Neoplasia Practice Questions

Q: A newborn child is noted to have a bulky abdominal tumor. CT scans reveal that the mass involves the right abdomen and retroperitoneum. The tumor is resected, revealing ganglion cells and primitive, small, round cells occasionally organized in rosettes, embedded in a fibrillary pink matrix. Special studies confirm the likely diagnosis. Which of the following features of this tumor is associated with a poorer prognosis?

Amplification of N-myc gene. ***Amplification of N-myc gene*** - **N-myc (MYCN) gene amplification** is the most critical adverse prognostic factor in **neuroblastoma**, indicating a more aggressive tumor with rapid growth and poor response to therapy [1]. - Its presence is associated with **increased likelihood of metastasis**, treatment failure, and poor survival regardless of stage [1]. - MYCN amplification is found in ~20-25% of neuroblastomas and is an independent predictor of poor outcome. *Advanced stage at diagnosis* - While advanced stage (stage 3 or 4) generally correlates with a poorer prognosis, **N-myc amplification** is an independent molecular marker that is a stronger predictor of outcome [1]. - Tumors with MYCN amplification can have an aggressive course even at lower stages, and conversely, some high-stage tumors without MYCN amplification may have a better outcome. *Numerous ganglion cells* - The presence of numerous **ganglion cells** indicates a more differentiated tumor type, such as a **ganglioneuroma** or **ganglioneuroblastoma** (intermixed type). - Increased differentiation is generally associated with a **better prognosis** in neuroblastic tumors, representing favorable histology. *Cellular aneuploidy* - In neuroblastoma, **hyperdiploidy** (DNA index >1, with more than the diploid number of chromosomes) is associated with a **favorable prognosis**, particularly in infants [1]. - In contrast, **diploid tumors** (DNA index = 1) tend to have a worse prognosis [1]. - The term "cellular aneuploidy" alone is ambiguous; the prognostic significance depends on whether it refers to hyperdiploidy (favorable) or diploidy (unfavorable). **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.

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

A newborn child is noted to have a bulky abdominal tumor. CT scans reveal that the mass involves the right abdomen and retroperitoneum. The tumor is resected, revealing ganglion cells and primitive, small, round cells occasionally organized in rosettes, embedded in a fibrillary pink matrix. Special studies confirm the likely diagnosis. Which of the following features of this tumor is associated with a poorer prognosis?

Accepted Answer

Amplification of N-myc gene

Answer

Advanced stage at diagnosis

Answer

Numerous ganglion cells

Answer

Cellular aneuploidy

Question 2

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 3

All of the following are small round blue cell tumours except

Accepted Answer

Wilms tumour

Answer

Ewing's sarcoma

Answer

Rhabdomyosarcoma

Answer

Retinoblastoma

Question 4

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 5

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 6

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 7

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 8

Tumor NOT commonly seen in first decade of life?

Accepted Answer

Ameloblastoma

Answer

Neuroblastoma

Answer

Wilm’s tumor

Answer

Retinoblastoma

Question 9

Skeletal metastasis is common in:

Accepted Answer

Cancer breast

Answer

Hepatoma

Answer

Cancer stomach

Answer

Cancer pancreas

Question 10

All of the following are associated with malignancy except:

Accepted Answer

Fragile X syndrome

Answer

Down syndrome

Answer

Fanconi anemia

Answer

Bloom syndrome

Neoplasia — MCQs

Neoplasia — MCQs

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