Neoplasia Practice Questions

Q: All are good prognostic factors in Neuroblastoma except

Trk B expression. ***Trk B expression*** - **Trk B expression** is associated with poor prognosis and increased tumor aggressiveness in neuroblastoma. - Neuroblastomas expressing Trk B often show **MYCN amplification** and are more likely to have advanced disease at diagnosis [1]. *Stage 2* - **Stage 2 neuroblastoma** generally represents a localized tumor with complete gross resection, and often indicates a favorable outcome. - It differs from Stages 3 and 4 which involve more extensive local invasion or metastatic disease. *Trk A expression* - **Trk A expression** is a well-established marker for favorable prognosis in neuroblastoma. - Tumors expressing TrkA often undergo spontaneous differentiation and have a higher likelihood of regression [2]. *Age of 1 year* - An **age less than 12-18 months** at diagnosis is a strong independent predictor of favorable prognosis in neuroblastoma [1]. - Younger patients often have less aggressive tumors and respond better to therapy. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 486-487. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-484.

Q: A small nodule of well-developed and normally organized pancreatic tissue found in the submucosa of the small intestine is an example of __________.

Choristoma. ***Choristoma*** - A **choristoma** is a normal tissue found in an abnormal location, such as pancreatic tissue in the submucosa of the small intestine. - These are typically benign and represent a developmental anomaly where a focus of normal cells develops in an ectopic site. *Papilloma* - A **papilloma** is a benign epithelial tumor growing exophytically (outwardly) in finger-like projections. - It usually arises from the surface epithelium and is not characterized by the presence of normal tissue in an abnormal location. *Hamartoma* - A **hamartoma** is a benign, localized malformation of mature differentiated cells and tissues that are native to the organ in which it arises, but are disorganized and present in abnormal proportions. - While it involves normal tissue components, they are disorganized and typically found in the correct organ, not in an entirely different organ as described. *Teratoma* - A **teratoma** is a tumor composed of several different types of tissue, such as hair, muscle, teeth, or bone, derived from more than one germ layer. - These are typically much more complex and involve tissues not normally associated with the location of development, and often occur in gonads.

Q: Which of the following is not a premalignant condition?

Fragile X syndrome. ***Fragile X syndrome*** - This is a genetic condition causing **intellectual disability** and developmental problems, primarily due to an **FRM1 gene mutation** leading to a fragile site on the X chromosome [1]. - It is **not associated with an increased risk of malignancy** and is therefore not considered a premalignant condition. *Bloom's syndrome* - This is a rare genetic disorder characterized by **growth deficiency**, a **photosensitive rash**, and a significantly **increased risk of various cancers**, including leukemias, lymphomas, and solid tumors. - It is associated with a mutation in the **BLM gene**, which plays a role in DNA repair and replication, leading to chromosomal instability. *Down's syndrome* - Individuals with Down's syndrome (trisomy 21) have a **20-fold increased risk of acute lymphoblastic leukemia (ALL)** and a 10-fold increased risk of acute myeloid leukemia (AML), particularly transient myeloproliferative disorder in infancy. - While it is a developmental disorder, the increased incidence of specific cancers classifies it as a **premalignant condition**. *Fanconi anemia* - This is a rare genetic disorder characterized by **bone marrow failure**, physical abnormalities, and an extraordinarily high risk of developing **acute myeloid leukemia (AML)** and myelodysplastic syndrome (MDS). - It is caused by mutations in genes involved in **DNA repair**, making cells highly susceptible to DNA damage and cancer.

Q: LMP 1 gene plays a role in oncogenesis induced by

Epstein Barr virus. ***Epstein Barr virus*** - The **LMP1 (Latent Membrane Protein 1)** gene is a key **oncogene** encoded by the **Epstein-Barr virus (EBV)**. - LMP1 acts as a **constitutively active receptor**, mimicking CD40 and leading to the activation of several cellular signaling pathways (**NF-κB, JNK/p38, PI3K/Akt**) crucial for cell proliferation, survival, and differentiation. *Hepatitis B virus* - **Hepatitis B virus (HBV)** is associated with **hepatocellular carcinoma (HCC)**, but its oncogenic mechanisms primarily involve the **HBx protein**, which deregulates cell cycles and impacts host gene expression. - HBV does not encode an LMP1 equivalent; its oncogenesis is linked to chronic inflammation, hepatocyte regeneration, and integration of viral DNA. *Human T cell leukemia virus type 1* - **Human T-cell leukemia virus type 1 (HTLV-1)** is linked to **Adult T-cell Leukemia/Lymphoma (ATL)**, with its primary oncogene being **Tax**. - **Tax protein** activates NF-κB and other pro-proliferative pathways, but HTLV-1 does not have an LMP1 gene. *Human papilloma virus* - **Human papillomavirus (HPV)** is responsible for cervical and other anogenital cancers, with major oncogenes being **E6 and E7**. - **E6 targets p53** for degradation, and **E7 inactivates Rb**, leading to uncontrolled cell division; HPV does not encode an LMP1 gene.

Q: Tumor NOT commonly seen in first decade of life?

Ameloblastoma. ***Ameloblastoma*** - Ameloblastomas are **odontogenic tumors** that typically affect adults between the **third and seventh decades of life**, making them uncommon in the first decade. - Symptoms include **painless swelling** in the jaw, facial asymmetry, and malocclusion, usually presenting much later in life. *Neuroblastoma* - **Neuroblastoma** is a common tumor of early childhood, with the majority of diagnoses occurring before the **age of 5** [1], [2]. - It originates from **neural crest cells** and can arise in the adrenal glands, abdomen, chest, or neck [1], [2]. *Wilm’s tumor* - **Wilm's tumor (nephroblastoma)** is a kidney cancer that most commonly affects children between the **ages of 3 and 4**, making it prevalent in the first decade of life [1], [2]. - It often presents as an **abdominal mass**, abdominal pain, or hematuria. *Retinoblastoma* - **Retinoblastoma** is a malignant tumor of the retina that almost exclusively affects young children, typically diagnosed before the **age of 5** [1], [2]. - Key signs include **leukocoria** (white pupillary reflex) and strabismus [3]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-484. [2] 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. 211-212. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 737-738.

Q: Skeletal metastasis is common in:

Cancer breast. ***Cancer breast*** - **Breast cancer** is one of the most common primary malignancies that metastasize to bones (part of the classic **"osteophilic pentad"**: breast, prostate, lung, kidney, and thyroid) [1]. - Preferentially metastasizes to **axial skeleton** including the **spine**, **pelvis**, and **ribs**. - Bone metastases from breast cancer can be **osteolytic**, **osteoblastic**, or mixed, often causing pain and **pathological fractures**. - Approximately **70% of patients with advanced breast cancer** develop bone metastases. *Hepatoma* - **Hepatocellular carcinoma (HCC)**, or hepatoma, commonly metastasizes to the **lungs** and regional lymph nodes via hematogenous spread [2]. - While bone metastases can occur, they are **uncommon** (occurs in <5% of cases), much less frequent than with breast, prostate, or lung cancers [1]. - Bone involvement often indicates advanced disease. *Cancer stomach* - **Gastric cancer** primarily metastasizes to nearby **lymph nodes**, the **liver**, and the **peritoneum** (Krukenberg tumor to ovaries, Sister Mary Joseph nodule). - Bone metastases from gastric cancer are **relatively rare**, occurring in less than 10-15% of cases, and represent a poor prognostic sign. - Not among the common cancers with bone tropism [1]. *Cancer pancreas* - **Pancreatic cancer** frequently metastasizes to the **liver** (most common), **peritoneum**, and **lungs**. - Bone metastases are **uncommon** in pancreatic cancer (<5% of cases), typically indicating widespread disease and a very poor prognosis. - Not part of the osteophilic group of cancers [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 671-672. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, p. 282.

Q: All of the following are associated with malignancy except:

Fragile X syndrome. ***Fragile X syndrome*** - Fragile X syndrome is primarily associated with **intellectual disability** and developmental delays, not an increased risk of malignancy [1]. - It is caused by an expansion of the **CGG repeat** in the FMR1 gene, leading to reduced or absent FMRP protein. *Down syndrome* - Individuals with Down syndrome (Trisomy 21) have a significantly increased risk of developing certain cancers, particularly **acute lymphoblastic leukemia (ALL)** and **acute myeloid leukemia (AML)**. - This increased leukemia risk is several times higher than in the general population. *Fanconi anemia* - Fanconi anemia is a rare genetic disorder characterized by **bone marrow failure** and an increased predisposition to various cancers. - Patients are at a higher risk for **acute myeloid leukemia (AML)** and **myelodysplastic syndromes (MDS)**, as well as head and neck squamous cell carcinomas. *Bloom syndrome* - Bloom syndrome is a very rare genetic disorder associated with an exceptionally high risk of developing a wide range of cancers early in life. - This increased susceptibility is due to **genomic instability** and a high frequency of chromosomal rearrangements.

Question 1

All are good prognostic factors in Neuroblastoma except

Accepted Answer

Trk B expression

Answer

Stage 2

Answer

Trk A expression

Answer

Age of 1 year

Question 2

All of the following are small round blue cell tumours except

Accepted Answer

Wilms tumour

Answer

Ewing's sarcoma

Answer

Rhabdomyosarcoma

Answer

Retinoblastoma

Question 3

A small nodule of well-developed and normally organized pancreatic tissue found in the submucosa of the small intestine is an example of __________.

Accepted Answer

Choristoma

Answer

Papilloma

Answer

Hamartoma

Answer

Teratoma

Question 4

Which of the following is not a premalignant condition?

Accepted Answer

Fragile X syndrome

Answer

Bloom's syndrome

Answer

Down's syndrome

Answer

Fanconi anemia

Question 5

LMP 1 gene plays a role in oncogenesis induced by

Accepted Answer

Epstein Barr virus

Answer

Hepatitis B virus

Answer

Human T cell leukemia virus type 1

Answer

Human papilloma virus

Question 6

Which of the following is not a characteristic of malignant lesions?

Accepted Answer

Pear-shaped

Answer

Absence of encapsulation

Answer

Ulcerated borders

Answer

Ill-defined borders

Question 7

Tumor NOT commonly seen in first decade of life?

Accepted Answer

Ameloblastoma

Answer

Neuroblastoma

Answer

Wilm’s tumor

Answer

Retinoblastoma

Question 8

Skeletal metastasis is common in:

Accepted Answer

Cancer breast

Answer

Hepatoma

Answer

Cancer stomach

Answer

Cancer pancreas

Question 9

All of the following are associated with malignancy except:

Accepted Answer

Fragile X syndrome

Answer

Down syndrome

Answer

Fanconi anemia

Answer

Bloom syndrome

Question 10

What is an example of radiation-induced cancer?

Accepted Answer

Papillary carcinoma of thyroid

Answer

Hepatoma

Answer

Lymphoma

Answer

Follicular carcinoma of thyroid

Neoplasia — MCQs

Neoplasia — MCQs

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