⁨⁨⁨أسئلة المحاضرة الرابعة أورام باطنة الدلتا ⁩  ⁩
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Questions and Answers

Which of the following syndromes is primarily associated with ectopic production of erythropoietin by cancer cells?

  • Eosinophilia
  • Thrombocytosis
  • Erythrocytosis (correct)
  • Granulocytosis

What is the typical hematocrit level in men diagnosed with paraneoplastic erythrocytosis?

  • >52% (correct)
  • >50%
  • >55%
  • >48%

Which type of cancer is NOT typically associated with granulocytosis?

  • Lung cancer
  • Renal cancer (correct)
  • Ovarian cancer
  • Gastrointestinal cancer

What is the primary cytokine involved in the development of eosinophilia in paraneoplastic syndromes?

<p>IL-5 (B)</p> Signup and view all the answers

What type of cancers is primarily associated with thrombocytosis?

<p>Lung cancer (A)</p> Signup and view all the answers

Which of the following is a common symptom of paraneoplastic erythrocytosis?

<p>Elevated hematocrit (A)</p> Signup and view all the answers

Which of the following cytokines plays a role in both granulocytosis and thrombocytosis?

<p>IL-6 (D)</p> Signup and view all the answers

What is the relationship between the severity of paraneoplastic syndromes and cancer progression?

<p>They parallel each other (D)</p> Signup and view all the answers

What should be measured in patients with erythrocytosis due to renal cell cancer, hepatoma, or CNS cancer?

<p>Red cell mass (B)</p> Signup and view all the answers

What is indicated if the red cell mass in a patient is elevated?

<p>Measurement of serum erythropoietin level (B)</p> Signup and view all the answers

Which treatment is likely to resolve erythrocytosis in patients with cancer?

<p>Successful resection of the cancer (A)</p> Signup and view all the answers

In cancer patients, granulocytosis is typically observed in which stage of the disease?

<p>Advanced-stage disease (D)</p> Signup and view all the answers

What prognosis is associated with thrombocytosis in cancer patients?

<p>Poorer prognosis (A)</p> Signup and view all the answers

Which condition is the most common thrombotic event in patients with cancer?

<p>Deep venous thrombosis (D)</p> Signup and view all the answers

What may be an initial manifestation of cancer, according to migratory or recurrent cases?

<p>Migratory or recurrent thrombophlebitis (C)</p> Signup and view all the answers

Which symptom would typically present in cancer patients developing deep venous thrombosis?

<p>Swelling or pain in the leg (A)</p> Signup and view all the answers

Which condition is characterized by dark discoloration in body folds and creases?

<p>Acanthosis Nigricans (C)</p> Signup and view all the answers

Which symptom is NOT associated with Dermatomyositis?

<p>Joint effusion (C)</p> Signup and view all the answers

What is a common inflammatory effect of Leukocytoclastic Vasculitis?

<p>Tissue destruction in small blood vessels (C)</p> Signup and view all the answers

Which condition presents with painful, edematous, and erythematous lesions on the skin?

<p>Sweet Syndrome (A)</p> Signup and view all the answers

Which of the following is a characteristic symptom of Hypertrophic Osteoarthropathy?

<p>Digital clubbing (B)</p> Signup and view all the answers

Which paraneoplastic syndrome is known as a fatal blistering disease?

<p>Paraneoplastic Pemphigus (A)</p> Signup and view all the answers

Which treatment approach is recommended for managing dermatologic and rheumatologic paraneoplastic syndromes?

<p>Standard treatment of non-paraneoplastic counterparts plus cancer-directed therapy (A)</p> Signup and view all the answers

Which symptom is typically associated with Sweet Syndrome?

<p>Fever and leukocytosis (D)</p> Signup and view all the answers

Which of the following symptoms are commonly observed in patients with pulmonary embolism?

<p>Dyspnea, chest pain, and syncope (A)</p> Signup and view all the answers

What is the typical treatment regimen for a patient diagnosed with pulmonary embolism?

<p>LMWH for at least 5 days then consider oral anticoagulants (D)</p> Signup and view all the answers

Which of the following cancers is most commonly associated with hypercalcemia?

<p>Squamous cell neoplasms (B)</p> Signup and view all the answers

What is a likely mechanism behind neurological paraneoplastic syndromes?

<p>Production of tumor-directed antibodies attacking nervous system components (A)</p> Signup and view all the answers

Which of the following is not commonly associated with neurological paraneoplastic syndromes?

<p>Initial brain cancer (B)</p> Signup and view all the answers

What are the treatments for neurological paraneoplastic syndromes?

<p>Removal of the antigen and treating the cancer (B)</p> Signup and view all the answers

Paraneoplastic encephalomyelitis may include which additional condition?

<p>Sensory neuronopathy (C)</p> Signup and view all the answers

Which neurological condition is not typically a result of paraneoplastic syndromes?

<p>Multiple sclerosis (B)</p> Signup and view all the answers

Which of the following features indicates a severe presentation of pulmonary embolism on physical examination?

<p>Tachycardia and hypotension (C)</p> Signup and view all the answers

In which situation could paraneoplastic syndrome manifest before cancer diagnosis?

<p>In response to developing cancer before its detection (B)</p> Signup and view all the answers

What defines a paraneoplastic syndrome?

<p>Clinical manifestations not directly related to a tumor’s physical presence. (B)</p> Signup and view all the answers

Which pathway is NOT a mechanism through which paraneoplastic syndromes occur?

<p>Direct invasion of tumor cells (D)</p> Signup and view all the answers

Cushing Syndrome as a paraneoplastic syndrome primarily results from the production of which hormone?

<p>Adrenocorticotropic hormone (ACTH) (D)</p> Signup and view all the answers

Which of the following symptoms is commonly associated with Hypercalcemia of malignancy?

<p>Altered mental status (D)</p> Signup and view all the answers

What type of cancer is most commonly associated with the Syndrome of Inappropriate Antidiuretic Hormone secretion (SIADH)?

<p>Lung cancer (C)</p> Signup and view all the answers

Which treatment is NOT typically used for managing Hypercalcemia of malignancy?

<p>Fluid restriction (C)</p> Signup and view all the answers

What characterizes endocrinal paraneoplastic syndromes?

<p>They arise from the production of hormones or peptides. (B)</p> Signup and view all the answers

Which of the following is NOT a typical tumor type associated with Cushing Syndrome?

<p>Breast cancer (C)</p> Signup and view all the answers

What is a common presentation symptom of SIADH?

<p>Anorexia (D)</p> Signup and view all the answers

Which syndrome is characterized by muscle weakness, hypertension, and weight gain?

<p>Cushing Syndrome (D)</p> Signup and view all the answers

Which symptom is most commonly associated with hypercalcemia?

<p>Lethargy (A)</p> Signup and view all the answers

What role does vasopressin play in paraneoplastic syndromes?

<p>Contributes to SIADH (C)</p> Signup and view all the answers

Which of these is NOT classified as a type of paraneoplastic syndrome?

<p>Vascular paraneoplastic syndromes (D)</p> Signup and view all the answers

Which protein is primarily responsible for stimulating erythrocytosis in paraneoplastic syndromes?

<p>Erythropoietin (EPO) (D)</p> Signup and view all the answers

Which of the following is a characteristic hematocrit level in men presenting with paraneoplastic erythrocytosis?

<blockquote> <p>52% (A)</p> </blockquote> Signup and view all the answers

Which cancer type is typically associated with eosinophilia in paraneoplastic syndromes?

<p>Lung cancer (D)</p> Signup and view all the answers

What is the typical clinical presentation of erythrocytosis in paraneoplastic syndromes?

<p>Asymptomatic with an elevated hematocrit (C)</p> Signup and view all the answers

Which condition is NOT commonly associated with thrombocytosis in cancer patients?

<p>Cervical cancer (D)</p> Signup and view all the answers

What type of cancer is associated with ectopic production of Granulocyte Colony-Stimulating Factor (G-CSF)?

<p>Lung cancer (B)</p> Signup and view all the answers

In the context of paraneoplastic syndromes, which symptom is commonly linked to thrombophlebitis?

<p>Swelling and pain in limbs (C)</p> Signup and view all the answers

Which interleukin is associated with both granulocytosis and thrombocytosis in paraneoplastic syndromes?

<p>Interleukin-6 (IL-6) (D)</p> Signup and view all the answers

Which of the following accurately describes the association between granulocytosis and cancer stages?

<p>Granulocytosis is more prevalent in advanced-stage cancer. (C)</p> Signup and view all the answers

What indirect treatment for erythrocytosis might be considered when tumors cannot be resected?

<p>Phlebotomy to alleviate symptoms of erythrocytosis. (C)</p> Signup and view all the answers

Which of the following statements regarding thrombocytosis in cancer patients is true?

<p>Asymptomatic presentation is common in patients with thrombocytosis. (A)</p> Signup and view all the answers

What is the significance of elevated serum erythropoietin levels in a cancer patient with erythrocytosis?

<p>It confirms the presence of paraneoplastic syndrome. (D)</p> Signup and view all the answers

What defines Trousseau's syndrome in patients diagnosed with cancer?

<p>Peripheral venous thrombosis linked with visceral carcinoma. (C)</p> Signup and view all the answers

Which symptom is generally associated with deep venous thrombosis in cancer patients?

<p>Swelling or pain in the leg. (D)</p> Signup and view all the answers

What is a critical factor in diagnosing paraneoplastic erythrocytosis in a patient with cancer?

<p>Measurement of the red cell mass. (A)</p> Signup and view all the answers

Which of the following conditions is most commonly associated with thrombosis in cancer patients?

<p>Deep venous thrombosis. (C)</p> Signup and view all the answers

Which condition is primarily characterized by painful, edematous, and erythematous lesions on the skin?

<p>Sweet Syndrome (B)</p> Signup and view all the answers

What symptom is NOT characteristic of Dermatomyositis?

<p>Digital clubbing (A)</p> Signup and view all the answers

Which paraneoplastic syndrome is known for causing a fatal blistering disease?

<p>Paraneoplastic Pemphigus (B)</p> Signup and view all the answers

Which of these symptoms is primarily linked to Hypertrophic Osteoarthropathy?

<p>Joint effusion (D)</p> Signup and view all the answers

What is the primary management strategy for dermatologic and rheumatologic paraneoplastic syndromes?

<p>Cancer-directed therapy plus standard treatments (C)</p> Signup and view all the answers

Which symptom is NOT commonly associated with Sweet Syndrome?

<p>Arthralgias (A)</p> Signup and view all the answers

Which of the following accurately describes Acanthosis Nigricans?

<p>Characterized by dark discoloration in body folds (A)</p> Signup and view all the answers

What condition is indicated by inflammation in small blood vessels leading to tissue destruction?

<p>Leukocytoclastic Vasculitis (C)</p> Signup and view all the answers

What is the typical initial treatment for patients diagnosed with pulmonary embolism?

<p>Low molecular weight heparin (LMWH) for at least 5 days (A)</p> Signup and view all the answers

Which of the following statements about paraneoplastic neurological syndromes is accurate?

<p>All cancers can potentially cause neurological paraneoplastic syndromes except brain cancer. (C)</p> Signup and view all the answers

Which symptom is least likely to be seen during a physical examination of a patient with pulmonary embolism?

<p>Hypertension (B)</p> Signup and view all the answers

Paraneoplastic syndromes related to neurological symptoms typically involve which mechanism?

<p>Tumor-directed antibodies attacking nervous system components (D)</p> Signup and view all the answers

What is the potential benefit of suppressing the immune response in treating paraneoplastic syndromes?

<p>It reduces the attack on healthy nervous tissue. (C)</p> Signup and view all the answers

Which of the following neurological conditions is specifically associated with paraneoplastic syndromes?

<p>Paraneoplastic cerebellar degeneration (PCD) (A)</p> Signup and view all the answers

In the context of paraneoplastic syndromes, which condition is primarily linked to a hypercalcemia diagnosis?

<p>Squamous cell neoplasms (A)</p> Signup and view all the answers

Which of the following is NOT a paraneoplastic neurological disease?

<p>Neurofibromatosis type 1 (D)</p> Signup and view all the answers

What distinguishes paraneoplastic syndromes from direct cancer effects?

<p>They are caused by immune reactions to tumor proteins. (C)</p> Signup and view all the answers

Which type of cancer is least likely to cause neurological paraneoplastic syndromes?

<p>Brain cancer (C)</p> Signup and view all the answers

What is the primary mechanism by which paraneoplastic syndromes are mediated?

<p>Cross reacting antibodies (C)</p> Signup and view all the answers

Which of the following conditions is a hematologic paraneoplastic syndrome?

<p>Thrombocytosis (D)</p> Signup and view all the answers

What factor is primarily responsible for the condition known as hypercalcemia of malignancy?

<p>Parathyroid hormone-related protein (PTHrP) (D)</p> Signup and view all the answers

Which symptom is least likely to be associated with the Syndrome of Inappropriate Antidiuretic Hormone secretion (SIADH)?

<p>Muscle rigidity (D)</p> Signup and view all the answers

In the context of endocrinal paraneoplastic syndromes, what effect does successful treatment of the underlying tumor often have?

<p>Often improves these conditions (C)</p> Signup and view all the answers

Which type of tumor is NOT typically associated with Cushing Syndrome?

<p>Melanoma (C)</p> Signup and view all the answers

What is a common treatment approach for managing hypercalcemia of malignancy?

<p>Saline hydration (D)</p> Signup and view all the answers

Which type of paraneoplastic syndrome is characterized by alterations in glucose metabolism?

<p>Endocrinal paraneoplastic syndrome (C)</p> Signup and view all the answers

Which condition is most commonly recognized in patients with paraneoplastic hypercalcemia?

<p>Altered mental status (A)</p> Signup and view all the answers

What hormonal change occurs in Cushing Syndrome?

<p>Increased cortisol levels (C)</p> Signup and view all the answers

Which of the following conditions may precipitate confusion in patients suffering from SIADH?

<p>Hyponatremia (D)</p> Signup and view all the answers

What is the typical metabolic derangement associated with hypercalcemia of malignancy?

<p>Hypercalcemia (D)</p> Signup and view all the answers

Which clinical feature is most characteristic of paraneoplastic endocrine syndromes?

<p>Metabolic derangements (C)</p> Signup and view all the answers

Which condition is a serious potential consequence of untreated hypercalcemia of malignancy?

<p>Acute renal failure (A)</p> Signup and view all the answers

Flashcards

Paraneoplastic Erythrocytosis

A condition where the red blood cell count is abnormally high due to a cancer, often specifically a renal cell cancer, hepatoma, or CNS cancer. It is diagnosed with a red cell mass test and an elevated erythropoietin level.

Granulocytosis

A higher than normal number of white blood cells, specifically neutrophils, in the blood. Often asymptomatic, it's more common in patients with advanced-stage disease and usually resolves with successful cancer treatment.

Paraneoplastic Thrombocytosis

A higher than normal number of platelets in the blood, often linked to advanced-stage cancers. It's usually asymptomatic and does not cause thrombosis directly. Though, patients with thrombocytosis have a poorer prognosis.

Migratory or Recurrent Thrombophlebitis

A condition characterized by recurrent blood clots typically in the legs. It's often the first sign of a cancer. The combination of blood clots and internal cancer, especially pancreatic cancer, is called Trousseau's syndrome.

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Deep Venous Thrombosis (DVT)

A common thrombotic complication in patients with cancer. This complication can occur in deep veins of the legs, causing swelling and pain.

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Pulmonary Embolism (PE)

A blood clot that travels from the legs to the lungs, leading to a blockage. It's a serious complication that can be deadly. It often occurs in patients with cancer.

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What is the main cause of paraneoplastic eryhtrocytosis?

The primary cause of paraneoplastic erythrocytosis is the overproduction of a hormone called erythropoietin by cancer cells.

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What is erythropoietin?

Erythropoietin (EPO) is a hormone that stimulates the production of red blood cells in the bone marrow.

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What is the hematocrit level in patients with paraneoplastic Erythrocytosis?

Paraneoplastic erythrocytosis typically results in a significantly elevated hematocrit level, which is the percentage of red blood cells in the blood.

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What are the symptoms of paraneoplastic eryhtrocytosis?

In most cases, paraneoplastic erythrocytosis is asymptomatic, meaning that the patient does not experience any noticeable symptoms.

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How is paraneoplastic eryhtrocytosos diagnosed?

Paraneoplastic erythrocytosis is often detected during a routine blood count, when the hematocrit level is found to be abnormally high.

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What types of cancers are associated with paraneoplastic eryhtrocytosis?

Paraneoplastic erythrocytosis is associated with various types of cancers, including renal cell carcinoma, hepatocellular carcinoma, and cerebellar hemangioblastomas.

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How does the severity of paraneoplastic eryhtrocytosis relate to the cancer?

The severity of paraneoplastic erythrocytosis often parallels the progression of the underlying cancer, meaning that as the cancer worsens, the erythrocytosis may also become more severe.

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What are paraneoplastic syndromes?

Disorders that accompany benign or malignant tumors but are not directly related to mass effects or invasion by the primary tumor or its metastases.

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How are paraneoplastic syndromes mediated?

Paraneoplastic syndromes can be caused by a variety of mechanisms, including cross-reacting antibodies, production of physiologically active factors, interference with normal metabolic pathways, and idiopathic causes.

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What are the four main types of paraneoplastic syndromes?

Paraneoplastic syndromes are classified into four main types: endocrine, hematologic, neurological, and dermatological.

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What is the cause of endocrinal paraneoplastic syndromes?

These syndromes result from the production of hormones or peptides that lead to metabolic derangements.

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How is the treatment of the underlying tumor related to endocrinal paraneoplastic syndromes?

Successful treatment of the underlying tumor often improves these conditions.

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Does the presence of endocrinal paraneoplastic syndromes correlate with cancer stage or prognosis?

The development of these disorders does not necessarily correlate with cancer stage or prognosis.

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What are some common endocrinal paraneoplastic syndromes?

Cushing syndrome, hypercalcemia, syndrome of inappropriate antidiuretic hormone secretion (SIADH), and hypoglycemia.

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What are the symptoms of Cushing syndrome?

The excess production of ACTH (adrenocorticotropic hormone) can lead to a variety of symptoms, including muscle weakness, peripheral edema, hypertension, weight gain, and centripetal fat distribution.

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What are the typical tumor types that can cause Cushing syndrome?

Cushing syndrome can be caused by tumors in various locations, including lung, thymus, pancreatic islet, and medullary thyroid carcinoma.

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What are the symptoms of hypercalcemia of malignancy?

The excess production of PTHrP (parathyroid hormone-related protein) can lead to a variety of symptoms, including altered mental status, weakness, ataxia, lethargy, hypertonia, renal failure, hypertension, and bradycardia.

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What are the typical tumor types that can cause hypercalcemia of malignancy?

Hypercalcemia of malignancy can be caused by tumors in various locations, including squamous cell (head and neck, lung, skin), breast, genitourinary, and gastrointestinal.

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What are the symptoms of SIADH (Syndrome of Inappropriate Antidiuretic Hormone Secretion)?

The excess production of vasopressin can lead to a variety of symptoms, including gait disturbance, falls, headache, nausea, fatigue, cramps, anorexia, confusion, lethargy, seizures, and coma.

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What are the typical tumor types that can cause SIADH?

SIADH can be caused by tumors in various locations, including lung (squamous, small cell), gastrointestinal, genitourinary, and ovary.

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Why are paraneoplastic hematologic syndromes less well-characterized than endocrinal syndromes?

The paraneoplastic hematologic syndromes in patients with solid tumors are less well characterized than the endocrine syndromes because the ectopic hormone(s) or cytokines responsible have not been identified in most of these tumors.

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Paraneoplastic Neurological Syndromes

A disorder where the body's immune system mistakenly attacks parts of the nervous system in response to a developing cancer.

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Onconeural Antibodies

Antibodies produced by the body that target cancer cells but also mistakenly attack components of the nervous system.

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Paraneoplastic Encephalomyelitis with Sensory Neuronopathy (PEM/PSN)

A common paraneoplastic neurological syndrome characterized by inflammation of the brain and spinal cord, often accompanied by sensory nerve damage.

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Limbic Encephalitis (LE)

A paraneoplastic syndrome that affects the hippocampus and other limbic system structures, leading to memory problems, behavior changes, and seizures.

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Brainstem Encephalitis (BE)

A paraneoplastic syndrome characterized by inflammation and damage to the brainstem, causing problems with balance, coordination, and swallowing.

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Paraneoplastic Cerebellar Degeneration (PCD)

A paraneoplastic neurological syndrome that causes degeneration of the cerebellum, leading to problems with coordination, movement, and balance.

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Paraneoplastic Opsoclonus-Myoclonus (POM)

A paraneoplastic syndrome characterized by rapid, involuntary eye movements (opsoclonus) and muscle jerks (myoclonus), often seen in children.

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Motor Neuron Syndromes

A paraneoplastic neurological syndrome that affects the motor neurons, leading to weakness, muscle atrophy, and paralysis.

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Myasthenia Gravis

A paraneoplastic neurological syndrome that affects the neuromuscular junction, causing muscle weakness and fatigue.

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Polymyositis/Dermatomyositis (PM/DM)

A paraneoplastic neurological syndrome that affects the muscles directly, leading to inflammation, weakness, and pain.

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Acanthosis Nigricans

A skin condition characterized by dark discoloration in body folds and creases, typically affecting the armpits, groin, and neck.

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Dermatomyositis

A disease that causes muscle weakness and skin rash. Symptoms include a red or purple rash on sun-exposed skin and eyelids, calcium deposits under the skin, muscle weakness, and difficulty speaking or swallowing.

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Hypertrophic Osteoarthropathy

A syndrome associated with a variety of cancers, characterized by clubbing of the fingers, increased bone growth, joint pain, and swelling.

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Leukocytoclastic Vasculitis

Inflammation of small blood vessels causing tissue damage. It is a common paraneoplastic syndrome.

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Paraneoplastic Pemphigus

A rare, severe, and often fatal mucocutaneous blistering disease associated with cancer.

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Sweet Syndrome

A syndrome characterized by painful, edematous, and erythematous papules, plaques, or nodules on the skin, accompanied by fever and elevated white blood cell count.

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Paraneoplastic Syndromes

A group of disorders that develop in association with cancer, often preceding a cancer diagnosis or recurrence.

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Management of Paraneoplastic Syndromes

Management of dermatologic and rheumatologic paraneoplastic syndromes usually involves treating the underlying cancer and managing the non-cancerous symptoms.

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What are the 4 main types of paraneoplastic syndromes?

Endocrine, Hematologic, Neurological, and Dermatological.

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What are the causes of endocrine paraneoplastic syndromes?

Hormone or peptide production by the cancer, leading to metabolic issues.

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What is Cushing Syndrome?

The overproduction of the hormone ACTH, leading to symptoms like muscle weakness, weight gain, and high blood pressure.

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What is Hypercalcemia of malignancy?

High calcium levels in the blood due to the cancer producing PTHrP.

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What is SIADH?

Too much vasopressin is produced, leading to low sodium in the blood and water retention.

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What is Hypoglycemia in paraneoplastic syndromes?

Low blood sugar levels caused by the cancer producing insulin-like factors.

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What are paraneoplastic neurological syndromes?

The body's immune system attacks parts of the nervous system in response to a cancer.

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What are onconeural antibodies?

Antibodies mistakenly attack parts of the nervous system.

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What is PEM/PSN?

Inflammatory condition of the brain and spinal cord, sometimes accompanied by sensory nerve damage.

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What is Limbic Encephalitis?

A syndrome that primarily affects the hippocampus, leading to memory problems, behavioral changes, and potentially seizures.

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What is Brainstem Encephalitis?

Inflammation of the brainstem, leading to problems with coordination, swallowing, and balance.

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Erythropoietin

The hormone that stimulates the production of red blood cells in the bone marrow.

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Ectopic Erythropoietin Production

Cancer cells, not the usual source, produce erythropoietin, leading to an abnormally high red blood cell count.

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Migratory Thrombophlebitis

A common paraneoplastic syndrome characterized by recurrent blood clots, often in the legs, that can be a first sign of cancer.

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Hematocrit

The percentage of red blood cells in the blood, often elevated in paraneoplastic erythrocytosis.

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Paraneoplastic Granulocytosis

A condition characterized by an increased number of neutrophils (a type of white blood cell) in the blood, often seen in advanced cancer.

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Deep Venous Thrombosis (DVT) and Pulmonary Embolism (PE) in Cancer

The occurrence of blood clots, typically in the deep veins of the legs, as a complication of cancer.

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Trousseau's Syndrome

The combination of peripheral venous thrombosis (blood clots in the legs) with internal cancer, particularly pancreatic cancer.

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How paraneoplastic erythrocytosis is caused

A condition involving a rise in red cell mass due to the cancer's production of erythropoietin.

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Treatment for Paraneoplastic Erythrocytosis

Successful tumor removal often resolves the erythrocytosis, and phlebotomy can control symptoms if the tumor cannot be removed.

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How are Paraneoplastic Syndromes managed?

The management often involves treating the underlying cancer and managing the non-cancerous symptoms.

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Study Notes

Paraneoplastic Syndromes (L4)

  • Paraneoplastic syndromes are disorders that accompany benign or malignant tumors.
  • They are not directly related to the tumor's mass effects or invasion.
  • Atypical clinical findings in a cancer patient should raise suspicion of a paraneoplastic syndrome.

Pathogenesis

  • Paraneoplastic syndromes are mediated through:
    • Cross-reacting antibodies
    • Production of physiologically active factors
    • Interference with normal metabolic pathways
    • Idiopathic mechanisms

Types of Paraneoplastic Syndromes

  • Endocrine paraneoplastic syndromes
  • Hematologic paraneoplastic syndromes
  • Neurological paraneoplastic syndromes
  • Dermatological paraneoplastic syndromes

Endocrine Paraneoplastic Syndromes

  • Result from hormone/peptide production causing metabolic disturbances.
  • Successful tumor treatment often improves these conditions.
  • Common syndromes include:
    • Cushing syndrome (ectopic ACTH production)
    • Hypercalcemia
    • SIADH (Syndrome of Inappropriate Antidiuretic Hormone secretion)
    • Hypoglycemia

Hypercalcemia of Malignancy

  • Caused by PTHrP (Parathyroid hormone-related protein).
  • Clinical presentation includes altered mental status, weakness, ataxia, lethargy, hypertonia, renal failure, hypertension (HTN), and bradycardia.
  • Typical tumor types: Squamous cell (head & neck, lung, skin), breast, genitourinary, and gastrointestinal.
  • Treatment: Saline hydration, forced diuresis with loop diuretics, and bisphosphonates.

SIADH (Syndrome of Inappropriate Antidiuretic Hormone secretion)

  • Caused by ectopic vasopressin production.
  • Clinical presentation includes gait disturbance, falls, headache, nausea, fatigue, cramps, anorexia, confusion, lethargy, seizures, and coma.
  • Typical tumor types: Lung (squamous, small cell), gastrointestinal, genitourinary, and ovarian.
  • Treatment: Treatment of the cause, fluid restriction, and hypertonic saline.

Hematologic Paraneoplastic Syndromes

  • Less well characterized than endocrine syndromes.

  • Severity often parallels the course of the cancer.

  • Common syndromes include erythrocytosis, granulocytosis, thrombocytosis, eosinophilia, thrombophlebitis, and thrombophlebitis.

  • Erythrocytosis associated with: Renal cancers, hepatocarcinoma, cerebellar hemangioblastomas, lung cancer, gastrointestinal cancer, and ovarian cancer

  • Granulocytosis associated with: Lung cancer, gastrointestinal cancer, ovarian cancer, genitourinary cancer, and Hodgkin's disease.

  • Thrombocytosis associated with: Lung cancer, gastrointestinal cancer, breast cancer, ovarian cancer, lymphomas, lymphoma, leukemia.

  • Thrombophlebitis associated with Lung cancer, pancreatic cancer, gastrointestinal cancer, breast cancer, genitourinary cancer, ovarian cancer, and lymphoma, prostate cancer

Neurological Paraneoplastic Syndromes

  • Immune-mediated diseases targeting the nervous system are caused by onconeural antibodies and associated onconeural antigen-specific T lymphocytes.
  • Common syndromes include:
    • Paraneoplastic encephalomyelitis
    • Limbic encephalitis
    • Brainstem encephalitis
    • Paraneoplastic cerebellar degeneration (PCD)
    • Paraneoplastic opsoclonus-myoclonus (POM)
    • Paraneoplastic retinal degeneration
    • Myelitis, Motor neurone syndromes, Sensory neuropathy, Sensorimotor neuropathy, Motor neuronopathy, Myasthenia gravis, Polymyositis/dermatomyositis (PM/DM), Acute necrotising myopathy

Dermatologic & Rheumatologic Paraneoplastic Syndromes

  • Many dermatologic and rheumatologic paraneoplastic syndromes occur without associated malignancy.
  • Management often involves cancer-directed therapy along with standard treatments for the corresponding non-paraneoplastic conditions.
  • Development often precedes or accompanies cancer diagnosis/recurrence.
  • Examples:
    • Acanthosis nigricans (dark skin discoloration)
    • Dermatomyositis (muscle weakness/skin rash)
    • Hypertrophic osteoarthropathy (digital clubbing, bone changes)
    • Leukocytoclastic vasculitis (small blood vessel inflammation)
    • Paraneoplastic pemphigus (mucous membrane blistering)
    • Sweet syndrome (painful skin lesions)

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