甲胎蛋白异质体在儿童肿瘤中的研究进展

甲胎蛋白(alpha-fetal protein,AFP)是一种糖蛋白,自发现伊始,甲胎蛋白一直在相关儿童肿瘤(肝母细胞瘤、卵黄囊瘤、胰母细胞瘤等)的临床诊断中起到重要作用,但诊断作用并不完美,甲胎蛋白有三种异质体(AFP-L1,AFP-L2,AFP-L3),近年来,对其作用机制及功能的研究愈发加深.     

Treatment of a stage I testicular yolk sac tumor with vascular invasion

There is controversy concerning the treatment of stage I yolk sac tumor of the testis, particularly of those with histological factors that indicate a high risk of relapse. Usually orchiectomy alone is sufficient and adjuvant chemotherapy is unnecessary. Retroperitoneal lymphadenectomy is indicated for patients with persistently high alpha-fetoprotein. Once recurred, treatment at that time is thought to be curative. However, postoperative chemotherapy may be necessary for patients with a tumor expressing histological factors that predict possible relapse. In this paper we report on a case of a 2 year old boy whose tumor invaded the testicular veins. The patient suffered from recurrent disease but was successfully treated by chemotherapeutic regimens including cisplatin and retroperitoneal lymphadenectomy. The importance of the histological factors in making a decision on the treatment strategy for stage I testicular yolk sac tumor is discussed.     

Elevated serum alpha-fetoprotein in Wilms tumor may follow the same pattern of other fetal neoplasms after treatment: evidence from three cases

Alpha-fetoprotein (AFP) is a common tumoral marker in pediatric neoplasms; increased serum levels are usually encountered in tumors arising from tissues producing AFP during fetal life. However, elevation of such protein is rarely found in patients with Wilms tumor (WT). Three patients with WT and elevated serum AFP were studied over the course of the disease. One patient had left WT with invasion of aorto-caval lymph nodes and lung metastases. The second patient was referred to our center for abdominal recurrence of bilateral WT whereas the third showed right WT with inferior vena cava thrombosis. AFP levels demonstrated a trend parallel to decrease/increase of tumor size, with further elevation in patients with metastases. Elevated AFP serum levels in patients with WT could be related to peculiar histological features and serial dosage might be a useful diagnostic and prognostic test.     

Living donor liver transplantation in a pediatric patient with preexisting yolk sac tumor

There is ongoing discussion regarding the indications and timing of LT for patients with a preexisting extrahepatic malignancy. We herein report a pediatric case that underwent LDLT after therapy for YST. The patient, a 13‐year‐old female with biliary atresia, had undergone portoenterostomy at 2 months of age. She developed a left ovarian tumor with a high serum alpha‐fetoprotein concentration at 10 years of age. She underwent left oophorectomy and was diagnosed with ovarian YST (Stage I). After surgery, hepatopulmonary syndrome progressed gradually. She was examined carefully and exhibited no findings to suggest the recurrence of YST. We decided to perform LDLT at 3 years and 6 months of age after the surgery for YST. The patient is currently alive and doing well without recurrence of YST at approximately 2 years after transplantation. There is no significant difference between the recurrence rate of preexisting extrahepatic malignancy and the incidence of de novo malignancy if specific cases are selected. The indications and period from surgery for preexisting extrahepatic malignancy to LT should thus be determined according to the type and stage of cancer.     

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.     

Endodermal Sinus Tumor of the Parotid Gland in a Child

We report a case of a 16-month-old girl with a primary endodermal sinus tumor (EST) of the parotid gland. The girl was admitted to the hospital with a left side cervical tumor with a quick growth (within 3 wk). The tumor was surgically resected. The gross examination showed a 7.0-cm hemorrhagic and fragmentized mass. Diagnosis of an EST was established on the hematoxylin and eosin-stained slides from the resected material. Elevated serum alpha-fetoprotein (AFP) levels were detected and follow-up examinations of the patient did not show ESTs in other locations or other manifestations due to the tumor. The patient received chemotherapy after the surgery and remained alive without evidence of disease at the time of this writing (2 years after the diagnosis). As far as we know, this is the second report in the literature of a parotid glands EST.     

Is yolk sac tumor related to the pathophysiology of low birthweight?

An 8‐year‐old Japanese girl was admitted with an ovarian yolk sac tumor. Regarding birth history, the patient had been delivered by cesarean section at 25 weeks of gestation with a birthweight of 711g. She had required neonatal intensive care including oxygenation, various medications, and tests. After surgery and chemotherapy, there was no recurrence for 2 years, at the time of writing. Yolk sac tumor, which is a malignant germ cell tumor, is rare in children. Although the cause and risk factors are unclear, it has been reported that malignant germ cell tumors in childhood have been associated with pathophysiology at birth. Given that premature infants are more likely to survive due to advances in perinatal care, it is expected that such cases will increase in the near future. We suggest that children born prematurely require careful follow up.     

Malignant transformation of germinoma 14 years after onset: Favorable efficacy of oral etoposide

We report the case of a 19‐year‐old woman with a highly malignant intracranial germ cell tumor (GCT) that developed 14 years after treatment for neurohypophyseal germinoma. Magnetic resonance imaging (MRI) showed a large neurohypophyseal mass and a synchronous lesion in the pineal region. Plasma α‐fetoprotein was elevated to 3038 ng/mL. Although the tumor shrank and tumor marker levels normalized after chemotherapy and craniospinal irradiation, treatment was switched to oral etoposide for the residual tumor because of adverse events. MRI after oral etoposide introduction showed additional tumor shrinkage for 27 months after the onset of the second tumor. To the best of our knowledge, this is the longest interval between germinoma onset and the development of highly malignant recurrent GCT to be reported in the English‐language literature. Oral etoposide prevented regrowth of the GCT, which has a poor prognosis, and decreased the size of the residual tumor.     

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.     

Ovarian germ cell tumors in children: a clinical study of 66 patients

BACKGROUND: Ovarian germ cell tumors are rare in childhood. The aim of this study is to review clinical presentation, management, and outcome in a two-center series of girls with ovarian germ cell tumor. PROCEDURE: The records of 66 patients (median age 9 years) with histologically proven ovarian germ cell tumor (either benign or malignant), treated over a 44-year-span, were reviewed. RESULTS: Pain and an abdominal mass were the most frequent symptoms. The tumors were right-sided in 35, left-sided in 28, and bilateral in 3. Most patients (52) were stage I, 4 were stage II, 6 stage III, and 1, with liver metastases, stage IV. Sixteen patients had an emergency operation for tumor torsion. Unilateral salpingo-oophorectomy was the most frequently performed procedure (n = 46), and ovarian-sparing tumorectomy was performed in 9 patients (one bilaterally). Histologically, teratomas were found most frequently (mature: 45, immature: 9), followed by mixed tumors (n = 7), yolk sac tumors (n = 3), dysgerminoma (n = 2), gonadoblastoma (n = 2), and embryonal carcinoma (n = 1). Surgical removal of the tumor with or without the ovary and/or adnex was the sole treatment in 55 patients, chemotherapy was administered in 10 and radiotherapy + chemotherapy in one. Intra-operative spillage of tumoral fluid occurred in six; this did not influence outcome in five. Recurrence was observed in three patients. Two patients, with malignant disease, died. The 64 survivors are now between 8 months and 44 years after treatment. CONCLUSIONS: With a recurrence rate of 4.5% and a mortality rate of 3%, this series confirms the excellent prognosis for girls with ovarian germ cell tumor (GCT).     

小儿颅脑损伤急性期血糖变化的临床意义

目的　探讨小儿颅脑外伤急性期血糖变化与格拉斯哥昏迷评分 (GCS)和预后 (GOS)的关系 ,以及高血糖的处理。方法　测定并分析 1998年 1月至 2 0 0 2年 1月期间收治并从中选择的 14 5例颅脑外伤患儿的急性期血糖值。结果　按GCS评分分为三组 (GCS 3～ 8分 2 7例 ,9～ 12分 2 7例 ,13～ 15分 91例 ) ,入院时血糖平均值分别为 (16 .2 6± 2 .33)mmol/L、(10 .4 7± 2 .15 )mmol/L、(5 .92± 1.2 1)mmol/L ;按预后分为三组 (良好 12 6例 ,差 13例 ,死亡 6例 ) ,其入院时血糖值分别为 (7.73±3.2 5 )mmol/L、(12 .2 5± 2 .99)mmol/L、(2 2 .5 4± 3.97)mmol/L。随机选取 2 8例高血糖患儿予控制糖入量、使用胰岛素等降糖处理 ,并在伤后≤ 6h、2 4h、4 8h作血糖动态监测 ,血糖值分别为 (12 .70±3.0 2 )mmol/L、(8.34± 2 .71)mmol/L、(5 .6 3± 1.75 )mmol/L ,致残率、死亡率显著下降 ,预后良好。结论　血糖测定有助于评价小儿颅脑损伤的严重程度及预后 ,积极的综合治疗可改善高血糖患儿的预后     

Imaging of pediatric liver tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper

Primary hepatic malignancies are relatively rare in the pediatric population, accounting for approximately 1%–2% of all pediatric tumors. Hepatoblastoma and hepatocellular carcinoma are the most common primary liver malignancies in children under the age of 5 years and over the age of 10 years, respectively. This paper provides consensus-based imaging recommendations for evaluation of patients with primary hepatic malignancies at diagnosis and follow-up during and after therapy.     

A case of malignant rhabdoid tumor mimicking yolk sac tumor

Malignant rhabdoid tumors (MRTs) are rare, highly aggressive embryonal neoplasms caused by biallelic alterations of the SMARCB1 gene. MRTs may occur in any soft tissue, but extracranial extrarenal MRTs are extremely rare. Diagnosis of MRTs in unusual locations and with an uncharacteristic cytomorphology that mimics other tumors is difficult. This was an atypical case of MRT in a 15‐year‐old female with tumors that closely resembled yolk sac tumors. It was extremely challenging to diagnose the tumors without confirming the SMARCB1 status.     

Hepatic hematoma in a neonate with a high level of alpha-fetoprotein

Hepatic hematomas in neonates are uncommon lesions. When they are large or subcapsular in location, they can rupture with clinical signs of hemoperitoneum. We report a case of subcapsular hepatic hematoma (SHH) associated with a high level of alpha-fetoprotein (AFP), for which diagnosis was made with conservative management, following up with the reduction in size at ultrasound examination and the reduction of the level of AFP.     

Immature ovarian teratoma with peritoneal gliomatosis and elevated serum alpha-fetoprotein associated with a second mature teratoma

A 7-year-old girl had a huge immature grade 2 teratoma of the left ovary with peritoneal gliomatosis and elevated serum alpha-fetoprotein (AFP). The serum AFP level reached 3543 ng/ml and returned to normal after left salpingo-oophorectomy and chemotherapy. Twenty-two months later, a second mature teratoma was removed from the left subdiaphragmatic region. The AFP level had been within the normal range and the patient had been symptom-free for 3 years following the previous operation. AFP was positive in the tubular epithelium of the immature tissues on immunohistochemical study. Offprint requests to: A. Hokama