Intracranial calcification and brachydactyly mimicking Albright's hereditary osteodystrophy in an adult patient with lingual thyroid and prolactinoma-like lesion.
Hyperprolactinemia and prolactinoma in patients with long-term primary hypothyroidism have been recognized for decades. We report a case of 57-year-old female patient with lingual thyroid and cretinism who had a high serum prolactin level (greater than 200 ng/ml) and a pituitary tumor with suprasellar and parasellar extension. The tumor regressed to a size undetectable by CT scan after 2 years of thyroid hormone replacement therapy, but complete normalization of the hyperprolactinemia required additional bromocriptine therapy. This patient showed generalized short metacarpal and phalangeal bones, calcification of the basal ganglia and dentate nuclei bilaterally, and subcutaneous calcification at both gluteal regions, while serum calcium, phosphorus and c-PTH levels were all normal. Thus in addition to short stature, brachydactyly, a round face, and obesity, which are related to hypothyroidism, she also presented features uniquely mimicking the Albright's hereditary osteodystrophy seen in patients with pseudohypoparathyroidism and pseudopseudohypoparathyroidism. Since she had no family history of pseudohypoparathyroidism and had a normal level of Gs alpha protein on the membrane of the red blood cells, there is no evidence of pseudopseudohypoparathyroidism. The cause of the ectopic calcification remains unknown.
Department of Clinical Pathology, National Taiwan University Hospital, Taipei, R.O.C.
K S
KS
Tsai
D J
DJ
Wu
S M
SM
Lai
S T
ST
Gong
D001927:Brain Diseases / Q000175:diagnosis*; D002114:Calcinosis / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D005385:Fingers / Q000002:abnormalities*; D006801:Humans; D008875:Middle Aged; D010911:Pituitary Neoplasms / Q000175:diagnosis*; D015175:Prolactinoma / Q000175:diagnosis*; D011547:Pseudohypoparathyroidism / Q000175:diagnosis*; D013961:Thyroid Gland / Q000002:abnormalities*