Introduction • We present a case of a female neonate with a presacral mass

detected prenatally.

Visual Diagnosis: Neonate with Sacral MassChristina Pabustan MD; Ma Teresa Ambat MD; Davina Lopes De Menezes MD ;

Harry Wilson; MD Department of Pediatrics

Texas Tech University Health Sciences Center - Paul L. Foster School of Medicine, El Paso Texas

Abstract

• A newborn female of Asian-american descent was transferred to our NICU due to a presacral mass.

• She was born to a 25-year-old G1P0 woman of no known medical illnesses. The pregnancy was complicated by prenatal detection of a presacral mass first noted at 18 weeks gestation. Fetal MRI at 28 weeks gestation revealed a mixed solid-cystic mass in the sacrococcygeal region. Serial antenatal ultrasound monitored fetal status and tumor size. Otherwise, pregnancy progressed well and fetal status remained stable without signs of compromise.

• The results of routine maternal prenatal screening tests were negative. The mother denied family history of congenital birth defects.

• An elective CS was performed at 37 completed weeks of gestation due to progressive enlargement of the mass.

• Apgar scores were 8 and 8 at 1 and 5 minutes respectively. Birth weight was 4090 grams plotted at 95 percentile or age. The size of the tumor accounted for a great proportion of her birth weight. Physical and neuromuscular maturity rating was consistent with 38 weeks gestation.

• The mass was prominently located at the presacral area and measured 15cm x 10cm at its widest dimension. It was non- erythematous, non-tender, predominantly solid but cystic areas. Blood vessels were found coursing the surface of the mass but did not transilluminate. She is neurologically intact, tone and reflexes were appropriate for gestational age. In particular, the movement of all extremities were spontaneous and equal. The anal sphincter tone was liekewise intact. There were no other major anomalies found on examination.

Case Summary

Conclusions

References1. Bhat RA, Kushtagi P, Bhat KA. Fetal sacrococcygeal teratoma. The Internet

Journal of Gynecology and Obstetrics. 2005;4(2). Available at:http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijgo/vol4n2/teratoma.xml

2. Tuladhar R, Patole SK, Whitehall JS. Sacrococcygeal teratoma in the perinatal period. Postgrad Med J. 2000;76:754-759

3. Barth RA, Rubesova E. Fetal magnetic resonance imaging: anomalies of the neck, chest, and abdomen. Neoreviews. 2007;8:e313-e335

4. http://neoreviews.aappublications.org/site/case60/diagnose.xhtml

2013 Texas Pediatric Society Electronic Poster Contest

• Sacrococcygeal teratoma is the most common tumor of the newborn period. Most cases are diagnosed prenatally (and incidentally) during routine ultrasound imaging. Once diagnosed, serial ultrasound studies are needed to monitor tumor growth. If fetal hydrops develops, intervention should be done asap to minimize fetal morbidity (or mortality). Management depends on fetal lung maturity and tumor size.

• Most cases are benign and require only minimal intervention. Once fetal maturity is achieved at 37 weeks, scheduled delivery is planned. Complete resection of tumor including coccyx is vital to prevent malignant recurrence. Strict follow up and AFP monitoring is important. Small percentage of these tumors become malignant and can occur even after tumor removal.

Discussion

Fig 4 : Pre-op MRI

Differential Diagnoses of presacral mass in fetus and newborn: •Chordoma•Lipoma•Lymphangioma•Meningocele, meningomyelocele•Rhabdomyosarcoma•Sacrococcygeal teratoma

The Diagnosis:•Sacrococcygeal teratoma is a congenital germ cell tumor arising from embryologically multipotent cells of Hensen’s node, which lies within the coccyx. It occurs in 1 per 20,000-40,000 births and is the most common tumor of the newborn period..

•This usually benign tumor is composed of a wide diversity of tissue foreign to the sacrum. Presence of disorganized tissue originating from >1 germ cell layer is hallmark of SCT. Neuroglias are the most common histologic finding in sacrococcygeal teratoma .

• The tumors are subdivided and classified according to their location:1.Type I (46.7%) - predominantly external tumor with minimal presacral content2.Type II (34.7%) - a large external tumor with significant intrapelvic extention3.Type III (8.8%) - a mass that is apparent externally but whose mass is predominantly pelvic or abdominal or both 4.Type IV (9.8%) - a tumor that is presacral and entirely internal with no external manifestations

Diagnosis/Treatment/Follow-up:•These tumors can be diagnosed prenatally as early as 13 weeks of gestation. Ultrasonography reveals a nonhomogenous sacral mass, with calcification in one third of cases. It also shows the size and extent of tumor as well as mass displacement of the bladder and rectum. Fetal magnetic resonance imaging is superior to ultrasonography because it permits accurate definition of cephalic extent and total dimension of the tumor, colon displacement, urinary tract dilatation, and intraspinal extent of a SCT. Such findings are important for surgical planning. Prenatally diagnosed tumors should be monitored closely for development of fetal hydrops.

•Potential complications in utero include polyhydramnios, tumor hemorrhage, anemia, congestive heart failure, and nonimmune hydrops fetalis. Development of hydrops is an ominous sign and is associated with very poor prognosis. In utero resection of tumor may benefit affected fetuses who develops hydrops prior to 28 to 30 weeks’ gestation. If development of hydrops is recognized after 30 weeks’ gestation, delivery is recommended as soon as lung maturity is documented. For fetuses that have tumors larger than 5 cm, cesarean delivery should be considered to prevent dystocia or tumor rupture.

• The recommended treatment is resection of the tumor en bloc with the coccyx in the first week after birth because long delays may be associated with a higher rate of malignancy.

•All patients who have SCTs should be re-evaluated every 3 to 6 months for 3 years because there is a small but definite risk of malignant recurrence. The outpatient visit should include a thorough physical examination, including a rectal examination, and measurement of AFP.

Figure 2: Actual mass showing cystic and solid components.

Figure 7: Management Algorithm of Sacroccocygeal Teratoma

• SCT should always be considered in a fetus/neonate with a sacral mass. Prenatal diagnosis is usually made with ultrasound findings of sacral mass in fetus. Continuous fetal monitoring (serial Ultrasounds, Doppler, MRI) should be done after diagnosis is made.

• Course of management depend on presence or absence of hydrops and fetal lung maturity. Early prenatal sonographic detection of sacrococcygeal teratoma allows for optimal perinatal obstetric and surgical management.

• Definitive treatment is en bloc resection of tumor in 1st week of life. Follow up and reevaluation every 3-6 months for 3 years is vital due to risk of malignant recurrence. Prognosis for those without complications or malignancies is usually very good.

• Pre-op MRI showed a large non-homogenous sacral mass with calcifications causing upward displacement of pelvic structures. Diagnosis of sacrococcygeal teratoma was made and pediatric surgical team was consulted.

• Surgical resection was done with the coccyx excised in continuity with the tumor on day 1.

• Post operative course was uneventful. Hematology–Oncology specialist was also consulted. Serial alpha-fetoprotein measurement showed a consistent downward trend (AFP >100000 26,2012500376.8).

Figure 1: The infant with a large presacral mass as described above.

Diagnosis and NICU Course

Figure 3: Pathology - skin & mature teratoma tissues with brain-like mature neural component showing gliosis & fibrosis with disordered fibrofatty & fibromuscular connective tissue elements, no malignant or atypical features identified.

Figure 5: Post-op MRI – Changes consistent with recent excision, no residual tumor or surgical complication.

Figure 6: Altman Classification

Immature Lungs Mature Lungs

Placentomagaly/hydrops

Pregnancy termination/Fetal surgery

ElectiveCS

Term NVSD

Emergency CS

YES NO

Serial US

Placentomegaly/hydrops

YES NO

LargeSymptomatic tumor

Small orAsymptomatic tumor