Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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Sacrococcygeal teratoma
Etiology

The most frequent site of congenital teratomas is the sacrococcygeal region. The tumor arises from totipotential cells of the primitive node (Hensens node) located near the coccyx.

Clinical signs
  • incidence: 1 : 30 000 – 70 000, more frequent in females
  • the prognosis in a fetus correlates inversely with the tumor size and rate of tumor growth, the prognosis is worsened with the presence of hydrops fetalis and placental edema
  • hydrops is a result of high output cardiac failure and precedes the intrauterine demise
  • teratoma is often large and obstructs the delivery (caesarean section or punction of the cystic part of the tumor before the delivery are inevitable)
  • significant mortality and morbidity is due to premature delivery
  • survivors undergo a surgical resection of the tumor and the coccyx as well, otherwise there is a risk of local reccurence
  • the tumor is behaves benignly, the risk of malignant transformation increases if the resection is delayed or if the tumor is incompletely excised.
Macroscopic appearance
  • large tumor deforming the sacral region
  • appearance is solid (on the cut mainly grey-white tender nervous tissue) or cystic

Sacrococcygeal teratoma (73062)

Sacrococcygeal teratoma (73063)

Classification
  • type 1: predominantly external, minimal intrapelvic component
  • type 2: predominantly external, significant intrapelvic component
  • type 3: minor external component, predominantly internal component extending into the abdomen
  • type 4: only intrapelvic component

Type 1 and 2 common and include 80% of the cases.

Histology

Tumor consists of different somatic tissues of all germ layers. The largest part is made of immature nervous tissue (represents the ectoderm).

Pictures

Sacrococcygeal teratoma, fetus: Sacrococcygeal teratoma, HE 40x (72158)