Presacral teratoma presenting with congenital urinary ascites

Congenital teratomas occur most frequently in the sacrococcygeal region. Most grown into a large perineo-sacral swelling that is conspicuous externally. Infrequently, the neoplasm is contained almost entirely within the pelvis in the presacral space. Congenital urinary ascites is observed in patients with obstructive uropathy; posterior urethral valves in a newborn is one of the most prominent causes of urinary ascites. We report a case of presacral teratoma leading to rupture of the urinary bladder due to outflow obstruction and causing urinary ascites. The ascites was drained, the bladder was repaired, and the teratoma was successfully excised. A review of the literature did not reveal any similar case. Accepted: 12 November 1996     

Recurrent yolk sac tumor following resection of a neonatal immature gastric teratoma

Gastric teratomas are very rare and usually benign. Only a few cases of gastric teratomas with malignant components have been reported. This report describes recurrence of a yolk sac tumor following resection of a neonatal immature gastric teratoma. Gastric teratoma recurring as a malignant lesion has not been previously reported. Recurrence of immature gastric teratomas should be considered, and a periodic follow-up check with alpha-fetoprotein level should be mandatory.     

The growing teratoma syndrome

Growing teratoma syndrome is defined as enlarging masses of mature teratoma following chemotherapy for malignant nonseminomatous germ-cell tumors. Typically, there is associated normalization of initially elevated serum tumor markers. We describe clinical and imaging findings in a case of growing teratoma syndrome originating from immature teratoma of the ovary in a 12-year-old girl. Familiarity with this unusual entity is important to avoid confusion with advancing malignancy.     

The management of prenatally diagnosed sacrococcygeal teratoma

Two of our own and 18 other reported cases with prenatally diagnosed sacrococcygeal teratoma that were delivered by cesarean section are reviewed. Six infants delivered at a gestational age of from 25 to 32 weeks died perinatally. Causes of death were immaturity, intratumoral hemorrhage, and tumor rupture. It is concluded that an attempt should be made to continue pregnancy until 32 weeks' gestation and that after this age infants should be delivered by elective cesarean section as soon as possible. Offprint requests to: H. Ikeda     

Mechanisms of sudden death in patients with congenital teratoma

Patients with congenital teratomas usually have a favourable prognosis provided that adequate surgical excision is performed early in life and that no malignancy is detected within the excised tissue. Three patients who died suddenly and unexpectedly as a result of the critical locations of their congenital teratomas demonstrate an alternative outcome. The clinicopathological features and mechanisms of death are described. A 4.8-kg, term boy failed to establish normal respiration and died after 1 h. At autopsy the chest cavity was almost completely occupied by a grossly distended pericardial sac that contained a large teratoma arising from the root of the aorta. A 31-week-gestation boy who was delivered by caesarean section for fetal distress died after 1 h of respiratory distress due to upper airway obstruction caused by a large anterior cervical teratoma. A 1.7-kg, 36-week-gestation girl who was admitted in a moribund state died during attempted surgical removal of a large sacrococcygeal teratoma that had caused marked haemorrhage during delivery. Antenatal diagnosis had not been made in any of the patients. These cases demonstrate that sudden death due to variable mechanisms such as cardiorespiratory compromise, airway obstruction, pulmonary hypoplasia, and traumatic haemorrhage should always be considered as possible complications of congenital teratoma that may occur prior to definitive surgery. Correspondence to: R. W. Byard     

Successful surgical management of a prenatally diagnosed intrapericardial teratoma

Congenital intrapericardial teratoma is a rare, usually benign tumour frequently associated with massive pericardial effusion, cardiac compression, and severe cardiorespiratory distress shortly after birth. Surgical removal is not only curative but also potentially lifesaving because these lesions often become fatal if not promptly excised. We present a case of a newborn with a huge intrapericardial teratoma diagnosed in utero. After normal delivery the infant underwent surgical removal and has had clinical follow-up for more than 11 months.     

Late urologic sequelae after surgery for congenital sacrococcygeal teratoma

Forty-five patients treated for congenital sacrococcygeal teratoma were examined for late urologic outcome. The follow-up ranged from 4 to 42 (median 22) years. Only 8 patients experienced subjective urinary complaints. Sonography of the urinary tract revealed unilateral hydronephrosis in 2 patients, an ectopic kidney in 1, and a severely scarred kidney in 1; 1 patient had asymptomatic bacteriuria. Urodynamic screening using uroflowmetry was abnormal in 35 patients (78%). The risk of significant postoperative complications and subsequent late sequelae was greatest among patients with intrapelvic extension of the primary tumor.Supported by the Foundation for Pediatric Research, Helsinki, Finland     

Massive congenital intracranial teratoma: a report of two cases

Although intracranial teratoma is a well-recognized entity in the differential diagnosis of pediatric brain tumors, massive congenital intracranial teratoma replacing the cerebral hemispheres of a neonate has seldom been reported. We describe two such instances that histologically exhibit predominantly neuroepithelial differentiation. In 1 case serial prenatal ultrasonography revealed ventricular dilatation prior to identification of the lesion. Theories of pathogenesis are briefly discussed.     

胎儿骶尾部畸胎瘤的预后评估研究进展

骶尾部畸胎瘤(sacrococcygeal teratoma,SCT)是并不少见的胎儿及新生儿肿瘤,在出生活婴中发病率是1/40 000～1/35 000,男女比例1∶3～1∶4.通常认为SCT胎儿出生后经手术治疗预后良好,但据文献报道,产前诊断为SCT的胎儿死亡率却高达16％～63％.因此,SCT胎儿的预后评估指标和产前诊疗策略是当前研究的重点.本文对近年来SCT胎儿预后评估的研究进展进行了综述.肿瘤囊实性评分、TFR、STV∶ HV、STVI、肿瘤生长速度与CVPS等是SCT胎儿预后评估的有意义指标,这些指标各有利弊.在临床上,建议同时评估肿瘤囊实性比例与TFR这2个简便易行的指标,有条件的产前诊断中心也可选测STV∶ HV或STVI任一个指标.复查过程应监测肿瘤生长速度.CVPS可用于评估SCT胎儿心力衰竭的发生及进展,以决定干预时机.若SCT胎儿出现进展性高输出型心衰,孕28周前首先考虑胎儿手术,孕28周后可予治疗性早产.目前,SCT胎儿的预后评估尚处于探索阶段,本文所述产前策略亦并不完善,有待更多医学工作者投入研究.     

Cervical teratoma: prenatal detection and management in the neonate

Routine prenatal ultrasonography in a 34-year-old woman at 28 weeks gestation, revealed a large right cervical mass in the fetus. Subsequent sonographies showed no increase in size of the mass, but polyhydramnios developed. Cesarean section was performed at 38 weeks. At birth a very right cervical mass was present which on palpation was partly cystic and partly solid. CT scan revealed heterogeneous tissue with numerous irregular calcifications. Because of increasing difficulty with breathing and swallowing the tumor was removed surgically. The microscopic findings were compatible with teratoma. The postoperative course was uneventful. Although malignancy has been described, mainly in adults, histologically cervical teratoma is a benign tumor. The importance of its recognition in utero is stressed, as this allows the planning of obstetrical and surgical strategy.     

Sacrococcygeal teratoma: an analysis of 37 cases

Thirty seven cases of sacrococcygeal teratoma were treated in Children Hospital, B.H.U. from 1974 to 1982. Thirty-one of these were benign and 6 were malignant. In thirty two cases a swelling was present since birth and in the remaining the swelling appeared later and these all were malignant. Most of them (70%) were below one year on admission. Usual presentation was a swelling on the sacrococcygeal region (94·6%) with an intrapelvic extension. Immature neural elements were present only in malignant cases. Benign treatomas were treated by early total surgical excision; recurrence was noted in one case only. Malignant cases however showed poor results following surgical excision and post-operative radiotherapy and chemotherapy.     

Fetus in Fetu: a Fetiform Teratoma

Examination of a retroperitoneal fetus in fetu, diagnosed preoperatively, revealed previously unreported histologic findings in the vascular pedicle and membranous capsule that indicated that these structures are not “umbilical cord” or “amnion.” These findings include nervous innervation of both structures and a well-defined lamina elastica interna and vaso vasorum in the artery of the vascular pedicle. Thus, strong support is provided for the theory that many examples of fetus infetu are remarkably complex, well differentiated, highly organized teratomas. Additional arguments that favor the identity of fetus in fetu and teratoma are presented.     

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.     

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     

Management of pineal non-germinoma germ cell tumor with residual teratoma and normal alpha-fetoprotein

A 16-year-old white male presented with multiple abnormal extraocular movements secondary to an enhancing pineal tumor. Subtotal resection of the lesion revealed a mixed malignant germ cell tumor. The pre-operative serum alpha-fetoprotein (AFP) was markedly elevated at 155 IU/L. The patient subsequently received radiotherapy and adjuvant chemotherapy consisting of cisplatin rotating monthly with vincristine and cyclo-phosphamide, with dramatic tumor regression and return of AFP to normal. Eighteen months later the persistence of a substantial tumor mass despite a normal AFP raised concern for residual active tumor. Histological examination of the resected lesion revealed benign teratoma and fibrous tissue. Repeat surgical intervention may contribute to the management of mixed malignant germ cell tumors, which demonstrate a persistent mass following an initial response to treatment. © 1994 Wiley-Liss, Inc.     

新生儿先天性梅毒诊断准确性的回顾性分析

目的探讨制定统一先天性梅毒(CS)诊断标准的必要性。方法回顾分析2016年1月—2020年2月因母亲梅毒感染而入院的85例新生儿的临床资料,分别采用性传播疾病管理指南(2015)/2015美国儿科学会感染委员会报告(美国指南)、2014年欧洲梅毒管理指南(欧洲指南)和2015年版预防艾滋病、梅毒和乙肝母婴传播工作实施方案(中国方案)进行评估诊断,比较上述三种标准诊断CS的一致性。结果将确诊/高度疑似或疑似病例归入CS,美国指南共诊断32例,欧洲指南诊断40例,中国方案诊断15例,三个标准共诊断40例,其中三个标准均符合者14例。美国指南和中国方案诊断病例均包含于欧洲指南诊断病例,美国指南和中国方案诊断病例重复例数为14例。10例梅毒螺旋体(Tp)试验阳性+长骨X线异常患儿中,7例因母亲孕前或孕期接受规范治疗未被美国指南诊断,其余仅3例因母孕期不规范治疗而被美国指南诊断,10例均未被中国方案诊断。Tp试验阳性+Tp-IgM阳性1例未被美国指南诊断,但均被其他两个标准所诊断。Tp试验阳性+其他指标阴性且母孕期不规范治疗15例未被中国方案诊断,但均被其他两个标准诊断。美国指南与欧洲指南、美国指南和中国方案一致性较强,加权κ值分别为0.611和0.705,而欧洲指南与中国方案一致性一般,加权κ值仅为0.304。结论临床上制定统一的CS诊断标准非常必要。     

Sacrococcygeal teratoma in Northeastern Nigeria: 18-years experience

Although sacrococcygeal teratomas are rare, there have been several reports from various parts of Nigeria, outside the Northeast region. This report reviews the experience with this tumour from the northeastern part of Nigeria. A retrospective study of 21 children with histologically confirmed sacrococcygeal teratoma managed in 18 years. Over the 18-year-period, 21 histologically confirmed cases of sacrococcygeal teratoma were seen—80% were girls. Thirteen (60.9%) presented during the neonatal period. Eighteen patients presented with benign sacrococcygeal teratoma while three patients and a recurrence from an initial benign tumour had malignant sacrococcygeal teratoma. All malignant tumours had a significant presacral component. There were associated congenital malformations in 3 (14.3%) of the patients. All the benign tumours were resectable. Using the American Academy of Pediatrics (Altman’s) classification (Altman et al., J Pediatr Surg, 9:389–398, 1974), 13 patients were type I, 5 patients type II, 3 patients type Ill and none was for type IV. The commonest post-operative complication was wound infection in six patients. Others were wound dehiscence in three and post-operative diarrhoea in two patients. Three deaths were recorded in this series. The size of mass greater than 5 cm did not correlate with the incidence of post-operative complications (P=0.367). The post-operative follow-up was very poor; but there was a recurrence in a girl, two-years after the initial surgery. Our study revealed that most of our patients with sacrococcygeal teratoma present early to hospital with benign lesions and the immediate post-operative results were excellent. However, the follow-up was poor as such long-term post-operative results are difficult to study. It is to be recommended that sacrococcygeal teratoma presenting early to hospital should have prompt surgical intervention.