[Gigantism and amenorrhea: eunuchoid gigantism].
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Adult↗
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Results for “Gigantism”
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The pathology of congenital localized gigantism.
Two cases of congenital localized gigantism are described. They illustrate a form of segmental limb disproportion, characterized pathologically by an excessive linear growth of the skeletal structures and by soft tissue lipomatosis. The gigantism was confined to bones of the right great toe and adjacent metatarsus in one case; it affected the long bones of a lower extremity in the second case. Case 2, followed for over 7 years, developed a fibrovascular hamartomatous neoplasm of rapid growth and some cytological atypia in infancy--but this was well controlled by conservative surgery. This type of gigantism of the limb appears to be compatible with a normal life expectancy, and it is conceptually useful to distinguish it from hemihypertrophy. The number of cases reported is insufficient to support a significant association of gigantism with malignant tumors or with other life-jeopardizing anomalies.
Child↗
A case of cerebral gigantism and hepatocarcinoma.
A 14-year-old boy, who had the physical and neurological characteristics of cerebral gigantism (Sotos syndrome), developed hepatocarcinoma. This tumor is rare in children and has never, to our knowledge, been recorded in a patient with cerebral gigantism. An autopsy was performed, the first we are aware of in a patient with cerebral gigantism without increased size in ventricles.
Abnormalities, Multiple↗
Prolactin-secreting pituitary adenoma in a man with gigantism: a case report.
A prolactin-secreting pituitary adenoma was removed trans-sphenoidally from a 37 years old man with gigantism (218 cm). Serum levels of prolactin (PRL) were elevated pre-operatively and decreased after administration of L-Dopa with no increase after TRH as is usually observed in PRL-secreting adenomas. Growth hormone (GH) and somatomedin serum levels were normal with no modification of GH after insulin hypoglycemia, oral glucose loading or L-Dopa. Morphological examination of the tumour demonstrated the presence of lactotrophs by light and electron microscopy and by immunofluorescense staining. No somatotrophs were found. In this unique case, the relationship between a PRL-secreting adenoma and gigantism is discussed.
Adenoma↗
Cerebral gigantism (Sotos' syndrome) and cataracts.
A five-year-old girl with cerebral gigantism (Sotos' syndrome) and cataracts is described. Sotos' syndrome, characterized by generalized gigantism with normal endocrine studies has rarely been reported with ocular abnormalities and never with cataracts. It is important to study any child with cataracts for systemic disease.
Age Determination by Skeleton↗
[Cerebral gigantism. Review of the literature apropos of one case].
The authors report a case of cerebral gigantism and review 83 cases found in the world literature. The diagnosis depends on the association of several factors, none of which alone are specific but which, on the whole, form a fairly characteristic picture. Large size at birth, leading only rarely to gigantism at adult age. Facial dysmorphia of acromegaloid type. Ecephalopathy with mental deficiency without any neuroradiological abnormality. Finally, endocrine investigations and somatotropic function were normal. No etiology has been found up to now. The most satisfactory theory is that of a hypothalamopituitary disturbance. Treatment may simple avoid excess height at adult age and is based on sex hormone therapy to accelerate bony maturation.
Acromegaly↗
[Cerebral gigantism (Sotos-syndrome) (author's transl)].
Case report of a nine month old girl with Cerebral Gigantism. It is a gigantism syndrome characterized by advanced bone age, a typical craniofacial dysmorphia and a not progredient mental retardation. Etiology and pathogenesis of this syndrome are unknown.
Abnormalities, Multiple↗
Syndrome of fetal gigantism, renal hamartomas, and nephroblastomatosis with Wilms' tumor.
A new case of the fetal gigantism-renal hamartomas-nephroblastomatosis syndrome is described, in which a Wilms' tumor occurred. It is considered that this observation provides strong evidence for the interrelationship between renal dysplasia and renal neoplasia.
Facial Expression↗
Cerebral gigantism and primary hypothyroidism: pleiotropy or incidental concurrence.
An 8.5-month-old baby girl had cerebral gigantism and primary hypothyroidism with generalized large muscles (the Kocher-Debré-Semelaigne syndrome). The significance of this association remains to be determined.
Cephalometry↗
Nervous lesions in a case of local gigantism.
A case of local gigantism of the thumb was studied by light and electron microscopy. In the lesion, proliferation of fibroadipose tissue and typical alterations of the nerves were found. The alterations in the nerve were isolated hypertrophy of the perineural cells. The relation of the changes to other peripheral nerve tumours especially in neurofibromatosis, is discussed.
Adult↗
Dominant inheritance of cerebral gigantism.
Cerebral gigantism is a syndrome consisting of characteristic dysmorphic features, accelerated growth in early childhood, and variable degrees of mental retardation. Its etiology and pathogenesis have not been defined. Three families are presented with multiple affected members. The vertical transmission of the trait and equal expression in both sexes in these families indicates a genetic etiology with a dominant pattern of inheritance, probably autosomal. As in previously reported cases, extensive endocrine evaluation failed to define the pathogenesis of the accelerated growth present in this disorder.
Adolescent↗
Familial occurrence of cerebral gigantism, Sotos' syndrome.
Since the original description of cerebral gigantism, about 85 cases have been reported. Four papers comment on familial occurrence but never in parents and their children. This paper describes the syndrome in a mother and her child, which, together with facts pointing towards prenatal etiology, such as excessive birthweight, striking mutual resemblance and abnormal dermatoglyphics, points to a genetic defect. Previous endocrine studies are enlarged by the findings of normal serum somatomedin and serum prolactin.
Abnormalities, Multiple↗
Gigantism associated with a pituitary tumour secreting growth hormone and prolactin and cured by transsphenoidal hypophysectomy.
An 18-year old male is reported who presented with a history of a growtn spurt over the year preceding his admission. His height was above the 97th percentile, and he had incompletely developed secondary sexual characters. Pituitary evaluation demonstrated a moderately elevated level of growth hormone (hGH) not suppressible by a glucose load and not stimulable by TRH or by L-DOPA. Serum prolactin (PRL) concentration was also increased while gonadotrophin, thyroid and adrenal function were all subnormal. There was clear radiological evidence of a large pituitary tumour with suprasellar extension and transsphenoidal total hypophysectomy was performed. A mixed chromophobe and acidophilic adenoma was found and both growth hormone and prolactin were demonstrable in different cells of the tumour by the immunoperoxidase technique. Post-operatively the patient has hypopituitarism and levels of growth hormone and prolactin have remained low or undetectable after 6 months. Thus early diagnosis and surgical treatment of gigantism of this mixed hGH-PRL secreting pituitary tumour was associated with a cure, which contrasts with the unfavourable outcome of many of the patients previously reported.
Adenoma, Acidophil↗
[Cerebral gigantism in twins].
Two probably monozygotic twins with cerebral gigantism are reported. They showed three typical features: pneumo-encephalography demonstrated a fifth anterior ventricular dilatation; in both patients dermatoglyphic findings showed a thenar exit of the Aline and a vertical palmar alignement; Growth hormone and sulfatation factor blood concentration were low but within normal limits.
Brain Diseases↗
[Reflexions and thoughts about one case of acromegalic gigantism (author's transl)].
Having observed a 22 year old man with a case of acromegalic-gigantism (2,30 m.) whose growth still continued after the ablation of a hypophyseal micro-adenoma, by trans-sphenoidale tract, the authors after making various neuroendocrine comments study from a psychological point of view the myth of the giant which appears to be linked to the myth of origins, from a phylogentical point of view (for example Atlantis) as well as from the ontogenetical point of view (identification to the father). The example is particularly ambivalent: at the same time idealized (the Heros), and rejected (the Ogre)--this is explained by the evolution of the father complex, and by the connections between, on the one hand the ego, and on the other hand the ideal ego and the couple ego ideal-super ego.
Acromegaly↗
[Cerebral gigantism: report on two familial cases (author's transl)].
Two cases of cerebral gigantism occurring in related boys (cousins of 3rd degree) are discussed. It is difficult to argue from these cases in favour of a precise type of hereditary transmission. The hypothesis of a dominant trait with weak penetrance cannot be excluded. A genetic heterogeneity of the Sotos syndrome is very likely.
Abnormalities, Multiple↗
An unusual case of local gigantism.
A 38-year-old female with an unusual degree of gigantism of the left leg is described. There was no other known association. Angiogram did not reveal any abnormality. The patient refused surgical intervention.
Adult↗