Unicameral bone cyst: Difference between revisions
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*Various theories have been proposed concerning the pathogenesis of unicameral bone cyst: | *Various theories have been proposed concerning the pathogenesis of unicameral bone cyst: | ||
**Blockage in the venous drainage is the most favored mechanism which occurs in a rapidly growing and remodeling portion of cancellous bone. | **Blockage in the venous drainage is the most favored mechanism which occurs in a rapidly growing and remodeling portion of cancellous bone. | ||
** | **Increased internal pressure of involved bone as compared to normal pressure of bone marrow.<ref>Chigira M., Maehara S., Arita S., Udagawa E. The aetiology and treatment of simple bone cysts. Bone & Joint Journal. 1983;65(5):633–637.</ref> | ||
**Lower partial pressure of oxygen of cyst fluid than arterial or venous blood suggesting a venous obstruction.<ref>Chigira M., Maehara S., Arita S., Udagawa E. The aetiology and treatment of simple bone cysts. Bone & Joint Journal. 1983;65(5):633–637.</ref> | |||
**Increased levels of lysosomal enzymes in the cyst than serum. Enzymes include prostaglandins, interleukin 1β, nitrate and nitrites levels, proteolytic enzymes, tumor necrosis factor ⍺ and interleukins 1β and 6.<ref name="pmid10968539">{{cite journal| author=Komiya S, Inoue A| title=Development of a solitary bone cyst--a report of a case suggesting its pathogenesis. | journal=Arch Orthop Trauma Surg | year= 2000 | volume= 120 | issue= 7-8 | pages= 455-7 | pmid=10968539 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10968539 }} </ref> | |||
*Unicameral bone cyst typically occur in the metaphysis adjacent to the physis of the long bones. | |||
*The bones often involved are proximal femur, distal tibia, ilium, calcaneus, and occasionally metacarpals, phalanges, or distal radius. | |||
===Genetics=== | |||
*Unicameral bone cyst may have association with genetic abnormalities on chromosome 4, 6, 8, 16, 21, and both chromosomes 12. | |||
*Translocation t (16; 20) (p11.2; q13) has been found in cases with unicameral bone cyst. | |||
==Causes== | ==Causes== | ||
There are no established causes for unicameral bone cyst. | |||
==Differentiating Unicameral Bone Cyst from Other Diseases== | |||
Unicameral bone cyst must be differentiated from following bone disorders: | |||
{| | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |'''Bubbly lytic lesion on x-ray''' | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |'''Lakes of Blood on histology''' | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Diagnosis | |||
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Treatment is curretage and bone grafting | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Unicameral bone cyst | |||
| align="center" style="background:#F5F5F5;" + | + | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + |[[Radiology]] and [[biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | - | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Aneurysmal bone cyst | |||
| align="center" style="background:#F5F5F5;" + | + | |||
| align="center" style="background:#F5F5F5;" + | + | |||
| align="center" style="background:#F5F5F5;" + |[[Radiology]] and [[biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | + | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Non ossifying fibroma | |||
| align="center" style="background:#F5F5F5;" + | + | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + |[[Radiology]] and [[biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | - | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Giant cell tumor | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + |[[Radiology]] and [[Biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | + | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Chondroblastoma | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + |[[Biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | + | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Chondromyxoid Fibroma | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + |[[Radiology]] and [[biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | + | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Osteoblastoma | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + |[[Radiology]] and [[biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | + | |||
|- | |||
! align="center" style="background:#DCDCDC;" + |Telangiectatic osteosarcoma | |||
| align="center" style="background:#F5F5F5;" + | - | |||
| align="center" style="background:#F5F5F5;" + | + | |||
| align="center" style="background:#F5F5F5;" + |[[Radiology]] and [[biopsy]] | |||
| align="center" style="background:#F5F5F5;" + | - | |||
|} | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
*Unicameral bone cyst constitute approximately 3% of all bone tumors. | |||
*Adolescents and children are most affected by unicameral bone cyst.<ref name="pmid24788445">{{cite journal| author=Pretell-Mazzini J, Murphy RF, Kushare I, Dormans JP| title=Unicameral bone cysts: general characteristics and management controversies. | journal=J Am Acad Orthop Surg | year= 2014 | volume= 22 | issue= 5 | pages= 295-303 | pmid=24788445 | doi=10.5435/JAAOS-22-05-295 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24788445 }} </ref> | |||
*The age distribution of unicameral bone cyst is between 5-15 years.<ref name="pmid11856945">{{cite journal| author=Biermann JS| title=Common benign lesions of bone in children and adolescents. | journal=J Pediatr Orthop | year= 2002 | volume= 22 | issue= 2 | pages= 268-73 | pmid=11856945 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11856945 }} </ref> | |||
*The mean age of the patients with unicameral bone cyst is 9 years. | |||
*Men are more commonly affected than women, with a 2:1 ratio.<ref name="pmid4874360">{{cite journal| author=Boseker EH, Bickel WH, Dahlin DC| title=A clinicopathologic study of simple unicameral bone cysts. | journal=Surg Gynecol Obstet | year= 1968 | volume= 127 | issue= 3 | pages= 550-60 | pmid=4874360 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4874360 }} </ref> | |||
*There is no racial predilection to chondroblastoma. | |||
The | |||
==Risk Factors== | ==Risk Factors== | ||
There are no established risk factors for | There are no established risk factors for unicameral bone cyst. | ||
==Screening== | ==Screening== | ||
There is insufficient evidence to recommend routine screening for | There is insufficient evidence to recommend routine screening for unicameral bone cyst. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
*Common complications of unicameral bone cyst (UBC) include: | |||
**Pathological fracture | |||
**Premature epiphyseal closure | |||
***Limb-length discrepancy | |||
Common complications of | ***Angular deformity | ||
**Malignant transformation. | |||
*Prognosis is generally excellent for Unicameral bone cyst. | |||
**As a patient approaches skeletal maturity, a unicameral bone cyst will often decrease in size and may heal after growth is complete. | |||
Prognosis is generally excellent | **Fracture healing usually does not lead to cyst resolution. | ||
**It requires close follow up while in active phase due to recurrence and risk of fracture or growth arrest. | |||
==Diagnosis== | ==Diagnosis== |
Revision as of 22:19, 18 January 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rohan A. Bhimani, M.B.B.S., D.N.B., M.Ch.[2]
Synonyms and keywords:
Overview
Historical Perspective
- In mediveal times, Lagier et al identified a unicameral bonce cyst in the femur from the remains of a child.[1]
- In 1891, Virchow reported it as “cystic structures” that resulted due to anomalies in the local circulation.[2]
- In 1942, Jaffe and Lichenstein later recognized it as a distinct entity.[3]
Classification
Unicameral bone cyst can be classified based on imaging findings.
Enneking (MSTS) Staging System
- The Enneking surgical staging system (also known as the MSTS system) for benign musculoskeletal tumors based on radiographic characteristics of the tumor host margin.[4]
- It is widely accepted and routinely used classification.
Stages | Description |
---|---|
1 | Latent: Well demarcated borders |
2 | Active: Indistinct borders |
3 | Aggressive: Indistinct borders |
Pathophysiology
- The exact pathogenesis of unicameral bone cyst is not fully understood.
- Various theories have been proposed concerning the pathogenesis of unicameral bone cyst:
- Blockage in the venous drainage is the most favored mechanism which occurs in a rapidly growing and remodeling portion of cancellous bone.
- Increased internal pressure of involved bone as compared to normal pressure of bone marrow.[5]
- Lower partial pressure of oxygen of cyst fluid than arterial or venous blood suggesting a venous obstruction.[6]
- Increased levels of lysosomal enzymes in the cyst than serum. Enzymes include prostaglandins, interleukin 1β, nitrate and nitrites levels, proteolytic enzymes, tumor necrosis factor ⍺ and interleukins 1β and 6.[7]
- Unicameral bone cyst typically occur in the metaphysis adjacent to the physis of the long bones.
- The bones often involved are proximal femur, distal tibia, ilium, calcaneus, and occasionally metacarpals, phalanges, or distal radius.
Genetics
- Unicameral bone cyst may have association with genetic abnormalities on chromosome 4, 6, 8, 16, 21, and both chromosomes 12.
- Translocation t (16; 20) (p11.2; q13) has been found in cases with unicameral bone cyst.
Causes
There are no established causes for unicameral bone cyst.
Differentiating Unicameral Bone Cyst from Other Diseases
Unicameral bone cyst must be differentiated from following bone disorders:
Disease | Bubbly lytic lesion on x-ray | Lakes of Blood on histology | Diagnosis | Treatment is curretage and bone grafting |
---|---|---|---|---|
Unicameral bone cyst | + | - | Radiology and biopsy | - |
Aneurysmal bone cyst | + | + | Radiology and biopsy | + |
Non ossifying fibroma | + | - | Radiology and biopsy | - |
Giant cell tumor | - | - | Radiology and Biopsy | + |
Chondroblastoma | - | - | Biopsy | + |
Chondromyxoid Fibroma | - | - | Radiology and biopsy | + |
Osteoblastoma | - | - | Radiology and biopsy | + |
Telangiectatic osteosarcoma | - | + | Radiology and biopsy | - |
Epidemiology and Demographics
- Unicameral bone cyst constitute approximately 3% of all bone tumors.
- Adolescents and children are most affected by unicameral bone cyst.[8]
- The age distribution of unicameral bone cyst is between 5-15 years.[9]
- The mean age of the patients with unicameral bone cyst is 9 years.
- Men are more commonly affected than women, with a 2:1 ratio.[10]
- There is no racial predilection to chondroblastoma.
Risk Factors
There are no established risk factors for unicameral bone cyst.
Screening
There is insufficient evidence to recommend routine screening for unicameral bone cyst.
Natural History, Complications, and Prognosis
- Common complications of unicameral bone cyst (UBC) include:
- Pathological fracture
- Premature epiphyseal closure
- Limb-length discrepancy
- Angular deformity
- Malignant transformation.
- Prognosis is generally excellent for Unicameral bone cyst.
- As a patient approaches skeletal maturity, a unicameral bone cyst will often decrease in size and may heal after growth is complete.
- Fracture healing usually does not lead to cyst resolution.
- It requires close follow up while in active phase due to recurrence and risk of fracture or growth arrest.
Diagnosis
Diagnostic Study of Choice
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
OR
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
OR
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
OR
There are no established criteria for the diagnosis of [disease name].
History and Symptoms
The majority of patients with [disease name] are asymptomatic.
OR
The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
Physical Examination
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
OR
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
OR
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
Laboratory Findings
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
OR
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
OR
[Test] is usually normal among patients with [disease name].
OR
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
OR
There are no diagnostic laboratory findings associated with [disease name].
Electrocardiogram
There are no ECG findings associated with [disease name].
OR
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
X-ray
There are no x-ray findings associated with [disease name].
OR
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with [disease name].
OR
Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
CT scan
There are no CT scan findings associated with [disease name].
OR
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
MRI
There are no MRI findings associated with [disease name].
OR
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Other Imaging Findings
There are no other imaging findings associated with [disease name].
OR
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
There are no other diagnostic studies associated with [disease name].
OR
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
OR
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
OR
The majority of cases of [disease name] are self-limited and require only supportive care.
OR
[Disease name] is a medical emergency and requires prompt treatment.
OR
The mainstay of treatment for [disease name] is [therapy].
OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.
OR
[Therapy] is recommended among all patients who develop [disease name].
OR
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
OR
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
OR
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
OR
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
Surgery
Surgical intervention is not recommended for the management of [disease name].
OR
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
OR
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
OR
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
OR
Surgery is the mainstay of treatment for [disease or malignancy].
Primary Prevention
There are no established measures for the primary prevention of [disease name].
OR
There are no available vaccines against [disease name].
OR
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
OR
[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].
Secondary Prevention
There are no established measures for the secondary prevention of [disease name].
OR
Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].
References
- ↑ Lagier R, Kramar C, Baud CA (1987). "Femoral unicameral bone cyst in a medieval child. Radiological and pathological study". Pediatr Radiol. 17 (6): 498–500. PMID 3317251.
- ↑ Wilkins RM (2000). "Unicameral bone cysts". J Am Acad Orthop Surg. 8 (4): 217–24. PMID 10951110.
- ↑ Jaffe HL, Lichtenstein L. Solitary unicameral bone cyst with emphasis on the roentgen picture, the pathologic appearance and the pathogenesis. Arch Surg. 1942. 44:1004-25.
- ↑ Jawad MU, Scully SP (2010). "In brief: classifications in brief: enneking classification: benign and malignant tumors of the musculoskeletal system". Clin Orthop Relat Res. 468 (7): 2000–2. doi:10.1007/s11999-010-1315-7. PMC 2882012. PMID 20333492.
- ↑ Chigira M., Maehara S., Arita S., Udagawa E. The aetiology and treatment of simple bone cysts. Bone & Joint Journal. 1983;65(5):633–637.
- ↑ Chigira M., Maehara S., Arita S., Udagawa E. The aetiology and treatment of simple bone cysts. Bone & Joint Journal. 1983;65(5):633–637.
- ↑ Komiya S, Inoue A (2000). "Development of a solitary bone cyst--a report of a case suggesting its pathogenesis". Arch Orthop Trauma Surg. 120 (7–8): 455–7. PMID 10968539.
- ↑ Pretell-Mazzini J, Murphy RF, Kushare I, Dormans JP (2014). "Unicameral bone cysts: general characteristics and management controversies". J Am Acad Orthop Surg. 22 (5): 295–303. doi:10.5435/JAAOS-22-05-295. PMID 24788445.
- ↑ Biermann JS (2002). "Common benign lesions of bone in children and adolescents". J Pediatr Orthop. 22 (2): 268–73. PMID 11856945.
- ↑ Boseker EH, Bickel WH, Dahlin DC (1968). "A clinicopathologic study of simple unicameral bone cysts". Surg Gynecol Obstet. 127 (3): 550–60. PMID 4874360.