Professional Documents
Culture Documents
Log in
Bookshelf
Search databaseBooksAll
DatabasesAssemblyBiocollectionsBioProjectBioSampleBooksClinVarConserved
DomainsdbGaPdbVarGeneGenomeGEO DataSetsGEO ProfilesGTRHomoloGeneIdentical Protein
GroupsMedGenMeSHNLM CatalogNucleotideOMIMPMCPopSetProteinProtein ClustersProtein Family
ModelsPubChem BioAssayPubChem CompoundPubChem
SubstancePubMedSNPSRAStructureTaxonomyToolKitToolKitAllToolKitBookgh
Search term
Search
Browse Titles
Advanced
Help
Disclaimer
StatPearls [Internet].
Show details
Search term
Aneurysmal Bone Cysts
Kyle J. Stevens; James A. Stevens.
Go to:
Introduction
Aneurysmal bone cysts are non-malignant, tumor-like, vascular lesions comprised of blood-
filled channels. Although they can occur in any bone, they are most common in the femur,
tibia, and vertebrae.[1]Their expansile nature may result in pain and inflammation, and
disruption of joints and growth plates. They can grow aggressively, be locally destructive,
and weaken bones to the point of pathologic fracture.
Go to:
Etiology
The etiology of aneurysmal bone cysts is currently unknown, although they appear to be due
to vascular malformations in the bone.
There are three main theories for their etiology:[2]
Aneurysmal bone cysts occur as a result of a separate primary bone tumor; this may
be due to the relatively high rate (1 in 3) of an accompanying bone lesion. These
lesions are frequently chondromyxoid, fibromas, chondrosarcomas, fibrous dysplasia,
giant cell tumors of the bone, osteoblastomas, osteosarcomas, among others.
They form as the primary tumor.
They form at a site of previous trauma.
A portion of aneurysmal bone cysts now appears to be neoplastic. Genetic studies of the
tumor have revealed that up to 69% of primary aneurysmal bone cysts contain a clonal
t(16,17) translocation. The t(16,17) fusion causes an upregulation of the TRE17/USP6
oncogene, which activates NF-kB and matrix metalloproteinases (MMPs). The MMPs break
down the extracellular matrix, allowing for the swift growth of the lesions.[3] Secondary
aneurysmal bone cysts have not been found to contain this translocation.
Go to:
Epidemiology
Aneurysmal bone cysts are a rare osseous tumor, comprising 1% to 6% of primary osseous
tumors. According to a retrospective study, the incidence of primary aneurysmal bone cysts
was 0.14 per 10 people. The majority of patients diagnosed with an aneurysmal bone cyst (8
out of 10) are children and adolescents less than 20 years old. There is a slightly increased
incidence rate seen in females (1 to 1.3).[4]Because aneurysmal bone cysts primarily
manifest in pediatric patients, growth plate involvement and permanent limb length
deformities are of great concern.
By far, the majority of aneurysmal bone cysts present in the metaphysis of long bones (67%).
They also occur in the spine (15%), particularly the posterior elements, pelvis (9%), and less
frequently, they can appear in the craniofacial bones and epiphyses.[5]
Go to:
Pathophysiology
Researchers believe aneurysmal bone cysts to be due to a vascular malformation, with the
definitive cause being unknown. The current thinking is that the vascular malformations lead
to increased pressure and expansion in the bone itself, causing erosion and resorption of the
involved bone.[2] The majority of primary aneurysmal bone cysts lesions possess a t(16,17)
gain-of-function translocation that upregulates the TRE17/USP6 oncogene, which halts
osteoblastic maturation.[6]
Go to:
Histopathology
Grossly, aneurysmal bone cysts are composed of blood and hemosiderin-filled cystic
cavities, enclosed in a subperiosteal shell of reactive bone. The cavities contain septa of
trabeculated bone or osteoid tissue, without endothelium. The stroma is comprised of
fibroblasts, spindle cells, osteoid, and multinucleated giant cells.[3]These cells are typically
seen at the periphery of the cystic cavities, giving an appearance described as “pigs at the
trough.” Mitotic figures commonly present in the lesions, but atypical figures should be
absent.
Go to:
Evaluation
The evaluation of aneurysmal bone cysts primarily consists of imaging studies, which can
typically provide critical clues to the diagnosis.
Radiographs illustrate cystic or osteolytic lesions with thin “eggshell” sclerotic borders. The
enclosed cavity contains many dividing septa.[2]The surrounding bone may be pushed
outwards from its normal anatomy, demonstrating the aggressive, expansile nature of the
lesion.
Computed tomography reveals similar characteristics as plain radiographs, although it may
define the cystic septa to a greater degree, highlighting the “eggshell” rim. Fluid-fluid levels
may be present within the cavities due to the separation of the cellular debris from the serum.
Magnetic resonance imaging again demonstrates similar findings as CT. T1 contrast-
enhanced and T2 weighted images can emphasize the septa within the lesion, revealing rims
of low T1 and T2 signal [2]. Variably aged blood contained within the cystic cavities are
visible on MR as focal areas of hyperintense signal on both T1 and T2 weighted sequences,
and double density fluid levels may also be visible. A pathologic fracture may be evident, as
demonstrated by osseous and soft tissue edema.
Laboratory studies hold a minimal benefit in the workup and diagnosis of aneurysmal bone
cysts, although bears mentioning that alkaline phosphatase levels may present as increased
due to the elevated activity of osteoblasts.
Go to:
Treatment / Management
Once diagnosed with an aneurysmal bone cyst, the patient should obtain a referral to an
orthopedic oncologist. Surgical intervention is typically the treatment of choice to prevent
pathological fracture. Based on the lesion size and the region of bone involved, either
intralesional curettage, intralesional excision, or en bloc (complete) excision may be an
option.
Intralesional curettage involves evacuating the cavity of its contents and filling the remaining
space with bone graft or cement to strengthen the bone.
Intralesional excision is the preferred treatment of choice, which is similar to curettage: the
surgeon makes a broad opening through the osseous wall of the lesion and removes the
contents. This process allows a greater amount of the bone to remain intact to reduce patient
morbidity when compared to en bloc excision. After the removal of the cystic contents, the
surgeon can fill the lesion via bone grafting or other material to supply strength and promote
healing of the bone. Differing from curettage, this type of treatment allows the use of various
forms of adjuvant therapy to reduce the rates of recurrence, due to the broad opening made to
gain access to the cavity. Adjuvant therapies include high-speed burr, argon beam
coagulation, phenol, and cryotherapy. This type of excision is also beneficial in aneurysmal
bone cysts that occur near joints and other structures where the desire to maintain normal
anatomy is crucial for functionality.
En bloc excision consists of removing the entire cavitary lesion from the bone that contains
it. When considering en bloc excision, the surgeon must weigh the risks and benefits of the
procedure, considering the possible loss of functionality of the area, especially when
operating near a joint. Due to the result of significant patient morbidity, en bloc resection is
typically reserved for patients with recurrent lesions that were not adequately controlled by
less invasive means.[3]
Selective arterial embolization (SAE) may be considered before surgery as an adjuvant, or as
a primary treatment if the clinician suspects a severe loss of function or destabilization as a
result of local or wide excision of the lesion. In up to 40% of patients being treated primarily
with SAE, a second or third embolization attempt is necessary.[7]
Radiotherapy has also found utility as adjuvant therapy in recurrent aneurysmal bone cysts
cases. However, clinicians must consider the significant risks associated with radiation
therapy. There is at least one documented instance of radiation-induced sarcoma of a patient
receiving treatment with external beam radiation for a vertebral aneurysmal bone cyst.[8]
Medical therapies with monoclonal antibodies for non-surgical candidates is an area
currently being explored.
Go to:
Differential Diagnosis
Chondroblastoma – These are cartilaginous tumors typically found in the epiphyses (95%)
and apophyses of long bones. Chondroblastomas are nonmalignant and usually discovered
when a child has constant pain and swelling in an extremity that is unrelated to activity.
Radiographically, they demonstrate sclerotic, well-circumscribed lesions located in the
epiphysis that may be crossing the growth plate. A central region of intermediate to high
signal on T2/STIR images are visible on MR, as well as central enhancement post-
gadolinium. Peritumoral edema is frequently associated, as evidenced by surrounding
hyperintense areas on T2 images.[9] Fluid-fluid levels may be present.
Fibrous dysplasia – Predominantly a process found in children and young adults, fibrous
dysplasia is a benign, tumor-like process in which healthy bone becomes replaced with
fibrous connective tissue and immature trabecular bone. These lesions are due to an error in
osteoblastic differentiation and maturation. Usually found incidentally, patients with fibrous
dysplasia are typically asymptomatic and require no intervention. However, morbidity can
arise when the lesion is expanding and pressing upon nearby structures. They can be
monostotic (involving only one bone) or polyostotic (involving multiple bones).
Radiographically, the lytic lesions show cortical thinning with endosteal scalloping and well-
circumscribed borders. A ground glass matrix is present, and, occasionally, a “rind sign” is
present (a thick layer of sclerotic reactive bone around the lesion).[10] CT and MRI are
usually not warranted for further evaluation unless pathologic fracture or stress reaction is
suspected. A “shepherd’s crook” boney deformity may present in the setting of multiple
microfractures and progressive varus stresses in the femoral neck.[11]
Giant cell tumor (GCT) – GCT is a nonmalignant tumor of the bone that typically presents in
adults who have reached skeletal maturity. They are aggressive osteolytic neoplasms that
may have distant metastases to the lungs; these metastases are usually benign. Although the
majority are nonmalignant, up to 8% of GCTs will undergo malignant transformation.
[12] GCT commonly presents as chronic pain and swelling at the site, with limitation of
movement of the nearby joint. If weight-bearing bones are involved, a pathologic fracture
may occur. Radiographs will show a lesion, typically in the epiphysis, with a non-central
lytic focus. Subchondral plate involvement is not uncommon. CT will show profound
cortical thinning and reveal the absence of matrix mineralization. GCT is the most
commonly involved tumor seen with secondary aneurysmal bone cysts.[13]
Telangiectatic osteosarcoma (TOS) – TOS is similar in presentation and appearance to
aneurysmal bone cysts. MRI with gadolinium can help differentiate the two by
demonstrating a nodular appearance to the tissue surrounding the cavities in TOS, which
corresponds to sarcomatous tissue. TOS will also reveal cortical destruction and a
corresponding soft-tissue mass.[3] Necrosis may also be present in TOS. However, the only
definitive way to differentiate the two diseases is through biopsy of the lesion, which will
reveal malignant tumor cells in TOS.
Unicameral bone cyst (UBCs) – Appearing in the first two decades of life, UBCs are fluid-
filled lesions surrounded by a fibrous lining of mesothelial cells. Patients will commonly
present with pain due to a pathological fracture located in the metaphysis of a long bone.
Radiography will reveal an expansile lesion that extends across the entire diameter of the
involved bone.[14] Well-defined margins are present without reactive sclerosis. Unlike
aneurysmal bone cysts, the diameter of a UBC will not exceed the diameter of the involved
bone. The “fallen fragment’ sign is pathognomonic for UBCs, in which fractured
intramedullary fragments are radiographically visible inside the cystic cavity.[15]
Go to:
Staging
The staging of aneurysmal bone cysts has its basis in Enneking’s staging of benign
musculoskeletal neoplasms [16]:
Stage 1: Latent (inactive) - These tumors are usually found incidentally and are
asymptomatic.
Stage 2: Active - These tumors are discovered due to the symptomatic discomfort of
the patient. They are steadily growing and may be palpable.
Stage 3: Aggressive - These tumors are typically found due to significant patient
discomfort and a possible visible abnormality with inflammation. Although these
tumors are benign, they act very similarly to low-grade malignancy.
Go to:
Prognosis
Surgical excision of aneurysmal bone cysts is usually curative; however, historically, a
spontaneous recurrence rate of 19% has been seen.[17] Recurrences tend to happen within
the first year of excision, but patients should be regularly evaluated for recurrence up to 5
years after surgery. In patients that have not yet reached skeletal maturity, recurrence could
affect future bone growth and cause deformities.
Go to:
Complications
Complications during treatment tend to vary depending on the lesion’s location in the
skeleton. As with any surgical procedure, the risk of bleeding and infection is of the utmost
concern, not only for a superficial wound infection but osteomyelitis as well. Damage to the
physis is possible if the lesion is near the growth plate. Pulmonary embolism is also a
concern due to venous thromboembolism or fat embolism, as it is with other orthopedic
surgeries.
Go to:
Postoperative and Rehabilitation Care
The patient’s return to activity is dependent on the location of the lesion, the extent of
excision (if excised), and the extent of the reconstruction (bone grafting vs. cement).
Physical and/or occupational therapy are essential to help reduce morbidity.
Go to:
Review Questions
Access free multiple choice questions on this topic.
Comment on this article.
Figure
Radiograph Ankle ABC Aneurysmal Bone Cyst Well Defined Expansile Lytic Lesion in
Child with Internal Septations. Contributed by Scott Dulebohn, MD
Figure
Tuberculosis Benign Bone Cyst, Radius, Ulnar, Central giant-of internal malleolus of tibia,
cell tumor of lower end of radius. Contributed by The Journal Of Radiology (1920)
Figure
Bone cyst in proximal femur. Image courtesy S Bhimji MD
Go to:
References
1.
Schreuder HW, Veth RP, Pruszczynski M, Lemmens JA, Koops HS, Molenaar WM.
Aneurysmal bone cysts treated by curettage, cryotherapy and bone grafting. J Bone
Joint Surg Br. 1997 Jan;79(1):20-5. [PubMed]
2.
Cottalorda J, Bourelle S. Modern concepts of primary aneurysmal bone cyst. Arch
Orthop Trauma Surg. 2007 Feb;127(2):105-14.[PubMed]
3.
Park HY, Yang SK, Sheppard WL, Hegde V, Zoller SD, Nelson SD, Federman N,
Bernthal NM. Current management of aneurysmal bone cysts. Curr Rev
Musculoskelet Med. 2016 Dec;9(4):435-444.[PMC free article] [PubMed]
4.
Boubbou M, Atarraf K, Chater L, Afifi A, Tizniti S. Aneurysmal bone cyst primary--
about eight pediatric cases: radiological aspects and review of the literature. Pan Afr
Med J. 2013;15:111. [PMC free article] [PubMed]
5.
Mascard E, Gomez-Brouchet A, Lambot K. Bone cysts: unicameral and aneurysmal
bone cyst. Orthop Traumatol Surg Res. 2015 Feb;101(1 Suppl):S119-27. [PubMed]
6.
Lau AW, Pringle LM, Quick L, Riquelme DN, Ye Y, Oliveira AM, Chou MM.
TRE17/ubiquitin-specific protease 6 (USP6) oncogene translocated in aneurysmal
bone cyst blocks osteoblastic maturation via an autocrine mechanism involving bone
morphogenetic protein dysregulation. J Biol Chem. 2010 Nov 19;285(47):37111-
20. [PMC free article] [PubMed]
7.
Rossi G, Rimondi E, Bartalena T, Gerardi A, Alberghini M, Staals EL, Errani C, Bianchi
G, Toscano A, Mercuri M, Vanel D. Selective arterial embolization of 36 aneurysmal
bone cysts of the skeleton with N-2-butyl cyanoacrylate. Skeletal Radiol. 2010
Feb;39(2):161-7.[PubMed]
8.
Papagelopoulos PJ, Currier BL, Shaughnessy WJ, Sim FH, Ebsersold MJ, Bond JR, Unni
KK. Aneurysmal bone cyst of the spine. Management and outcome. Spine (Phila Pa
1976). 1998 Mar 01;23(5):621-8. [PubMed]
9.
Aboulafia AJ, Kennon RE, Jelinek JS. Begnign bone tumors of childhood. J Am Acad
Orthop Surg. 1999 Nov-Dec;7(6):377-88.[PubMed]
10.
Levine SM, Lambiase RE, Petchprapa CN. Cortical lesions of the tibia: characteristic
appearances at conventional radiography. Radiographics. 2003 Jan-Feb;23(1):157-
77. [PubMed]
11.
Biermann JS. Common benign lesions of bone in children and adolescents. J Pediatr
Orthop. 2002 Mar-Apr;22(2):268-73.[PubMed]
12.
Amelio JM, Rockberg J, Hernandez RK, Sobocki P, Stryker S, Bach BA, Engellau J,
Liede A. Population-based study of giant cell tumor of bone in Sweden (1983-
2011). Cancer Epidemiol. 2016 Jun;42:82-9. [PubMed]
13.
Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ.
From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative
granuloma of bone: radiologic-pathologic correlation. Radiographics. 2001 Sep-
Oct;21(5):1283-309. [PubMed]
14.
Copley L, Dormans JP. Benign pediatric bone tumors. Evaluation and
treatment. Pediatr Clin North Am. 1996 Aug;43(4):949-66.[PubMed]
15.
Struhl S, Edelson C, Pritzker H, Seimon LP, Dorfman HD. Solitary (unicameral) bone
cyst. The fallen fragment sign revisited. Skeletal Radiol. 1989;18(4):261-5. [PubMed]
16.
Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop Relat
Res. 1986 Mar;(204):9-24. [PubMed]
17.
Vergel De Dios AM, Bond JR, Shives TC, McLeod RA, Unni KK. Aneurysmal bone cyst.
A clinicopathologic study of 238 cases. Cancer. 1992 Jun 15;69(12):2921-
31. [PubMed]
Disclosure: Kyle Stevens declares no relevant financial relationships with ineligible companies.
Disclosure: James Stevens declares no relevant financial relationships with ineligible companies.
Copyright © 2023, StatPearls Publishing LLC.
This book is distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0
the work, provided that the article is not altered or used commercially. You are not required to obtain permission to distribute
this article, provided that you credit the author and journal.
Views
PubReader
Print View
Cite this Page
In this Page
Continuing Education Activity
Introduction
Etiology
Epidemiology
Pathophysiology
Histopathology
History and Physical
Evaluation
Treatment / Management
Differential Diagnosis
Staging
Prognosis
Complications
Postoperative and Rehabilitation Care
Deterrence and Patient Education
Enhancing Healthcare Team Outcomes
Review Questions
References
Bulk Download
Bulk download StatPearls data from FTP
Related information
PMC
PubMed
Similar articles in PubMed
Aneurysmal Bone Cyst of the Head of the Fibula: An Unusual Presentation.[Cureus.
2022]
Review Bone cysts: unicameral and aneurysmal bone cyst.[Orthop Traumatol Surg Res.
2015]
Recent Activity
ClearTurn Off
See more...
FOLLOW NCBI
Connect with NLM
National Library of Medicine
8600 Rockville Pike
Bethesda, MD 20894
Web Policies
FOIA
HHS Vulnerability Disclosure
Help
Accessibility
Careers
NLM
NIH
HHS
USA.gov