Skip to main content
Erschienen in: Journal of Medical Case Reports 1/2012

Open Access 01.12.2012 | Case report

Nonossifying fibroma presenting as an aneurysmal bone cyst: a case report

verfasst von: Akio Sakamoto, Takeaki Ishii, Yoshinao Oda, Yukihide Iwamoto

Erschienen in: Journal of Medical Case Reports | Ausgabe 1/2012

download
DOWNLOAD
print
DRUCKEN
insite
SUCHEN

Abstract

Introduction

Nonossifying fibroma is a common fibrous bone lesion in children that occurs in the metaphysis of the long bones of the lower extremities. The lesion rarely leads to aneurysmal bone cyst, which is characterized as a blood-filled space.

Case presentation

We present the case of a 13-year-old Japanese boy with a complaint of discomfort in the thigh and a small, well-defined, osteolytic lesion with cortical thinning located in the medullary space of the distal diaphysis of the femur. At 10-month follow-up, the size of the lesion had increased. Gadolinium-enhanced magnetic resonance imaging failed to detect any solid area. Curettage and bone graft were performed and confirmed a blood-filled cystic lesion. The pathological diagnosis of the cyst wall was that of nonossifying fibroma, suggesting aneurysmal bone cyst as a secondary change. An aneurysmal bone cyst is rarely found secondary to nonossifying fibroma, and the diaphyseal location is atypical for nonossifying fibroma, both of which made diagnosis challenging.

Conclusion

The current case is a reminder to clinicians that, although rare, nonossifying fibroma can be associated with aneurysmal bone cyst, and both can occur in the diaphysis of long bones.
Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1752-1947-6-407) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

AS drafted the manuscript. AS and TI treated the patient. YO and YI participated in the design of the study. All authors read and approved the final manuscript.
Abkürzungen
ABC
Aneurysmal bone cyst
NOF
Nonossifying fibroma.

Introduction

Nonossifying fibroma (NOF) is a common type of benign fibrous lesion that occurs in the metaphysis of the long bones of the lower extremities[1, 2]. On plain radiographs, NOF appears as a small, cortically based osteolytic lesion with a thin sclerotic rim. Histologically, NOF is composed of spindle-shaped fibroblasts, multinucleated giant cells, and foamy histiocytes[2]. NOF is typically asymptomatic and the lesion is found incidentally. NOF is considered a developmental bone defect, not a neoplastic lesion, because it is usually self-limiting[13].
In contrast to NOF, aneurysmal bone cyst (ABC) is a locally destructive bone lesion[4]. Primary ABC typically occurs during the first two decades of life, and most frequently in the metaphyses of long bones, and less frequently in the spine or flat bones[4]. On plain radiographs, ABC appears as an eccentric and expansile lytic lesion[4]. ABC is considered a reactive process secondary to various precursor conditions, including benign and malignant bone neoplasms[2, 5].
We report a rare case of NOF associated with ABC that occurred at the diaphysis of the femur.

Case presentation

A 13-year-old Japanese boy with discomfort in his right thigh was evaluated at an orthopedic hospital where an abnormality in the distal diaphysis of the femur was noticed on plain radiographs, and the patient was referred to our institute. The plain radiographs showed a well-defined osteolytic lesion with two-cm longitudinal diameter. The adjacent cortex was slightly expanded and thinned (Figure1A). Magnetic resonance imaging (MRI) demonstrated the intramedullary lesion with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. Internal fibrous septa were seen as low signal intensity on both T1- and T2-weighted images (Figure2A and2B). There were no signal abnormalities in the intramedullary bone surrounding the lesion, and no extraosseous extension or adjacent soft-tissue abnormality was recognized. Because the features of MRI ruled out aggressive neoplasm or infection, the diagnosis was a benign lesion, and follow-up was recommended.
On follow-up 10 months later, the size of the lesion on plain radiograph had increased to 5cm on longitudinal diameter and MRI confirmed the enlargement (Figures1B and2C). MRI with gadolinium contrast enhanced only the periphery of the lesion, a result that suggested the lesion was cystic (Figure2C). Curettage and artificial bone graft were performed. The gross operative finding was of a cystic lesion containing blood and no solid material. The histology of the curetted tissue from the cyst wall was composed of fibrohistiocytic spindle cells arranged in vague fascicles, accompanied by occasional multinucleated giant cells and foamy histiocytes. The histological diagnosis was that of NOF (Figures3A and3B). The final diagnosis based upon the histology, image results, and clinical appearance was ABC change secondary to NOF.

Discussion

The pathogenesis of ABC is presumably a local circulatory abnormality that causes increased venous pressure and results in dilation of the vascular network[2, 5]. Primary ABC has a different genetic pathogenesis from secondary ABC, in spite of their morphological similarities, because primary ABC has been reported to have rearrangements of CDH11 or USP6 that are not seen in secondary ABC[6].
Typically, the diagnosis of NOF can be easily made based on images and clinical findings[3, 7]. The natural course of NOF is self-limiting involution and, thus, NOF has come to be known as a ‘don’t touch’ lesion because more aggressive diagnostics or treatment are unnecessary[1]. It has been reported that NOF can be found in approximately 30% of young individuals within the first and second decade of life[2]. ABC change secondary to NOF appears to be extremely rare, although the frequency is difficult to assess. Of interest, this case of NOF was located at the femoral diaphysis, but the typical location of NOF is the metaphyseal region. According to Brenner and colleagues[8], NOF can be divided into three phases on the basis of the intensity of uptake on bone scan: active, healing, and inactive. The low uptake, combined with the diaphyseal location, may suggest the current case was a long standing lesion in the inactive phase. The diaphyseal location made it difficult to arrive at the correct diagnosis of NOF based on the imaging results.

Conclusion

In conclusion, this report presents a rare case of NOF associated with ABC. In addition, the diaphyseal location was atypical for NOF. We present this case report as a reminder to clinicians to remain aware that, although rare, NOF can be associated with ABC, and both can occur in the diaphyses of long bones.
Written informed consent was obtained from the patient’s legal guardian for publication of this manuscript and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://​creativecommons.​org/​licenses/​by/​2.​0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

AS drafted the manuscript. AS and TI treated the patient. YO and YI participated in the design of the study. All authors read and approved the final manuscript.

Unsere Produktempfehlungen

e.Med Interdisziplinär

Kombi-Abonnement

Für Ihren Erfolg in Klinik und Praxis - Die beste Hilfe in Ihrem Arbeitsalltag

Mit e.Med Interdisziplinär erhalten Sie Zugang zu allen CME-Fortbildungen und Fachzeitschriften auf SpringerMedizin.de.

© Springer Medizin

Bis 11. April 2024 bestellen und im ersten Jahr 50 % sparen!

e.Med Allgemeinmedizin

Kombi-Abonnement

Mit e.Med Allgemeinmedizin erhalten Sie Zugang zu allen CME-Fortbildungen und Premium-Inhalten der allgemeinmedizinischen Zeitschriften, inklusive einer gedruckten Allgemeinmedizin-Zeitschrift Ihrer Wahl.

© Springer Medizin

Bis 11. April 2024 bestellen und im ersten Jahr 50 % sparen!

Anhänge

Authors’ original submitted files for images

Literatur
1.
Zurück zum Zitat Hetts SW, Hilchey SD, Wilson R, Franc B: Case 110: Nonossifying fibroma. Radiology. 2007, 243: 288-292. 10.1148/radiol.2431040427.CrossRefPubMed Hetts SW, Hilchey SD, Wilson R, Franc B: Case 110: Nonossifying fibroma. Radiology. 2007, 243: 288-292. 10.1148/radiol.2431040427.CrossRefPubMed
2.
Zurück zum Zitat Dorfman HD, Czerniak B: Bone tumors. 1998, St Louis: Mosby Dorfman HD, Czerniak B: Bone tumors. 1998, St Louis: Mosby
3.
Zurück zum Zitat Betsy M, Kupersmith LM, Springfield DS: Metaphyseal fibrous defects. J Am Acad Orthop Surg. 2004, 12: 89-95.CrossRefPubMed Betsy M, Kupersmith LM, Springfield DS: Metaphyseal fibrous defects. J Am Acad Orthop Surg. 2004, 12: 89-95.CrossRefPubMed
4.
Zurück zum Zitat Vergel De Dios AM, Bond JR, Shives TC, McLeod RA, Unni KK: Aneurysmal bone cyst. A clinicopathologic study of 238 cases. Cancer. 1992, 69: 2921-2931. 10.1002/1097-0142(19920615)69:12<2921::AID-CNCR2820691210>3.0.CO;2-E.CrossRefPubMed Vergel De Dios AM, Bond JR, Shives TC, McLeod RA, Unni KK: Aneurysmal bone cyst. A clinicopathologic study of 238 cases. Cancer. 1992, 69: 2921-2931. 10.1002/1097-0142(19920615)69:12<2921::AID-CNCR2820691210>3.0.CO;2-E.CrossRefPubMed
5.
Zurück zum Zitat Kransdorf MJ, Sweet DE: Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am J Roentgenol. 1995, 164: 573-580.CrossRefPubMed Kransdorf MJ, Sweet DE: Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am J Roentgenol. 1995, 164: 573-580.CrossRefPubMed
6.
Zurück zum Zitat Oliveira AM, Perez-Atayde AR, Inwards CY, Medeiros F, Derr V, Hsi BL, Gebhardt MC, Rosenberg AE, Fletcher JA: USP6 and CDH11 oncogenes identify the neoplastic cell in primary aneurysmal bone cysts and are absent in so-called secondary aneurysmal bone cysts. Am J Pathol. 2004, 165: 1773-1780. 10.1016/S0002-9440(10)63432-3.CrossRefPubMedPubMedCentral Oliveira AM, Perez-Atayde AR, Inwards CY, Medeiros F, Derr V, Hsi BL, Gebhardt MC, Rosenberg AE, Fletcher JA: USP6 and CDH11 oncogenes identify the neoplastic cell in primary aneurysmal bone cysts and are absent in so-called secondary aneurysmal bone cysts. Am J Pathol. 2004, 165: 1773-1780. 10.1016/S0002-9440(10)63432-3.CrossRefPubMedPubMedCentral
7.
Zurück zum Zitat Hod N, Levi Y, Fire G, Cohen I, Ayash D, Somekh M, Horne T: Scintigraphic characteristics of non-ossifying fibroma in military recruits undergoing bone scintigraphy for suspected stress fractures and lower limb pains. Nucl Med Commun. 2007, 28: 25-33. 10.1097/MNM.0b013e328012e3de.CrossRefPubMed Hod N, Levi Y, Fire G, Cohen I, Ayash D, Somekh M, Horne T: Scintigraphic characteristics of non-ossifying fibroma in military recruits undergoing bone scintigraphy for suspected stress fractures and lower limb pains. Nucl Med Commun. 2007, 28: 25-33. 10.1097/MNM.0b013e328012e3de.CrossRefPubMed
8.
Zurück zum Zitat Brenner RJ, Hattner RS, Lillien DL: Scintigraphic features of nonosteogenic fibroma. Radiology. 1979, 131: 727-730.CrossRefPubMed Brenner RJ, Hattner RS, Lillien DL: Scintigraphic features of nonosteogenic fibroma. Radiology. 1979, 131: 727-730.CrossRefPubMed
Metadaten
Titel
Nonossifying fibroma presenting as an aneurysmal bone cyst: a case report
verfasst von
Akio Sakamoto
Takeaki Ishii
Yoshinao Oda
Yukihide Iwamoto
Publikationsdatum
01.12.2012
Verlag
BioMed Central
Erschienen in
Journal of Medical Case Reports / Ausgabe 1/2012
Elektronische ISSN: 1752-1947
DOI
https://doi.org/10.1186/1752-1947-6-407

Weitere Artikel der Ausgabe 1/2012

Journal of Medical Case Reports 1/2012 Zur Ausgabe