Subscribe to RSS
Download PDF
DOI: 10.1055/s-0038-1630494
Fibröse Knochenerkrankungen im Kindes- und Jugendalter – Diagnostik und therapeutische Strategien
Fibrous diseases of the bones in children and adolescents – diagnostic and therapeutic strategiesPublication History
Eingegangen:
29 May 2008
angenommen:
13 June 2008
Publication Date:
25 January 2018 (online)
Zusammenfassung
Das Skelettsystem des Menschen geht aus dem mesenchymalen Gewebe hervor und besteht hauptsächlich aus Knochen-und Knorpelgewebe. Zwischen den Knochenbälkchen und den Haver’schen Kanälchen findet sich Bindegewebe, aus dem tumoröse Veränderungen entstehen können. Die fibrösen Knochenerkrankungen sind gekennzeichnet durch ein fibröses Grundgerüst, das Fibroblasten enthält. Eine Vielzahl von fibrösen Knochenveränderungen wurde beschrieben. Die fibröse Dysplasie und das nicht ossifizierende Knochenfibrom als benigne Knochenläsionen stellen eine häufige Fehlentwicklung dar, die Kinder und Jugendliche befällt. Im Laufe des Wachstums nehmen die Herde an Größe und Anzahl zu. Es können sich Schmerzen, Frakturen und Deformitäten entwickeln, die eine lokale oder systemische Therapie erforderlich machen. Maligne fibröse Knochenerkrankungen sind ein seltenes Krankheitsbild und werden meist im jungen Erwachsenenalter diagnostiziert. Kinder und Jugendliche sind nur selten betroffen. Die Erkrankungen bedürfen eines interdisziplinären Behandlungsansatzes, da die Diagnostik und die Therapie eine ärztliche Herausforderung bedeuten.
Summary
The human skeletal system emanates from mesenchymal tissue and consists mainly of bone and cartilage tissue. The connective tissue to be found between bone trabeculae and Haversian canals can be susceptible to tumorous change. These fibrous bone diseases are characterized by a fibrous matrix containing fibroblasts. A multiplicity of fibrous bone degenerations has been described. Fibrous dysplasia and non-ossifying bone fibroma are two frequent types of benign bone lesion especially children and adolescents are afflicted with. In the course of adolescence, size and number of these lesions increase. Pain, fractures, and deformities can develop, requiring local or systemic therapy. Malign fibrous bone diseases are a clinical picture seldom seen, mostly found in early adult age, almost never in children or adolescents. These diseases call for an interdisciplinary treatment concept, as diagnostics and therapy pose a challenge to any physician.
-
Literatur
- 1 Ackerman LV, Spjut HJ. Tumors of bone and cartilage, Fascicle 4. Washington/DC: Armed Forces Institute of Pathology; 1962
- 2 Albright F, Butler AM, Hampton AO, Smith P. Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation, and endocrine dysfunction with precocious puberty in females: report of 5 cases. N Engl J Med 1937; 216: 727-746.
- 3 Adler CP. Knochenkrankheiten. Berlin, Heidelberg, New York: Springer; 1997
- 4 Bianco PM, Riminucci A, Majolagbe SA. et al. Mutation of the GNAS 1 gene, stromal cell dysfunction, and osteomalacic changes in non-McCune-Albright fibrous dysplasia of bone. J Bone Miner Res 2000; 15: 120-128.
- 5 Bianco P, Robey PG, Wientroub S. Fibous dysplasia. In: Juppner H. (ed). Pediatric bone: biology and disease. Academic. New York: Press/Elsevier; 2003: 509-539.
- 6 Bertoni F, Bacchini P, Staals E. Malignancy in giant cell tumors. Skeletal Radiol 2003; 32: 143-146.
- 7 Brandenberger G, Gronfier C, Chapotot F. et al. Effect of sleep deprivation on overall 24 h growthhormone secretion. Lancet 2000; 356: 1408.
- 8 Campanacci M. Osteofibrous dysplasia of long bones. A new clinical entity. Ital J Orthop Traumatol 1976; 2: 24-36.
- 9 Campanacci M. Bone and soft tissue tumors. Wien New York: Springer; 1990
- 10 Cecchetto G, Carli M, Alaggio R. et al. Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group studies (1979–1995). J Surg Oncol 2001; 78: 225-231.
- 11 Collins MT, Bianco P. Fibrous dysplasia. In: Favus MJ. (ed). Primer on the metabolic bone diseases and disorders of mineral metabolism. Washigton/DC: American Society for Bone and Mineral Research; 2003: 466-470.
- 12 Dorfman HD, Czerniak B. Fibroosseous lesions. In: Czerniak B. (ed). Bone tumors. St.Louis: Mosby; 1998: 441-491.
- 13 Enneking WF, Spanier SS, Googman MA. A system for the surgical staging of musculoskeletal sarcomas. Clin Orthop 1980; 153: 106-120.
- 14 Freyschmidt J, Ostertag H. Knochentumoren. Berlin, Heidelberg, New York: Springer; 1998
- 15 Ghert M, Rizzo M, Harrelson J, Scully S. Giantcell tumor of the appendicular skeleton. Clin Orthop 2002; 400: 201-210.
- 16 Hart ES, Bostan EE, Borman P. et al. Onset, progression, and plateau of skeletal lesions in fibrousdysplasia, and the relationship to functional outcome. J Bone Miner Res 2007; 18: 1235-1246.
- 17 Hefti F. Kinderorthopädie in der Praxis. Berlin, Heidelberg, New York: Springer; 2006
- 18 Ippolito E, Caterini R, Farsetti P, Potenza V. Surgical treatment of fibrous dysplasia of bone in McCune-Albright syndrome. J Pediatr Endocrinol Metab 2003; 155 (Suppl. 03) 939-944.
- 19 Kransdorf MJ, Sweet DE, Buetow PC. et al. Giant cell tumor in skeletally immature patients. Radiology 1992; 184: 233-237.
- 20 Leet AL, Collins MT. Current approach to fibrous dysplasia of bone and McCune-Albright syndrome. J Child Orthop 2007; 1: 3-17.
- 21 Lichtenstein L. Bone tumors, 4th ed. Saint Louis: Mosby; 1972
- 22 Lin P, Guzel V, Moura M. et al. Long-term followup of patients with giant cell tumor of the sacrum treated with selective arterial embolization. Cancer 2002; 95: 1317-1325.
- 23 Lodwick GS, Wilson AJ. Determining growth rates of focal lesions of the bone from radiographs. Radiology 1980; 134: 577-583.
- 24 McCune DJ. Osteitis fibrosa cystica: the case of a nine-year-old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism. Am J Dis Child 1936; 52: 743-744.
- 25 Niethard FU, Pfeil J. Orthopädie. Stuttgart: Thieme; 2003
- 26 Ozaki T, Lindner N, Blasius S. Dedifferentiated chondrosarcoma in Albright syndrome. A case report and review of the literature. J Bone Joint Surg Am 1977; 79: 1545-1551.
- 27 Plottkin H, Rauch F, Zeitlin L. et al. Effect of pamidronate treatment in children with polyostic fibrous dysplasia of the bone. J Clin Endocrinol Metab 2003; 88: 4569-4575.
- 28 Pollandt K, Engels C, Werner M, Delling G. Fibrous Dysplasia. Pathologe 2002; 23: 351-356.
- 29 Rödl R, Götze C. Fibröse Dysplasie. Orthopäde 2008; 37: 49-55.
- 30 Schajowicz F. Histological typing of bone tumors. Edited by WHO. International histological classification of tumors. Berlin, Heidelberg, New York: Springer; 1993
- 31 Schajowicz F. Tumor and tumorlike lesions of bone. Pathology, radiology, and treatment. Berlin, Heidelberg, New York: Springer; 1993
- 32 Schutte HE, Taconis WK. Giant cell tumor in children and adolescent. Skelet Radiol 1993; 22: 173-176.
- 33 Weinstein RS. Long-term aminobisphosphonate treatment of fibrous dysplasia: spectacular increase in bone density. J Bone Miner Res 1997; 12: 1314-1315.