Lynch Syndrome II

Hereditary nonpolyposis colorectal neoplasms associated with other malignancies, more commonly of ovarian or uterine origin. When also associated with SEBACEOUS GLAND NEOPLASMS, it is called MUIR-TORRE SYNDROME.

Also Known As:

Networked: 15 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Hereditary Nonpolyposis Colorectal Neoplasms (Hereditary Nonpolyposis Colorectal Cancer)
2.	Colonic Neoplasms (Colon Cancer)
3.	Tetraploidy
4.	Neoplasms (Cancer)
5.	Carcinoma (Carcinomatosis)

Experts

1.	Lynch, J F: 1 article (01/2001)
2.	Lynch, H T: 1 article (01/2001)
3.	Shaw, T G: 1 article (01/2001)
4.	Deters, C A: 1 article (01/2001)
5.	Brand, R E: 1 article (01/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lynch Syndrome II:

1.	Lynch cancer family syndrome 2IBA 01/01/1993 - "The aggregation of colon, endometrial, ovarian, and possibly breast cancers in families has been described as a "cancer family syndrome" (now called Lynch syndrome II). " 11/01/1992 - "Our preliminary data not only show familial and probably heritable tendencies for endometrial adenocarcinoma, but further suggest that there are at least two distinct forms: (1) the previously described Lynch syndrome II (cancer family syndrome), and (2) a heretofore unemphasized entity characterized by a tendency to endometrial adenocarcinoma alone." 11/01/1992 - "In four other families, multiple first- and second-degree relatives of probands had adenocarcinoma of the uterus, colon, or ovary, presumably representing a cancer family syndrome (Lynch syndrome II). " 04/01/1992 - "These findings get seen as partially coincidental with some features of the cancer family syndrome (Lynch syndrome II), and we have considered that genetics conditions, like those of Lynch syndrome II, could to explain some multiple neoplasms in patients carrying then." 10/01/1991 - "The authors concluded that endometrial carcinoma is a significant component of cancer family syndrome and should be included in the main criteria of Lynch syndrome II."
2.	Biological Markers (Surrogate Marker)IBA 02/01/1992 - "Lynch syndrome II may be exceedingly difficult to diagnose due to an absence of premonitory clinical signs or biomarkers of genotypic susceptibility. " 10/01/1991 - "There are no premonitory physical signs or biomarkers which can identify the genotypic status in Lynch syndrome II. Diagnosis is therefore dependent on the pedigree, with attention to cancer of all anatomic sites, inclusive of those cardinal features of its natural history. " 08/15/1985 - "Nine families with the cancer family syndrome (CFS), or Lynch syndrome II, and two with hereditary site-specific colonic cancer (HSSCC), or Lynch syndrome I, were investigated for the following potential biomarkers of genotype status: in vitro tetraploidy of dermal fibroblast monolayer cultures; tritiated thymidine uptake (3HdThd) labeling of colonic mucosa; cytogenetics of peripheral blood mononuclear leukocytes; quantitative serum immunoglobulin determinations; methionine dependence in dermal fibroblasts in tissue culture; segregation analysis; and the study of gene linkage with respect to 25 landmark serum and blood group markers. "
3.	ThymidineIBA 08/15/1985 - "Nine families with the cancer family syndrome (CFS), or Lynch syndrome II, and two with hereditary site-specific colonic cancer (HSSCC), or Lynch syndrome I, were investigated for the following potential biomarkers of genotype status: in vitro tetraploidy of dermal fibroblast monolayer cultures; tritiated thymidine uptake (3HdThd) labeling of colonic mucosa; cytogenetics of peripheral blood mononuclear leukocytes; quantitative serum immunoglobulin determinations; methionine dependence in dermal fibroblasts in tissue culture; segregation analysis; and the study of gene linkage with respect to 25 landmark serum and blood group markers. "
4.	Methionine (L-Methionine)FDA Link 08/15/1985 - "Nine families with the cancer family syndrome (CFS), or Lynch syndrome II, and two with hereditary site-specific colonic cancer (HSSCC), or Lynch syndrome I, were investigated for the following potential biomarkers of genotype status: in vitro tetraploidy of dermal fibroblast monolayer cultures; tritiated thymidine uptake (3HdThd) labeling of colonic mucosa; cytogenetics of peripheral blood mononuclear leukocytes; quantitative serum immunoglobulin determinations; methionine dependence in dermal fibroblasts in tissue culture; segregation analysis; and the study of gene linkage with respect to 25 landmark serum and blood group markers. "
5.	Immunoglobulins (Immunoglobulin)IBA 08/15/1985 - "Nine families with the cancer family syndrome (CFS), or Lynch syndrome II, and two with hereditary site-specific colonic cancer (HSSCC), or Lynch syndrome I, were investigated for the following potential biomarkers of genotype status: in vitro tetraploidy of dermal fibroblast monolayer cultures; tritiated thymidine uptake (3HdThd) labeling of colonic mucosa; cytogenetics of peripheral blood mononuclear leukocytes; quantitative serum immunoglobulin determinations; methionine dependence in dermal fibroblasts in tissue culture; segregation analysis; and the study of gene linkage with respect to 25 landmark serum and blood group markers. "
6.	Muramidase (Lysozyme)IBA 07/01/1987 - "This report provides medical genetic/pathologic findings on an HNPCC kindred from southern Italy that shows criteria consistent with Lynch syndrome II. An international collaborative effort led to extension of this kindred with disclosure of a potentially new spectrum of phenotypic findings: an excess of gastric carcinoma; complete intestinal metaplasia and chronic atrophic gastritis restricted to the antrum; an apparent excess of colonic mucosal macrophagia, which by special stain appeared to be positive for mucin, with a constant content of both sialo and sulfomucin, a lack of iron, and an inconstant positivity for lysozyme obtained by immunoperoxidase technique; and findings of crypt atrophy of the colonic mucosa. "
7.	MucinsIBA 07/01/1987 - "This report provides medical genetic/pathologic findings on an HNPCC kindred from southern Italy that shows criteria consistent with Lynch syndrome II. An international collaborative effort led to extension of this kindred with disclosure of a potentially new spectrum of phenotypic findings: an excess of gastric carcinoma; complete intestinal metaplasia and chronic atrophic gastritis restricted to the antrum; an apparent excess of colonic mucosal macrophagia, which by special stain appeared to be positive for mucin, with a constant content of both sialo and sulfomucin, a lack of iron, and an inconstant positivity for lysozyme obtained by immunoperoxidase technique; and findings of crypt atrophy of the colonic mucosa. "
8.	IronIBA 07/01/1987 - "This report provides medical genetic/pathologic findings on an HNPCC kindred from southern Italy that shows criteria consistent with Lynch syndrome II. An international collaborative effort led to extension of this kindred with disclosure of a potentially new spectrum of phenotypic findings: an excess of gastric carcinoma; complete intestinal metaplasia and chronic atrophic gastritis restricted to the antrum; an apparent excess of colonic mucosal macrophagia, which by special stain appeared to be positive for mucin, with a constant content of both sialo and sulfomucin, a lack of iron, and an inconstant positivity for lysozyme obtained by immunoperoxidase technique; and findings of crypt atrophy of the colonic mucosa. "
9.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 12/01/1998 - "Although an increased inherent predisposition of the patient to exhibit those neoplasms, similar to Lynch syndrome II, is to be discussed, her previous treatments with cyclophosphamide and external radiotherapy are likely to explain at least the occurrence of urothelial cancer. "
10.	Hereditary pancreatitisIBA 01/01/2001 - "Hereditary pancreatic cancer (PC) appears to be exceedingly heterogeneous, as evidenced by its association with a variety of integrally associated diverse cancers and/or differing mendelian inherited cancer syndromes, which include the Lynch syndrome II variant of hereditary nonpolyposis colorectal cancer, hereditary breast-ovarian cancer syndrome in families with the BRCA2 mutation, hereditary pancreatitis, Peutz-Jeghers polyposis and the familial atypical multiple-mole melanoma syndrome in families with the CDKN2A (p16) germline mutation. "

Therapies and Procedures

1.	Radiotherapy 12/01/1998 - "Although an increased inherent predisposition of the patient to exhibit those neoplasms, similar to Lynch syndrome II, is to be discussed, her previous treatments with cyclophosphamide and external radiotherapy are likely to explain at least the occurrence of urothelial cancer. "
2.	Colectomy 05/01/1995 - "HNPCC is characterized by an autosomal dominant inherited predisposition to early age of onset (= 44 years) of CRC with proximal predominance (= 70% proximal to the splenic flecture) with an excess of synchronous and metachronous CRC (45% 10 years after initial hemicolectomy or segmental resection as opposed to subtotal colectomy), features which characterize the Lynch syndrome I variant, while the Lynch syndrome II variant of HNPCC shows all of these features, but in addition, there is a marked excess of carcinoma of the endometrium, ovary, small bowel, stomach, pancreas, and transitional cell carcinoma of the ureter and renal pelvis, lesions which are integral to this syndrome. "