Master MEN 2B (Multiple Endocrine Neoplasia) with Picmonic for Medicine

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MEN 2B (Multiple Endocrine Neoplasia)

2 Men in (B) black
Picmonic
Multiple Endocrine Neoplasm (MEN) 2B is a group of neoplasms occurring together in characteristic organs due to mutation in the RET oncogene. The neoplasms typically originate from the medullary cells of the thyroid gland, mucosal ganglionic cells, and chromaffin cells of the adrenal gland which results in pheochromocytoma. Additionally, patients have a characteristic marfanoid appearance.
6 KEY FACTS
PATHOPHYSIOLOGY
RET Gene
Roulette

RET is a tyrosine kinase receptor on chromosome 10. It is an oncogene that develops a gain of function mutation in the MEN2A and MEN2B syndromes.

Autosomal Dominant
Domino

The disease is passed in an autosomal dominant fashion. Therefore, there is often a significant family history in patients that present with this disease.

SIGNS AND SYMPTOMS
Medullary Thyroid Carcinoma
Medal Thyroid

Medullary carcinomas of the thyroid are neuroendocrine tumors derived from the C cells, or parafollicular cells of the thyroid and are often seen in MEN 2A and MEN2B syndromes. Cases associated with the MEN syndrome occur in younger persons while sporadic cases develop much later in life. These tumors secrete calcitonin, and altered calcitonin polypeptides can deposit as amyloid, which has a characteristic red stain on Congo dye.

Pheochromocytoma
Phiat-chrome

This is a neuroendocrine tumor of the chromaffin cells which secrete catecholamines. This tumor is of neural crest cell origin and is often found in the adrenal medulla. It often presents with the classic triad of intermittent palpitations, headache and diaphoresis.

Marfanoid Body Habitus
Marfan-the-martian

Patients with marfanoid body habitus display long limbs, arachnodactyly, and hyperlaxity. This type of body habitus is seen in MEN2B, homocystinuria, and Marfan syndrome.

Oral Ganglioneuromatosis
Neurons with Mouths

This is a type of mucosal neuroma characteristically found in the GI tract. It is a benign growth of ganglioma cells.

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