Endocrine Pathology Practice Questions

Q: A patient presents with headaches, palpitations, hypertension, and urine VMA positivity. The biopsy findings are shown in the image. Which of the following statements is correct?

Associated with MEN 2A. ***Associated with MEN 2A*** - The clinical presentation (headaches, palpitations, hypertension) and positive **urine VMA (vanillylmandelic acid)** strongly suggest a **pheochromocytoma**. - Pheochromocytomas are tumors of the adrenal medulla that secrete catecholamines and are frequently associated with **Multiple Endocrine Neoplasia Type 2A (MEN 2A)**, along with medullary thyroid carcinoma and primary hyperparathyroidism. *Mostly malignant* - Pheochromocytomas are generally benign, with approximately **10% being malignant** ("rule of 10s"). - Malignancy is difficult to predict based on histology alone and is usually defined by the presence of **metastases**. *Mostly in children* - While pheochromocytomas can occur at any age, they are **more common in adults**, typically between 30 and 50 years old. - When they do occur in children, they are more often bilateral, extra-adrenal, or associated with genetic syndromes. *Mostly bilateral* - The majority of pheochromocytomas (approximately **90%**) are **unilateral**. - Bilateral pheochromocytomas are often seen in genetic syndromes such as **MEN 2**, von Hippel-Lindau disease, and neurofibromatosis type 1.

Q: The following is a histopathological image of thyroid pathology. What is the diagnosis?

Medullary carcinoma of thyroid. ***Medullary carcinoma of thyroid*** - This image shows sheets and nests of **polygonal to spindle-shaped cells**, which are characteristic of medullary thyroid carcinoma, especially when mixed with an **amyloid stroma** (seen as amorphous eosinophilic material) [2]. - The presence of **neuroendocrine features** and the production of **calcitonin** are hallmarks of these C-cell tumors [1], [2]. *Papillary carcinoma of thyroid* - Characterized by **papillary architecture**, **ground-glass (Orphan Annie eye) nuclei**, nuclear grooves, and intranuclear cytoplasmic inclusions. - These features are not prominently seen in the provided image. *Follicular carcinoma of thyroid* - Defined by an invasive growth pattern of **well-differentiated follicular cells** forming follicles, with either capsular or vascular invasion [2]. - The image does not show classic follicular architectural patterns or clear evidence of invasion in the absence of a capsule. *Anaplastic carcinoma of thyroid* - This is a highly aggressive and undifferentiated tumor with **marked pleomorphism**, bizarre giant cells, and high mitotic activity [2]. - While there is some pleomorphism, the overall pattern and cellular morphology in the image are more consistent with medullary carcinoma than the extreme anaplasia. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1102-1103. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 428-431.

Q: A thyroid FNA shows 'bubble gum' colloid. Which nuclear feature would best support papillary thyroid carcinoma?

Ground glass nuclei. ***Ground glass nuclei*** - **Ground glass nuclei**, also known as **Orphan Annie eye nuclei** [1][2], are the most **characteristic and recognized nuclear feature** of **papillary thyroid carcinoma (PTC)** on FNA cytology. [1] - This appearance results from **fine, evenly dispersed chromatin** that gives the nucleus a clear, empty, or translucent appearance with a prominent nuclear membrane. [1] - Among the given options, this is the **single best feature** that would support a PTC diagnosis when 'bubble gum' colloid is present. *Nuclear grooves* - **Nuclear grooves** are a common and highly supportive feature of PTC, particularly when **prominent and numerous**. - However, as a **single finding**, they are less definitive than ground glass nuclei, as grooves can occasionally be seen in benign conditions (though usually less prominent). - In combination with other features, nuclear grooves are highly specific for PTC. *Prominent nucleoli* - **Prominent nucleoli** are more frequently associated with **follicular neoplasms**, **medullary thyroid carcinoma**, or anaplastic thyroid carcinoma. - Classical PTC typically has **inconspicuous nucleoli**, so prominent nucleoli would suggest an alternative diagnosis or a tall cell variant of PTC. *Salt and pepper chromatin* - **Salt and pepper chromatin** (finely stippled chromatin) is a classic cytological feature of **medullary thyroid carcinoma** (MTC). - This chromatin pattern reflects neuroendocrine differentiation and is distinct from the nuclear characteristics of PTC. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1098-1100. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 429-430.

Q: Pheochromocytoma produces all except?

Secretin. Secretin - **Secretin** is a hormone primarily produced by **S cells in the duodenum and jejunum** [3] in response to acidic chyme, stimulating pancreatic bicarbonate secretion. - Pheochromocytomas originate from **chromaffin cells** of the adrenal medulla and are known for producing catecholamines [2], not secretin. *Vasoactive intestinal polypeptide* - **Vasoactive intestinal polypeptide (VIP)** can be produced by some **neuroendocrine tumors** [1], including pheochromocytomas, though it's more commonly associated with VIPomas. - VIP acts as a **vasodilator** and can contribute to symptoms like flushing and diarrhea in rare cases of VIP-producing pheochromocytomas. *Calcitonin* - While calcitonin is primarily associated with **medullary thyroid carcinoma**, it can rarely be produced ectopically by **neuroendocrine tumors**, including some pheochromocytomas. - This ectopic production is part of the broader concept of **paraneoplastic syndromes** seen in various malignancies. *Norepinephrine* - **Norepinephrine** is a major catecholamine produced by pheochromocytomas, leading to classic symptoms such as **hypertension, palpitations, and sweating** [2]. - The tumor cells (chromaffin cells) are specialized to synthesize and release **catecholamines**, including norepinephrine and epinephrine [2].

Q: Malignancy in a multinodular goiter is most often:-

Papillary carcinoma. ***Papillary carcinoma*** - **Papillary carcinoma** is the most common type of thyroid cancer, accounting for about 80-85% of all thyroid malignancies [1], [2]. - It often arises in the setting of multifocal disease or within a **multinodular goiter**, particularly when a dominant nodule undergoes malignant transformation [2]. *Anaplastic carcinoma* - **Anaplastic carcinoma** is a very aggressive and rare form of thyroid cancer, typically presenting as a rapidly growing neck mass in elderly patients [2]. - While it can occur in a multinodular goiter, it is far less common than papillary carcinoma and carries a much poorer prognosis [2]. *Follicular carcinoma* - **Follicular carcinoma** is the second most common type of thyroid cancer (10-15%) and is often difficult to distinguish from benign follicular adenomas without surgical excision [2]. - While it can be found in a multinodular goiter, **papillary carcinoma** is still statistically more frequent in this context [1]. *Medullary carcinoma* - **Medullary carcinoma** originates from the parafollicular C cells of the thyroid and accounts for about 1-2% of all thyroid cancers [2]. - It is often associated with inherited syndromes like **Multiple Endocrine Neoplasia type 2 (MEN2)** and is distinct from tumors arising from follicular cells within a multinodular goiter [2]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1098-1099. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 429-430.

Q: Nesidioblastoma is due to hyperplasia of?

Beta cell. ***Beta cell*** - Nesidioblastoma is a rare pancreatic disorder characterized by the **hyperplasia of pancreatic islet beta cells**. - This over-proliferation of **insulin-producing beta cells** leads to excessive insulin secretion and recurrent hypoglycemia [2]. *Alpha cell* - Hyperplasia of **alpha cells** would primarily lead to an overproduction of **glucagon**, potentially causing hyperglycemia [1], [2]. - Nesidioblastoma is specifically linked to hypoglycemia due to excessive insulin. *D cells* - **D cells** (delta cells) in the pancreas produce **somatostatin**, which inhibits the secretion of insulin and glucagon [2]. - Hyperplasia of D cells would more likely dampen both insulin and glucagon effects rather than cause insulin-driven hypoglycemia. *Acinus* - The **acinus** refers to the exocrine portion of the pancreas, responsible for producing digestive enzymes [1]. - Nesidioblastoma originates from the **endocrine islet cells**, not the exocrine acinar cells. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 433-434. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1109, 1125.

Q: A 14-year-old male presents with type I diabetes mellitus. His mother wants to know if the boy's brother might also have an increased risk of getting the disease. Which of the following genotypes, if present in the brother, would be associated with the greatest risk of developing diabetes?

DR3/DR4. ***DR3/DR4*** - The combination of **HLA-DR3** and **HLA-DR4** is the strongest genetic risk factor for **Type 1 diabetes mellitus (T1DM)**, particularly in individuals of European descent. - These alleles are associated with increased susceptibility to autoimmune destruction of pancreatic beta cells [1]. *DR2/DR4* - While **DR4** is a risk allele for Type 1 diabetes, **DR2 (specifically DRB1*1501)** is generally considered to be protective against the disease. - The presence of the protective DR2 allele would likely mitigate the increased risk conferred by DR4. *DR2/DR2* - **HLA-DR2 (DRB1*1501)** is largely recognized as a protective allele against **Type 1 diabetes**, meaning individuals with this genotype have a lower risk of developing the disease [1]. - Its presence is associated with a reduced susceptibility to autoimmune conditions like T1DM. *B27/B27* - **HLA-B27** is primarily associated with **seronegative spondyloarthropathies** such as ankylosing spondylitis and reactive arthritis, and not directly with an increased risk of Type 1 diabetes mellitus. - This allele is involved in different autoimmune pathways than those implicated in T1DM.

Q: Neuroblastoma originates from –

All of these. ***All of these*** - **Neuroblastoma** arises from primitive **sympathetic neural crest cells** and can originate from **any location** where these cells exist during development [1]. - The tumor can develop in the **adrenal medulla**, **sympathetic ganglia** along the paravertebral sympathetic chain (abdomen, chest, neck, pelvis), making all the listed options correct sites of origin [1], [2]. - The anatomical distribution reflects the **embryological migration pattern** of neural crest cells. **Adrenal Glands (Most Common)** - The **adrenal medulla** is the most frequent primary site, accounting for approximately **40-50%** of all neuroblastomas [1]. - This is because the adrenal medulla is derived from sympathetic neural crest cells. - Abdominal neuroblastomas (adrenal and extra-adrenal) together comprise about **65%** of cases. **Mediastinum (Posterior)** - Approximately **15-20%** of neuroblastomas originate in the **posterior mediastinum** from sympathetic ganglia. - These are the **second most common site** and typically present as posterior mediastinal masses on chest imaging. - Often discovered incidentally on chest X-rays. **Neck (Cervical)** - **Cervical neuroblastoma** arises from sympathetic ganglia in the neck region. - This accounts for about **1-5%** of cases and is relatively uncommon. - May present as a palpable neck mass or Horner syndrome if the cervical sympathetic chain is involved. **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. Common Clinical Problems From Liver And Biliary System Disease, pp. 419-420.

Question 1

A patient presents with headaches, palpitations, hypertension, and urine VMA positivity. The biopsy findings are shown in the image. Which of the following statements is correct?

Accepted Answer

Associated with MEN 2A

Answer

Mostly malignant

Answer

Mostly in children

Answer

Mostly bilateral

Question 2

The following is a histopathological image of thyroid pathology. What is the diagnosis?

Accepted Answer

Medullary carcinoma of thyroid

Answer

Papillary carcinoma of thyroid

Answer

Follicular carcinoma of thyroid

Answer

Anaplastic carcinoma of thyroid

Question 3

A thyroid FNA shows 'bubble gum' colloid. Which nuclear feature would best support papillary thyroid carcinoma?

Accepted Answer

Ground glass nuclei

Answer

Nuclear grooves

Answer

Prominent nucleoli

Answer

Salt and pepper chromatin

Question 4

Pheochromocytoma produces all except?

Accepted Answer

Secretin

Answer

Vasoactive intestinal polypeptide

Answer

Norepinephrine

Answer

Calcitonin

Question 5

Malignancy in a multinodular goiter is most often:-

Accepted Answer

Papillary carcinoma

Answer

Anaplastic carcinoma

Answer

Follicular carcinoma

Answer

Medullary carcinoma

Question 6

Pseudohermaphroditism (Disorder of Sex Development - DSD) is most commonly seen with which tumor?

Accepted Answer

Virilizing adrenal carcinoma

Answer

Adrenal adenoma

Answer

Leydig cell tumor

Answer

Granulosa cell tumor

Question 7

Nesidioblastoma is due to hyperplasia of?

Accepted Answer

Beta cell

Answer

Alpha cell

Answer

D cells

Answer

Acinus

Question 8

A 14-year-old male presents with type I diabetes mellitus. His mother wants to know if the boy's brother might also have an increased risk of getting the disease. Which of the following genotypes, if present in the brother, would be associated with the greatest risk of developing diabetes?

Accepted Answer

DR3/DR4

Answer

DR2/DR4

Answer

DR2/DR2

Answer

B27/B27

Question 9

Neuroblastoma originates from –

Accepted Answer

All of these

Answer

Mediastinum

Answer

Adrenals

Answer

Neck

Question 10

Which of the following occurs in long standing goitre:

Accepted Answer

Follicular Ca

Answer

Medullary Ca

Answer

Papillary Ca

Answer

Anaplastic Ca

Endocrine Pathology — MCQs

Endocrine Pathology — MCQs

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