Neoplasia Practice Questions

Q: Which of the following is the most common tumor of the fetus and newborn?

Sacrococcygeal teratoma. **Explanation:** **Sacrococcygeal Teratoma (SCT)** is the most common tumor of the fetus and newborn [1], occurring in approximately 1 in 20,000 to 40,000 live births [1]. These tumors arise from the **totipotent cells** of the primitive streak (Hensen’s node) that fail to migrate or involute. They are more common in females (4:1 ratio) [1]. While most are benign (mature), the risk of malignancy increases with age at diagnosis. **Analysis of Incorrect Options:** * **Neuroblastoma:** This is the most common **extracranial solid tumor of childhood** and the most common malignancy in infants (under 1 year), but it is not more frequent than SCT in the immediate neonatal/fetal period. * **Wilms Tumor (Nephroblastoma):** This is the most common **primary renal tumor of childhood**, typically presenting between ages 2 and 5. It is rare in the neonatal period. * **Leukemia:** While leukemia is the most common childhood cancer overall, it is not the most common tumor at birth. Congenital leukemia is a rare entity. **High-Yield Clinical Pearls for NEET-PG:** * **Altman Classification:** Used for SCT based on the location (Type I is predominantly external; Type IV is entirely presacral/internal). * **Tumor Markers:** Elevated **Alpha-fetoprotein (AFP)** in a teratoma often suggests a yolk sac component (malignancy). * **Associated Complication:** Large fetal SCTs can cause **high-output cardiac failure** and hydrops fetalis due to significant vascular shunting (steal phenomenon). * **Most common malignancy in infants:** Neuroblastoma. * **Most common childhood cancer:** Acute Lymphoblastic Leukemia (ALL). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 482-483.

Q: Which of the following malignancies is associated with the characteristic Zellballen pattern on histopathology?

Carotid body tumor. **Explanation:** The correct answer is **C. Carotid body tumor**. **1. Why Carotid Body Tumor is correct:** A carotid body tumor is a type of **paraganglioma** (extra-adrenal pheochromocytoma) [1]. The characteristic histopathological hallmark of paragangliomas is the **Zellballen pattern**. This consists of nests or clusters of round-to-oval neuroendocrine cells (Chief cells) surrounded by a delicate vascular stroma and peripheral spindle-shaped sustentacular cells. The term "Zellballen" is German for "cell balls." **2. Why other options are incorrect:** * **A. Gastrointestinal stromal tumor (GIST):** Characterized by bundles of spindle cells or epithelioid cells. The diagnostic marker is **CD117 (c-KIT)**. * **B. Astrocytoma:** Low-grade astrocytomas show a fibrillary background; high-grade (GBM) shows pseudopalisading necrosis. They do not form nested patterns. * **C. Retinoblastoma:** Characterized by **Flexner-Wintersteiner rosettes** (true rosettes with a central lumen) and Homer Wright rosettes. **3. High-Yield Clinical Pearls for NEET-PG:** * **Location:** Carotid body tumors occur at the bifurcation of the common carotid artery [1]. * **Clinical Sign:** **Fontaine’s Sign** – The mass is mobile horizontally but fixed vertically (due to its attachment to the carotid artery). * **Immunohistochemistry (IHC):** Chief cells are positive for **Synaptophysin/Chromogranin**, while Sustentacular cells are positive for **S-100**. * **Rule of 10s:** While traditionally associated with pheochromocytoma, remember that carotid body tumors are the most common head and neck paragangliomas [1]. * **Salt and Pepper Chromatin:** A common feature of neuroendocrine tumors, including the cells within the Zellballen nests. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Head and Neck, pp. 748-749.

Q: MIC-2 is a marker of?

Ewing sarcoma. **Explanation:** **MIC-2** (also known as **CD99**) is a cell surface glycoprotein that is highly characteristic of the **Ewing Sarcoma/Primitive Neuroectodermal Tumor (PNET)** family. It is encoded by the *MIC2* gene located on the pseudoautosomal region of the X and Y chromosomes. 1. **Why Ewing Sarcoma is Correct:** In Ewing Sarcoma, MIC-2/CD99 shows a distinctive **strong, diffuse, membranous staining** pattern on immunohistochemistry (IHC). While not 100% specific, it is a highly sensitive marker used to differentiate Ewing sarcoma from other "small round blue cell tumors" of childhood. 2. **Why Other Options are Incorrect:** * **Chronic Lymphocytic Leukemia (CLL):** The hallmark markers for CLL are **CD5, CD19, CD20, and CD23**. MIC-2 is not used for its diagnosis. * **Mantle Cell Lymphoma (MCL):** MCL is characterized by the overexpression of **Cyclin D1** (due to t(11;14)) and markers like **CD5** and **SOX11** [1]. 3. **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** Ewing Sarcoma is most commonly associated with **t(11;22)(q24;q12)**, leading to the **EWS-FLI1** fusion gene. * **Radiology:** Classically presents with an **"onion-skin"** periosteal reaction. * **Morphology:** Small round blue cells with scant cytoplasm; **Homer-Wright rosettes** may be seen (indicating neural differentiation). * **Differential Diagnosis:** Other CD99-positive tumors include lymphoblastic lymphoma and synovial sarcoma, but in the context of bone tumors, it strongly points to Ewing's. **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. 610-612.

Question 1

Malignancies associated with BRCA 1 and 2 include all of the following except?

Accepted Answer

Mesothelioma

Answer

Breast cancer

Answer

Prostate cancer

Answer

Papillary serous carcinoma of peritoneum

Question 2

Which gene is involved in Retinoblastoma?

Accepted Answer

13Q14

Answer

12P13

Answer

13P14

Answer

12Q13

Question 3

Russel bodies are seen in which of the following conditions?

Accepted Answer

Multiple Myeloma

Answer

Rabies

Answer

Parkinsonism

Answer

Intracranial neoplasm

Question 4

Which of the following is the most common tumor of the fetus and newborn?

Accepted Answer

Sacrococcygeal teratoma

Answer

Neuroblastoma

Answer

Wilm's tumor

Answer

Leukemia

Question 5

Tumor arising from a totipotent cell is:

Accepted Answer

Teratoma

Answer

Seminoma

Answer

Myoma

Answer

Lipoma

Question 6

Which of the following malignancies is associated with the characteristic Zellballen pattern on histopathology?

Accepted Answer

Carotid body tumor

Answer

Gastrointestinal stromal tumor

Answer

Astrocytoma

Answer

Retinoblastoma

Question 7

Which tumor suppressor gene is primarily responsible for lung squamous cell carcinoma?

Accepted Answer

p53

Answer

Rb

Answer

PTEN

Answer

p63

Question 8

Microdeletion leading to instability of DNA, causing the cell to undergo degeneration, is seen as a marker in which of the following cancers?

Accepted Answer

Colon carcinoma

Answer

Medullary carcinoma of thyroid

Answer

Small cell lung cancer

Answer

Gastric lymphoma

Question 9

MIC-2 is a marker of?

Accepted Answer

Ewing sarcoma

Answer

Chronic lymphocytic leukemia

Answer

Mantle cell lymphoma

Answer

All of these

Question 10

Smoking is most commonly associated with which of the following carcinomas?

Accepted Answer

Urinary bladder carcinoma

Answer

Liver carcinoma

Answer

Gall bladder carcinoma

Answer

Stomach carcinoma

Neoplasia — MCQs

Neoplasia — MCQs

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