|Title: Neurofibromatosis and
Multiple endocrine neoplasm
type III. Report of a case.
Contact Person: Alberto
G Pizarro (email@example.com)
Multiple endocrine neoplasia
type III (MEN III), also known as MEN IIb or
mucosal neuroma syndrome, can often be recognized
at an early age by its typical facies, marfanoid
habitus, and characteristic mucosal neuromas.
These features are usually present before
development of the more serious, life-threatening
complications that consist of medullary thyroid
carcinoma (MTC) and
Pheochromocytoma is the most
frequent cause of adrenal sudden death, which is
the unique sign in 1.5% of cases.
Pheochromocytoma is an explosive clinical
syndrome that may be characterized by either
severe hypertension associated with cerebral,
cardiac, and renal complications or hypotension,
or even shock and sudden death. Other emergencies
include lactic acidosis, hypoglycemia,
hypercalcemic crisis, severe hypokalemic
alkalosis, and acute bowel obstruction due to
bowel ischemia, necessitating prompt surgical
Many difficulties are
encountered by clinicians in attempting to
diagnose pheochromocytomas. Saudi (8) describe
several patients with unusual clinical features.
These include sudden death, cerebral hemorrhage,
refractory congestive heart failure, acute
abdominal pain, and hypercalcemia.
The condition may be familial,
having a pattern of inheritance consistent with
autosomal dominance. (9,10,11,12,13,14) Von
Recklinghausen´s Neurofibromatosis is inherited
as an autosomal dominant. It is characterized by
a wide variety of cutaneous neurologic,
endocrine, skeletal, ocular and visceral
manifestations. The findings in the skin provide
the best means of diagnosis. These mat range from
café au lait spots, usually more than six, to
multiple neurofibromas. Spinal and cranial nerves
may be involved. Superficial neurofibromata
seldom undergo malignant change : neurogenic
sarcoma may arise.(10,15,16,17)
Pheochromocytomas are tumors of
the adrenal gland which produce excess
adrenaline. Pheochromocytoma, a tumor arising
from the chromaffin tissue, is a rare and unique
disease which is mainly characterized by a great
deal of variability in its biological activity
and therefore in its clinical manifestation.
Pheochromocytomas arise from
the central portion of the adrenal gland which is
called the adrenal medulla. The adrenal medulla
is responsible for the normal production of
adrenaline which our body requires to help
maintain blood pressure (15) Pheochromocytoma
occurs with high incidence in this disease, your
frequency is 5-20 %.
Pheochromocytomas are often
referred to as the "ten percent tumor"
because they do many things about ten percent of
the time: malignant, bilateral, extraadrenal, in
children, familial, recurrent associated with MEN
syndromes, presnet with a stroke.
The Multiple endocrine neoplams
have neurofibromatosis and pheocromocytoma and
others neoplams are MEN type III o IIB.
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