National University-Sudan

Faculty of Medicine
Third Year
Semester (5)
Endocrine System (ME-ENDO-315)
Lecture (40)
Lecture Title: Multiple Endocrine Neoplasia
 ‫بسم هللا الرحمن الرحيم‬

Multiple Endocrine
Neoplasia
Learning objectives
• By the end of this lecture the student should be able to:
• Define multiple endocrine neoplasia syndrome.
• Mention the component of MEN1
• Mention the components of MEN2.
 Multiple Endocrine Neoplasia
• Are a group of inherited diseases resulting in proliferative lesions
(hyperplasias, adenomas, and carcinomas) of multiple endocrine
organs.
Endocrine tumors arising in the context of MEN syndromes have
certain distinctive features:

1.These tumors occur at a younger age than sporadic cancers.

2.They arise in multiple endocrine organs. either synchronously or
metachronously.
3.Are often multifocal.
4. Usually preceded by an asymptomatic stage of endocrine
hyperplasia involving the cell of origin of the tumor.
5.Usually more aggressive and recur in a higher proportion.
Multiple Endocrine Neoplasia Type 1:
• Autosomal dominant.

• The gene (MEN1) is located at 11q13 and is a tumor

suppressor gene; thus, inactivation of both alleles of the
gene is believed to be the basis of tumorigenesis.
 Organs commonly involved include: 3P

1. Parathyroid (95%)
2. Pancreas (∼40%).
3. Pituitary (∼30%)
The "3 ps."
 Parathyroid:
• Primary hyperparathyroidism, arising from multiglandular
parathyroid hyperplasia, is the most consistent feature of
MEN-1.
• Occurs at younger ages (19 years) than sporadic counterpart (50 years)
 Pancreas:
• These tumors are usually aggressive and present with metastatic
disease or multi-focality.
• Pancreatic endocrine tumors are often functional (i.e., they
secrete hormones).
• Familial Zollinger-Ellison syndrome (ZES) associated with
gastrinomas: are most common pancreatic tumors.
• Complications include bleeding, perforation, and esophageal
strictures.
• In 90% of MEN1 patients with ZES, lesions are often multiple, and
located in duodenum
 Pancreas
• Insulinomas:
• 2nd most frequent pancreatic tumor in setting of MEN1 and
hypoglycemia, related to insulinomas, are common.
• VIPomsa, glucagonomas, are rare.

• Nonfunctioning pancreatic endocrine tumors:

20-40% of MEN1 patients.
When misdiagnosed, often discovered after local compression
&/or hepatic metastases
a small hypervascular lesion
in the pancreatic body with
regional lymph node
metastases.
Pancreatic gastrinomas and
insulinomas are commonly
observed in MEN1 patients.
 Pituitary:
• The most frequent pituitary tumor in MEN-1 patients is a
prolactin-secreting macroadenoma.
• Some individuals develop acromegaly from
somatotrophin-secreting tumors.
MULTIPLE ENDOCRINE NEOPLASIA TYPE 2:
• MEN type 2 is two distinct groups of disorders that are
caused by activating mutations of the RET protooncogene.
• Inherited in an autosomal dominant pattern.
• Has two types A&B.
 Multiple Endocrine Neoplasia Type 2A ,organs
involved include:
1.Thyroid:
Medullary carcinoma of the thyroid develops in virtually all
cases.
2.Adrenal medulla:
50% of patients develop adrenal pheochromocytomas.
3.Parathyroid:
Parathyroid gland hyperplasia with primary
hyperparathyroidism
Multiple Endocrine Neoplasia Type 2B

organs involved include:

• Thyroid
• Adrenal medulla.
Unlike MEN-2A, patients with MEN-2B: Do not develop
primary hyperparathyroidism
MEN 2 B
• It develops at an early age in infants , it characterized by :
 Medullary thyroid carcinoma MTC
 pheochromocytomas
Neuromas.
Marfan`s syndrome.
Thank you