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CHAPTER MENU
Simple Bone Cyst, 271
– Frequency, 272
–
– Age, 273
–
– Sex, 273
–
– Site, 273
–
●● Pathogenesis, 278
●● Histopathology, 280
●● Treatment, 281
– Frequency, 281
–
– Age, 281
–
– Sex, 282
–
– Site, 282
–
●● Pathogenesis, 284
●● Histopathology, 285
●● Treatment, 285
●● Pathogenesis, 286
●● Histopathology, 286
Shear’s Cysts of the Oral and Maxillofacial Regions, Fifth Edition. Paul M. Speight.
© 2022 John Wiley & Sons Ltd. Published 2022 by John Wiley & Sons Ltd.
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This chapter considers a number of lesions of the jaws that Classification and Terminology
present clinically or radiologically as cystic lesions, but are
A notable feature of all the publications is uncertainty
empty or fluid-filled bony cavities without an epithelial
about the nature of this lesion and this is reflected in the
reviewed.
rhagic bone cyst (Howe 1965) or solitary bone cyst
(Rushton 1946). In the long bones, the lesion is often
referred to as unicameral bone cyst. Although this term is
Simple Bone Cyst
Diagnostic Criteria
gory of ‘bone cysts’. In the first edition it was called simple
Simple bone cyst is a cystic radiolucency that on gross bone cyst (Pindborg and Kramer 1971), but in the second
or histological examination does not have an epithelial edition it was solitary bone cyst (Kramer et al. 1992).
lining, but shows a cavity that is empty or contains Subsequent editions (Barnes et al. 2005; El-Naggar
small amounts of fluid. et al. 2017), including the latest (fifth) edition (WHO 2022a),
have consistently called this lesion simple bone cyst with
D ag c r ra
‘solitary’, ‘traumatic’, and ‘haemorrhagic’ as synonyms.
i nosti C ite i
and bone tumours also prefers the term simple bone cyst
● The cavity is empty or contains small amounts of fluid
term (WHO 2022b).
●
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272 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
For these reasons we suggest that simple bone cyst should Gomez (2019c) identified 284 papers that reported 1253
always be used. This reflects the simple nature of the lesion cysts. Table 17.1 summarises a number of selected larger
and makes no presumptions regarding pathogenesis or case series (>20 cases) with a broad geographical distribu-
clinical findings. Although it is not a true cyst, it presents tion, as well as the findings of the review by Chrcanovic
clinically and radiologically as a cystic lesion and the term and Gomez (2019c). The simple bone cyst shows a number
‘cyst’ remains acceptable and widely used. Simple bone of characteristic features (Box 17.2) that assist in making a
cyst is also the preferred term for all lesions, no matter diagnosis, but ultimately a final diagnosis is made by exclu-
where in the skeleton they are encountered. sion of any known cause of a cystic cavity in the jaws
There is some evidence, discussed in detail in the follow- (Box 17.1).
ing sections, that the simple bone cyst is not a single clin-
icopathological entity, but may be a common end result of Frequency
a number of causative factors (see ‘Pathogenesis’). The vast Simple bone cysts account for about 3% of bone lesions and
majority of lesions are single or solitary, affecting the man- are most common in children and adolescents. Most
dible, but about 5.0% of patients may have multiple cysts lesions (about 80%) are found in the proximal metaphyseal
(Chrcanovic and Gomez 2019c). In addition, there are a regions of the humerus (50%) or femur (30%), with most of
number of reports of lesions similar to simple bone cysts the remainder found in the proximal tibia or other long
arising in association with cemento-osseous dysplasia (e.g.
bones. Jaw lesions are rare and represent less than 5% of all
Chadwick et al. 2011; Yeom and Yoon 2020). Shimoyama skeletal lesions and about 1% of all jaw cysts.
et al. (1999) suggested that the simple bone cyst may take In his review of cases from the University of the
three distinctive clinicopathological forms: simple solitary Witwatersrand, Shear found only 35 specimens during the
lesions, multiple lesions that may progress or recur, and 46-year period under review, constituting 1% of all jaw
cysts associated with other lesions, primarily cemento- cysts, and 7.2% of non-odontogenic cysts (see Table 1.1). In
osseous dysplasia. In the following sections solitary and a review of Sheffield specimens there were only 36 cases
multiple lesions will be considered together as variants of reported over a 30-year period, representing 0.5% of 6862
the same entity, but lesions associated with cemento- cysts of the jaws (Jones and Franklin 2006a,b) and only
osseous dysplasia will be discussed separately (see later 0.07% of all oral biopsies. Other studies have shown similar
Box 17.3 and ‘Simple Bone Cysts Associated with Cemento- low frequencies, with only 4 cases reported among 2030 jaw
osseous Dysplasia’). cysts (0.2%) in a study from Italy (Lo Muzio et al. 2017) and
9 cases among 5295 cysts in Greece (0.2%; Tamiolakis
et al. 2019).
Clinical Features
Most simple bone cysts are found below the age of
Most reports of simple bone cyst in the jaws are single case 20 years and so they are relatively more common in chil-
reports or small series. Lima et al. (2020) found 29 pub- dren. Jones and Franklin found an overall frequency of
lished case series (5 cases or more) between 1951 and 2019, 0.5% of jaw cysts, but the frequency in their paediatric pop-
reporting almost 800 lesions. In a detailed systematic ulation was 3.4% (19 cases among 556 jaw cysts; Jones and
review, including single case reports, Chrcanovic and Franklin 2006b), which was more than 10 times greater
Table 17.1 Age, sex, and site distribution, and frequency of scalloping, of simple bone cysts from selected case series and the
References Country n Age mean (range) Male (%) Mandible (%) Scalloped (%)
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Box 17.2 Simple Bone Cyst: Key Features
Clini cal
●● 60–70% arise in the second decade
●● Average age is 20 years
Rad iolo gy
●● A well-demarcated radiolucency
than the frequency of 0.3% in adults (Jones and a slight female predilection. In their large systematic
Franklin 2006a). They also found that in children the simple review, Chrcanovic and Gomez (2019c) found that 51.7% of
bone cyst represented 51.4% of non-odontogenic cysts (19
cases arose in females and 48.3% in males. However, it has
cases among 37), compared with only 6.7% in adults. Other been shown that multiple lesions and lesions associated
but smaller studies have found higher frequencies in children. with cemento-osseous dysplasia arise more commonly in
In a study from Israel, Manor et al. (2012) encountered 95 jaw females and in older age groups. This is discussed later in
cysts in children over a 20-year period and found 17 (17.9%)
this chapter.
simple bone cysts. Over the same period, they recorded no
cases in adults (≥17 years). Resnick et al. (2016) reported 45
Site
cases of simple bone cyst diagnosed over a 15-year period at
Simple bone cysts involving the jaws are almost always
the Boston Children’s Hospital (Boston, MA, USA). found in the mandible, with each case series only reporting
one or two cases in the maxilla (Table 17.1). Studies have
Age consistently shown that almost all the maxillary cases have
The simple bone cyst occurs in young individuals, with the involved the anterior regions and the majority of the man-
vast majority of cases occurring below the age of 20 years. dibular cases have been reported in the body (about 65%)
There is a wide age range, with cases reported in patents up or symphyseal areas (about 25%) (Copete et al. 1998;
to 74 years and with an overall average of between about 20
Cortell-Ballester et al. 2009; Resnick et al. 2016; Flores
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274 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
extending further posteriorly or anteriorly. They also found Lima et al. (2020) identified 23 published case series that
that 129 lesions (16.5%) crossed the midline of the mandi- had recorded any history of trauma and found that 5
ble and 6 (0.8%) involved the coronoid process. reported that no patients could recollect a traumatic event.
Occasional cases have been reported in the mandibular The remaining 18 reports recorded trauma in between 5.0%
condyle (Persson 1985; Rubin and Murphy 1989; Telfer and 87.5% of patients, with an overall average of 22.2%
et al. 1990; Magliocca et al. 2007). (range 0.0–87.5%).
It seems that early case reports may have been more fas-
Clinical Presentation tidious in recording possible traumatic events. In his review
Most simple bone cysts are found incidentally when a of 60 cases reported between 1929 and 1963, Howe (1965)
patient undergoes radiological examination for another found details of trauma in 33 (55%) cases. Of these, 11 were
purpose. In the case series shown in Table 17.1, three of the sporting injuries, 5 were road accidents (including 1 hit by
studies reported that all their cases were incidental find- a trolley car), 4 were due to falls (1 after an explosion), and
ings with no clinical signs or symptoms (Cortell-Ballester 4 followed dental extraction. A further 10 cases were
et al. 2009: You et al. 2017; Roma et al. 2021). In the studies recorded as miscellaneous, including a gunshot to the face,
that recorded clinical findings, the frequency of symptoms a blow with an axe, and being trapped in swing doors.
was 18.3% (Lima et al. 2020) and 9.5% (Flores et al. 2017). A number of authors have noted an association with
In their review of the literature, Lima et al. (2020) identi- orthodontic treatment (Copete et al. 1998; Velez et al. 2010;
fied 25 case series that had recorded clinical findings and Resnick et al. 2016). Velez et al. (2010) found that 10
found that in 9, all the cases were incidental findings on (22.7%) of their patients had undergone orthodontic treat-
radiology, with no clinical signs or symptoms. Of the ment and considered this to be an important factor in the
remaining 16 reports, signs and symptoms were found in pathogenesis of the lesion. They suggested that the ‘micro-
between 2.2% and 30.4% of patients, with an overall aver- trauma’ associated with tooth movement may increase vas-
age of 15.2% (range 0.0–30.4%). Chrcanovic and Gomez cular activity and osteoclast stimulation, or that a
(2019c) found that clinical findings were recorded for 836 piezoelectric affect may promote bone cavity formation
patients and that only 88 (10.5%) reported symptoms. (Velez et al. 2010). In support of this hypothesis, they did
In almost all reports, the only clinical symptoms are provide some evidence of a true association with orthodon-
complaints of swelling associated with pain or tenderness. tic treatment. They compared the incidence of orthodontic
Flores et al. (2017) found that 4 of their 42 patients were treatment in their group of patients with simple bone cysts
aware of swelling, but none had pain, whereas Lima et al. to the incidence in an age- and sex-matched control group
(2020) stated that 11 of their patients complained of pain or without cysts. Of the cyst patients 23% had undergone
swelling. Hansen (1974) detailed clinical findings in 61 orthodontic treatment, compared with only 13% of the
patients and found that 44 (72.1%) were incidental findings matched controls.
and only 8 (13.1%) complained of pain or tenderness. In Others suggest, however, that the association with ortho-
the remaining 9 patients the symptoms were vague and dontic treatment is entirely coincidental, since the peak
non-specific. No patients complained of swelling, although
age of presentation of the simple bone cyst (the second dec-
on clinical or radiological examination 14 (23.0%) cases ade) is the same as the age at which most adolescents have
showed some evidence of bone expansion. Howe (1965) orthodontic treatment and have radiographs taken (Copete
reviewed 60 early cases and found that 20 (33.3%) patients et al. 1998). Resnick et al. (2016) noted that all the cases in
reported symptoms. Of these, 16 (26.7%) had noted swell- their series were found incidentally on radiographs taken
ing and in 2 cases this was accompanied by pain. The during orthodontic treatment or routine dental care.
remaining 4 cases reported pain alone (2 cases) or pain Routine radiology of the jaws at a young age may also be
with paraesthesia (2 cases). the reason why the peak age of presentation is in the sec-
The simple bone cyst has been thought to be caused by ond decade. It is possible that many lesions may remain
trauma (see ‘Pathogenesis’), but overall only about 25% of symptomless and undiscovered until later in life when
patients report an episode of trauma (Chrcanovic and radiographs are taken to investigate other disorders. This
Gomez 2019c). In the series in Table 17.1, trauma was would explain the much lower prevalence in older age
recorded in 4 of the reports. The proportion of patients groups, when routine panoramic radiographs are much
reporting an episode of trauma was 28.6% (Flores less common. This same premise may also account for the
et al. 2017), 23.8% (Cortell-Ballester et al. 2009), 11.7%
association with trauma. Young people are more likely to
(Lima et al. 2020), and 7.4% (You et al. 2017). In their series sustain a traumatic injury and undergo radiological exami-
of 45 paediatric patients, Resnick et al. (2016) found that nation, whereupon an asymptomatic lesion may be
only 6 (13.3) patients had a history of trauma. discovered.
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Although simple bone cysts often embrace the teeth, the 30.8% (Flores et al. 2017), and 25.0% (Velez et al. 2010). In
teeth are found to be vital in about 95% of cases (Chrcanovic their systematic review, Chrcanovic and Gomez (2019c)
and Gomez 2019c; Lima et al. 2020). found that 41.7% of 561 cases where the feature was
Simple bone cysts of the long bones are one of the most recorded showed scalloping. MacDonald-Jankowski (1995)
common causes of pathological fracture in children and regarded this feature as typical of the simple bone cyst and
adolescents, and about 80% of cases present with a fracture used the wonderfully descriptive term of the tooth roots or
of the humerus or femur. In the jaws, however, pathologi- apices ‘hanging’ within the cavity of the cyst (Figure 17.1).
cal fractures are very rare, and have only been encountered In most cases the lamina dura and periodontal space are
in 0.6% of cases (Chrcanovic and Gomez 2019c). intact and normal, but scalloped lesions may closely
embrace the teeth, and the lamina dura may be lost
(Figure 17.1). Suei et al. (2010) reviewed the radiological
Radiological Features
findings of 30 cases and found that 9 (30%) had a scalloped
Most simple bone cysts are symptomless and a final diag- margin, and that in all 9 there was some loss of the lamina
nosis is made on the basis of radiological examination and dura. Conversely, 21 cases had a smooth margin and only 4
a lack of specific pathological features. This means that the of these (19.0%) showed any loss of lamina dura. Cases
diagnosis is always made on the basis of radiology followed with loss of lamina dura were also larger, may show root
by exclusion of other causes of a radiolucent cystic cavity resorption, and were more likely to have recurred. Overall,
(Box 17.1). however, root resorption or displacement of the teeth is
The cyst appears as a radiolucent area that is well demar- very rare and has been noted in only about 1.0% of cases
cated, with an irregular but definite margin and slight cor- (Chrcanovic and Gomez 2019c).
tication (Figure 17.1). The majority of lesions (85–90%) are Another characteristic radiological feature that is almost
unilocular (Velez et al. 2010; Chrcanovic and Gomez 2019c; diagnostic of the simple bone cyst is the presence of a cone-
Roma et al. 2021), but a scalloped margin is common and is shaped margin. Copete et al. (1998) described this charac-
regarded as a characteristic feature. teristic cone-shaped morphology in 28 (63.6%) of their 44
The scalloped margin is at the superior aspect of the cases. The typical shape was of a lesion that was oval or
lesion, where extensions of the cyst rise up between the rounded towards the posterior aspect of the mandible, but
tooth roots (Figure 17.1). This feature is so characteristic as with a cone shape at the anterior margin, with two planes
to be regarded as almost diagnostic of the simple bone cyst. converging at a 45° angle (Figure 17.1, arrows). This mor-
However, it is not seen in all cases (Table 17.1). The reported phology was present in 23 (52.3%) of their cases. Two cases
frequencies of a scalloped margin have been 72.4% (Lima showed a double cone shape, with cones projected at the
et al. 2020), 71.7% (Howe 1965), 68.2% (Copete et al. 1998), anterior and posterior margins, and three cases had ‘half-
involving an extensive area in the right body of the mandible. Flores et al. 2017; You et al. 2017; Roma et al. 2021;
This example has a well-defined margin with cortication.
roots appear to ‘hang’ into the cystic cavity. The anterior margin
of the cyst is cone shaped (arrows). Source: Courtesy of Prof. cancellous bone, often with minimal buccal or lingual
Christoffel Nortjé. expansion. In the larger reviews or case series, the
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276 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
proportion that have shown expansion has been between Simple Bone Cyst Associated
12% and 25% (Velez et al. 2010; Flores et al. 2017; with Cemento-osseous Dysplasia
Chrcanovic and Gomez 2019c; Lima et al. 2020), but the
In 1976, Melrose et al. described a series of cases of
images and descriptions suggest that expansion is minimal
cemento-osseous dysplasia that were extensive, with mul-
and tends to be smooth and fusiform (Suei et al. 2010).
It has been reported that in about 5.0% of patients, sim- dysplasia. Subsequently there have been a number of small
ple bone cysts may be multiple or bilateral. In the case case series and single case reports (Table 17.2; Horner and
series shown in Table 17.1, bilateral lesions were Forman 1988; Higuchi et al. 1988; Tong et al. 2003;
reported in 2 cases (3.4%) by Lima et al. (2020) and in 2 Mahomed et al. 2005; Velez et al. 2010; Zillo Martini
cases (7.4%) by You et al. (2017). Howe (1965) reviewed et al. 2010; Peacock et al. 2015; Yeom and Yoon 2020).
60 patients and found 1 (1.7%) who had two lesions. A It is uncertain whether these lesions should be regarded
number of studies have reported multiple lesions that as simple bone cysts or as cystic change that may occur as
have arisen in association with cemento-osseous dyspla-
part of the natural history and development of cemento-
sia, but these may be a separate entity and are discussed osseous dysplasia. In this respect, it is well documented
in the next section. that a number of fibro-osseous lesions, including cemento-
It is important to note that Chrcanovic and Gomez osseous dysplasia, show cystic change both on radiology
(2019c), in their systematic review, excluded cases that and on histological examination. Su et al. (1997a,b) under-
were associated with cemento-osseous or fibrous dysplasia
took a detailed analysis of 241 cemento-osseous dysplasias
on the basis that they behave differently. They found a total and found that about 30% showed radiolucent lesions with
of 1253 cysts in 1187 patients. There were 58 patients (4.9%) well-demarcated margins that may resemble simple bone
with multiple cysts, of which 45 patients had two cysts, cysts. On histological examination, they noted that large
11 had three cysts, and 2 had many cysts. They also found blood-filled spaces are common. In a more recent review of
that patients with multiple cysts were more often female fibro-osseous lesions, Eversole et al. (2008) noted that
(71.3% vs 49.5% for solitary cysts) and older (26.6 years vs
empty bone cavities and circumscribed radiolucent areas
19.7 years) and that multiple cysts were more often scal-
are a characteristic feature of fibro-osseous lesions, includ-
loped (67.1% vs 37.4%), were more likely to cause bone ing ossifying fibroma and cemento-osseous dysplasia. They
expansion (36.2% vs 24.6%) and to recur or persist (12.0% noted that in florid cemento-osseous dysplasia, these cavi-
that multiple or bilateral lesions could be regarded as a however, cystic change has not been prominent or has not
separate type of simple bone cyst that is distinctive from been noted as a specific feature. Cavalcanti et al. (2018)
solitary lesions (Box 17.3). reviewed the cone beam computed tomography (CBCT)
Table 17.2 Age and sex distribution of simple bone cysts that have been reported to be associated
with cemento-osseous dysplasia, from selected case series and the review of Yeom and Yoon (2020).
Note: The review by Yeom and Yoon (2020) incorporates some of the included case series.
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features of 82 cemento-osseous dysplasias, and although
stroma of heavily mineralised lesions. Histological examina-
their images show a number of well-demarcated cystic
tion showed a variable pattern of mineralisation, but the
radiolucencies, they did not regard any cases as being asso- authors noted areas of cellular osteoid associated with resorp-
ciated with simple bone cyst. Many of the hypodense tion that created fluid- or blood-filled spaces that coalesced to
(lucent) lesions that they illustrated contained areas of form empty, smooth-surfaced cystic spaces. The authors pro-
of the jaws. They identified five areas that they described as gest cystic spaces within the stroma of the lesions surrounded
simple bone cyst, characterised as hypodense (lucent) cor- or lined by lesional tissue, rather than a cavity within the
ticated lesions that caused expansion and perforation of bone, which is a feature of the simple bone cyst.
the cortical bone. However, the case that they illustrated We would suggest that this process represents a form of
showed a corticated hypodense area containing the roots of cystic degeneration that might be a common feature of
a molar tooth with hypercementosis and irregular deposits fibro-osseous lesions generally, rather than a specific exam-
did not find any cases that they described as being associ- fibro-osseous lesions and blood-filled cystic spaces that
ated with simple bone cysts. They did find, however, that have been called secondary aneurysmal bone cyst. These
florid cemento-osseous dysplasia typically shows multiple
almost certainly represent degenerative change and are not
radiolucent and mixed radiolucent/radiopaque areas. true aneurysmal bone cysts, which are now known to be a
Their illustrations show radiolucencies that resemble sim- neoplasm. The term aneurysmal bone cyst should no
ple bone cysts, with scalloping of the margins and the longer be used in the context of these secondary, reactive
‘hanging’ roots feature. Furthermore, they showed that changes (WHO 2022b). Aneurysmal bone cyst is discussed
early and intermediate lesions showed multiple well- in more detail later in this chapter.
demarcated radiolucencies and suggested that cemento- Table 17.2 summarises demographic data from five
osseous dysplasia is a progressive disease that becomes reports of simple bone cysts associated with cemento-
increasingly mineralised over time. osseous dysplasia, and from a review of 45 cases by Yeom
This progression was also shown in the original series and Yoon (2020). In addition, there are reports of cysts aris-
described by Melrose (1976). He found that 14 of his ing within cemento-osseous dysplasia in some of the larger
patients had cystic radiolucencies that proved to be empty series of simple bone cysts, including 6 cases included in a
on surgical exploration or histological examination. series of 44 cysts by Velez et al. (2010), 6 cases among 20
Although he called these simple bone cysts, he also sug- cysts by Peacock et al. (2015), and 1 case in each of the
gested that they were a stage in the progression of the series of 60 and 44 cysts reported by Lima et al. (2020) and
cemento-osseous dysplasia. He described and illustrated
Copete et al. (1998), respectively. In total about 50 cases
progressive mineralisation of the radiolucencies over a have been reported.
period of 1–29 years. On histological examination, he also
The data in Table 17.2 show a different demographic of
found that cases often showed ‘aneurysmally’ dilated ves- patients to those summarised in Table 17.1. Patients
sels and blood-filled spaces that he thought might repre-
reported as having cysts associated with cemento-osseous
sent early cyst development. Other cystic spaces were lined dysplasia were almost all female and had an average age of
by a thin layer of fibrous tissue with scattered osteoclasts. about 45 years, with a peak in the fifth and sixth decades,
These features are in keeping with the concept that fibro- compared with patients with simple bone cysts alone who
osseous lesions show cystic degeneration of the stroma, show an equal sex distribution and an average age of about
either in early lesions before mineralisation (Pereira 20 years, with a peak in the second decade. In reports where
et al. 2016) or in the stroma of later mineralised lesions (Su the ethnicity is recorded, cases associated with cemento-
et al. 1997a,b; Eversole et al. 2008). Such degeneration may osseous dysplasia have, almost without exception, been
result in empty or blood-filled spaces, often resembling sim-
reported in black females. This demographic is the same as
ple bone cyst or aneurysmal bone cyst (van Heerden for cemento-osseous dysplasia generally (Su et al. 1997b;
et al. 1989; Raubenheimer et al. 2016). Raubenheimer et al. Eversole et al. 2008; Pereira et al. 2016). A review of the
(2016) described 18 cases (in 14 patients) of large expansile papers and of the illustrations shows that most cases have
osseous dysplasia. Of these cases, 5 were described as con- presented as typical florid cemento-osseous dysplasia with
taining simple bone cysts, but these were illustrated as multiple radiolucencies, many of which are mixed with
hypodense areas on computed tomography (CT) within the focal areas of mineralisation.
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278 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
strated an empty bone cavity (Horner and Forman 1988; osseous dysplasia and simple bone cysts. On the one hand,
Higuchi et al. 1988; Tong et al. 2003; Mahomed et al. 2005; cystic areas and well-demarcated radiolucencies are a
Velez et al. 2010; Zillo Martini et al. 2010; Peacock well-established feature of fibro-osseous lesions, includ-
et al. 2015). Taken together, the findings of multiple radio- ing cemento-osseous dysplasia (van Heerden et al. 1989;
lucencies and an empty cavity have been taken to confirm Su et al. 1997a,b; Eversole et al. 2008; Pereira et al. 2016;
a diagnosis of simple bone cyst. However, in many of the Raubenheimer et al. 2016; Cavalcanti et al. 2018; Kato
reports, the radiolucent cystic spaces often contained foci et al. 2020). These may be seen in early lesions that have
of mineralisation and the associated teeth showed hyperce- not yet mineralised or in later or large lesions that may
mentosis. These features have been reported in most cases undergo cystic degeneration. In this respect, therefore,
of cemento-osseous dysplasia and radiolucent, empty
these cystic spaces may not be simple bone cysts, but
cystic spaces are a consistent feature, especially in early merely part of the spectrum of radiological and histologi-
and developing lesions (Su et al. 1997b; Pereira et al. 2016; cal features that are seen in a variety of fibro-osseous
used to confirm the association between the two lesions similar pathogenic mechanism (see ‘Pathogenesis’) with a
and to suggest that up to one-third of simple bone cysts
common end result. This explanation is supported by the
may be associated with florid cemento-osseous dysplasia
observation that most of the cysts described in cemento-
(e.g. Raubenheimer et al. 2017). However, care must be osseous dysplasia have been empty cavities with character-
taken in interpreting this report. First, it is not clear istic radiological features similar to simple bone cysts in
whether Chadwick et al. (2011) reported a sequential series young people (Horner and Forman 1988; Higuchi
of cases, or rather reported and compared selected cohorts et al. 1988; Tong et al. 2003; Mahomed et al. 2005; Velez
of 68 solitary and 23 cemento-osseous dysplasia-associated
et al. 2010; Zillo Martini et al. 2010; Peacock et al. 2015).
cysts. Secondly, the 23 cases recorded as simple bone cysts This concept supports the proposal of Shimoyama et al.
in cemento-osseous dysplasia did not meet the criteria for
(1999) that the simple bone cyst takes three clinicopatho-
diagnosis (Box 17.1), but were diagnosed only on the radio- logical forms: simple solitary lesions, multiple lesions that
logical findings, with no exclusion of other pathologies by may progress or recur, and cysts in older patients associated
surgical intervention or histological analysis. It is possible, with cemento-osseous dysplasia (Box 17.3).
therefore, that many of the multiple radiolucent lesions A number of authors regard simple bone cysts associated
reported as simple bone cysts were in fact early lesions of with cemento-osseous dysplasia as distinctive and separate
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cohort of young people without cysts. It is quite possible at operation to be empty, the fact that some contain
that the association is entirely coincidental due to the blood or serosanguineous fluid tends to support the
frequent radiological examination of young people follow-
concept of a h aematoma breaking down. As noted pre-
did provide some evidence of a true association by showing haemorrhage to produce empty or blood- f illed cavities
that 23% of their cyst patients had undergone orthodontic resembling simple bone cyst or aneurysmal bone cyst
treatment compared to only 13% of matched controls. (van Heerden et al. 1989; Raubenheimer et al. 2016).
Olech et al. (1951) first suggested that trauma was the Raubenheimer et al. (2016) in particular noted areas of
cause and proposed that trauma to a bone results in cellular osteoid that were associated with resorption
intramedullary haemorrhage, which on occasion may fail that created fluid- or blood-f illed spaces that coalesced
to organise and can resorb to leave an empty cystic cavity. to form empty, smooth- s urfaced cystic spaces. They
They suggested that cysts seem to develop only after injury proposed that this process represented the formation of
to those areas of the skeleton where cancellous bone is simple bone cysts, and it is possible that a similar
enclosed in a heavy compact cortical layer. This would mechanism may occur within an organising haema-
explain the most frequent sites in the metaphyses of long toma following trauma.
bones and in the mandible. It would also explain the fact Shimoyama et al. (1999) reviewed the literature and
that most simple bone cysts develop in young individuals. found that there had been eight different theories about the
This proposal by Olech et al. (1951) has never been pathogenesis of the simple bone cyst, but these can be con-
substantiated nor refuted and most authors still regard solidated into three potential mechanisms (Harnet
some form of trauma as the most likely cause. The et al. 2008): trauma, a disorder or imbalance in bone growth
breakdown of haematomas and their failure to organ- or turnover, and degeneration of a bone lesion or tumour.
ise, particularly if they are large, is a well- k nown prob-
Harnet et al. (2008) regarded trauma as the most likely
lem in surgery and it is perfectly conceivable that this and the most widely accepted cause of the simple bone
could occur following intramedullary haemorrhage. cyst. They suggested that the process first proposed by
Although the majority of simple bone cysts are found Olech et al. (1951), of intramedullary haemorrhage
Solit ar Cysts
y
● The cysts are empty or contain fluid, but often contain focal mineralisation
●
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280 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
Summary and Conclusions
As can be seen from this discussion, these three pathogenic
mechanisms have similar elements and are not mutually
exclusive. Overall, it seems that the simple bone cyst is not
a single clinicopathological entity, but can be regarded as a
spectrum with different causes although with a similar
pathogenesis and a common end result – an intraosseous
cystic cavity. This is in keeping with the concept that there
Figure 17.2 A simple bone cyst is lined by loose vascular
are three clinicopathological variants of the simple bone
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281
decorin. These authors undertook a histological and immu- Hisatomi et al. 2019; Morita et al. 2021).
nohistochemical analysis of 51 simple bone cysts, includ- With regard to terminology, a number of names have
ing 20 from the mandible. They found that 12 (23.5%) been used for this lesion, including static bone cavity, static
contained deposits of hypocellular eosinophilic fibrin-like bone cyst, static bone defect, idiopathic bone cavity or cyst,
latent bone cavity or cyst, lingual mandibular bone defect
material. Immunohistochemistry demonstrated that in all
cases this material was negative for fibrin, but showed or depression, and mandibular concavity. However, the
expression of collagens I or III and decorin. most widely used term pays homage to Stafne, and most
authors call it the Stafne bone cavity.
Treatment
Clinical Features
Simple bone cysts are usually treated as part of the diagnos-
tic process. To determine the nature of the radiolucency, the Frequency
lesion is opened to reveal an empty cavity, as described Stafne bone cavity is almost always encountered inciden-
above. The cyst wall is then curetted causing haemorrhage tally during radiological examination for another purpose.
into the cavity, and in the vast majority of cases this results It is rarely classified as a jaw cyst and is not usually included
in uneventful healing. It is presumed that granulation tis- in reports of jaw lesions from pathology departments.
sue and eventually new bone proliferate and replace the Overall the condition is not uncommon and has been
haemorrhage caused as a result of the surgery. In their sys- found as an incidental finding in about one in every 1000
tematic review, Chrcanovic and Gomez (2019c) found that radiographs.
99.2% of all cases had been treated by surgical access only Philipsen et al. (2002) reported 69 new cases from Japan that
(32.7%) or by surgical access and curettage (66.5%). had been found on examination of 42 600 consecutive radio-
Recurrence is unusual, but many papers have reported graphs, giving a frequency of 0.16%. Others have reported
lesions that persist, and often do not differentiate between similar frequencies. Oikarinen and Julku (1974) examined
recurrence and persistence. Overall, however, Chrcanovic 10 000 orthopantomograms and found 10 examples, a fre-
and Gomez (2019c) found that of 691 lesions that had been quency of 0.1%. MacDonald and Yu (2020) reviewed 6252 con-
followed up, only 32 (4.6%) persisted during a follow-up secutive radiographs and found 3 cases (0.05%) and Assaf et al.
(2014) found 11 (0.08%) cases on review of 14 005 radiographs.
period of between 1 and 216 months.
Age
Stafne Bone Cavity The cavities are found in adults with an average age of
between 50 and 60 years (Stafne 1942; Schneider
The Stafne bone cavity is not a cyst, but it appears as an et al. 2014; Hisatomi et al. 2019; Aps et al. 2020; Morita
intraosseous cystic lesion on a plain radiograph. Nor can it et al. 2021). About 65% of all cases are encountered in the
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282 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
fifth and sixth decades. The age range has been shown to
be 11–87 years. No cases have ever been reported in an
et al. 2002).
Sex
About 85% of cases occur in males (male : female ratio of
about 6 : 1). Philipsen et al. (2002) reviewed 316 clinical
Site
Stafne bone cavities are only found in the mandible and the
vast majority are located on the lingual aspect, at the angle
of the mandible below the inferior dental (ID) canal
(Figures 17.4 and 17.5). However, cases are occasionally
seen in the anterior mandible, and very rarely in the ramus.
Philipsen et al. (2002) found that of 316 clinical cases, 270
(85.4%) were located on the lingual aspect of the posterior
mandible, 40 (12.7%) on the lingual aspect of the anterior
mandible, and 6 (1.9%) on the lingual aspect of the ramus.
Among the 267 archaeological or museum specimens that
they reviewed, they found a similar distribution (85.0%,
12.0%, and 1.5%, respectively), but also found a report of a
single case on the buccal aspect of the ascending ramus
(Shields 2000). Figure 17.5 Stafne bone cavity. A plain radiograph shows a
Posterior cases are always located below the ID canal and well-demarcated and corticated oval radiolucency. The cavity is
below the inferior dental canal and impinges onto the lower
are associated with the submandibular gland below the
border of the mandible. Source: Courtesy of Dr Hilton Mirels.
mylohyoid muscle in the submandibular space. Anterior
cases, however, are associated with the sublingual gland
Radiological Features
and are located above the mylohyoid muscle. A more
detailed analysis of the location of the cavities and the rela- On plain radiographs the cavities appear as a round or oval
tionship to the teeth and ID canal is discussed later (see cystic radiolucency that is well demarcated, often with a
‘Radiological Features’). characteristic ‘punched-out’ appearance (Figure 17.5).
plaints. However, a small number of cases in the posterior yses by CT or direct observation it can be seen that the cav-
mandible may disrupt the continuity of the lower border ity is an indentation in the lingual aspect of the mandible
and an indentation may be felt if the region is palpated (Figures 17.4 and 17.6). Mann (1992) studied 10 cases in
extraorally (Stafne 1942). Hisatomi et al. (2019) showed dried bone archaeological specimens and prepared moulds
that 17% of cases caused a discontinuity of the lower using silicone impression material. He found that the max-
border of the mandible, but they did not report on clinical
imum dimensions ranged from 7 to 14 mm and that 7 of the
findings or whether these cases were detectable on 10 cases had a ‘mushroom’ shape, in that the internal
palpation. dimensions were greater than the opening. This feature is
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283
5%
30%
10%
35% 10%
10%
lingual aspect of the mandible. Note that the internal superior– within the mandible. Source: Data from Aps et al. (2020), rounded
inferior dimension is greater than than the opening. to the nearest 5% for clarity and to aid recall.
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284 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
investigation, but because they are simple radiolucencies, a ●● The cavity is an indentation on the lingual aspect of
definitive diagnosis cannot be made on plain radiographs the mandible
alone. In early studies, the finding of a radiolucency, ●● Not uncommon: frequency is about 1 in every 1000
presumed to represent an intraosseous cystic lesion, led to
radiographs (0.1%)
surgical intervention, whereupon the surgeon would find ●● Predominantly found in males (85%)
an empty cavity that was continuous with the soft tissues at ●● Average age about 55 years. Peak in fifth and
the lingual aspect of the mandible. This would confirm the sixth decades
diagnosis, but had involved an unnecessary surgical ●● Symptomless and found as an incidental finding on
procedure. radiographs
The majority of lesions are well-demarcated radiolucen-
●● Well-demarcated, usually corticated radiolucency
cies in the molar/angle region of the mandible and are ●● More than 80% are found in the posterior mandible
located below the ID canal. This appearance is typical of ●● Always below the ID canal
the Stafne bone cavity and excludes a lesion of odontogenic ●● About 12% are found in the anterior mandible, above
origin, since these are located in the tooth-bearing alveolar
the mylohyoid muscle
bone above the ID canal. When such a radiolucency is seen ●● Rarely found (<5.0%) in the ramus
incidentally on a routine radiograph, the diagnosis should ●● They contain lobules of normal salivary gland
be confirmed by CT or MRI. Schneider et al. (2014) pre-
sented an algorithm for diagnosis of the Stafne bone cavity,
in which they suggest that a unilocular radiolucency in the proposal seems extremely unlikely given that the cavities
posterior mandible with corticated margins and below the are never seen in children and have a peak age of presenta-
ID canal is diagnostic. If these four criterion are not met, tion in the fifth and sixth decades. It is now generally
then CBCT or MRI can be used to confirm the diagnosis by accepted that the defect is acquired as a result of resorption
demonstrating a lingual opening or presence of soft tissue of the bone due to pressure from a lobe of the submandibu-
in the cavity. Schneider et al. state that surgical exploration lar or sublingual glands. This is supported by the observa-
can be avoided in almost all cases by careful consideration tion that the cavities almost always contain salivary tissue.
of the radiological findings. If such a mechanism were to apply, then it would be
If the radiolucency does prove to be intraosseous, then a expected that the cavities might grow over time. This has
bone lesion is the likely diagnosis. A central giant cell been shown by Friedrich et al. (2020), who reported and
lesion is one of the most commonly encountered non-
illustrated four cases that gradually increased in size over
odontogenic radiolucent lesions of the jaws, but even these periods ranging from 1 to 12 years. These authors also pro-
are rare at this site (Chrcanovic et al. 2018). Other possibili- posed that very early lesions might manifest only as depres-
ties include tumour metastases, ossifying fibroma, osteoma, sions of the lingual cortex that would not be visible on
or osteoblastoma, but all are uncommon and some will radiological examination. This is supported by direct exam-
show a mixed radiolucent/radiopaque lesion. Note that the ination of dried mandibles from museum or archaeological
simple bone cyst arises above the ID canal and should not collections that show a higher incidence of bone defects
be confused with a posteriorly located Stafne bone cavity. than seen in clinical cases detected on radiology (Harvey
Anterior bone cavities are more problematic because they and Noble 1968; Philipsen et al. 2002). It has also been
arise in the tooth-bearing areas (above the mylohyoid mus-
shown that larger lesions are seen in older individuals
cle) and occasionally may be located at the apex of a tooth. (Oikarinen and Julku 1974).
For anterior radiolucencies, therefore, a provisional diagno- Although pressure resorption is regarded as the most
sis of an odontogenic cyst is most likely to be the first likely cause, there have been no explanations as to how this
consideration and a final diagnosis will depend on further
might happen. Some have proposed that it may be associ-
imaging, but will often necessitate surgical intervention. ated with vascular changes resulting in increased pressure
from the facial artery that results in cavitation into which
salivary tissue might proliferate (Lello and Makek 1985).
Pathogenesis
There is very little evidence for this, however, and it does
Early papers suggested that the bone defect was congenital not explain cavities at sites away from vascular areas.
and of developmental origin, caused by entrapment or pro- The ‘glandular hypothesis’ is therefore the most widely
trusion of salivary tissue into the lingual aspect of the man- accepted. Philipsen et al. (2002) suggested that inflamma-
dible during ossification (Fordyce 1956). However, this tion and fibrosis in a salivary gland might increase the
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r 285
Focal
Osteoporotic Bone
Ma
row Defec
pressure on the mandibular cortex. This theory also seems made by careful radiological examination alone (Schneider
unlikely. If it were the case that an altered texture of the et al. 2014; see ‘Radiological Features’) and surgical inter-
gland was necessary, then it might be expected that bone vention is rarely needed. Although most cavities remain
cavities would be associated with salivary gland tumours, static, progression has been reported, especially in younger
and might be more commonly encountered in the ramus individuals (Friedrich et al. 2020). Periodic radiological
adjacent to the parotid gland, where tumours are most follow-up may be advisable until it can be confirmed that
common. Nevertheless, local resorption over time is still the defect is static.
the most accepted theory and is given credence by the
observation that the surface of the bone in the depth of the
defects is irregular and shows pitting consistent with osteo- Focal Osteoporotic Bone
treatment is needed. In most cases the diagnosis can be encountered in the fourth to sixth decades.
Table 17.3 Age, sex, and site distribution of focal osteoporotic bone marrow defect from the two largest case series and the review
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286 Pseudocysts of the Jaws: Simple Bone Cyst and Stafne Bone Cavity
The vast majority of cases have been reported as symp- Shankland and Bouquot (2004) discussed the possible
tomless and have been diagnosed after a chance finding pathogenic mechanisms and proposed that the defect may
during radiological examination for other reasons. represent an early manifestation of osteoporosis and bone
However, Shankland and Bouquot (2004) noted that 57% of marrow oedema, associated with ischaemia and a local
their 100 patients reported tenderness on palpation, and malfunction of blood flow. They suggest that local trauma
Makek and Lello (1986) found that 25% of their patients may be one factor that could initiate these changes.
had experienced pain.
Histopathology
Radiological Features When explored surgically, the cavities contain soft tissue
The focal osteoporotic bone marrow defect is a round or with small amounts of residual bone. In most cases histo-
oval cystic radiolucency that may be well demarcated, logical examination shows normal haematopoietic marrow
but only infrequently shows evidence of cortication. The with varying amounts of fat. Barker et al. (1974) examined
degree of radiolucency is variable and some cases may the histology of 181 defects and found that normal marrow
be difficult to visualise on a standard plain radiograph. occupied more than half the contents in 61.3% of cases. In
Cases in the mandible are always located in the alveolar 38 (21.0%) cases the contents were almost entirely fat.
bone above the ID canal. Schneider et al. (1988) exam- Shankland and Bouquot (2004) found that all cases con-
ined 20 cases and found corticated margins in only 2 tained viable trabeculae of bone, with marrow that was
(10.0%). They also found that in all cases, the normal essentially normal, although 79% was fatty and only 21%
bony trabeculae were still visible and in 55% there were contained red (haematopoietic) elements. They also
areas of radiopacity. Barker et al. (1974) reported that showed that 55% of cases showed evidence of inflamma-
most cases had ill- defined borders and occasionally had
tion and 51% had small focal areas of ischaemia or necrosis.
a ‘moth-eaten’ appearance. They illustrated a number of
tion of the contents of the cavity is necessary to exclude 24–84 years) and 66.6% were female. Cases were distrib-
known pathological entities and to confirm the diagnosis. uted equally between the mandible and maxilla, but the
most common sites were the mandibular molar region
(32.3% of cases) or the maxillary canine region (18.5%). The
Pathogenesis
authors regarded NICO as a localised form of chronic
The cause of these defects is not known, but the most com- osteomyelitis of the jaws, and believed that it is a form of
pelling explanation is of an accumulation of hyperplastic ischaemic osteonecrosis similar to that associated with
marrow in an area of bone healing. This would account for bone pain in other parts of the skeleton, especially the
the fact that the defects are often found at a site of a previ- femur or humerus (Bouquot and McMahon 2003).
ous tooth extraction. Other theories have suggested an Although Bouquot et al. (1992) introduced the term NICO,
association with anaemias, where there is an increased they were not the first to note an association between facial
demand for haematopoiesis, or persistence of haematopoi- neuralgia and bone cavities. In their paper, they recorded
etic marrow into adulthood. almost 2000 cases that had been reported between 1976
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y Bo Cys 287
Aneur
smal
ne
and 1991, and credited Ratner et al. (1979) with the first of long bones, especially the femur, tibia, humerus, and verte-
definitive description. brae. Only about 1.5% arise in the jaw bones.
More recently, members of a German research group Aneurysmal bone cysts, including jaw lesions, have been
have described similar lesions that they have called FDOJ divided into primary and secondary types, where the sec-
(Lechner et al. 2017, 2019, 2020). They first described 24 ondary type arises in association with other lesions, most
patients with bone cavities associated with systemic immu- often fibro-osseous or giant cell lesions (Arora et al. 2014).
nological disorders or facial pain (Lechner et al. 2017). In In the previous edition of this book and in the 2005 WHO
this and a later study, they demonstrated that the bone classification of head and neck tumours (Barnes
cavities showed chronic inflammation and fatty necrosis et al. 2005), the aneurysmal bone cyst was defined as pri-
and were almost always aseptic and associated with mary or secondary and it was thought that most lesions
increased expression of the pro-inflammatory chemokine
were reactive in nature. It is now known that this concept
CCL5 (RANTES; Lechner et al. 2019). The lesions were is wrong and outdated.
similar to the cavities described by Bouquot et al. (1992) Aneurysmal bone cyst has been shown to be a neoplasm
and the authors suggested that NICO and FDOJ are the associated with rearrangements of the USP6 gene with a
same lesion. In a later paper, Lechner et al. (2020) stated wide range of fusion partners (Oliveira et al. 2004, 2005;
that FDOJ is also synonymous with osteoporotic bone mar- Oliveira and Chou 2014; Warren et al. 2017; Šekoranja
row defect. Since the authors have provided few details et al. 2020). Specifically, primary aneurysmal bone cysts
about the clinical context of this lesion, further research is are neoplasms that show USP6 gene rearrangements, but
needed to establish the relationship between osteoporotic so-called secondary aneurysmal bone cysts do not show
bone marrow defects and these reported osteonecrotic USP6 rearrangements and should be regarded as secondary
cavities. changes in pre-existing lesions (Oliveira et al. 2004; Arora
In the absence of further research, these lesions have et al. 2014; Flanagan and Speight 2014). True neoplastic
never been widely accepted and, shamefully, the original aneurysmal bone cyst does occur in the jaw bones, but very
authors who described NICO have been the subject of ridi- few cases have yet been reported (Oliveira et al. 2004;
cule, personal abuse, and litigation (Bouquot and Brooks et al. 2019; McMullen et al. 2019; Cleven et al. 2020).
McMahon 2003). A number of authors have reviewed the In the fourth (2017) and fifth (2022) editions of the WHO
controversy around NICO (Zuniga 2000; Sciubba 2009) classification of head and neck tumours, the aneurysmal
and have suggested that further research and clinical trials bone cyst is defined as a cystic or multicystic, osteolytic
are needed. neoplasm associated with blood-filled spaces lined by
can arise at any site, but have a predilection for the metaphysis confusion with rare but truly neoplastic lesions.
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