Multiple endocrine neoplasm type III. Report of a case.

Contact Person: Alberto G Pizarro (rediegal@homonet.com.mx)

Discussion & Conclusion

We showed a Multiple endocrine neoplasia type III (MEN 3), also known as MEN 2b, is a syndrome that may be recognized at a young age by its characteristic numerous mucosal neuromas of stomachand. These features are generally evident before the development of pheochromocytoma. This syndrome most likely results from a dysplasia of neuroectodermal tissue. (5) (21) Pheochromocytoma is the most frequent cause of adrenal sudden death, which is the unique sign in 1.5% of cases. After a short review of the literature, we report a case of a 38 years-old symptomatic woman, who died suddenly and unexpectedly while receiving antihypertensive treatment. Our patient showed signs of shock and an acute pulmonary edema was confirmed.(22) Coagulative myocytolysis, found in all the present cases, always seen in the outer zone of an early infarct and in the surrounding normal myocardium at any stage of the repair process in most of the acute infarcts and in most cases of sudden death. The myocardial cell dies in a hypercontracted state, with early myofibrillar rhexis, and anomalous irregular cross-band formations. This tetanic death is similar to that seen in pheochromocytoma and in experimental catecholamine-induced necrosis.(23) A plausible death pathway is the following: a single catecholaminic peak might have induced myocardic vessel spasm, which in turn could be responsible for a lethal arrhythmia; the dramatic pressure drop can be the effect of pump failure or of a paradoxical disproportionate beta stimulation. (24) The importance of diagnosing the syndrome at an early stage and of investigating the relatives of a patient manifesting this potentially fatal syndrome are stressed.(5)

Gross examination did not allow to reveal cause of death.

Microscopy revealed signs of acute myocardial ischemia, in the lack of any coronary and catecholaminic heart disease. Azan-Mallory trichromic stain was found to be necessary in revealing myocardial lesions. (24) Histologic pattern showed extensive myocardial fibrosis and some acute myocytolytic areas. This pattern is equal to that of prolonged and decompensated stress cardiomyopathy. These pathologic pictures are both caused by catecholamines cardiotoxicity.(25)

A plausible death pathway is the following: a single catecholaminic peak might have induced myocardic vessel spasm.

Difficulty in the correct diagnosis is testified by the fact that quite often the tumor may go unrecognized in life and up to 50% is found incidentally at post-mortem.(10,11) The tumor is generally suspected on clinical ground for the presence of paroxysmal hypertension but this sign is largely aspecific and often absent. Conversely, each incidental adrenal mass should be suspected of being a pheochromocytoma even in the absence of evident cardiovascular manifestations. The diagnosis of pheochromocytoma has to be based on laboratory tests demonstrating dysregulation and/or overproduction of chromaffin secretory products, namely catecholamines (7,12,15).

Conclussions:

This patient illustrates many of the characteristics of endocrine tumors, such as multiplicity of glandular involvement and a rather benign she died of heart failure by effect of elevated catecholamines. She had Multiple endocrine neoplams type III or IIb with Systemic Neurofibromatosis lacated in skin, stomach, spin cord, pheochromocytoma, paraganglioma, thecofibroma, hyperplasia of parathyroids. She had sudden death by acute myocardial ischemia.

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