Diffuse Neuroendocrine System — MCQs
Which of the following cells in the lungs are classified as neuroendocrine cells?
VIPoma is associated with which syndrome?
FDG-PET negative tumor is:
All of the following statements are true about Gleason score, except:
Submucosal neuroma is associated with
Which of the following glands is NOT involved in Type I MEN?
MC location of gastrinoma in MEN-1 syndrome?
What is the most common type of acute myeloid leukemia in patients with Down's syndrome?
A 25-Year-old male presented with a 2cm thyroid nodule. A thyroidectomy was done. The histology picture is given below. What could be the diagnosis?
A 28-year-old woman has noticed increasing lower limb swelling and shortness of breath, with a 2-year history of facial rash, hair loss, arthralgias, and thrombocytopenia. On examination, her blood pressure is 150/90 mmHg, pulse 80/min, with a maculopapular rash on her face, JVP of 4 cm, normal heart sounds, clear lungs, and pedal and periorbital edema. Her creatinine is very high, and a urinalysis reveals many RBCs and RBC casts. For this patient with glomerulonephritis, select the most likely diagnosis on renal biopsy.
Diffuse Neuroendocrine System Indian Medical PG Practice Questions and MCQs
Question 1: Which of the following cells in the lungs are classified as neuroendocrine cells?
- A. Dendritic cells
- B. Type I pneumocytes
- C. Type II pneumocytes
- D. Kulchitsky cells (Correct Answer)
Explanation: ***Kulchitsky cells*** - **Kulchitsky cells**, also known as pulmonary neuroendocrine cells (PNECs), are found in the bronchial and bronchiolar epithelium. - They produce various **bioactive peptides and amines**, regulating airway tone and contributing to lung development and pathology. *Dendritic cells* - **Dendritic cells** are **immune cells** that act as antigen-presenting cells, initiating adaptive immune responses. - They are not classified as neuroendocrine cells and primarily function in immune surveillance. *Type I pneumocytes* - **Type I pneumocytes** are **squamous epithelial cells** that form the primary surface for gas exchange in the alveoli [1]. - Their main function is to facilitate **diffusion of gases** (oxygen and carbon dioxide) and they lack endocrine properties [1]. *Type II pneumocytes* - **Type II pneumocytes** are cuboidal cells responsible for producing and secreting **surfactant**, which reduces alveolar surface tension [1]. - They also act as progenitor cells for Type I pneumocytes but are not considered neuroendocrine cells [1].
Question 2: VIPoma is associated with which syndrome?
- A. Cushing's syndrome
- B. Verner-Morrison syndrome (Correct Answer)
- C. Carcinoid syndrome (serotonin syndrome)
- D. Zollinger-Ellison syndrome
Explanation: ***Verner-Morrison syndrome*** - **VIPoma** is a neuroendocrine tumor that secretes **vasoactive intestinal peptide (VIP)**, causing symptoms like watery diarrhea, hypokalemia, and achlorhydria (WDHA syndrome) [1]. - This clinical presentation is also known as **Verner-Morrison syndrome**, directly linking the VIPoma to this syndrome [1]. *Cushing's syndrome* - Characterized by excessive **cortisol** production, leading to symptoms like central obesity, moon facies, and hypertension [3]. - This syndrome is not directly associated with VIP excess or the watery diarrhea seen in VIPoma [2]. *Carcinoid syndrome (serotonin syndrome)* - Caused by tumors, typically in the gastrointestinal tract, that produce **serotonin**, leading to flushing, diarrhea, and bronchospasm [1]. - While it involves diarrhea, the primary mediator is serotonin, not VIP, and the other classic VIPoma symptoms (hypokalemia, achlorhydria) are absent [1]. *Zollinger-Ellison syndrome* - Characterized by a **gastrin-producing tumor (gastrinoma)**, which causes excessive gastric acid secretion and severe peptic ulcer disease [4]. - The hormonal excess is **gastrin**, not VIP, and symptoms are related to acid overproduction rather than massive watery diarrhea and hypokalemia [4].
Question 3: FDG-PET negative tumor is:
- A. Typical carcinoid (Correct Answer)
- B. Atypical carcinoid
- C. Small cell carcinoma
- D. Large cell neuroendocrine carcinoma
Explanation: ***Typical carcinoid*** - **Typical carcinoid tumors** generally have a low metabolic rate and thus do not avidly take up **FDG (fluorodeoxyglucose)**, appearing **FDG-PET negative**. - These tumors are characterized by low mitotic activity and lack of necrosis, contributing to their low glucose metabolism. *Atypical carcinoid* - **Atypical carcinoid tumors** have a higher proliferative index and increased metabolic activity compared to typical carcinoids. - They tend to be **FDG-PET positive** due to their higher glucose utilization. *Small cell carcinoma* - **Small cell carcinoma** is a highly aggressive tumor type with rapid proliferation and high metabolic activity. - It is typically **FDG-PET positive**, reflecting its significant glucose uptake. *Large cell neuroendocrine carcinoma* - **Large cell neuroendocrine carcinoma (LCNEC)** is also an aggressive tumor with a high mitotic rate and often exhibits necrosis. - Similar to small cell carcinoma, LCNEC is generally **FDG-PET positive** due to its high metabolic demand.
Question 4: All of the following statements are true about Gleason score, except:
- A. Used for grading prostate cancer
- B. Higher the score, poorer the prognosis
- C. Helps in planning management
- D. Scores range from 2-10 (Correct Answer)
Explanation: ***Scores range from 2-10*** - The **Gleason score** is obtained by summing the two most predominant architectural patterns of tumor growth, with each pattern graded from 1 to 5. The lowest possible sum is 2 (1+1) and the highest is 10 (5+5). However, **Gleason scores are now reported from 6 to 10** for clinical purposes, as grade 1 and 2 patterns are rarely seen in biopsies, and for practical purposes, anything less than a 3+3=6 is considered benign or low-risk. [1] - While mathematically the range is 2-10, in modern clinical practice, a score of 6 is the lowest grade assigned to prostate cancer, making the statement that scores range from 2-10 clinically inaccurate. *Used for grading prostate cancer* - The **Gleason score** is a well-established and widely used histological grading system specifically for prostate adenocarcinoma. [2] - It assesses the **architectural patterns** of glandular differentiation in prostate cancer to predict its aggressiveness. *Higher the score, poorer the prognosis* - A **higher Gleason score** indicates a more poorly differentiated and aggressive tumor, which correlates with a greater likelihood of metastasis and recurrence. [2] - This directly translates to a **poorer prognosis** for the patient. *Helps in planning management* - The **Gleason score** is a critical factor, along with PSA levels and clinical staging, in determining the risk stratification of prostate cancer. - This risk stratification guides treatment decisions, such as whether to pursue active surveillance, radiation therapy, surgery, or systemic therapy. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lower Urinary Tract and Male Genital System, pp. 990-992. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Lower Urinary Tract and Male Genital System, pp. 993-994.
Question 5: Submucosal neuroma is associated with
- A. MEN 2 A
- B. MEN 2B (Correct Answer)
- C. MEN 1
- D. None of the options
Explanation: ***MEN 2B (Multiple Endocrine Neoplasia Type 2B)*** - **Submucosal neuromas** are a distinctive feature of MEN 2B, specifically noticeble as mucosal neuromas on the lips, tongue, and gastrointestinal tract. - This syndrome is characterized by the presence of **medullary thyroid carcinoma**, **pheochromocytoma**, and mucocutaneous neuromas, without hyperparathyroidism. *MEN 2A (Multiple Endocrine Neoplasia Type 2A)* - MEN 2A is characterized by **medullary thyroid carcinoma**, **pheochromocytoma**, and **primary hyperparathyroidism**. - It does not typically feature extensive **submucosal neuromas** as a primary diagnostic criterion. *MEN 1 (Multiple Endocrine Neoplasia Type 1)* - MEN 1 involves tumors of the **parathyroid glands**, **anterior pituitary**, and **pancreatic islet cells** (the '3 Ps'). - **Submucosal neuromas** are not a component of the MEN 1 syndrome. *None of the options* - This option is incorrect because **submucosal neuromas** are a characteristic finding in MEN 2B.
Question 6: Which of the following glands is NOT involved in Type I MEN?
- A. Parathyroid
- B. Adrenal (Correct Answer)
- C. Pituitary
- D. Pancreas
Explanation: ***Adrenal*** - The **adrenal glands** are predominantly involved in **Multiple Endocrine Neoplasia type 2 (MEN2)**, particularly with pheochromocytomas, rather than MEN type 1. - While adrenal lesions (e.g., adenomas, hyperplasia) can occur sporadically or rarely in MEN1, they are not considered a primary or core component of the MEN1 syndrome as defined by the classic "3 Ps." [1] *Pancreas* - The **pancreas** is a primary gland involved in MEN1, frequently developing **neuroendocrine tumors** (e.g., gastrinomas, insulinomas). - These pancreatic tumors are a major cause of morbidity and mortality in MEN1 patients. *Pituitary* - The **pituitary gland** is one of the classic "3 P's" involved in MEN1, commonly developing **adenomas**, especially **prolactinomas**. [1] - These pituitary tumors can cause hormonal imbalances and mass effects within the sella turcica. *Parathyroid* - The **parathyroid glands** are almost universally involved in MEN1, with **hyperplasia** leading to **primary hyperparathyroidism**. [1] - This is often the earliest and most common clinical manifestation of MEN1.
Question 7: MC location of gastrinoma in MEN-1 syndrome?
- A. Jejunum
- B. Ileum
- C. Duodenum
- D. Pancreas (Correct Answer)
Explanation: ***Pancreas*** - In **Multiple Endocrine Neoplasia type 1 (MEN-1) syndrome**, gastrinomas are most commonly found in the **pancreas**. - While sporadic gastrinomas are frequently duodenal, the **genetic predisposition of MEN-1** shifts the primary location to the pancreas. *Duodenum* - **Sporadic gastrinomas** without MEN-1 syndrome are most frequently located in the **duodenum**, particularly the first and second parts. - However, in the context of **MEN-1**, the pancreas becomes the predominant site for gastrinoma development. *Jejunum* - The jejunum is an **uncommon location** for gastrinomas in both sporadic cases and those associated with MEN-1. - Gastrinomas found in the jejunum are typically **rare** and often associated with more aggressive disease or disseminated metastasis. *Ileum* - The ileum is an **extremely rare site** for gastrinomas. - Gastrinomas developing in the ileum are usually **ectopic** and are not typically the primary location in either sporadic cases or MEN-1 syndrome.
Question 8: What is the most common type of acute myeloid leukemia in patients with Down's syndrome?
- A. Acute megakaryoblastic leukemia M7 (Correct Answer)
- B. Acute myeloid leukemia M1
- C. Acute promyelocytic leukemia M3
- D. Acute myeloid leukemia M2
Explanation: ***Acute megakaryoblastic leukemia M7*** - **Acute megakaryoblastic leukemia (AML M7)** is significantly more common in children with **Down's syndrome (trisomy 21)**, particularly those under 5 years of age. - This association is thought to be due to an increased copy number of certain genes on **chromosome 21** that are involved in hematopoiesis and leukemogenesis. [3] *Acute myeloid leukemia M1* - This subtype, characterized by proliferation of **myeloblasts without maturation**, is not specifically associated with Down's syndrome. [1] - It is a more undifferentiated form of AML. *Acute promyelocytic leukemia M3* - Characterized by the t(15;17) translocation involving the **PML-RARα fusion gene**, resulting in a block in myeloid differentiation at the promyelocyte stage. [2], [4], [5] - This subtype is associated with a specific genetic abnormality and is not preferentially seen in patients with Down's syndrome. *Acute myeloid leukemia M2* - This subtype involves **myeloblasts with maturation** and a characteristic t(8;21) chromosomal translocation. [2] - While it's a common form of AML, it does not show the specific strong association with Down's syndrome that AML M7 does. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 607-608. [2] 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, p. 620. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 170-171. [4] 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. 621-622. [5] 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. 620-621.
Question 9: A 25-Year-old male presented with a 2cm thyroid nodule. A thyroidectomy was done. The histology picture is given below. What could be the diagnosis?
- A. Papillary thyroid carcinoma (Correct Answer)
- B. Follicular thyroid adenoma
- C. Graves' disease
- D. Adenomatous goiter
Explanation: ***Papillary carcinoma thyroid*** - Characterized by **papillary structures** and **nuclear features** such as nuclear grooves and overlapping nuclei on histology [1]. - Often presents in young adults and can show **psammoma bodies**, which are indicative of malignancy. *Follicular adenoma* - Generally shows well-circumscribed **follicular structures** without nuclear atypia [2,3]. - Lacks the typical **papillary architecture** and associated aggressive features found in carcinoma. *Graves disease* - Primarily presents with **hyperthyroidism** and diffuse goiter rather than a solitary nodule. - Histologically, it is characterized by **hyperplastic follicles** and does not display features of malignancy. *Adenomatous goitre* - Refers to **nodular enlargement** of the thyroid with benign hyperplastic nodules. - Lacks the **malignant features** present in papillary carcinoma, such as nuclear atypia and invasion. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, p. 1099. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1096-1097. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 428-429.
Question 10: A 28-year-old woman has noticed increasing lower limb swelling and shortness of breath, with a 2-year history of facial rash, hair loss, arthralgias, and thrombocytopenia. On examination, her blood pressure is 150/90 mmHg, pulse 80/min, with a maculopapular rash on her face, JVP of 4 cm, normal heart sounds, clear lungs, and pedal and periorbital edema. Her creatinine is very high, and a urinalysis reveals many RBCs and RBC casts. For this patient with glomerulonephritis, select the most likely diagnosis on renal biopsy.
- A. diffuse proliferative GN (Correct Answer)
- B. crescentic glomerulonephritis
- C. focal segmental glomerulosclerosis
- D. membranoproliferative glomerulonephritis
Explanation: ***diffuse proliferative GN*** - This patient's constellation of symptoms, including **facial rash, hair loss, arthralgias, thrombocytopenia**, and **renal involvement** (elevated creatinine, RBCs, and RBC casts), strongly points to **systemic lupus erythematosus (SLE)** [2], [4]. - **Diffuse proliferative glomerulonephritis (DPGN)** is the **most common and severe form of lupus nephritis** (WHO Class IV), characterized by widespread involvement of glomeruli with significant proliferation and inflammation [1]. - On renal biopsy, DPGN shows **subendothelial immune complex deposits** ("wire loop" lesions) and **proliferation of mesangial and endothelial cells** [1]. *crescentic glomerulonephritis* - While crescentic glomerulonephritis can cause a rapid decline in renal function and is associated with some autoimmune conditions, it is a **histological pattern** seen in various rapidly progressive glomerular diseases, not a specific underlying disease entity itself [3]. - The clinical picture here strongly suggests SLE, and while crescent formation can occur in severe lupus nephritis, **DPGN is the more specific and prevalent form** of nephritis for this presentation [1]. *focal segmental glomerulosclerosis* - **Focal segmental glomerulosclerosis (FSGS)** is a common cause of **nephrotic syndrome** and can lead to renal failure, but it is **not typically associated with the systemic symptoms** (rash, arthralgias, hair loss, thrombocytopenia) seen in this patient, which are characteristic of SLE. - While FSGS can rarely occur in lupus nephritis, it is less common than DPGN and lacks the widespread immune-mediated systemic features. *membranoproliferative glomerulonephritis* - **Membranoproliferative glomerulonephritis (MPGN)** can present with nephritic or nephrotic syndrome and may be associated with some autoimmune conditions like **cryoglobulinemia** or chronic infections (Hepatitis C). - However, the overall clinical context of **systemic inflammation, multi-organ involvement** (rash, arthralgias, thrombocytopenia), and the characteristic findings strongly favor **lupus nephritis**, for which **DPGN is the most characteristic finding** [1]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, p. 232. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 230-232. [3] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 528-529. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, p. 230.
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