Neoplasia Practice Questions

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

Histopathology. ***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.

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

Gastric cancer. **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**.

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

CEA. ***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.

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

Loss of contact inhibition in cells. ***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.

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

Middle 1/3rd. ***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.

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

CDH1. ***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.

Q: Most common type of sarcoma of the breast is?

Angiosarcoma. ***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.

Q: Lethal midline granuloma arises from which cell type?

NK cells. ***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.

Q: 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?

Immunohistochemical detection of chromogranin is not useful for diagnosis.. ***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.

Question 1

What is the most important prognostic factor of Wilms tumour?

Accepted Answer

Histopathology

Answer

Mutation of WT1 gene

Answer

Ploidy of cells

Answer

Age < 1 yr

Question 2

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

Accepted Answer

Gastric cancer

Answer

Intestinal cancer

Answer

Pancreatic cancer

Answer

Thyroid cancer

Question 3

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

Accepted Answer

CEA

Answer

hCG

Answer

AFP

Answer

CA-15-3

Question 4

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

Accepted Answer

Loss of contact inhibition in cells

Answer

Loss of orientation

Answer

Formation of microtumor cells

Answer

Change in size of cells

Question 5

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

Accepted Answer

Middle 1/3rd

Answer

Upper 1/3rd

Answer

Lower 1/3rd

Answer

Gastroesophageal junction

Question 6

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

Accepted Answer

Dysplasia

Answer

Atypical Hyperplasia

Answer

Invasive Carcinoma

Answer

Squamous Metaplasia

Question 7

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

Accepted Answer

CDH1

Answer

PTEN

Answer

APC

Answer

RB (Retinoblastoma)

Question 8

Most common type of sarcoma of the breast is?

Accepted Answer

Angiosarcoma

Answer

Kaposi sarcoma

Answer

Synovial sarcoma

Answer

Rhabdomyosarcoma

Question 9

Lethal midline granuloma arises from which cell type?

Accepted Answer

NK cells

Answer

B-cells

Answer

T-cells

Answer

Macrophages

Question 10

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?

Accepted Answer

Immunohistochemical detection of chromogranin is not useful for diagnosis.

Answer

70-80% of tumors are associated with elevated production of catecholamines.

Answer

Rearrangement or deletion of short arm of chromosome 1 is seen in 25-35% of cases.

Answer

The most common site of tumor is adrenal medulla.

Neoplasia — MCQs

Neoplasia — MCQs

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