Cancer In Werner Syndrome

WS patients are at increased risk of developing cancer (Goto et al., 1996; Monnat, 2001; Monnat, 2002). The elevated risk of neoplasia in WS patients is of particular biological interest. As discussed later, neoplasia may be an expression of important mechanistic links between WRN function in vivo, genome stability assurance, and the limitation of cell proliferation defects. The elevated risk of neoplasia in WS is selective in that only a small subset of neoplasms are clearly elevated in incidence as compared with general population controls (see Table 80.2). The following neoplasms, in order of decreasing frequency, have been observed most often in WS patients and occur at higher or much higher frequency than in normal population controls: soft tissue sarcomas, thyroid carcinoma, meningioma, malignant melanoma, malignant or preneoplastic hematologic disease, and osteosarcoma. Many other neoplasms, including common adult epithelial malignancies, have been observed in WS patients. However, it is not clear whether the risk of developing these neoplasms is significantly elevated. Of note, the histopathologic spectrum of neoplasms observed in WS overlaps with, though is distinct from, that observed in patients with Bloom syndrome and Rothmund-Thomson syndrome,

TABLE 80.2 Neoplasia in Werner syndrome

Frequent

Less common

(2/3 of neoplasms)

(1/3 of neoplasms)

Soft tissue sarcomas

Non-melanoma

malignant fibrous

skin cancer

histiocytoma

Hepatobiliary carcinomas

leiomyosarcoma

hepatocellular

fibrosarcoma

cholangiocarcinoma

malignant schwannoma

gallbladder

synovial sarcoma

Genito-urinary

rhabdomyosarcoma

bladder carcinoma

Thyroid carcinoma

uterine/ovarian carcinoma

follicular

renal cell carcinoma

papillary

prostate carcinoma

anaplastic

seminoma

Malignant melanoma

Gastro-intestinal

acral lentigenous melanoma

carcinoma

mucosal malignant

gastric

melanoma

esophagus

Meningioma

pancreas

benign

colon

multiple/malignant

Breast carcinoma

Hematological

Oro-pharyngeal

acute myelogenous

carcinoma

leukemias (M1-5)

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