2015, Volume 31, Number 2, Page(s) 153-157
Gingival Rhabdomyosarcoma in an Adult: A Unique Entity
Priya SAHNİ1, Abhishek SİNGHVİ1, Meghanand T. NAYAK1, Shakti Singh DEORA2
1Department of Oral & Maxillofacial Pathology, Vyas Dental College & Hospital, Jodhpur, RAJASTHAN, INDIA
2Department of Oral & Maxillofacial Surgery, Mathura Das Mathur Hospital, Jodhpur, RAJASTHAN, INDIA
Keywords: Rhabdomyosarcoma, Embryonal, Gingiva, Adult
Rhabdomyosarcoma is a disease that predominantly affects children.
Approximately 40 per cent are located in the head and neck region
but it is rare in the oral cavity.
This article describes an interesting case of an embryonal
rhabdomyosarcoma in a 36-year-old male, involving the mandibular
gingiva. The lesion showed radiolucency with ill-defined margins
that was crossing the midline. The history revealed a similar lesion
six months back at the same site and the lesion had been completely
excised. The biopsy reports confirmed the diagnosis of embryonal
rhabdomyosarcoma after which en-bloc resection of the tumor was
performed with administration of chemotherapy and radiotherapy.
Due to high recurrence rate of rhabdomyosarcomas in adults,
multimodal therapy should be planned for proper care of the patient.
Clinical, radiological, histopathological and management aspects are
Rhabdomyosarcoma (RMS) is a mesenchymal malignant
neoplasm, first described by Weber in 1854, and accounts
for 6% of all the malignancies in children under 15 years
. It exhibits skeletal muscle cells of varying
. Histopathology of RMS is
analogous to myogenesis in the developing embryo,
yielding clues to the biology of these lesions. For this
reason, it is correct to define RMS as a tumor derived from
primitive mesenchyme and exhibiting a profound tendency
towards myogenesis than to define it as a cancer arising
from skeletal muscle4
. This concept was eloquently
expressed by Masson in 1970 who referred to RMS as
“rhabdopoietic sarcomas,” and this explains the observation
that more tumors arise from viscera and axial soft tissues
than from the extremities5
. The head and neck region
and genitourinary tract are the most common sites for
RMS. In the head and neck region the commonly affected sites are the orbit, paranasal sinuses, and the neck6
RMS is rare, and when it occurs it is more frequent in the
The incidence of RMS appears to be higher in the African-
American and Caucasian population than Asians7,8.
Two-third of RMS cases are diagnosed in children 2 to
15 years of age with a mean age of 6 years, and there is a
small male predominance1,2,7. RMS is rare in adults,
accounting for 2 to 5 per cent of adult soft tissue sarcomas
and they show different biological behavior compared to
children with tumors that are more aggressive and have an
overall worse survival rate9,10,11.
Histologically, embryonal RMS is characterized by zones
of loose and dense cellularity, remarkably recapitulate
normal embryonal myogenesis, in which loose primitive
mesenchyme condenses to form nascent muscle12.
In this article, we describe an unusual case of gingival embryonal RMS occurring in an adult where the
diagnosis was made only by means of histopathology and
A 36-year-old male was referred for evaluation of a
symptomatic lesion in the anterior right mandibular
region. The clinical examination of the patient revealed a
painless mass, firm in consistency, measuring about 8x5 cm
on the mandibular right second molar region and crossing
the midline, to involve the mandibular left anterior region.
The borders were ill-defined and the mucosa covering the
lesion was reddish in color with spontaneous bleeding on
palpation (Figure 1
). The patient had noticed a similar
lesion six months back at the present site and had undergone complete excision of the lesion. A histopathological
diagnosis of embryonal RMS was made. However, the
lesion recurred and grew rapidly to the presenting size.
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|Figure 1: Intra-oral examination reveals an irregular mass
involving the right mandibular gingiva and alveolar mucosa with
obliteration of the buccal vestibule.
OPG and CT scan study showed an expansile, osteolytic
lesion involving the right inferior alveolus and body of
the mandible (Figure 2a). An extraosseous soft tissue
component was noticed both anterior and posterior to
the mandible (Figure 2b). Loss of the right mandibular
lateral incisor with loosening of other teeth was noticed in
the right mandibular quadrant (Figure 3). No significant
cervical lymphadenopathy was noticed.
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|Figure 2: A) Computerized tomographic (CT) axial image of the lesion showing irregular destruction of the anterior mandibular bone.
B) CT shows mesio-distal extension of the lesion in the mandibular region.
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|Figure 3: Orthopantomograph image shows loss of right mandibular lateral incisor and loosening of adjacent teeth.
An incisional biopsy was performed and the specimen
was referred for histopathological examination. Under
light microscopic examination, the tumor was observed
to be composed of dense cellular connective tissue stroma.
Cells were round to oval with hyperchromatic nuclei,
frequent mitotic figures and indistinct cytoplasm (Figure
4). The pathology evaluation showed embryonal RMS
that was verified by a immunohistochemistry panel. The
immunohistochemical tests were performed with antibodies
against desmin, myogenin and myoglobin. Among these
markers, desmin and myogenin revealed strong positive
staining whereas immunoreactivity to myoglobin was not
detected (Figure 5, 6).
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|Figure 4: Sections showing round undifferentiated cells with
hyperchromatic nuclei and myxoid area (H&E; x400).
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|Figure 5: Positive immunohistochemical staining for desmin
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|Figure 6: Positive immunohistochemical staining for myogenin
Following the histological diagnosis, en bloc resection of the
tumor with a wide margin of normal tissue was performed
(Figure 7). After removal of the lesion, a pectoralis
myocutaneous graft was placed along with an arch bar.
An excisional biopsy process was performed and revealed
findings similar to the incisional biopsy. Subsequent to
surgery, the patient underwent chemotherapy with a
vincristine, ifosfamide and actinomycin D regime. After three cycles of chemotherapy in 16 weeks the patient was
followed with radiotherapy, which was administered for
four weeks in once daily fractions of 1.8 Gy. To date of this
report, more than 6 months since diagnosis and treatment,
he has no evidence of the disease.
RMS is a rare malignant lesion, more common in children
and with a peak age of incidence between two to six years1,2,7
. The reported patient was 36 years of age and it is
very rare to see RMS at this age.
In the presented case, gingiva and alveolar mucosa of the right body of the mandible and anterior portion of the left mandible were involved. Only a few cases of gingival RMS have been published13. The present report adds to the
literature of adult gingival RMS.
Clinical presentation of RMS is variable and influenced by
site, age and the presence or absence of distant metastases.
In general the primary lesion is a non-tender mass. The
head and neck lesion may cause impairment of hearing,
vision, speech, and swallowing, and respiratory symptoms
may develop14. The clinical features noted in the present
case were facial asymmetry and obliteration of the labial
and buccal vestibule due to the painless mass.
RMS is very rare in adults so the data on clinical presentation
is inadequate. A number of studies have described that these
tumors are more aggressive in adults compared to children
and adolescents9,11,15. This case presented as a rapid
recurrence and growth of lesion due to inadequate surgery.
Radiological examination of the lesion may reveal the size
and extension of the lesion and extent of bone destruction16. Through computerized tomography and panoramic
radiography, we could determine that the lesion affected the
body of the mandible with missing and loosening of teeth.
Horn and Enterline classified RMS histologically in four
subtypes: embryonal, botryoid, alveolar, and pleomorphic.
Botryoid RMS abuts an epithelial surface, with condensation
of tumor cells in the immediate subepithelial zone. The
alveolar variant has aggregates of round to oval neoplastic
cells, separated by irregularly shaped fibrous trabeculae
forming ill-defined alveolar spaces. The pleomorphic or
classical variant is the adult type seen in adults over 40
years of age12. The histopathological appearance of this
case was of the embryonal subtype. Embryonal RMS has a
favorable prognosis when compared to the other subtypes17.
Immunohistochemically, cytoplasm-specific markers for
RMS are myoglobin, desmin, muscle-specific actin while
nucleus-specific markers are Myo D and myogenin. Desmin
and muscle-specific actin are the most accepted makers and
are used commonly in the diagnosis of RMS. Muscle transcription
factors Myo D and myogenin are the most sensitive
markers for immunohistochemical diagnosis of RMS.
Both of these markers are expressed at high levels in RMS,
even when cells are showing poor differentiation12. In
the presented case, we used a panel of immunohistochemical
markers and found positive expression of desmin and
RMS in adults is an aggressive tumor and has a higher incidence
of recurrence. The best possible clinical outcome is therefore achieved via a multimodal approach. Sophisticated
surgical techniques with reconstruction are used
as the primary modality9. RMS is a radiocurable lesion
but results in radiation-induced secondary tumors3. In
addition, multiagent chemotherapy, including vincristine,
adriamycin, cyclophosphamide, and actinomycin D reduces
micro metastases and malignant cell population,
thus favoring long-term survival with fewer complications18. Due to the high recurrence rate and association with
distant metastases of RMS, a high-quality treatment plan is
Sarcomas are extremely rare in the oral cavity. However
in our day-to-day practice we may come across the rarest
of the lesions with an unusual clinical presentation.
Therefore, each and every lesion of the oral cavity should
be examined thoroughly and all diagnostic techniques such
as radiography, histopathology and immunohistochemistry
should be considered to achieve a correct diagnosis. Lesions
on the gingiva and the alveolar area may be due to RMS
or any other sarcomas and these pathologies should be
considered in the differential diagnosis.
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