Restorative Dentistry

Enhanced CPD DO C

Angharad Truman

Claire Forbes-Haley

The Dental Effects of Head

and Neck Rhabdomyosarcoma
Treatment: A Case Series
Abstract: Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm of skeletal muscle origin. Approximately 40% of cases of RMS are
sited in the head and neck region and nearly 65% of cases are diagnosed in children less than six years of age. Treatment for RMS involves a
multimodality approach including surgery, chemotherapy and radiotherapy. When children are exposed to such treatments there are often
associated long-term adverse effect which may affect the patients’ dento-alveolar growth and development. Three cases are described
highlighting these adverse effects and the treatments used for oral rehabilitation.
CPD/Clinical Relevance: To inform readers of the long-term effects of rhabdomyosarcoma treatment on the dento-alveolar development.
Dent Update 2022; 49: 639–644

Rhabdomyosarcoma (RMS) is a malignant  Parameningeal: including the nose, cases), and pleomorphic (5% cases).
soft tissue neoplasm of skeletal muscle origin. nasopharynx, paranasal sinuses, middle Embryonic cases mainly occur in those
It is reportedly the third most common ear, mastoid, infratemporal fossa and aged up to 10 years, alveolar cases tend
extracranial solid tumour of childhood after pterygopalatine fossa; to present at around 10–25 years and
Wilms’ tumour and neuroblastoma.1–4 RMS  Non-parameningeal: including the scalp, pleomorphic RMS present most commonly
is the most frequent soft tissue sarcoma orbit, parotid gland, oral cavity, oropharynx, in those aged 40+ years.1 In the head
encountered and accounts for approximately and larynx.3 RMS of the oral cavity accounts and neck region, embryonal RMS is most
6% of all malignancies in children under 15 for 10–12% of all head and neck RMS prevalent. The cells of embryonal RMS
years of age.3 Approximately 65% of cases are cases.3 The most common site in the oral resemble that of the developing muscle
diagnosed in children younger than 6 years cells of a 6–8-week-old fetus.1
cavity is the tongue, followed by the soft
of age.2,5 There is a slight predilection for RMS appears to be sporadic in nature
palate, hard palate, and buccal mucosa.6,7
disease in males, with a male-to-female ratio and no definitive aetiological factors have
Jaw involvement is extremely rare.6
of 1.3–1.5.2 been reported. However, there is some
Other commonly reported areas are the
The most common site for RMS,
genitourinary tract, retroperitoneum and evidence suggesting gene abnormalities
accounting for approximately 40% of cases,
the extremities. may have a role.8 Furthermore, familial
is the head and neck region.1 RMS of the
syndromes such as neurofibromatosis,
head and neck are anatomically divided into Three basic types of RMS are described:
and the Li–Fraumeni syndrome have been
two categories: embryonal (60% cases), alveolar (20–30%
associated with RMS.2,9
Clinical manifestations of RMS in the
head and neck region may vary from a fast-
Angharad Truman, BDS (Hons), MFDS, M Pros, FDS (Rest Dent) RCSEd, PGCME, growing and extensive facial swelling to a
FHEA, Consultant in Restorative Dentistry, Somerset NHS Foundation Trust.
small cutaneous nodule. Symptoms may
Claire Forbes‑Haley, BDS, MJDF RCS, FGDP UK, FDS Res Dent RCS, Consultant
include pain, ptosis, trismus, paraesthesia,
Restorative Dentistry. University Hospitals Plymouth NHS Trust.
facial palsy and nasal discharge; however,
email: angharad.truman@plymouth.ac.uk
RMS may also be symptomless.3,10,11

September 2022 DentalUpdate 639

Restorative Dentistry
Of consideration are possible differential
diagnoses of RMS, which may include
Ewing sarcoma, Wilms’ tumour, lymphoma,
neuroblastoma, alveolar soft-part sarcoma,
fibrosarcoma and leiomyosarcoma.1
Once appropriately diagnosed,
treatment for RMS involves a multimodality
approach with surgery, chemotherapy and
radiotherapy. When children are exposed to
such treatments, there are often associated
long-term adverse effects that may affect
the patients dento-alveolar growth and
development, and may adversely affect the
patient’s quality of life. It is often reported
that the younger the age of treatment and
the greater the dose of radiotherapy and/
or chemotherapy, the increased severity of
side effects.12
Some of the side effects of radiation
and chemotherapy can occur immediately
after treatment, such as mucositis, increased
risk of candidiasis and oral infection, or can
be delayed, such as delayed eruption of
teeth, hypodontia/regional oligodontia and
retarded tooth and bone development. This
disruption to tooth development can affect
odontogenesis leading to enamel, dentine
and cementum malformation affecting the
crowns and roots of the teeth.1,5–7,12–15 Not
only may this have functional and aesthetic
issues for the patient, for the restorative
dentist, it poses an increased challenge
for restoration and a possibly decreased
predictability for any treatment provided.
In one study by Estilo et al,5 bony
hypoplasia or facial asymmetry was the
most common late presenting clinical
finding after radiotherapy treatment
for RMS. Radiographically, evidence of
underdevelopment of the mandible and root
formation disturbance, such as incomplete
root development, root stunting/tapering
and in severe cases root agenesis, were most
often discovered.5
Further effects may include tooth
discolouration, hyposalivation/xerostomia,
increased risk of caries, trismus, altered/loss
of taste and scar-tissue formation.1,5,12,13,15 It is
well established that osteoradionecrosis can
occur following radiotherapy to a region, and
this must be monitored for and taken into
consideration when planning any treatment.
These side effects can make oral rehabilitation
by the restorative dentist challenging. This
case series looks at three patients who were
successfully treated for RMS in the head and
neck region. It highlights the dento-alveolar
issues that can arise and discusses the
restorative treatment approaches that may
be used for oral rehabilitation.
640 DentalUpdate September 2022
Figure 1. An OPG was taken to assess the dentition of Patient A. Grade 1 quality. Radiographic findings:
bone levels were good. No obvious carious cavities; however, reduced radiodensity evident that was
consistent with the hypomineralized URE and LRE. An unerupted LR6 and a horizontally impacted
UR5. Possible fusion of the LRE and LR5. Generalized root shortening with almost root agenesis of
the permanent molar teeth. No obvious apical pathology. No obvious crypts for the developing third
permanent molars.
Case A teeth or teeth tender to percussion. A
diagnosis of generalized severe hypodontia,
Patient A was 17 years old at the time of
generalized microdontia, failure of complete
presentation to the adult dental health
root development, hypomineralized URE
unit. She had no complaints with regards to
and LRE, impaction of UR5 and failure of
symptoms, function or the aesthetics of her
eruption LR6.
dentition. She had an awareness of retained
primary teeth and that the roots of her teeth
had been affected by her RMS treatment. Case B
Medically, she was diagnosed with Patient B was 16-years old at the time of
retropharyngeal embryonal RMS at the age presentation to the adult dental health unit.
of 3 years. The treatment undertaken at He was concerned with the aesthetics of a
that time was intravenous chemotherapy. palatally placed lateral incisor, the mobility
She also reported hypopituitarism as a of his maxillary central incisors and the
consequence of RMS treatment, with future of his dentition due to the stunted
the patient taking pituitary hormone root development.
replacement injections. Extra-oral Medically, Patient B had been diagnosed
examination highlighted the patient had with metastatic embryonal nasopharyngeal
mild facial asymmetry that caused her no RMS at the age of 4 years. The treatment
concern. Intra-orally, oral hygiene was good undertaken at that time was radiotherapy
and the gingival tissues healthy. Generalized (41.4 Gy) followed by chemotherapy.
microdontia was evident. Retained primary He also reported hypopituitarism and
teeth included a hypomineralized URE, LRE hypothyroidism as a consequence of RMS
and an infra-occluded URE. The LRE and LR5 treatment, with the patient taking growth
appeared to be fused coronally with the LR5 hormones and thyroxine.
having severe microdontia. The patient had Extra-oral examination highlighted the
congenitally missing UR8, UR7, UL7, UL8, LL8 patient had facial asymmetry, which caused
LL7, LR7, LR8. some concern to the patient, and he was
Radiographic examination was assessing the option of further facial surgery
undertaken (Figure 1). This highlighted in an attempt to correct this issue. Intra-
an unerupted LR6, possible fusion of the orally, the oral hygiene was fair. Generalized
LRE and LR5 and a horizontally impacted microdontia was evident. Missing teeth
UR5. A lack of complete root formation included the UR3 and UL3. UR2, UL2 and
was evident affecting the entire dentition. UL7 were grade I mobile, and UR1 and UL1
Special investigations revealed no mobile grade II mobile.
 Restorative Dentistry

including conventional bridge work,

resin-retained bridge work, and implant-
supported prostheses. However, for these
patients, there is difficulty providing
conventional fixed restorative treatment,
ie bridge work, because the lack of root
development leaves no suitable abutment
teeth. This would lead to the need for
either a removable option, which many
young patients do not wish to have, or an
implant-retained prosthesis, which, at the
patients’ stage of growth and development,
is contraindicated due to the risk of further
growth leading to submerging implants.16
Both patients consented to a
conservative treatment plan. Oral hygiene
Figure 2. An OPG was taken to assess the dentition of Patient B. Grade 1 quality. Radiographic findings: instruction (OHI) and caries preventive
bone levels were good. Unerupted UR3 and UL3. No obvious carious cavities. Generalized root advice was given to help maintain the
agenesis of the maxillary and mandibular teeth with root shortening of the mandibular incisors. No dentition in good oral health, along
obvious apical pathology. with the requirement for regular review.
No active treatment was undertaken at
that time. Should the teeth fail, simple
removable mucosal-borne appliances
without clasping would be provided in the
first instance.

Case C
Patient C was first referred to the adult
dental health clinic at 18 years of age in
2012. She complained of a recent loss of
a front tooth, which her general dental
practitioner had splinted to her adjacent
teeth as a temporary measure because
she did not wish to have a removable
prosthesis. She was also aware of a
mobile symptomatic tooth (UL2). She had
awareness that the roots of her teeth had
been affected by her RMS treatment.
Medically, Patient C had been
diagnosed with right parameningeal
Figure 3. An OPG was taken to assess the dentition of Patient C. Grade 1 quality. Radiographic (right ear, skull and pterigoids) embryonal
findings: Bone levels were good. Splinted retained UR2 evident with a wire and composite splint UR3,
RMS at the age of 3 years. The treatment
UR1 and UL1. No obvious carious cavities. An unerupted UR7, UL7, partially erupted LR7 and a disto-
undertaken at that time was intravenous
horizontally impacted LL8. Generalized root agenesis of the maxillary teeth, and root shortening of the
mandibular incisors. No obvious apical pathology.
chemotherapy and radiotherapy to
the middle cranial fossa (45 Gy) and
pituitary gland (25 Gy). She also reported,
as a consequence of RMS treatment,
Radiographic examination was undertaken RMS sites were treated in childhood. hypopituitarism, facial hypoplasia of the
(Figure 2). This highlighted an unerupted Treatment options were discussed using right side, sensorineural hearing loss in
LR6, possible fusion of the LRE and LR5, and a a multidisciplinary approach (MDT), with the right ear and psychological issues.
horizontally impacted UR5. A lack of complete orthodontics to re-align the dentition and Current medication included growth
root formation was evident affecting the bring the impacted UR5 into the arch. hormone injections.
entire dentition. A diagnosis of generalized However, owing to shortened roots, this was Extra-oral examination highlighted
microdontia, generalized root agenesis and deemed to be of high risk and could lead to the patient had facial asymmetry and a
unerupted UR3 and UL3 was made. tooth loss. decreased development of the mid-third
The risk of tooth loss for these patients of the face. Intra-orally, the oral hygiene
is high. Options to replace missing teeth was good, and the gingival tissues healthy.
Treatment for Cases A and B often include removable options, such The UR2 was splinted to the UR3, UR1 and
The treatment options available to A and B as partial or complete dentures, which UL1. The UL2 was grade 2 mobile. Teeth
where quite similar even though different may be implant retained, or fixed options missing included the UR7, UL7, LL7, LR7.

September 2022 DentalUpdate 641

Restorative Dentistry
Figure 4. A CBCT of the maxilla was taken to assess the current bone volume for treatment planning for Patient C. Grade 1 quality. CBCT findings: in general,
it is evident that there is a lack of bone volume height and width available for implant placement without additional bone grafting. The view is centred on
the maxillary incisor region highlighting this lack of bone volume.
Radiographic examination was
undertaken (Figure 3). This highlighted
an unerupted UR7, UL7 and LR7. The LL8
had a disto-horizontal impaction. A lack of
complete root formation was evident, with
root agenesis affecting the entire maxillary
dentition and root shortening of the lower
incisors. A diagnosis of root agenesis of the
maxillary dentition and partial root formation
lower incisors, disto-horizontal impaction of
LR8 and acquired tooth loss was given.
Treatment for Case C
The very poor long-term prognosis of the
entire maxillary dentition was discussed
with the patient in a sympathetic manner.
The hopeless prognosis of the UL2 was
also addressed. Treatment options were
discussed with the patient as described for
patients A and B. For Patient C, the maxilla
had been included in the radiotherapy field,
and the risk of osteoradionecrosis following
implant placement was discussed at length.
As discussed in Cases A and B, conventional
tooth replacement options were limited
because of the poor abutment teeth. Patient
C desired a fixed prosthetic option rather
than a removable replacement.
In the first instance the splinted UR2 was
removed, and the UL2 extracted. These were
replaced with a resin-retained bridge of
double abutment with cantilever design off
642 DentalUpdate September 2022
the central incisors. Although the UR1 and
UL1 were of poor long-term prognosis, the
patient accepted that their use as abutments
risked their loss, but this was preferable to
wearing a removable prosthesis.
Regular review with the GDP was
required and yearly reviews at Bristol Dental
hospital were undertaken for an element of
shared care to ensure the good dental health
of the patient.
From 2012 to 2016, no further active
treatment was required for the patient.
However, in 2016, Patient C presented with
grade III mobile maxillary canine teeth, and
had exfoliated her posterior mandibular
molars. The resin-retained bridge work
replacing the upper lateral incisors was
sound. As previously, treatment options
to replace teeth were re-discussed and
resin-retained bridge work was provided
as a single cantilever design from the first
premolars to replace the canines.
During 2017 the patient presented with
further pain and mobility associated with
her maxillary premolar teeth on both sides.
An immediate partial denture was created.
At this point, Patient C was 23 years old
and her dentition entailed the maxillary
central incisors being used as resin-bridge
abutments to replace the lateral incisors and
first permanent molars only.
Further discussions were undertaken
regarding the options of a removable
prosthesis or implant-retained prosthesis. A
lack of sufficient bone volume in the maxilla
was confirmed with cone beam CT scans
(Figure 4). Using the multidisciplinary team
and liaising closely with the oncologist to
discuss radiotherapy fields and the risks
of undertaking surgical procedures in the
maxilla, the patient consented to a treatment
plan of extraction of the UR6, UR1, UL1,
UL6 and an immediate complete denture
was fitted. Following this, onlay rib bone
grafting and bilateral sinus lift procedures
were undertaken under general anaesthetic
(Figure 5).
In mid-2018, at the age of 24 years,
Patient C received six dental implants in the
central incisors, canines and premolar region
prior to the construction of fixed implant-
retained bridges (Figure 6).
Discussion
Treatment for RMS includes surgery,
radiotherapy and chemotherapy. Radiation
can directly and indirectly affect the
developing dentition, directly inhibiting
mitotic activity of odontoblasts. Children
are more susceptible to the effects owing
to the large quantity of rapidly dividing
pre-secretory odontoblasts. Indirectly,
radiation affects amelogenesis by inducing
formation of ‘osteodentine’ instead of normal
dentine. This osteodentine has reduced
 Restorative Dentistry

Figure 5. A CBCT of the maxilla was taken after the onlay rib bone graft and bilateral sinus lift to assess the bone volume for treatment planning for Patient
C. Grade 1 quality. CBCT findings: in general, it is evident that there is sufficient bone volume for short implant placement. The view is centred on the
maxillary incisor region.

Figure 6. Intra-oral peri-apical radiographs were taken to assess the post cementation of the three cement-retained implant-supported bridges and provide
baseline bone records for Patient C. Grade 1 quality. Radiographic findings: bone levels were good, no obvious pathology associated with implants. The
cemented restorations were seating well and there was no evidence of cement below the gingival margin.

phosphorylated phosphoprotein, inhibiting
nucleation of enamel crystals leading to
deficient enamel mineralization.12
Chemotherapy treatment consists of
antineoplastic medications that suppress
the autoimmune system and/or directly
kill cells. Most antineoplastic agents affect
normal cells, as well as tumour cells.
Antineoplastic agents, such as vincristine,
actinomycin, and cyclophosphamide, can
interfere with odontogenesis.1 The delayed
effects of such treatment can be delayed
eruption of teeth, hypodontia and retarded
tooth and bone development. Not only may
this have functional and aesthetic issues
for the patient, for the restorative dentist it
poses an increased challenge to restore, and
potentially a less predictable outcome for
any treatment provided.
It is of utmost importance that
sympathetic discussions are undertaken with
the patient, and that the patient is informed
of the risks of any treatment provided,
including the potential loss of the entire
dentition in severely affected cases.
Issues with prosthetic replacement must
be discussed. Often the teeth available
provide poor abutments, and their use
may lead to an increased rate of loss of
the abutment teeth. A multidisciplinary
approach is often required, with the team
including restorative dentists, orthodontists
and maxillofacial surgeons. Continued
communication with the patients’
medical team, such as the oncologists,
endocrinologists, cardiologists, plastic
surgeon and psychologists, is also required
to ensure a holistic approach is taken in the
best interest of the patient.
Conclusion
This article highlights the effects of RMS
treatment on dento-alveolar growth and
development, and the issues this creates for
the patient aesthetically and functionally.
The issues surrounding restorative treatment
are discussed, particularly the compromised
approach to be undertaken in the first

September 2022 DentalUpdate 643

Restorative Dentistry

instance and prior to possible loss of most of the dentition. Age is a

limiting factor for rehabilitation, and a removable prosthesis is likely

Absolutely Amazing! to be required in the short to long term. Implant-retained prostheses

are not always an option for these patients owing to the high risks of
surgery on irradiated bone and also the general lack of bone volume
because of the reduced development of the dento-alveolar complex.
There is often a requirement for bone grafting procedures. For these
cases, tailored and careful MDT planning is required to consider the best
management to gain the safest and most effective solution.

Compliance with Ethical Standards

Conflict of Interest: The authors declare that they have no conflict
of interest.
Informed Consent: Informed consent was obtained from all individual
participants included in the article.

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644 DentalUpdate September 2022