Open Journal of Stomatology, 2011, 1, 50-54 OJST
doi:10.4236/ojst.2011.12009 Published Online June 2011 (http://www.SciRP.org/journal/OJST/).
Published Online June 2011 in SciRes. http://www.scirp.org/journal/OJST
Maxillofacial sarcomas: a ugandan epidemiological survey
Kamulegeya Adriane
Oral Maxillofacial Surgery Unit Department of Dentistry, College of Health Sciences, Mulago Hospital, Makerere University,
Kampala, Uganda.
Received 27 April 2011; revised 3 June 2011, accepted 15 June 2011.
ABSTRACT
We reviewed the case notes of 203 patients who were
treated for sarcomas of the oral and maxillofacial re-
gion over a period of 5 years. There were 98 male cases
(mean age 31.4 ± 12.8 years) and 105 female cases
(mean age 29.3 ± 10.4). Kaposi’s sarcoma accounted
for 82.8% cases, and rhabdomyosarcoma 6.9% fol-
lowed by osteosarcoma and chondrosarcoma at 2.5%
each. Except for Kaposi’s sarcoma, surgery in combi-
nation with radiotherapy and/or chemothe- rapy was
the main stay of treatment. Survival data was not
available for most of our patients.
Keywords: Maxillofacial Sarcomas, Kaposi’s Sarcoma,
Maxillofacial Tumors, Head and Neck Tumors
1. INTRODUCTION
Sarcomas of the head and neck region are rare mali-
gnancies often without a clear etiology. They present
with variable symptoms usually dependent on the af-
fected anatomical structure. Symptoms range from pain-
less masses, hearing loss, vertigo, tinnitus, to facial pa-
ralysis. Masses that affect the oral cavity can induce den-
tal pain and, loosening of teeth. When they affect vital
structures of the neck they may cause dysphagia, hoarse-
ness, and even dyspnea [1]. Diagnosis is based on histo-
logical evaluation highly dependent on expert and ex-
perienced pathologists who at times need to employ spe-
cial immunohistochemical staining techniques to confirm
the diagnosis and when in doubt outside opinion is
sought. Both these avenues may not readily be avail- able
in developing countries therefore reliance on the consen-
sus of a local panel of pathologist for co nfirmation is the
norm [2].
Generally oral maxillofacial malignan cies contribute a
small percentage to the overall incidence of this disease
entity [3,4]. Sarcomas are even rarer contributing 1% to
15% of maxillofacial tumors [1,5-7].
Pathologic classification has been reported as critical
to the ulti mate treatment and prognosis of head and neck
sarcomas. Different studies have reported Kaposis’ sar-
coma, osteosarcoma, rhabdomyosarcoma, malignan t fib-
rous histiocytoma, and angiosarcoma as the most com-
mon types that occur in the head and neck region [8-10].
However, the HIV pandemic has changed the overall
incidence and prevalence of sarcomas in sub Saharan
Africa due to an increase in Kaposi’s sarcoma [11]. In
recent times an increase in availability of highly active
retroviral therapy has been reported to reduce Kaposis’
sarcoma incidence among HIV/AIDS patients [12]. De-
cline in Kaposi’s sarcoma has been reported in Tanzania
with an increase in antiretroviral therapy availability
[13]. We didn’t find a report on head and neck sarcomas
in Uganda therefore we felt the need to carry out a ret-
rospective survey o f prevalence of this en tity in the max-
illofacial region.
2. MATERIALS AND METHODS
Patient data were obtained from a retrospective search of
medical records at the Maxillofacial Unit, Mulago hos-
pital, a national referral and Teaching Hospital of Make-
rere University College of Health Sciences seen from
January 2006 to December 2010. Records of cases of hi-
stopathologically diagnosed as sarcomas were selected
and patient files traced. The histological type, gender,
age at initial diagnosis, presenting complaint, site of
tumor and treatment were retrieved. Comparison with
published data was done using simple students T test (p
0.05) with the help of Medcalc.
3. RESULTS
3.1. Prevalence and Histopathological Types
There were 2084 cases biopsied at the oral and maxillo-
facial unit within the study period. Out of which 203
(9.7%) were sarcomas. Eleven histological types of sar-
coma were found from the retrieved data. Kaposis’ sar-
coma was the most common lesion followed by rhabdo-
myosarcoma then osteosarcoma and chondrosarcoma
(Table 1).

K. Adriane / Open Journal of Stomatology 1 (2011) 50-54 51
Table 1. Gender and histopathological distribution of sarcoma.
Sex
Neoplasm Female Male No. (%)
Kaposi’s sarcoma 87 81 168 (82.8)
Rhabdomyosarcoma 5 4 9 (4.4)
Osteosarcoma 3 3 6 (2.9)
Embryonal rhabdomyosarcoma 3 2 5 (2.5)
Chondrosarcoma 2 3 5 (2.5)
Hemagio pericytoma 1 3 4 (1.9)
Malignant fibrous hystiocytoma 0 2 2 (1)
Myxoid liposarcoma 1 0 1 (0.5)
Fibro sarcoma 1 0 1 (0.5)
Dermato fibrosarcoma 1 0 1 (0.5)
Reticular cell sarcoma 1 0 1 (0.5)
Total 105 98 203 (100)
3.2. Sex and Age Distribution
There was no gender preponderance, with a male to fe-
male ratio of 1:1.1 (Table 1). The age range was 1 to 76
years (mean 30.3 ± 11.6 years, median and mode were
both at 30 years) in Table 2. The third and fourth decade
equally dominated at 32% each.
3.3. Tumor Site Distribution
There were more soft tissue sarcomas in the maxillofa-
cial region than hard tissue (5.4%). Vascular tumors as a
group (Kaposi’s sarcoma and hemagiopericytoma) were
the commonest followed by the muscular group (rhab-
domyosarcoma and embryonal rhabdomyosarcoma). Ka-
posi’s sarcoma mainly was seen on the palate but the
oral cavity as a whole accounted for the second highest
reported site. The mandible was the most commonly
affected jaw bone by both rhabdomyosarcoma, osteosar-
coma and chondrosarcoma (Table 3).
3.4. Clinical Features and Duration of
Symptoms
As per Table 4, a combinatio n of p ain and swellin g were
the commonest chief presenting complaints of the pa-
tients (43.6%). This was followed by swelling alone.
Seven (6.4%) cases all of Kaposi’s sarcoma came be-
cause they were referred. Patients presented within 1 -
205 weeks of onset of symptoms. However, the 205
weeks case was an outlier diagnosed as a fibrosarcoma.
The others that presented after long durations were Ka-
posi’s sarcoma cases. Two cases of chondrosarcoma were
initially diagnosed as pleomorphic adenoma and chon-
droma hence they were treated conservatively with sur-
gery only to fail loco-regionally. We don’t routinely
stage but we always have chest x-rays and abdominal
ultrasound scans done before treatment except for Ka-
posi’s sarcoma. In all cases they were no significant
findings.
3.5. Treatment and Follow-Up
The treatment modalities differed as per the diagnosis.
Kaposis sarcoma was mainly managed by chemotherapy
and highly active antiretrov iral therap y (HAART). Th ere
is an ongoing blinded study treatment Kaposi’s sarcoma
so we couldn’t establish who received what. However,
we were unable to establish HIV/AIDS management
situation for 41.2% of the patients with Kaposi’s sarcoma.
Out of those whose HIV treat m e nt hi story was retri evabl e,
17.7% were on HAART at time of diagnosis, while
11.3% were on cotrimazole (septrin) prophylaxis and an
equal percentage reported not being on any treat- ment.
1.0% had stopped taking HAART due to perceived in-
crease in sickness. Otherwise non Kaposi’s sarcoma
cases were managed by a combination of surgery fol-
lowed by chemotherapy and/or radiotherapy (Table 5).
Specific drugs used, dosages and number of courses
were determined by a consultant oncologist whereas
those who underwent radiotherapy were handled by
consultant radiotherapy oncologists. One of the patients
with chondrosarcoma of the mandible had total man-
dibulectomy followed by radiotherapy but died after
Table 2. Distribution of sarcomas according to age groups.
Age (years)
Neoplasm 0 - 9 10 - 1920 - 2930 - 3940 - 4950 - 5960+ Mean
Kaposi’s sarcoma 7 9 58 58 28 5 1 30.9 ± 10. 0
Rhabdomyosarcoma 2 2 2 1 1 - 1 26.6 ± 21.9
Osteosarcoma 1 - 1 2 2 - - 32.3 ± 13.7
Embryonal rhabdomyosarcoma - 3 2 - - - - 8.0 ± 6.4
Chondrosarcoma - - 2 2 1 - - 30.8 ± 7.7
Hemagio pericytoma 1 - - 1 1 1 - 32.5 ± 19.1
Malignant fibrous h ystiocytoma - - - 1 1 - - 38.5 ± 2.1
Myxoid liposarcoma - 1 - - - - - 27*
Fibro sarcoma - - - - - - 6 62*
Dermato fibrosarcoma - 1 - - - - - 14*
Reticular cell sarcoma - - 1 - - - - 23*
Total 11 16 66 65 34 6 8
*Denotes age of the lone patient in the gro up; #2 Patients did not have their age specified.

K. Adriane / Open Journal of Stomatology 1 (2011) 50-54 52
Table 3. Distribution of sarcomas as per affected anatomical site.
Site (%) Total %
Neoplasm Palate Whole body TongueMandibleMaxillaOral
cavity Parotidlip Retro molar area Row total
Vascular
Kaposi’s sarcoma 22.6 14.3 13.1 2.3 1.2 19.1 0.6 1.8 0.6 75.6
Hemagio pericytoma - - - - - - 25.0 50.0 - 70.0
Muscular
Rhabdomyosarcoma - - - 44.4 11.1 33.3 - - - 100
Embryonal
rhabdomyosarcoma - - - 60.0 40.0 - - - - 100
Bony
Osteosarcoma - - - 83.3 16.4 - - - - 100
Cartilagenous
Chondrosarcoma - - - 60.0 40.0 - - - - 100
Fibrous
Malignant fibrous
hystiocytoma 100 - - - - - - - - 100
Fibro sarcoma - - - - - 100 - 100 - 100
Dermato fibrosarcoma - - - - - - - - - 100
Fatty
Myxoid liposarcoma - - - - - 100 - - - 100
Unclear Histiogenesis
Reticular cell sarcoma - - - - - - 100 - - 100
The missing row percentages are for those cases whose site was not established.
Table 4. Distribution of sarcomas as per chief complaint/reason for coming to hospital.
Neoplasm Chief complaint
of
p
atient N
)
Duration
(
weeks
)
Swelling Referral Pain+
swelling Bleeding PainDifficult eating Swelling+ bleeding Pain+ Numbness Wound
Kaposi’s sarcoma 30 7 37 5 3 5 2 1 2 1 - 156
Rhabdomyosarcoma 1 - 2 - - - - - - 1 - 24
Osteosarcoma 1 - 3 - - - - 1 - 6 - 22
Embryonal
rhabdomyosarcoma 1 - - - - - - - - 4*
Chondrosarcoma 1 - 3 - - - - - - 3 - 60
Hemagio pericytoma - - 1 - - - - - - 3*
Malignant fibrous
hystiocytoma - - 1 - - - - - - 7*
Myxoid liposarcoma 1 - - - - - - - - 8*
Fibro sarcoma - - 1 - - - - - - 250
Dermato fibrosarcoma 1 - - - - - - - - 4
Reticular cell sarcoma - - - - - - - - - 1*
Total 36 7 48 5 3 5 2 2 2 1 - 205
*Data available for one case or onl y one case was found.

K. Adriane / Open Journal of Stomatology 1 (2011) 50-54 53
Table 5. Distribution of sarcomas as per treatment modality.
Treatment (% of cases)
Neoplasm Chemo and or HAARTSurgRadioSurg RadioSurg+ChemoSurg Chemo Radio Not established
Kaposi’s sarcoma 73.3 - - - 0.6 - 26.2
Rhabdomyosarcoma - - - 55.6 - - 44.4
Osteosarcoma 16.7 - - 16.7 50.1 16.7 -
Embryonal rhabdomyosarcoma 20.0 - - - - - 80.0
Chondrosarcoma - - - - 20.0 80.0 -
Hemagio pericytoma 50.0 50.0- - - - -
Malignant fibrous hystiocytoma 50.0 - - - - - 50.0
Myxoid liposarcoma 100 - - - - - -
Fibro sarcoma 100 - - - - - -
Dermato fibrosarcoma 100 - - - - - -
Reticular cell sarcoma - - - - - - 100
Those under not established are cases whose files couldn’t be traced.
the second dose of chemotherapy. We were only able to
follow a few patients due to distances involved.
4. DISCUSSION
Little is known of the epidemiology of oral maxillofacial
sarcomas in a Ugandan population. The data analyzed
here differed a bit with the general epidemiology of the
disease probably due to the high number of Kaposi’s
sarcoma cases (Table 1) seen at our centre [1,3,7,8].
Although a study f rom Ken ya [8 ] reported Kaposi’s sar-
coma as the commonest sarcoma, their percentage was
not as high as that seen in this stu dy (P 0.001 chi 72.19).
On the other hand, a Nigerian [7] study reported only
one case therefore either other centers have most of the
biopsies for Kaposi’s sarcoma diagnosis taken by other
specialties or the oral facial component of the disease is
the main manifestation among our population. This has
been alluded to by Ziegler et al. [14]. All Kaposi’s sar-
coma patients were HIV positive with up to 17.7% being
on HAART at the time of diagnosis. This was not a sur-
prise given the fact that increased incidence of HIV/
AIDS associated Kaposis sarcoma has been reported by
other authors [15]. We could not ascertain the exact
treatment that our Kaposi’s sarcoma patients got due to
an ongoing research randomizing them in HAART
group and other arms. However, the main stay of treat-
ment in the cou n tr y is HAA RT ex c ep t in a fe w cas e s th at
are given chemotherapy as well. It has become standard
practice in this era of HAART to expect a resolution of
the lesions with improving immunity and increase in
CD4 cell count [16,17].
Rhabdomyosarcoma was the second commonest sar-
coma in our series followed by osteosarcoma and then
chondrosarcoma. The data in this study is slightly dif-
ferent in that respect when compared to other reports
that rank rhabdomyosarcoma second to fourth in preva-
lence [1,7,8,18,19]. Chondrosarcoma is reported to be
rare in the head and neck region [1,7,18] therefore the
prevalence in this study was rather high but not statisti-
cally significantly different from that reported by a Ke-
nyan study that picked just 3 cases over a ten year period
(P = 0.12 chi 2.38). A Nigerian [7] report had only 2
cases of chondrosarcoma that were loco-regional failures
from other centre in a twenty year study. It is hard to tell
if the prevalence in Uganda is higher or it’s because our
unit is the only fu nctional maxillofacial uni t in the coun-
try at present. Unfortunately the risk factors for sarco-
mas have not been well addressed and it was not any
different in this study therefore we could not establish if
there are any particular factors to explain this high oc-
currence.
The majority of our patients presented with swelling
and pain as the chief complaints. This differed with re-
ports from developed country [18,20]. Sarcomas are
known to present as painless swelling therefore pain on
set is either because of advancement or supra infection.
In this study painful swellings were the commonest chief
complaints. This probably was due to delayed reporting
and supra infection. In fact one of the chondrosarcoma
patients decided to try traditional healers un til pain set in
before she came back for surgery.
In this study surgical intervention for non Kaposi’s
sarcoma disease was the primary treatment modality just
as reported by other authors [1,7,8,18]. Surgery was ac-
companied by some form of adjuvant therapy with either
radiation and/or chemotherapy. However, the few whose
outcome was established, the results were discouraging
as many died within a short time.

K. Adriane / Open Journal of Stomatology 1 (2011) 50-54 54
Future research should focus on survival of patients
and the factors that affect treatment outcomes. A mecha-
nism for long term follow up has to be implemented.
With increasing mobile teleph one penetration, a window
of opportunity is available to aid us in follow up.
5. ACKNOWLEDGEMENTS
The authors acknowledge the contributions of the pathologist at Mak-
erere University department of pathology, radio oncologists of Mulago
hospital and medical oncologists of Uganda cancer institute in the
diagnosis and treatment of the patien ts.
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