Sacrococcygeal teratoma

Abstract Eight cases of sacrococcygeal teratoma are described. Seven patients were diagnosed at birth, and one presented at 3 1/2 months of age with urinary retention. Two patients had associated congenital anomalies (arachnoid cyst, external angular dermoid and bifid sacrum), while one had a family history of sacrococcygeal teratoma. All had the tumour removed together with the coccyx as soon as the diagnosis was made. None had recurrence of the tumour.     

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.     

Long-term outcomes of surgery for malignant sacrococcygeal teratoma: 20-year experience of a regional UK centre

Background  The timing of surgery for malignant sacrococcygeal teratoma is controversial. The long-term outcomes and complications of surgery for this rare tumour are presented. Methods  All cases of malignant sacrococcygeal teratoma in the 20-year period 1987–2006 were identified and the case notes retrieved. The age at diagnosis, investigations, presentation, type of surgery, early complications, recurrence rates, long-term complications and outcomes were recorded. Results  Twelve patients (three males, nine females) were identified. Mean age at presentation was 20.8 months (range: 12–39 months). All had the Carboplatin–Etoposide–Bleomycin chemotherapeutic protocol. The average time of follow-up was 10.6 years (range: 1–17 years). Ten patients had excision of their tumours following chemotherapy, whilst two patients had excision prior to chemotherapy. Two patients had recurrence of their tumours. There was one death (8%), which was due to disseminated metastasis. The other 11 children were all well at the last follow-up. Conclusion  Surgery for malignant sacrococcygeal teratoma is safe and has a low complication rate. The long-term outcomes are favourable with minimal side effects.     

Management of benign sacrococcygeal teratoma: reducing mortality and mornidity

Between 1954 and 1987, 43 children with sacrococcygeal teratoma have been seen in our institution. There was 1 stillbirth, 4 neonates (9%) died during transfer, and there were 2 operative deaths (4.5%). Median age at operation was 4 days (range 1 day to 3 years). The survivors have been followed up for a median of 5.5 years (range 5 months to 32 years). Four patients have faecal and/or urinary incontinence. One developed a malignant teratoma subsequent to excision of a benign tumour. Since 1982, 4 patients have been diagnosed by antenatal ultrasound scanning, there have been no deaths related to sacrococcygeal teratomas either during transit or at operation, and morbidity has been minimal. Increasing use of antenatal ultrasound and improved neonatal anaesthetic and surgical care are the main contributory factors.     

Head and neck teratomas in children

A retrospective review of seven patients with teratomas of the head and neck treated at out center over the past 5 years, which represented 2% of all teratomas (sacrococcygeal, ovarian, and retroperitoneal) seen over the past 20 years, was performed. After investigation to exclude associated anomalies, all but one of the children underwent surgery for removal of the tumour. All excised tumours were subjected to histopathological examination to confirm their teratomatous nature. The patients were followed up at regular intervals for up to 4 years. The patients' ages ranged from newborn to 2.5 years. There were three cervical, two oral, and two skull teratomas. The youngest patient had a cervical teratoma with respiratory compromise, requiring tracheal intubation. All but one patient (skull teratoma) had excision of the tumours with a satisfactory outcome. Histopathological examination of the excised tumours showed mature tissue from the three germinal layers in all specimens. Specific components included glandular epithelium, keratinising epithelium, and muscle fibres. Follow-up did not show any recurrence in the operated children. The three with cervical teratomas had normal levels of thyroid hormone post-operatively. Head and neck teratomas in children are mostly benign lesions amenable to curative excision. In cervical teratomas airway management takes priority. Accepted: 8 November 1999     

Familial triad of anorectal,sacrococcygeal, and presacral anomalies that includes sacrococcygeal teratomas

We present two Australian families with the triad of anorectal, sacrococcygeal, and presacral anomalies. In one family four members had sacral deformities, three of which were associated with presacral masses and anal stenosis. The presacral masses were: one meningocoele combined with a teratoma; one teratoma; and the third remains undiagnosed and unresected at the patient's request. In the second family, one child had an infected presacral teratoma with a small sacral defect and the father had a similar sacral defect with computed tomographic evidence of a presacral meningocoele and teratoma. The presence of anal stenosis and constipation with a sacral defect should raise the possibility of a presacral mass. The mass may take the form of a meningocoele, teratoma, enteric cyst, or any combination of these. Once detected, other family members should be investigated.     

Evaluación funcional y estética de los teratomas sacrococcígeos. No todo termina con la cirugía

Introduction

Sacrococcygeal teratoma is the most common solid neonatal tumour. The improvement in survival has meant that postoperative sequelae can be diagnosed and treated. The aim of this article is to evaluate the long-term outcomes of patients treated in our centre.

Somatic malignant transformation in a sacrococcygeal teratoma in a child and the use of F18FDG PET imaging

A 6-year-old female presented with a subcutaneous sacral mass. Biopsy revealed an adenocarcinoma most likely arising from a sacrococcygeal teratoma (SCT). CT imaging revealed a massive tumour consistent with SCT. F¹⁸FDG Positron Emission Tomography (PET) scan confirmed marked metabolic activity in the tumour mass and regional lymph node involvement. After chemotherapy repeat CT and PET studies revealed a poor response but no evidence of peritoneal or distant metastases. Radical abdomino-pelvic and gluteal surgery was performed with removal of the entire tumour confirmed as a moderately differentiated adenocarcinoma arising in an immature teratoma. Follow up imaging including PET scanning 5 months after her surgery revealed widespread peritoneal, hepatic and pulmonary metastases. Somatic malignant transformation of an SCT in a child of this age has not been previously reported.     

Malignant sacrococcygeal teratoma: improved survival with chemotherapy

Between 1954 and 1985, 15 cases of malignant sacrococcygeal teratoma were treated in our institution. Median age at presentation was 20 months (range 9–46 months); there were 14 females and 1 male. Six cases presenting prior to 1970 were treated without chemotherapy with a median survival of 2 months (range 1–7 months). Five patients treated from 1971 to 1979 had tumour excision plus vincristine, actinomycin D, and cyclophosphamide (VAC). Three received radiotherapy in addition and median survival was 11 months (range 0–31 months). Four patients presenting since 1980 had chemotherapy alone after tumour excision. 1 receiving VAC and adriamycin and the remaining patients cisplatinum, bleomycin, and VP16. All these are alive and disease free (median follow-up 27 months; range 15–96 months). It is concluded that initial surgery aimed at complete excision followed by aggressive chemotherapy gives good results. Offprint requests to: D. C. S. Gough     

Sacrococcygeal tumors in infancy and childhood; a retrospective histopathological review of 85 cases

This study retrospectively examines the spectrum of sacrococcygeal tumors reported in a tertiary paediatric pathology department during a 15-year period. There were 85 sacrococcygeal tumors identified in total, including 79 (93%) sacrococcygeal germ cell tumors, of which 62 (78%) were benign, whereas 17 (22%) contained malignant yolk sac tumor elements. The median age at examination in cases with malignant elements present was significantly greater than in those with benign sacrococcygeal teratoma only (median 2 years, range birth--3 years versus median 1 week, range birth--10 years, respectively; p < .01). Of the 85 cases of total sacrococcygeal lesions 6 (7%) represented pathologies other than sacrococcygeal teratoma, including one case each of neuroblastoma, ganglioneuroma, myxopapillary ependymoma, primitive neuroectodermal tumor, lipomatous tumor, and unclassifiable inflammatory tumor. Of these 6 cases 3 were malignant (50%) compared with 17 of the 79 cases of sacrococcygeal germ cell tumors (22%; Z =1.59, p = .08). The median age in the group of non-germ-cell sacrococcygeal masses was 3 years (range 5 months to 13 years).     

Gastroschisis with extracorporeal liver and stomach associated with sacrococcygeal teratoma and limb abnormalities

Gastroschisis is the herniation of abdominal viscera through a paramedian abdominal wall fusion defect without involvement of the umbilical cord. Evisceration usually contains intestinal loops and has no surrounding membrane. Rarely, herniation of other major viscera such as stomach and liver occurs, which makes the prognosis worse. Gastroschisis is usually not associated with sacrococcygeal teratoma. In the present report, a very rare case of gastroschisis associated with sacrococcygeal teratoma is described. The gastroschisis had complete evisceration of the stomach, bowel and extracorporeal liver. A large sacrococcygeal mass was located on the posteroinferior part of the trunk and gluteal region, and was completely external. The fetus also showed a malrotated lower limb and talipes equinovarus.     

Paediatric gastric teratoma.

AIM: This paper reviews our experience with patients presenting with gastric teratoma treated over an eighteen-year period. This rare paediatric tumour has an excellent prognosis after curative resection. METHODS: There were seven patients, all males. Investigations included a haematological profile, plain radiography and ultrasound of the abdomen. All patients were operated and the specimens subjected to histopathological examination. RESULTS: The age at presentation ranged from 1 month to 7 years. Two patients presented with melaena and the rest with progressively increasing abdominal mass. Our oldest patient had a massive tumour occupying nearly the whole abdomen. None of the children had metastases and none of the lesions had invaded adjacent structures. The tumours could be completely resected and all patients had an uneventful recovery. Histology of the tumours showed mature elements arising from the three germ layers in 6 children, one child had an immature teratoma. The average follow-up was 3 years (range 2 - 6 years). None of the patients has had a recurrence or any other problem. CONCLUSIONS: Gastric teratoma is a rare tumour but amenable to curative resection with an excellent prognosis.     

Sacrococcygeal Teratoma in Nigerian Children

Two-thirds of all teratomas seen in 31 Nigerian children at Ibadan were situated in the sacrococcygeal region. About one-quarter of all the sacrococcygeal teratomas were malignant as shown on radiological and pathological examinations. The malignant tumours had papillary carcinomatous pattern, probably derived from the choroid plexus. The clinicopathological features of one patient with malignant change is described to illustrate the biological behaviour of this tumour. Malignancy in all the sacrococcygeal tumours appears to be associated with growth of the tumour into the pelvic cavity and metastasis initially to inguinal lymph nodes and later to distant sites. Inferior venacavogram can be of great value in defining the extent of the intrapelvic mass and the displacement of the genito-urinary organs, particularly when surgery is contemplated.     

Sacrococcygeal teratoma: results of a retrospective multicentric study in Belgium and Luxembourg.

Eighteen patients, operated upon for sacrococcygeal teratoma in 7 different centres in Belgium and Luxembourg between 1992 and 1996, were reviewed. From an epidemiological point of view, this series compares very well to others. Although excellent results were obtained, with all patients surviving, some imperfection in diagnosis, timing of delivery and of operation, and in operative technique was observed. Therefore, it is stated that for optimal treatment of sacrococcygeal teratoma to be achieved, these cases should be treated in just a very few centres of neonatal surgery.     

Huge Fetal Sacrococcygeal Teratoma with a Completely Formed Eye and Intratumoral DNA Ploidy Heterogeneity

Preterm infants and newborns with sacrococcygeal teratomas are at high risk for prenatal and perinatal complications. The prognosis depends on size and histology of the tumor, degree of prematurity, associated malformations, route of delivery, and prompt and complete surgical removal. Virtually any tissue can be present in a sacrococcygeal teratoma, but to date, ocular lens has been documented only as lentinoids (lens-like cells), whereas flow-cytometric data have been variably interpreted. We describe a case of a sacrococcygeal teratoma in an infant of 29 weeks gestational age that is remarkable for the weight (4500 g), the presence of a completely formed eye, and intratumoral DNA ploidy heterogeneity. Received October 10, 1997; accepted January 21, 1998.     

骶尾部畸胎瘤术后复发高危因素分析

目的 探讨和分析引起骶尾部畸胎瘤术后复发的高危因素.方法 回顾性分析2003年1月至2012年12月收治的107例骶尾部畸胎瘤患儿的临床资料,使用单因素和多因素危险度Lo-gistic回归法,从Altman分类、手术年龄、肿瘤大小、肿瘤是否完整切除、术中肿瘤是否溃破以及破溃成分、病理类型等方面分析肿瘤复发的高危因素.结果 肿瘤复发共16例,复发时间为6～59个月,平均(16.25±14.01)个月.恶性肿瘤复发均为术后常规随访发现,良性肿瘤仅2例常规随访发现,其余因出现排便困难、骶尾部出现肿块等临床症状就诊.根据Altman分类,Ⅰ～Ⅳ型的复发病例(复发率)分别为8例(15.4％)、2例(10.5％)、1例(10.0％)和5例(31.3％).病理类型中,成熟性畸胎74例中复发9例,其中5例病理性质复发后转变为未成熟或恶性畸胎瘤;未成熟畸胎瘤6例中复发2例;恶性畸胎瘤17例中复发5例.肿瘤复发后的5年生存率(64.8％)与未复发病例(95.0％)相比,差异有统计学意义(P=0.002).单因素及多因素危险度分析提示术中肿瘤实质成分溃破(P=0.028)、肿瘤残留(P=0.000)、未成熟(P=0.029)及恶性畸胎瘤(P=0.026)是骶尾部畸胎瘤复发的危险因素,而Altman分类、手术年龄、肿瘤大小以及囊液破裂并不是肿瘤复发的危险因素.结论 肿瘤复发是影响骶尾部畸胎瘤预后的重要因素.术中避免肿瘤实质成分溃破,尽可能完整切除肿瘤可减少肿瘤复发.重视骶尾部畸胎瘤,特别是成熟性畸胎瘤的术后随访,有利于尽早发现肿瘤复发,提高治愈率.     

腹腔镜辅助下小儿骶尾部畸胎瘤切除术15例

目的探讨腹腔镜辅助切除小儿Ⅲ型骶尾部畸胎瘤的可行性、安全性及操作要点。方法回顾分析腹腔镜辅助手术治疗15例小儿Ⅲ型骶尾部畸胎瘤的临床资料。先经腹腔镜结扎肿瘤供应血管并游离瘤体的腹腔部分,后经骶尾部完整切除肿瘤。结果 12例顺利完成手术,3例中转开腹。术后均顺利恢复,经半年以上的随访未发现复发病例。结论腹腔镜辅助小儿骶尾部畸胎瘤切除术是有效、安全可行且损伤小的手术方法。     

Neonatal sacrococcygeal teratoma communicating with the rectum

A unique case of benign sacrococcygeal teratoma communicating with the rectal lumen at birth is reported. A large air-fluid level in the teratoma was seen on the plain X-ray film.     

Intra-abdominal (Type IV) sacrococcygeal teratoma presenting with buttock hemangioma

Sacrococcygeal teratoma is the most common type of extragonadal germ cell tumor in infants and young children. Sacrococcygeal teratomas can uncommonly present in an intra-abdominal fashion, with minimal clinical findings. Dermatologic lesions overlying the vertebrae or buttocks are often associated with sacrococcygeal teratoma and may be the only clue to their presence. Here, we report a case of an intra-abdominal sacrococcygeal teratoma presenting with an anatomically separate buttock hemangioma.     

骶尾部未成熟畸胎瘤神经组织分化与增殖的免疫组织化学研究

目的 了解骶尾部未成熟畸胎瘤神经组织分化与增殖的变化。方法 对14例骶尾部未成熟畸胎瘤手术标本进行神经元特异性烯醇酶、神经纤维细丝蛋白、S100蛋白、波形蛋白、Ki-67及增殖细胞核抗原免疫组化染色，14例患儿收治年龄为1d～4岁，平均304．8d，其中男6例，女8例。术后多处切取瘤体组织置10％中性甲醛固定2h，常规石蜡包埋。石蜡切片、染色后行病理诊断并根据参考文献所列标准分级，其中Ⅰ级6例，Ⅱ级4例，Ⅲ级4例。所有患儿术后均接受化疗并进行长期随访，随访内容有肿瘤有无复发、是否有远处转移、有无大便失禁等。笔者分析这些染色结果与病理分级间的关系。结果 神经元特异性烯醇酶与神经纤维细丝蛋白的表达随病理分级的提高阳性率下降，而波形蛋白、Ki-67及增殖细胞核抗原的表达随病理分级的提高阳性率上升，但S100的表达变化与病理分级无关。结论 神经标记物和细胞增殖标记物的免疫组化染色可为分析骶尾部未成熟畸胎瘤神经组织分化程度提供有用的资料。