Diseases Associated with Gastrointestinal and Extra-Intestinal Cancers
There are certain diseases which are alerts for physicians to go deep in search of gastrointestinal cancers, either those that may already exist or those that are likely to develop in the future.
These can be a group of diseases or conglomerate of symptoms, called syndromes in sufferers who are mostly genetically susceptible and can include:
- Esophageal cancer is the eighth most common cancer and the sixth most common cause of cancer-related mortality in the world. Histologically, approximately 90% of esophagus cancer cases are squamous cell carcinoma. It is frequently found in smokers and alcoholics; and is associated with ingestion of hot food and less intake of fruits and vegetables.
A hereditary disease clearly associated with it is tylosis. Tylosi is characterized by a group of symptoms to be termed as a syndrome; most common manifestations being palmar-plantar keratoderma (PPK). Here, there is increased abnormal thickening of skin of palms and soles with formation of callosities in the pressure points. Oral leukoplakia, a thickening of cheek mucosa and esophageal mucosal thickening may accompany PPK.
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Barrett’s esophagus, often manifests as gastro-esophageal reflux disease (GERD) is a precursor of cancer esophagus.
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Breast carcinoma suppressor gene (BRCA) and esophageal cancer; it has been seen that a mutation in BRCA gene, which is a predisposing factor for breast cancer is also associated with cancer of the esophagus.
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Lynch syndrome; otherwise called hereditary non-polyposis colorectal cancer (HNPCC) accounts for 2-7% of all colorectal cancers and is associated with cancers of endometrium of the uterus, ovary, stomach, hepato-billiary system, pancreas, small bowel, brain and urinary system. It develops due to an automatism dominant mutation in at least one of the DNA mismatch repair genes. As these mutations are highly penetrant, multiple generations of a family are frequently affected by cancer.
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Familial atypical multiple mole melanoma syndrome is associated with hereditary pancreatic cancer and syndromes like Peutz-Jeghers syndrome and familial breast cancer syndrome. About 5% of pancreatic cancer in the US is hereditary.
- Peutz-Jogher syndrome (PJS); characterized by polyps in the gastrointestinal tract, mucocutaneous melanin pigmentation and various neoplasms. It is also associated with breast cancer, cervical cancer and endometrium of the uterus. The incidence of cancer among patients suffering with PJS has been estimated to be 15-fold higher than the incidence of cancer in the general population. PJS presents with characteristic flat, pigmented, freckle-like cutaneous lesions mainly on the lower lip, perioral area, buccal mucosa, periorbital area and eyelids. Continued on the next page
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