MEN2 is rare genetic disorder causes tumors in multiple endocrine glands. RET proto-oncogene germline mutations cause autosomal dominant MEN2 transmission.
The two types of MEN2 syndromes are:
MEN2A
MEN2B
Medullary Thyroid Carcinoma is a cancer of the thyroid gland that originates from C-cells.
Primary hyperparathyroidism overactivity of the parathyroid glands to increase levels of parathyroid hormone and high calcium levels.
Pheochromocytoma is a tumor of the adrenal glands that can cause high blood pressure and other symptoms due to excessive production of catecholamines.
Genotype-phenotype correlation in inherited MTC can personalize risk management for childhood carriers.
MEN2A and MEN2B are inherited in an autosomal dominant pattern with only one altered gene copy needed.
Epidemiology
The overall frequency of MEN2 in the United States is 1 case per 50000 population.
50% MEN2A patients with RET gene mutations develop disease up to 50 years old.
Genetic background and healthcare access influence reported incidences. MEN2B may be more prevalent in specific populations.
Family history of MEN2 increases risk due to autosomal dominant inheritance.
Anatomy
Pathophysiology
GDNF critical in enteric nervous system and renal organogenesis pathways for normal function.
Intracellular tyrosine kinase core triggers signalling events through second messenger molecules downstream.
MEN2A patients have bilateral, multicentric MTC with C-cell hyperplasia, while sporadic MTC is unilateral.
Pheochromocytomas in MEN2A patients are benign and diagnosed with medullary thyroid cancer.
Etiology
The causes for MEN2 are:
Genetic mutations
Inheritance Pattern
Autosomal Dominant
Oncogenesis
Tumor Formation
Genetic Testing
Genetics
Prognostic Factors
Prognosis in MTC patients is influenced by disease stage at diagnosis. MEN2B-associated MTC is most aggressive, while FMTC without other malignancies is least aggressive.
Prompt detection of MTC saves lives; monitoring for pheochromocytomas prevents hypertension episodes.
Adrenal medulla tumors found in 50% of MEN2 patients in their late 30s.
Surgery should be considered for confirmed adrenal mass enlargement and bilateral masses require specific procedure.
Clinical History
Collect details including family and medical history to understand clinical history of patient.
Physical Examination
Neck Examination
Skeletal Examination
Oral Examination
Skin Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Symptoms for MEN 2A&2B are:
Palpable lumps, hoarseness, difficulty in swallowing, kidney stones, bone pain, abdominal discomfort, hypertension, palpitations, sweating, and headaches
Differential Diagnoses
Hyperparathyroidism
Multiple Endocrine Neoplasia Type 1
Neurofibromatosis Type 1
Medullary Thyroid Carcinoma
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Radiation therapy is used alone or with surgery to treat tumors. It may include prostaglandin inhibitors for medullary thyroid cancer.
Pheochromocytomas should be evaluated and removed before other surgeries to ensure patient stability.
Thyroid hormone needed after total thyroidectomy in RET mutation carriers or medullary thyroid carcinoma.
Initiate 24-hour urine catecholamine collections and annual serum calcium/PTH tests to detect pheochromocytoma early.
Annual screening for hyperparathyroidism with calcium and PTH levels.
Vandetanib can benefit late-stage MTC patients with symptomatic disease ineligible for surgery due to its dual kinase inhibition.
In the initial treatment phase, evaluation of MEN2 tumor and imaging test to confirm diagnosis.
Pharmacologic therapy is effective in the treatment phase as it includes use of tyrosine kinase inhibitors, diuretics, calcium metabolism modifiers, thyroid Hormones, and corticosteroids.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention therapies.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
Not safe and efficacious for children less than 10 years
For more than 10 years (Victoza only)- 0.6 mg subcutaneously daily
1 week later- 0.6 mg each day. Increase the dose to 1.2 mg/day if additional glycemic level is desired
»
Home » CAD » Multiple Endocrine Neoplasia Type 2 (MEN2)
Multiple Endocrine Neoplasia Type 2 (MEN2)
Updated :
September 26, 2024
MEN2 is rare genetic disorder causes tumors in multiple endocrine glands. RET proto-oncogene germline mutations cause autosomal dominant MEN2 transmission.
The two types of MEN2 syndromes are:
MEN2A
MEN2B
Medullary Thyroid Carcinoma is a cancer of the thyroid gland that originates from C-cells.
Primary hyperparathyroidism overactivity of the parathyroid glands to increase levels of parathyroid hormone and high calcium levels.
Pheochromocytoma is a tumor of the adrenal glands that can cause high blood pressure and other symptoms due to excessive production of catecholamines.
Genotype-phenotype correlation in inherited MTC can personalize risk management for childhood carriers.
MEN2A and MEN2B are inherited in an autosomal dominant pattern with only one altered gene copy needed.
The overall frequency of MEN2 in the United States is 1 case per 50000 population.
50% MEN2A patients with RET gene mutations develop disease up to 50 years old.
Genetic background and healthcare access influence reported incidences. MEN2B may be more prevalent in specific populations.
Family history of MEN2 increases risk due to autosomal dominant inheritance.
GDNF critical in enteric nervous system and renal organogenesis pathways for normal function.
Intracellular tyrosine kinase core triggers signalling events through second messenger molecules downstream.
MEN2A patients have bilateral, multicentric MTC with C-cell hyperplasia, while sporadic MTC is unilateral.
Pheochromocytomas in MEN2A patients are benign and diagnosed with medullary thyroid cancer.
The causes for MEN2 are:
Genetic mutations
Inheritance Pattern
Autosomal Dominant
Oncogenesis
Tumor Formation
Genetic Testing
Prognosis in MTC patients is influenced by disease stage at diagnosis. MEN2B-associated MTC is most aggressive, while FMTC without other malignancies is least aggressive.
Prompt detection of MTC saves lives; monitoring for pheochromocytomas prevents hypertension episodes.
Adrenal medulla tumors found in 50% of MEN2 patients in their late 30s.
Surgery should be considered for confirmed adrenal mass enlargement and bilateral masses require specific procedure.
Collect details including family and medical history to understand clinical history of patient.
Neck Examination
Skeletal Examination
Oral Examination
Skin Examination
Symptoms for MEN 2A&2B are:
Palpable lumps, hoarseness, difficulty in swallowing, kidney stones, bone pain, abdominal discomfort, hypertension, palpitations, sweating, and headaches
Hyperparathyroidism
Multiple Endocrine Neoplasia Type 1
Neurofibromatosis Type 1
Medullary Thyroid Carcinoma
Radiation therapy is used alone or with surgery to treat tumors. It may include prostaglandin inhibitors for medullary thyroid cancer.
Pheochromocytomas should be evaluated and removed before other surgeries to ensure patient stability.
Thyroid hormone needed after total thyroidectomy in RET mutation carriers or medullary thyroid carcinoma.
Initiate 24-hour urine catecholamine collections and annual serum calcium/PTH tests to detect pheochromocytoma early.
Annual screening for hyperparathyroidism with calcium and PTH levels.
Vandetanib can benefit late-stage MTC patients with symptomatic disease ineligible for surgery due to its dual kinase inhibition.
Endocrinology, Metabolism
Organized routine screenings detect tumors early in thyroid, parathyroid, and adrenal glands.
Daily physical activity and exercise to improve the strength, endurance and overall wellness of the patients.
Diet changes improve primary hyperparathyroidism symptoms and complications with hydration balance.
Proper awareness about MEN2 should be provided and its related causes with management strategies.
Appointments with a physician and preventing recurrence of disorder is an ongoing life-long effort.
It increases utilization and mobilization of glycogen stores to promote gluconeogenesis.
Endocrinology, Metabolism
Calcitriol:
It is used in the management of hypocalcemia in patients with postsurgical hypoparathyroidism.
Endocrinology, Metabolism
Fludrocortisone acetate:
It provides a partial replacement therapy for primary and secondary adrenocortical insufficiency.
Endocrinology, Metabolism
It inhibits osteocalstic activity to bind with the ligand receptor of serum calcium.
Endocrinology, Metabolism
Thyroidectomy is indicated in prophylactic surgery for medullary thyroid carcinoma.
Parathyroid surgery is used as surgical intervention for primary hyperparathyroidism.
Adrenal Surgery involves surgical removal of pheochromocytoma.
Endocrinology, Metabolism
In the initial treatment phase, evaluation of MEN2 tumor and imaging test to confirm diagnosis.
Pharmacologic therapy is effective in the treatment phase as it includes use of tyrosine kinase inhibitors, diuretics, calcium metabolism modifiers, thyroid Hormones, and corticosteroids.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention therapies.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
MEN2 is rare genetic disorder causes tumors in multiple endocrine glands. RET proto-oncogene germline mutations cause autosomal dominant MEN2 transmission.
The two types of MEN2 syndromes are:
MEN2A
MEN2B
Medullary Thyroid Carcinoma is a cancer of the thyroid gland that originates from C-cells.
Primary hyperparathyroidism overactivity of the parathyroid glands to increase levels of parathyroid hormone and high calcium levels.
Pheochromocytoma is a tumor of the adrenal glands that can cause high blood pressure and other symptoms due to excessive production of catecholamines.
Genotype-phenotype correlation in inherited MTC can personalize risk management for childhood carriers.
MEN2A and MEN2B are inherited in an autosomal dominant pattern with only one altered gene copy needed.
The overall frequency of MEN2 in the United States is 1 case per 50000 population.
50% MEN2A patients with RET gene mutations develop disease up to 50 years old.
Genetic background and healthcare access influence reported incidences. MEN2B may be more prevalent in specific populations.
Family history of MEN2 increases risk due to autosomal dominant inheritance.
GDNF critical in enteric nervous system and renal organogenesis pathways for normal function.
Intracellular tyrosine kinase core triggers signalling events through second messenger molecules downstream.
MEN2A patients have bilateral, multicentric MTC with C-cell hyperplasia, while sporadic MTC is unilateral.
Pheochromocytomas in MEN2A patients are benign and diagnosed with medullary thyroid cancer.
The causes for MEN2 are:
Genetic mutations
Inheritance Pattern
Autosomal Dominant
Oncogenesis
Tumor Formation
Genetic Testing
Prognosis in MTC patients is influenced by disease stage at diagnosis. MEN2B-associated MTC is most aggressive, while FMTC without other malignancies is least aggressive.
Prompt detection of MTC saves lives; monitoring for pheochromocytomas prevents hypertension episodes.
Adrenal medulla tumors found in 50% of MEN2 patients in their late 30s.
Surgery should be considered for confirmed adrenal mass enlargement and bilateral masses require specific procedure.
Collect details including family and medical history to understand clinical history of patient.
Neck Examination
Skeletal Examination
Oral Examination
Skin Examination
Symptoms for MEN 2A&2B are:
Palpable lumps, hoarseness, difficulty in swallowing, kidney stones, bone pain, abdominal discomfort, hypertension, palpitations, sweating, and headaches
Hyperparathyroidism
Multiple Endocrine Neoplasia Type 1
Neurofibromatosis Type 1
Medullary Thyroid Carcinoma
Radiation therapy is used alone or with surgery to treat tumors. It may include prostaglandin inhibitors for medullary thyroid cancer.
Pheochromocytomas should be evaluated and removed before other surgeries to ensure patient stability.
Thyroid hormone needed after total thyroidectomy in RET mutation carriers or medullary thyroid carcinoma.
Initiate 24-hour urine catecholamine collections and annual serum calcium/PTH tests to detect pheochromocytoma early.
Annual screening for hyperparathyroidism with calcium and PTH levels.
Vandetanib can benefit late-stage MTC patients with symptomatic disease ineligible for surgery due to its dual kinase inhibition.
Endocrinology, Metabolism
Organized routine screenings detect tumors early in thyroid, parathyroid, and adrenal glands.
Daily physical activity and exercise to improve the strength, endurance and overall wellness of the patients.
Diet changes improve primary hyperparathyroidism symptoms and complications with hydration balance.
Proper awareness about MEN2 should be provided and its related causes with management strategies.
Appointments with a physician and preventing recurrence of disorder is an ongoing life-long effort.
It increases utilization and mobilization of glycogen stores to promote gluconeogenesis.
Endocrinology, Metabolism
Calcitriol:
It is used in the management of hypocalcemia in patients with postsurgical hypoparathyroidism.
Endocrinology, Metabolism
Fludrocortisone acetate:
It provides a partial replacement therapy for primary and secondary adrenocortical insufficiency.
Endocrinology, Metabolism
It inhibits osteocalstic activity to bind with the ligand receptor of serum calcium.
Endocrinology, Metabolism
Thyroidectomy is indicated in prophylactic surgery for medullary thyroid carcinoma.
Parathyroid surgery is used as surgical intervention for primary hyperparathyroidism.
Adrenal Surgery involves surgical removal of pheochromocytoma.
Endocrinology, Metabolism
In the initial treatment phase, evaluation of MEN2 tumor and imaging test to confirm diagnosis.
Pharmacologic therapy is effective in the treatment phase as it includes use of tyrosine kinase inhibitors, diuretics, calcium metabolism modifiers, thyroid Hormones, and corticosteroids.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention therapies.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
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