Familial multilocular cystic disease of the jaws

 # Which of the following is also known as familial multilocular cystic disease of the jaws?

A. Osteopetrosis

B. Cleidocranial dysostosis

C. Hemifacial microsomia

D. Cherubism

The correct answer is D. Cherubism.

Cherubism, a non-neoplastic hereditary bone lesion that is histologically similar to central giant cell granuloma, affects the jaws of children bilaterally and symmetrically, usually producing the so-called cherubic look (Fig. 17-13). The disease was first described in 1933 by Jones, who called it familial multilocular disease of the jaws. The term ‘cherubism’, was introduced by Jones and others to describe the clinical appearance of affected patients. According to the WHO classification, cherubism belongs to a group of non-neoplastic bone lesions affecting only the jaws. It is a rare, benign condition with autosomal dominant inheritance, and it is one of the very few genetically determined osteoclastic lesions in the human body. It appears to have 100% penetrance in males and only 50–70% penetrance in females. There is great variation in the clinical expression.

Reference: Shafer’s Textbook of ORAL PATHOLOGY, 7th Edition, Page No: 715

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Cause of osteogenesis imperfecta is:

# Cause of osteogenesis imperfecta is:

A. Defect in type I collagen

B. Defect in type II collagen

C. Defect in maturation process

D. Defect in calcification process

The correct answer is A. Defect in type I collagen.

Osteogenesis imperfecta (OI) is a serious disease, the molecular pathogenesis of which is being elucidated and it bears a superficial relatedness to dentinogenesis imperfecta, a milder condition affecting mesodermal tissues. It is a condition resulting from abnormality in the type I collagen, which most commonly manifests as fragility of bones. Although osteogenesis imperfecta is generally recognized as representing a hereditary autosomal dominant characteristic, autosomal recessive and nonhereditary types also occur.

Reference: Shafer’s Textbook of ORAL PATHOLOGY, 7th Edition, Page No: 699

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Dysplastic changes, carcinoma in situ or even early invasive cancer is seen in:

# Which of the following clinical lesions is most likely to harbor dysplastic changes, carcinoma in situ or even early invasive cancer?

A. Erythroplakia

B. Lichen planus

C. Leukoplakia

D. Stomatitis nicotiana

The correct answer is A. Erythroplakia.

Erythroplakia

(Erythroplasia of Queyrat)

Whilst leukoplakia is a relatively common condition, erythroplakia is rare. In contrast to leukoplakia, erythroplakia is almost always associated with premalignant changes histologically and is, therefore, the most important precancerous lesion.

The high rate of premalignant and malignant changes noticed in erythroplakia is true for all clinical varieties of this lesion and not solely a feature of speckled erythroplakia. Different studies have demonstrated that 80–90% of erythroplakias are histopathologically either severe epithelial dysplasia, carcinoma in situ, or invasive carcinoma.  

 Reference: Shafer’s Textbook of ORAL PATHOLOGY, 7th Edition, Page No: 95

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Most sensitive to radiation induced cancer?

 # Which of the following is the most sensitive to radiation induced cancer?

A. Nerves

B. Female breast

C. Thyroid

D. Skin

The correct answer is B. Female breast.

Susceptibility of Different Organs to Radiation-Induced Cancer

High: Colon, Stomach, Lung, Bone marrow (leukemia), female breast

Intermediate: Bladder, Liver, Thyroid

Low: Bone surface, Brain, Salivary glands, skin

Reference: Oral Radiology Principles and Interpretation, 7th Edition, Page no 26

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