Abstract
Camurati–Engelmann disease (CED) is a rare, progressive diaphyseal dysplasia characterized as diaphyseal hyperostosis and sclerosis of the long bones. Corticosteroids, bisphosphonates, and losartan have been reported to be effective systemic medications used to reduce CED symptoms. There are no reports of osteoblastoma in patients with CED, and osteoblastoma in the distal radius is rare. We present a patient diagnosed with CED, based on radiological and histological examinations, at 11 years old. At 22 years old, she experienced severe pain in her right forearm and was treated with bisphosphonate, losartan, and prednisolone; however, the pain continued. An expansive and sclerotic lesion at the distal radius was observed on radiography. A follow-up plain radiograph indicated that the lesion was growing. Fluorodeoxyglucose positron emission tomography revealed solitary, intense radiotracer uptake, and a biopsy and surgical resection were performed due to suspected malignancy. Pathologic analysis showed anastomosing bony trabeculae rimmed by osteoblasts observed in a loose fibrovascular stroma. The lesion was diagnosed as an osteoblastoma. Following bone excision and artificial bone grafting, the patient’s severe pain almost completely disappeared. At final follow-up, no evidence of osteoblastoma recurrence was noted. To our knowledge, this is the first case report of osteoblastoma arising in a patient with CED. Bone excision and artificial bone grafting may be a treatment option for local symptomatic osteoblastoma in patients with CED.
Similar content being viewed by others
Data Availability
Data are available from the corresponding author upon reasonable request.
References
Van Hul W, Boudin E, Vanhoenacker FM et al (2019) Camurati-Engelmann disease. Calcif Tissue Int 104:554–560. https://doi.org/10.1007/s00223-019-00532-1
Janssens K, Vanhoenacker F, Bonduelle M et al (2006) Camurati-Engelmann disease: review of the clinical, radiological, and molecular data of 24 families and implications for diagnosis and treatment. J Med Genet 43:1–11. https://doi.org/10.1136/jmg.2005.033522
Cockayne EA (1920) Case for Diagnosis. Proc R Soc Med 13:132–136
Camurati M (1922) Di uno raro caso di osteite simmetrica ereditaria degli arti inferiori. Chir Organi Mov 6:662–665
Engelmann G (1929) Ein fall von osteopathia hyperostotica (sclerotisans) multiplex infantilis. Fortschr Geb Roentgenstr Nukl 39:1101–1106
Nishimura G, Nishimura H, Tanaka Y et al (2002) Camurati-Engelmann disease type II: progressive diaphyseal dysplasia with striations of the bones. Am J Med Genet 107:5–11. https://doi.org/10.1002/ajmg.10079
Inaoka T, Shuke N, Sato J et al (2001) Scintigraphic evaluation of pamidronate and corticosteroid therapy in a patient with progressive diaphyseal dysplasia (Camurati-Engelmann disease). Clin Nucl Med 26:680–682
Minford AM, Hardy GJ, Forsythe WI et al (1981) Engelmann’s disease and the effect of corticosteroids: a case report. J Bone Joint Surg Br 63:597–600
Iba K, Takada J, Kamasaki H et al (2008) A significant improvement in lower limb pain after treatment with alendronate in two cases of Camurati-Engelmann disease. J Bone Miner Metab 26:107–109. https://doi.org/10.1007/s00774-007-0783-7
Castro GR, Appenzeller S, Marques-Neto JF et al (2005) Camurati-Engelmann disease: failure of response to bisphosphonates. Report of two cases. Clin Rheumatol 24:398–401. https://doi.org/10.1007/s10067-004-1056-7
Shaker JL (2009) Paget’s disease of bone: a review of epidemiology, pathophysiology and management. Ther Adv Musculoskelet Dis 1:107–125
Kinoshita A, Saito T, Tomita H et al (2000) Domain-specific mutations in TGFB1 result in Camurati-Engelmann disease. Nat Genet 26:19–20. https://doi.org/10.1038/79128
Carlson ML, Beatty CW, Neff BA et al (2010) Skull Base Manifestations of Camurati-Engelmann disease. Arch Otolaryngol Head Neck Surg 136:566–575. https://doi.org/10.1001/archoto.2010.68
Berry M, Mankin H, Gebhardt M et al (2008) Osteoblastoma: a 30-year study of 99 cases. J Surg Oncol 98:179–183. https://doi.org/10.1002/jso.21105
Kwee TC, de Klerk JMH, Nix M et al (2017) Benign bone conditions that may be FDG-avid and mimic malignancy. Semin Nucl Med 47:322–351. https://doi.org/10.1053/j.semnuclmed.2017.02.004
Nagasawa H, Okada K, Nanjo H et al (2010) Unusual association between enchondroma and Camurati-Engelmann disease: a case report. Ups J Med Sci 115:157–160. https://doi.org/10.3109/03009730903406777
Angelini A, Varela-Osorio AF, Trovarelli G et al (2017) Osteoblastoma of the elbow: analysis of 13 patients and literature review. Eur J Orthop Surg Traumatol 27:787–795. https://doi.org/10.1007/s00590-017-1997-6
Siris ES, Lyles KW, Singer FR et al (2006) Medical management of Paget’s disease of bone: indications for treatment and review of current therapies. J Bone Miner Res 21:94–98. https://doi.org/10.1359/jbmr.06s218
Ayyavoo A, Derraik JG, Cutfield WS et al (2014) Elimination of pain and improvement of exercise capacity in Camurati-Engelmann disease with losartan. J Clin Endocrinol Metab 99:3978–3982. https://doi.org/10.1210/jc.2014-2025
Higuchi T, Yamamoto N, Hayashi K et al (2018) Calcium phosphate cement in the surgical management of benign bone tumors. Anticancer Res 38:3031–3035
Acknowledgements
We thank Issei Nomura, MD, PhD (Department of Orthopaedic Surgery, Kanazawa Disabled Children’s Hospital), for preparation of medical record. We also thank Editage (www.editage.com) for English language editing and publication support.
Funding
The research did not receive any specific grant from any funding agency in the public, commercial, or non-profit sector.
Author information
Authors and Affiliations
Contributions
HY, KH, NY, AT, KT, SM, KI, HK, YA, SM, YA, YA, KS, HI, TN, and HT were involved in the diagnosis and clinical management of the patient. All authors revised the paper critically for intellectual content and approved the final version. All authors agree to be accountable for this work.
Corresponding author
Ethics declarations
Conflict of interest
Hirotaka Yonezawa, Katsuhiro Hayashi, Norio Yamamoto, Akihiko Takeuchi, Kaoru Tada, Shinji Miwa, Kentaro Igarashi, Hiroaki Kimura, Yu Aoki, Sei Morinaga, Yoshihiro Araki, Yohei Asano, Keisuke Sakurakichi, Hiroko Ikeda, Takayuki Nojima, and Hiroyuki Tsuchiya declare that they have no conflict of interest.
Consent for Publication
The participant has consented to the submission of the case report to the journal.
Ethical Approval
All procedures performed in this study were in accordance with the ethical standards of the institutional and national research committees and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Ethical approval was obtained from Medical Ethics Committee of Kanazawa University.
Informed Consent
Informed consent was obtained from the patient included in this study.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Yonezawa, H., Hayashi, K., Yamamoto, N. et al. Significant Improvement After Surgery for a Symptomatic Osteoblastoma in a Patient with Camurati–Engelmann Disease: Case Report and Literature Review. Calcif Tissue Int 108, 819–824 (2021). https://doi.org/10.1007/s00223-021-00813-8
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00223-021-00813-8