Benign Sacrococcygeal Teratoma Research Articles

Prenatal Management of High Risk Fetal Sacrococcygeal Teratoma

Introduction: Fetal sacrococcygeal teratomas (SCGT), the most common fetal neoplasm diagnosed in fetuses, are a rare condition with an estimated incidence of 1 in 40,0000 live births. Fetal SCGT is usually suspected when characteristic findings are seen on ultrasound such as a mass that is cystic and/or solid and is located in the sacral region. MRI may also be used to more accurately characterize the tumor and to help distinguish between SCGTs and distal neural tube defects like sacral meningomyelocele. Despite advances in ultrasound and MRI, only approximately 50% are diagnosed prenatally. The detection of fetal SCGT is important because it changes pregnancy monitoring and management as it is associated with fetal hydrops, intrauterine death, polyhydramnios, mirror syndrome, and mortality rates as high as 43%. Once an SCGT has been identified, serial ultrasound evaluation of the fetus, placenta, and SCGT is important to identify early signs of fetal hydrops, fetal anemia, or cardiac failure and to intervene as appropriate. Case Report: A 40-year-old G5P4004 was referred to Maternal Fetal Medicine (MFM) due to findings of a 1 cm mass near the fetal bladder on first trimester ultrasound. She had a level II ultrasound performed by MFM at 16.2 weeks where a 3.6 x 2.3 x 2.6 cm mass was identified. This mass was adjacent to the sacrococcygeal region. Due to its location and characteristic appearance, with both cystic and solid components with high vascularity, a fetal sacrococcygeal teratoma was suspected. There were no signs of hydrops or fetal anemia. At this time, the recommendation for management was: ultrasounds every 2 weeks to assess for hydrops and fetal anemia, growth ultrasound every 4 weeks, an echocardiogram by pediatric cardiology at 22-24 weeks, a fetal MRI at 28 weeks, and delivery between 37-39 weeks or earlier pending clinical course. At 24.2 weeks, she developed polyhydramnios with a maximum vertical pocket (MVP) of 10.5 cm. She had also failed her 1-hour glucose tolerance test with a value of 287 mg/dL. Due to the new finding of polyhydramnios and diagnosis of gestational diabetes, her monitoring was increased to weekly, with biophysical profile (BPP) starting at 28 weeks and weekly nonstress tests (NST) at 32 weeks. A fetal MRI was performed at 26.4 weeks and showed a large lesion extending off the sacrococcygeal region measuring 9.6 x 6.3 x 7.7 cm that was composed of both solid and cystic components. By 32.2 weeks, she developed severe polyhydramnios with an AFI of 36.0 cm and accelerated fetal growth due to an AC > 99th percentile. The last ultrasound she had at 34.2 weeks the SCGT measured 17.7 x 13.3 x 13.6 cm and severe polyhydramnios persisted. The patient went into preterm labor at 35.2 weeks and had a cesarean section due to known SCGT measuring greater than 5 cm. Immediately after delivery, the neonate was evaluated by the neonatal team and transferred to the Neonatal Intensive Care Unit. The infant had surgical resection of the mass and will have tumor markers, AFP and LDH, monitored for development of recurrent immature or mature teratoma. The pathology report confirmed the diagnosis for benign sacrococcygeal teratoma. Discussion: Failure to diagnose a fetal sacrococcygeal teratoma has potentially catastrophic consequences for the fetus and mother. Maternal complications include hyperemesis, preeclampsia, mirror syndrome, preterm labor, and HELLP syndrome. Significant obstetric complications can be as high as 81%. Intrauterine fetal complications include fetal hydrops from high-output failure, fetal anemia, intratumoral hemorrhage, polyhydramnios, genitourinary obstruction leaking to hydronephrosis and or hydroureter. SCGT size >10 cm or tumor with rapid growth has > 50% perinatal mortality. Tumor characteristics associated with higher mortality rates are early diagnosis, solid components, high vascularity, tumor size > 10 cm, and rapid growth > 150 cm3/week. Another way to risk stratify these patients is by calculating a tumor volume to fetal weight ratio (TFR), which if > 0.12 at < 24 weeks is predictive of an overall poor prognosis. When comparing this case to literature review of sacrococcygeal teratomas, the patient’s fetal SCGT would be classified as high risk of mortality due to the tumor characteristics of early diagnosis, TFR at 20 weeks being 0.16, large size, rapid growth, and pronounced vascularity. Overall, in this high risk case of SCGT, the close follow up and evidence-based management with utilization and collaboration of a multidisciplinary team including Maternal Fetal Medicine, NICU, and Pediatric Surgery in a tertiary center resulted in a successful outcome.

Prenatal Management of High Risk Fetal Sacrococcygeal Teratoma

Introduction: Fetal sacrococcygeal teratomas (SCGT), the most common fetal neoplasm diagnosed in fetuses, are a rare condition with an estimated incidence of 1 in 40,0000 live births. Fetal SCGT is usually suspected when characteristic findings are seen on ultrasound such as a mass that is cystic and/or solid and is located in the sacral region. MRI may also be used to more accurately characterize the tumor and to help distinguish between SCGTs and distal neural tube defects like sacral meningomyelocele. Despite advances in ultrasound and MRI, only approximately 50% are diagnosed prenatally. The detection of fetal SCGT is important because it changes pregnancy monitoring and management as it is associated with fetal hydrops, intrauterine death, polyhydramnios, mirror syndrome, and mortality rates as high as 43%. Once an SCGT has been identified, serial ultrasound evaluation of the fetus, placenta, and SCGT is important to identify early signs of fetal hydrops, fetal anemia, or cardiac failure and to intervene as appropriate. Case Report: A 40-year-old G5P4004 was referred to Maternal Fetal Medicine (MFM) due to findings of a 1 cm mass near the fetal bladder on first trimester ultrasound. She had a level II ultrasound performed by MFM at 16.2 weeks where a 3.6 x 2.3 x 2.6 cm mass was identified. This mass was adjacent to the sacrococcygeal region. Due to its location and characteristic appearance, with both cystic and solid components with high vascularity, a fetal sacrococcygeal teratoma was suspected. There were no signs of hydrops or fetal anemia. At this time, the recommendation for management was: ultrasounds every 2 weeks to assess for hydrops and fetal anemia, growth ultrasound every 4 weeks, an echocardiogram by pediatric cardiology at 22-24 weeks, a fetal MRI at 28 weeks, and delivery between 37-39 weeks or earlier pending clinical course. At 24.2 weeks, she developed polyhydramnios with a maximum vertical pocket (MVP) of 10.5 cm. She had also failed her 1-hour glucose tolerance test with a value of 287 mg/dL. Due to the new finding of polyhydramnios and diagnosis of gestational diabetes, her monitoring was increased to weekly, with biophysical profile (BPP) starting at 28 weeks and weekly nonstress tests (NST) at 32 weeks. A fetal MRI was performed at 26.4 weeks and showed a large lesion extending off the sacrococcygeal region measuring 9.6 x 6.3 x 7.7 cm that was composed of both solid and cystic components. By 32.2 weeks, she developed severe polyhydramnios with an AFI of 36.0 cm and accelerated fetal growth due to an AC > 99th percentile. The last ultrasound she had at 34.2 weeks the SCGT measured 17.7 x 13.3 x 13.6 cm and severe polyhydramnios persisted. The patient went into preterm labor at 35.2 weeks and had a cesarean section due to known SCGT measuring greater than 5 cm. Immediately after delivery, the neonate was evaluated by the neonatal team and transferred to the Neonatal Intensive Care Unit. The infant had surgical resection of the mass and will have tumor markers, AFP and LDH, monitored for development of recurrent immature or mature teratoma. The pathology report confirmed the diagnosis for benign sacrococcygeal teratoma. Discussion: Failure to diagnose a fetal sacrococcygeal teratoma has potentially catastrophic consequences for the fetus and mother. Maternal complications include hyperemesis, preeclampsia, mirror syndrome, preterm labor, and HELLP syndrome. Significant obstetric complications can be as high as 81%. Intrauterine fetal complications include fetal hydrops from high-output failure, fetal anemia, intratumoral hemorrhage, polyhydramnios, genitourinary obstruction leaking to hydronephrosis and or hydroureter. SCGT size >10 cm or tumor with rapid growth has > 50% perinatal mortality. Tumor characteristics associated with higher mortality rates are early diagnosis, solid components, high vascularity, tumor size > 10 cm, and rapid growth > 150 cm3/week. Another way to risk stratify these patients is by calculating a tumor volume to fetal weight ratio (TFR), which if > 0.12 at < 24 weeks is predictive of an overall poor prognosis. When comparing this case to literature review of sacrococcygeal teratomas, the patient’s fetal SCGT would be classified as high risk of mortality due to the tumor characteristics of early diagnosis, TFR at 20 weeks being 0.16, large size, rapid growth, and pronounced vascularity. Overall, in this high risk case of SCGT, the close follow up and evidence-based management with utilization and collaboration of a multidisciplinary team including Maternal Fetal Medicine, NICU, and Pediatric Surgery in a tertiary center resulted in a successful outcome.

Sacrococcygeal teratoma in Nigerians: A case report and clinico-pathologic review

Introduction: Sacrococcygeal Teratomas (SCTs) are rare childhood tumors, commonly seen in the neonatal period. The mainstay of their diagnosis is both clinical and radiological. Early gross-total surgical excision has the best prognostic outcome, especially for benign tumors. Case report: We present the case of a 12-year old girl with recurrent infected right gluteal benign SCT who was successfully managed in a secondary-level health facility in Nigeria. She has remained symptom-free with no recurrence four years post-surgery. We also reviewed the medical literature on SCT in Nigeria. Discussion: Our case report and others from Nigeria are consistent with the global trend on SCTs. They mostly occur within the first 15 years of life, with a bimodal peak in the first 4 years and at 8-11 years. 75-90% are benign, and the tumors often contain tissues from the three germ layers. Complete surgical excision is the treatment of choice. Conclusion: SCTs are rare tumors, but require early diagnosis and complete surgical excision to prevent malignant transformation. The case report draws attention to the need for a multi-institutional tumor registry in Nigeria and other sub-Saharan African countries. Keywords: sacrococcygeal teratoma; surgical excision; nigeria; literature review.

An unusual cause for recurrent perianal sepsis in Currarino syndrome: Case report and review of the literature

Abstract Currarino syndrome (CS) is a rare symptom complex comprising an anorectal malformation, a sacral bone abnormality and a presacral mass. Recurrent sepsis of the perianal area is a well-described post-operative morbidity. We report the case of a 4-year-old boy with CS and chronic perianal sepsis in the form of a draining sinus at the level of S5, which had plagued him since removal of a benign sacrococcygeal teratoma at the age of 1 year. Normal alfa feto protein and beta-human chorionic gonadotropin levels ruled out malignant recurrence of a sacrococcygeal teratoma, whilst an MRI showed a large presacral abscess with no fistulous connection to the rectum. Biopsy and drainage of the abscess was performed through a posterior sagittal approach and pus was sent for microscopy, culture and sensitivity. Microscopy demonstrated the presence of acid-fast bacilli, confirming infection with Mycobacterium tuberculosis (TB). A review of the literature revealed paucity in the documentation of microbes related to this common complication with no previous reports of TB as the aetiological pathogen. This case is of particular interest as the patient was not immunocompromised, had no TB contacts or history of previous TB infection, and highlights the importance of actively excluding the cause of recurrent perianal sepsis, particularly in syndromes where sepsis is a common complication.

Evaluation of chemotherapeutic sequelae and quality of life in survivors of malignant sacrococcygeal teratoma.

The impact of chemotherapeutic sequelae on long-term quality of life (QoL) for survivors of malignant sacrococcygeal teratoma (SCT) is unknown. The incidence of chemotherapeutic toxicity in patients treated for malignant SCT and possible effects on the QoL were analyzed. Retrospective chart review of patients ≥18years treated for SCT in the Netherlands was performed. Present QoL was evaluated using the SF-36 questionnaire. The results of survivors of malignant SCT were compared to those of patients treated for benign SCT. Fifty-one of 76 traceable patients consented to participate. The results of 47 (92.2%), 9 men and 38 women (median age 25.4years, range 18.3-41.2), were analyzed. Eleven had been treated for malignancy; 63.6% suffered from at least one chemotherapeutic sequel with hearing loss as the most common one. Results for both groups were similar on all but one SF-36 subcategory; those treated for malignant tumor scored significantly lower on the subcategory physical functioning (p=0.02). Despite the high incidence of chemotherapeutic sequelae among survivors of malignant SCT, their QoL does not differ from that of those treated for benign SCT. Even though their physical functioning is restricted, daily activities and psychosocial functioning of survivors of malignant SCT are not restricted.

Long-Term Outcomes of Sacrococcygeal Germ Cell Tumors in Infancy and Childhood

Purpose. The aim of this study was to evaluate long-term outcomes of sacrococcygeal germ cell tumors (SC-GCTs) over a 15-year period. Materials and Methods. A retrospective review was conducted of all pediatric patients treated for SC-GCTs at our hospital from 1998 to 2012. Results. Fifty-seven patients were treated for SC-GCTs with the most common in Altman's classification type I. Age at surgery ranged from one day to 5.6 years. Tumor resection and coccygectomy were primarily performed in about 84% of the cases. Pathology revealed mature, immature, malignant sacrococcygeal teratomas (SCTs), and endodermal sinus tumors (ESTs) in 41 (72%), 4 (77%), 6 (10.5%), and 6 (10.5%), respectively. Recurrence of discase occurred in 3 of 41 patients with mature teratomas (7.3%); 2 recurrences with mature teratomas and one recurrence with EST. Five of 6 malignant SCTs and 3 of 6 ESTs responded well to the treatment. Alpha-fetoprotein (AFP) level was elevated in both malignant teratomas and ESTs. No immediate patient death was noted in any of the 57 cases, but 4 patients with malignant tumors and distant metastasis succumbed at home within 2 years of the initial treatment. Conclusion. Benign SCTs have a significant recurrence rate of approximately 7%. Close follow-up with serial AFP level monitoring should be done for 5 years after initial tumor resection and coccygectomy. The survival rate for malignant SC-GCTs with distant metastasis was unfavorable in the present study.

Turnbull–Cutait abdominoperineal pull-through: a safe approach for recurrent sacrococcygeal teratoma complicated by rectovaginal fistula

Sacrococcygeal teratomas are rarely seen and are classified as type I germ cell tumors. While surgery is the main treatment option, poor postoperative urological and anal functional outcomes and recurrence are major concerns [1]. An extensive resection with proctectomy is one of theoptions offering a curative treatment for recurrent sacrococcygeal teratomas. Maintenance of intestinal continuity is another component of a comprehensive therapeutic approach and should be considered for the sake of the patient’s quality of life after surgery. We hereby present the Turnbull–Cutait abdominoperineal pull-through anastomosis (T–C) technique in a patient with recurrent sacrococcygeal teratoma complicated by a rectovaginal fistula. A 41-year-old female had undergone surgery for a benign sacrococcygeal teratoma 24 years ago and had no complaints until last year. After she sought help for tailbone pain at an outside hospital, a recurrent presacral mass was discovered with a computer tomography scan. Fine-needle biopsy performed at an outside hospital confirmed the diagnosis of a recurrent sacrococcygeal teratoma. The initial operation which was performed to excise the recurrent lesion failed, and a rectovaginal fistula developed on postoperative day 20. A diverting ileostomy was then created to control pelvic sepsis, and the patient was eventually referred to our institution. At presentation, a baseline pelvic magnetic resonance imaging scan was requested, which revealed the recurrent sacrococcygeal teratoma with operative changes related to prior surgery (Fig. 1a). A stricture at 7 cm from the anal verge was found where pus drainage was observed in rectal examination which was performed under general anesthesia. The patient had type 1 diabetes, a body mass index of 21.9 kg/m and an American Society of Anesthesiologists (ASA) score of 3. Her surgical history included cholecystectomy and total abdominal hysterectomy in addition to prior surgical intervention for sacrococcygeal teratoma. It was determined that a total mesorectal excision was necessary in order to achieve total removal of the neoplastic lesion. The patient was consented preoperatively for colovaginal fistula takedown and colorectal anastomosis plus possible abdominoperineal resection. During the operation, after resection of the rectum, stapled anastomosis was deemed inappropriate due to poor tissue quality and the decision was made to proceed with our traditional two-staged T–C technique [2]. The operation was completed uneventfully. The perineal colonic segment was resected on postoperative day 5, and hand-sewn coloanal anastomosis was performed (Fig. 1b). Postoperatively, the patient had persistent pelvic pain and developed a pelvic abscess which was diagnosed on postoperative day 12 and was drained percutaneously. Intravenous antibiotic therapy was started, and the patient was discharged on postoperative day 20 after pain control was achieved. A T–C pull-through with delayed coloanal anastomosis, T–C, was first described by Turnbull and Cutait for the treatment of rectal cancer, Hirschsprung’s disease and megacolon in Chagas disease [3, 4]. Basic steps of the Turnbull–Cutait abdominoperineal pull-through anastomosis were shown in the Fig. 1c–f. T–C is not typically preferred for low pelvic anastomoses, since advanced stapler technology is now used effectively. However, we believe that T–C has its place in low coloanal anastomoses, The study was supported by the Ed and Joey Story Endowed Chair in Colorectal Surgery.

Sacrococcygeal teratoma in infants and children

The purpose of this study is to share our experience with extensive sacrococcygeal teratoma, paying particular attention to neurological function, intraoperative hemorrhage, rate of tumor recurrence, and the surgical procedure. Infants and children with sacrococcygeal teratoma were selected and generally examined in order to detect other associated anomalies. Patients with giant sacrococcygeal tumor and high output heart failure were managed by the abdominosacral route while patients with small sacrococcygeal tumor and normal cardiac function were managed by the sacral approach. All operated patients were observed and followed-up regularly for a period of 1-3 years after tumor excision. This study presents 22 patients with sacrococcygeal teratoma managed over a period of 40 months; 18 patients presented in the first 2 months of life and four children aged 2-4 years. Fifteen patients were treated by sacral excision and seven patients were corrected by abdomenosacral excision and temporary colostomy. Benign teratoma presented in 17 patients, one of whom died (6%), and five patients had malignant tumor, one of whom died (20%). The fecal and urinary state was good in all patients treated by the sacral route. Patients corrected by abdominosacral excision showed varied degrees of urinary incontinence and constipation. Survival in excised malignant tumor was good following chemotherapy; three patients received chemotherapy with a 70% survival rate. Benign sacrococcygeal teratoma has an excellent outcome after early surgery, but the incidences of malignancy increase if resection is delayed.

Intraocular Teratoma in Association With Sacrococcygeal Teratoma: A Clinicopathologic Report

A benign sacrococcygeal teratoma was resected from an infant at birth. Four weeks later, intraocular tumors were identified in the right eye. The eye expanded in size and was removed at 16 weeks. A benign cystic intraocular teratoma was identified.

MRI diagnosis of benign sacrococcygeal teratomas of the infants

To investigate the value of MR imaging in the diagnosis of benign sacrococcygeal teratomas of the infants. MR imaging of benign sacrococcygeal teratomas in 6 cases proved by surgery and pathology was retrospectively reviewed. In all patients, a fast imaging sequence, fast spin echo sequence was employed, together with short time inversion recovery sequence and contrast enhancement scanning by intravenous injection of Gd-DTPA. There were 6 benign sacrococcygeal teratomas, which were heterogeneous masses and arose from the distal sacrococcygeal region in the pelvis. The MR imaging appearances of the benign sacrococcygeal teratomas were characteristic, T1- and T2-weighted images demonstrated a large mass containing round, well-defined areas of varying signal intensity representing its cystic, solid, and sometimes fat, calcification within the lesions. MR imaging provides definitive information of benign sacrococcygeal teratomas and clearly shows both extra-and intra-pelvic components, and even better anatomic details, which facilitates the surgical planning adequately.

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, 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.

Feasibility of perineal sagittal approaches in patients without anorectal malformations

Perineal sagittal approaches (posterior sagittal anorectoplasty and anterior and posterior sagittal transanorectal approaches) allow complete anatomic exposure of the perineum and lower pelvis. Moreover, they reduce the risk of damaging important structures because the incision is led in the midline. Therefore, many surgeons have used these approaches to treat diseases other than anorectal malformations (ARM), including intestinal dysganglionosis, trauma, pseudohermaphroditism, presacral mass, and rectal duplication. The aim of this study was to describe a small series of patients operated on via these approaches at Gaslini Children's Hospital over a 5-year period. We retrospectively evaluated 10 patients consecutively operated on via a perineal sagittal approach, with or without sphincteric structure involvement, between January 1997 and December 2001. All of these patients were without ARM. Indications included retrorectal abscesses (two), iatrogenic anal canal stenosis (one), postinflammatory anal canal stenosis (one), internal anal sphincter neurogenic achalasia (one), female pseudohermaphroditism (one), benign sacrococcygeal teratomas (two), malignant sacrococcygeal teratoma (one), and perineal rhabdomyosarcoma (one). Protective colostomy was used in four patients. The parameters that we analysed included technical details, possible complications, perineal cosmetic appearance, and outcome. No complications were experienced. The postoperative cosmetic perineal appearance was excellent in all patients, and continence, when assessed, was always considered satisfactory. All tumours underwent complete gross resection. However, one patient with malignant sacrococcygeal teratoma died as a result of the malignant process 2 years after surgery. Although our study was carried out on a small series of patients, it confirmed that perineal sagittal approaches can be used not only for ARM but also for other conditions involving perirectal pouches, presacral space, and urogenital structures, as these approaches are safe and provide excellent cosmetic results as well as satisfactory functional outcome. Although tumours can be treated via these approaches, outcome remains related to the nature and malignancy of the disease itself.

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).

Sacrococcygeal teratoma. Outcome and management. An analysis of 17 cases.

The authors compare their experience of 17 cases of sacrococcygeal teratoma (SCT) with the literature in an attempt to clarify the natural history of this tumor and to identify factors related to its prognosis and management. The obstetrical, neonatal and surgical data were analyzed for 17 cases of SCT observed between July 1985 and December 1998. Three fetuses died in utero or shortly after birth. In the remaining 14, the tumors were removed. Twelve of the infants are currently tumor-free, with good sphincter control and lower-limb function. The remaining two died: one had a malignant tumor, and the other had a recurrence of an embryonal carcinoma. Recurrent tumors (mature histotypes) were also removed from two of the 12 patients who survived. Benign SCTs generally have favorable prognosis. Negative prognostic factors for SCT include solid tumors, those detected early in pregnancy, malignant histotypes, polyhydramnios, placentomegaly, and fetal hydrops.

Prenatal diagnosis and management of sacrococcygeal teratoma.

Five fetuses, each with a sacrococcygeal teratoma (SCT) were delivered at the Royal Women's Hospital while 2 fetuses, each with a SCT were delivered at Monash Medical Centre in 1998. The number of cases reported in this series is higher than expected but it most likely occurred due to chance. The diagnosis was made prenatally in all cases. Three of the SCT were entirely external while the remaining 4 were external with intrapelvic extension. Rapid growth of the SCT occurred in 3 fetuses. This was associated with polyhydramnios in 2 fetuses. No fetus developed nonimmune hydrops. Six infants were liveborn (perinatal mortality rate of 14%), 3 of whom were delivered prior to 37 weeks' gestation. Two infants were delivered by classical Caesarean section. The remaining 4 infants were delivered by lower uterine segment Caesarean section. There was 1 perinatal death. This stillborn infant was delivered vaginally. The 6 surgical resections were performed between the 4th and 10th postnatal days. Histological examination confirmed the diagnosis of benign SCT in each. One infant developed a recurrence at 2 months of age and required chemotherapy.

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.

Neonatal benign sacrococcygeal teratoma may recur in adulthood and give rise to malignancy: P. Lahdenne, M. Heikinheimo, V. Nikkanen, et al. Cancer 72:3727–3731, (December 15), 1993

Neonatal benign sacrococcygeal teratoma may recur in adulthood and give rise to malignancy: P. Lahdenne, M. Heikinheimo, V. Nikkanen, et al. Cancer 72:3727–3731, (December 15), 1993

Lower Urinary Tract Dysfunction in Children with Benign Sacrococcygeal Teratoma

We evaluated 8 girls and 3 boys with benign sacrococcygeal teratoma for lower urinary tract dysfunction. All children underwent ultrasonography of the urinary tract, voiding cystourethrography and at least 2 standard urodynamic evaluations. Mean patient age at first urodynamic study was 59 months (range 1 to 136). Nine of the 11 children had abnormal studies, including detrusor instability and associated pelvic floor overactivity during micturition in 2, an anatomical infravesical obstruction in 2 and neurogenic bladder-sphincter dysfunction in 5 (2 detrusor hyperreflexia with sphincter dyssynergia, 2 hyporeflexic bladder-sphincter function and 1 detrusor hyporeflexia with normal sphincter function). Although the major cause of neurogenic lower urinary tract dysfunction seems to be related to surgical trauma of the pelvic plexus and sacral nerves, we observed tethered spinal cord as a primary cause in 2 patients. The incidence of voiding dysfunction in children with sacrococcygeal teratoma is high and is usually neurogenic in origin.

Association of tumour necrosis factor and haemodynamic shock in a newborn undergoing surgery for sacrococcygeal teratoma

A newborn who was operated upon for a benign sacrococcygeal teratoma at the age of 2 weeks developed haemodynamic instability with a shock episode at the time of operation. The serum level of tumour necrosis factor alpha (TNF-Alpha) during this event rose to 158 pg/ml (normal <15 pg/ml). Preoperatively TNF-Alpha was undetectable, while post-operatively the level was 23 pg/ml. Serum levels of the cytokine interleukin-1 beta (IL-1 beta) were undetectable throughout the study. The baby was treated successfully by fluid challenge and dopamine. This case represents a temporal association between haemodynamic instability during surgical intervention and a high serum level of TNF-Alpha, which is an important mediator in the pathogenesis of septic shock.

Anorectal function in adults operated for a benign sacrococcygeal teratoma

Fecal continence and quality of life were evaluated by a questionnaire in 26 adult patients (mean age, 30 years; 6 men, 20 women) who had undergone surgery for a benign sacrococcygeal teratoma in infancy. The fecal continence was assessed by a score described by Holschneider. Twenty-six healthy people with a similar age and sex distribution were used as controls. Good fecal continence was reported by 88% of the patients; however, only 27% had completely normal bowel habits. Some fecal soiling was present in 27% of the patients. No correlation between the severity of aberrations in anorectal functions and the degree of intrapelvic extension of the tumor was found. Social problems related to deficient anorectal function were reported by 27% of the patients. Other health problems including urinary incontinence were reported by 50% of the patients. All the controls had good fecal continence; 77% of them had completely normal bowel habits. The present study shows that at the adult age, a significant proportion of patients who have undergone surgery for sacrococcygeal teratoma suffer from deficient anorectal function and a diminished quality of life.