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2. Primary intraosseous carcinoma is a very rare but well
recognized entity.
First described by Loos in 1913 and named as intra- alveolar
epidermoid carcinoma by Wills in 1948.
Term primary intraosseous carcinoma (PIOC) was suggested
by Pindborg et al in 1972.
The term primary intraosseous odontogenic carcinoma
(PIOC) has been primarily used to describe a squamous cell
carcinoma within the jaws as de novo.
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3. According to WHO classification, PIOC is an odontogenic
carcinoma defined as “ a squamous cell carcinoma arising
within the jaw, having no initial connection with the oral
mucosa, and presumably developing from residues of the
odontogenic epithelium.”
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4. Waldron & Mustoe (1989), classification of odontogenic
carcinomas -
Type 1. Arising ex odontogenic cyst
Type 2. Arising ex ameloblastoma
A. well differentiated (malignant ameloblastoma)
B. poorly differeentiated (ameloblastic carcinoma)
Type 3. Primary Intraosseous carcinoma arising de nova (PIOC)
A. non-keratinizing
B. Keratinizing
Type 4. Intraosseous mucoepidermoid carcinoma
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5. CLINICAL FEATURES –
Occurs in adult patients in sixth to seventh decade of life.
Occurs only in the jaw bones & predominantly in the
posterior mandible.
Up to 2/3rd
of odontogenic carcinoma arise due to its
malignant transformation within Odontogenic cyst or
tumour while PIOC arising de novo is relatively rare.
Affects men more than women with ratio about 2.2 : 1
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6. The most common symptoms is pain and swelling.
Facial asymmetry.
Overlying mucosa or skin intact.
Sensory disturbance like paresthesia and numbness.
Cotical plate expansion.
Regional lymphadenopathy.
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9. 60 yr. old female visited the department of Oral Medicine &
Radiology, Guru Nanak Dev Dental College and
Research Institute, Sunam with the chief complaint of
pain & swelling over the right side of face since 8-9
months.
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10. History of presenting illness revealed that there was small swelling
over the right side of face 8-9 months back which gradually
increased in size from last 1 month to attain the present size.
H/o pain in the same region since 1 month which was dull, aching,
continuous and non- radiating in nature. Aggravated while
chewing and opening the mouth and relieved temporarily by
taking medication.
Difficulty in eating food.
Paresthesia of lower lip since 3-4 months.
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11. Excessive salivation
No history of trauma, sinus formation or pus discharge.
Patient denied the history of any recent development of
ulcer or other soft tissue lesion in the oral cavity.
No history of fever or decrease in weight in recent past
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12. Medical history revealed that -
Patient is known hypertensive since 10 years and not taking
medications regularly.
Never hospitalized in the past.
Not allergy to any drug was reported.
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13. Patient underwent extraction of 47 from local dentist 1 month back
because of mobility of tooth . After that pain and progressive
swelling of the extraction wound persisted.
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14. Family history was not contributary
Personal history –
Married
Vegetarian diet
No history of tobacco or betal nut chewing .
Oral hygiene bad , brushes once in 15 – 20 days.
Mild to moderate bleeding from gums .
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15. General examination –
Gait – no abnormality detected
Height – 5’3’’
Weight – 64 kg
Average built and moderately nourished.
Mild pallor, no cyanosis and clubbing of fingers
Except blood pressure (146/ 90mm of Hg) all vitals signs were in
normal limits.
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16. Extra oral examination revealed a single
oval swelling over the right, lower 1/3rd
of face leading to gross facial
asymmetry.
.ANT – POT. EXTENSION – starts
from angle of mouth and goes upto
posterior border of ramus of mandible.
SUPERIO- INFERIOR EXTENSION
– 1.5cm below the ala-tragus line and
inferiorly it goes beyond the lower
border of the mandible.
Skin over the swelling was stretched with
no change of color. www.indiandentalacademy.com
17. On palpation , swelling was tender , hard in consistency
without localized increased in temperature
Step deformity was appreciated on the right lower border
of body of mandible.
Two right submandibular lymph nodes both measuring not
more than 1.5 cms in size, hard in consistency, tender on
palpation, one lymph node was fixed to underlying tissues
and other was freely mobile in all directions.
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18. Intraoral examination –
Single oval swelling present over the
right alveolar ridge in the region of
45 46 47
Examination revealed an intact
Overlying normal -appearing mucosa
except for 47 region were
Extraction socket was present.
Medially swelling starts from floor of
mouth, Laterally it obliterates the
right vestibule w.r.t 45 46 47.
On palpation swelling was tender ,
hard in consistency with expansion
of buccal & lingual cortical plates.www.indiandentalacademy.com
19. Teeth missing – 17 24 32 41 42 43 44 45
46 47 48
Dental caries – 18 (tender on percussion) 26 36
Mobile – grade I (16 23 26 )
Grade II ( 37)
Generalized attrition of all the teeth.
Severe gingival recession.
Bleeding on probing present
Both hard and soft deposits present.
Vestibule obliterated w.r.t 46 47.www.indiandentalacademy.com
20. Based on history and clinical examination provisional diagnosis of
tumour of mandible was made and differential diagnosis of-
Residual cyst
Ameloblastoma
Osteosarcoma
Metastatic carcinoma
Chronic osteomylitis
Primary Intra-osseous carcinoma
Carcinoma arising in odontogenic cyst were considered.www.indiandentalacademy.com
25. Right lateral oblique view of mandible showing large ill-defined radiolucency
with infiltrative borders involving body of mandible.
Discontinue inferior cortex of body of mandible suggestive of pathological
fracture.
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26. Large non- homogenous radiolucency involving the right side of body of
mandible and ascending ramus with specks of radio-opacities.
Ill defined margins with infiltrative borders.
Discontinuity of inferior cortical margin of right side of jaw at angle region
suggestive of pathological fracture. Erosion of cortical margins of dental canal.
Generalized horizontal bone loss.
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29. Incisional biopsy of the lesion was performed and the
specimen was send for histopathology examination.
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30. The histopathological report of multiple grayish white soft tissue
mass was suggestive of WELL DIFFERENTIATED –
SQUAMOUS CELL CARCINOMA
Eosinophilic cytoplasm
Keratin pearls
N/C increased
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32. As confirmed of malignancy, patient was referred to oral and
Maxillofacial Surgery department for further management.
Commando surgery was performed with right side functional neck
dissection and hemimandibulectomy. The resected specimen was
send for histopathological examination
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33. Intact mucosa lining
Foreign body giant
cellsKeratin pearls
H/P report was suggestive of Well- differentiated Primary
intra-osseous carcinoma of mandible.
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34. Based upon the history, clinical features,
radiographic features and histopathological examination final
diagnosis of Well -differentiated keratinizing Primary
Intra-osseous carcinoma (de nova) of mandible was made.
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36. Till now 97 case of Primary intra osseous carcinoma (de nova) have
been reported.
Tumour is believed to arise from Odontogenic epithelial cell rests.
(Lucas et al )
The common factor may be reactive inflammatory stimulus with or
without a predisposing genetic cofactor, inducing neoplastic formation.
The clinical features were non specific while pain (54.8%) and
sometimes sensory disturbances (16.1%) presented in most of cases.
Diagnosis of PIOC is difficult, partly the initial symptoms are often
thought to be of dental origin. (Mc Gowan RH 1980).
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37. Radiographically there is great variation in the appearance of
borders ranging from well defined smoothly contoured to ill-
defined infiltrative that makes them indistinguishable from other
benign or malignant tumours. (Nolan R 1976)
Kaffe et al have proposed that an important feature of PIOC is
the presence of indistinct margins without sclerotic outline.
PIOC type 3 shows equal tendency toward keratinizing and non-
keratinizing types .
Prognosis of PIOC is quite poor, and emphasis should be given to
early diagnosis so that suitable treatment can be given at the
earliest opportunity. (2-year survival rate in 40% patients)
(To E H W et al 1991)
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38. CONCLUSION
Diagnosing this very rare and well recognized entity is
challenging task for oral physician because of its close
resemblance to many other benign & malignant lesions both
clinically and radiographically.
Proper diagnosis at an early stage is important for better
prognosis.
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