Variable mechanisms of sudden death at or soon after birth in patients with congenital teratoma

Abstract

Congenital teratomas are generally benign tumours that are curable by adequate surgical resection. Problems may arise in cases where there is malignant tissue present, e.g. where there is an association with another germ cell tumour of much poorer prognosis such as endodermal sinus tumour, or where the tumour is located at a critical site. Three patients who died suddenly as a result of the particular location of their “benign” congenital teratomas are presented to demonstrate the variable mechanisms of death that were involved. Case 1: A 4.8 kg term boy failed to establish normal respirations and died at 1 hr. At autopsy the chest cavity was almost completely occupied by a grossly distended pericardial sac which contained a 40x60x70mm teratoma arising from the root of the aorta. Case 2: A 31 week gestation boy, who was delivered by caesarean section for fetal distress, died after 1 hour of respiratory distress due to upper airway obstruction caused by a 100mm diameter teratoma located in the anterior cervical area. Case 3: A 1.7 kg 36 week gestation girl was admitted in a moribund state following marked haemorrhage during delivery from a 100mm diameter sacrococcygeal teratoma. Death occurred during surgery. These cases demonstrate the clinicopathological features and mechanisms of sudden death in three patients with congenital teratomas located at different anatomical sites that resulted in fatal outcomes. Although the tumours were histologically benign their critical locations led to death from cardiorespiratory compromise, airway obstruction (both with associated pulmonary hypoplasia) and traumatic haemorrhage, respectively. Antenatal diagnosis may have improved the chances of survival in at least two of the patients. The occurrence of sudden death, at or shortly after birth, due to a variety of mechanisms, should always be considered as a possible complication of congenital teratoma. Congenital teratomas are generally benign tumours that are curable by adequate surgical resection. Problems may arise in cases where there is malignant tissue present, e.g. where there is an association with another germ cell tumour of much poorer prognosis such as endodermal sinus tumour, or where the tumour is located at a critical site. Three patients who died suddenly as a result of the particular location of their “benign” congenital teratomas are presented to demonstrate the variable mechanisms of death that were involved. Case 1: A 4.8 kg term boy failed to establish normal respirations and died at 1 hr. At autopsy the chest cavity was almost completely occupied by a grossly distended pericardial sac which contained a 40x60x70mm teratoma arising from the root of the aorta. Case 2: A 31 week gestation boy, who was delivered by caesarean section for fetal distress, died after 1 hour of respiratory distress due to upper airway obstruction caused by a 100mm diameter teratoma located in the anterior cervical area. Case 3: A 1.7 kg 36 week gestation girl was admitted in a moribund state following marked haemorrhage during delivery from a 100mm diameter sacrococcygeal teratoma. Death occurred during surgery. These cases demonstrate the clinicopathological features and mechanisms of sudden death in three patients with congenital teratomas located at different anatomical sites that resulted in fatal outcomes. Although the tumours were histologically benign their critical locations led to death from cardiorespiratory compromise, airway obstruction (both with associated pulmonary hypoplasia) and traumatic haemorrhage, respectively. Antenatal diagnosis may have improved the chances of survival in at least two of the patients. The occurrence of sudden death, at or shortly after birth, due to a variety of mechanisms, should always be considered as a possible complication of congenital teratoma.