Paraneoplastic syndromes are rare disorders triggered by an altered immune response to cancer. They involve non-metastatic effects that accompany malignant disease. Common types include neurological, endocrine, dermatological, and hematological syndromes. Evaluation involves ruling out other potential causes through testing like bloodwork, imaging, and biopsies to identify underlying cancers. While some symptoms may improve with cancer treatment, paraneoplastic syndromes can also result in irreversible organ damage or progression of cancer.

1. Paraneoplastic
Syndrome
Presented By
Dr.Md.Mushfequr Rahman Khan
FCPS Core Medicine Trainee
Medicine Unit-VI,DMCH

2. Objetives:
• Definition
• Epidemiology
• Mechanism
• Classification
• Explain how to evaluate a patient for paraneoplastic syndrome
• Treatment option available for Paraneoplastic syndrome
• Differential Diagnosis
• Prognosis
• Reference

3. Definition :
• Paraneoplastic Syndromes are rare disorders that are triggered by an
altered immune system response to a neoplasm.
• They are defined as clinical syndromes involving non-metastatic
systemic effects that accompany malignant disease.
• The first report of a paraneoplastic syndrome dates back to the 19th
century.

4. Epidemiology:
• The precise incidence and prevalence of paraneoplastic syndrome are
unknown because of the rarity of the disease.
• A literature review suggests that paraneoplastic syndrome occurs in up
to 8% of cancer patients.
• Neurology manifestation in the form of neuropathies are the most
common presentation.
• Male and female are affected equally.

5. Mechanisms:
• The Mechanisms of how cancers affect distant sites are not
understood precisely
.Cross –reacting Abs
. Production of physiologically active substances.
. Interference with normal metabolic pathways.
. Idiopathic.

6. Classification :
• Endocrine: Hormone secreting tumours.
• Neurological: Immune Mediated.
• Dermatological
• Haematological
• Rheumatological
• Others

7. Endocrine :
• Cushing’s syndrome
• SIADH
• Hypoglycaemia
• Hypercalcemia
• Carcinoid Syndrome
• Hyperaldesteronism

8. Neurological:

9. Neurological:

10. Dermatological :
• Acanthosis nigricans
• Dermatomyositis
• Laser-Trelat sign
• Necrolytic migratory Erythema
• Sweet’s syndrome
• Florid cutaneous
• Papillomatosis
• Pyoderma gangrenosum
• Acquired generalised hypertrichosis

11. Haematological:
• Granulocytosis
• Polycythaemia
• Trousseau’s sign
• Nonbacterial thrombotic endocarditis
• Anaemia
• DIC

12. Rheumatological :
• Paraneoplastic polyarthritis
• Polymyalgia Rheumatica
• Multicentric reticulohistiocytosis
• Hypertrophic osteoarthropathy

13. Others:
• Membranous glomerulonephritis
• Tumour-induced osteomalacie
• Stauffer syndrome
• Neoplastic fever
• Weight loss and anorexia- Lung, Gastrointestinal tract
• Fatigue

14. Endocrine :Cushing’s syndrome

15. Endocrine :SIADH

16. Endocrine: Hypoglycaemia

17. Endocrine :Hypercalcemia

18. Endocrine :Hypercalcemia

19. Endocrine : Hypercalcemia

20. Endocrine :Carcinoid Syndrome

21. Endocrine :Carcinoid syndrome

22. Neurological:

23. Neurological:

24. Neurological:LEMS
• Inv: Diagnosis is made by Electrophysiologically.
• Treatment :
🧵Symptomatic:
. Acetylcholinesterase inhibitor: Pyridostigmine.
. Guanidine
. Aminopyridine

25. Neurological: LEMS
• 🧵Immunological therapies:
. Plasma exchange
. Iv-immunoglobulines
. Oral immunosuppressive( prednison/Azathioprine)

26. LEMS VS Myasthenia Gravis

27. Neurological:

28. Neurological:

29. Neurological:

30. Opsoclonus and myoclonus syndrome

31. Dermatological:

32. Dermatological:

33. Dermatological:

34. Sweet Syndrome :

35. Dermatological :Pyoderma Gangrenosum
• Painful papules,ulcers,violaceous border & purulent exudates
• Malignancy: Multiple Myeloma,SCC,Mycosis fugoides
• Treatment :Respond to steroids

36. Haematological: Granulocytosis
• Protein: G-CSF,GM-CSF,IL-6
• Clinical Presentation: Asymtomatic
• Lab investigation : Elevated Neutrophils
• Associtated cancer: Lung cancer, GI cancer,Ovarian
cancer,Genitourinary cancer,Hodgkin’s disease.

37. Haematological:

38. Rheumatological:

39. Rheumatological:

40. Rheumatological:
• Multicentric Reticulohistocytosis :
.Violaceous papules over joints with
arthritis mutilans in 50%
patients.
. Ca.pancreas,SCC lung,Melanoma
. Due to destructive effects of
proteinases.

41. Renal:Membranous Nephropathy
• M/A: Ab deposition & complement activation against tumour Ag
deposited in glomeruli.
• C/F: That of nephrotic syndrome ( heavy proteinuria,renal vein
thrombosis,minimal haematuria)
• Associated Cancer: CA lung,stomach,colon.

42. Stauffer Syndrome:

43. Evaluation :
• Paraneoplastic syndrome is a diagnosis of exclusion and all possible
etilogies should be ruled out.
• Complete Blood count with differential
• Comprehensive metabolic panel.
• Urinalysis
• Tumour markers.
• Ectopic hormone level like PTHrP,ACTH,ADH.

44. Evaluation :
• Imaging- Xray,CT scan,MRI,PET scan
• Electrodiagnostic studies.
• Cerebrospinal fluid analysis (CSF)
• Protein electrophoresis of serum and CSF.
• Assay of paraneoplastic antibodies in the blood and CSF.
• Skin Biopsy
• Muscle Biopsy

45. Differential Diagnosis :
• Toxic Metabolic encephalopathy-rule out underlying infections &
electrolyte abnormalities.
• Infectious encephalitis :rule out any bacterial, viral or fungal etiologies
• Myelitis:rule out infectious or inflammatory causes.
• Chronic fatigue syndrome
• Mixed connective tissue disorder
• Bone marrow failure

46. Prognosis:
• Paraneoplastic syndrome have diverse clinical manifestations and hence
prognosis may vary.
• Few paraneoplastic disorders may resolve spontaneously or with primary
cancer treatment.
• Death may result from the underlying :
-progression of cancer
-from complications of cancer treatments
-an irreversible system impairment

47. Reference :
• 1) Davidson 23rd edition
• 2) https://www.slideshare.net/jameswheeler001/paraneoplastic-
syndromes-36986377
• 3) https://www.ncbi.nlm.nih.gov/books/NBK507890/
• 4) https://www.slideshare.net/IhabSamy/para-neoplastic-malignant-
syndromes
• 5) Wikipedia