Neoplasia Practice Questions

Q: What is the origin of the Pindborg tumour?

Stratum intermedium. **Explanation:** The **Pindborg tumor**, medically known as **Calcifying Epithelial Odontogenic Tumor (CEOT)**, is a rare, benign but locally aggressive odontogenic neoplasm. **Why Option B is correct:** The tumor cells in CEOT histologically resemble the cells of the **stratum intermedium** of the enamel organ. This is supported by the presence of high alkaline phosphatase activity and the characteristic polyhedral epithelial cells with prominent intercellular bridges (desmosomes) seen on histopathology. **Analysis of Incorrect Options:** * **Option A (Reduced Enamel Epithelium):** This is the origin of the **Dentigerous cyst** and is also associated with the Adenomatoid Odontogenic Tumor (AOT), but not CEOT. * **Option C (Dental Lamina):** Remnants of the dental lamina (Rests of Serres) are typically the origin of the **Odontogenic Keratocyst (OKC)** and Ameloblastoma [1]. * **Option D:** Since the origin is specifically linked to the stratum intermedium, "All of the above" is incorrect. **NEET-PG High-Yield Pearls for Pindborg Tumor:** 1. **Radiology:** Classically presents as a unilocular or multilocular radiolucency with a **"Driven Snow" appearance** (radiopaque foci due to calcification). 2. **Histopathology:** Characterized by **Liesegang rings** (concentric calcifications) and **Liesegang-like amyloid-containing areas** that stain with Congo Red and show **apple-green birefringence** under polarized light. 3. **Location:** Most commonly occurs in the **posterior mandible** (molar-ramus area) and is often associated with an impacted tooth. 4. **Nature:** It is a slow-growing, painless swelling but has a recurrence rate of about 14%. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Head and Neck, pp. 741-742.

Q: A 15-year-old boy presents with pain and swelling in his right thigh. A biopsy of the mass demonstrates osteosarcoma. His mother was diagnosed with breast cancer one year ago, and his maternal grandmother died of breast cancer ten years ago. The child has three younger siblings. Siblings are at an increased risk of developing which of the following cancers?

Glioma. ### Explanation **Concept: Li-Fraumeni Syndrome (LFS)** The clinical presentation describes a classic case of **Li-Fraumeni Syndrome**. This is an autosomal dominant condition caused by a germline mutation in the **TP53 tumor suppressor gene** (located on chromosome 17p) [1]. The syndrome is characterized by a strong family history of early-onset cancers. **Why Glioma is Correct:** The "core" cancers associated with LFS are remembered by the mnemonic **SBLA**: **S**arcoma (Osteosarcoma/Soft tissue), **B**reast cancer, **L**eukemia, and **A**drenal cortical carcinoma. However, the spectrum also significantly includes **Brain tumors (specifically Gliomas)**. Since the siblings share the same genetic risk, they are at a high risk for these specific malignancies. **Why Other Options are Incorrect:** * **Wilms tumor (A):** Associated with WAGR syndrome, Denys-Drash, or Beckwith-Wiedemann syndrome (WT1/WT2 mutations), not typically TP53. * **Neuroblastoma (B):** Most common extracranial solid tumor in children; associated with N-MYC amplification, but not a core component of LFS. * **Hepatoblastoma (C):** Associated with Familial Adenomatous Polyposis (FAP) and Beckwith-Wiedemann syndrome, but not LFS. **High-Yield Clinical Pearls for NEET-PG:** * **TP53:** Known as the "Guardian of the Genome." It regulates the cell cycle at the G1-S checkpoint. * **LFS Criteria:** Proband diagnosed with sarcoma <45 years + First-degree relative with any cancer <45 years + Another first/second-degree relative with any cancer <45 years or sarcoma at any age. * **Most Common Cancer in LFS:** Breast cancer (females) and Sarcomas (males/children). * **Choroid Plexus Carcinoma:** In a child, this specific brain tumor is highly suggestive of a TP53 mutation. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 298-300.

Q: Which of the following functions is associated with the oncogenic MYC in lung cancer?

Nuclear binding protein. **Explanation:** **Why the correct answer is right:** The **MYC** gene (specifically *c-MYC*, *n-MYC*, and *l-MYC*) encodes a **nuclear transcription factor** [1] that plays a central role in cell cycle progression, metabolism, and apoptosis. Once synthesized, the MYC protein translocates to the nucleus, where it binds to specific DNA sequences (E-box sequences) via a basic helix-loop-helix leucine zipper domain. In lung cancer (particularly Small Cell Lung Cancer), *L-MYC* amplification leads to the constitutive expression of these nuclear proteins, driving uncontrolled cellular proliferation [1], [2]. **Why the other options are wrong:** * **A. Protein Kinase:** This function is characteristic of oncogenes like **ABL** (non-receptor tyrosine kinase) or **ERBB1/EGFR** (receptor tyrosine kinase) [1]. * **B. GTP Binding Protein:** This describes the **RAS** family of oncogenes (K-RAS, H-RAS, N-RAS). RAS proteins act as molecular switches by cycling between GDP-bound (inactive) and GTP-bound (active) states [2]. * **C. Growth Factor:** This refers to oncogenes like **PDGF-̢** (SIS oncogene), which act as extracellular signals to trigger cell growth. **High-Yield Clinical Pearls for NEET-PG:** * **MYC Family Associations:** * **c-MYC:** Burkitt Lymphoma (t[8;14]). * **N-MYC:** Neuroblastoma (Double minute chromosomes). * **L-MYC:** Small Cell Carcinoma of the Lung [2]. * **Mechanism:** MYC upregulates **Cyclin-Dependent Kinases (CDKs)** and downregulates CDK inhibitors, effectively "pushing" the cell through the G1/S checkpoint [2]. * **Warburg Effect:** MYC is a key mediator in reprogramming cell metabolism to aerobic glycolysis. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 292-293. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 296-297.

Question 1

Which of the following is true about p53?

Accepted Answer

Encodes 53 kDa protein

Answer

Tumor suppressor gene

Answer

Proto-oncogene

Answer

Pro-apoptotic

Question 2

Which of the following is seen in African children with Burkitt's lymphoma?

Accepted Answer

Chromosome 8q24 to chromosome 14q32 translocation

Answer

Absence of EBV

Answer

T cell neoplasia

Answer

Deletion of C-myc gene

Question 3

Which of the following is considered as a high-risk susceptibility gene for malignant melanoma?

Accepted Answer

CDKN2A

Answer

CDKN1A

Answer

CDKN3A

Answer

CDKN4A

Question 4

Women carrying the BRCA1 gene have a higher likelihood of developing which type of breast carcinoma?

Accepted Answer

Medullary

Answer

Lobular

Answer

Colloid

Answer

Secretory

Question 5

Which of the following conditions is characterized by a Zellballen pattern?

Accepted Answer

Carotid body tumor

Answer

Lymphoepithelial cyst

Answer

Cholesteatoma

Answer

Thyroglossal cyst

Question 6

Which of the following is not a cyclin-dependent kinase (CDK) inhibitor?

Accepted Answer

p53

Answer

p21

Answer

p27

Answer

p57

Question 7

What is the origin of the Pindborg tumour?

Accepted Answer

Stratum intermedium

Answer

Reduced enamel epithelium

Answer

Dental lamina

Answer

All of the above

Question 8

Arrange the following in increasing order for risk of malignancy: Fibroadenoma, Sclerosing adenosis, Atypical ductal hyperplasia, Lobular carcinoma in situ.

Accepted Answer

Fibroadenoma, Sclerosing adenosis, Atypical ductal hyperplasia, Lobular carcinoma in situ

Answer

Sclerosing adenosis, Fibroadenoma, Atypical ductal hyperplasia, Lobular carcinoma in situ

Answer

Fibroadenoma, Sclerosing adenosis, Lobular carcinoma in situ, Atypical ductal hyperplasia

Answer

Sclerosing adenosis, Fibroadenoma, Lobular carcinoma in situ, Atypical ductal hyperplasia

Question 9

A 15-year-old boy presents with pain and swelling in his right thigh. A biopsy of the mass demonstrates osteosarcoma. His mother was diagnosed with breast cancer one year ago, and his maternal grandmother died of breast cancer ten years ago. The child has three younger siblings. Siblings are at an increased risk of developing which of the following cancers?

Accepted Answer

Glioma

Answer

Wilms tumor

Answer

Neuroblastoma

Answer

Hepatoblastoma

Question 10

Which of the following functions is associated with the oncogenic MYC in lung cancer?

Accepted Answer

Nuclear binding protein

Answer

Protein kinase

Answer

GTP binding protein

Answer

Growth factor

Neoplasia — MCQs

Neoplasia — MCQs

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