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Congenital sacrococcygeal teratomas: effect of gestational age on size, morphologic pattern, ploidy, p53, and ret
M E Herrmann1, K Thompson, E M Wojcik
1Department of Pathology, Stritch School of Medicine, Loyola University Medical Center, Maywood, IL 60153, USA.
Insights
Sacrococcygeal teratomas (SCTs) in infants show poorer prognosis with earlier gestational age (GA). Immature neural tissues and aneuploidy in SCTs correlate with rapid growth and lower survival rates before 30 weeks GA.
Area of Science:
- Oncology
- Developmental Biology
- Genetics
Background:
- Sacrococcygeal teratomas (SCTs) are congenital tumors with variable infant prognosis.
- Gestational age (GA) significantly impacts SCT outcomes, with survival rates dropping sharply before 30 weeks.
- Understanding factors like tumor composition and genetic alterations is crucial for explaining survival disparities.
Purpose of the Study:
- To investigate correlations between gestational age (GA), tumor morphology, ploidy, and expression of p53 and ret in sacrococcygeal teratomas (SCTs).
- To identify potential biological markers associated with the aggressive growth of early-gestation SCTs.
Main Methods:
- Morphological analysis of seven SCTs (GA 21-41 weeks).
- Assessment of DNA ploidy in teratoma tissues.
- Immunohistochemical quantification of p53 and ret expression, correlated with morphology and GA.
Main Results:
- Tumor size relative to infant weight and immature neural tissue content inversely correlated with advancing GA.
- Yolk sac tumors (YST) and immature tissues exhibited aneuploid cell populations.
- p53 accumulation was observed in various YST patterns and immature SCT components; ret reactivity was noted in specific cellular contexts.
Conclusions:
- Rapid SCT growth before 30 weeks GA is linked to increased immature neural tissues.
- The study confirms aneuploidy in YST and suggests its presence in immature SCT tissues.
- p53 accumulation is common in YST and immature SCTs, while the role of ret requires further investigation.
Abstract:
Prognosis of infants born with sacrococcygeal teratomas (SCTs) correlates with gestational age (GA). The survival rate after 30 weeks of gestation is 75%, compared to 7% before 30 weeks of gestation. Studies correlating GA with size, morphologic composition of teratomas, ploidy or expression of cell cycle control proteins such as p53, and ret [a tyrosine kinase receptor of the GDNF (glial cell line-derived neurotrophic factors)] receptor family may provide information explaining differences in survival. Seven SCTs (GA 21 to 41 weeks), ranging in size from 5 to 15 cm, were evaluated for morphologic composition. DNA ploidy was assessed in mature and immature neural elements. Immunohistochemical reactivity with monoclonal antibodies recognizing p53, and ret was quantitated and correlated with morphological pattern and GA. Relative size of teratomas to infants' weight and content of immature neural tissues correlated inversely with advancement of GA. Yolk sac tumor (YST) and immature tissues showed aneuploid cell populations. Nuclear p53 reactivity was apparent in the teratoma with YST in the microcystic patterns, the neuroectodermal rosettes, and the glandular patterns. Ret reactivity was seen in osteoclasts adjacent to bone formation surrounding developing teeth in an immature teratoma, and in rare mature neural cells of one SCT of 35 weeks GA. The rapid growth of SCT (GA <30 weeks) correlates with increase in immature neural tissues. Our study confirms aneuploidy in YST and suggests aneuploid populations within immature tissues. p53 accumulates in a variety of patterns of YST and may be seen in immature components of SCTs. To understand the possible role of ret, further studies comparing ret expression in immature human tissues are needed.