Endocrine Pathology Practice Questions

Q: RET proto-oncogene is associated with the development of

Medullary carcinoma thyroid. ***Medullary carcinoma thyroid*** - The **RET proto-oncogene** mutation is strongly linked to medullary thyroid carcinoma, particularly in Multiple Endocrine Neoplasia (MEN) type 2 syndrome [1]. - This carcinoma originates from **C-cells** of the thyroid, which produce calcitonin, and its presence is typically assessed via genetic testing. *Hurthle cell tumor thyroid* - Hurthle cell tumors, while associated with the thyroid, are not typically linked to **RET proto-oncogene** mutations but rather are considered a variant of follicular thyroid carcinoma. - They primarily appear as **eosinophilic cells** and are related to **radioactive iodine resistance**, which differs from medullary carcinoma. *Paraganglioma* - Paragangliomas arise from **neuroendocrine cells** and are associated with mutations in genes such as **SDHB** or **SDHD**, not RET. - They commonly present as **extra-adrenal pheochromocytomas** and are not related to thyroid pathology. *Astrocytoma* - Astrocytomas are primary **central nervous system tumors** that originate from astrocytic cells and have no known association with RET proto-oncogene mutations. - The mutations in this type of tumor are different, often involving genes like **TP53** and **IDH1**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1102-1103.

Q: In a patient presenting with an adrenal mass, what is the most common cause of a malignant adrenal mass?

Metastasis from another solid tissue tumor. ***Metastasis from another solid tissue tumor*** - This is the **most common cause** of malignant adrenal masses, with **lung, breast, and melanoma** as usual primary sites [1]. - Adrenal metastases often present as **bilateral lesions**, frequently detected during imaging for another condition. *Adrenocortical carcinoma* - While this is a type of **primary adrenal cancer**, it is less common than metastatic disease [2]. - Adrenocortical carcinoma typically presents with **hormonal symptoms** or local invasiveness rather than being the most common cause. *Malignant phaeochromocytoma* - Though it is a malignant adrenal tumor, it is **rare** compared to metastases. - Symptoms may include **hypertension and episodic headache**, but it does not commonly account for adrenal masses. *Lymphoma* - Might involve the adrenal gland, but it is more commonly found in **extra-nodal sites** such as the abdomen. - The frequency of adrenal lymphoma is significantly lower than that of **metastatic adrenal lesions**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lung, pp. 724-725. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1135-1137.

Q: All of the following are features of granulomatous thyroiditis except?

Painless. ***Painless*** - Granulomatous thyroiditis is characterized by **painful** thyroid gland inflammation, which is a distinguishing feature. - Thus, describing it as **painless** contradicts the typical clinical presentation. *Hyperthyroidism* - Granulomatous thyroiditis may lead to **hyperthyroidism** initially due to the release of thyroid hormones from damaged follicles [1]. - However, this condition can also lead to transient thyroid function changes or even permanent hypothyroidism later on [1]. *Hypothyroidism* - While **hypothyroidism** can occur post-thyroiditis, it is not a feature of granulomatous thyroiditis at the outset like the **painless** descriptor. - This condition often starts with hyperthyroid symptoms and may evolve later, differing from primary hypothyroid disorders. *Giant cells on histology* - Histological examination typically reveals **multinucleated giant cells**, a hallmark of granulomatous inflammation, as seen in this thyroid condition. - This significant finding helps in differentiating it from other thyroid disorders. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1091-1092.

Q: Which chromosomal translocation is associated with follicular thyroid carcinoma?

PAX8 - PPARγ. ***PAX8 - PPARγ*** - The **PAX8-PPARγ fusion oncogene** is a well-established molecular marker directly associated with **follicular thyroid carcinoma (FTC)**. - This translocation leads to the expression of a fusion protein that contributes to **thyroid cell proliferation** and **tumorigenesis**. *ALK - NPM1* - The **ALK-NPM1 fusion** is primarily observed in some types of **anaplastic large cell lymphoma**, not thyroid cancers. - This translocation typically results in an **activated anaplastic lymphoma kinase (ALK)**, driving lymphoproliferation. *ETV6 - NTRK3* - The **ETV6-NTRK3 rearrangement** is characteristic of **secretory carcinoma** (formerly mammary analogue secretory carcinoma), often affecting salivary glands, and is not a common finding in thyroid malignancies. - This fusion leads to the activation of the **NTRK3 receptor tyrosine kinase**, involved in cell growth and survival. *RET - PTC1* - The **RET-PTC1 rearrangement (RET/papillary thyroid carcinoma 1)** is specifically associated with **papillary thyroid carcinoma (PTC)**, which is histologically distinct from follicular thyroid carcinoma. - This fusion activates the **RET proto-oncogene**, promoting cell proliferation and survival in papillary thyroid cancer.

Q: Calcified pulmonary metastasis is seen in which carcinoma?

Thyroid carcinoma. ***Thyroid carcinoma*** - **Papillary** and **medullary thyroid carcinomas** can produce **calcified pulmonary metastases**. - In **papillary thyroid cancer**, calcification occurs due to **psammoma bodies** (concentrically laminated calcified structures). - In **medullary thyroid cancer**, calcification can occur through **dystrophic calcification** within the tumor tissue. - Other common causes of calcified lung metastases include **osteosarcoma** and **chondrosarcoma**. *Pancreatic carcinoma* - Pancreatic carcinoma rarely causes **calcified pulmonary metastases**; metastatic lesions are typically **non-calcified**. - Metastases are more commonly found in the **liver** and **peritoneum**. - Primary pancreatic tumors may show calcification, but metastases usually do not. *Endometrial carcinoma* - Endometrial carcinoma metastases to the lungs are usually **non-calcified** and appear as **soft tissue nodules**. - While it can metastasize to the lungs, **calcification** is not a typical feature of its pulmonary spread. *None of the options* - This option is incorrect because **thyroid carcinoma** (particularly papillary type) is a well-recognized cause of **calcified pulmonary metastases**. - Among epithelial malignancies, thyroid carcinoma is one of the classic causes of this finding.

Q: All the following are true of craniopharyngioma except:

Present in sella or infra-sellar location. ***Present in sella or infra-sellar location*** - Craniopharyngiomas are typically located in the **suprasellar region**, above the **sella turcica**, where they can compress the optic chiasm. - While they can extend into the sella, their primary location is rarely exclusively intrasellar or infrasellar. *Derived from Rathke's pouch* - This statement is true; craniopharyngiomas arise from remnants of **Rathke's pouch**, the embryonic precursor of the anterior pituitary gland. - This origin explains their characteristic location near the pituitary stalk and third ventricle. *Contains epithelial cells* - This statement is true as **craniopharyngiomas** are benign **epithelial tumors**, specifically adamantinomatous or papillary types. - They are composed of stratified squamous epithelium, often with calcifications and cystic components. *Causes visual disturbances* - This statement is true because the **suprasellar location** of a craniopharyngioma often leads to compression of the **optic chiasm**, resulting in characteristic visual field deficits like bitemporal hemianopsia. - Visual disturbances are a common presenting symptom due to their proximity to the visual pathways.

Q: Hurthle cell carcinoma is a variant of which type of carcinoma?

Follicular carcinoma. **Follicular carcinoma** - **Hürthle cell carcinoma**, also known as **oxyphilic follicular carcinoma**, is a specific variant of **follicular carcinoma of the thyroid**. - It is characterized by the presence of large polygonal cells with abundant eosinophilic, granular cytoplasm known as **Hürthle cells** (or oxyphil cells) within the neoplastic growth. *Medullary carcinoma* - **Medullary carcinoma** originates from the **parafollicular C cells** of the thyroid, which produce calcitonin. - It is histologically distinct, featuring nests or cords of cells often associated with **amyloid deposits**, and is not related to Hürthle cell morphology. *Papillary carcinoma* - **Papillary carcinoma** is the most common type of thyroid cancer, characterized by distinctive **nuclear features** such as **Orphan Annie eye nuclei**, nuclear grooves, and intranuclear cytoplasmic inclusions. - Its histological origin and morphological appearance are different from Hürthle cell neoplasms, which are follicular in origin. *Anaplastic carcinoma* - **Anaplastic carcinoma** is a highly aggressive and undifferentiated thyroid malignancy with a very poor prognosis. - It is characterized by pleomorphic, giant, and spindle cells and lacks the specific differentiation seen in follicular or Hürthle cell tumors.

Q: Orphan Annie nuclei are characteristic of which of the following?

Papillary thyroid carcinoma. ***Papillary carcinoma thyroid***[1][2] - Characterized by **Orphan Annie nuclei**[1], which are large and round with a clear or empty appearance due to the presence of intranuclear cytoplasmic inclusions[1]. - Often associated with **thyroid follicular structures** and is the most common type of thyroid cancer[2]. *Meningioma* - Typically presents with **dural-based tumors** and does not exhibit Orphan Annie nuclei. - Histologically, it may demonstrate **whorled patterns** or calcifications instead. *Carcinoma pituitary* - Involves **adenomatous changes** in the pituitary gland but does not demonstrate the characteristic Orphan Annie nuclei. - More commonly shows **varied cellular morphology** depending on the type of secretory cells (e.g., prolactin, ACTH). *Paraganglioma* - Derived from **neuroendocrine cells**, and presents with **zellballen pattern** rather than Orphan Annie nuclei. - Often shows **chromaffin cells** and is typically associated with catecholamine secretion. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, p. 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: Which of the following is a definitive feature of malignant pheochromocytoma?

Presence of metastases in non-chromaffin tissues. ***Presence of metastases in non-chromaffin tissues*** - The definitive diagnosis of malignant pheochromocytoma is based solely on the presence of **metastases** in sites where chromaffin cells are not normally found, such as bone, liver, lung, or lymph nodes. - Unlike many other cancers, histological features like pleomorphism, mitotic activity, or tumor size do not reliably distinguish between benign and malignant pheochromocytomas. *Capsular invasion* - While **capsular invasion** suggests more aggressive behavior, it is not a definitive criterion for malignancy in pheochromocytoma. - Benign pheochromocytomas can also exhibit some degree of capsular invasion, making it an unreliable indicator of metastatic potential. *Vascular invasion* - Similar to capsular invasion, **vascular invasion** indicates an increased risk of recurrence or more aggressive behavior, but it is not a singular definitive feature of malignancy. - The presence of tumor cells within blood vessels does not automatically confirm metastatic disease without evidence of spread to distant sites. *None of the options are definitive features of malignancy* - This statement is incorrect because the presence of metastases in non-chromaffin tissues is indeed the **definitive feature** for diagnosing malignant pheochromocytoma. - Without evidence of metastasis, distinguishing between benign and malignant pheochromocytoma based on other features is often challenging and unreliable.

Q: Tumor marker for medullary carcinoma of the thyroid is:

Calcitonin. Everything in the original explanation remains unchanged as none of the provided references [1, 2, 3, 4, 5] were relevant to Medullary Thyroid Carcinoma or Calcitonin. ***Calcitonin*** - **Calcitonin** is a hormone produced by the parafollicular C cells of the thyroid gland. Medullary thyroid carcinoma originates from these C cells. - Elevated serum calcitonin levels are a highly sensitive and specific **tumor marker** for both diagnosis and monitoring of medullary thyroid carcinoma. *Albumin* - **Albumin** is a protein primarily produced by the liver, essential for maintaining oncotic pressure and transporting various substances in the blood. - It is not a tumor marker for any specific thyroid cancer. *TSH* - **Thyroid-stimulating hormone (TSH)** is produced by the pituitary gland and regulates thyroid hormone production by follicular cells. - While TSH levels are crucial in evaluating other thyroid disorders, they are not a specific tumor marker for medullary thyroid carcinoma, which arises from C cells, not follicular cells. *Thyroglobulin* - **Thyroglobulin** is a protein produced by the follicular cells of the thyroid gland and is the precursor to thyroid hormones. - It serves as a tumor marker for **differentiated thyroid cancers** (papillary and follicular carcinoma), but not for medullary thyroid carcinoma.

Question 1

RET proto-oncogene is associated with the development of

Accepted Answer

Medullary carcinoma thyroid

Answer

Astrocytoma

Answer

Paraganglioma

Answer

Hurthle cell tumor thyroid

Question 2

In a patient presenting with an adrenal mass, what is the most common cause of a malignant adrenal mass?

Accepted Answer

Metastasis from another solid tissue tumor

Answer

Adrenocortical carcinoma

Answer

Malignant phaeochromocytoma

Answer

Lymphoma

Question 3

All of the following are features of granulomatous thyroiditis except?

Accepted Answer

Painless

Answer

Hyperthyroidism

Answer

Giant cells on histology

Answer

Hypothyroidism

Question 4

Which chromosomal translocation is associated with follicular thyroid carcinoma?

Accepted Answer

PAX8 - PPARγ

Answer

ALK - NPM1

Answer

ETV6 - NTRK3

Answer

RET - PTC1

Question 5

Calcified pulmonary metastasis is seen in which carcinoma?

Accepted Answer

Thyroid carcinoma

Answer

Pancreatic carcinoma

Answer

Endometrial carcinoma

Answer

None of the options

Question 6

All the following are true of craniopharyngioma except:

Accepted Answer

Present in sella or infra-sellar location

Answer

Derived from Rathke's pouch

Answer

Contains epithelial cells

Answer

Causes visual disturbances

Question 7

Hurthle cell carcinoma is a variant of which type of carcinoma?

Accepted Answer

Follicular carcinoma

Answer

Medullary carcinoma

Answer

Papillary carcinoma

Answer

Anaplastic carcinoma

Question 8

Orphan Annie nuclei are characteristic of which of the following?

Accepted Answer

Papillary thyroid carcinoma

Answer

Paraganglioma with Zellballen pattern

Answer

Meningioma with psammoma bodies

Answer

Pituitary adenoma with atypical nuclei

Question 9

Which of the following is a definitive feature of malignant pheochromocytoma?

Accepted Answer

Presence of metastases in non-chromaffin tissues

Answer

Vascular invasion

Answer

None of the options are definitive features of malignancy

Answer

Capsular invasion

Question 10

Tumor marker for medullary carcinoma of the thyroid is:

Accepted Answer

Calcitonin

Answer

Albumin

Answer

TSH

Answer

Thyroglobulin

Endocrine Pathology — MCQs

Endocrine Pathology — MCQs

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