Prognostic and therapeutic factors influencing the clinical outcome of hepatoblastoma after liver transplantation: A single‐institute experience

LT has contributed to an elevation in cure rates for patients with unresectable HB; however, patients with recurrent HB after LT have poor prognosis. To analyze the prognostic and therapeutic factors that influence the clinical outcome of patients with HB receiving LT, we retrospectively analyzed 24 patients with HB who underwent LT between 1997 and 2015. The 5‐year OS rate of all patients was 69.6±9.7%. The 5‐year OS rate of 11 patients receiving salvage LT for recurrent tumor after a primary resection was comparable to that of 13 patients receiving primary LT. Among 12 evaluable patients receiving primary LT, six of 10 patients with a decline of serum AFP >95% at LT are currently alive and in remission, whereas two patients with a decline of AFP ≤95% experienced post‐LT relapse. Among 9 evaluable patients receiving salvage LT, all three patients with any decline of AFP at LT are currently alive in remission, and three of six patients with no response to pre‐LT salvage chemotherapy are also alive and in remission. Response to chemotherapy may be a reliable marker for prediction of post‐LT relapse, even for patients receiving salvage LT.     

Alpha1-fetoprotein: physiology, pathology and diagnosis especially in childhood (author's transl)]

Alpha1-fetoprotein (AFP) is an alpha1-glycoprotein which can be found in high concentration during fetal development in many mammals, birds, sharks and, also, man. The alpha-fetoproteins of various species have similar physico-chemical properties and often common antigenic determinants. Differences of microheterogeneity depend on a different content of sialin-acid. During human fetal development the serum AFP concentration falls with increasing gestational age. 4-5 weeks after birth AFP can be detected usually in low serum concentrations. Using more sensitive immunulogic techniques e.g. radioimmunoassay there was shown that AFP is present in sera of normal adults in concentrations of 10-20 ng/ml. AFP serum concentrations rise physiologically during pregnancy up to 500-550 ng/ml. During fetal development liver, yolk sac and gastrointestinal tract are the major sites of synthesis. In primary liver cell carcinoma, hepatoblastoma and in teratoblastoma containing yolk sac tissue AFP synthesis rises in tumor cells; the AFP serum concentration increases above 2 microgram/ml. In patients with benign liver diseases e.g. virus hepatitis, a transient rise of AFP serum concentrations was seen. Moreover, increased levels of AFP were found in hereditary diseases e.g. congenital tyrosinemia, ataxia-telangiectasia and in the amniotic fluid in congenital nephrosis of Finnish type. AFP assay in serum is clinically important for the control of course and treatment of primary liver cell carcinoma and teratoblastoma. AFP assay in amniotic fluid is a method for the prenatal detection of neural tube defects and the fetal distress syndrome, especially.     

Actual half-life of alpha-fetoprotein as a prognostic tool in pediatric malignant tumors

In a retrospective study, the prognostic value of monitoring the decay of alpha-fetoprotein (AFP) was assessed. Serum AFP was determined serially in 18 children with malignant germ-cell or hepatic tumors: 7 endodermal sinus tumor, 3 embryonal carcinoma, 5 malignant teratoma, 2 hepatoblastomas, and 1 hepatocellular carcinoma. The actual half-life (AHL) of AFP was computed after surgical resection of the tumor. In group 1, which had complete resection and no recurrence during follow-up (n = 13), the AHL of AFP was 4.0 ± 0.9 days. In group 2, which had incomplete resection or recurrence during follow-up (n = 5), the AHL of AFP was 24.8 ± 20 days, significantly longer than that of group 1 (P = 0.0026). The increased AHL of AFP indicated residual active tumor after surgical resection. The AHL of AFP may be more sensitive than serial monitoring of AFP in detecting preclinical recurrence after surgical resection of AFP-secreting tumors. Treatment strategies can be based on AFP clearance, and prospective clinical trials are warranted.     

The prognostic significance of serum alpha 1-fetoprotein in children and adolescents with malignant extracranial non-testicular germ cell tumors]

The cooperative therapy study MAKEI 83/86 included an examination of the prognostic value of the AFP in children and adolescents with extracranial non-testicular yolk sac tumors. The serum AFP values of 72 protocol- and follow-up-patients were documented at diagnosis and up to the ninth month of treatment. 32 of these patients had saccrococcygeal tumors, 27 had tumors of the ovary and 13 suffered from extragonadal germ cell-tumors. 4 children showed progressive disease under initial chemotherapy and 1 patient died of therapy, 10 of 72 patients relapsed. The AFP measurements were plotted on semilogarithmic charts. They were compared to the measurements of healthy children up to the age of 1 year. According to the development of the patients' AFP values compared to the reference curves the following classifications could be made: 1. Patients with a normal AFP-decrease id est 50% in less than or equal to 6 days during the 1st month of therapy: 48/72 patients 2. Patients with slow AFP-decrease: 17/72 patients 3. Patients with transient AFP-decrease: 5/72 patients 4. Patients with no AFP-decrease: 2/72 patients According to Kaplan-Meier life table analysis, patients with a normal AFP-decrease had an event-free survival of 89% +/- 4%, whereas all other patients showed an event-free survival of 63% +/- 10% (p less than 0.05). Regarding primary therapy id est tumor resection or preoperative chemotherapy an equal distribution of the patients among those with a normal and slow AFP-decrease was observed.(ABSTRACT TRUNCATED AT 250 WORDS)     

IMMUNOLOGICAL STUDIES IN CHILDREN BEFORE AND AFTER SPLENECTOMY

Abstract. Fourteen children underwent splenectomy for congenital spherocytosis, splenomegaly, or thrombocytopenia. The patients were studied twice before the operation, three times during the first postoperative month, and one year later. A transitory rise in neutrophils and serum IgA was seen postoperatively; there was a modest but long-lasting increase in lymphocytes and a marked elevation of eosinophils. An immediate decline in serum IgM concentration was observed only in patients with an uncomplicated postoperative course, but one year after splenectomy the average IgM concentration had decreased by 23 %. The in vitro lymphocyte transformation response to a panel of mitogens and antigens fell in the immediate postoperative period but was largely normalized 10 days postoperatively, except in the youngest of the patients who had repeated infections following the splenectomy. One year postoperatively the transformation response and the number of T- and B-lymphocytes in the blood were normal.     

Alpha fetoprotein is increasing with age in ataxia–telangiectasia

The elevated serum alpha fetoprotein (AFP) concentration in ataxia-telangiectasia (A-T) patients has been known for decades, but the individual variation of AFP levels over time has not been studied. We have followed 12 patients (five girls and seven boys) for 1-12 years (mean 5.5 years) measuring in each patient AFP 2-8 (mean 4) times. Serum AFP levels were increased in all patients, mean 168.7 (range 40-373) kU/L, and without significant differences between the patients. There was a significant age related difference in the serum AFP level. A positive linear relationship (r=0.61, p=0.04) could be found between AFP level and age. Albumin levels were within normal range and did not change with age. Four patients had slightly increased aspartate aminotransferase (AST) levels. None of the patients had serological evidence of infectious hepatitis, and none had increased levels of carcinoembryonic antigen. Repeated standardized observations of gait function revealed no major difference in neurological deterioration between our patients. All had classical A-T disease and mainly truncating mutations; 21 out of 24 possible mutations were either frameshift or nonsense. Four were homozygous for the Norwegian ATM founder mutation. No correlation between serum AFP levels and the different ATM genotypes could be found. We conclude that serum AFP is not only elevated, but also is continuously increasing with age in patients with classical A-T disease.     

Alpha-fetoprotein following neonatal resection of sacrococcygeal teratoma

The sacrococcygeal teratoma (SCT) is the most frequent tumor in the neonatal period. Alpha-fetoprotein (AFP) levels fall after tumoral resection, although the time required for level normalization has not been established. The authors evaluate the time required for AFP levels to reach normal levels in 6 infants with prenatal diagnosis of SCT who underwent surgery for resection. The mean time required for AFP level normalization was 9 months. All patients were alive and 1 had neurogenic bladder. AFP has a progressive decrease, which may last 9 months in average and should not be confused with tumoral relapse.     

Alpha1-fetoprotein in neonatal hepatobiliary disease.

It has been suggested that the quantitative estimation of serum alpha-1-fetoprotein may help in distinguishing the neonatal hepatitis syndrome from biliary atresia. We measured the serum AFP concentration in 52 neonates and infants with various hepatobiliary disorders, including neonatal hepatitis syndrome (group I), biliary atresia (group II), and other hepatopathies such as choledochal cyst (group III). The mean serum AFP concentration in patients with neonatal hepatitis was significantly greater than the mean concentration in the other two groups. There was no significant difference between the mean serum AFP concentrations in patients with biliary atresia and in group III patients. Patient age was noted to be an important factor: Serum AFP levels greater than 35 microgram/ml in infants one to four months of age suggpst the diagnosis of neonatal hepatitis syndrome. Serum AFP levels below 10 microgram/ml in infants less than four months of age suggest the diagnosis of biliary atresia or hepatopathies other than neonatal hepatitis. However, the variable and significant overlapping of serum AFP values between 10 and 35 microgram/ml limit the diagnostic value of this test.     

75例肝母细胞瘤治疗回顾

目的 回顾小儿肝母细胞瘤(HB)的治疗经验,探讨影响疗效的临床、病理因素.方法 分析15年间75例HB的临床资料,按时间先后分为Ⅰ组(1990年1月至1998年12月,32例)和Ⅱ组(1999年1月至2005年1月,43例).Ⅰ组的化疗方案为ICE+A等多种方案,Ⅱ组统一参照日本小儿肝肿瘤研究组的JPLT-1方案进行规范治疗.对各临床、病理参数进行Kaplan-Meier单因素和Cox模型多凶素分析.结果 中位随访时间为52个月(24～156个月).Ⅰ组术后2年和5年生存率分别为43.6%和21.9%,Ⅱ组分别为81.4%和72.0%,二组比较,差异有统计学意义(P＜0.01);Ⅰ组术后2年和5年无瘤生存率为37.5%和15.6%,Ⅱ组为74.4%和62.5%,二组比较,差异有统计学意义(P＜0.01).单因素分析提示PRETEXT分期,血清AFP在术后2个月内降至正常、肝硬化、腔静脉和门静脉瘤栓、肿瘤多灶性、规范化疗方案、根治性肝切除术(手术切缘距肿瘤2 cm以上)是HB的预后指标;Cox模型多因素分析则提示PRETEXT分期、腔静脉和门静脉瘤栓、肿瘤多灶性、规范化疗方案、根治性肝切除术是影响HB预后的因素.结论 规范化综合治疗可使HB的疗效显著提高,根治性手术切除和完备的术前、术后化疗是提高HB患儿生存率的关键.     

Yolk sac tumor with a unique uniform hepatoid pattern histology

Abstract Background: Yolk sac tumors (YST) exhibit several different histological subtypes and the mechanisms of cellular differentiation and prognosis of each subtype remain unknown.
Results: We report two infants with sacrococcygeal YST; one represented a typical histological subtype and the other showed a hepatoid subtype with uniform histology. The isoform of alpha-fetoprotein (AFP) in the patient with the hepatoid pattern was examined by lectin-affinity Immunoelectrophoresis and represented as a YST. but not hepatocellular, subtype. The patient with typical YST responded well to VAB-6 combination chemotherapy. However, this regimen was only partially effective to the patient with the pure hepatoid histological subtype, and an etoposide with ifosfamide and cisplatin (VIP) regimen as a salvage chemotherapy combined with complete tumor resection was useful to achieve complete remission (CR). Both of the patients have been in CR for more than four years.     

Pretransplant trends in α‐fetoprotein levels as a predictor of recurrence after living donor liver transplantation for unresectable hepatoblastoma: A single‐institution experience

LT is a practical therapeutic alternative for unresectable hepatoblastoma; however, deciding when to perform LT is difficult. The aim of this study was to optimize the timing of LT for hepatoblastoma using pretransplant trends in AFP levels. Trends in pretransplant AFP levels and their influence on post‐transplant outcomes were retrospectively evaluated. All patients who underwent living donor LT for hepatoblastoma in our institution since 2002 were included. Variables analyzed included history of prior tumor resection, pretransplant AFP responses to chemotherapy, metastatic disease at diagnosis, and post‐transplant chemotherapy. Eight patients (seven boys and one girl; median age, 35 months; range, 15 months‐12 years) were transplanted. The overall post‐transplant recurrence‐free survival rate was 62.5% (5/8) with a mean follow‐up of 77 months. Patients with post‐transplant recurrence showed a 0.573 log increase in AFP levels after the last chemotherapy session before LT. This was significantly higher than the 0.279 log decrease observed in patients without post‐transplant recurrence (P = .024). Because the AFP response cannot be accurately predicted before each cycle of chemotherapy, it may be appropriate to perform LT when AFP levels do not decrease after the last cycle and before they are found to be elevated again.     

Diagnostic value of lectin reactive alpha-fetoprotein for neoinfantile hepatic tumors and malignant germ cell tumors: preliminary study

BACKGROUND AND PURPOSE: The serum alpha-fetoprotein (AFP) level has been used as a tumor marker for hepatoblastoma, and malignant germ cell tumors in pediatric patients. The AFP has 3 isoforms (L1, L2, L3), and the usefulness of the L3 fraction as a diagnostic marker for the adult hepatocellular carcinoma is well known. However, there are few reports dealing with various pediatric malignant tumors. In the current study, we analyzed the diagnostic value of AFP fractions for pediatric diseases, in particular, those occurring in the neoinfantile period. MATERIALS AND METHODS: From 2003 to 2006, two cases of hepatoblastoma, and 5 cases of germ cell tumor, all of which were neoinfantile, were treated in our department. In our analytical system (LiBASys), the level of the L3 fraction contains the majority of the L2 fraction. The total AFP (ng/mL) level and the L3 fraction (%) were measured to assess the usefulness of the L3 fraction as a diagnostic marker. RESULTS: In all cases of hepatoblastoma and yolk sac tumor, both the total AFP and the L3 fraction were high, either before treatment or in the presence of malignant tumors. Most of the cases of neonatal immature teratoma showed a high total AFP level during the neoinfantile period, however, the L3 fraction was around 10%, and decreased after surgical treatment. Only 1 case of the immature teratoma demonstrated malignant transformation, when the patient was 8 months old. As the total AFP and the AFP-L3 fraction were proportionally elevated, the patient was treated with additional surgical resection and chemotherapy. In the case of neonatal mature teratoma, the L3 fraction was below 0.5%, even when the total AFP level was high. DISCUSSION: Our results indicated that the level of the L3 fraction accurately confirmed the existence, or the malignant potential of hepatic tumor or germ cell tumor. The L3 fraction is useful as a tumor marker during the neoinfantile period.     

Endogenous clearance rate and synthesis of alphafoetoprotein during and after blood exchange transfusion in hyperbilirubinaemic newborns

Plasma alphafoetoprotein (AFP) concentration was monitored in 22 hyperbilirubinaemic infants during blood exchange transfusion at the age of 0-4 days, in order to determine the endogenous clearance rate and demonstrate possible postnatal synthesis of AFP. The rapid fall in plasma AFP during the transfusion to 19 +/- 4% (mean +/- S.D.) was followed by an increase to 81 +/- 15% of the initial concentration; only a 68% rise was expected after the establishment of a new equilibrium between the intra- and extravascular pools of AFP. Repeated transfusion in the same individuals gave similar findings. In two infants the total amount of AFP in these pools increased by 4.0-7.4 mg after the transfusions. The results could be accounted for by postnatal AFP synthesis. Because of the diffusion of AFP from the extravascular compartment to the intravascular pool the actual turnover rate of AFP could not be measured. The estimated magnitude of endogenous turnover rate of AFP, 0.02-0.09 min-1, gave a mean half-life of about 20 min, which is distinctly shorter than that of AFP in plasma (3-5 days); this could be due to either continuous synthesis of AFP or transfer of presynthesized AFP from the liver cells to extracellular pool. We postulate that either the synthesis or the turnover rate of AFP is controlled by the plasma AFP concentration via a negative feedback mechanism.     

Biphasic reduction and concanavalin A binding properties of serum alpha-fetoprotein in preterm and term infants.

Reference values for postnatal serum alpha-fetoprotein (AFP) and concanavalin A (ConA) binding subfractions of AFP in preterm and term infants are presented. Preterm infants had 10-fold higher serum concentrations of AFP than did term infants at birth. The reduction of serum values of AFP was biphasic in both groups and differed significantly between the two groups. The first declining phase continued for approximately 4 months in preterm and for 2 months in term infants, and was related to the degree of prematurity. The AFP values reached adult levels by 12 months in preterm and by 9 months in term infants. The developmental pattern of the carbohydrate moiety of AFP was determined by ConA fractioning. The proportion of the ConA nonreactive subfraction of AFP in preterm and term infants at birth was 6% and 13%, respectively; it increased more rapidly in term than in preterm infants but reached 85% to 95% by the age of 6 months in both infant groups. Our results indicate that the postnatal maturation of AFP synthesis is dependent on gestational age. Malignant recurrences of neonatal sacrococcygeal teratomas were associated with an increase in serum concentration of AFP and a decrease in the proportion of the ConA nonreactive subfraction of AFP.     

Mesenchymal hamartoma of the liver associated with features of Beckwith-Wiedemann syndrome and high serum alpha-fetoprotein levels.

A 5-month-old girl with clinical features of Beckwith-Wiedemann syndrome (BWS), including a repaired omphalocele, an earlobe crease, enlarged adrenal glands, renal size discrepancy, and hyperinsulinemic hyperglycemia, presented with a 1.9-cm liver nodule. Markedly increased serum alpha-fetoprotein (AFP) levels (1,060,000 mg/L), highly suspicious for hepatoblastoma, were detected, and resection of the liver mass was performed. Histologic sections showed features characteristic of a mesenchymal hamartoma of the liver (MHL). No features of embryonal or fetal hepatocellular proliferation or heterologous stromal components were noted. By immunohistochemistry, the hepatocytes expressed AFP, but no nuclear accumulation of beta-catenin was present. Electron microscopy revealed normal, mature hepatocytes. Here we address the diagnostic challenge of the uncommon association of MHL and BWS in the setting of markedly elevated serum AFP levels. In addition, we analyze the unusual pancreatic lesion (focal endocrine adenomatosis) leading to severe hyperinsulinemic hypoglycemia in a patient with possible BWS. We emphasize that MHLs may present with markedly increased serum AFP levels, mimicking hepatoblastomas, and may also be part of the expanding spectrum of findings of BWS.     

High survival rates after liver transplantation for hepatoblastoma and hepatocellular carcinoma

Kosola S, Lauronen J, Sairanen H, Heikinheimo M, Jalanko H, Pakarinen M. High survival rates after liver transplantation for hepatoblastoma and hepatocellular carcinoma.
Pediatr Transplantation 2010: 14:646–650. © 2010 John Wiley & Sons A/S. Abstract: Unresectable malignant liver tumors may be treated by LTx. We evaluated the results of LTx for HB and HCC. All patients transplanted for HB or HCC between 1990 and 2007 were included. Effects of histologic tumor type, primary tumor resection, disease staging, and serum AFP levels at diagnosis and at transplantation on disease recurrence and survival were evaluated. Twelve patients with median age of five (range, 2–16) were transplanted and followed for a median of 11 (2–18) yr. Six patients had HB and six had HCC. At diagnosis, eight patients were staged as PRETEXT III and four patients as PRETEXT IV. Two patients had pulmonary metastases. All patients received neoadjuvant chemotherapy. Median time from diagnosis to LTx was seven (2–133) months. At LTx, none of the patients had radiological evidence of extrahepatic disease, and the median AFP level was 85 (6–15 180) μg/L. No routine chemotherapy after LTx was used.The overall one‐, five‐, and 10‐yr cumulative survival rates were 100%, 80%, and 67%, respectively. Survival was comparable between the two tumor types (4/6 for both). Two deaths occurred secondary to tumor recurrence, one of each tumor type. Both of these patients had an AFP response of <99%. Six of eight patients with primary LTx survived, when compared to two of four transplanted after primary resection. PRETEXT tumor staging had no effect on survival. LTx even without post‐transplantation chemotherapy is an effective treatment option for unresectable HB and HCC with comparable survival. Incomplete AFP response to chemotherapy and primary tumor resection were associated with decreased survival.     

婴幼儿肌纤维瘤病的临床病理学观察

目的 探讨婴幼儿肌纤维瘤病的临床病理特征、诊断及鉴别诊断.方法 12例患儿均以无痛性肿块为主要表现,发病的中位数年龄为4.5个月,病变以头颈部、肩背部、腋下及腿等浅表部位多见;手术切除标本采用HE染色及免疫组织化学技术进行病理学观察,并结合临床资料进行分析.结果 12例患儿都采用了肿瘤完整切除,术后无放疗、化疗,手术切除标本组织学表现:肿瘤大部分由短梭形的、胞质嗜伊红的细胞构成,排列呈螺旋状或束状,肿瘤内血管丰富,呈血管外皮瘤样结构,并见部分血管腔内肿瘤生长,局部见坏死和核异型;免疫组化:肿瘤细胞Vim(+),CD34(+),SMA(+),Des(-),S-100蛋白(-);术后临床平均随访时间约4年,2例出现复发,进行了再次手术,无恶性变发生.结论 婴幼儿肌纤维瘤病是少见的先天性疾病,临床上多表现为体表孤立或多发性无痛性结节,病理学形态复杂,鉴别诊断范围广,本病有一定自限性,局部切除可治愈,但有一定的复发率.     

小儿骶尾部内胚窦瘤的治疗

目的探讨骶尾部内胚窦瘤的治疗和预后。方法回顾性分析1999年1月-2005年12月收治12例骶尾部内胚窦瘤患儿。该组病例术前经B超、CT或MRI检查发现骶尾部肿瘤,经检测血清甲胎蛋白均升高,采用手术切除全部或大部分肿瘤,所有患儿均采用BEP（顺铂十足叶乙甙＋博来霉素）方案化疗。结果该组病例术前经B超或CT检查发现骶尾部肿瘤,经检测血清甲胎蛋白（AFP）均升高,采用手术切除全部或大部分肿瘤,病理证实为内胚窦瘤。患儿均采用BEP（顺铂＋足叶乙甙＋博来霉素）方案化疗。随访6年,生存9例,死亡3例,复发5例。结论以顺铂为基础的联合化疗可改善内胚窦瘤疗效,AFP检测及B超联合应用对小儿内胚窦瘤诊断及复发监测有重要意义,选择恰当的手术方式并辅以联合化疗可提高患儿长期生存率。     

ENDOGENOUS CLEARANCE RATE AND SYNTHESIS OF ALPHAFOETOPROTEIN DURING AND AFTER BLOOD EXCHANGE TRANSFUSION IN HYPERBILIRUBINAEMIC NEWBORNS

Abstract. Teppo, A.-M. and Simell, O. (Department of Paediatrics, University of Helsinki, Helsinki, Finland). Endogenous clearance rate and synthesis of alphafoetoprotein during and after blood exchange transfusion in hyperbilirubinaemic newborns. Acta Paediatr Scand, 69: 491, 1980. —Plasma alphafoetoprotein (AFP) concentration was monitored in 22 hyperbilirubinaemic infants during blood exchange transfusion at the age of 0–4 days, in order to determine the endogenous clearance rate and demonstrate possible postnatal synthesis of AFP. The rapid fall in plasma AFP during the transfusion to 19±4% (mean ±1 S.D.) was followed by an increase to 81±15% of the initial concentration; only a 68% rise was expected after the establishment of a new equilibrium between the intra- and extravascular pools of AFP. Repeated transfusions in the same individuals gave similar findings. In two infants the total amount of AFP in these pools increased by 4.0–7.4 mg after the transfusions. The results could be accounted for by postnatal AFP synthesis. Because of the diffusion of AFP from the extravascular compartment to the intravascular pool the actual turnover rate of AFP could not be measured. The estimated magnitude of endogenous turnover rate of AFP, 0.02–0.09 min^-1, gave a mean half-life of about 20 min, which is distinctly shorter than that of AFP in plasma (3–5 days); this could be due to either continuous synthesis of AFP or transfer of presynthesized AFP from the liver cells to extracellular pool. We postulate that either the synthesis or the turnover rate of AFP is controlled by the plasma AFP concentration via a negative feedback mechanism.     

Alpha-fetoprotein levels correlate with the pathologic grade and surgical outcomes of pediatric retroperitoneal teratomas

Introduction  Retroperitoneal teratomas (RTs) are rare neoplasms that comprise 2–5% of all pediatric teratomas and 10% of all pediatric retroperitoneal tumors. Although alpha-fetoprotein (AFP) is a recognized tumor marker for immature teratomas, its prognostic value in neonates and children with RTs is unknown due to physiologic elevation of this marker. The aim of this study was to determine whether AFP level correlates with pathologic grade of pediatric RT and patient outcome. Methods  A retrospective analysis of all RTs seen at our institution over a 20-year period was performed. Thirteen patients with RTs were identified; their AFP levels were correlated with tumor grade, patient age, and outcome. Results  Twelve patients were less than 1 year of age at diagnosis. There were nine patients with immature, and four with mature RTs. All patients with mature RTs had AFP levels within the normal range, while eight out of nine patients with immature RTs had elevated AFP levels. A statistically significant elevation in AFP was noted in grade III RTs compared with mature RTs (P = 0.036), regardless of age. Major complications including vascular injury, sepsis, and death were more prevalent in high-grade RTs. Conclusion  We conclude that AFP levels correlate with tumor immaturity and predict operative morbidity even in patients less than 2 months of age. Despite the increased morbidity associated with surgical excision of high-grade RTs, complete resection results in long-term disease-free survival in the majority of infants with this rare pediatric neoplasm.