Take you to understand the special types of multiple endocrine tumors (MEN-1)

Other tumors associated with MEN 1 include gastric carcinoids, neuroendocrine tumors of the lung and thymus, adrenal adenomas or carcinomas, lipomas and cutaneous hemangioma, thyroid adenomas, and breast cancer may also be related to MEN 1

Gastric carcinoid

Gastric carcinoid

Gastric carcinoid is an inert, small malignant tumor distributed throughout the gastrointestinal tract including the fundus of the stomach

Inert, small, malignant enterochromaffin cells distributed throughout the gastrointestinal tract including the fundus of the stomach , mainly due to chronic excessive secretion of gastrin, causing gastric acidemia and atrophic gastritis.

Thymic Carcinoid

Thymic Carcinoid

Thymus and lung neuroendocrine tumors appear in less than 8% of MEN 1 patients, thymic carcinoids account for less than 5% of anterior mediastinal tumors, and males predominate in patients with MEN 1, the ratio is 2:1

Thymic carcinoid usually presents as a large mass, often accompanied by local invasion and distant metastasis

Large lumps, often accompanied by local invasion and distant metastasis, are aggressive,

Many thymic neuroendocrine tumors are accidentally discovered after chest X-ray screening, or after symptoms of compression, displacement, or invasion of the mediastinum and chest CT or X-ray examination

Ga-DOTA-TATE

Thymic carcinoids are treated with surgical resection

Surgical resection for cisplatin or carboplatin combined with etoposide chemotherapy and radiotherapy

Bronchial carcinoid

Bronchial carcinoid

Bronchial carcinoids are essentially indolent tumors

Males and smokers have hemoptysis or recurrent pneumonia, which is small in size (<1cm), is multifocal, and is mostly located around the lungs

Adrenal gland disease

Adrenal gland disease

Adrenal diseases such as hyperplasia, adenoma, or cancer have a higher incidence in MEN 1 patients, and symptoms can be subdivided according to endocrine function

MRI or CT scan of the adrenal glands must be performed.

Aldosteronoma

Aldosteronoma causes Conn syndrome, which is characterized by hypertension and hypokalemia

Hypertension and hypokalemia with more than three kinds of antihypertensive aldosterone tumors are usually unilateral, about 1-2 cm in size

Cortisol-producing adenoma

Cortisol-producing adenomas can cause Cushing’s syndrome, which is often accompanied by weight gain, muscle wasting, fat deposits, buffalo back, osteoporosis, hyperglycemia/diabetes, hypertension, amenorrhea, and depression

Cushing’s syndrome is often accompanied by weight gain, muscle wasting, fat deposits, buffalo back, osteoporosis, hyperglycemia/diabetes, hypertension, amenorrhea, and depression

Dehydroepiandrosterone adenoma

   Dehydroepiandrosterone adenomas (DHEAs) subtypes are extremely rare in MEN 1

Masculinization, including darkening of the tone, hair growth, menstrual disorders, and acne.
Detection of plasma dehydroepiandrosterone levels

Pheochromocytoma

Pheochromocytoma

Fewer than 10 cases of pheochromocytoma have been reported in MEN 1 syndrome
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Patients may experience intermittent palpitations, sweating, and headaches
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By measuring norepinephrine and norepinephrine in plasma and/or 24-hour urine, and then performing imaging examinations, including CT or MRI of the adrenal glands
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Before surgery, the patient must be treated with alpha adrenergic receptor blockers, followed by beta blockers (if needed)
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Once they have orthostatic hypotension, they can undergo surgery
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A professional team composed of endocrinologists, surgeons and anesthesiologists is a necessary condition for good results
.

Patients with intermittent palpitations, sweating, and headaches must be treated with alpha adrenergic receptor blockers, followed by beta blockers (if needed)
.

Adrenal cortical carcinoma

Adrenal cortical carcinoma

Only about 19 cases of adrenocortical carcinoma are related to MEN 1
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Most patients present with Cushing's syndrome, and the prognosis is poor
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These tumors are generally larger than 11 cm.
Biopsy should not be performed due to the risk of tumor spread
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Open surgical resection is recommended to reduce the risk of capsule rupture and spread
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However, this proposal is controversial
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The median 5-year overall survival rate is 15-44%
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The older, the incomplete resection, the worse the prognosis
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In addition, functional tumors tend to have a worse prognosis
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Mitotane has been approved for use in metastatic disease where other treatments have failed
.

More than 11 cm, because of the risk of tumor dissemination, biopsy should not be performed.
The older the age, the incomplete resection, the worse the prognosis
.
Mitotane has been approved for metastatic disease where other treatments have failed

Original source:

Thompson R, Landry CS, Multiple endocrine neoplasia 1: a broad overview.
Ther Adv Chronic Dis 2021;12

Multiple endocrine neoplasia 1: a broad overview.


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