Neoplasia — MCQs

A 3-year-old boy is found to have spontaneous bursts of non-rhythmic conjugate eye movements in various directions, as well as hypotonia and myoclonus. Physical examination also reveals an abdominal mass. A CT scan shows a mass in the adrenal gland. Which of the following statements is false regarding the patient's condition?

Neoplasia Indian Medical PG Practice Questions and MCQs

Question 901: Which tumor marker is most commonly associated with lung and breast carcinoma?

A. CEA (Correct Answer)
B. hCG
C. AFP
D. CA-15-3

Explanation: ***CEA*** - **Carcinoembryonic antigen (CEA)** is a tumor marker commonly associated with **lung** and **breast cancers** [1]. - Elevated levels of CEA are often observed in **various malignancies**, making it useful for monitoring treatment response and recurrence. *CA-15-3* - While **CA-15-3** is a breast cancer marker, it is less specific than CEA and often used primarily for **monitoring** but not for initial diagnosis. - It is primarily elevated in **breast carcinoma**, not typically associated with **lung cancer**. *11CG* - This ppears to be incorrectly referenced and may not exist as a recognized tumor marker for lung or breast cancer. - There are no clinical associations with lung or breast cancer, making it irrelevant in this context. *AFP* - **Alpha-fetoprotein (AFP)** is primarily associated with **liver** and **germ cell tumors**, not commonly associated with lung or breast cancers [1]. - Elevated AFP levels do not correlate with lung or breast carcinomas, distinguishing it from CEA's relevance. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 346.

Question 902: E-cadherin gene deficiency is associated with which type of cancer?

A. Intestinal cancer
B. Pancreatic cancer
C. Thyroid cancer
D. Gastric cancer (Correct Answer)

Explanation: **Correct: Gastric cancer** - **E-cadherin** is a crucial cell adhesion molecule, and its deficiency is strongly linked to **diffuse-type gastric cancer**. - Mutations in the **CDH1 gene**, which encodes E-cadherin, predispose individuals to **hereditary diffuse gastric cancer (HDGC)** due to loss of cell-cell adhesion. - This is a classic tumor suppressor gene, and germline mutations lead to an autosomal dominant cancer syndrome. *Incorrect: Intestinal cancer* - While E-cadherin plays a role in various epithelial cancers, its deficiency is not the primary driver or defining feature of intestinal cancer (colorectal cancer). - **Colorectal cancer** is more commonly associated with mutations in genes like **APC**, **KRAS**, **TP53**, and mismatch repair genes. *Incorrect: Thyroid cancer* - E-cadherin expression can be altered in thyroid cancers, but its deficiency is not the hallmark genetic event. - **Thyroid cancer** (especially papillary and follicular types) is more frequently linked to gene rearrangements (e.g., **RET/PTC**, **PAX8/PPARγ**) or point mutations (e.g., **BRAF**, **RAS**). *Incorrect: Pancreatic cancer* - Although E-cadherin can be down-regulated in pancreatic cancer, it is not the principal genetic deficiency. - **Pancreatic ductal adenocarcinoma** typically involves mutations in **KRAS**, **TP53**, **SMAD4**, and **CDKN2A**.

Question 903: What is the most important prognostic factor of Wilms tumour?

A. Mutation of WT1 gene
B. Histopathology (Correct Answer)
C. Ploidy of cells
D. Age < 1 yr

Explanation: ***Histopathology*** - The presence of **anaplastic histology**, particularly diffuse anaplasia, is the most significant adverse prognostic factor in Wilms tumor. - Tumors with favorable histology (triphasic, blastemal, stromal, or epithelial predominant) have an excellent prognosis, while those with anaplastic features have significantly worse outcomes [1]. *Ploidy of cells* - While **aneuploidy** (specifically, **hyperdiploidy**) has been associated with improved prognosis in some childhood cancers, its role as an independent prognostic factor in Wilms tumor is less significant than histology [2]. - It is not the most important factor in determining the overall outcome. *Age < 1 yr* - **Younger age** (typically less than 1 year) at diagnosis is generally associated with a **more favorable prognosis** in Wilms tumor. - This is because these tumors are often smaller, less aggressive, and more likely to have favorable histology. *Mutation of WT1 gene* - **WT1 gene mutations** are implicated in the development of Wilms tumor, particularly in syndromes like WAGR (Wilms tumor, aniridia, genitourinary anomalies, intellectual disability). - While critical for pathogenesis, the mere presence of a WT1 mutation is **not the primary determinant** of prognosis compared to tumor histology. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 488-490. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 486-487.

Question 904: Which of the following is a key characteristic of virus-induced tumor cells?

A. Loss of orientation
B. Loss of contact inhibition in cells (Correct Answer)
C. Formation of microtumor cells
D. Change in size of cells

Explanation: ***Loss of contact inhibition in cells*** - **Contact inhibition** is a critical regulatory mechanism in normal cells, where cell growth and division are arrested upon contact with neighboring cells, preventing uncontrolled proliferation. - Virus-induced tumor cells often lose this inhibition, leading to **uncontrolled proliferation** and the formation of multi-layered foci in culture, a hallmark of their transformed state. *Loss of orientation* - While transformed cells may exhibit **disorganized growth patterns**, the primary functional hallmark of oncogenic transformation relevant to tumor formation is the uncontrolled proliferation, not merely a loss of physical orientation. - Loss of orientation is a general characteristic of many cell types under various conditions and lacks the specificity as a key characteristic of virus-induced tumor cells. *Formation of microtumor cells* - The term "microtumor cells" is not a standard characteristic or recognized cellular phenomenon describing virus-induced transformation. - Virus-induced tumor cells are known for their **uncontrolled growth** and ability to form macroscopic tumors, not specifically for forming uniquely "micro" tumor cells. *Change in size of cells* - While transformed cells can exhibit changes in cell size and morphology (e.g., pleomorphism), this is a general characteristic of many pathological processes and is not as defining of virus-induced tumor cells as the loss of contact inhibition. - Changes in cell size can occur due to various factors, including stress or metabolic alterations, and are not unique to oncogenic transformation.

Question 905: Squamous cell carcinoma of the esophagus is most commonly located at:

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

Explanation: ***Middle 1/3rd*** - **Squamous cell carcinoma** of the esophagus most frequently develops in the **middle segment** of the esophagus [1]. - This location accounts for approximately **50% of all esophageal squamous cell carcinomas** due to various risk factors acting on its lining [1]. - The middle third extends from approximately 24-32 cm from the incisors. *Upper 1/3rd* - While possible, squamous cell carcinomas in the **upper 1/3rd** of the esophagus are less common, representing about **15-20% of cases**. - Cancers in this region are often associated with different etiologies, such as **Plummer-Vinson syndrome** or post-cricoid web. *Lower 1/3rd* - The **lower 1/3rd** of the esophagus accounts for approximately **30-35% of squamous cell carcinomas**. - This region is also the most common site for **adenocarcinoma**, which is typically associated with Barrett's esophagus and GERD [1]. *Gastroesophageal junction* - The **gastroesophageal junction** is a distinct anatomical landmark at the distal-most portion of the esophagus where it meets the stomach. - **Adenocarcinoma** is the predominant histological type at this location, often arising from Barrett's esophagus. - While squamous cell carcinoma can extend to this area, it rarely arises as a primary tumor here. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, pp. 766-767.

Question 906: What is the earliest change of neoplastic transformation observed at the microscopic level?

A. Dysplasia (Correct Answer)
B. Atypical Hyperplasia
C. Invasive Carcinoma
D. Squamous Metaplasia

Explanation: ***Dysplasia*** - Dysplasia represents the **earliest microscopic changes** in neoplastic transformation, indicating **abnormal growth** or development of cells [1,4]. - It is characterized by changes in **cell shape, size, and organization**, often seen before the development of invasive cancer [1]. *Metaplasia* - Metaplasia involves the **replacement of one differentiated cell type** with another, often as an adaptation to chronic irritation or injury [1]. - While it can be a precursor to dysplasia, it does not represent the **initial cellular changes** indicative of neoplastic transformation. *Carcinoma insitu* - Carcinoma insitu represents a more advanced pre-invasive stage where abnormal cells are present but have not invaded **surrounding tissues** [3]. - It occurs after dysplastic changes and signifies a higher level of malignancy, not the **earliest changes** [3]. *Hyperplasia* - Hyperplasia is characterized by an **increase in the number of cells** but typically maintains normal cell structure and function [1]. - It is a reactive process and does not indicate neoplastic transformation, which is marked by **cellular atypia** seen in dysplasia. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, p. 723. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Female Genital Tract Disease, pp. 467-468. [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. 209-210.

Question 907: Which tumor suppressor gene is associated with familial gastric cancer and lobular breast carcinoma?

A. PTEN
B. APC
C. CDH1 (Correct Answer)
D. RB (Retinoblastoma)

Explanation: ***CDH1*** - The **CDH1** gene encodes for **E-cadherin**, a cell adhesion protein; mutations are strongly linked to **hereditary diffuse gastric cancer** and **lobular breast carcinoma**. - A germline mutation in **CDH1** significantly increases the lifetime risk for both these types of cancers. *RB (Retinoblastoma)* - The **RB gene** is a tumor suppressor gene primarily associated with **retinoblastoma** (a rare childhood eye cancer). - It is also implicated in other cancers like **osteosarcoma** and small cell lung cancer, but not directly familial gastric cancer or lobular breast carcinoma. *PTEN* - **PTEN** is a tumor suppressor gene associated with **Cowden syndrome**, which increases the risk of breast, thyroid, and endometrial cancers. - While it has broad tumor suppressor functions, it is not the primary gene associated with familial gastric cancer or lobular breast carcinoma. *APC* - The **APC gene** is a tumor suppressor gene famously associated with **familial adenomatous polyposis (FAP)**, which leads to numerous colon polyps and a high risk of colorectal cancer. - Mutations in APC are not characteristic of familial gastric cancer or lobular breast carcinoma.

Question 908: Most common type of sarcoma of the breast is?

A. Angiosarcoma (Correct Answer)
B. Kaposi sarcoma
C. Synovial sarcoma
D. Rhabdomyosarcoma

Explanation: ***Angiosarcoma*** - **Angiosarcoma** is the most frequent type of primary breast sarcoma, accounting for a significant portion of these rare tumors [1]. - It often arises de novo or as a complication of **radiation therapy** for breast cancer or chronic lymphedema (Stewart-Treves syndrome) [1]. *Kaposi sarcoma* - **Kaposi sarcoma** is a vascular tumor strongly associated with **HIV infection** or other forms of immunosuppression [4]. - While it can affect various organs, including the skin and lymph nodes, primary breast involvement is exceedingly rare and not considered the most common type of breast sarcoma [4]. *Synovial sarcoma* - **Synovial sarcoma** typically originates near large joints in the extremities, especially the lower extremities [3]. - It is a tumor of presumed synovial origin, and its occurrence in the breast is extremely uncommon, making it an unlikely most common type [3]. *Rhabdomyosarcoma* - **Rhabdomyosarcoma** is a malignant tumor of skeletal muscle origin, predominantly affecting children and adolescents in sites like the head and neck, genitourinary tract, or extremities [2]. - Its incidence in the breast is very rare, usually presenting as a metastatic lesion rather than a primary breast sarcoma [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 527-528. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1224-1225. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1225-1226. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 526-527.

Question 909: Most common benign tumor of the stomach is?

A. Lipoma
B. Hamartoma
C. Adenoma
D. Leiomyoma (Correct Answer)

Explanation: ***Leiomyoma*** - **Leiomyomas** are the most frequently encountered benign tumors of the stomach, originating from the **smooth muscle layer** (muscularis propria) of the gastric wall [1]. - They are typically asymptomatic but can present with symptoms like **bleeding** or abdominal pain if large or ulcerated. *Adenoma* - Gastric adenomas are less common than leiomyomas and are considered **premalignant lesions**, often arising in the setting of chronic gastritis or intestinal metaplasia [1]. - They are characterized by **glandular dysplasia** and carry a risk of progression to adenocarcinoma [1]. *Lipoma* - **Lipomas** are benign tumors composed of **adipose tissue** and are relatively rare in the stomach compared to other benign gastric tumors. - They usually grow in the submucosal layer and are often asymptomatic unless they become large enough to cause obstruction or bleeding. *Hamartoma* - Gastric **hamartomas** are uncommonly found in the stomach and represent a disorganized overgrowth of mature tissue components normally present in the stomach wall. - They are benign but can be associated with certain **syndromes** like Peutz-Jeghers syndrome. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Alimentary System Disease, pp. 354-355.

Question 910: A 3-year-old boy is found to have spontaneous bursts of non-rhythmic conjugate eye movements in various directions, as well as hypotonia and myoclonus. Physical examination also reveals an abdominal mass. A CT scan shows a mass in the adrenal gland. Which of the following statements is false regarding the patient's condition?

A. Immunohistochemical detection of chromogranin is not useful for diagnosis. (Correct Answer)
B. 70-80% of tumors are associated with elevated production of catecholamines.
C. Rearrangement or deletion of short arm of chromosome 1 is seen in 25-35% of cases.
D. The most common site of tumor is adrenal medulla.

Explanation: ***Immunohistochemical detection of chromogranin is not useful for diagnosis.*** - This statement is **false** because **neuroblastoma** cells, which originate from neural crest cells, commonly express **chromogranin A** and C, along with other neuroendocrine markers like **synaptophysin** and **neuron-specific enolase (NSE)** [1]. - **Immunohistochemical staining** for chromogranin is thus a **useful diagnostic tool** to confirm the neuroendocrine differentiation of the tumor [1]. *The most common site of tumor is adrenal medulla.* - This statement is **true**. Approximately **50% of neuroblastomas** originate in the **adrenal glands**, specifically the adrenal medulla, because it is derived from neural crest cells, the precursor cells for neuroblastoma [1]. - Other common sites include the paraspinal ganglia, such as the posterior mediastinum, pelvis, and neck. *70-80% of tumors are associated with elevated production of catecholamines.* - This statement is **true**. Neuroblastoma cells often retain the ability to synthesize and secrete **catecholamines** (**epinephrine, norepinephrine, dopamine**) [1]. - Elevated levels of **vanillylmandelic acid (VMA)** and **homovanillic acid (HVA)**, which are the **breakdown products (metabolites) of catecholamines**, are detected in the urine of 70-80% of patients and serve as **important diagnostic and prognostic markers** [1]. *Rearrangement or deletion of short arm of chromosome 1 is seen in 25-35% of cases.* - This statement is **true**. **Deletion** or **rearrangement** of the **short arm of chromosome 1 (1p36)** is a common **cytogenetic abnormality** found in 25-35% of neuroblastomas. - This genetic alteration is often associated with **poor prognosis** and more aggressive disease behavior. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-487.

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Neoplasia — MCQs

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