Recent Comprehensive Article on SCTs

I thought the group might be interested in the recent publication of a comprehensive retrospective analysis of 30 cases of fetal SCTs. The article is entitled "Sacrococcygeal Teratoma: Prenatal Assessment, Fetal Intervention, and Outcome" (Hedrick et al. 39 J. of Ped. Surg. pp. 430-438 March 2004). The paper was authored by a group of pediatric surgeons and Ob/Gyns working out of Childrens Hospital of Philadelphia (CHOP). It analyzes the authors' experiences with all 30 cases of fetal SCTs that presented at CHOP between September 1995 and January 2003. This study represents the largest series of fetal SCTs studied to date. A summary of the article follows.

Enrollment and Associated Anomalies

During the seven year study period, thirty women presented with an average maternal age of 29 years (range, 18 to 40). The mean gestational age at the time of CHOP evaluation was 23.9 weeks (range, 19 to 38.5). Tumor composition on prenatal ultrasound scan consisted of 19 solid, 5 cystic, and 6 mixed solid/cystic tumors. The most common associated anomaly diagnosed by ultrasound or MRI was urinary obstruction (13/30 or 43%). Musculoskeletal problems included hip dislocation (n=2) and club feet (n=2). Severe pulmonary hypoplasia was found in 3 fetuses secondary to complications of inadequate amniotic fluid.

Overall Outcomes

4 Abortions
5 Fetal Deaths
7 Neonatal Deaths
14 Survivors

Fetal Deaths

Intrauterine fetal death occurred in 5 fetuses at a mean gestational age of 26.6 weeks (range, 24.6 to 28.7). In 3 of the 5 fetal deaths, high-output cardiac failure and hydrops were present before death. In the remaining 2 fetal death cases, Doppler studies revealed decreasing end-diastolic flow volumes and reversal of diastolic flow. Altman tumor types were as follows: Type I (n=1), Type II (n=2), and Type III (n=2). All tumors in the fetal death group were solid.

Neonatal Deaths

Seven neonatal deaths occurred at a mean gestational age of 28.6 weeks (range, 25.3 to 32.3). Five of the seven tumors in this group ruptured and bled either in utero during preterm labor or during surgery. Altman tumor types for this group were as follows: Type I (n=4), Type II (n=2), Type III (n=1). Six of the tumors in this group were solid and one was mixed solid/cystic.

Standard Postnatal Care

11 of the 14 survivors underwent standard postnatal tumor resection (the paper does not discuss what happened with the remaining 3 survivors). Altman tumor types for this group were: Type I (n=5) and Type II (n=6). The composition of the tumors was cystic (n=5), mixed solid/cystic (n=5) and solid (n=1). In the 9 patients treated at CHOP, the mean gestational age at birth was 36 weeks (range, 33 to 39). All survivors were delivered by c-section. All survivors underwent SCT removal surgery on day 1 or day 2 of life. Two patients had tumor recurrences detected after AFP levels increased at 5 and 16 months of age. Both children underwent reresection. The 16 month old patient underwent a course of chemotherapy. Both patients are now alive and well with no evidence of the disease. Mean hospital stay was 19 days (range, 6 to 37).

Selection for Fetal Intervention

There were 14 instances of fetal intervention among the 30 subjects. Fetal intervention was defined as amnioreduction (n=3), amnioinfusion (n=1), cyst aspiration (n=6), and open fetal surgery (n=4). The fetus who received amnioinfusion (due to inadequate amniotic fluid) did not survive. This fetus received amnioinfusion at weeks 29, 31 and 32 in an (ultimately unsuccessful) effort to facilitate lung growth. The only other non-surviving fetus in this group was a baby who died from complications arising out of a prior open fetal surgery procedure. The goal of prenatal amnioreduction and cyst aspiration was maternal comfort and reduction of uterine irritability. In 3 cases, cyst aspiration was performed just before delivery to preclude birth trauma. Criteria for consideration of open fetal surgery for debulking of the tumor mass include the following: no maternal risk factors for anesthesia or surgery, a single fetus with normal chromosome pattern, absence of significant associated anomalies, affirmative evidence of impending high-output cardiac failure, gestational age < 30 weeks, and favorable tumor anatomy (Altman Type I or II).

Maternal Morbidity

Significant obstetrical complications occurred in 81% of the 26 continuing pregnancies as follows: excessive amniotic fluid (n=7), inadequate amniotic fluid (n=4), preterm labor (n=13), preeclampsia (n=4), gestational diabetes (n=1), HEELP syndrome (n=1), and hyperemesis (n=1).

Radiofrequency Ablation

The authors clearly view RFA as still very experimental. They reference a report on 4 patients treated with RFA. 2 of the 4 patients died secondary to blood loss after a large portion of the tumor mass was ablated. The remaining 2 patients were delivered at 28 and 31 weeks gestation with evidence of perineal and gluteal dead tissue requiring additional surgeries. A second report on use of RFA described intrauterine fetal death on postoperative day 2. A third report on use of RFA described a newborn in whom RFA resulted in a large soft tissue defect, hypoplastic hip joint, and loss of sciatic nerve function. The authors conclude their discussion on RFA by stating that it may be promising but that there needs to be additional testing of the physical and thermal effects of the ablation techniques in normal fetal animal models before further clinical application.

While reviewing this study, I was struck by how little experience even our leading medical institutions have with fetal SCTs. In a seven year period, only 30 cases of fetal SCT were presented at a leading institution such as CHOP. In other words, CHOP sees less than 5 fetal SCTs in any given year. The other leading institution for the treatment of SCTs, Univ. of Cal. at San Francisco, only saw a total of 17 SCT fetuses over a 12 year period from 1986 to 1998. The figure of 1 in 40,000 live births as an incidence of SCTs would yield approximately 300 babies born with an SCT annually in the United States alone. The low numbers seen by CHOP and UCSF suggest that many neonates with SCT's were not diagnosed with the condition in utero. If the SCT develops later in the pregnancy or grows very slowly, it is entirely possible that it would not be picked up on the 1 or 2 ultrasounds a normal pregnancy typically yields. It would seem that the group of prenatally diagnosed SCT babies is a small subset of an already small group of babies born with an SCT.

The second thing I was struck by when reading the paper was the complete absence of any discussion of the relative condition of the 4 babies whose parents elected to abort. It would have been instructive to see whether there was any correlation, for example, between the voluntary termination of the pregnancy and the degree of issues presented by the SCT in each case. My gut tells me that there is no correlation between the relative seriousness of the appearance of the tumor and the decision to abort. Based on my own experience and the shared experiences of others, I believe that medical professionals are frequently guilty of subtle steering of a parent's decision towards termination of the SCT pregnancy (even in the context of "non-directed" counseling). When a physician chooses to identify only the risks and threats and ignores or fails to discuss the positive signs present (e.g. external presentation, cystic composition, non-vascularity) the physician is effectively steering certain parents towards termination in a manner that does not even produce informed consent to the termination procedure.

Michael

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