Paraneoplastic Syndromes — MCQs

10 questions

The electrolyte abnormality seen in Ramesh, a 40-year-old patient presenting with polyuria, pain abdomen, nausea, vomiting, and altered sensorium, who was found to have bronchiogenic carcinoma, would be.

Radiation exposure can lead to which type of thyroid carcinoma?

Malignancy-associated hypercalcemia is due to?

A 26-year-old male presents to the outpatient department with a discrete thyroid swelling. On neck ultrasound, an isolated cystic swelling of the gland is seen. What is the risk of malignancy associated with this finding?

Which of the following is the MOST characteristic feature of Eaton-Lambert syndrome?

Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen

What is the correct statement about thymoma?

What is the most common type of acute myeloid leukemia in patients with Down's syndrome?

Which of the following has the least malignant potential?

Q10

Priapism in a young male could occur because of

Paraneoplastic Syndromes Indian Medical PG Practice Questions and MCQs

Question 1: The electrolyte abnormality seen in Ramesh, a 40-year-old patient presenting with polyuria, pain abdomen, nausea, vomiting, and altered sensorium, who was found to have bronchiogenic carcinoma, would be.

A. Hyperkalemia
B. Hypercalcemia (Correct Answer)
C. Hypocalcemia
D. Hypokalemia

Explanation: ***Hypercalcaemia*** - Bronchiogenic carcinoma can lead to **paraneoplastic hypercalcaemia**, often due to osteolytic metastasis or secretion of parathyroid hormone-related peptide (PTHrP) [2]. - Symptoms such as **polyuria, nausea, vomiting**, and **altered sensorium** are common manifestations of elevated calcium levels in the blood [1], [2]. *Hypocalcaemia* - Usually presents with **muscle cramps**, **tetany**, or **neuromuscular irritability**, unlike the symptoms described here. - Contrary to the patient's case, it does not typically result from bronchiogenic carcinoma. *Hyperkalemia* - Often causes **muscle weakness**, **arrhythmias**, and **ECG changes**, which are not aligned with the patient's presentation. - More commonly associated with conditions like **renal failure** or **adrenal insufficiency**, rather than bronchiogenic carcinoma. *Hypokalemia* - Typically results from conditions causing **excessive fluid loss**, such as **vomiting**, but leads to **muscle weakness** rather than altered sensorium [3]. - Rather unlikely in the context of a cancer patient showing signs that suggest an **elevated calcium level** instead.

Question 2: Radiation exposure can lead to which type of thyroid carcinoma?

A. Lymphoma
B. Papillary carcinoma (Correct Answer)
C. Medullary carcinoma
D. Follicular carcinoma

Explanation: ***Papillary carcinoma*** - Papillary thyroid carcinoma is strongly associated with **radiation exposure**, particularly during childhood [1]. - It is the most prevalent type of thyroid cancer and typically has a **good prognosis** [1]. *Lymphoma* - Thyroid lymphoma is rare and generally not linked to **radiation exposure**; it often presents as a **rapidly enlarging goiter**. - It is more commonly associated with **autoimmune thyroiditis**, not primary radiation effects. *Follicular carcinoma* - Follicular carcinoma shows a correlation with **iodine deficiency** rather than radiation exposure [1]. - Its presentation is more subtle, compared to the classical association of **radiation with papillary carcinoma**. *Medullary carcinoma* - Medullary thyroid carcinoma is primarily linked to **familial syndromes** like MEN 2 and not radiation exposure. - It arises from **parafollicular C cells**, making it clinically distinct from radiation-related types. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1098-1099.

Question 3: Malignancy-associated hypercalcemia is due to?

A. Vitamin D secretion by tumors
B. Cytokine-mediated osteoclast activation
C. Bone metastases causing osteolysis
D. Parathyroid hormone-related peptide (PTHrP) (Correct Answer)

Explanation: Malignancy-associated hypercalcemia is primarily due to **parathyroid hormone-related peptide (PTHrP)**, which mimics the action of parathyroid hormone, causing increased calcium release from bones [1][2]. This condition is often seen in **solid tumors**, particularly squamous cell carcinomas, and can lead to significant **hypercalcemia** [2][3]. *Tumor lysis syndrome* - Tumor lysis syndrome results from rapid cell lysis leading to **release of intracellular contents**, causing hyperuricemia, not directly hypercalcemia. - It is characterized by **electrolyte imbalances** such as hyperkalemia, hyperphosphatemia, but not primarily hypercalcemia. *IGF-b* - Insulin-like Growth Factor (IGF) is primarily involved in **growth processes** and does not lead to hypercalcemia. - While it has an association with cancer, it does not function in the pathway that elevates serum calcium levels. *IL-7* - Interleukin-7 (IL-7) is mainly related to **T-cell development** and does not play a role in hypercalcemia associated with malignancy. - While cytokines can influence various pathways in cancer, IL-7 is not implicated in **calcium metabolism**.

Question 4: A 26-year-old male presents to the outpatient department with a discrete thyroid swelling. On neck ultrasound, an isolated cystic swelling of the gland is seen. What is the risk of malignancy associated with this finding?

A. 48%
B. 12%
C. 24%
D. 3% (Correct Answer)

Explanation: ***3%*** - **Purely cystic thyroid nodules** (as described in this case with "isolated cystic swelling") have a **very low risk of malignancy**, typically **2-3%** or less. - According to **ATA guidelines** and **TIRADS classification**, purely cystic nodules are considered **low suspicion** lesions. - The cystic nature suggests a **benign process** such as a degenerated adenoma, colloid cyst, or simple cyst. - **Fine needle aspiration (FNA)** may still be considered if the nodule is >2 cm or has any suspicious solid components, but is often not required for purely cystic lesions. *48%* - This percentage is **significantly higher** than the actual malignancy risk for a purely cystic thyroid swelling. - Such a **high risk** would typically be associated with **solid nodules** exhibiting highly suspicious ultrasound features such as: - Microcalcifications - Irregular or spiculated margins - Taller-than-wide shape - Marked hypoechogenicity - Extrathyroidal extension *24%* - This percentage represents a **moderate to high risk** of malignancy, which is **not characteristic** of an isolated purely cystic thyroid swelling. - A risk in this range might be seen with: - **Mixed solid-cystic nodules** with predominantly solid components - Solid nodules with **intermediate suspicious features** on ultrasound *12%* - While lower than 24% or 48%, 12% is still **considerably higher** than the generally accepted malignancy risk for purely cystic thyroid nodules. - This risk level could be plausible for: - **Predominantly cystic nodules** with some eccentric solid components - Solid nodules with **mildly suspicious** features on ultrasound

Question 5: Which of the following is the MOST characteristic feature of Eaton-Lambert syndrome?

A. Repeated electrical stimulation enhances muscle power in it. (Correct Answer)
B. Neostigmine is not effective for this syndrome.
C. It is commonly associated with small cell lung cancer.
D. It can affect the ocular muscles.

Explanation: ***Repeated electrical stimulation enhances muscle power in it.*** - A hallmark feature of **Lambert-Eaton Myasthenic Syndrome (LEMS)** is the **potentiation of muscle strength** with repeated or high-frequency nerve stimulation [2]. - This is due to the disease pathophysiology where repeated stimulation allows the accumulation of **intracellular calcium**, leading to increased acetylcholine release at the neuromuscular junction. *Neostigmine is not effective for this syndrome.* - While it's largely true that **acetylcholinesterase inhibitors** like neostigmine are less effective in LEMS compared to myasthenia gravis, they can still provide some minor symptomatic relief [1]. - Therefore, stating it's *not effective* might be an oversimplification, and it's not the *most characteristic* feature. *It is commonly associated with small cell lung cancer.* - Although LEMS is frequently a **paraneoplastic syndrome** linked to **small cell lung cancer (SCLC)**, this association is a cause/etiology, not a direct characteristic feature of the neuromuscular dysfunction itself [1], [2]. - Approximately 50-60% of LEMS cases are paraneoplastic, with SCLC being the most common underlying malignancy [2]. *It can affect the ocular muscles.* - **Ocular muscle involvement** (e.g., ptosis, diplopia) is a prominent and often initial symptom in **myasthenia gravis** [2]. - In LEMS, ocular muscle weakness is **much less common** and typically mild, if present, distinguishing it from myasthenia gravis.

Question 6: Match the following: A) Caplan syndrome- 1) Found first in coal worker B) Asbestosis- 2) Upper lobe predominance C) Mesothelioma- 3) Involves lower lobe D) Sarcoidosis- 4) Pleural effusion is seen

A. A-3, B-4, C-2, D-1
B. A-1, B-4, C-3, D-2 (Correct Answer)
C. A-4, B-2, C-3, D-1
D. A-2, B-4, C-3, D-1

Explanation: **A-1, B-4, C-3, D-2** - **Caplan syndrome** was first described in **coal workers** with **rheumatoid arthritis** and progressive massive fibrosis. - **Asbestosis** is often associated with **pleural effusion**, which can be benign or malignant. - **Mesothelioma** typically involves the **lower lobes** of the lungs, specifically the pleura, and is strongly linked to asbestos exposure. - **Sarcoidosis** is characterized by **non-caseating granulomas**, which have a predilection for the **upper lobes** of the lungs. *A-3, B-4, C-2, D-1* - This option incorrectly states that Caplan syndrome involves the lower lobe; **Caplan syndrome** is defined by the presence of large nodules in the lungs of coal workers with rheumatoid arthritis, and their specific lobar distribution is not a defining characteristic. - This option incorrectly states that Mesothelioma has an upper lobe predominance; **Mesothelioma** is a pleural malignancy and typically involves the **lower lobes**, extending along the pleura. *A-4, B-2, C-3, D-1* - This option incorrectly associates Caplan syndrome with pleural effusion; **Caplan syndrome** manifests as rheumatoid nodules in the lungs, not primarily pleural effusion. - This option incorrectly states that Asbestosis has an upper lobe predominance; **Asbestosis** predominantly affects the **lower lobes** of the lungs, causing interstitial fibrosis. *A-2, B-4, C-3, D-1* - This option incorrectly states that Caplan syndrome has an upper lobe predominance; the defining feature of **Caplan syndrome** is the combination of rheumatoid arthritis and pneumoconiosis, not specific lobar involvement. - This option correctly identifies pleural effusion with asbestosis and lower lobe involvement with mesothelioma, but **Caplan syndrome** is not characterized by upper lobe predominance.

Question 7: What is the correct statement about thymoma?

A. Chest X-ray is the investigation of choice for the diagnosis of thymoma.
B. Thymoma is primarily located in the posterior mediastinum.
C. Thymoma is the most common neoplasia of the thymus. (Correct Answer)
D. Thymoma is usually asymptomatic and only occasionally causes symptoms.

Explanation: ***Thymoma is the most common neoplasia of the thymus.*** [1] - **Thymoma** is the most common primary tumor of the thymus, accounting for approximately **40-50% of anterior mediastinal masses** in adults. - It is a slow-growing tumor originating from the **epithelial cells** of the thymus [1]. *Chest X-ray is the investigation of choice for the diagnosis of thymoma.* - While a **chest X-ray** may show a widened mediastinum or an anterior mediastinal mass, it is not the investigation of choice for definitive diagnosis or staging [2]. - **CT scan** of the chest with contrast is the preferred imaging modality for evaluating thymomas, providing better anatomical detail and assessing invasiveness [2]. *Thymoma is typically asymptomatic and rarely causes any symptoms.* - Approximately **30-50% of patients with thymoma are asymptomatic** at diagnosis, with the tumor discovered incidentally on imaging [2]. - However, the remaining **50-70% of patients present with symptoms** related to **mass effect** (e.g., chest pain, dyspnea, cough) or **paraneoplastic syndromes** like myasthenia gravis, pure red cell aplasia, or hypogammaglobulinemia [2]. - Therefore, it is incorrect to say thymoma "rarely" causes symptoms. *Thymoma is primarily located in the posterior mediastinum.* - **Thymoma** is characteristically located in the **anterior mediastinum**, which is the most common site for thymic tissue. - Tumors primarily found in the posterior mediastinum are more commonly **neurogenic tumors**. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 571-574. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 572-574.

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: Which of the following has the least malignant potential?

A. Hamartomatous polyps associated with Peutz-Jeghers syndrome
B. Adenomatous polyps associated with Familial adenomatous polyposis
C. Juvenile polyps associated with juvenile polyposis syndrome (Correct Answer)
D. Adenomatous polyps associated with Lynch syndrome (HNPCC)

Explanation: ***Juvenile polyps associated with juvenile polyposis syndrome*** - **Isolated juvenile polyps** are benign hamartomas with **minimal intrinsic malignant potential**. - While **juvenile polyposis syndrome (JPS)** as a condition carries an increased lifetime colorectal cancer risk (15-38%), this is primarily due to the development of **co-existing adenomatous polyps** or dysplastic changes, not from the typical juvenile polyp histology itself [1]. - Among the listed options, juvenile polyps have the **least malignant potential**. *Hamartomatous polyps associated with Peutz-Jeghers syndrome* - **Peutz-Jeghers syndrome (PJS)** is characterized by distinctive **hamartomatous polyps** and carries a significantly increased lifetime risk of various cancers, including colorectal, gastric, small intestine, and pancreatic cancers (cumulative risk ~93% by age 70) [1]. - Although hamartomas are benign lesions, these polyps can undergo **malignant transformation** or harbor areas of **adenomatous change and dysplasia**, contributing to the cancer risk [4]. *Adenomatous polyps associated with Familial adenomatous polyposis* - **Familial adenomatous polyposis (FAP)** is caused by a germline mutation in the **APC gene** and is characterized by hundreds to thousands of **adenomatous polyps** in the colon [2]. - Without colectomy, there is a nearly **100% lifetime risk of developing colorectal cancer** due to the malignant transformation of these adenomas [3]. - This represents the **highest malignant potential** among the options. *Adenomatous polyps associated with Lynch syndrome (HNPCC)* - **Lynch syndrome (hereditary nonpolyposis colorectal cancer, HNPCC)** is caused by mutations in DNA mismatch repair genes, leading to an increased risk of various cancers, most notably **colorectal cancer** (lifetime risk 50-80%) [2]. - The polyps associated with Lynch syndrome are typically **adenomatous polyps**, which develop at an earlier age and progress more rapidly to cancer (2-3 years vs 10-15 years for sporadic adenomas) compared to sporadic adenomas [3]. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, p. 813. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, p. 817. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, pp. 821-822. [4] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Gastrointestinal Tract, pp. 813-814.

Question 10: Priapism in a young male could occur because of

A. leukaemia (Correct Answer)
B. carcinoid tumour of appendix
C. testicular cancer
D. penile cancer

Explanation: ***Leukaemia*** - In leukaemia, especially **myeloid leukaemia**, immature white blood cells can accumulate in the **corpus cavernosa**, leading to stasis and **venous occlusion**. [1] - This cellular congestion prevents venous outflow from the penis, causing a prolonged and **painful erection (priapism)**. *Carcinoid tumour of appendix* - A carcinoid tumour of the appendix is typically associated with **carcinoid syndrome**, which involves symptoms like flushing and diarrhoea. - It does **not directly cause priapism**, as its mediators (e.g., serotonin) do not typically induce this specific local vascular event. *Testicular cancer* - Testicular cancer primarily manifests as a **painless lump in the testis** and can cause symptoms related to metastasis, but **priapism is not a typical direct presenting symptom**. - While some cancers can cause paraneoplastic syndromes, testicular cancer is not associated with priapism. *Penile cancer* - Penile cancer typically presents as a **lesion, ulcer, or mass on the penis**, often associated with pain, bleeding, or discharge. - While it affects the penis, it does **not typically cause prolonged erections (priapism)**, but rather local tissue destruction or obstruction.

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