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Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor: a rare case of McCune-Albright syndrome. 2011

Kenshi Sakayama, and Yoshifumi Sugawara, and Teruki Kidani, and Taketsugu Fujibuchi, and Katsumi Kito, and Nozomu Tanji, and Atsushi Nakamura
Department of Bone and Joint Surgery, Ehime University Graduate School of Medicine, Shitsukawa, Toon, Ehime 791-0204, Japan. kenshi@m.ehime-u.ac.jp

We report a rare case of polyostotic fibrous dysplasia on endocrine hyperfunction with elevated human growth hormone and normal serum level of prolactin. There were some differential points of gender, gigantism, endocrine function, and GNAS gene from McCune-Albright syndrome. Malignant transformation was suspected in the pelvic tumor from imaging because rapid growth of the tumor by imaging was observed; however, no malignant change occurred in this case.

UI MeSH Term Description Entries
D008297 Male Males
D010386 Pelvic Neoplasms Tumors or cancer of the pelvic region. Cancer of Pelvis,Pelvic Cancer,Cancer of the Pelvis,Neoplasms of Pelvis,Pelvis Cancer,Pelvis Neoplasms,Cancer, Pelvic,Cancer, Pelvis,Cancers, Pelvic,Cancers, Pelvis,Neoplasm, Pelvic,Neoplasm, Pelvis,Neoplasms, Pelvic,Neoplasms, Pelvis,Pelvic Cancers,Pelvic Neoplasm,Pelvis Cancers,Pelvis Neoplasm
D010900 Pituitary Diseases Disorders involving either the ADENOHYPOPHYSIS or the NEUROHYPOPHYSIS. These diseases usually manifest as hypersecretion or hyposecretion of PITUITARY HORMONES. Neoplastic pituitary masses can also cause compression of the OPTIC CHIASM and other adjacent structures. Adenohypophyseal Diseases,Hypophyseal Disorders,Neurohypophyseal Diseases,Anterior Pituitary Diseases,Pituitary Disorders,Pituitary Gland Diseases,Posterior Pituitary Diseases,Adenohypophyseal Disease,Anterior Pituitary Disease,Disease, Adenohypophyseal,Disease, Anterior Pituitary,Disease, Neurohypophyseal,Disease, Pituitary,Disease, Pituitary Gland,Disease, Posterior Pituitary,Diseases, Adenohypophyseal,Diseases, Anterior Pituitary,Diseases, Neurohypophyseal,Diseases, Pituitary,Diseases, Pituitary Gland,Diseases, Posterior Pituitary,Disorder, Hypophyseal,Disorder, Pituitary,Disorders, Hypophyseal,Disorders, Pituitary,Hypophyseal Disorder,Neurohypophyseal Disease,Pituitary Disease,Pituitary Disease, Anterior,Pituitary Disease, Posterior,Pituitary Diseases, Anterior,Pituitary Diseases, Posterior,Pituitary Disorder,Pituitary Gland Disease,Posterior Pituitary Disease
D011388 Prolactin A lactogenic hormone secreted by the adenohypophysis (PITUITARY GLAND, ANTERIOR). It is a polypeptide of approximately 23 kD. Besides its major action on lactation, in some species prolactin exerts effects on reproduction, maternal behavior, fat metabolism, immunomodulation and osmoregulation. Prolactin receptors are present in the mammary gland, hypothalamus, liver, ovary, testis, and prostate. Lactogenic Hormone, Pituitary,Mammotropic Hormone, Pituitary,Mammotropin,PRL (Prolactin),Hormone, Pituitary Lactogenic,Hormone, Pituitary Mammotropic,Pituitary Lactogenic Hormone,Pituitary Mammotropic Hormone
D001849 Bone Diseases, Endocrine Diseases of the bones related to hyperfunction or hypofunction of the endocrine glands. Bone Disease, Endocrine,Disease, Endocrine Bone,Diseases, Endocrine Bone,Endocrine Bone Disease,Endocrine Bone Diseases
D001859 Bone Neoplasms Tumors or cancer located in bone tissue or specific BONES. Bone Cancer,Cancer of Bone,Cancer of the Bone,Neoplasms, Bone,Bone Neoplasm,Neoplasm, Bone
D002864 Chromogranins A group of acidic proteins that are major components of SECRETORY GRANULES in the endocrine and neuroendocrine cells. They play important roles in the aggregation, packaging, sorting, and processing of secretory protein prior to secretion. They are cleaved to release biologically active peptides. There are various types of granins, usually classified by their sources. Chromogranin,Granin,Secretogranin,Secretogranins,Granins
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005359 Fibrous Dysplasia, Polyostotic FIBROUS DYSPLASIA OF BONE affecting several bones. When melanotic pigmentation (CAFE-AU-LAIT SPOTS) and multiple endocrine hyperfunction are additionally associated it is referred to as Albright syndrome. Albright's Syndrome,McCune-Albright Syndrome,Albright Syndrome,Albright's Disease,Albright's Disease of Bone,Albright's Syndrome with Precocious Puberty,Albright-Mccune-Sternberg Syndrome,Albright-Sternberg Syndrome,Fibrous Dysplasia with Pigmentary Skin Changes and Precocious Puberty,Polyostotic Fibrous Dysplasia,Dysplasia, Polyostotic Fibrous,Dysplasias, Polyostotic Fibrous,Fibrous Dysplasias, Polyostotic,McCune Albright Syndrome,Polyostotic Fibrous Dysplasias,Syndrome, Albright,Syndrome, Albright's,Syndrome, McCune-Albright
D005877 Gigantism The condition of accelerated and excessive GROWTH in children or adolescents who are exposed to excess HUMAN GROWTH HORMONE before the closure of EPIPHYSES. It is usually caused by somatotroph hyperplasia or a GROWTH HORMONE-SECRETING PITUITARY ADENOMA. These patients are of abnormally tall stature, more than 3 standard deviations above normal mean height for age. Pituitary Gigantism,Gigantism, Pituitary

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