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imaging is not helpful in these individuals, and bilateral neck exploration

should be performed in these patients. The genetic analysis in the index patient

showed frame-shift mutation in MEN1 gene, the patient underwent bilateral

neck exploration and 3.5 glands were removed, and the histopathology of the

excised gland showed parathyroid adenoma in one gland and the rest showed

hyperplasia. The biochemical workup for other endocrine organ involvement

was noncontributory. However, the patient should be followed up annually or

with any new onset of symptom, as the multiple endocrine organ involvement

may evolve over time.

8. How to follow up a patient of MEN1 who has a single endocrine gland involve-
   ment at presentation?

The majority of endocrine tumors associated with MEN1 are benign, while

nonfunctioning pancreatic neuroendocrine tumors are usually malignant

and contribute to increased morbidity and mortality in these patients.

Therefore, periodic imaging is recommended to identify the disease at the

earliest possible in those individuals who have MEN1 mutation. The bio-

chemical tests are recommended to identify functional tumors and imaging

for nonfunctional tumor/s. The time schedule for recommended work up

and imaging modalities for patients with MEN1 are summarized in the table

given below.

Endocrine tumor

Age to screen

(years) Biochemistry (annually) Imaging

Parathyroid 8 Calcium and iPTH –

Gastrinoma 20 Gastrin –

Insulinoma 5 Fasting glucose and

C-peptide

Nonfunctional

pancreatic NET

<10 Chromogranin A MRI, CT, or

endoscopic

ultrasound

(annually)

Anterior pituitary 5 Prolactin, IGF1 MRI (3 yearly)

Adrenal <10 Unless symptomatic or

tumor size >1 cm

CT (annually)

Thymic and

bronchial carcinoid

15 – CT or MRI

(annually)

9. What are the characteristics of pituitary tumor associated with MEN1 syndrome?

Pituitary tumors are present in 30–40 % of patients with MEN1 syndrome. On the

contrary, 3 % of patients with pituitary tumor have MEN1 syndrome. These tumors

are more common in women and are usually macroadenomas (85 %). Further,

they have aggressive behavior and are usually resistant to medical treatment. Most

common pituitary tumor in MEN1 syndrome is prolactinoma (20 %), followed

by somatotropinoma (10 %), and rarely corticotropinoma and nonfunctioning

11 Multiple Endocrine Neoplasia