Paraneoplastic Syndromes

PARANEOPLASTIC
SYNDROMES
Tumors may produce signs and symptoms at
sites distant from the primary tumor or its
metastases and these are referred to as
paraneoplastic syndromes.
-symptom complexes in cancer-bearing patients
tumor production of substances that directly or indirectly

cause distant symptoms,
depletion of normal substances that leads to a paraneoplastic
manifestation
host response to the tumor that results in the syndrome.
important
they may represent the earliest

manifestation of a hidden neoplasm
in affected patients, they may represent
significant clinical problems and may even be
deadly
they may mimic metastatic disease and
therefore confound treatment
Among the best characterized of the paraneoplastic
syndromes are those producing polypeptide hormones,
such as adrenocorticotropin (ACTH) or parathyroid
hormone, that affect organ function at remote sites.
Additional tumor-derived proteins responsible for

paraneoplastic syndromes have been identified,
including various growth factors and cytokines such as
interleukin-1 (IL-1) and tumor necrosis factor (TNF).
PARANEOPLASTIC SYNDROMES
endocrinopathies
Clinical syndrome Major forms of underlying cancer Causal mechanism
Cushing syndrome Small cell carcinoma of lung ACTH or ACTH-like substance

Pancreatic carcinoma
Neural tumors
Syndrome of inappropriate antidiuretic Small cell carcinoma of lung; Antidiuretic hormone or atrial
hormone secretion intracranial neoplasms natriuretic hormones
Hypercalcemia Squamous cell carcinoma of lung Parathyroid hormone-related peptide,

Breast carcinoma TGF-alpha, TNF-alpha, IL-1
Renal carcinoma
Adult T-cell leukemia/lymphoma
Ovarian carcinoma
hypoglycemia Fibrosarcoma Insulin or insulin-like substance

Other mesenchymal sarcomas
Hepatocellular carcinoma
Carcinoid syndrome Bronchial adenoma (carcinoid)

Pancreatic carcinoma Serotonin, bradykinin, histamine
Gastric carcinoma
Polycythemia Renal carcinoma Erythropoietin

Cerebellar hemangioma
Hepatocellular carcinoma
The endocrinopathies are frequently encountered paraneoplastic
syndromes. Because the native cells giving rise to the cancer are
not of endocrine origin, the functional activity is referred to as
ectopic hormone production.
Among the endocrinopathies, Cushing syndrome (ACTH-
producing adenoma) is the most common.
-hypercortisolism is the most common cause of Cushingoid

features
50 % of patients with this endocrinopathy have carcinoma of

the lung
Hypercalcemia is the most common paraneoplastic
syndrome
-symptomatic hypercalcemia is most often related to some form of
cancer rather than to hyperparathyroidism.
2 general processes involved in cancer-associated hypercalcemia:
osteolysis induced by cancer, whether primary in bone, such as

multiple myeloma, or metastatic to bone from any primary lesion,
the production of calcemic humoral substances by extraosseous

neoplasms Hypercalcemia owing to skeletal metastases is not a
paraneoplastic syndrome
Many solid tumors produced PTH related peptide (PTHrP) which
shares homology with PTH in the first 13 aa and binds the PTH
receptor, thus mimicking the effect of PTH on bone and the
kidney.
Parathyroid related
-Solitary adenomas
-Multiple endocrine neoplasia
Malignancy-related
-Solid tumor with metastases (breast)
-Solid tumor with humoral mediation of hypercalcemia (lung, kidney)
-Hematologic malignancies (multiple myeloma, lymphoma, leukemia)
Hypoglycemia
-hepatomas or adrinocortical tumors may cause
hypoglycemia
-tumors often secrete incompletely processed insulin-
like growth factor II (IGF-II), a hormone capable of
activating insulin receptors and causing hypoglycemia
-rarely, hypoglycemia is due to insulin secretion by a
non-islet carcinoma
Nerve and muscle syndromes
Clinical Major forms of Causal mechanism
Syndrome underlying cancer
Myasthenia Bronchogenic carcinoma Immunologic
Disorders of the Breast carcinoma

central and peripheral
nervous systems
Dermatologic disorders
Acanthosis nigricans Gastric carcinoma Immunologic,secretion of epidermal
factor growth
Lung carcinoma
Uterine carcinoma
Dermatomyositis Bronchogenic, breast immunologic
cancer
The neuromyopathic paraneoplastic syndromes take diverse take
diverse forms such as peripheral neuropathies,cortical
cerebellar degenaration, polymyopathy and a myasthenic
syndromes similar to myasthenia gravis (MG).
MG- neuromascular disorder characterized by weakness and

fatigability of skeletal muscles.
* * the cause of these syndromes are poorly understood.

Acanthosis nigricans- can also be a reflection of an internal
malignancy most commonly of the GIT.
-may also be a reflection of an endocrinonopathy such as
acromegaly, Cushings syndrome, polycystic or ovary syndrome or
insulin-resistant diabetes mellitus.
-gray-black patches of verrucous
-50% of cases, >40, appearance of some lesions is associated with
some form of cancer.
Hypertrophic osteoarthropathy (HOA)
-clubbing of the digits
-periosteal new bone formation and synovial effusions
Primary or familial form-usually begins in childhood
Secondary is associated with intrathoracic malignancies and

variety of other disorders-adult
** clubbing is almost always a feature of HOA but can occur as an
isolated manifestation.
Osseous, articular, and soft tissue changes
Clinical syndromes Major forms of Causal mechanism

underlying cancer
Hypertrophic Bronchogenic carcinoma Unknown
osteoarthropathy and
clubbing of the fingers
Vascular and hematologic changes

Venous thrombosis (Trousseau Tumor products (mucins that
phenomenon) Pancreatic carcinoma activate clotting)
Bronchogenic carcinoma
Other cancers
Nonbacterial thrombotic Advanced cancers Hypercoagulability

endocarditis
Anemia Thymic neoplasms Unknown
Others ; nephrotic syndrome various cancers tumor antigens, immune complexes

The vascular and hematologic manifestations may appear in
association with a variety of form of cancer.
-deep venous thrombosis and pulmonary embolism.

-migratory or recurrent thrombophlebitis may be the initial
manifestation of cancer
** Trousseaus syndrome- coexistence of peripheral venous

thrombosis with visceral carcinoma particularly pancreatic
cancer

Paraneoplastic Syndromes

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PARANEOPLASTIC

tumor production of substances that directly or indirectly

they may represent the earliest

Additional tumor-derived proteins responsible for

Cushing syndrome Small cell carcinoma of lung ACTH or ACTH-like substance

Hypercalcemia Squamous cell carcinoma of lung Parathyroid hormone-related peptide,

hypoglycemia Fibrosarcoma Insulin or insulin-like substance

Carcinoid syndrome Bronchial adenoma (carcinoid)

Polycythemia Renal carcinoma Erythropoietin

-hypercortisolism is the most common cause of Cushingoid

50 % of patients with this endocrinopathy have carcinoma of

2 general processes involved in cancer-associated hypercalcemia:

osteolysis induced by cancer, whether primary in bone, such as

the production of calcemic humoral substances by extraosseous

Myasthenia Bronchogenic carcinoma Immunologic

Disorders of the Breast carcinoma

MG- neuromascular disorder characterized by weakness and

* * the cause of these syndromes are poorly understood.

Primary or familial form-usually begins in childhood

Secondary is associated with intrathoracic malignancies and

Clinical syndromes Major forms of Causal mechanism

Vascular and hematologic changes

Nonbacterial thrombotic Advanced cancers Hypercoagulability

Anemia Thymic neoplasms Unknown

Others ; nephrotic syndrome various cancers tumor antigens, immune complexes

-deep venous thrombosis and pulmonary embolism.

** Trousseaus syndrome- coexistence of peripheral venous

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