By Dr. Zahoor 1. Endocrine manifestation of cancer are usually paraneoplastic syndrome i.e. rare...
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Transcript of By Dr. Zahoor 1. Endocrine manifestation of cancer are usually paraneoplastic syndrome i.e. rare...
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ENDOCRINE MANIFESTATION OF MALAGNANCYPARANEOPLASTIC SYNDROME
By Dr. Zahoor
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Paraneoplastic Syndrome
Endocrine manifestation of cancer are usually paraneoplastic syndrome i.e. rare manifestations of malignancy, but are not due to direct effect of cancer cells
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Paraneoplastic Syndrome
Why we get paraneoplastic syndrome?
The mechanism involves the production of hormones or other substances that act in endocrine or paracrine manner resulting in systemic manifestation
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Paraneoplastic Syndrome
Paraneoplastic Syndrome arise due to tumor secretion of hormones, peptides or cytokines
Paraneoplastic Syndrome may affect other organ systems like Endocrine, Neurologic, Dermatologic, Rheumatologic and Hematologic systems
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Paraneoplastic Syndrome
Most commonly associated malignancies include small cell lung cancer, breast cancer, gynecologic tumor
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Paraneoplastic Syndrome
History More than 100 years ago, it was
recognized that certain cancers cause various symptoms not attributable to direct tumor invasion or compression
These symptoms were labeled as paraneoplastic syndrome in 1940, but were poorly understood
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Paraneoplastic Syndrome (PNS)
History (cont) Now we know these syndrome occur
due to secretion of peptide or hormone (endocrine PNS) or cross activity between tumor and normal host tissue (neurologic paraneoplastic syndrome)
Note – PNS can manifest before a cancer diagnosis specially in neurologic paraneoplastic disorders
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Paraneoplastic Endocrine Syndrome
These usually occur due to production of hormones or peptide
The commonest endocrine paraneoplastic syndrome include
1- Inappropriate anti-diuretic hormone secretion (SIADH) 2- Hypercalcaemia 3- Cushing Syndrome There may be presenting features of an
underlying malignancy We will study each one
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Paraneoplastic Endocrine Syndrome
Syndrome of inappropriate ADH secretion (SIADH)
Increased ADH secretion causes hyponatremia, hypo-osmolality
Cause of SIADH- small cell lung cancer, which produces ADH
Patient may need hypertonic saline to correct serum sodium level
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Paraneoplastic Endocrine Syndrome
Hypercalcemia Hypercalcemia can occur in cancer
patients due to secretion of parathyroid hormone related protein (PTHrP) by tumor cells
Tumors which cause hypercalcemia - Squamous cell carcinoma lung,
breast cancer, multiple myeloma, lymphoma
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Paraneoplastic Endocrine Syndrome
Cushing Syndrome Due to increased secretion of ACTH, which
increases release of Cortisol from adrenal gland
Cancer which cause hypercalcemia - Small cell lung cancer Patient may present with Cushing
syndrome (features of hypertension, hypokalemia, muscle weakness) before the diagnosis of cancer is made
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Paraneoplastic Neurologic Syndrome
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Paraneoplastic Neurologic Syndrome (PNS)
They occur due to immune cross reactivity between tumor cells and components of nervous system
Patient produces tumor related antibodies and they can attack the nervous system
Patient condition can be diagnosed by PNS before cancer is diagnosed in 80% of cases
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Paraneoplastic Neurologic Syndrome (PNS)
PNS - CNS symptoms include - Personality changes - Cranial nerve deficits - Weakness or numbness - Lambert – Eaten myasthenic
syndrome - Myasthenia gravis
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Paraneoplastic Neurologic Syndrome (PNS)
PNS - CNS symptoms include (cont)
- Cerebellar degeneration - Sensory neuropathy - Autonomic neuropathy NOTE – These conditions can occur
itself due to other causes and may not be paraneoplastic
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Paraneoplastic Neurologic Syndrome (PNS)
Lambert – Eaten myasthenic syndrome There is muscle weakness, which improves
on exercise It is associated with small cell lung cancer
Myasthenia gravis Patient complains of muscle weakness
which increases on exercise Myasthenia gravis is associated with
Thymoma
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Paraneoplastic Dermatologic and Rheumatologic Syndrome
Paraneoplastic Dermatologic Syndrome
Acanthosis Nigricans It is characterized by thickened, hyper-
pigmented skin, predominantly in axilla and neck region
Most common cancer associated with Acanthosis nigricans is gastric adenocarcinoma
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Acanthosis nigricans
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Acanthosis nigricans
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Paraneoplastic Dermatologic Syndrome
Dermatomyositis It is inflammatory Myopathy, there is
proximal Myopathy Heliotrope rash (purple color) on
upper eyelid Erythromatous rash on face, neck,
chest Commonly associated malignancies
– breast, ovary, lung, prostate Creatine phosphokinase is raised
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Heliotrope rash (purple color) on upper eyelid
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Paraneoplastic Dermatologic Syndrome
Sweet Syndrome Sudden onset of painful,
Erythematosus plaques, papule, nodules on the face, trunk and extremities. There is fever and neutrophalia
Association - acute myeloid leukemia
and other tumors like breast, GIT
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Sweet Syndrome
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Paraneoplastic Rheumatologic Syndrome
Hypertrophic osteoarthropathy It is characterized by periostosis and
sub periosteal bone formation along the shaft of long bones and phalanges, joint swelling and pain
Associated with cancer lung (Bronchogenic carcinoma)
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Hypertrophic osteoarthropathy - Periostitis, Knee and Ankle
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Paraneoplastic Hematologic Syndrome
These conditions are usually detected after the cancer diagnosis
Paraneoplastic Eosinophilia Associated with lymphoma and
Leukemias but may be seen with carcinoma lung, GIT, gynecologic tumors
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Paraneoplastic Hematologic Syndrome
Pure red cell aplasia Associated with thymoma
Polycythemia Associated with renal tumor
Paraneoplastic Thrombocytosis
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Conclusion
The ability to recognize and treat paraneoplastic syndrome may have effect on clinical outcome ranging from early cancer diagnosis, to improved quality of life and increased delivery of tumor directed therapy
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Thank you