Neoplasia Practice Questions

Q: Which of the following is NOT considered a premalignant condition?

Retinitis pigmentosa. **Explanation:** The correct answer is **Retinitis pigmentosa (Option A)**. **1. Why Retinitis Pigmentosa is the correct answer:** Retinitis pigmentosa is a group of rare, **genetic disorders** involving a breakdown and loss of cells in the retina (photoreceptors). It is characterized by progressive vision loss, night blindness, and "bone-spicule" pigmentation. Crucially, it is a **degenerative condition**, not a neoplastic or pre-neoplastic one. It does not involve cellular dysplasia or carry an inherent risk of transforming into a malignancy. **2. Analysis of Incorrect Options (Premalignant Conditions):** * **Ulcerative Colitis (UC) & Crohn’s Disease (B & C):** Both are forms of Inflammatory Bowel Disease (IBD) [1]. Chronic inflammation leads to increased cellular turnover and oxidative stress, which can cause dysplastic changes. UC carries a higher risk of **Colorectal Carcinoma** than Crohn’s, but both require regular endoscopic surveillance. Inflammation invariably involves the rectum and spreads proximally; in long-standing cases, dysplasia characterized by heaping of cells within crypts can occur [1]. * **Leukoplakia (D):** This is a clinical term for a white patch or plaque on the oral mucosa that cannot be rubbed off. It is a classic premalignant lesion, with a significant percentage progressing to **Squamous Cell Carcinoma (SCC)**. **Clinical Pearls for NEET-PG:** * **High-Yield Premalignant Lesions:** Actinic keratosis (leads to SCC), Barrett’s esophagus (leads to Adenocarcinoma), and Xeroderma pigmentosum (leads to BCC, SCC, and Melanoma). * **IBD Risk:** The risk of malignancy in IBD increases with the **duration** of the disease (usually after 8–10 years) and the **extent** of colonic involvement (pancolitis > left-sided colitis). * **Retinitis Pigmentosa Association:** Often associated with **Usher Syndrome** (hearing loss) and **Laurence-Moon-Biedl Syndrome**.

Q: A 5-year-old child presents with a lesion in the right eye. Histopathology reveals the presence of Flexner-Wintersteiner rosettes. What is the likely diagnosis?

Retinoblastoma. ***Retinoblastoma*** - The presence of **Flexner-Wintersteiner rosettes**, which are characteristic arrangements of columnar cells around a central lumen, is the pathognomonic histological feature of well-differentiated **retinoblastoma** [1].- This tumor is the most common **intraocular malignancy** of childhood, typically presenting as **leukocoria** (white pupillary reflex) in children under the age of 5. *Optic nerve glioma*- These tumors are typically low-grade astrocytomas, most frequently **pilocytic astrocytomas**, characterized by glial cells, not neuronal-like rosettes.- They involve the **optic nerve** itself and are strongly associated with **Neurofibromatosis type 1 (NF1)**. *Rhabdomyosarcoma*- This is the most common **pediatric orbital malignancy** (outside the globe), typically presenting with rapid onset of **proptosis** (exophthalmos) and eyelid swelling.- Histologically, it is a small round blue cell tumor derived from mesenchymal cells, showing **rhabdomyoblasts**, and does not form Flexner-Wintersteiner rosettes. *Ocular melanoma*- This malignancy is extremely rare in the pediatric population and is overwhelmingly a disease of **adults**.- Histopathology shows malignant cells derived from **melanocytes** (spindle or epithelioid cells) containing melanin, lacking photoreceptor differentiation structures like rosettes. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Eye, p. 1342.

Q: HPV infection is most commonly associated with which type of cancer?

Oropharyngeal cancer. ***Oropharyngeal cancer*** - HPV (particularly HPV-16 and HPV-18) is strongly associated with oropharyngeal cancers, especially those affecting the **base of tongue and tonsils** [2], [3] - HPV-positive oropharyngeal cancers represent a distinct epidemiological entity with **better prognosis** compared to tobacco/alcohol-related cases - Among the options listed, oropharyngeal cancer is the **only HPV-associated malignancy** [3] - Note: Cervical cancer is the **most common HPV-related cancer overall** (not listed in options) [1], [3] *Incorrect: Esophageal cancer* - Primarily associated with **tobacco, alcohol, Barrett's esophagus, and chronic GERD** - Not significantly associated with HPV infection *Incorrect: Lung cancer* - Main risk factors include **smoking, radon exposure, asbestos, and air pollution** - No established HPV association *Incorrect: Stomach cancer* - Associated with **H. pylori infection, dietary factors (nitrosamines), and chronic gastritis** - Not linked to HPV infection **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Female Genital Tract, pp. 1007-1008. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, pp. 739-741. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 219-220.

Q: Which of the following is true about p53?

It inhibits telomerase activity. ***It inhibits telomerase activity*** - **p53 transcriptionally represses hTERT** (telomerase reverse transcriptase), effectively downregulating telomerase activity as part of its tumor suppressor function. - This inhibition prevents **cellular immortalization** and contributes to p53's role as the "**guardian of the genome**" in preventing malignant transformation. *It increases telomere length in the liver* - p53 has **no tissue-specific function** to increase telomere length in any organ, including the liver. - p53 actually **opposes telomere maintenance** by inhibiting telomerase, which would shorten rather than lengthen telomeres. *It synthesizes RNA* - p53 is a **transcription factor** that regulates gene expression but does **not synthesize RNA directly**. - **RNA synthesis** is performed by **RNA polymerase enzymes**, while p53 modulates their activity at specific gene promoters. *It retains the telomerase enzyme in the nucleus* - p53 **represses telomerase activity** by downregulating hTERT expression, rather than retaining the enzyme. - Loss of p53 function in **cancer cells** allows telomerase reactivation, contributing to unlimited replicative potential.

Q: A three-year-old boy has been observed by his parents to be increasingly clumsy for the past 6 months. On a physical examination, there is a cauliflower-like growth in the left eye, leukocoria, and an absent red reflex. The microscopic appearance of the specimen shows Flexner-Wintersteiner rosettes. Which gene is involved in the given condition?

Rb gene. ***Rb gene***- The constellation of leukocoria, absent red reflex, and the microscopic finding of **Flexner-Wintersteiner rosettes** are pathognomonic for **retinoblastoma** [1, 3]. - The **Rb gene** (Retinoblastoma gene) is a critical **tumor suppressor gene** located on chromosome 13q14, and its inactivation leads to the development of this malignancy [1, 2]. *ATP 7B gene* - This gene is responsible for **Wilson disease**, an autosomal recessive disorder causing excessive **copper accumulation** in the liver, brain, and cornea (Kayser-Fleischer rings). - It is not associated with primary malignant retinal tumors like retinoblastoma. *Rhodopsin gene* - Mutations in the **rhodopsin gene** are the most common cause of **autosomal dominant retinitis pigmentosa**, which presents with progressive night blindness and visual field loss. - This condition involves retinal degeneration and does not cause a mass-forming neoplasm with leukocoria. *FBN 1 gene* - The **FBN1 gene** codes for **Fibrillin-1** and is mutated in **Marfan syndrome**, a connective tissue disorder. - While Marfan syndrome often causes ocular issues like **ectopia lentis** (lens dislocation), it does not cause retinoblastoma. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 737-738. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 300. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Eye, p. 1342.

Q: Masaoka staging is used for staging:

Thymoma. ***Thymoma*** - The **Masaoka staging system** is specifically designed for evaluating the extent of **thymomas**, a type of tumor originating from the thymus gland [1]. - This system assesses tumor invasion into surrounding structures, such as the mediastinal fat, pleura, pericardium, and great vessels, which is critical for determining prognosis and treatment [1],[2]. *Germ cell tumours* - **Germ cell tumors** are typically staged using systems specific to their primary site (e.g., testicular, ovarian, mediastinal) that often involve imaging, tumor markers (e.g., AFP, beta-hCG), and histopathological findings. - While germ cell tumors can occur in the mediastinum, the Masaoka system is not their primary staging method. *Neurogenic tumours* - **Neurogenic tumors** encompass a broad range of tumors arising from nervous tissue (e.g., neuroblastoma, schwannoma, ganglioneuroma) and are staged using various systems depending on the specific tumor type and location (e.g., INPC staging for neuroblastoma). - The Masaoka system is not applicable to these tumors. *Lymphoma* - **Lymphomas** are staged using the **Ann Arbor classification system** (or modified Lugano classification), which primarily considers the number and location of involved lymph node regions, as well as extranodal involvement. - This system is distinct from the Masaoka staging system, which is anatomically focused on the thymus and its surrounding structures. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 634-635. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 572-574.

Question 1

Which of the following statements is TRUE about Tumor suppressor gene p53?

Accepted Answer

Its activity in the cells decreases following UV irradiation and stimulates cell cycle.

Answer

It regulates certain genes involved in cell cycle regulation.

Answer

Its increased levels can induce apoptosis.

Answer

Mutations of the p53 gene are the most common genetic alteration seen in human cancer.

Question 2

S100 is a marker used in the diagnosis of which of the following conditions, EXCEPT?

Accepted Answer

Basal cell carcinoma

Answer

Melanoma

Answer

Schwannoma

Answer

Histiocytoma

Question 3

Which of the following is NOT considered a premalignant condition?

Accepted Answer

Retinitis pigmentosa

Answer

Crohn's disease

Answer

Ulcerative colitis

Answer

Leukoplakia

Question 4

A 5-year-old child presents with a lesion in the right eye. Histopathology reveals the presence of Flexner-Wintersteiner rosettes. What is the likely diagnosis?

Accepted Answer

Retinoblastoma

Answer

Optic nerve glioma

Answer

Rhabdomyosarcoma

Answer

Ocular melanoma

Question 5

HPV infection is most commonly associated with which type of cancer?

Accepted Answer

Oropharyngeal cancer

Answer

Esophageal cancer

Answer

Lung cancer

Answer

Stomach cancer

Question 6

Which of the following is not related to disease progression?

Accepted Answer

Duration

Answer

Depth

Answer

Stage of Cancer

Answer

Site

Question 7

Which of the following is true about p53?

Accepted Answer

It inhibits telomerase activity

Answer

It retains the telomerase enzyme in the nucleus

Answer

It increases telomere length in the liver.

Answer

It synthesizes RNA

Question 8

A three-year-old boy has been observed by his parents to be increasingly clumsy for the past 6 months. On a physical examination, there is a cauliflower-like growth in the left eye, leukocoria, and an absent red reflex. The microscopic appearance of the specimen shows Flexner-Wintersteiner rosettes. Which gene is involved in the given condition?

Accepted Answer

Rb gene

Answer

Rhodopsin gene

Answer

FBN 1 gene

Answer

ATP 7B gene

Question 9

A 3-year-old child has presented with abdominal lump and peculiar appearance of eyes. All genes are responsible for development of this condition except:

Accepted Answer

K-ras gene

Answer

WT1

Answer

P53

Answer

Beta-catenin

Question 10

Masaoka staging is used for staging:

Accepted Answer

Thymoma

Answer

Germ cell tumours

Answer

Neurogenic tumours

Answer

Lymphoma

Neoplasia — MCQs

Neoplasia — MCQs

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