We experienced one case of Beckwith syndrome. This one day aged male neonate manifested macroglossia, umbilical hernia, visceromegaly, hypoglycemia, gigantism and polycythemia. A brief review of related literature is also presented.
Gigantism ; Hernia, Umbilical ; Humans ; Hypoglycemia ; Infant, Newborn ; Macroglossia ; Male ; Polycythemia

D-penicillamine, a chelating agent of copper, is the drug of choice for the treatment of Wilson's disease. Breast enlargement is a rare complication arising from its use, and we report a case of breast gigantism which developed after it had been used for ten months to treat this condition. Mammography demonstrated bilaterally enlarged dense breasts; ultrasonography, similarly, demonstrated enlargement, revealing the presence of a mass, shown at biopsy to be benign, in the left one.
Biopsy ; Breast* ; Copper ; Gigantism* ; Hepatolenticular Degeneration ; Mammography ; Penicillamine* ; Ultrasonography

3 cases of pituitary adenomas, two hormone-secreting adenomas, and one non-secreting chromophobe adenoma, were treated surgically by the sublabial transsphenoidal approach with use of surgical microscope and radiofluroscopic image intensification at the Dept. of Neurosurgery, Yonsei University Medical College. The 2 cases of hormone secreting adenomas were an eosinophilic microadenoma manifestating gigantism and a prolaction secreting adenoma with amenorrhea and galactorrhea. The simplicity of this technique, criteria for the treatment of the hormone secreting and non-secreting adenomas and their biologic behaviors were described and discussed with review of literatures.
Adenoma ; Adenoma, Chromophobe ; Amenorrhea ; Eosinophils ; Female ; Galactorrhea ; Gigantism ; Neurosurgery ; Pituitary Neoplasms ; Pregnancy

3 cases of pituitary adenomas, two hormone-secreting adenomas, and one non-secreting chromophobe adenoma, were treated surgically by the sublabial transsphenoidal approach with use of surgical microscope and radiofluroscopic image intensification at the Dept. of Neurosurgery, Yonsei University Medical College. The 2 cases of hormone secreting adenomas were an eosinophilic microadenoma manifestating gigantism and a prolaction secreting adenoma with amenorrhea and galactorrhea. The simplicity of this technique, criteria for the treatment of the hormone secreting and non-secreting adenomas and their biologic behaviors were described and discussed with review of literatures.
Adenoma ; Adenoma, Chromophobe ; Amenorrhea ; Eosinophils ; Female ; Galactorrhea ; Gigantism ; Neurosurgery ; Pituitary Neoplasms ; Pregnancy

Adolescent ; Female ; Gigantism ; diagnosis ; surgery ; Humans ; Limb Salvage ; methods ; Lipomatosis ; diagnosis ; surgery ; Upper Extremity ; pathology ; surgery

The Beckwith-Wiedemann syndrome, which included congenital anomalies such as macroglossia, exomphalos, postnatal somatic gigantism, have a substantially increased risk for the development of tumor. We report a case of testicular yolk sac tumor associated with Beckwith-Wiedemann syndrome, a previously unreported association. Pathologic examination showed Schiller-Duval body with evidence of testicular yolk sac tumor. This finding appears to represent a previously unreported association between Beckwith-Wiedemann syndrome and testicular yolk sac tumor.
Beckwith-Wiedemann Syndrome* ; Endodermal Sinus Tumor* ; Gigantism ; Hernia, Umbilical ; Macroglossia ; Testis ; Yolk Sac*

Klinefelter syndrome is a congenital disease that is associated with the existence of an extra X chromosome, and is one of the most common causes of male primary hypogonadism. In addition to hypogonadism-associated manifestations such as testicular atrophy and infertility, it is also well known that this syndrome may be associated with other systemic comorbidities. In this report, we describe a typical case of Klinefelter syndrome that was differentially diagnosed as a cause of gigantism. A 20-year-old male was admitted to evaluate the cause of tall stature. His height was 193.4 cm, and all screening tests for gigantism were negative. Physical examination revealed no clear evidence of secondary sexual characteristics, and the results of a hormonal assay were highly suspicious for primary hypogonadism. Based on these findings, we performed a chromosomal analysis and confirmed Klinefelter syndrome with a 47, XXY karyotype.
Atrophy ; Chromosome Disorders ; Comorbidity ; Gigantism ; Humans ; Hypogonadism ; Infertility ; Karyotype ; Klinefelter Syndrome ; Male ; Mass Screening ; Physical Examination ; X Chromosome ; Young Adult

gigantism and asymmetry of the limbs, plantar hyperplasia, hemangiomas, lipomas, lymphangiomas, varicosities, verrucous epidermal nevi, macrocephaly, cranial hyperostosis, and long bone overgrowth. We diagnosed Proteus syndrome in a male patient who visited our hospital with a chief complaint of limited mouth opening and report the case because we obtained a good healing outcome after treating the condition with a corrective osteotomy.]]>
Congenital Abnormalities ; Extremities ; Gigantism ; Hemangioma ; Humans ; Hyperostosis ; Hyperplasia ; Lipoma ; Lymphangioma ; Macrocephaly ; Male ; Mouth ; Nevus ; Osteotomy ; Proteus ; Proteus Syndrome

gigantism, hemihypertrophy, hypoglycemia, and the increased risk of solid tumor development from multiple cell lines. A macroglossia is a key component of the syndrome, and can lead to cosmetic, functional and psychologic disorder. This report shows a 5-year-old patient with Beckwith-Wiedemann syndrome, who had macroglossia and received reduction glossectomy.]]>
Abdominal Wall ; Beckwith-Wiedemann Syndrome ; Cell Line ; Child, Preschool ; Gigantism ; Glossectomy ; Humans ; Hypoglycemia ; Incidence ; Live Birth ; Macroglossia

Overgrowth and hypertrophy of one or more extremities is an infrequentIy encountered congenital or acguired anomaly, It is referred to in the literature by different authors as local gigantism, hypertrophy etc. The etiology remains unexplained and the condition is only seldom found to be familiar. Hypertrophy of left lower extremity associated with neurofibromatosis of the sural nerve in the calf, in a 8 years old school boy, is presented with a review of the literature. Chief complaints were enlargement of left lower leg and limping gait since 5 years old. There was no change in sensory and motor function of the involved leg. X-ray films showed enlargement of tibia and fibula and diffuse hypertrophied soft tissue shadow in the left leg. One case of local excision of hypertrophied sural nerve and subcutaneous fat tissues was presented.
Extremities ; Fibula ; Gait ; Gigantism ; Humans ; Hypertrophy ; Leg ; Lower Extremity ; Male ; Neurofibromatoses ; Subcutaneous Fat ; Sural Nerve ; Tibia ; X-Ray Film