Recently Antich et al. [1995] reported on a pair of sibs with Schinzel-Giedion syndrome, one of whom had resection of a sacrococcygeal teratoma at 1 month with local recurrence at 14 months. This was the third report of a sacrococcygeal tumor in a patient with Schinzel-Giedion syndrome. In 1994 RodrΔ± Β΄guez et al. described a patient with Schinzel-Giedion syndrome and a ''primitive'' neuroectodermal tumor arising in the lumbosacral area. A year earlier, Robin had reported a sacrococcygeal teratoma in a patient with otherwise typical Schinzel-Giedion syndrome . We report a fourth patient with Schinzel-Giedion syndrome and a sacrococcygeal tumor.
NR was born at 37 weeks to a healthy primigravid woman after a pregnancy complicated by polyhydramnios. Fetal hydronephrosis and ambiguous genitalia were recognized on ultrasonography at 34 weeks, and fetal karyotype in amniocytes was 46,XY. The birth weight of 2,926 g and OFC of 34 cm were appropriate for gestational age, but the infant was hypotonic and required ventilatory support after birth. He had a huge anterior fontanelle, high forehead, shallow orbits with borderline hypertelorism, retracted midface, choanal stenosis, anteverted nose, apparently lowset ears with thick helices, and macroglossia with intact palate (Fig. ). He was hirsute and had hyperconvex narrow fingernails, single palmar flexion creases, and adducted, slightly small thumbs. His chest was normally formed with hypoplastic nipples. There was no evidence of congenital heart disease. His abdomen was distended and ultrasonography confirmed hydronephrosis. He had micropenis with a normal sized scrotum and palpable testes. His back initially appeared normal, but within a week, he was noted to have an asymmetric swelling in the sacral area. X-ray films showed broad ribs, hypoplasia of the first ribs, and unossified pelvis.
His respiratory status improved gradually. He had mildly elevated creatinine with normal urine output. Tube feedings were required because of poor suck and reflux. Seizures developed in the first week of life and were controlled with phenobarbitol. At 1 month, he underwent resection of his sacral mass. Histologic study