Osteofibrous Dysplasia (Kempson-Campanacci's Disease): Long Term Follow-up Study on Natural History, Results of Treatment and Relationship With Adamantinoma
Conditions: Osteofibrous Dysplasia
Sponsor: Istituto Ortopedico Rizzoli

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“Osteofibrous Dysplasia (Kempson-Campanacci’s disease): Long Term 
Follow-up Study on Natural History, Results of Treatment and 
Relationship with Adamantinoma” 
 
Study code DOFOL 
Sponsor’s Name and 
Address: 
Istituto Ortopedico Rizzoli 
Via di Barbiano 1/10 
40136 Bologna 
Italy 
Study 
Number/Version/Date: 
Vers 1.0  
08 Mar 2019 
Coordinating Center: IRCCS Istituto Ortopedico Rizzoli 
Pathology Unit 
Via Pupilli 1 
40136 Bologna, Italy 
Coordinating 
Investigator and 
address: 
Alberto Righi MD 
Phone: 051-6366.665 
Email: alberto.righi@ior.it 
Scientific/Medical study 
responsible and 
developer 
Alberto Righi MD PhD 
 
Methodology: Retrospective study 
(Single insitution case series review of clinical data) 
Type: Academic 
 
Founding: None 
 
Principal Investigator 
Signature 
I confirm that I’ve read this protocol and I accept to run the study 
in compliance with what is stated in the protocol and with the 
ICh-GCP and all applicable law 
 
Alberto Righi MD 
 
Firma 
 
_________________________ 
 

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TABLE of CONTENTS 
Background 
 
 
 
 
 
 
 
 
 
pg. 3 
Objective of the study 
 
 
 
 
 
 
 
pg.  3 
Study design   
 
 
 
 
 
 
 
 
pg  3 
Population     
 
 
 
 
 
 
 
 
pg.  3 
Material and Methods   
 
 
 
 
 
 
 
pg.  4 
Statistics 
 
   
 
 
 
 
 
 
 
pg.  4 
Enrolment procedure   
 
 
 
 
 
 
 
pg.  4 
Data Collection            
  
 
 
 
 
 
 
pg.  4 
Informed Consent   
 
  
 
 
 
 
 
 
pg.  5 
Ethic and quality assurance   
 
 
 
 
 
 
pg.  5 
Confidentiality                                                                                       
 
 
pg.  6 
Publication of results                                                        
 
 
             pg.  6 
Sponsor Role and Responsibility                                                                                   pg.  6 
References 
                                                                                       
 
 
pg.  7 
 
 

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BACKGROUND 
Osteofibrous dysplasia, also called Kempson-Campanacci disease, is a rare benign fibro-osseous lesion 
typically involving the tibia or the tibia and fibula, usually diagnosed during early childhood or pre-
adolescent age (1,2). Despite benign self-limiting course it may cause anterior bowing, bone expansion and 
moderate pain. Osteofibrous dysplasia is rare, with reported incidence of 0.2% of all primary bone tumors 
(1-3). It is slightly prevalent in males and is usually diagnosed within the first 2 decades of life, most 
commonly in children less than 12 years old (1-4). Since malignant progression to adamantinoma has been 
reported, management of this condition remains somewhat controversial (1-7). The potential role of 
osteofibrous dysplasia as precursor lesion capable of progression to adamantinoma has received support 
by the more recent description of osteofibrous dysplasia-like adamantinoma or differentiated 
adamantinoma, an intermediate lesion possibly representing the missing link between osteofibrous 
dysplasia and adamantinoma (5-7). However, consensus has not been reached on the histologic criteria 
differentiating osteofibrous dysplasia, osteofibrous dysplasia-like adamantinoma and adamantinoma and 
progression to adamantinoma has not been convincingly documented so far (1-7). 
 
  
OBJECTIVE OF THE STUDY  
The aim of the present study is to obtain long term follow-up in patients with osteofibrous dysplasia, to 
assess natural history of the disease, late results of treatment and in particular the potential and risk of 
progression to adamantinoma. 
The study will exam all the cases of osteofibrous dysplasia treated between 1943 and 2018.  
 
STUDY DESIGN 
This is single institution cases series review of histological and clinical data 
 
POPULATION 
Inclusion criteria 
1) Male and female patients treated at Rizzoli Institute from 01 Jan 1943 to 31 Dic 2011 
2) Diagnosis of osteofibrous dysplasia pathologically confirmed 
3) Written informed consent prior to any study-specific analysis and/or data collection  
According to the Italian “Autorizzazione generale n. 9/2016 al trattamento dei dati personali 
effettuato per scopi di ricerca scientifica” of the Privacy Tutor (and the corresponding regulation in 
the other participating countries) the informed consent is not required to be obtained by the 

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deceased subjects, as long as all the other enrolment criteria are met and the study has been 
approved by the Ethic Committee (refers to INFORMED CONSENT section). 
 
MATERIAL AND METHODS 
We will retrieve from the database of the Rizzoli institute all the cases with a histological diagnosis 
of Ewing sarcoma from 01 Jan 1943 to 31 Dic 2011.  
We aspect to find approximately 55 cases.  
We will review all the medical records, radiological imaging, and histological data of these cases.  
 
STATISTICS 
To the case series will be applied a descriptive statistic. 
 
ENROLLMENT PROCEDURE 
Patients considered eligible will be included in the study, after providing a written informed 
consent.  
Since we will include cases of several decades ago, it would be possible that some eligible subjects 
will be deceased. 
 
DATA COLLECTION 
Clinical data will be retrieved by patient charts. 
A protocol-specific CRF reporting the results of the review will be provided. 
A CRF is required and should be completed for each included subject. 
 
ETHICS AND QUALITY ASSURANCE 
The clinical trial protocol and its documents will be sent before initiating the study to the 
competent Authorities and Ethics Committees of each participating country for its approval. 
The responsible investigator will ensure that this study is conducted in agreement with either the 
most updated Declaration of Helsinki and all the international and local laws that apply to clinical 
trials and to patient protection.  
The protocol has been written, and the study will be conducted according to the principles of the 
ICH Harmonized Tripartite Guideline for Good Clinical Practice  
(ref: http://www.emea.eu.int/pdfs/human/ich/013595en.pdf).  

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INFORMED CONSENT 
All patients will be informed, by the investigator, of the aims of the study, the possible risks and 
benefits that will derive from the study participation. * 
The Investigator must clearly inform that the patient is free to refuse participation in the study and 
that can withdraw consent at any time and for any reason.  
They will be informed as to the strict confidentiality of their patient data, but that their medical 
records may be reviewed for trial purposes by authorized individuals other than their treating 
physician.  
The informed consent procedure must conform to the ICH guidelines on Good Clinical Practice. 
This implies that "the written informed consent form should be signed and personally dated by the 
patient or by the patient's legally acceptable representative".   
The Investigator must also sign the Informed Consent form, and will keep the original at the site 
and a copy of the original must be handed to the patient.  
The competent ethics committee for each Institution participating to the study must validate local 
informed consent documents before the study can be opened. It will be emphasized that the 
participation is voluntary and that the patient is allowed to refuse further participation in the 
study whenever he/she wants. This will not prejudice the patient's subsequent care.  
Due to the high incidence of mortality of the disease under investigation, it would be possible that 
some potential eligible subjects will be deceased.  
In order to allow and promote the increase in the knowledge of this rare disease that could be 
beneficial for other patient that are or will be affected, according to the Italian “Autorizzazione 
generale n. 9/2016 al trattamento dei dati personali effettuato per scopi di ricerca scientifica” of 
the Privacy Tutor and the corresponding regulation in the other participating country, as well as 
the EU General Data Protection Regulation 679/2016 (that will be applicable from 25 May 2018), 
the informed consent is not required to be obtained by the deceased subjects according the 
aforementioned laws/dispositions. 
 
CONFIDENTIALITY 
In order to ensure confidentiality of clinical trial data as disposed the national and European 
applicable regulation, data will be only accessible for the trial Sponsor and its designees, for 

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monitoring/auditing procedures, the Investigator and collaborators, the Ethics Committee of each 
corresponding site and the Health Authority. 
Investigator and the Institution will allow access to data and source documentation for 
monitoring, auditing, Ethic Committee revision and inspections of Health Authority, but 
maintaining at all times subject personal data confidentiality as specified in the “Directive 
95/46/EC of the European Parliament and of the Council of 24 October 1995”.  
 The Investigator must guarantee that patient anonymity is kept at all times and their identity 
must be protected from unauthorized persons and institutions.  
All patients included in the study will be identified with a numeric code, so that no identifiable 
personal data will be collected (pseudo anonymization) 
The Investigator must have and conserve a patients’ inclusion registry where it figures the 
personal data of the patient: name, surname, address and corresponding identification code into 
the study, this register will be kept on the Investigator File. 
 
PUBBLICATION OF RESULTS  
The results from this study will be published or shown at scientific conferences.  
The final publication of the study results will be written by the Principal Investigator. 
 
SPONSOR ROLE AND RESPONSIBILITY 
The sponsor is the sole owner of the data and is responsible of all the clinical trial activities from 
study design, development, data collection, management, analysis, interpretation of data, writing 
and the decision to submit the report for publication written by the Principal Investigator,  
 
 
 
 
 
 
 

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REFERENCES 
[1] Vigorita VJ, Hogendoorn PCW and Sawyer JR. Osteofibrous dysplasia. WHO Classification of 
Tumours of Soft Tissue and Bone, 4th edn. Lyon: IARC Press, 354-355, 2013.  
[2] Puchner SE, Varga R, Hobusch GM, Kasparek M, Panotopoulos J, Lang S, et al. Long-term 
outcome following treatment of Adamantinoma and Osteofibrous dysplasia of long bones. Orthop 
Traumatol Surg Res. 2016 Nov;102(7):925–32.  
[3] Campanacci M, Laus M. Osteofibrous dysplasia of the tibia and fibula. J Bone Joint Surg Am. 
1981 Mar;63(3):367-75.  
[4] Gleason BC, Liegl-Atzwanger B, Kozakewich HP, Connolly S, Gebhardt MC, Fletcher JA, et al. 
Osteofibrous dysplasia and adamantinoma in children and adolescents: a clinicopathologic 
reappraisal. Am J Surg Pathol. 2008 Mar;32(3):363–76.  
 [5] Czerniak B, Rojas-Corona RR, Dorfman HD. Morphologic diversity of long bone adamantinoma. 
The concept of differentiated (regressing) adamantinoma and its relationship to osteofibrous 
dysplasia. Cancer. 1989 Dec 1;64(11):2319–34.  
 [6] Scholfield DW, Sadozai Z, Ghali C, Sumathi V, Douis H, Gaston L, et al. Does osteofibrous 
dysplasia progress to adamantinoma and how should they be treated? Bone Joint J. 2017 Mar 
1;99–B(3):409–16. 
[7] Benassi MS, Campanacci L, Gamberi G, et al. Cytokeratin expression and distribution in 
adamantinoma of the long bones and osteofibrous dysplasia of the tibia and fibula. An 
immunoistochemical study correlated to histogenesis. Histopathology 1994; 25 :71-76.