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| Discussion |
Case Report: A 25-year-old married woman with a three history of pituitary macroadenoma treated with carbegoline comes with complaints of dull aching pain in the abdomen & fainting episodes since two years. Physical examination was unremarkable
Laboratory findings
Hb- 14.02 gm %, : Random blood sugar 25 mg%,
T3- 95 ng %, T4 -5.00 mcg % , TSH -2.75 µIu/ml, B cortisol 9.30 mcg % ,
TSH- 4.94 μIU/ml, Prolactin -3.60 ng /ml.
Radiological findings: Plain films of the lumbar spine showed a lft renal calculus. There was evidence of early osteomalacia (Figs 1,2)
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Fig. 1 |
Fig. 2 |
Ultrasonography of the abdomen – (Figs. 3,4,5) revealed:
- Atrophic pancreas with pancreatic calcification
-Bilatral tiny renal calculi with Left 8mm renal calculus near upper pole.(fig4&5)
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Fig. 3 |
Fig. 4 |
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Fig. 5 |
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In view of the possibility of hyperparathyroidism, sonography of the neck was performed. (Figs. 6,7,8,9).
This showed:
-Bilateral bulky inferior parathyroids suggestive of parathyroid hyperplasia with possible a adenoma of right inferior parathyroid.
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Fig. 6 |
Fig. 7 |
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Fig. 8 |
Fig. 9 |
15 mci 99 m Tc Sestaemibi scan confirmed an adenomatous right inferior parathyroid.
In view of biochemical findings consistent with insulinoma , a contrast enhanced CT abdomen (arterial phase) was performed to search for an insulinoma. However it could not confirm endocrinological findings . But incidentally b/l bulky adrenals suggestive of mild adrenal hyperplasia was detected. (Fig-10, 11- plain CT; Fig12-arterial phase; and Fig13)
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Fig. 10 |
Fig. 11 |
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Fig. 12 |
Fig. 13 |
In view of the low random blood sugar, further biochemical evaluation done. The values were as follows:
C Peptide 1.72 ng /ml Insulin 6.67 µIu/ml.
FT4 0.90 ng/dl Fasting Gastrin 29.00 pg/ml
Serum Ca-10.6, P-1.4, PTH - 573pg.ml
Further evaluation with endoscopic ultrasound was performed (Fig 14). This revealed a small 2.0 x 1.1 cm sized hypoechoic lesion in the head of the pancreas adjoining duodenum which would most likely represent insulinoma.
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Fig. 14 |
MR Brain (Fig 15,16) This revealed a residual pituitary macroadenama
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Fig. 15 |
Fig. 16 |
DIAGNOSIS:
In view of these biochemical and radiological findings, a diagnosis of Multiple Endocrine NeoplasiaI syndrome.( Prolactinoma +Parathyroid adenoma +Insulinoma + mild b/l adrenal hyperplasia) was made.
TREATMENT
1. Prolactinoma Medical treatment with cabergoline ( .5 mg) 2 x /week
2. Primary hyperparathyriodsm : Subtotal parathyriodectory 3 ½ gland removal, leaving behind 50 mg viable parathyroid tissue,additionally trancervical thymectomy. Inj Vit D 6 lac units im to be given 12 hours before surgery
3. Insulinoma – Enuclearation of the adenoma
First reported in 1963 by Wermer, multiple endocrine neoplasia (MEN) syndromes consist of rare, autosomal dominant mutations in genes that regulate cell growth.
Current classification recognizes type 1 MEN and type 2 MEN, with subcategories type 2A MEN (Sipple syndrome) and type 2B MEN. The menin protein produced from the MENIN gene is a tumor suppressor on chromosome11. Loss of this protein allows tumors to arise. Alternatively, Ret protein produced from the RET gene, a proto-oncogene, can be constitutively activated, causing abnormal cell proliferation.
Type 1 MEN is defined by hyperfunctioning tumors in all 4 parathyroid glands, pancreatic islets (eg, gastrinoma, insulinoma, glucagonoma, vasoactive intestinal peptide tumor [VIPoma], pancreatic polypeptide–producing tumor [PPoma]), and the anterior pituitary (eg, prolactinoma, somatotropinoma, corticotropinoma, nonfunctioning tumors). Other associated tumors include lipomas, angiofibromas, or those located in the adrenal gland cortex (rarely, in the adrenal medulla).
Type 2A MEN is defined by medullary thyroid carcinoma (MTC), pheochromocytoma (about 50% of cases), and hyperparathyroidism caused by parathyroid gland hyperplasia (about 20% of cases).
Familial MTC is also recognized. Familial MTC is hereditary MTC without other associated endocrinopathies, although adrenomedullary hyperplasia secondary to a germline RET mutation may still be present but undiagnosed.
Type 2B MEN is defined by medullary thyroid cancer and pheochromocytoma. Associated abnormalities include mucosal neuromas, medullated corneal nerve fibers, and marfanoid habitus.
Imaging
Pitutary- MR of sella with contrast study along with biochemical evaluation is the appropriate imaging modality.
Parathyroid- USG is more sensitive than CT for the detection of enlarged parathyroids & also helpful in planning surgical removal. Sestamibi scan detcts adenomatous parathyroid.
Insulinoma-Contrast enhanced CT in the early arterial phase can detect insulinomas due to their characteristic early brilliant enhancemet pattern .
Insulinomas & gastrinomas are also detectable on endoscopic ultrasound.