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Nigerian Journal of Surgical Research
Surgical Sciences Research Society, Zaria and Association of Surgeons of Nigeria
ISSN: 1595-1103
Vol. 8, Num. 3-4, 2006, pp. 135-139
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Untitled Document
Nigerian Journal of Surgical Research, Vol. 8, No. 3-4, Jul-Dec, 2006, pp. 135-139
Oral
and Maxillo-facial soft tissue sarcomas in an Africa population
E T Adebayo , S O Ajike , A. Adebola and E. O Adekeye
Department of Dental surgery 44 Nigerian Army Reference
Hospital, P.M.B. 2413, Kaduna 80000144,Maxillofacial Unit,Ahmadu Bello University Teaching Hospital Kaduna, Nigeria.
Request for Reprints to Dr.
Ezekiel Taiwo Adebayo,Department of Dental Surgery, 44 Nigerian Army Reference
Hospital,P.M.B. 2413, Kaduna 80000144, E-mail: taiwo_adebayo@yahoo.com
Code Number: sr06032
Abstract
Background:Soft tissue sarcomas (STS) are not
common and account for less than 1% of all malignancies in the maxillofacial
region. These tumours are under reported in Africa South of the Sahara in
current literature.This report is a review of our
experience with STS over a 23 year period at a busy Tertiary Oral care centre
in Kaduna, Northern Nigeria.
Methods: Patients presenting to the
Maxillofacial Unit, of the Ahmadu Bello University Teaching Hospital, Kaduna, with oral and maxillofacial malignancies between the years 1977 and 1999 were retrospectively
studied. Soft tissue sarcomas were studied. Demographic features, clinical presentation,
treatment options , outcome and histologic variants were studied.
Results: There were 38(<1%) soft tissue sarcomas out of 415
oral and maxillofacial malignancies recorded within the study period. Male to female
ratio was 1.6:1. Age range was 24 days to 60 years ( median 28 years). There
were more adults (79%) than children (21%). There were eleven histologic types but
the more frequent ones were rhabdomyosarcoma (10/38,(), fibrosarcoma (10/38 (26%),
liposarcoma ( 5/38 (13%) and malignant fibrous histiocytoma (5/38(13%) . Site
of primary occurrence was mainly the mandible (32%), palate (21%) and cheek (18%).
Surgery was the mainstay of treatment 28/38 (74%) while 10/38(26%) patients had
no active treatment.
Conclusion: Most patients present late with difficult deforming
tumours. We can blame patients knowledge, Socio-economic
Key words: soft tissue sarcoma, oral, maxillofacial, poverty, ignorance,
treatment, African, management, tumours
Introduction
Soft tissue sarcomas (STS) are solid malignancies
mostly of mesodermal origin whose rarity and variety
present formidable challenges in characterization and
treatment. According to Pisters and Brennan, STS
make up less than 1% of all oral malignancies[1].
Like other tumours, they can occur at any anatomical
site and at any age. The extremities and trunk are
favoured sites for occurrence and only about 5-10% of
STS occur in the head and neck region 1, 2 . Some STS
are of ectodermal origin but all STS are considered as
a group because of the similarities in their clinical
features, natural history, treatment and disease
outcome. Rhabdomyosarcoma is the most common
histologic type of STS in the oral and maxillofacial
region, followed by the malignant fibrous
histiocytoma (MFH) and the fibrosarcoma 2 . Adults
and children are affected and in most reports 80-90%
are adults and 10-20% are children 3. Reports from
Africa on the clinical behaviour of oral and
maxillofacial STS are scanty and are mostly short reports. This study presents oral and maxillofacial soft
tissue sarcomas in a Native African population
Northern Nigeria in the Savanna belt south of the
Sahara desert.
Methods
Medical
records of patients with histopathologically diagnosed malignant oral and
maxillofacial tumours between January 1977 and December 1999 were
retrospectively reviewed at the Maxillofacial Unit, Ahmadu Bello University
Teaching Hospital, Kaduna, Nigeria. The Unit is the leading, best staffed and
equipped center for the management of oro-facial diseases in Northern Nigeria
(estimated population 60 million in 1998). The catchment area of this center is
the 19 states in Northern Nigeria. Patient records examined included case filesoperative
records and histopathology results. Cases of STS were selected from other
malignancies and analysed for age at presentation,
sex, tumour site, clinical features, histopathological type, treatment and
follow up reports. Tumour staging and grading was not done routinely within the
study period.
Results
From January 1977 to December 1999, there were 406
cases of histopathologically diagnosed oral and maxillofacial malignancies of
which 38(<1%) were STS. Mean age of cases of STS was 28.4 ± 15.6years,
median age 28.5 years (range 24days to 60years). 79% of patients were above 18 years
and (21%) were, < 18 years old. Male to female ratio was 1.6:1. Common
oral and maxillofacial sites of STS were mandible (n=12, 32%), palate (n=8, 21%),
cheek (n=7, 18%) and the maxilla (n=6, 16%). There were 11 histologic types of
STS studied (Table 1). More common were rhabdomyosarcoma (n=10, 26%),
fibrosarcoma (n=10, 26%), liposarcoma (n=5, 13%) and MFH (n=5, 13%). The most
frequent treatment option of STS was surgery (n=20, 74%). Some patients had
chemotherapy (n=6, 22%). Six patients defaulted after confirmatory diagnosis
while four tumours were deemed inoperable and patients palliated with
analgesics and terminal care.
Discussion
Many
STS are of mesodermal tissue origin such as rhabdomyosarcoma, fibrosarcoma and
liposarcoma while a few are of neuroectodermal origin such as malignant
schwannoma and Ewing’s sarcoma. STS of the head and neck are quite rare with
only a few reports in English literature [4,5,6]. Some case reports from Africa
are on specific tumours. Sts make up less than 1 % of all malignancies in the
head
and neck region and between 5% and 10% of all body sarcomas 1, 2. In
this 23 years survey at our tertiary oral care center in Nigeria, STS was less
than 1% (38 cases) of all malignancies of the oral and maxillofacial region confirming
its frequently reported rarity. Though no age group is
exempt from sarcomas, maxillofacial STS generally affect the younger age group
less commonly. According to Eeles et al., 7 STS occurs at a median
age of 32 years with 53% of patients being males. Between 80-90% of STS occur
in adults while 10-20% of patients are children3. The median age of
STS occurrence of the 38 cases seen in this study was 28years with fewer less
females than males. Male predilection was also observed by other authors 5,6.
The
most common type of STS differs in different reports. Greagar and colleagues, 8 observed that fibrosarcoma is commonest (27%) followed by malignant schwannoma
(15%),while Weber et al., 2 noted that rhabdomyosarcoma (18%) was more common
followed by followed by MFH and fibrosarcoma. In his report of 164 adult STS
Tran
et al.,9 angiosarcoma (16%) was the commonest followed by fibrosarcoma (12%)
and unclassified sarcomas (11%). Quite recently, Pandey et al., 10 reviewing 22 adult patients from India found unclassified spindle-cell sarcomas
(32%) and MFH and rhabdomyosarcoma (14% each). In our series involving 38 cases,
the foremost STS were rhabdomyosarcoma and fibrosarcoma (26% each) and MFH and
liposarcoma (13% each) Rhabdomyosarcoma is the neoplastic analogue of the embryogenesis
of
skeletal muscle. The rhabdomyoblast recapitulates skeletal muscle embryogenesis
in a highly disorganised manner 11 . The tumour appears to be commoner
among
Caucasians than Negroes causing speculation of a genetic predilection 12 .
The paucity
of reports on head and neck rhabdomyosarcoma fromAfrica makes these conclusions
tenuous. Ten cases of rhabdomyosarcoma seen in this study had a male to female
ratio of 1.5:1. Among the 10 cases in this six had histological subtyping
available; five were embryonal and one alveolar. The cheek was the commonest
site overall (40%) followed by the mandible (20%). Four histologic varieties;
pleomorphic, alveolar, embryonal and botryoid types have been described13.
Among these forms, the embryonal is reportedly commoner in the maxillofacial
region most commonly affecting the hard palate7, 13. Rhabdomyosarcoma
is the most common soft tissue malignancy of the head and neck in children,
14.In the younger adults the tumour is more frequently found affecting the
extremities and trunk. In 110 embryonal rhabdomyosarcomas of the body, Enzinger
and Shiraki 15 found the lesion occurring between 10 and 20 years
of
age (mean age 15years). A similar age range was recorded in three cases whose
subtype was not disclosed by Pandey et al., 6. Patients in this
study were between 24 days and 40years old (mean 14 years). From the five
cases of embryonal subtype in this series, all but one was below 10years of
age. Presenting features of oral and maxillofacial rhabdomyosarcoma was
frequently as swelling (90%), pain (60%) while ulceration of skin or mucosa was
uncommon (20%). These have reported
duration was 1.4 months from. According to Maurer et al., 16 a
combination of surgery, radiation treatment and multichemotherapy could give
dramatic survival
rates in patients with rhabdomyosarcoma. This form of treatment has dramatically
improved the outlook for these patients. Surgery is the mainstay while
radiotherapy and cytotoxic chemotherapy are adjuvants16 Pelliteri
et al., 17 believe that while the influence of the size of head and
neck STS on prognosis is controversial; the possibility of successful en-bloc
resection of tumour is limited in large tumours due to involvement of critical
neurovascular structures. Hence, poor local control and survival would
accompany surgical therapy of large lesions in the head and neck. In this
report, four cases were treated surgically, three with cytotoxic chemotherapy
and one case referred for radiotherapy . Out of the four cases treated
surgically, only that treated by resection could be said to have had optimum
initial treatment (Cases no 9) though no review reports were available.Also,
during this study period, facilities for head and neck radiotherapy were not
available
within a 700km radius of our center while the cost of radical surgery with need
for reconstruction remains unaffordable to many of our patients unlike the
Indian cases reported by Pandey et al., 6 It should be noted that
there is no functional health insurance scheme for most citizens of sub-Saharan
Africa including Nigeria. Despite the gruesome sizes on presentation of many
tumour cases in Nigeria 18, cases of default after diagnosis and
inoperable cases abound. This is due to ignorance of medical problems among
patients and their relatives, poor access to modern health care facilities,
native superstitions forbidding surgical treatment and the social problem of
poverty. Hence, the combination of ignorance, poverty and inadequate medical
infrastructure limit treatment options and survival of STS patients in our
environment. The impact of treatment on survival of the cases seen in this
report could not be assessed, as follow-up review visits was poor in this
study. Stout19 states that 45% of fibrosarcomas occur between the
ages of 20 and 40 years while Dahlin and Ivins , 2.0 found 13 of 114
central bone cases of the lesion originating in the mandible. There were 10
cases of fibrosarcoma seen at Kaduna, Nigeria occurred between 8 and 60 years
of age with 70% below the age of 40years.Among these cases , the mandible was
the commonest site (60%) followed by the palate (20%). Fibrosarcoma grows
rapidly or slowly 13. Duration ranging from 3-72 months was recorded
in this study; most cases (80%) however, presented within 6months. The most
acceptable form of treatment for fibrosarcoma is radical resection. Any form
of
limited excision especially due to its atimes pseudo encapsulation inevitably
leads to recurrence 13,21. Eight cases were managed during the
period of this study especially by surgery (n=6). One case managed surgically
by tumour resection was followed up for 6months with no record of recurrence.
It is noted that this follow-up period is too short to assess recurrence. No
survivor was recorded 5years after treatment of fibrosarcoma of the jaws in the
Netherlands 22.. Even though fat tissue is abundant in the oral
and maxillofacial region, liposarcoma in this region is exceptionally rare.
Between 1958 and1965, only four cases were seen in southern England 23. .
Hudson et al., . 24, reported the first case of
liposarcoma from our center in 1978. This tumour is rare below the age of 30
years and shows a slight male preponderance (55%) according to Enzinger and Winslow, 25 . Table 4 shows 80% of the cases in this study where above 30
years old while the male to female ratio was 1.5:1.
Liposarcoma occurred mostly as cheek swellings (60%),
the rest were in the palate (20%) and maxilla (20%) in .Several histological
sub
classifications of liposarcoma have been described. One classification
describes the cell types as myxoid, round cell, adult and pleomorphic 26. .
It has been noted that majority (71% of 35 cases) of liposarcomas of the
maxillofacial area are of the myxoid cell type, are well differentiated with
good prognosis 27. Among our five cases, 60% were of the myxoid
variety . Surgical excision with or without radiotherapy is used for treating
liposarcoma with prognosis related to several factors especially tumour cell
type 13. Four cases of liposarcoma were treated, two by surgery and
one with chemotherapy. One patient was referred for radiotherapy. No follow-up
record of treated cases was available. MFH is regarded as one of the
commonest STS of later life28 where it affects the musculature of
the limbs. It is rare in the maxillofacial region. In a large series of 200
cases, Blitzer et al., 29 found only six in the head and neck.
According to Pandey et al., 6, all three cases of MFH from a
Regional Cancer Centre in India occurred between 13 and 54 years of age, all
are males. Table 2 shows that 80% of cases of MFH seen at Kaduna, Nigeria, were
above the third decade of life, (mean age 39.4years). All our cases were also
males. This raises the possibility of male predilection for occurrence. The
tumour is believed to occur mostly in the sixth decade of life 21. The
tumour is rare in the oral soft tissues; most are central lesions of the
jawbones30., In the report by Pandey et al., 6, 66% of
cases of MFH were in the jawbones. Four of the five cases in our series
occurred at the jawbones similar to the findings of Pandey et al., 6 Radical local excision is the most useful form of treatment for this lesion 2.1 Malignant schwannoma can occur de novo or less frequently
from pre-existing lesions of neurofibromatosis. While cases occurring de
novo present later in life, those arising secondarily are generally seen in
adolescents and young adults13. . Cases in this report had no
history of malignant schwannoma arising secondarily. The two cases seen were
males aged 46-48years. Any of the oral soft tissues such as the lip, palate,
gingiva or the hard tissues such as the jawbones can be involved in the lesion.
Patients managed in this report presented with swelling, pain and ulceration
of the palate and nostril.. Case no 6 recurred within two months of resection of
tumour. Patient then opted to leave the hospital for home care. Prognosis of
malignant schwannoma varies greatly and factors responsible are tumour
location, degree of differentiation, duration at presentation, and method of
treatment. Tumours secondary to von Recklinghausen`s disease have a worse
outcome compared to primary tumours13. Recurrence following local
excision can be up to 80% with a 5year survival between 15-30% 31. Adjuvant
radiotherapy and chemotherapy do not improve the efficacy of local control and long-term
prognosis,21.. The management of STS requires close collaboration
between an experienced head and neck surgeon, medical oncologist, pathologist,
radiation oncologist and diagnostic radiologist in a comprehensive cancer centre6.
There is a role for the oral/maxillofacial surgeon and prosthodontist in
assessment and follow-up care of some of these cases. The guarantee for optimum
patient treatment is lacking in much of sub-Saharan Africa. Better patient
care would result from the establishment and equipping of regional centres
for the management of malignancies as available in most developed world and Indian
subcontinent. Only by this can our patients escape the devastation form the
relatively uncommon STS
Conclusion
The frequency of STS in Nigeria (1%) is comparable to
that in other parts of the world. However the quality of life of patients and
survival after treatment is not comparable. Better patient care would be
possible with the establishment of regional centers for the management of
malignancies and to benefit from the skilled manpower in Africa. Patient
education should be encouraged to seek treatment early in the course of disease
to reduce the problems of delayed management of malignancies. Patients follow
up should be meticulous and structured to catch early those who recur post
intervention.
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