What is Hereditary Non-Polyposis Colorectal Cancer?

Hereditary Non-Polyposis Colorectal Cancer
Endoscopy images, gross pathology, and histopathology from case 2. a Colonoscopy revealed an ulcerated, partially obstructing, large mass in the transverse colon (marked by asterisk). The pediatric colonoscope could not completely traverse this large mass due to tumor obstruction and alteration in the normal colon anatomy. b Upper endoscopy showed a large malignant ulcerated mass (marked by asterisk) with no bleeding in the second part of the duodenum. c Part of the surgical specimen including colon (marked by single arrow), duodenum (marked by double arrow), with the mass (marked by asterisk) with a diffusely mucinous appearance. d Hematoxylin and eosin stain of primary colonic adenocarcinoma (viewed at ×40) with large pools of mucin evident. The tumor was 14 cm, low grade mucinous adenocarcinoma of the colon with loss of mismatch repair proteins and high microsatellite instability. There was mild to moderate intratumoral lymphocytes, mild to moderate peritumor lymphocytes, with low grade tumor budding, and no lymphovascular invasion. Margins were negative and 22 lymph nodes were negative (not shown). Pancreaticoduodenectomy for locally advanced colon cancer in hereditary nonpolyposis colorectal cancer. Zhu R, Grisotti G, Salem RR, Khan SA - World journal of surgical oncology (2016). Not Altered. CC.

Hereditary non-polyposis colorectal cancer is a condition in which the tendency to develop colorectal cancer is inherited. “Nonpolyposis” means that colorectal cancer can occur when only a small number of polyps are present. In families with HNPCC, cancer usually occurs on the right side of the colon.

What is the Pathology of Hereditary Non-Polyposis Colorectal Cancer?

The pathology of hereditary non-polyposis colorectal cancer is:

-Etiology: The cause of hereditary non-polyposis colorectal cancer is an inherited mutation or abnormality.

-Genes involved: hMSH2.

-Pathogenesis: The sequence of events that lead to hereditary non-polyposis colorectal cancer are inherited mutations of DNA mismatch repair MMR genes responsible for the correction of DNA mismatches, such as base substitution mismatch and insertion-deletion mismatch.

-Histology: The histology associated with hereditary non-polyposis colorectal cancer shows that these cancers are more often mucinous, poorly differentiated, signet ring cells with lymphocytic infiltrate, or rimmed by a germinal center producing a lymphoid reaction.

How does Hereditary Non-Polyposis Colorectal Cancer Present?

Patients with hereditary non-polyposis colorectal cancer typically are all genders at age range before 40 years. The symptoms, features, and clinical findings associated with hereditary non-polyposis colorectal cancer include: rectal bleeding, stomach pain , weight loss and fatigue.

How is Hereditary Non-Polyposis Colorectal Cancer Diagnosed?

Hereditary non-polyposis colorectal cancer is diagnosed by sigmoidoscopy, colonoscopy, and biopsy.

How is Hereditary Non-Polyposis Colorectal Cancer Treated?

Hereditary non-polyposis colorectal cancer is treated by surgery.

What is the Prognosis of Hereditary Non-Polyposis Colorectal Cancer?

The prognosis of hereditary non-polyposis colorectal cancer is good. The 5-year survival rate in patients with hereditary nonpolyposis colorectal cancer HNPCC is estimated to be approximately 60%, compared with 40-50% for sporadic cases.

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Last updated on May 25, 2022