| مستخلص: | Background: McCune-Albright syndrome is a relatively rare disease characterized by the trio of fibrous dysplasia, café-au-lait pigmentation, and endocrine disturbances. It predominantly affects females and may be associated with sarcomatous degeneration in 0.4% to 4% of the cases. Case Report: This article reports on the case of a 24-year-old female patient who had presented a mass in the oral cavity for 30 days, probably originating from the left ramus of the mandible. She had a previous diagnosis of polyostotic fibrous dysplasia, along with café-au-lait spots and endocrine disorders, thus characterizing McCune-Albright syndrome. Histopathological examination of a biopsy specimen revealed osteosarcoma of the mandible. The patient underwent chemotherapy and a surgical procedure for lesion resection. One year has now passed since the patient's operation, and the disease is under control. Discussion: The patient presented sarcomatous degeneration in areas of fibrous dysplasia, consisting of mesenchymal cells that produced osteoid. Malignant degeneration is rare when it is not associated with McCune-Albright syndrome. There is no curative treatment for the syndrome. Attending physicians need to bring endocrine disorders under control, with surgical treatment in cases of significant deformities, as well as providing clinical and psychological care. |
| References: | Orphanet J Rare Dis. 2008 May 19;3:12. (PMID: 18489744) Am J Med Sci. 1964 Jan;247:1-20. (PMID: 14106874) Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008 Jul;106(1):e49-55. (PMID: 18585612) J Child Orthop. 2007 Mar;1(1):3-17. (PMID: 19308500) J Craniofac Surg. 2010 Mar;21(2):601-3. (PMID: 20489463) Int J Clin Oncol. 2006 Jun;11(3):229-35. (PMID: 16850130) Pediatr Endocrinol Rev. 2007 Aug;4 Suppl 4:412-8. (PMID: 17982388) Arch Orthop Trauma Surg. 2009 Apr;129(4):439-44. (PMID: 18560851) Int J Oral Maxillofac Surg. 1997 Jun;26(3):198-204. (PMID: 9180230) |
| تواريخ الأحداث: | Date Created: 20110906 Date Completed: 20130122 Latest Revision: 20211020 |
| رمز التحديث: | 20221213 |
| DOI: | 10.1007/s10006-011-0286-5 |
| PMID: | 21892759 |
| قاعدة البيانات: | MEDLINE |
Find this article in full text from Springer Verlag. 
Full Text Finder | تدمد: | 1865-1569 |
|---|---|
| DOI: | 10.1007/s10006-011-0286-5 |