Multiple endocrine neoplasia type 2a associated with cutaneous lichen amyloidosis

Article Abstract:

Individuals with multiple endocrine neoplasia type 2a (MEN 2a) typically suffer from hyperplasia (excessive proliferation of cells) or malignant tumors of the endocrine glands. Those with type 2a develop thyroid cancer, parathyroid (an endocrine gland) hyperplasia and pheochromocytomas, a type of tumor. MEN 2a is a rare genetic disease, but many studies have been done on this condition as a part of hereditary malignancy research. The gene or genes involved in this disorder have been sought, and mapping and screening techniques have been used for this purpose. Similarly, cutaneous lichen amyloidosis (a skin disease characterized by the deposition of amyloid, a starchy substance) is a rare disorder that may also be genetically passed. A skin lesion characteristic of amyloidosis was detected in family members suffering from MEN 2a. In an attempt to find an association between the two disorders, a family pedigree with 19 members was evaluated for cutaneous lichen amyloidosis in a group with MEN 2a. After the results of biochemical tests for immunologic factors were analyzed, it was found that MEN 2a and cutaneous amyloidosis constitute a clearly defined autosomal (non-sex chromosome) dominant hereditary syndrome. Also, when the results of staining were evaluated, the keratin-like peptides rather than the calcitonin-like peptides were associated with dermal amyloid. It was suggested that individuals with hereditary lichen amyloidosis be screened to verify the syndrome frequency. (Consumer Summary produced by Reliance Medical Information, Inc.)

Amyloidosis

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Pancreatic carcinoma as a cause of unexplained pancreatitis: report of ten cases

Article Abstract:

Pancreatic cancer is often hard to diagnose. Retrospective study of patients with pancreatic cancer showed that 10 patients exhibited symptoms of inflammation of the pancreas (pancreatitis). In four patients, the signs and symptoms of pancreatitis resolved; in another four patients, pain and raised blood levels of amylase persisted; two patients had pancreatitis that recurred after a few months. When the diagnosis persisted, the patients underwent further testing and were found to have cancer as the underlying cause of their pancreatitis. Cancer should be considered in the diagnostic evaluation of patients with unexplained acute pancreatitis. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, Causes of, Pancreatic cancer, Pancreatitis, Cancer diagnosis

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Lessons learned from the management of a rare genetic cancer

Article Abstract:

Children who have a family history of multiple endocrine neoplasia type 2 (MEN-2) should have a genetic test for a mutation of the RET gene. If they have the mutation, they should have their thyroid gland removed to prevent thyroid cancer. Thyroid cancer is one of the manifestations of MEN-2.

Care and treatment, Editorial, Prevention, Thyroid cancer

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