Neoplasia Practice Questions

Q: Von Hippel-Lindau (VHL) syndrome is most commonly associated with which carcinoma?

Renal cell carcinoma. **Explanation:** **Von Hippel-Lindau (VHL) syndrome** is an autosomal dominant hereditary cancer syndrome caused by a germline mutation in the **VHL tumor suppressor gene** located on **chromosome 3p25**. **Why Renal Cell Carcinoma (RCC) is correct:** The VHL protein normally acts as part of a ubiquitin ligase complex that targets **Hypoxia-Inducible Factor 1α (HIF-1α)** for degradation. In VHL syndrome, the loss of this protein leads to the constitutive stabilization of HIF-1α, which triggers the overexpression of growth factors like VEGF and PDGF. This "pseudo-hypoxia" state drives angiogenesis and cell proliferation, specifically predisposing patients to **Clear Cell Renal Cell Carcinoma** [1]. Approximately 70% of VHL patients develop RCC, often bilaterally and multicentrically. **Why other options are incorrect:** * **Lung Carcinoma:** Primarily associated with smoking or mutations in EGFR/ALK; not a component of the VHL spectrum. * **Endometrial Carcinoma:** Linked to Lynch Syndrome (HNPCC) and Cowden Syndrome (PTEN mutation), but not VHL. * **Hepatocellular Carcinoma:** Associated with Chronic Hepatitis (B/C) and Cirrhosis; VHL is associated with liver cysts, but rarely malignancy. **High-Yield Clinical Pearls for NEET-PG:** * **VHL Manifestations (mnemonic: HARP):** **H**emangioblastomas (Cerebellum/Retina), **A**ngiomatosis, **R**CC (Clear cell type), and **P**heochromocytoma. * **Pancreatic involvement:** Serous cystadenomas and Neuroendocrine tumors (NETs). * **Epididymal papillary cystadenomas** are a highly specific finding. * **Genetics:** Follows the "Knudson Two-Hit Hypothesis" (one inherited mutation, one acquired somatic mutation). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 958-959.

Question 1

What is the term for the study of neoplastic growths?

Accepted Answer

Oncology

Answer

Tetralogy

Answer

Anaplasia

Answer

Neoplasia

Question 2

Cancer cells primarily derive energy from which metabolic process?

Accepted Answer

Glycolysis

Answer

Oxidative metabolism

Answer

Increased mitochondria

Answer

None of the above

Question 3

Cowden syndrome is commonly associated with which of the following?

Accepted Answer

Hamartoma

Answer

Renal cell carcinoma

Answer

Thymic carcinoid

Answer

Cerebellar haemangioblastoma

Question 4

Which syndrome is associated with an increased risk of leukemia?

Accepted Answer

Klinefelter syndrome

Answer

Plummer-Vinson syndrome

Answer

Sturge-Weber syndrome

Answer

Multiple hamartoma

Question 5

Presence of more than two germ cell layers in a tumor is called:

Accepted Answer

Teratoma

Answer

Dysgerminoma

Answer

Theca cell tumor

Answer

Seminoma

Question 6

Which of the following cancers is least associated with genetic and familial causes?

Accepted Answer

Lung

Answer

Ovarian

Answer

Prostate

Answer

Breast

Question 7

In which of the following malignancies is histological grade a good prognostic indicator?

Accepted Answer

Soft tissue sarcoma

Answer

Melanoma

Answer

Renal cell carcinoma

Answer

Lung cancer

Question 8

A 63-year-old woman discovers a lump in her right breast. Mammography confirms the presence of a suspicious mass, and a needle core biopsy reveals that the mass is cancerous with amplification of the Her-2/neu oncogene. What kind of protein is the gene product of Her-2/neu?

Accepted Answer

Receptor tyrosine kinase

Answer

GTPase

Answer

GTPase-activating protein

Answer

Nuclear transcription factor

Question 9

Orphan Annie-eye nuclei are seen in which of the following conditions?

Accepted Answer

Papillary carcinoma of thyroid

Answer

Medullary carcinoma of thyroid

Answer

Anaplastic carcinoma of thyroid

Answer

Follicular carcinoma of thyroid

Question 10

Von Hippel-Lindau (VHL) syndrome is most commonly associated with which carcinoma?

Accepted Answer

Renal cell carcinoma

Answer

Lung carcinoma

Answer

Endometrial carcinoma

Answer

Hepatocellular carcinoma

Neoplasia — MCQs

Neoplasia — MCQs

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