paraneoplastic syndromes Flashcards
Trousseau’s Sign
- Trousseau’s Sign = Migratory superficial thrombophlebitis
- associated with adenocarcinoma
- Most commonly seen in pancreatic adenocarcinoma
- 6 fold increase chance of hematologic manifestation in lung adenocarcinomas that metastasize
- Due to increased mucin release by the tumor – increasing endothelial response
- Symptoms begin after primary tumor metastasizes to the liver
- Flushing, diarrhea, wheezing and right side valvular disease
- Niacin deficiency leading to
- dementia
- diarrhea
- dermatitis
What does these describe, and what would we find in this patient?
carcinoid syndrome
high levels of 5-HT NOT broken down by the liver
SCLC Criteria for the diagnosis of paraneoplastic endocrine syndromes
- Abnormal endocrine function without physiologic feedback regulation
- The absence of metastasis in the respective endocrine gland
- Improvement in endocrine function with the treatment of the tumor
- Evidence of the presence of hormones in the tumor or hormone synthesis by the tumor
SIADH: which condition does this arise and what is its criteria
an SCLC Endocrine Syndrome
SIADH
- Euvolemic, hypoosmolar, hyponatremic
- Headache, general fatigue, and muscle weakness
- Severe if Na <125 mEq/L
- Tx: demeclocycline and treat the tumor
Ectopic Cushing Syndrome
what cancer does this associate with and what are its criteria
SCLC ass. syndrome
what cancer does this associate with and what are its criteria
- Uncontrolled ACTH from non-pituitary tissue
- Carcinoid tumor and SCLC are most common causes of ECS
- Moon face, acne, purple striae, proximal muscle weakness,
- peripheral edema, hypertension, and metabolic alkalosis with hypokalemia
- Poor prognosis
A BIG FIB!
Cushing’s Syndrome: A BIG (all up arrows) FIB (all down arrows)
↑ appetite
↑ blood mmHg
↑ insulin resistance
↑ gluconeogenesis, lipolysis, proteolysis (because of decreased glucose utilization)
decrease in fibroblast activituy
decreases in inflammatory and immunse responses
decreased bone formation (decrease osteoblast activity)
Neurological syndromes SCLC general info
- SCLC – Neurological Syndromes
- Anti-Hu syndromes where Abs attack cancer cells
- Been cases of spontaneous regression of the SCLC in patients with Anti-Hu Abs
- Onconeural Abs attack components of the nervous system and portions of the tumor
- Less than 50% of the PNS have the antibodies
- Neurological symptoms typically occur before the actual cancer
- presents (~80%)
- PNS can effect any part of the nervous system – central, peripheral, NMJ
SCLC – Neurological Syndromes
Opsoclonus-myoclonus ataxia
- “dancing eyes, dancing feet”
- Neuroblastoma in children
- SCLC in adults
- https://www.youtube.com/watch?v=zX4j0IsFbAk
Paraneoplastic cerebellar degeneration
- Abs against Purkinje cell proteins (antigens)
- Acute: dizziness, nausea, and vomiting,
- Later Onset: gait instability, oscillopsia, diplopia, both truncal and appendicular ataxia, dysarthria, and dysphagia
Lambert-Eaton Syndrome
- Abs against pre-synaptic voltage gated Ca+ channels
- Gets better with more movement
- -helps distinguish between myasthenia gravis
Most common PNS and its causes
- Squamous Cell Carcinoma induced hypercalcemia
- Two causes:
-
PTHrP Release by squamous cells (lymphoma – Increase in Vit D); most common cause of hypercalcemia in SCC
- Act on the DCT and collecting duct of the kidney and bone resorption; does not increase vit D, no GI reabsorption
- Lytic bone lesions
-
PTHrP Release by squamous cells (lymphoma – Increase in Vit D); most common cause of hypercalcemia in SCC
- Two causes:
Lab Features and S/S
- Increased Ca2+
- Normal Vit D
- Normal PTH (different than PTHrP)
- Circulatory: thirst, polyuria, dehydration, and renal failure,
- GI: anorexia, nausea, vomiting, abdominal pain, and constipation,
- Neurologic effects: fatigue, muscular weakness, confusion, lethargy, irritability, and coma
- Psychiatric manifestations: depression, anxiety, etc.
what DOESNT this have?
nests: SCLC- it is a poorly differentiated neuroendocrine tumor
What is this? What is this patient most likely to have?
Nests in a carcinoid tumor. If this were part of a syndrome we might also seea niacin defiency leading to dementia and dermatitis, wheezing, and right side valvular disease
- Headache, general fatigue, and muscle weakness; severe if Na <125 mEq/L
- Moon face, acne, purple striae, proximal muscle weakness, peripheral edema, hypertension, and metabolic alkalosis with hypokalemia
- spontaneous regression in patients with anti-HU antibodies
- when would neurological symptoms in a patient with SCLC appear: before or after cancer detection?
- dizziness, nausea, and vomiting, gait instability, oscillopsia, diplopia, both truncal and appendicular ataxia, dysarthria, and dysphagia
- Movement disorder that improves with more movement
- “dancing eyes, dancing feet”
all of them are SCLC:
- SIADH SCLC
- Ectopic Cushings SCLC
- Factoid about SCLC
- after
- Paraneoplastic cerebellar degeneration- SCLC
- Lambert Eaton Syndrome- SCLC
- Opsoclonus-myoclonus- SCLC
Most common PNS
hypercalcemia in squamous cell carcinoma
most common cause of hypercalcemia in PNS
- PTHrP Release by squamous cells: most common cause of hypercalcemia in SCC