Paraneoplastic Syndrome

Aka: Paraneoplastic Syndrome

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II. Epidemiology

  1. Paraneoplastic Syndromes affect 1 to 8% of cancer patients

III. Mechanism

  1. Neoplasms secrete biologically active substances (e.g. Hormones, peptides, Cytokines)
    1. May have direct effects (e.g. endocrine) or stimulate an immune-mediated response
  2. Released substances may affect multiple organ systems
    1. Endocrine
    2. Neurologic
    3. Dermatologic
    4. Rheumatologic
    5. Hematologic
  3. Associated neoplasms (most common)
    1. Small Cell Lung Cancer
    2. Breast Cancer
    3. Gynecologic neoplasms (e.g. Ovarian Neoplasms)
    4. Hematologic malignancies (e.g. Leukemia)

IV. Precautions

  1. Early cause identification may significantly impact morbidity and mortality
    1. Reduce high symptom burden associated with Paraneoplastic Syndrome
    2. Counter Electrolyte and hormonal effects
    3. Early diagnosis of associated malignancy (paraneoplastic symptoms are often the cancer presentation)

V. Types: Endocrine

  1. Syndrome Inappropriate ADH Secretion (SIADH)
    1. Presents with symptoms related to isovolemic hyposmolar Hyponatremia
    2. Multiple associated cancers
  2. Hypercalcemia of Malignancy
    1. Presents with Altered Mental Status, Ataxia, hypertonia, Vomiting, Renal Failure
    2. Multiple associated cancers
  3. Cushing Syndrome
    1. Presents with Muscle Weakness, Peripheral Edema, weight gain, Hypertension
    2. Associated with Lung Cancer (50-60% of cases)
    3. Also associated with thymoma, Medullary Thyroid Cancer, Pancreatic Cancer, adrenal cancer, Ovarian Cancer
  4. Hypoglycemia
    1. Associated with Lung Cancer (including Mesothelioma), Sarcomas and gastrointestinal tumors
    2. IGF2 or Insulin secretion by Non-Islet cell tumors (NICTH)
    3. More commonly occurs with primary islet cell tumors (Insulinomas)

VI. Types: Neurologic

  1. Limbic Encephalitis
    1. Presents with mood changes, Hallucinations, Memory Loss and Seizures
    2. Various associated antibodies
      1. Anti-Hu (Small Cell Lung Cancer)
      2. Anti-Ma2 (Testicular Cancer - germ cell)
      3. Anti-CRMP5
      4. Anti-NMDA (see NMDA Encephalitis, associated with ovarian malignancies)
    3. Associated with Small Cell Lung Cancer (40-50%), testicular germ cell cancer (20%) or Breast Cancer (8%)
    4. Other associated cancers include thymoma, Teratoma, Hodgkin Lymphoma
  2. Paraneoplastic Cerebellar Degeneration
    1. Presents with Ataxia, Diplopia, Dysphagia and Dysarthria
    2. Associated with Small Cell Lung Cancer, gynecologic cancer, Hodgkin Lymphoma, Breast Cancer
  3. Lambert-Eaton Myasthenic Syndrome (LEMS)
    1. Presents with proximal leg weakness, diaphragmatic weakness and bulbar symptoms (speach, swallow)
    2. Associated with Small Cell Lung Cancer, Prostate Cancer, Cervical Cancer, Lymphomas
  4. Myasthenia Gravis
    1. Presents with presents with ocular, bulbar and limb fatigable weakness and diaphragmatic weakness
    2. Associated with thymoma (15% of Myasthenia Gravis patients)
  5. Autonomic Neuropathy
    1. Presents with Orthostatic Hypotension, gastrointestinal symptoms
    2. Associated with Small Cell Lung Cancers and thymomas
  6. Peripheral Sensory Neuropathy
    1. Presents with initial arm>leg Paresthesias, followed by Ataxia
    2. Associated with Small Cell Lung Cancer (70-80% of cases)
    3. Also associated with Breast Cancer, Ovarian Cancer, Sarcomas, Hodgkin Lymphoma

VII. Types: Dermatologic and Rheumatologic

  1. Acanthosis Nigricans
    1. Associated with abdominal adenocarcinomas (esp. Gastric Cancer)
  2. Dermatomyositis
    1. Presents with heliotrope rash, Gottron Papules, and erythematous rash face/neck/trunk
    2. Associated with Ovarian Cancer, Breast Cancer, Prostate Cancer, Lung Cancer, Colorectal Cancer
    3. Also associated with nasopharyngeal cancers and Non-Hodgkin Lymphoma
  3. Erythroderma
    1. Presents with a diffuse erythematous, exfoliating rash
    2. Associated with CLL, Cutaneous T-Cell Lymphoma, myeloproliferative disorders and GI cancers
  4. Hypertrophic Osteoarthropathy
    1. Presents with Digital Clubbing, bone thickening of wrists and ankles, large joint effusions
    2. Associated with intrathoracic tumors, lung metastases, Rhabdomyosarcoma, nasopharyngeal cancers
  5. Leukocytoclastic Vasculitis
    1. Presents with digital erosions and Cyanosis, leg palpable Purpura, Renal Insufficiency, Peripheral Neuropathy
    2. Associated with Leukemia, Lymphoma, Myelodysplastic Syndrome, Multiple Myeloma
    3. Also associated with Colon Cancer, Lung Cancer, urologic cancer and Rhabdomyosarcoma
  6. Paraneoplastic Pemphigus (PNP)
    1. Presents with severe mucosal erosions and skin bullae/erosions (esp. on palms/soles)
    2. Associated with Non-Hodkin Lymphoma, CLL, Leukemia, thymoma, Castleman disease, Sarcoma
  7. Polymyalgia Rheumatica
    1. Presents with proximal Muscle pain, stiffness
    2. Associated with Leukemia, Lymphoma, Myelodysplastic Syndrome
    3. Also associated with Colon Cancer, Lung Cancer, Renal Cancer, Prostate Cancer, Breast Cancer
  8. Sweet Syndrome
    1. Presents with tender erythematous Papules, Nodules, and Plaques on face, upper trunk, arms
    2. Associated with Leukemia (esp. AML), Non-Hodgkin Lymphoma, Myelodysplastic Syndrome, Multiple Myeloma
    3. Also associated with Breast Cancer, GI/GU/Gyn cancers, Melanoma, Testicular Cancer

VIII. Types: Hematologic

  1. Eosinophilia
    1. Presents with respiratory symptoms (e.g. Dyspnea, Wheezing)
    2. Associated with Lymphoma, Leukemia (CML, ALL), Lung Cancer, Thyroid Cancer, GI cancer, Renal Cancer, Breast Cancer
  2. Granulocytosis
    1. Typically asymptomatic
    2. Associated with Hodgkin Lymphoma, GI/GU cancer, Lung Cancer, Breast Cancer, brain cancer
  3. Pure Red Cell Aplasia
    1. Presents with Dyspnea, pallor, Syncope, Fatigue
    2. Associated with thymoma (most common), Leukemia, Lymphoma, Myelodysplastic Syndrome
  4. Thrombocytosis
    1. Typically asymptomatic
    2. Associated with head and neck cancers, Glioblastoma and Lymphoma
    3. Also associated with GI/Gyn cancers, Lung Cancer, Breast Cancer, Renal Cell Cancer, Prostate Cancer

IX. Resources

  1. Paraneoplastic Syndromes (StatPearls)
    1. https://www.ncbi.nlm.nih.gov/books/NBK507890/

X. References

  1. Henry (2019) Semin Diagn Pathol 36(4):204-10 +PMID: 30876820 [PubMed]
  2. Pelosof (2010) Mayo Clin Proc 85(9):838-54 +PMID: 20810794 [PubMed]

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Related Studies

Ontology: Paraneoplastic Syndromes (C0030472)

Definition (NCI) A classification for rare disorders of diverse organ systems (endocrine, neuromuscular, gastrointestinal, renal, dermatologic, rheumatologic, hematologic) that are affected by substances secreted by a distant neoplasm but not by the action of the neoplasm itself metastasizing to that organ or tissue. Less than 1 % of neoplasms are associated with these syndromes. An immune-mediated response to neoplasm-elaborated proteins may be the cause of these syndromes. Additionally, their manifestation may signal the presence of an occult neoplasm, potentially at an earlier stage of disease thereby leading to a better clinical outcome. Constitutional signs may include fever, night sweats, anorexia and cachexia. Clinical course is usually progressive. Prognosis is variable depending on the effective treatment of the underlying neoplasm.
Definition (NCI_NCI-GLOSS) A group of symptoms that may develop when substances released by some cancer cells disrupt the normal function of surrounding cells and tissue.
Definition (MSH) In patients with neoplastic diseases a wide variety of clinical pictures which are indirect and usually remote effects produced by tumor cell metabolites or other products.
Concepts Neoplastic Process (T191)
MSH D010257
SnomedCT 49783001
English Paraneoplastic Syndromes, Syndrome, Paraneoplastic, Syndromes, Paraneoplastic, Paraneoplastic syndromes, PARANEOPL SYNDROMES, Paraneoplastic Syndromes [Disease/Finding], paraneoplastic syndromes, paraneoplastic syndrome, Paraneoplastic syndrome, Paraneoplastic syndrome (disorder), Paraneoplastic Syndrome
Italian Sindrome paraneoplastica, Sindromi paraneoplastiche
Dutch paraneoplastische syndromen, paraneoplastisch syndroom, Paraneoplastisch syndroom, Paraneoplastische syndromen, Syndromen, paraneoplastische, Syndroom, paraneoplastisch
Portuguese Síndromes paraneoplásicas, Síndroma paraneoplásico, Síndromes Paraneoplásicas
Japanese 腫瘍随伴症候群, ｼｭﾖｳｽﾞｲﾊﾝｼｮｳｺｳｸﾞﾝ
Swedish Paraneoplastiska syndrom
Czech paraneoplastické syndromy, Paraneoplastické syndromy, Paraneoplastický syndrom
Finnish Paraneoplastiset oireyhtymät
Russian PARANEOPLASTICHESKIE SINDROMY, ПАРАНЕОПЛАСТИЧЕСКИЕ СИНДРОМЫ
Croatian PARANEOPLASTIČNI SINDROMI
Polish Zespoły paraneoplastyczne, Zespoły paranowotworowe
Hungarian Paraneoplasiás syndroma, Paraneoplasiás syndromák
Norwegian Paraneoplastiske syndromer
Spanish síndrome paraneoplásico (trastorno), síndrome paraneoplásico, Síndrome paraneoplásico, Síndromes Paraneoplásicos
French Syndrome paranéoplasique, Syndromes paranéoplasiques
German paraneoplastisches Syndrom, Paraneoplastische Syndrome
Derived from the NIH UMLS (Unified Medical Language System)

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