Endocrine Pathology Practice Questions

Q: Which tumor arises from the organ of Zuckerkandl?

Paraganglioma. **Explanation:** **Correct Answer: A. Paraganglioma** The **Organ of Zuckerkandl** is a collection of extra-adrenal chromaffin tissue derived from the neural crest [1]. It is typically located at the origin of the inferior mesenteric artery or near the aortic bifurcation [1]. A tumor arising from these extra-adrenal chromaffin cells is termed a **Paraganglioma** [1]. While histologically identical to a Pheochromocytoma (which arises from the adrenal medulla), the term Paraganglioma is specifically used for extra-adrenal sites [1]. The Organ of Zuckerkandl is the most common site for extra-adrenal paragangliomas [1]. **Why other options are incorrect:** * **B. Schwannoma:** These are benign nerve sheath tumors arising from Schwann cells, not chromaffin tissue. * **C. Astrocytoma:** These are primary central nervous system (CNS) tumors arising from astrocytes (glial cells). * **D. Medulloblastoma:** This is a highly malignant primitive neuroectodermal tumor (PNET) located in the cerebellum (posterior fossa) of children [2]. **NEET-PG High-Yield Pearls:** * **Rule of 10s for Pheochromocytoma:** 10% are extra-adrenal (Paragangliomas), 10% are bilateral, 10% are malignant, and 10% occur in children. * **Zuckerkandl Clinical Presentation:** Like pheochromocytomas, these can secrete catecholamines, leading to the classic triad of episodic headache, sweating, and palpitations (hypertension) [1]. * **Staining:** Paragangliomas are positive for **Chromogranin** and **Synaptophysin**. The characteristic histological pattern is the **"Zellballen" appearance** (nests of cells surrounded by vascular stroma). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Head and Neck, pp. 748-749. [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: Which of the following statements is false regarding pituitary adenoma?

Reticular stain is positive with pituitary adenoma.. **Explanation:** Pituitary adenomas are benign neoplasms of the anterior pituitary and represent the most frequent cause of hyperpituitarism [1]. Understanding the histological architecture is key to distinguishing them from normal pituitary tissue. **Why Option B is False (The Correct Answer):** In a normal pituitary gland, cells are arranged in small nests or "acini" surrounded by a robust **reticulin network**. In a **pituitary adenoma**, this reticulin framework is **disrupted or absent**. Therefore, a reticulin stain will show a **loss of the normal reticular pattern**, making the statement "reticular stain is positive" (implying a preserved/normal pattern) histologically incorrect. This loss of reticulin is a diagnostic hallmark used to differentiate an adenoma from hyperplasia. **Analysis of Other Options:** * **Option A:** This is a **true** statement. Pituitary adenomas (especially functional ones like prolactinomas) are the leading cause of excess hormone production from the anterior pituitary [1]. * **Option C:** This is a **true** statement. Pituitary adenomas are a classic component of **MEN-1 (Wermer Syndrome)**, alongside Parathyroid hyperplasia and Pancreatic islet cell tumors (the "3 Ps") [1]. **NEET-PG High-Yield Pearls:** * **Most common type:** Prolactinoma (presents with amenorrhea, galactorrhea, and infertility) [1]. * **Microadenoma vs. Macroadenoma:** Defined by a size cutoff of **10 mm (1 cm)**. * **Genetics:** Mutations in the **GNAS1** gene (encoding the Gsα protein) are common in GH-secreting adenomas [1]. * **Clinical Sign:** Bitemporal hemianopia due to compression of the optic chiasm [1]. * **Histology:** Monomorphism (uniform cell type) and absence of a reticulin network are the two most important diagnostic features. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1079-1081.

Q: Medullary carcinoma of thyroid is associated with mutation in which gene?

RET. **Explanation:** **Medullary Carcinoma of the Thyroid (MTC)** is a neuroendocrine tumor arising from the **Parafollicular C-cells** (which secrete calcitonin) [3]. These cells are embryologically derived from the neural crest [3]. 1. **Why RET is correct:** The **RET proto-oncogene** (located on chromosome 10q11.2) encodes a receptor tyrosine kinase. Mutations in RET lead to constitutive activation of the receptor, driving oncogenesis. * **Germline mutations** in RET are responsible for nearly 100% of hereditary MTC cases (MEN 2A and 2B syndromes) [2]. * **Somatic mutations** in RET are found in approximately 50% of sporadic MTC cases. 2. **Why the other options are incorrect:** * **RAS:** Mutations in the RAS family (HRAS, KRAS, NRAS) are commonly associated with **Follicular Thyroid Carcinoma** and the follicular variant of Papillary Thyroid Carcinoma, but not typically MTC. * **NF (Neurofibromatosis):** Mutations in NF1 or NF2 are associated with Neurofibromatosis types 1 and 2, leading to tumors like neurofibromas, optic gliomas, and acoustic neuromas. * **Rb (Retinoblastoma):** The Rb tumor suppressor gene mutation is the hallmark of Retinoblastoma and Osteosarcoma. **High-Yield Clinical Pearls for NEET-PG:** * **MEN 2A:** MTC + Pheochromocytoma + Parathyroid Hyperplasia [1]. * **MEN 2B:** MTC + Pheochromocytoma + Mucosal Neuromas/Marfanoid habitus [1]. * **Tumor Marker:** Calcitonin is used for diagnosis and monitoring recurrence [3]. * **Histology:** Characterized by nests of cells in a prominent **Amyloid stroma** (stained with Congo Red). * **Prophylaxis:** In families with known RET mutations, prophylactic thyroidectomy is often performed in early childhood.

Question 1

Which tumor arises from the organ of Zuckerkandl?

Accepted Answer

Paraganglioma

Answer

Schwannoma

Answer

Astrocytoma

Answer

Medulloblastoma

Question 2

A 14-year-old girl noticed gradual neck enlargement during the past 8 months. On physical examination, her thyroid gland is diffusely enlarged. Her serum TSH level is normal. A dietary history is most likely to reveal that she has begun eating more of which of the following foods?

Accepted Answer

Cabbage

Answer

Fava beans

Answer

Fish

Answer

Plantains

Question 3

Which of the following is NOT a characteristic of medullary carcinoma of the thyroid?

Accepted Answer

TSH dependent

Answer

Contains amyloid

Answer

Secretes calcitonin

Answer

Patients should be screened for RET point mutation on chromosome 10

Question 4

All are true regarding medullary carcinoma of thyroid except?

Accepted Answer

It is dependent on TSH

Answer

It arises from C cells

Answer

Secrete high levels of calcitonin

Answer

Most cases are familial

Question 5

Which of the following statements is false regarding pituitary adenoma?

Accepted Answer

Reticular stain is positive with pituitary adenoma.

Answer

It is the most common cause of hyperpituitarism.

Answer

It is associated with MEN-1 syndrome.

Answer

All of the above statements are true.

Question 6

FNAC is useful in all the following types of thyroid carcinoma except?

Accepted Answer

Follicular

Answer

Papillary

Answer

Anaplastic

Answer

Medullary

Question 7

Which one of the following variants of papillary carcinoma thyroid occurs in younger individuals, including children, with lymphonodal metastases in almost all cases and morphologically simulates Hashimoto thyroiditis?

Accepted Answer

Diffuse sclerosing variant

Answer

Tall-cell variant

Answer

Follicular variant

Answer

Oncocytic variant

Question 8

Medullary carcinoma of thyroid is associated with mutation in which gene?

Accepted Answer

RET

Answer

RAS

Answer

NF

Answer

Rb

Question 9

Pheochromocytoma is a neoplasm derived from which of the following cell types?

Accepted Answer

Chromaffin cells

Answer

Paraganglia in the neck

Answer

Adrenal gland tumor due to nephrogenic rest

Answer

Primitive totipotential cells

Question 10

A 65-year-old female patient presented with a neck swelling that moved with deglutition, along with multiple episodes of diarrhea, hoarseness, mild dysphagia, and mild shortness of breath. Lab findings revealed raised serum calcitonin levels and hypercalcemia. From which embryonic structure is the gland responsible for this condition derived?

Accepted Answer

Thyroid parafollicular cells (C cells)

Answer

Parathyroid glands

Answer

Thymus

Answer

Adrenal medulla

Endocrine Pathology — MCQs

Endocrine Pathology — MCQs

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