Association for Academic SurgeryPrenatal solid tumor volume index: novel prenatal predictor of adverse outcome in sacrococcygeal teratoma
Introduction
Sacrococcygeal teratoma (SCT) is the most common congenital tumor in neonates with an incidence of 1 in 23,000 to 40,000 births [1]. A majority of the tumors are detected during routine prenatal screening, which allows for enhanced maternal counseling, more intensive monitoring, and appropriate intervention when necessary. Despite the improvements in prenatal diagnosis, mortality still approaches 50% during the prenatal period secondary to maternal and fetal complications from the possible high flow vascular tumor pathophysiology [2], [3].
The high prenatal mortality stems from its unpredictable nature in terms of growth and vascularization [3], [4]. Tumors become large and vascular, resulting in internal tumor arteriovenous shunting. Venous overload leads to high output cardiac failure in the fetus and sequelae such as cardiac failure, hydrops, maternal mirror syndrome, and fetal demise can ensue [5], [6], [7]. This high output cardiac pathophysiology then becomes one of the relative indications that requires urgent prenatal intervention [2]. Attempts have been made to calculate how the tumor will behave but only a few characteristics have been correlated with poor outcomes, including large size and vascularity [8], [9], [10].
Radiological advances in high resolution ultrasound and ultra-fast magnetic resonance imaging (MRI) has increased the ability to diagnose many teratomas early during the prenatal course. The focus of care then becomes stratification of patients into low and high risk groups, which facilitates appropriate counseling and interval monitoring. Frequent cardiac, ultrasound, and MRI assessments for patients with worrisome tumor characteristics, such as tumor growth and signs of high output cardiac failure, permits earlier detection of complications that can be managed with measures such as intensive inpatient monitoring, open fetal surgery, early delivery, and ex utero intra partum therapy to resection [2], [4], [11], [12]. However, the risk-benefit ratio in regards to management can be difficult to assess. Early delivery is hindered by the mortality and morbidity associated with prematurity. Expectant management with closer monitoring can be disastrous as hydrops can manifest precipitously and rapidly progress to intrauterine fetal demise. Finally, fetal surgery can be a lifesaving intervention but may incur its own complications to both the mother and the baby. For the most part, the decision tree has been based on clinical expertise utilizing minimal objective indices in an algorithm [13].
The purpose of this study is to investigate the predictive value of solid tumor volume on outcome, specifically development of a high cardiac output state and hydrops. We aimed to develop a solid tumor volume index (STVI) that could be used to stratify patients into high risk groups and assist in their management algorithm, with the goal of optimizing the risk-benefit ratio for both mother and child.
Section snippets
Data collection
A retrospective review of all SCT patients prenatally evaluated at our fetal care center between the 2005 and 2012 was conducted. Approval was obtained from the Institutional Review Board of Cincinnati Children’s Hospital Medical Center (IRB #2011-2078). Patients were excluded from the study if pregnancy was voluntarily terminated prior to delivery, adequate postnatal data was not available, or if multiple tumors were present. Data collected included gestational age at evaluation, time of
Results
Thirty-eight patients were evaluated over the study period. Seven patients were excluded. Three patients had elective terminations for various reasons. One patient with twins was in labor at the initial evaluation, and we were therefore unable to gather all prenatal data prior to initiation of delivery. In that circumstance, the twin with the SCT died in utero while the other twin was vaginally delivered stillborn. Two other patients had inadequate postnatal delivery information, and we were
Discussion
The goal of this study was to investigate the predictive value of solid tumor volume on outcome, specifically development of a high cardiac output state and hydrops. Our data suggest that STVI and total tumor volume/EFW are reliable predictors of adverse outcome in fetuses with SCT. In the evaluation of our SCT population, we have shown that the volume of the solid component has a greater predictive value of outcome than the overall size of the tumor itself. Historically, a more solid SCT is
References (20)
- et al.
Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome
J Pediatr Surg
(2004) - et al.
Death due to high-output cardiac failure in fetal sacrococcygeal teratoma
J Pediatr Surg
(1990) - et al.
Prenatally diagnosed sacrococcygeal teratoma: a prognostic classification
J Pediatr Surg
(2006) - et al.
Tumor volume to fetal weight ratio as an early prognostic classification for fetal sacrococcygeal teratoma
J Pediatr Surg
(2011) - et al.
Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas
J Pediatr Surg
(2011) - et al.
Estimation of fetal weight with the use of head, body, and femur measurements—a prospective study
Am J Obstet Gynecol
(1985) - et al.
Comparison of the accuracy of CT volume calculated by circumscription to prolate ellipsoid volume (bidimensional measurement multiplied by coronal long axis)
Acad Radiol
(2009) - et al.
High-output cardiac failure in fetuses with large sacrococcygeal teratoma: diagnosis by echocardiography and Doppler ultrasound
J Pediatr
(1989) - et al.
Sacrococcygeal teratoma over two decades: birth prevalence, prenatal diagnosis, and clinical outcomes
Prenat Diagn
(2008) - et al.
Sacrococcygeal teratoma: prenatal surveillance, growth, and pregnancy outcome
Fetal Diagn Ther
(2009)
Cited by (24)
Approach and Technique for Cesarean Section to Immediate Resection for High-Risk Sacrococcygeal Teratomas
2023, Journal of Surgical ResearchNeurocognitive Effects of Fetal Exposure to Anesthesia
2021, Anesthesiology ClinicsCitation Excerpt :The decision on when and how to intervene in utero is one in which multiple predictive tools have been used to better predict which fetuses are likely to succumb to the condition in utero and therefore benefit from fetal intervention. Those models include a tumor-to-fetal ratio of greater than 0.12 before 24 weeks’ gestation,7 solid tumor volume index,8 and tumor growth rate9 to name a few. Ultimately, the goal is to avoid lethal high-output cardiac failure in utero, as this tumor can act as a large arteriovenous fistula.
Fetal body MRI for fetal and perinatal management
2021, Clinical RadiologyCitation Excerpt :Intra tumoural haemorrhage cannot be easily ascertained by ultrasound but is easily recorded on T1-weighted images; accordingly, intrauterine transfusion(s) can be considered for those fetuses whose high-output cardiac deterioration results from fetal anaemia rather than tumour-related vascular steal.48 Although US seems sufficient for evaluating the extrapelvic sacral mass of SCT, the more accurate characterisation of the intrapelvic and abdominal extent of the tumour (Fig 8) significantly improves prenatal counselling,48 enabling the aspiration/shunting of severe urogenital compressions that lead to oligohydramnios and pulmonary hypoplasia, and helps to select the most appropriate postnatal surgical approach.44,45 Lower urinary tract obstruction (LUTO) is a complex fetal genitourinary disorder that includes various anatomical abnormalities associated with significant morbidity and mortality due to severe oligohydramnios and pulmonary hypoplasia ultimately prompted by severe renal dysfunction.49
A large, highly vascularized sacrococcygeal teratoma in a preterm male infant: A case report
2021, Asian Journal of SurgeryFetal Tumours
2019, Fetal Medicine: Basic Science and Clinical PracticeSolid Cancers in the Premature and the Newborn: Report of Three National Referral Centers
2016, Pediatrics and NeonatologyCitation Excerpt :Between 1980 and 1999, only four newborn infants were referred to the three tertiary centers, whereas after 2000, thanks to the cooperation of gynecologists, obstetricians, surgeons, and oncologists, we implemented a referral system and more than 30 children were referred, with a significantly improved outcome. Germ-cell tumors and neuroblastoma are the most common malignant solid neoplasms in the newborn period, followed by soft-tissue sarcoma, renal tumors, brain tumors, and leukemia.3,13,16,18 This distribution was confirmed in our series, although we had a higher proportion of neuroblastomas, probably resulting from the exclusion of mature and immature teratomas.