Neoplasia — MCQs

Neoplasia Indian Medical PG Practice Questions and MCQs

Question 911: Lethal midline granuloma arises from which cell type?

A. B-cells
B. NK cells (Correct Answer)
C. T-cells
D. Macrophages

Explanation: ***NK cells*** - **Lethal midline granuloma**, also known as **extranodal NK/T-cell lymphoma, nasal type**, is **predominantly derived from Natural Killer (NK) cells** in 70-80% of cases. - These malignant NK cells are **CD56 positive** and show a **cytotoxic phenotype** with expression of cytotoxic molecules (granzyme B, perforin, TIA-1). - The disease is strongly associated with **Epstein-Barr virus (EBV)** infection and presents with **destructive midline facial lesions** involving the nasal cavity, paranasal sinuses, and palate. - **Angioinvasion and necrosis** are characteristic histologic features. *T-cells* - While a **minority of cases** (20-30%) can arise from **cytotoxic T-cells** with a similar clinical presentation, NK cell origin is the predominant type. - T-cell derived cases are CD3 positive but CD56 negative, distinguishing them from the more common NK cell type. - The term "NK/T-cell lymphoma" reflects that both origins are possible, but NK cells are the primary cell of origin. *B-cells* - **B-cell lymphomas** are distinct entities that do not typically present as lethal midline granuloma. - B-cell lymphomas may involve extranodal sites but lack the characteristic aggressive destruction of midline facial structures seen in NK/T-cell lymphoma. *Macrophages* - **Macrophages** are phagocytic cells of the mononuclear phagocyte system and are not the origin of lymphomas. - Histiocytic disorders (like Langerhans cell histiocytosis) arise from cells of macrophage/dendritic cell lineage, but these are distinct from lymphoid malignancies.

Question 912: Which tumor is not associated with von Hippel-Lindau disease?

A. Renal Cell Carcinoma
B. Pheochromocytoma
C. Cholangiocarcinoma (Correct Answer)
D. Hemangioblastoma of Cerebellum

Explanation: ***Cholangiocarcinoma*** - Cholangiocarcinoma is a **bile duct cancer** that is not typically associated with **von Hippel-Lindau (VHL) disease**. - While VHL disease affects various organs through abnormal blood vessel growth, the **bile ducts** are not common sites for VHL-related tumors. *Hemangioblastoma of Cerebellum* - **Hemangioblastomas**, particularly in the **cerebellum**, are characteristic benign tumors strongly associated with **VHL disease**. - These tumors are highly vascular and linked to mutations in the **VHL tumor suppressor gene** [2]. *Renal Cell Carcinoma* - **Clear cell renal cell carcinoma** is a major manifestation of VHL disease due to a mutated **VHL gene**, which affects oxygen sensing and leads to tumor formation [1]. - Patients with VHL disease have a significantly increased risk of developing **renal cysts** and these specific kidney cancers. *Pheochromocytoma* - **Pheochromocytomas**, which are neuroendocrine tumors of the **adrenal medulla**, are a well-documented feature of VHL disease. - They produce **catecholamines** and can lead to hypertension and other symptoms related to hormonal excess. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 958-959. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 306-307.

Question 913: Familial retinoblastoma is characterized by which of the following statements?

A. Due to mutations in the RB1 gene
B. Less common than sporadic retinoblastoma
C. More commonly bilateral
D. Has autosomal dominant inheritance (Correct Answer)

Explanation: ***Has autosomal dominant inheritance*** - Familial retinoblastoma is inherited in an **autosomal dominant pattern** with approximately **90% penetrance**, meaning a child who inherits the mutated RB1 gene has a 90% chance of developing the tumor [1]. - This is the **defining characteristic** that distinguishes familial from sporadic retinoblastoma - it represents a **germline mutation** present in all cells of the body from conception [2]. - The inheritance pattern allows the condition to be passed through generations, with each affected parent having a 50% chance of passing the mutation to their offspring. *Due to mutations in the RB1 gene* - While this statement is **medically accurate**, mutations in the RB1 tumor suppressor gene occur in **both familial and sporadic** forms of retinoblastoma [3]. - This does not **characterize** or distinguish familial retinoblastoma specifically, as the same gene is involved in all retinoblastoma cases [1]. - The key difference is that familial cases involve **germline** RB1 mutations, while sporadic cases typically involve **somatic** mutations [2]. *More commonly bilateral* - This statement is **factually correct** - familial retinoblastoma presents bilaterally in approximately **60-75%** of cases, compared to only 25-30% of sporadic cases. - However, bilaterality is a **clinical consequence** of having a germline mutation (multiple retinal cells at risk) rather than the primary defining characteristic [2]. - Bilateral presentation is better viewed as a **manifestation** of the inherited genetic defect rather than the characteristic itself. *Less common than sporadic retinoblastoma* - This is an **accurate epidemiological fact** - familial cases account for approximately **25-30%** of all retinoblastoma cases, with sporadic cases comprising the majority (~70-75%) [3]. - However, this describes the **relative frequency** rather than characterizing the nature or mechanism of familial retinoblastoma. - Prevalence data doesn't define what makes familial retinoblastoma unique from a genetic or clinical standpoint. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 298-300. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 300. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 737-738.

Question 914: A 12-year-old child presented with a maxillary mass, which on further investigation showed the presence of a "starry sky appearance" in the biopsy. All of the following statements are true except:

A. This is non-Hodgkin lymphoma
B. More commonly seen in adults (Correct Answer)
C. This condition is due to c-Myc gene activation
D. This is Burkitt lymphoma

Explanation: ***More commonly seen in adults*** - Burkitt lymphoma predominantly occurs in children and young adolescents, especially in endemic forms associated with malaria [1]. - While it can appear in adults, its incidence is significantly lower compared to pediatric populations [1]. *This condition is due to c-Myc gene activation* - Burkitt lymphoma is characteristically associated with translocations involving the **c-Myc gene**, primarily t(8;14). - This genetic alteration leads to **uncontrolled cell proliferation**, a hallmark of the disease. *This is non-Hodgkin lymphoma* - Burkitt lymphoma is indeed classified as a **non-Hodgkin lymphoma**, arising from B-lymphoid cells [1]. - It is known for its aggressive behavior and association with the **"starry sky appearance"** seen on biopsy [1]. *Burkitt lymphoma* - The **starry sky appearance** is a classic histopathological feature of Burkitt lymphoma, indicating high proliferation rates [1]. - It commonly presents as a maxillary or abdominal mass in children, confirming its diagnosis in this scenario [1]. **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. 605-606.

Question 915: What is the most common initial site for the spread of cancers?

A. Liver
B. Lungs
C. Brain
D. Lymph Nodes (Correct Answer)

Explanation: ***LN*** - Lymph nodes (LN) are the **primary site for metastatic spread** due to their role in filtering lymphatic fluid, making them a common initial site for cancer spread. - The presence of cancer cells in lymph nodes can indicate **lymphatic dissemination**, often correlating with advanced disease stages. *Brain* - While the **brain** can be affected by metastases, it is not the **commonest site** for initial spread of cancers compared to lymph nodes. - Metastasis to the brain typically occurs later in the disease process and is more common with certain cancers like melanoma or lung cancer. *Lungs* - The **lungs** are often involved in metastatic disease, but they are generally not the initial site of spread; rather, they serve as a common final destination for metastatic lesions [1]. - Lung involvement is usually detected in advanced cancer stages when multiple sites of metastasis have occurred. *Liver* - The **liver** is a common site for metastasis due to its extensive blood supply and involvement in circulation; however, it is not the first site where cancers typically spread [1]. - Liver metastases often arise later in the course of a malignancy and are usually a result of dissemination from other primary sites. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 282.

Question 916: A 67-year-old male smoker presents with haemoptysis and cough. Bronchoscopic biopsy revealed an undifferentiated tumour. The immunohistochemical marker that can be most helpful is:

A. Cytokeratin (Correct Answer)
B. Vimentin
C. GGT
D. Calretinin

Explanation: ***Cytokeratin*** - **Cytokeratin** is a **intermediate filament protein** found in epithelial cells and is a key marker for carcinomas, which are the most common type of lung cancer [1]. - Given the patient's history (smoker, haemoptysis) and an undifferentiated tumor, a **carcinoma** is highly probable, making cytokeratin the most helpful marker for classification [2]. *Vimentin* - **Vimentin** is an intermediate filament protein typically expressed in **mesenchymal cells** (e.g., sarcomas, lymphomas, melanoma). - Its presence would suggest a non-epithelial tumor, which is less likely in this clinical context of a lung mass in a smoker. *GGT* - **Gamma-glutamyl transpeptidase (GGT)** is an enzyme primarily used as a marker for **liver function** and bile duct obstruction. - It is not a tumor marker and has no role in the immunohistochemical diagnosis of an undifferentiated lung tumor. *Calretinin* - **Calretinin** is a nuclear and cytoplasmic protein that is a useful marker for **mesothelioma** and some other neuroendocrine tumors. - While mesothelioma can occur in the lung, it is less common than carcinoma, and calretinin would only be considered if mesothelioma was specifically suspected. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Respiratory Tract Disease, pp. 334-337. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, pp. 720-721.

Question 917: Which of the following statements about Psammoma bodies is false?

A. Seen in teratoma (Correct Answer)
B. Contains Calcium deposits
C. Concentric whorled appearance
D. Seen in meningioma

Explanation: ***Seen in teratoma*** - **Psammoma bodies** are not typically found in teratomas; they are more commonly associated with other tumors such as meningiomas and papillary thyroid carcinoma. - Teratomas usually consist of differentiated tissues from all three germ layers, and do not exhibit the characteristic **laminated** appearance of psammoma bodies. *Seen in meningioma* - **Meningiomas** are indeed associated with psammoma bodies, which appear as laminated calcified structures [1]. - These bodies signify **calcium deposition** and are a pathological feature of certain tumors, particularly **meningeal** tumors [1]. *Contains Calcium deposits* - Psammoma bodies are known for their **calcium deposits**, characterized by their laminated structure [1]. - They represent focal areas of calcification within tumors, particularly in conditions like **meningiomas** and **thyroid papillary carcinoma**. *Concentric whorled appearance* - Psammoma bodies display a **concentric** laminated appearance, often described as **whorled layers**. - This histopathological feature is a distinguishing characteristic of psammoma bodies in confirmed tumors. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1316-1317.

Question 918: Which of the following is the strongest predisposing factor for testicular germ cell tumors?

A. Testicular feminization syndrome
B. Family history of testicular cancer
C. Cryptorchidism
D. Previous testicular germ cell tumor (Correct Answer)

Explanation: ***Previous testicular germ cell tumor*** - A previous diagnosis of a **testicular germ cell tumor** is the strongest risk factor for developing a new, contralateral tumor. - This risk is significantly higher than that associated with other predisposing factors. *Testicular feminization syndrome* - While **gonadal dysgenesis** conditions like **androgen insensitivity syndrome** (sometimes referred to as testicular feminization syndrome) can increase the risk of gonadal tumors, it is not the strongest factor compared to a prior cancer history. - The risk is related to the presence of **undescended testes** or dysgenetic gonads within these syndromes. *Family history of testicular cancer* - A **family history** of testicular cancer does increase the risk, suggesting a genetic predisposition. - However, this risk is generally lower than the risk associated with having already had one testicular germ cell tumor. *Cryptorchidism* - **Cryptorchidism** (undescended testis) is a well-established risk factor for testicular germ cell tumors, with the risk increasing the higher the position of the testis [1], [2]. - Despite this, the risk associated with a history of cryptorchidism is still less potent than having a prior testicular cancer [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 508-509. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lower Urinary Tract and Male Genital System, pp. 976-977.

Question 919: What is the most common site of esophageal cancer?

A. Entire Esophagus
B. Lower 1/3rd
C. Upper 1/3rd
D. Middle 1/3rd (Correct Answer)

Explanation: ***Lower 1/3rd*** - The most common site for **esophageal cancer** is indeed the **lower third** of the esophagus, often correlating with the gastroesophageal junction [1]. - This area is associated with **risk factors** such as **gastroesophageal reflux disease (GERD)** and **Barrett's esophagus**, which can lead to adenocarcinoma [1]. *Middle 1/3 rd* - While esophageal cancer can occur here, it is significantly **less common** than in the lower third. - Typically, squamous cell carcinoma is more prevalent in the middle third [2], but not as frequent as adenocarcinoma in the lower third. *Upper 1/3 rd* - Similar to the middle third, it has a **lower incidence** of esophageal cancer compared to the lower segment. - This section is more frequently affected by **squamous cell carcinoma**, especially associated with factors like tobacco and alcohol use. *Lower end of esophagus* - While it may seem specific, it does not highlight the **most common site** as comprehensively as "lower 1/3rd," which encompasses adenocarcinoma risk factors. - This phrasing may confuse with the **anatomical definition**, as it doesn't clearly denote a region but rather a terminological description. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, pp. 765-766. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, pp. 766-767.

Question 920: Signet ring cells are characteristically seen in which ovarian tumor?

A. Dysgerminoma
B. Krukenberg tumor (Correct Answer)
C. Granulosa cell tumor
D. Teratoma

Explanation: ***Krukenberg tumor*** - The **signet ring appearance** is classically associated with Krukenberg tumors, which are metastatic tumors of the ovary often arising from gastric cancer [1]. - These tumors contain **mucin-filled cells** that resemble signet rings when viewed histologically [1]. *Teratoma* - Teratomas contain **multiple cell types** and may have various appearances, but they do not typically exhibit a signet ring configuration [3]. - More characteristic findings include **dermoid cysts** or tissue types from all three embryonic layers [3]. *Granulosa cell tumor* - Granulosa cell tumors are primarily characterized by **hormonal activity**, producing estrogen, and do not display signet ring cells. - Histologically, they are defined by **Call-Exner bodies** and small follicles rather than signet ring morphology. *Dysgerminoma* - Dysgerminomas, a type of germ cell tumor, typically show **solid sheets of uniform cells** without the signet ring feature [2]. - They are more associated with **lymphocytic infiltration** and often elevate serum levels of **LDH** [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, p. 779. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Female Genital Tract, pp. 1034-1035. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Female Genital Tract Disease, pp. 480-481.

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