PMC4194010_01
a Computed tomography of abdomen shows right (arrow) and left (open arrow) adrenal tumors. b
131I-MIBG scan reveals abnormal uptakes indicating bilateral pheochromocytomas
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Fig1
Bilateral adrenal tumors were identified by abdominal CT scan (maximum diameter of 4 cm for right mass and 9 cm for left mass) (Fig. 1a). Pheochromocytomas were diagnosed by 131I-metaiodobenzylguanidine (MIBG) scan (Fig. 1b), along with elevated urinary catecholamine and metabolite concentrations (Table 1), and she was, therefore, transferred to the Fujita Health University Hospital for surgical management.
a Microscopy of left adrenal tumor shows pheochromocytoma (hematoxylin & eosin, x100). B Microscopy of thyroid gland shows C-cell hyperplasia (hematoxylin &eosin, x100). c Microscopy of thyroid gland shows C-cell hyperplasia (calcitonin immunostaining, x100)
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Fig2
Bilateral adrenal tumors were surgically resected, and the diagnosis of pheochromocytomas was confirmed by histological examination (Fig. 2a). Histological examination demonstrated that multiple nodal lesions were scattered, indicating the presence of C-cell hyperplasia without any evidence of MTC (Fig. 2b, c).
a Macroscopy of the resected parathyroid glands shows slightly enlarged right upper and lower gland. (a
right upper gland, b
left upper gland, c
right lower gland, d
left lower gland) b Microscopy of right upper parathyroid gland shows no hyperplasic change (hematoxylin & eosin, x100)
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Fig3
Regarding the four resected parathyroid glands (Fig. 3a), two right glands were slightly enlarged (231 mg for superior and 118 mg for inferior glands) compared with the two left glands (48 mg for superior and 15 mg for inferior glands). However, no specific histological change was observed even in the enlarged glands (Fig. 3b).
PMC4194010_02
Pedigree of the family. The arrow indicates the proband. The II-3, who was affected by adrenal disease and died from cerebral vascular disease, might possibly carry the S891A mutation
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Fig4
RET mutation screening, and indeed, the S891A mutation was identified (Fig. 4). Routine chemical screening of the blood, including basal serum calcitonin levels, was all normal.