378
40 mEq/L (N <15), fasting plasma insulin 10.2 μIU/ml, C-peptide 3.19 ng/ml with cor-
responding plasma glucose 98 mg/dl, and HbA1c 5.2 % (N <5.7 %). One microgram
ACTH stimulation test showed a peak cortisol response 455 nmol/L (N >550 nmol/L).
Twenty-four-hour urinary metanephrine was 93 μg (N <350) and normetanephrine
161 μg (N <600). CEMRI sella showed a 5 × 4.6 × 3.2 cm sellar–suprasellar mass with
right parasellar extension. The CECT abdomen showed small enhancing focal lesion in
the head of the pancreas, circumferential wall thickening in D1 and D2 segment of the
duodenum, and bilateral adrenal gland enlargement. Endoscopic ultrasonography
showed two lesions in the pancreas (8 × 8 mm in the body and 6 × 4 mm in the tail of the
pancreas). DOTANOC–PET CT showed somatostatin receptor expressing lesions in the
sella turcica, thyroid gland, and posterior to the thyroid gland (parathyroid gland); how-
ever, focal non-avid hypodense lesions were identified in the pancreas and in the adrenal
gland. Upper gastrointestinal endoscopy showed multiple superficial ulcers in D1 and
D2 segment of the duodenum. Ultrasonography of the neck showed 1 × 0.9 cm size
heterogeneous lesion with central vascularity in antero-inferior aspect of left lobe of the
thyroid gland. Sestamibi parathyroid scan showed uptake in the right superior and the
left superior and inferior parathyroid glands. SPECT–CT fusion image showed increased
tracer uptake in the left superior parathyroid gland. The patient was initiated with
L-thyroxine and cabergoline. Patient underwent transsphenoidal surgery (TSS) and
resection of the pituitary tumor was carried out. Postoperatively, patient was continued
with L-thyroxine supplementation and hydrocortisone was added. Cabergoline was also
continued as there was large residual tumor postoperatively. After 3 months of pituitary
surgery, he underwent bilateral neck exploration, and three enlarged parathyroid glands
were identified (left superior and inferior parathyroid, and right inferior parathyroid
gland) and were excised. Simultaneously, open laparotomy was also performed; intra-
operative ultrasonography confirmed the lesions in the head and body of the pancreas,
and these were excised accordingly. Postoperatively 3 months after TSS, serum prolac-
tin was 23 ng/ml (on cabergoline) and GH was non-suppressible (nadir GH 1.3 ng/ml)
after glucose load, however, IGF 1 was normalized (226 ng/ml; N 101–267, age
matched). Repeat CEMRI sella revealed 1.5 × 2.8 × 2.8-cm sellar–suprasellar mass with
parasellar extension. After parathyroid surgery with one gland in situ, serum calcium
and phosphorus was normalized (9.3 mg/dl and 3.3 mg/dl, respectively), serum iPTH
was 46.6 pg/ml, and serum gastrin was undetectable (<10 pg/ml). Histopathology of the
sellar mass confirmed pituitary adenoma and showed diffuse positivity for GH and pro-
lactin on immunohistochemistry (IHC). Parathyroid gland histology showed parathy-
roid adenoma in all three resected glands. Pancreatic tumor histology was consistent
with neuroendocrine tumor. Genetic analysis for MEN1 gene demonstrated duplication
of C1546 gene in exon 10 and frame-shift mutation at P-Arg 516 consistent with MEN1
syndrome. The pedigree of the index patient is shown in the figure given below. The
patient was continued with cabergoline and received external beam radiotherapy (EBRT)
for residual pituitary lesion. However, serum GH still remained non-suppressible after
glucose load with elevated IGF1; therefore, he was initiated with octreotide LAR (20 mg
once a month). He did not have features of hungry bone syndrome postoperatively. He
was continued with proton pump inhibitor along with calcium carbonate and calcitriol
(Fig. 11.1).
11 Multiple Endocrine Neoplasia