CASE REPORT ON RHABDOMYOSARCOMA AT DHAKA DENTAL COLLEGE AN HOSPITAL
Rhabdomyosarcoma is an aggressive and highly malignant form of cancer that develops from skeletal muscle cells that have failed to fully differentiate. It is generally considered to be a disease of childhood, as the vast majority of cases occur in those below the age of 18,with an annual incidence of 4.5 cases per 1 million children, making it the third most prevalent extracranial solid tumor of childhood after neuroblastoma and Wilms tumor.
The first line of treatment for rhabdomyosarcoma typically lasts between five and 12 months. We tailor our treatments to child’s risk group and type of disease. In general, patients in the low-risk group need less therapy than those in the high-risk group, and alveolar rhabdomyosarcoma may need more-intensive therapy than embryonal rhabdomyosarcoma.
Rhabdomyosarcoma treatment usually includes chemotherapy to shrink the tumor and prevent new tumors from forming. Radiation therapy usually begins several months after chemotherapy starts. It’s another effective way of shrinking the tumor. A few patients have surgery after chemotherapy to take out the entire tumor.
Often we do a PET scan after chemotherapy and radiation to see how the tumor responded to the treatments. Study shows that this practice can help predict the chance that the rhabdomyosarcoma will be permanently controlled by chemotherapy and radiation therapy.
We present a case of embryonal rhabdomyosarcoma of anterior mandible of a 11 months old child, with occasional bleeding for 45 days. Incisional biopsy and histopathology revealed it was a case of small round cell tumor with embryonal rhabdomyosarcoma. The pathological tissue was excised from the ant. mandible, the wound site was healing with good facial aesthetics.
Dr. Nusrat Fahmida Trisha
Intern Doctor
Department of Oral and Maxillofacial Surgery
Dhaka Dental College and Hospital
Mirpur-14, Dhaka
2. INTRODUCTON
• Rhabdomyosarcoma is a tumor of skeletal muscle origin, is the most
common soft tissue sarcomas in childhood and adolescence accounting for
4-8% of all malignant lesions in those younger than 15 years of age.
• Genetic translocation producing new protein that interrupts skeletal muscle
differentiation
• Oral and maxillofacial region : 44% of all RMS, occurs mostly in the children
and teenagers
• 2% of RMS are present at birth
• 5% occur in individuals younger than 1 year
• Tumors have a peak incidence at the age 4 years and another at age 17
years
• HISTOPATHOLOGICAL REPORT: The diagnosis is established by means of a deep incisional
biopsy within the center of the tumor.
EMBRYONAL RMS ALVOLAR RMS PLEOMORPHIC RMS
• RADIOLOGY: Plain radiographs, a CT Scan, an MRI Scan is required to understand the tumor’s
size, spatial anatomic relationship and extend of bony destruction.
• The CT or MRI Scan should include detailed cuts of the base of the scull to assess potential
invasion into this area and include the entire skull to rule out brain extension or metastasis
DIAGNOSIS
Treatment Protocol
LOCAL
EXCISIONCHEMOTHERAPY RADIOTHERAPY
• Recently the treatment is done by chemotherapy followed by surgery
3. CASE REPORT
• Baby girl Ilma, 4 years old child, hailing from New market was referred to the
department of oral & maxillofacial surgery of Dhaka Dental College Hospital
in 30th October 2017, with a chief complaint of painless swelling in the
mandibular right posterior region of 25 days duration.
• The swelling was initially small size and quickly increased ( progressive
growth ). She had no past history of trauma and systemic disease. According
to the statement of patient’s attendance, there was no sign of pain,
sensation disorder, pus discharge and bleeding in the involved area.
• Extra oral examination showed facial asymmetry and a diffuse soft tissue to
firm swelling in the posterior region on the mandible with smooth surface, ill
defined margin, consistency soft but near angle of mouth swelling is hard
and fixed to underlying structure.
• Intraoral examination showed irregular ulcerated swelling, well defined
margin, consistency firm to hard, fixed to underlying structure.
Measurement of lesion : 4cm X 4cm
• She had no systemic diseases.
• Regional Lymph node:
right submandibular lymph node is palpable,
size almost 1.5cm, mobile.
• Incisional biopsy : soft tissue taken from right
side of the body, angle and ramus of the mandible. sections showed fragmented
pieces of tissue showing a neoplasm, composed of cells having irregular nuclear
membrane. Background is myxoid.
OPG
• The paneromic xray revealed radiolucent lesion with ill
defined borders.
• The lesion was extending anterioposteriorly, from the retro
molar area to the ramus of the mandible,
• Inferiorly up to the inferior alveolar canal of the mandible
• Superiorly to the alveolar ridge
INVESTIGATION
Fig : OPG showing bone destruction
at right side of mandible
4. CT shows facial asymmetry cortical perforation
• The computed tomography ( CT
scan ) showed the large aggressive
mass lesion ( 47.5 mm X 39.2 mm )
noted in right lower jaw involving
right ramus and adjacent part of
body of mandible
• The lesion having large soft tissue
component surrounding the
involved part of mandible
• Gross bony destruction noted in
involved part of mandible
• Medially the mass involved right
buccal region & laterally the mass
merged with muscles of
mastication
CT SCAN
5. DISCUSSION
• The latest treatment protocol is Chemotherapy
followed by surgical excision.
• So the patient was reffered to Oncology department
of Dhaka Medical College Hospital for better
management on 23rd November 2017
• Radiation therapy usually begins several months after
chemotherapy starts. It’s another effective way of
shrinking the tumor
• Often we do a PET scan after chemotherapy and
radiation is done to see how the tumor responded to
the treatments. Study shows that this practice can
help predict the chance that the rhabdomyosarcoma
will be permanently controlled by chemotherapy and
radiation therapy.
CONCLUSION
We conclude that in children, any swelling
should be carefully examined and treatment
outcomes should be regularly followed up.
High degree of suspicion, early diagnosis and a
multidisciplinary treatment approach would
be of great importance in such cases.
REFERENCES
• Oral and Maxillo-facial
pathology
Robert E. Marx,DDS
Diane Stern,DDS
• Oral Pathology
REGEZI
SCIUBBA
JORDAN
• The Intergroup Rhabdomyosarcoma Study Group (IRSG)
• Medical Wikipedia