Bilateral pheochromocytoma associated with paraganglioma and papillary thyroid carcinoma: report of an unusual case.
A 42-year old woman presented with headache, palpitation and facial flushing. Ultrasonograms and computed tomograms revealed tumors in both of the adrenal glands, anterior aspect of the inferior vena cava, and the right lobe of the thyroid gland. Fine needle aspiration biopsy of the thyroid nodule revealed papillary thyroid carcinoma. Serum calcitonin, CEA, intact PTH and calcium levels were within normal limits. Markedly elevated levels of urinary normetanephrine and vanillylmandelic acid, and the result of 131I-metaiodobenzylguanidine (131I-MIBG) scintigraphy indicated that both adrenal masses were pheochromocytoma. Bilateral adrenalectomy, paracaval mass removal and total thyroidectomy together with central lymph node dissection were performed. The final pathological diagnosis was bilateral adrenal pheochromocytoma, paraganglioma, papillary thyroid carcinoma and either parathyroid adenoma or hyperplasia. Analysis of the RET proto-oncogene mutation, von Hippel Lindau mutation, succinate dehydrogenase subunit B mutation, and succinate dehydrogenase subunit D mutation yielded negative results. The relationship of these lesions could not be determined. This is the first report of a combination of bilateral pheochromocytoma, abdominal paraganglioma, papillary thyroid carcinoma and either parathyroid adenoma or hyperplasia without hyperparathyroidism.
Yang
Jeong Hoon
JH
Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Korea.
Bae
Sung Jin
SJ
Park
Sanghui
S
Park
Hyun-Kyung
HK
Jung
Hye Seung
HS
Chung
Jae Hoon
JH
Min
Yong-Ki
YK
Lee
Myung-Shik
MS
Kim
Kwang-Won
KW
Lee
Moon-Kyu
MK
D019797:3-Iodobenzylguanidine; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D001707:Biopsy, Needle; D002291:Carcinoma, Papillary / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D006801:Humans; D008197:Lymph Node Excision; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis*; D010235:Paraganglioma / Q000175:diagnosis*; D010673:Pheochromocytoma / Q000175:diagnosis* / Q000601:surgery; D000090063:Proto-Oncogene Mas; D019275:Radiopharmaceuticals; D013964:Thyroid Neoplasms / Q000175:diagnosis* / Q000601:surgery; D013965:Thyroidectomy; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography
C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D019275:Radiopharmaceuticals; D019797:3-Iodobenzylguanidine