Macrogenitosomia Praecox

MACROGENITOSOMIA PRAECOX (from Greek makros - large, Latin genitalia - genital organs and Greek soma - body), a name proposed by Pellizzi to denote a developmental anomaly in which premature sexual and physical development is observed, while the psyche and intellect remain at the level of the child's actual age or are even below it." Some authors identify M. r. with pubertas praecox (see), however such an interpretation is incorrect. In the pubertal period of childhood, all features by which an adult differs from a child develop proportionally, harmoniously, and as a whole: growth and physical development, psyche and sexual maturation. Accordingly, under pubertas praecox one should understand only harmoniously and as a whole premature development. M. r., as the name itself shows, represents one form of disproportionate, one-sided premature development. Another form of one-sided premature development, observed exclusively in girls, is so-called hirsutism (see).- About 400 cases of M. r. have been described. In girls it is observed 3-4 times more often than in boys. The etiology is unknown. The possibility of genotypic origin of M. r. is indicated by the appearance of premature development in most cases already in the first years of life, as well as the noted simultaneous existence of this anomaly in parents and child in some cases.- Regarding pathogenesis, a connection between M. r. and lesions of the endocrine glands can be considered established: sexual, adrenal and pineal, as well as the central nervous system. Malignant tumors (sarcomas, carcinomas) were found in the sexual glands, which by their rapid growth caused enhanced functioning of the glands. Premature development in some cases followed the type of pubertas praecox, in others - M. r. In the adrenal glands, hypernephromas or hyperplasia of the cortical substance were observed, i.e., here too the mechanism of premature development is connected with hyperfunction of the gland. In girls, hypernephromas lead to the development of the hirsutism syndrome. In boys (4 cases), along with premature sexual maturation, the enhanced development of muscles and skeleton (Herculean boys) was striking; intellectual development was either below or above normal. In the pineal gland, tumors of various nature were observed-teratomas, sarcomas, cysts, etc. Most authors consider M. r. in tumors of this gland as a result of the loss of its function (hypo pinealism). In some cases, there were no changes in the endocrine glands, but they were found in the central nervous system (hydrocephalus, tumors of the bottom of the III ventricle); in these cases, one can speak of the participation of vegetative centers in the mechanism of development of M. r. Finally, cases are described in which there were no organic changes either in the endocrine glands or in the central nervous system; such cases Lenz (Lenz), Molchanov and others consider as "functional hypergenitalism".-The fate of children with M. r. is not the same. Those who have a tumor or other organic lesions soon die if an operation is not performed. Patients with "functional hypergenitalism" can live to a very old age. Their sexual life begins early, some had numerous offspring; in 10 cases, births occurred before 10 years of age. With the end of sexual maturation, the growth of children with M. r. stops, even if they have not yet reached the size of an adult; thus, M. r. ultimately leads to dwarf growth (nanismus genitalis), but the psyche and intellect continue to develop despite the cessation of growth.-X-ray treatment for tumors of the sexual glands and adrenal glands gives a brilliant effect; cessation of development, disappearance of secondary sexual characteristics and even reduction of the sexual organs themselves are observed. Attempts to remove tumors of the pineal gland ended in failure: death soon occurred. In functional hypergenitalism, organotherapy (preparations of the thyroid, pineal and other glands) was used, but without result.