Bilateral neuroblastoma in situ associated with microcephaly
Journal of Korean Medical Science
;
: 99-103, 1993.
Article
in English
| WPRIM
| ID: wpr-161579
ABSTRACT
We present an autopsy case of a two-day-old female infant with a very unusual combination of neuroblastoma in situ in both adrenals and microcephaly. This baby was born to a 28-year-old mother after 38 weeks of gestation, and died of respiratory difficulty 2 days later. At autopsy, the baby weighted 1,840gm, and the brain was extraordinarily small with a weight of 125gm. The gyral pattern was simplified and irregular. Microscopically massive migration defects, pachygyria, micropolygyria, leptomeningeal glioneuronal islands, small corticospinal tract and heterotopic Purkinje cells in the cerebellum were found. In addition, there were medullary nodules in both adrenals. They measured 0.7 x 0.4cm and 0.7 x 0.3cm, respectively. These nodules showed the typical histological features of undifferentiated neuroblastoma. The tumor nodules were confined to the medullary portion and did not extend to the cortex or contiguous structures meeting the criteria of neuroblastoma in situ. Based on these unusual and seemingly unrelated sets of findings, it is suggested that the histogenesis of neuroblastoma in situ could be a part of the generalized dysontogenic process.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Carcinoma in Situ
/
Adrenal Gland Neoplasms
/
Microcephaly
/
Neuroblastoma
Limits:
Adult
/
Female
/
Humans
/
Infant, Newborn
/
Pregnancy
Language:
English
Journal:
Journal of Korean Medical Science
Year:
1993
Type:
Article
Similar
MEDLINE
...
LILACS
LIS