The deficient development of the peripheral immunopoietic system in acardiac acephalous monsters cannot be explained only by the absence of the thymus. Similarly the adrenal gland hypoplasia does not depend upon the absence of the pituitary gland due to the concurrent condition of acephaly. The author suggests that the failed development of the above mentioned structures depends respectively upon disorders in the peripheral "microenvironment" which is needed by immature lymphoid cells to establish themselves in the peripheral lymphoid structures and upon the decreased sensibility of the adrenal glands to the corticotrophic stimulus. A hypothesis about the frequency of the association between acephaly and acardy is proposed. Finally the author proposes a study program in such cases.

Abnormalities, Severe Teratoid↗

PubMedDate not supplied

Long survival in two cases of acute myelogenous leukemia complicated by chronic active hepatitis.

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Adult↗

PubMedDate not supplied

[The prognostic effect of amplification of the MYCN oncogene in neuroblastoma. The preliminary results of the Italian Cooperative Group for Neuroblastoma (GCINB)].

Of 567 children with neuroblastoma diagnosed between November 1984 and May 1993 in 21 Italian institutions, 235 (41%) have been evaluated for MYCN oncogene amplification. The amplification (3 or more copies of the gene) was found in 39 patients (17%) and was more frequent in patients aged more than one year, abdominal primary site of the tumor, advanced stages, normal urinary excretion of vanillylmandelic acid (VMA), and high level of LDH, NSE and ferritin. The five-year survival of the 235 patients (62%) was significantly better in patients with normal copy number of MYCN (69% versus 29%). By correlating genomic amplification with clinical and biochemical characteristics, MYCN amplification was found associated with a worse prognosis even when patients were subdivided for age (under and above one year), disease extension (localized operable, localized but inoperable, and disseminated) with exception for Stage IV-S, VMA and homovanillic acid excretion, serum levels of NSE and ferritin, but not of LDH. These data confirm the unfavourable prognostic meaning of MYCN amplification, but are unable to define if it represents a new independent variable.

Adolescent↗

PubMedDate not supplied

[Neuroblastoma: update on clinical aspects and therapy].

Despite the remarkable amount of clinical and biologic information that has been cumulated on neuroblastoma, particularly in the last two decades, this embryonal tumor of early childhood remains one of the major challenges of pediatric oncology. It is now clear that the term encompasses at least two entities characterized by different features and outcome. The favorable entity includes patients with localized disease, infants and stage IV-s. The unfavorable entity refers to patients older than one year at diagnosis with disseminated disease. However, in both entities exceptions exploiting a clinical course different from expected do occur. Molecular biology is presently suggesting that specific genomic alterations may predict unfavorable events. Patients evaluation should thus include the study of biologic features since they may provide us with strongly predictive clues. In this article the Authors describe the main clinical features of the neoplasia and focus on some of its more peculiar patterns. They also refer on the criteria that the International Neuroblastoma Staging System (INSS) has recently designed regarding diagnosis, evaluation of disease extent, and response to therapy, in order to improve the cooperations and the understanding among the major Cooperative Groups. Finally, the treatment outlines of the main forms of clinical presentations are described.

Age Factors↗