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ENDOCRINE MANIFESTATION OF MALAGNANCY PARANEOPLASTIC SYNDROME

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Presentation on theme: "ENDOCRINE MANIFESTATION OF MALAGNANCY PARANEOPLASTIC SYNDROME"— Presentation transcript:

1 ENDOCRINE MANIFESTATION OF MALAGNANCY PARANEOPLASTIC SYNDROME
By Dr. Zahoor

2 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 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

3 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 Most commonly associated malignancies include small cell lung cancer, breast cancer, gynecologic tumor

4 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

5 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

6 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

7 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 3% saline (hypertonic) to correct serum sodium level

8 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

9 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

10

11 Paraneoplastic Neurologic Syndrome

12 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

13 Paraneoplastic Neurologic Syndrome (PNS)
PNS - CNS symptoms include - Personality changes - Cranial nerve deficits - Weakness or numbness - Lambert – Eaten myasthenic syndrome - Myasthenia gravis - Cerebellar degeneration - Sensory neuropathy - Autonomic neuropathy NOTE – These conditions can occur itself due to other causes and may not be paraneoplastic

14 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

15 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

16 Acanthosis nigricans

17 Acanthosis nigricans

18 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

19 Heliotrope rash (purple color) on upper eyelid

20 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

21 Sweet Syndrome

22 Paraneoplastic Rheumatologic Syndrome
Hypertrophic osteoarthropathy It is characterized by peristosis and sub periosteal bone formation along the shaft of long bones and phalanges, joint swelling and pain Associated with cancer lung (Bronchogenic carcinoma)

23 Hypertrophic osteoarthropathy - Periostitis, Knee and Ankle

24 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

25 Paraneoplastic Hematologic Syndrome
Pure red cell aplasia Associated with thymoma Polycythemia Associated with renal tumor Paraneoplastic Thrombocytosis

26 Multiple Endocrine Neoplasia (MEN) Syndrome
MEN I – Pituitary, Parathyroid, Pancreas (3 Ps) (It is also called Wermer MEN1 as he noted in 1954, this syndrome is Autosomal dominant) MEN IIa – Medullary Thyroid Ca, Pheochromocytoma, Parathyroid (1M, 2Ps) (Also called Sipple Syndrome as he described in 1961) MEN IIb - Medullary Thyroid Ca, Pheochromocytoma, Mucosal Neuroma (2Ms, 1P) (Described by William in 1966)

27 Multiple Endocrine Neoplasia Syndrome

28 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

29 Thank you


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