HNPCC-Associated Pheochromocytoma

Mutations Associated with HNPCC Predisposition — Update of ICG-HNPCC/INSiGHT Mutation Database

In 1994, the International Collaborative Group on Hereditary Nonpolyposis Colorectal Cancer (ICG-HNPCC) established an international database of mutations identified in families with Lynch (HNPCC) syndrome. The data are publicly available at http://www.nfdht.nl. The information stored in the database was systematically analyzed in 1997, and at that time, 126 different predisposing mutations wer...

Pheochromocytoma Associated with Pregnancy: Anaesthetic Management

Pheochromocytomas are the tumor of neural crest cells of adrenal gland secretes Catecholamines (norepinephrine being greater than epinephrine). It causes hypertension in 0.1% of cases.1 80-90 % are solitary tumors localized to single adrenal gland usually right side while 10% are bilateral. They may originate any where in neural crest side from neck to inguinal ligament e.g. right atrium, splee...

[Familial pheochromocytoma associated to neurofibromatosis type 1].

Lynch syndrome (HNPCC)

Lynch syndrome, also referred to as hereditary non-polyposis colorectal cancer (HNPCC), accounts for somewhere between 2 and 5% of all CRC. It has been shown that Lynch syndrome (LS) is a result of germline mutations in genes involved in DNA mismatch repair (MMR) MSH2, MLH1, MSH6, and PMS2, whereas as HNPCC refers to families that adhere to the Amsterdam criteria or iterations of it. More recen...

Recurrent pheochromocytoma associated with glucagonoma. A case report.

We treated a hypertensive patient with recurrent pheochromocytoma (paraganglioma) associated with glucagonoma. No clinical symptom of glucagonoma was found and it could be detected only by a slight elevation of plasma immunoreactive glucagon (IRG) while the extirpated pancreatic tumor contained much IRG. This case could not be classified as either multiple endocrine neoplasia (MEN) type I or ty...