Advertisement

Der Pathologe

, Volume 39, Issue 2, pp 132–138 | Cite as

Chondroblastom

  • G. Jundt
  • D. Baumhoer
Schwerpunkt: Knorpel, Knochen, Chorda – Molekulare Pathologie
  • 241 Downloads

Zusammenfassung

Chondroblastome sind sehr seltene, gutartige Knochentumoren, die fast ausschließlich Epi- und Apophysen meist der langen Röhrenknochen befallen. Sie treten überwiegend bei Kindern und Jugendlichen auf. Die Diagnose beruht auf der Kombination von Klinik, Röntgenbefund und Histologie. In Zweifelsfällen hilft der Nachweis der charakteristischen H3F3B-Punktmutation mittels Immunhistochemie oder Sequenzierung. Rezidive werden in 5–15 % beobachtet, selten (<1 %) kommen nach Rezidiven Lungenmetastasen vor, die offenbar auf einer intraoperativen Tumorverschleppung beruhen („metastatisches“ Chondroblastom), aber fast nie zum Tod des Patienten führen, wenn sie operativ entfernt werden. Histologisch „maligne“ Chondroblastome sind Einzelfälle, deren Genese bisher nicht verstanden ist. Als Therapie hat sich, auch in der Rezidivsituation, die lokale sorgfältige Kürettage bewährt.

Schlüsselwörter

Knochentumoren Chondroblastom Epiphyse Immunohistochemie Punktmutation 

Chondroblastoma

Abstract

Chondroblastomas are very rare benign primary bone tumors occurring preferentially in the epiphyses or apophyses of long bones in children and adolescents. In most cases the typical histological and imaging findings lead to a correct diagnosis that may be substantiated by demonstrating the highly specific point mutation in the H3F3B gene (p.K36M), either by sequencing or immunohistochemistry. Recurrences occur in 5–15% of cases, postsurgical metastatic deposits to the lungs are very rare (<1%). Histologically “malignant” chondroblastomas have been reported as single case reports. The treatment of choice is a thorough curettage, also in the case of local relapses.

Keywords

Bone neoplasms Chondroblastoma Epiphyses Immunohistochemistry Point mutation 

Notes

Danksagung

Die Autoren danken Frau Petra Huber, KTRZ Basel, für ihre Hilfe bei der Erstellung der Tabellen und Grafiken.

Förderung

G. Jundt und D. Baumhoer werden unterstützt durch die Stiftung Knochentumor-Referenzzentrum Basel. D. Baumhoer wird außerdem unterstützt durch die Gertrude von Meissner-Stiftung, die Gedächtnis-Stiftung Susy Rückert zur Krebsbekämpfung sowie die Stiftung für krebskranke Kinder, Regio Basiliensis.

Einhaltung ethischer Richtlinien

Interessenkonflikt

G. Jundt und D. Baumhoer geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

Literatur

  1. 1.
    Akpalo H, Lange C, Zustin J (2012) Discovered on gastrointestinal stromal tumour 1 (DOG1): a useful immunohistochemical marker for diagnosing chondroblastoma. Histopathology 60:1099–1106CrossRefPubMedGoogle Scholar
  2. 2.
    Amary F, Berisha F, Ye H et al (2017) H3F3A (Histone 3.3) G34W Immunohistochemistry: A Reliable Marker Defining Benign and Malignant Giant Cell Tumor of Bone. Am J Surg Pathol, 10.1097/pas.0000000000000859. May 12Google Scholar
  3. 3.
    Amary MF, Berisha F, Mozela R et al (2016) The H3F3 K36M mutant antibody is a sensitive and specific marker for the diagnosis of chondroblastoma. Histopathology 69:121–127CrossRefPubMedGoogle Scholar
  4. 4.
    Behjati S, Tarpey PS, Presneau N et al (2013) Distinct H3F3A and H3F3B driver mutations define chondroblastoma and giant cell tumor of bone. Nat Genet 45:1479–1482CrossRefPubMedGoogle Scholar
  5. 5.
    Bousdras K, O’donnell P, Vujovic S et al (2007) Chondroblastomas but not chondromyxoid fibromas express cytokeratins: an unusual presentation of a chondroblastoma in the metaphyseal cortex of the tibia. Histopathology 51:414–416CrossRefPubMedGoogle Scholar
  6. 6.
    Cleven AH, Briaire-De Bruijn I, Szuhai K et al (2016) DOG1 expression in giant-cell-containing bone tumours. Histopathology 68:942–945CrossRefPubMedGoogle Scholar
  7. 7.
    Codman EA (2006) The Classic: Epiphyseal chondromatous giant cell tumors of the upper end of the humerus. Surg Gynecol Obstet.1931;52:543. Clin Orthop Relat Res 450:12–16CrossRefPubMedGoogle Scholar
  8. 8.
    Coleman SS (1966) Benign chondroblastoma with recurrent soft-tissue and intra-articular lesions. J Bone Joint Surg Am 48:1554–1560DecCrossRefPubMedGoogle Scholar
  9. 9.
    De Silva MV, Reid R (2003) Chondroblastoma: varied histologic appearance, potential diagnostic pitfalls, and clinicopathologic features associated with local recurrence. Ann Diagn Pathol 7:205–213AugCrossRefPubMedGoogle Scholar
  10. 10.
    Edel G, Ueda Y, Nakanishi J et al (1992) Chondroblastoma of Bone. Virchows Arch A Pathol Anat 421:355–366CrossRefGoogle Scholar
  11. 11.
    Elek EM, Grimer RJ, Mangham DC et al (1998) Malignant chondroblastoma of the os calcis. Sarcoma 2:45–48CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Erlemann R (2001) Benigne Knorpeltumoren. Radiologe 41:548–559CrossRefPubMedGoogle Scholar
  13. 13.
    Erlemann R (2009) Basisdiagnostik von Knochentumoren. Radiologe 49:355–370CrossRefPubMedGoogle Scholar
  14. 14.
    Farfalli GL (2017) What Happens to the Articular Surface After Curettage for Epiphyseal Chondroblastoma? A Report on Functional Results, Arthritis, and Arthroplasty. Clinical Orthopaedics and Related Research. Slullitel Pai Muscolo Dl Et Al 475:760–766. https://doi.org/10.1007/s11999-016-4715–5Google Scholar
  15. 15.
    Fletcher CDM, Bridge JA, Hogendoorn PWC et al (Hrsg) (2013) WHO Classification of Tumours of the Soft Tissues and Bone. IARC Press, LyonGoogle Scholar
  16. 16.
    Huang L, Cheng YY, Chow LTC et al (2003) Receptor activator of NF-κB ligand (RANKL) is expressed in chondroblastoma: possible involvement in osteoclastic giant cell recruitment. Molecular. Pathology 56:116–120.  https://doi.org/10.1136/mp.56.2.116 Google Scholar
  17. 17.
    Huvos AG, Higinbotham NL, Marcove RC et al (1977) Aggressive chondroblastoma. Review of the literature on aggressive behavior and metastases with a report of one new case. Clinical orthopaedics and related research 126: 266–272Google Scholar
  18. 18.
    Jaffe HL, Lichtenstein L (1942) Benign Chondroblastoma of Bone: A Reinterpretation of the So-Called Calcifying or Chondromatous Giant Cell Tumor. The. Am J Pathol 18:969–991PubMedPubMedCentralGoogle Scholar
  19. 19.
    Jambhekar NA, Desai PB, Chitale DA et al (1998) Benign metastasizing chondroblastoma: a case report. Cancer 82:675–678FebCrossRefPubMedGoogle Scholar
  20. 20.
    Jundt G, Baumhoer D (2008) Chondrogene Tumoren des Skeletts. Der Pathologe 29(Suppl 2):223–231.  https://doi.org/10.1007/s00292-008-1025-z CrossRefPubMedGoogle Scholar
  21. 21.
    Kaim AH, Hugli R, Bonel HM et al (2002) Chondroblastoma and clear cell chondrosarcoma: radiological and MRI characteristics with histopathological correlation. Skeletal Radiol 31:88–95CrossRefPubMedGoogle Scholar
  22. 22.
    Kilpatrick SE, Romeo S (2013) Chondroblastoma. In: Fletcher CDM, Bridge JA, Hogendoorn PWC, Mertens F (Hrsg) WHO Classification of Tumours of the Soft Tissues and Bone. IARC Press, Lyon, S 262–263Google Scholar
  23. 23.
    Kirchhoff C, Buhmann S, Mussack T et al (2006) Aggressive scapular chondroblastoma with secondary metastasis—a case report and review of literature. Eur J Med Res 11:128–134MarPubMedGoogle Scholar
  24. 24.
    Kolodny A (1927) Bone Sarcoma. The Primary Malignant Tumors of Bone and the Giant-Cell Tumor. Surg Gynecol Obstet 44:1–214Google Scholar
  25. 25.
    Kurt A‑M, Unni KK, Sim FH et al (1989) Chondroblastoma of Bone. Hum Pathol 20:965–976CrossRefPubMedGoogle Scholar
  26. 26.
    Kyriakos M, Land VJ, Penning HL et al (1985) Metatstatic Chondroblastoma. Report of a fatal case with a review of the literatue on atypical, aggressive, and malignant chondroblastoma. Cancer 55:1770–1789CrossRefPubMedGoogle Scholar
  27. 27.
    Lehner B, Witte D, Weiss S (2011) Clinical and radiological long-term results after operative treatment of chondroblastoma. Arch Orthop Trauma Surg 131:45–52CrossRefPubMedGoogle Scholar
  28. 28.
    Mccarthy EF (1995) The Registry of Bone Sarcoma. A history. Iowa Orthop J 15:74–78PubMedPubMedCentralGoogle Scholar
  29. 29.
    Ostrowski ML, Johnson ME, Truong LD et al (1999) Malignant chondroblastoma presenting as a recurrent pelvic tumor with DNA aneuploidy and p53 mutation as supportive evidence of malignancy. Skeletal Radiol 28:644–650NovCrossRefPubMedGoogle Scholar
  30. 30.
    Posl M, Werner M, Amling M et al (1996) Malignant transformation of chondroblastoma. Histopathology 29:477–480NovCrossRefPubMedGoogle Scholar
  31. 31.
    Reyes CV, Kathuria S (1979) Recurrent and aggressive chondroblastoma of the pelvis with late malignant neoplastic changes. Am J Surg Pathol 3:449–455CrossRefPubMedGoogle Scholar
  32. 32.
    Romeo S, Hogendoorn PC, Dei Tos AP (2009) Benign cartilaginous tumors of bone: from morphology to somatic and germ-line genetics. Adv Anat Pathol 16:307–315CrossRefPubMedGoogle Scholar
  33. 33.
    Semmelink HJF, Pruszczynski M, Wiersma A et al (1990) Cytokeratin expression in chondroblastomas. Histopathology 16:257–263CrossRefPubMedGoogle Scholar
  34. 34.
    Sirsat MV, Doctor VM (1970) Benign chondroblastoma of bone. Report of a case of malignant transformation. The Journal of bone and joint surgery. British volume 52:741–745NovGoogle Scholar
  35. 35.
    Soder S, Oliveira AM, Inwards CY et al (2006) Type II collagen, but not aggrecan expression, distinguishes clear cell chondrosarcoma and chondroblastoma. Pathology 38:35–38CrossRefPubMedGoogle Scholar
  36. 36.
    Suneja R, Grimer RJ, Belthur M et al (2005) Chondroblastoma of bone: long-term results and functional outcome after intralesional curettage. J Bone Joint Surg Br 87:974–978.  https://doi.org/10.1302/0301-620x.87b7.16009 CrossRefPubMedGoogle Scholar
  37. 37.
    Xu H, Nugent D, Monforte HL et al (2015) Chondroblastoma of bone in the extremities: a multicenter retrospective study. J Bone Joint Surg Am 97:925–931CrossRefPubMedGoogle Scholar

Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2017

Authors and Affiliations

  1. 1.Knochentumor-Referenzzentrum und DÖSAK-Zentralregister am Institut für PathologieUniversitätsspital BaselBaselSchweiz

Personalised recommendations