Retroperitoneal immature teratoma.
A near term newborn male infant was evaluated for severe abdominal distension. He was born at 36 weeks of gestation weighing 3500 grams to a 24-year old mother via Cesarean section. Maternal history was pertinent for syphilis and chlamydia for which adequate treatment was received. The delivery was complicated by severe respiratory distress requiring positive pressure ventilation. Apgar scores were 1, 3 and 4 at one, five and ten minutes respectively. Vital signs were: heart rate 160 bpm, respiratory rate 90 bpm, and oxygen saturation 82% with fraction of inspired oxygen 100 percent. On physical examination, the infant was consistently tachypneic with subcostal retractions. Abdominal examination showed marked abdominal distension, visible dilated veins, and a palpable firm mass on the right lower quadrant extending to the left lower quadrant (Figure 1). The remainder of the examination was within normal limits. Basic metabolic panel, Beta human chronic gonadotropin, and human VanillyImandelic Acid levels were normal, but alpha feto-protein was elevated a 35,350 ng/ml (normal range, 33,113 [+ or -] 32,503). All other clinical labs were unremarkable. The fetal ultrasound was normal; however, postnatal abdominal ultrasound and Doppler flow examination revealed a heterogeneous mass of 11 x 10 x 9 cm in dimension with hypoechoic areas and vascular flow within the mass pushing the abdominal organs superiorly and the bowel loops in the pelvis (Figure 2). The liver, spleen, pancreas and right kidney were normal but left kidney was not visualized.
Echocardiogram reported a right to left shunt through the ductus arteriosus with pulmonary hypertension. Inhaled nitric oxide was started given the echocardiogram findings of persistent pulmonary hypertension. Because of increasing abdominal distension worsening the respiratory failure, an exploratory laparotomy was undertaken on day 9 of life. During the procedure a large mass was removed that had displaced the colon and the small intestines into the pelvis. The mass had clotted blood on the lateral inferior and posterior surface due to rupture. The mass was attached to the posterior wall of the stomach which necessitated resection of the posterior wall of the stomach. The remainder of the abdominal structures and the left kidney were normal. The patient had marked improvement in his respiratory status after the procedure and was extubated on day 14 of life. Surgical pathology of the mass reported a weight of 455grams with a smooth glistening surface and small necrotic area (Figure 3), it was jelly-like; soft with diffuse cystic areas. Serial sections of the mass showed tan gray soft cut surfaces with multilocular tiny gray areas intermingled with dark areas (Figure 4,5). This description is consistent with immature teratoma with abundant immature neuroepithelial tissues. Follow up magnetic resonance images of the abdomen and pelvis at 4 months of age showed recurrence of the tumor for which he underwent another resection. Follow up at 12 months of age showed no recurrence of the tumor.
The differential diagnosis of a large abdominal masses in neonates includes: neuroblastoma, nephroblastoma, feto-in-fetus, teratoma, and meconium cyst. Prenatal detection by ultrasound and post-natal evaluation by computed tomography are the mainstays of evaluation. Planning for surgical procedures is the first step in treatment of these varied diagnoses.
Embryonic tumors are masses arising from rudimentary or immature tissue during fetal life. The basic pathophysiological mechanism contributing to the development of embryonic tumors is instability of cell growth and proliferation. (4) During the embryonic period, primordial germ cells originate external to the embryo within the yolk sac wall between four and six weeks of gestation, and migrate to the germinal ridges where they continue to undergo mitosis. During the process of migration a few cells become isolated in an abnormal location giving rise to germ cell tumors (GCTs) in the fetus and neonates. (5,6) GCTs are believed to arise from totipotent primordial germ cells choosing alternate pathways of abnormal development. Histologically, GCTs are classified as germinomas, embryonic carcinomas, yolk sack tumors, malignant mixed GCTs and malignant teratomas. (7)
Teratomas are one of the most common neonatal tumors accounting for approximately 25 percent of all neonatal tumors with a yearly incidence of 1 in 40,000 live births. (8-9) Teratomas are embryonic tumors that are derived from two to three germinal layers. Based on the presence of malignant elements they are categorized into mature and immature teratomas. The types of teratomas vary by the site of the tumor, and have been reported more frequently in females displaying a bimodal incidence with the first peak between birth and 4 years of age, and the second peak at the onset of puberty. (10-16) Congenital teratomas are usually extragonadal and histologically benign with a mortality of 50 percent if the teratoma is diagnosed prenatally secondary to excessive uterine enlargement or polyhydramnios. (17-20) Peritoneal tumors are extremely rare in comparison to retroperitoneal tumors with surgical extirpation remaining the backbone of treatment. Because of extensive blood supply to these tumors, consultation with a radiologist is advised to determine the specific venous outflow prior to surgical resection, as the risk of massive bleeding is very high. Unfortunately, outcomes for patients with malignant GCTs of pure or mixed histology treated with surgery and/or radiotherapy are generally poor.
For grade 3 immature ovarian teratoma, relapse rates have been reported as high as 70 percent. (21) However, because of the different clinical presentation of the pediatric immature teratomas, the patient should be followed periodically with diagnostic imaging, and evaluation of serum tumor markers, including alpha fetoprotein, and BHCG.
In summary, GCTs are the most common type of teratomas in the newborn period with the majority of them diagnosed antenatally. Based on the size, location and complexity of these tumors, optimal management should involve a multidisciplinary team comprised of an obstetrician, neonatologist, surgeon, anesthesiologist, radiologist and oncologist.
Hassan Ibrahim, MD; Shehzad Huda, MD; Ashley Northcutt, MD; Donald Sorells, MD; Thomas Gates, MD; Hilary Tice, PharmD; Guillermo Sangster, MD
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(19.) Flake AW, Harrison MR, Adzick NS, et al. Fetal sacrococcygeal teratoma. J Pediatr Surg.1986;21:563-566.
(20.) Schmidt KG, Silverman NH, Harrison MR, et al. High-output cardiac failure in fetuses with large sacrococcygeal teratoma: diagnosis by echocardiography and Doppler ultrasound. J Pediatr. 1989;114:1023-1028.
(21.) Norris HJ, Zirkin HJ, Benson WL: Immature (malignant) teratoma of the ovary: A clinical and pathological study of 58 cases. Cancer 37:1976:2359-2372
Dr. Ibrahim and Dr. Gates are Associate Professors of Pediatric Neonatology at LSUHSC-Shreveport, LA; Dr. Huda is a Fellow of Neonatal Perinatal Medicine UAB, Birmingham AL; Dr. Northcutt is a surgical resident at LSUHSC-Shreveport, LA; Dr. Sorrells is a Clinical Assistant Professor of Pediatric Surgery at LSUHSC-Shreveport, LA; Dr. Tice is an Assistant Professor, Pharmacy, University of Louisiana at Monroe, LA, School of Pharmacy Shreveport, LA. Campus; Dr. Sangster is a Professor of Radiology at LSUHSC-Shreveport, LA.
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|Author:||Ibrahim, Hassan; Huda, Shehzad; Northcutt, Ashley; Sorells, Donald; Gates, Thomas; Tice, Hilary; San|
|Publication:||The Journal of the Louisiana State Medical Society|
|Article Type:||Clinical report|
|Date:||Mar 1, 2015|
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