Neoplasia Practice Questions

Q: Which syndrome represents a subset of Carney complex?

NAME syndrome. **Explanation:** **Carney Complex** is an autosomal dominant familial multiple neoplasia syndrome characterized by cardiac and cutaneous myxomas, spotty skin pigmentation (lentigines), and endocrine overactivity. It is primarily caused by mutations in the **PRKAR1A gene** (Protein Kinase A type 1-alpha regulatory subunit) [1]. **Why Option A is Correct:** **NAME syndrome** (Nevi, Atrial myxoma, Myxoid neurofibroma, and Ephelides) and **LAMB syndrome** (Lentigines, Atrial myxoma, Mucocutaneous myxomas, and Blue nevi) are historical acronyms that describe the clinical subsets of Carney complex. They represent the same genetic entity with varying phenotypic expressions of myxomatous and pigmented lesions. **Why Other Options are Incorrect:** * **B. LEOPARD Syndrome:** Now known as Noonan syndrome with multiple lentigines, it is caused by PTPN11 mutations. While it features lentigines, it is associated with hypertrophic cardiomyopathy and deafness, not myxomas. * **C. MERRF Syndrome:** A mitochondrial disorder (Myoclonic Epilepsy with Ragged Red Fibers) characterized by myoclonus, ataxia, and seizures. * **D. Wilkie’s Syndrome:** Also known as Superior Mesenteric Artery (SMA) syndrome, it involves compression of the third part of the duodenum by the SMA. **High-Yield Clinical Pearls for NEET-PG:** * **Carney Complex vs. Carney Triad:** Do not confuse them. **Carney Triad** is non-hereditary and includes GIST, Pulmonary Chondroma, and Paraganglioma. * **Endocrine involvement:** The most common endocrine manifestation in Carney Complex is **PPNAD** (Primary Pigmented Nodular Adrenocortical Disease), leading to ACTH-independent Cushing syndrome [1]. * **Psammomatous Melanotic Schwannoma:** A highly specific tumor associated with this complex. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1126-1127.

Q: The lesion shown below is caused by:

HPV. ***HPV*** - **HPV** causes characteristic **koilocytes** with **perinuclear halos** and **pyknotic nuclei**, which are pathognomonic cytopathic effects seen in cervical dysplasia and condylomas. - Associated with **squamous cell carcinomas** and **adenocarcinomas** of the cervix, vulva, and other anogenital regions through oncogenic strains (16, 18). *HSV* - **HSV** produces **multinucleated giant cells** with **intranuclear inclusion bodies** (Cowdry A bodies) and **ground glass nuclei**. - Causes **vesicular lesions** that ulcerate, not the keratinizing lesions with koilocytes typical of HPV infection. *HIV* - **HIV** does not directly cause specific histologic lesions but predisposes to **opportunistic infections** and **malignancies**. - Associated with **Kaposi's sarcoma**, **lymphomas**, and **cervical cancer** (through HPV co-infection), but doesn't produce koilocytic changes. *EBV* - **EBV** causes **atypical lymphocytes** and is associated with **infectious mononucleosis** and certain **B-cell lymphomas**. - Produces **Reed-Sternberg-like cells** in lymphoid tissue, not squamous epithelial changes with perinuclear halos.

Q: The RET proto-oncogene is associated with which carcinoma?

Multiple Endocrine Neoplasia (MEN). **Explanation:** The **RET proto-oncogene**, located on chromosome 10q11.2, encodes a receptor tyrosine kinase involved in cell growth and differentiation. Gain-of-function mutations in RET are strongly associated with **Multiple Endocrine Neoplasia (MEN) syndromes**, specifically **MEN 2A and 2B**, as well as Familial Medullary Thyroid Carcinoma (FMTC) [1]. In these syndromes, the mutation leads to constitutive activation of the receptor, driving the development of Medullary Thyroid Carcinoma and Pheochromocytoma [2]. **Analysis of Options:** * **Hepatocellular Carcinoma:** Primarily associated with chronic HBV/HCV infections, aflatoxin exposure, and mutations in the **TP53** gene or **CTNNB1** (β-catenin). * **Testicular Carcinoma:** Most germ cell tumors are associated with an **isochromosome of the short arm of chromosome 12 [i(12p)]**. * **Breast Carcinoma:** Frequently linked to mutations in **BRCA1, BRCA2, TP53 (Li-Fraumeni)**, and amplification of the **HER2/neu** (ERBB2) oncogene [1]. **High-Yield Clinical Pearls for NEET-PG:** * **MEN 2A:** Characterized by Medullary Thyroid Carcinoma, Pheochromocytoma, and Parathyroid Hyperplasia [2]. * **MEN 2B:** Characterized by Medullary Thyroid Carcinoma, Pheochromocytoma, Mucosal Neuromas, and Marfanoid habitus [2]. * **Prophylactic Thyroidectomy:** In children identified with RET mutations, a prophylactic thyroidectomy is often indicated because Medullary Thyroid Carcinoma occurs in nearly 100% of these patients. * **Papillary Thyroid Carcinoma:** RET can also be involved via chromosomal rearrangement (**RET/PTC**), whereas point mutations are specific to the MEN 2/Medullary Thyroid Carcinoma pathway [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 292. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1097-1137.

Question 1

Which syndrome represents a subset of Carney complex?

Accepted Answer

NAME syndrome

Answer

Leopard syndrome

Answer

MERRF syndrome

Answer

Wilkie's syndrome

Question 2

How will you differentiate a mediastinal mass being a thymoma or acute lymphoblastic leukemia?

Accepted Answer

Cytokeratin

Answer

CD1a

Answer

CD3

Answer

Tdt

Question 3

Invasive carcinoma differs from carcinoma in situ by:

Accepted Answer

Breached basement membrane

Answer

Abnormal nuclear morphology

Answer

Pleomorphism

Answer

Mitoses

Question 4

Which of the following is the most characteristic ultrastructural feature of paraganglioma on electron microscopy?

Accepted Answer

Dense core neuroendocrine granules

Answer

Shrunken mitochondria

Answer

Large Golgi apparatus

Answer

Frequent mitoses

Question 5

Which of the following is a tumor marker?

Accepted Answer

Alkaline phosphatase

Answer

Acid hydrolase

Answer

Melatonin

Answer

CPK-MB

Question 6

The lesion shown below is caused by:

Accepted Answer

HPV

Answer

HSV

Answer

HIV

Answer

EBV

Question 7

The RET proto-oncogene is associated with which carcinoma?

Accepted Answer

Multiple Endocrine Neoplasia (MEN)

Answer

Hepatocellular Carcinoma

Answer

Testicular Carcinoma

Answer

Breast Carcinoma

Question 8

Which of the following is not a benign neoplasm?

Accepted Answer

Seminoma

Answer

Chondroma

Answer

Leiomyoma

Answer

Papilloma

Question 9

What are the characteristic features of a pleomorphic adenoma?

Accepted Answer

Epithelial cells in a chondroid matrix

Answer

Columnar cells enclosing lymphoid stroma

Answer

Exclusively myoepithelial cells

Answer

Nests of squamous cells and vacuolated cells containing mucin

Question 10

N-MYC amplification is associated with which tumor?

Accepted Answer

Neuroblastoma

Answer

Burkitt lymphoma

Answer

Squamous cell carcinoma lung

Answer

Astrocytoma

Neoplasia — MCQs

Neoplasia — MCQs

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