Review on Bone: Osteoblastoma, with data on clinics, and the genes involved

de, Andrea CE

Hogendoorn, PCW

Atlas of Genetics and Cytogenetics in Oncology and Haematology

English

I-Revues

   
 
 
 
 
 
Solid Tumour Section 
Mini Review 
 
Atlas Genet Cytogenet Oncol Haematol. 2010; 14(1)  75 
Atlas of Genetics and Cytogenetics 
in Oncology and Haematology 
OPEN ACCESS JOURNAL AT INIST-CNRS 
Bone: Osteoblastoma 
Carlos Eduardo de Andrea, Pancras CW Hogendoorn 
Department of Pathology, Leiden University Medical Center, P1-30, Albinusdreef 2, 2333 ZA Leiden, The 
Netherlands (CEd, PCWH) 
 
Published in Atlas Database: February 2009 
Online updated version: http://AtlasGeneticsOncology.org/Tumors/OsteoblastomaID5343.html 
DOI: 10.4267/2042/44670 
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence. 
© 2010 Atlas of Genetics and Cytogenetics in Oncology and Haematology 
 
Identity 
Note 
Osteoblastoma (OB) is defined as a rare benign bone 
forming neoplasm which produces woven bone 
spicules, which are bordered by prominent osteoblasts 
(WHO, 2002).  
This tumour was first described in 1932 by Jaffe and 
Mayer in a case report of an osteoblastic osteoid tissue-
forming tumor of a metacarpal bone. It was been 
reported under various names, such as "osteogenic 
fibroma of bone" and "giant osteoid osteoma". 
Subsequently, in 1956, it was given its current name, 
when Jaffe and Lichtenstein independently proposed 
the term "benign osteoblastoma." 
Clinics and pathology 
Etiology 
The genetic cause of OB has not been established but 
there is a report of an unbalanced translocation 
resulting in the loss of the chromosome 17 short arm. 
MDM2 amplification was reported in one case and 
TP53 deletion at a splice region was demonstrated in an 
aggressive OB. Other reports described that recurrence 
was statistically correlated with p53 protein expression 
and high PCNA index. 
Epidemiology 
OB accounts for approximately 1% of all bone tumors. 
The tumor most commonly involves the vertebral 
column, particularly the posterior or dorsal elements, as 
well as the diaphyses of long bones, pelvis, talus, facial 
bones, and short bones of the hands and feet. 
Most of the patients affected by OB are in the second 
or third decades of life. 
Males are affected more commonly than females. 
 
Osteoblastoma. Well delineated, radiolucent/radiopaque lesion. 
 
Bone: Osteoblastoma de Andrea CE, Hogendoorn PCW 
 
 
 
 
 
Atlas Genet Cytogenet Oncol Haematol. 2010; 14(1)  76 
 
Osteoblastoma. Consisting of anatomosing woven bone trabeculae, lined by a single but prominent layer of osteoblasts, and intervening 
hypervascular, hypocellular intertrabecular spaces. 
 
Clinics 
Swelling, pain and tenderness are usually the general 
symptoms of OB. 
The clinical presentation of the spine tumours may 
include back pain, scoliosis and myelopathic and/or 
radicular symptoms, and suggest a herniated disc. 
Pathology 
OB is usually round to oval with a thinned cortex. If the 
cortex is destroyed always a thin periosteal reactive 
bone shell is found. Usually this tumour has an 
extremely rich vascular supply. 
Microscopically, OB is characterized by woven bone 
trabeculae and osteoid, lined by a prominent, single 
layer of osteoblasts, and an intervening hypocellular 
but highly vascular stroma. 
The osteoblasts are usually uniform and cytologically 
bland; however, about 10% of cases may be composed 
predominantly of "epithelioid" osteoblasts often 
proliferating in a multifocal pattern. 
The differentiation between OB and osteosarcoma (OS) 
is one of the most challenging problems in Orthopedic 
Pathology. OBs are often progressive tumours with a 
propensity for locally aggressive growth. 
Approximately 25% of patients have radiographic and 
histologic aspects that may suggest malignancy, such as  
 
epithelioid osteoblasts, bizarre cells, mitosis, and lace-  
or sheet-like osteoid production. The entity "aggressive 
osteoblastoma" was introduced by Dorfman and Weiss 
(1984) and Lucas et al.(1994) to describe lesions with 
features borderline between osteoblastoma and 
osteosar-coma. In OS cases, a permeative pattern of 
growth within intratrabecular spaces and a lack of 
matu-ration toward the periphery of the lesion have 
been described. The term "aggressive osteoblastoma" 
designates a tumor with local destructive behavior, but 
without metastatic potential. 
Treatment 
When possible, en bloc resection is the therapy of 
choice. 
Prognosis 
OB is a benign bone tumor with a recurrence potential 
as high as 22%. However, malignant transformation 
has been reported in rare cases and occurs in less than 
1% of cases. 
References 
Dorfman HD, Weiss SW. Borderline osteoblastic tumors: 
problems in the differential diagnosis of aggressive 
osteoblastoma and low-grade osteosarcoma. Semin Diagn 
Pathol. 1984 Aug;1(3):215-34 
Bone: Osteoblastoma de Andrea CE, Hogendoorn PCW 
 
 
 
 
 
Atlas Genet Cytogenet Oncol Haematol. 2010; 14(1)  77 
Bertoni F, Unni KK, McLeod RA, Dahlin DC. Osteosarcoma 
resembling osteoblastoma. Cancer. 1985 Jan 15;55(2):416-26 
Bertoni F, Bacchini P, Donati D, Martini A, Picci P, 
Campanacci M. Osteoblastoma-like osteosarcoma. The Rizzoli 
Institute experience. Mod Pathol. 1993 Nov;6(6):707-16 
Mascarello JT, Krous HF, Carpenter PM. Unbalanced 
translocation resulting in the loss of the chromosome 17 short 
arm in an osteoblastoma. Cancer Genet Cytogenet. 1993 
Aug;69(1):65-7 
Lucas DR, Unni KK, McLeod RA, O'Connor MI, Sim FH. 
Osteoblastoma: clinicopathologic study of 306 cases. Hum 
Pathol. 1994 Feb;25(2):117-34 
Radig K, Schneider-Stock R, Mittler U, Neumann HW, 
Roessner A. Genetic instability in osteoblastic tumors of the 
skeletal system. Pathol Res Pract. 1998;194(10):669-77 
Malcolm AJ, Schiller AL, Schneider-Stock R.. Osteoblastoma. 
In: Fletcher CDM, Unni KK, Mertens F, eds. World Health 
Organization Classification of Tumours. Pathology and 
Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC 
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Horvai A, Unni KK. Premalignant conditions of bone. J Orthop 
Sci. 2006 Jul;11(4):412-23 
Jones AC, Prihoda TJ, Kacher JE, Odingo NA, Freedman PD. 
Osteoblastoma of the maxilla and mandible: a report of 24 
cases, review of the literature, and discussion of its relationship 
to osteoid osteoma of the jaws. Oral Surg Oral Med Oral 
Pathol Oral Radiol Endod. 2006 Nov;102(5):639-50 
Rhode MG, Lucas DR, Krueger CH, Pu RT. Fine-needle 
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lymphangiomatosis. Diagn Cytopathol. 2006 Apr;34(4):295-7 
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Shimoda T, Kaya M, Matsumura T, Sonobe H, Kusumi T, 
Yamaguchi T, Hasegawa T. Utility of immunohistochemical 
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osteoblastoma and osteosarcoma. J Clin Pathol. 2007 
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Oliveira CR, Mendonça BB, Camargo OP, Pinto EM, 
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This article should be referenced as such: 
de Andrea CE, Hogendoorn PCW. Bone: Osteoblastoma. Atlas 
Genet Cytogenet Oncol Haematol. 2010; 14(1):75-77. 


Borderline osteoblastic tumors: problems in the differential diagnosis of aggressive osteoblastoma and low-grade osteosarcoma. Semin Diagn Pathol.

Osteoblastoma of the maxilla and mandible: a report of 24 cases, review of the literature, and discussion of its relationship to osteoid osteoma of the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod.

Bone: Osteoblastoma

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