Endocrine Pathology Practice Questions

Q: In which of the following conditions are 'Zellballens' typically seen?

Pheochromocytoma. **Explanation:** **1. Why Pheochromocytoma is correct:** 'Zellballen' (German for "cell balls") is the characteristic histological pattern seen in **Pheochromocytoma** and **Paragangliomas** [1]. This pattern consists of nests or clusters of large, polygonal chromaffin cells surrounded by a delicate vascular stroma and a peripheral layer of spindle-shaped **sustentacular cells** [1]. These cells contain granular cytoplasm due to the presence of catecholamine-containing membrane-bound vesicles [1]. **2. Why the other options are incorrect:** * **Gastric Carcinoma:** Typically presents with a glandular pattern (adenocarcinoma) or **Signet ring cells** (in diffuse types), where the nucleus is pushed to the periphery by a large mucin vacuole. * **Angiosarcoma:** A malignant vascular tumor characterized by anastomosing vascular channels lined by atypical, pleomorphic endothelial cells. * **Colon Carcinoma:** Characterized by malignant glands invading the basement membrane, often showing "dirty necrosis" (central luminal necrotic debris). **3. High-Yield Clinical Pearls for NEET-PG:** * **Rule of 10s for Pheochromocytoma:** 10% are bilateral, 10% are malignant, 10% are extra-adrenal (Paragangliomas), and 10% occur in children. * **Staining:** Sustentacular cells are positive for **S-100**, while the chief cells are positive for **Chromogranin** and **Synaptophysin**. * **Clinical Triad:** Episodic headache, sweating (diaphoresis), and tachycardia/palpitations in a hypertensive patient. * **Genetic Associations:** Often associated with MEN 2A, MEN 2B, VHL syndrome, and NF-1 [2]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 419-420. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, p. 1137.

Q: All are true regarding MEN-I syndrome, except?

Autosomal recessive inheritance. **Explanation:** **MEN-I Syndrome (Wermer Syndrome)** is a hereditary cancer syndrome characterized by tumors of the "3 Ps": **P**arathyroid, **P**ancreas (Islet cells), and **P**ituitary [1]. 1. **Why Option A is the Correct Answer (The Exception):** MEN-I syndrome follows an **Autosomal Dominant** inheritance pattern, not autosomal recessive. A single mutated copy of the *MEN1* gene inherited from a parent is sufficient to predispose an individual to tumor development (following Knudson’s "two-hit" hypothesis at the cellular level). 2. **Analysis of Other Options:** * **Option B (Hyperparathyroidism):** This is the most common manifestation (80–95% of cases). It typically presents as multiglandular parathyroid hyperplasia and is often the first clinical sign of the syndrome [1]. * **Option C (Islet cell tumor):** Pancreatic neuroendocrine tumors (NETs) are the leading cause of morbidity and mortality in MEN-I. Common types include Gastrinomas (Zollinger-Ellison Syndrome) and Insulinomas [1]. * **Option D (Chromosome 11):** The *MEN1* gene is a tumor suppressor gene located on the long arm of **chromosome 11 (11q13)** [1]. It encodes the protein **Menin**. **High-Yield Clinical Pearls for NEET-PG:** * **The 3 Ps:** Parathyroid (Hyperplasia), Pancreas (Islet cell tumors/NETs), and Pituitary (most commonly Prolactinoma) [1]. * **Genetic Marker:** Mutation in the *MEN1* gene on Chromosome 11q13 [1]. * **Associated Lesions:** Patients may also present with angiofibromas, collagenomas, and lipomas. * **Contrast with MEN-II:** MEN-II involves the *RET* proto-oncogene (Chr 10) and is characterized by Medullary Thyroid Carcinoma and Pheochromocytoma [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1104-1140.

Q: Which chromosomal translocation is characteristic of follicular carcinoma?

PAX8 - PPAR. **Explanation:** **Correct Option: A (PAX8 - PPAR̳)** Follicular Thyroid Carcinoma (FTC) is characterized by a specific chromosomal translocation, **t(2;3)(q13;p25)**. This results in the fusion of the **PAX8** gene (a transcription factor for thyroid development) and the **PPAR̳** (peroxisome proliferator-activated receptor gamma) gene. This fusion protein acts as an oncogene by interfering with normal thyroid differentiation and promoting uncontrolled cell proliferation. It is found in approximately 30–60% of follicular carcinomas. **Incorrect Options:** * **B. RET - PTC:** This rearrangement is characteristic of **Papillary Thyroid Carcinoma (PTC)**, specifically the classic and tall-cell variants [1]. It involves the fusion of the RET proto-oncogene with the PTC gene. * **C. ALK - NPM1:** The **t(2;5)** translocation involving NPM1 and ALK is the hallmark of **Anaplastic Large Cell Lymphoma (ALCL)**, not thyroid pathology. * **D. JAK - TEL:** This is not a standard translocation for thyroid cancers. (Note: ETV6-NTRK3 fusions, sometimes confused with TEL, are seen in secretory breast cancer and some thyroid variants). **High-Yield Clinical Pearls for NEET-PG:** * **RAS Mutations:** These are also common in Follicular Carcinoma (and Follicular Adenomas), highlighting the difficulty in distinguishing benign from malignant lesions on FNA [1]. * **Diagnosis:** FTC cannot be diagnosed by Fine Needle Aspiration (FNA) because the diagnosis requires evidence of **capsular or vascular invasion**, which can only be seen on histology [1]. * **Spread:** Unlike Papillary Carcinoma (lymphatic spread), Follicular Carcinoma spreads **hematogenously**, commonly to the bone and lungs. * **Iodine:** FTC is more common in areas of **dietary iodine deficiency** [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1097-1100.

Question 1

Pheochromocytomas arise from:

Accepted Answer

Neuroendocrine cells of the adrenal medulla

Answer

Nonendocrine cells of the adrenal cortex

Answer

Nonendocrine cells of the adrenal medulla

Answer

Neuroendocrine cells of the adrenal cortex

Question 2

What is the tumor marker for Medullary Carcinoma of the thyroid?

Accepted Answer

Calcitonin

Answer

TSH

Answer

T3, T4 and TSH

Answer

Alpha Feto protein

Question 3

Sheehan syndrome is due to:

Accepted Answer

Pituitary hemorrhage

Answer

Adrenal hemorrhage

Answer

Hypothalamic hemorrhage

Answer

Pancreatic hemorrhage

Question 4

Psammoma bodies are characteristically found in which of the following organs?

Accepted Answer

Thyroid

Answer

Stomach

Answer

Heart

Answer

Lungs

Question 5

In which of the following conditions are 'Zellballens' typically seen?

Accepted Answer

Pheochromocytoma

Answer

Gastric carcinoma

Answer

Angiosarcoma

Answer

Colon carcinoma

Question 6

Which of the following is true about anaplastic carcinoma of the thyroid?

Accepted Answer

Common in elderly

Answer

Surrounding neck tissues are free

Answer

Lymphatic infiltration occurs

Answer

p53 mutation

Question 7

Hürthle cells are seen in which of the following conditions?

Accepted Answer

Hashimoto thyroiditis

Answer

Papillary carcinoma

Answer

Granulomatous thyroiditis

Answer

Thyroglossal cyst

Question 8

All are true regarding MEN-I syndrome, except?

Accepted Answer

Autosomal recessive inheritance

Answer

Hyperparathyroidism

Answer

Islet cell tumor

Answer

Association with chromosome 11

Question 9

What is the commonest thyroid tumor in Multiple Endocrine Neoplasia (MEN)?

Accepted Answer

Medullary

Answer

Follicular

Answer

Papillary

Answer

Anaplastic

Question 10

Which chromosomal translocation is characteristic of follicular carcinoma?

Accepted Answer

PAX8 - PPAR

Answer

RET - PTC

Answer

ALK -NMP1

Answer

JAK - TEL

Endocrine Pathology — MCQs

Endocrine Pathology — MCQs

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