Congenital Sacrococcygeal Teratomas: Effect of Gestational Age on Size, Morphologic Pattern, Ploidy, p53, and ret Expression

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.

Congenital Teratoma

“…Eight of 22 tumors (case nos. [17][18][19][20][21][22] had neonatal resections of teratomas that presented at birth (''neonatal teratomas,'' group 3). 9) was an autopsy of a fetus that died after attempted in utero radiofrequency ablation of the tumor.…”

“…Presentation prior to 30 weeks' gestation is associated with an especially poor prognosis because of an increased risk of premature delivery or development of hydrops fetalis. 21,23,41 Patients presenting in utero are rarely specifically identified in clinical studies. 14 The behavior of congenital teratomas is poorly understood.…”

“…4,5,12,36 Fetal surgical interventions, including in utero tumor debulking, have been developed in an attempt to increase survival in high-risk patients, with some success. 7,18,21,34 The improved treatment and survival of patients with teratomas presenting in utero raise important questions regarding the prognostic significance of such histologic parameters as the presence of malignant germ cell elements such as conventional yolk sac tumor, degree of immaturity (grade), and tumor maturation from the fetal to subsequent neonatal resections. Most reports describe autopsy series or have only a small number of survivors.…”

Factors associated with recurrence and metastasis in sacrococcygeal teratoma

“…This point is further emphasized in the present study, in which only 1·5 per cent of the tumours were malignant when diagnosed and treated in the first week of life, whereas the rate of malignancy was nearly 40 per cent (11 of 28) in children older than 1 year. Further studies with other markers of malignancy may elucidate this phenomenon 19 .…”

Fetal Sakrokoksigeal Teratom-Prenatal Tanı ve Yönetim Olgu Sunumu

“…Otuzuncu gebelik haftasından sonra yaşam oranı %75 iken, 30.hafta öncesi bu oran %7'dir. Gestasyonel yaş, teratomun büyüklüğü ve histopatolojik içeriği sürviv üzerinde etkisi bulunan özel-liklerdir (8). Gestasyonel yaşın sürviv üzerindeki önemli etkisi nedeniyle hastamızda erken başla-yan eylem durdurulmaya çalışılmış, ancak hastanın poşu açıldığı için fetal akciğer matürasyonu için gerekli olan 24 saatlik süre kazanıldıktan sonra tokoliz durdurulup doğum kararı alınmıştır.…”