Sacrococcygeal teratoma: A case report

Sacrococcygeal teratomas (SCTs) are the most common extragonadal germ cell tumors, comprised different types of tissues that come from at least two of three germ cell layers. Depending on the tissues that are included, they are divided into mature, immature, and malignant. The incidence of SCT in infants and children is 1 in 35,000–40,000 live births. We are reporting a case of type I SCT. The patient was gravida 3, para 1, and abortion 1, which was diagnosed during antenatal ultrasound examination at 22 weeks of gestation and the termination was done after counseling the parents. A female fetus with a tumor in the sacrococcygeal region, weighing 800 g was delivered. The baby was sent to the department of anatomy. SCTs develop at the base of the coccyx and are thought to be derived from Henson’s node a rounded and elevated area at the cranial end of the primitive streak. This primitive streak consists of totipotent cells, which are able to transform into any type of cells.

Asymmetric twinning: A preterm baby with Sacrococcygeal Teratoma and parasitic twin born to a 16-year-old Primigravida / Philippine Journal of Obstetrics and Gynecology

@#A 16-year-old primigravida, at 33 weeks and 5 days age of gestation came in due to preterm labor. Sonographic examination revealed an incidental finding of a mass attached to the sacrococcygeal area. The mass has a cystic and solid component diagnosed as sacrococcygeal teratoma. Attached to the mass were two lower extremity structures identified as femurs with feet and was considered as an underdeveloped parasitic twin. A classical cesarean section was performed because of the advanced preterm labor, and a live female infant weighing 2500 g was delivered. The parasitic lower limbs, however, inadvertently detached during delivery. There was a high index of suspicion because of a larger fundic height of 37 cm compared to the age of gestation and the difficult palpation on Leopold's maneuver. Accuracy of ultrasound findings helped the obstetricians to a timely and prepared for delivery.

Manejo in útero de teratoma sacrocoxigeo y resección postnatal / In utero management of sacrocoxygeal teratoma and postnatal resection

Describimos un caso de teratoma sacro-coxígeo gigante diagnosticado a las 21 semanas de gestación. Debido a los signos de robo vascular y de insuficiencia cardíaca fetal a las 24 semanas de gestación, se planteó la esclerosis del vaso nutricio por medio de laser Diodo. El procedimiento se realizó mediante punción percutánea guiada por ecografía en tres oportunidades, a las 25 semanas, a las 27 y a las 30 semanas. No hubo efecto secundario inmediato post tratamiento. La desvascularización fue parcial, debido a la híper-vascularización periférica de la masa. Los signos de insuficiencia cardíaca fetal se normalizaron después de 1 semana con disminución de crecimiento del tumor. El parto se realizó por cesárea a las 34 semanas, por rotura prematura de membranas y trabajo de parto avanzado en presentación pelviana. Tras 6 horas posteriores a su nacimiento se realizó cirugía de exceresis tumoral por técnica convencional, donde se extirpó por completo el teratoma sacrocoxígeo con un peso total de 630 gramos, material que se envió a anatomía patológica, cuyo diagnóstico final fue teratoma inmaduro grado III. Conclusión: La cirugía mínimamente invasiva con esclerosis del vaso nutricio parece mejorar el resultado perinatal en casos de teratoma sacro-coxígeo (TSC) fetal de alto riesgo. La identificación y el tratamiento temprano nos pueden dar resultado exitoso

We describe a case of giant sacrococcygeal teratoma (SCT) diagnosed at 21 weeks of gestation. Due to the signs of vascular steal and fetal heart failure at 24 weeks of gestation, sclerosis of the nutrient vessel was proposed by a laser diode. The procedure was performed by percutaneous puncture guided by ultrasound on three occasions, at 25 weeks, at 27 and at 30 weeks. There was no immediate side effect after treatment. The devascularization was partial, due to the peripheral hypervascularization of the mass. Signs of fetal heart failure normalized after 1 week with decreased tumor growth. Delivery was performed by cesarean at 34 weeks, due to premature rupture of the membranes and advanced labor in pelvic presentation. Six hours after birth, tumor excision was performed using a conventional technique, in which SCT with a total weight of 630 grs was completely removed, material that was sent to the pathological anatomy. Final diagnosis was immature teratoma grade III. Conclusions: Minimally invasive surgery with sclerosis of the nutrient vessel seems to improve the perinatal outcome in cases of high-risk fetal SCT. Identification and early treatment can give us a successful outcome

Management mature sacrococcygeal teratoma: a case report

Mature sacrococcygeal teratoma (SCT) are uncommon neoplasm comprised of mixed elements derived from three germ layers. They attract attention because of their gross appearance and bizarre histology. Tumor of the sacrococcygeal region, referred to as sacrococcygeal teratomas (SCTs) in most reports, generally present in two distinct fashions: neonates with large predominately external lesions, which are detected in utero or at birth and are rarely malignant; and older infants and children who present with primarily hidden pelvic tumors with a much higher rate of malignancy. Sacrococcygeal teratomas are the most common extragonadal tumor in neonates, accounting for up to 70% of all teratomas in childhood. A 3 to 4:1 female to male ratio is generally reported. Surgical resection remains the mainstay of therapy and recurrence is rare following complete excision. A 14 years old girl was presented to us by her parents with a mass at the buttock since birth. She was delivered at home by traditional midwife after a term, unsupervised pregnancy to a 35 years old woman. Both pregnancy and delivery were uneventful. Direct rectal examination revealed a mass has displaced the recto-sigmoid anteriorly. The CT scan revealed a heterogenous mass with a solid, cystic, and multiple classification density at the anterior of coccygeus bone, and push the coccygeus to the posterior. The mass infiltrated the subcutis, and attached to the posterior aspect of rectum. A 14 years old girl presented by mature SCT since newborn comprising ectoderm, mesoderm, and endoderm tissue. She had a complete surgical excision (including coccygectomy) with primary wound closure. A complete surgical excision remains the mainstay of therapy of mature SCT.

Adult sacrococcygeal teratoma: a retrospective study over eight years at a single institution / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

Objective: To determine the clinical, imaging, and histological features, and surgical resection modalities and outcomes of adult sacrococcygeal teratoma (SCT). Methods: Adult patients with histopathologically diagnosed SCT were enrolled in our hospital between August 2010 and August 2018. Each patient’s characteristics and clinical information were reviewed. Results: There were 8 patients in the study (2 males, 6 females) with a median age of 34 years (range, 18–67 years). The time to clinical symptoms was 14 d to 35 years, with a median time of 4 years. Six patients presented with symptoms of sacrococcygeal pain, and four with signs of sacrococcygeal mass and ulceration in the sacrococcygeal region. Six patients were evaluated using a combination of computed tomography (CT) and magnetic resonance imaging (MRI). All patients showed a presacral tumor with heterogeneous intensity on CT images. All patients underwent surgical treatment, including 6 parasacral, 1 transabdominal, and 1 combined anterior-posterior surgery cases. Seven patients were histopathologically diagnosed with benign mature SCT, and have shown no recurrence. One patient had malignant SCT, with recurrence at 84 months after surgery. After a second surgery, the patient had no recurrence within 6 months follow-up after re-resection. Conclusions: Our retrospective study demonstrated: (1) adult SCT is difficult to diagnose because of a lack of typical clinical symptoms and signs; (2) a combination of CT and MRI examination is beneficial for preoperative diagnosis; (3) the choice of surgical approach and surgical resection modality depends on the size, location, and components of the tumor, which can be defined from preoperative CT and MRI evaluation; (4) most adult SCTs are benign; the surgical outcome for the malignant SCT patient was good after complete resection. Even for the patient with recurrent malignant SCT, the surgical outcome was good after re-resection.

Adult sacrococcygeal teratoma: a retrospective study over eight years at a single institution / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

OBJECTIVE@#To determine the clinical, imaging, and histological features, and surgical resection modalities and outcomes of adult sacrococcygeal teratoma (SCT).@*METHODS@#Adult patients with histopathologically diagnosed SCT were enrolled in our hospital between August 2010 and August 2018. Each patient's characteristics and clinical information were reviewed.@*RESULTS@#There were 8 patients in the study (2 males, 6 females) with a median age of 34 years (range, 18-67 years). The time to clinical symptoms was 14 d to 35 years, with a median time of 4 years. Six patients presented with symptoms of sacrococcygeal pain, and four with signs of sacrococcygeal mass and ulceration in the sacrococcygeal region. Six patients were evaluated using a combination of computed tomography (CT) and magnetic resonance imaging (MRI). All patients showed a presacral tumor with heterogeneous intensity on CT images. All patients underwent surgical treatment, including 6 parasacral, 1 transabdominal, and 1 combined anterior-posterior surgery cases. Seven patients were histopathologically diagnosed with benign mature SCT, and have shown no recurrence. One patient had malignant SCT, with recurrence at 84 months after surgery. After a second surgery, the patient had no recurrence within 6 months follow-up after re-resection.@*CONCLUSIONS@#Our retrospective study demonstrated: (1) adult SCT is difficult to diagnose because of a lack of typical clinical symptoms and signs; (2) a combination of CT and MRI examination is beneficial for preoperative diagnosis; (3) the choice of surgical approach and surgical resection modality depends on the size, location, and components of the tumor, which can be defined from preoperative CT and MRI evaluation; (4) most adult SCTs are benign; the surgical outcome for the malignant SCT patient was good after complete resection. Even for the patient with recurrent malignant SCT, the surgical outcome was good after re-resection.

Teratoma Sacrococcígeo: Reporte de Caso / Sacrococcygeal Teratoma: Case Report

El teratoma sacrococcígeo es el teratoma más común en los recién nacidos, con predominio en el sexo femenino. Se presenta el manejo de un neonato de 5 días de vida(AU)

Sacrococcygeal teratoma is the most common teratoma in newborn, predominantly in female sex. A case of a 5 day old newborn is presented(AU)

A Contrast Nephropathy in a Preterm Infant Following Preoperative Embolization of Giant Sacrococcygeal Teratoma

Newborn infants with huge and highly vascular sacrococcygeal teratoma (SCT) are frequently subjected to renal hypoperfusion secondary to high-output cardiac failure. Any underlying renal dysfunction is a significant risk factor for the development of contrast-induced nephropathy (CIN). However, reports on CIN in infants are rare. I report here a case of a premature infant born at 28 weeks and 3 days of gestation with a huge SCT who survived preoperative embolization and surgical resection but presented with persistent non-oliguric renal failure that was suggestive of CIN. During radiological intervention, a contrast medium had been administered at about 10 times the manufacturer-recommended dose for pediatric patients. Despite hemodynamic stabilization and normalization of urine output immediately following surgery, the patient's serum creatinine and cystatin-C levels did not return to baseline until 4 months after birth. No signs of reflux nephropathy were observed in follow-up imaging studies. Dosing guidelines for the use of a contrast medium in radiological interventions should be provided for infants or young patients.

Manejo prenatal del teratoma sacroccocígeo, a propósito del primer caso sometido a transfusión sanguínea intrauterina en el Perú / Prenatal management of sacroccocygeal teratoma, a propos of the first case subjected to intrauterine blood transfusion in Peru

El teratoma sacrococcígeo es una enfermedad rara en el feto, pero con alta mortalidad perinatal debido al secuestro de flujo sanguíneo y consiguiente desarrollo de anemia fetal severa. Presentamos el caso de una gestante de 27 semanas referida a nuestro servicio para manejo prenatal de un feto con teratoma sacrococcígeo gigante, que desarrolló anemia fetal severa y fue sometido a una transfusión intrauterina intravascular, la cual pudo prolongar el embarazo y mejorar los resultados perinatales.

Sacrococcygeal teratoma is a rare fetal disease but with high perinatal mortality due to sequestration of blood flow and consequent development of severe fetal anemia. We present the case of a 27 weeks pregnant woman referred to our service for prenatal management of a fetus with giant sacrococcygeal teratoma and severe anemia and who was subjected to intrauterine intravascular transfusion that could permit prolongation of the pregnancy and improve perinatal results.

Trapped after Coming Buttocks: A Consequence of Sacrococcygeal Teratoma: A Case Report.

Sacrococcygeal teratoma (SCT) is the commonest fetal tumour but a rare cause of obstructed labour. If undiagnosed during antenatal period, high index of suspicion is required during labour to make a diagnosis. In this case report, we present a case of sacrococcygeal teratoma (SCT) which was diagnosed at the time of delivery. The fetus, with a massive ruptured sacrococcygeal mass, was delivered by traction and suprapubic pressure. Histology report revealed malignant sacrococcygeal teratoma. The diagnosis of sacrococcygeal teratoma (SCT) should form part of the differential diagnoses when there is obstructed labour after the delivery of the fetal shoulders so that appropriate management can be instituted.

Teratoma sacrococcígeo: diagnóstico, evaluación y terapia prenatal / Sacrococcygeal teratoma: diagnosis, evaluation and prenatal therapy

El teratoma sacrococcígeo representa la lesión tumoral más frecuente durante la gestación. Si bien su frecuencia es baja, en asociación con hidrops puede determinar la aparición de insuficiencia cardiaca fetal y posterior muerte fetal in útero. La evaluación prenatal mediante ultrasonografía, debe orientarse hacia la selección de aquellos fetos candidatos a intervención prenatal para mejorar el resultado perinatal de fetos con compromiso hemodinámico que se encuentran bajo la viabilidad. El objetivo de la presente actualización es exponer las herramientas actualmente en uso para realizar dicha evaluación y los resultados de la cirugía prenatal. La puntuación del perfil cardiovascular fetal mediante la ecografía, se propone como una herramienta sensible y útil en la selección de fetos candidatos a intervención prenatal. Tanto la cirugía abierta como el abordaje mínimamente invasivo se exponen como alternativas en el manejo prenatal de fetos candidatos a intervención con sobrevida reportada en torno al 50 y 44 por ciento respectivamente.

The sacrococcygeal teratoma is the most common tumor during prenatal period. Although its frequency is low, together with hydrops could determine the occurrence of fetal heart failure, and subsequent fetal death in utero. Prenatal ultrasonographic evaluation, should be directed toward selection of those fetuses as candidates for prenatal therapy, to improve their perinatal outcome, when they present hemodynamic compromise while they are under fetal viability. The purpose of this update is to expose the tools currently used to conduct the assessment and the results of prenatal surgery. The fetal cardiovascular profile score by ultrasound is proposed as a sensible and useful tool in the selection of candidates for prenatal intervention. Both open surgery as minimally invasive approach are presented as alternatives in the prenatal management of fetus as candidates to intervention, has been reported a survival about 50 and 44 percent respectively.

Cardiac arrest during excision of a huge sacrococcygeal teratoma: A report of two cases / 대한마취과학회지

Resection of large sacrococcygeal teratomas (SCTs) in premature neonates has been associated with significant perinatal mortality, making this a high risk procedure requiring careful anesthetic management. Most deaths during resection of SCTs are due to cardiac arrest caused by electrolyte imbalances, such as hyperkalemia, and massive bleeding during surgery. We describe two premature neonates who experienced cardiac arrest, one due to hyperkalemia and the other not due to hyperkalemia, during excision of large, prenatally diagnosed SCTs. We present here the considerations for anesthesia in premature neonates with huge SCTs.

Teratoma sacrococcígeo: revisión de la literatura a propósito de un caso / Sacrococcygeal teratoma: literature review concerning a case

Clásicamente el teratoma es definido como un tumor compuesto de tejidos derivados de las 3 capas germinales del embrión (endodermo, mesodermo y ectodermo). El teratoma sacrococcígeo es un tumor ubicado en la base del cóccix. Tiene una incidencia de 1 por cada 35 000 a 40 000 nacimientos y es el tumor más común en los recién nacidos, con predominio en el sexo femenino. Se presenta el caso de un recién nacido del sexo femenino, con diagnóstico de teratoma sacrococcígeo de tipo II, de naturaleza benigna, y se ofrece una revisión sobre el tema.

Classically, the teratoma is defined like a tumor consisted of tissues from the three germinal layers of embryo (endoderm, mesoderm and ectoderm). Sacrococcygeal teratoma is a tumor located at the coccyx base with an incidence of 1 by 35 000 to 40 000 births and it is the commonest tumor in newborns with predominance in female sex. This is the case of a female newborn diagnosed with a benign sacrococcygeal type II teratoma. Authors offer a review on this subject.

Sacrococcygeal Teratoma in a Child: A Case Report.

Sacrococcygeal teratomas (SCT) are tumors that arise in the sacrococcygeal area containing tissue from all three germ layers. SCT has an incidence of approximately one in 35,000 – 40,000 live births with malignant transformation with increasing age. We report a case in a four year old which was excised completely via the postanal route with no operative or postoperative morbidity. The histopathology was that of yolk sac variant and she is planned for chemoradiotherapy.

Correlação entre os achados ultra-sonográficos e de ressonância magnética no teratoma sacrococcígeo fetal / Correlation between ultrasonographic and magnetic resonance imaging findings in fetal sacrococcygeal teratoma

OBJETIVO: Correlacionar os achados ultra-sonográficos e de ressonância magnética no teratoma sacrococcígeo fetal. MATERIAIS E MÉTODOS: Três pacientes com idade gestacional entre 30 e 35 semanas, com diagnóstico ultra-sonográfico suspeito de teratoma sacrococcígeo fetal, foram submetidas a ressonância magnética e, posteriormente, a ultra-sonografia para correlação dos achados. Tanto na ressonância magnética quanto na ultra-sonografia foram avaliadas as dimensões, a localização, a extensão e os conteúdos dos tumores. RESULTADOS: A ultra-sonografia e a ressonância magnética obtiveram resultados semelhantes em relação à localização, ao tamanho e ao conteúdo dos tumores. Todas as lesões localizavam-se na região sacrococcígea, com dimensões médias de 6,0 cm x 9,0 cm. Quanto ao conteúdo dos tumores, um dos casos era completamente cístico e dois eram sólidos e císticos. A extensão exata das lesões foi mais bem avaliada pela ressonância magnética do que pela ultra-sonografia, mostrando de forma adequada o acometimento pélvico nos três casos. CONCLUSÃO: A ressonância magnética fetal é capaz de complementar os achados ultra-sonográficos do teratoma sacrococcígeo fetal, uma vez que determina com melhor precisão o conteúdo e a extensão do tumor, auxiliando na conduta terapêutica e aumentando as chances de cura desses fetos.

OBJECTIVE: The present study was aimed at correlating ultrasonographic and magnetic resonance imaging findings in patients with fetal sacrococcygeal teratoma. MATERIALS AND METHODS: Three pregnant women between the 30th and 35th weeks of gestation were submitted to fetal magnetic resonance imaging because of previous ultrasonographic findings suggestive of fetal sacrococcygeal teratoma. Subsequently, they were submitted to ultrasonography for correlation of the imaging findings. Tumors size, location, extent and content were evaluated both at magnetic resonance imaging and ultrasonography. RESULTS: Findings regarding tumor location, size and content were similar for both methods. All the lesions were found in the sacrococcygeal region, with a mean size of 6.0 cm x 9.0 cm. As regards the tumors content, two of them were mixed solid-cystic, and one, entirely cystic. Magnetic resonance imaging was superior to ultrasonography in the evaluation of the exact tumor extent, accurately demonstrating pelvic involvement in all of the three cases. CONCLUSION: Fetal magnetic resonance imaging has showed to be a valuable adjunct to obstetric sonography in the evaluation of fetal sacrococcygeal teratoma, because of its higher accuracy in the determination of these tumors extent and content, playing a significant role in the therapeutic planning and increasing the chances of cure for these fetuses.

A Case of Sacrococcygeal Teratoma Diagnosed by Ultrasonography and MRI at 28 Weeks of Gestation / 대한주산의학회잡지

Sacrococcygeal teratoma is often diagnosed by ultrasound in the prenatal period. The fetus may develop high-output heart failure due to limited cardiac pumping ability and anemia secondary to sequestration of blood volume in the tumor. Eventually, fetal hydrops or placentomegaly may develop and these complications carry a grave prognosis. Therefore, careful monitoring by serial USG is needed, and any evidence of fetal hydrops should trigger prompt delivery or fetal intervention. Recently, fetal MRI may be helpful in assessing the exact tumor size, content, and intraabdominal extent to optimize prenatal and postnatal management. We experienced a case sacrococcygeal teratoma diagnosed by prenatal ultrasonography and fetal MRI at 28 weeks of gestation. At 31 weeks of gestation, preterm labor with sudden aggravation of polyhydramnios developed, we delivered the baby by Cesarean section due to concerns about fetal dystocia by the mass. Tumor was removed successfully 6 days after birth, and was diagnosed as immature teratoma. Thirteen months after delivery, the infant is healthy without any evidence of recurrence. So we present this case with a brief review of literatures.

Investigation of Magnetic Resonance Imaging Features and Treatment of Sacrococcygeal Teratoma in Children / 实用儿科临床杂志

Objective To analyze the magnetic resonance imaging (MRI) features of sacrococcygeal teratoma in children,and explore the diagnostic value of MRI and the critical suggestion for treatment.Methods The MRI features,clinical features,pathological data and operation complication of sacrococcygeal teratoma in 26 children were retrospectively analyzed. Phenotype teratoma in 9 cases grew from apex of coccyx to fundament.They presented spherical or ellipsoidal,distinct boundary and septatus in cystic MRI.All pathological data presented benign tumors.Mixed teratoma in 16 cases growed from fundament to cavitas pelvis.They presented dumbbell,distinct boundary,mixed signal of cystiform and solid density in MRI.There were 12 cases in benign tumors and 4 cases in malignant in pathology,the invisible teratoma in 1 case which lay between rectum and sacrum,extended to cavitas pelvis.Its boundary was not intact and segmented,and the pathological data presented malignant tumors.Results All of the 26 cases were completely excised only once and 21 of them received sacrococcygeal incision,5 cases had abdominosacral combined incision.Twenty-two cases were followed up (benign 20 cases,malignant 2 cases),and 2 cases of them died. 8 cases had complications,of the 8 cases,2 cases with necrosis of flap,1 case with chronic sinus tract and 2 cases with recidivism occurrd.Conclusions MRI may detect discovering the location and appearance of sacrococcygeal teratoma in children in diagnosis and in differential diagnosis of tumor nature.It is a very significant to reconstruct cavities pelvis and eradicase focal dead space in prevention and treatment of post-operation complication in childrens sacrococcygeal teratoma.

The role of fetal surgery in life threatening anomalies

The development and evolution of fetal surgery and the recognition of the fetus as a patient came from two sources. First, were those obstetricians and perinatologists who detected life threatening anomalies before birth, and re-described a hidden mortality arising from death in utero. Ultrasonography, color Doppler ultrasound and ultrafast fetal magnetic resonance imaging have since enhanced the accuracy of prenatal evaluation. Second, were those pediatricians responsible for treating newborn infants with extremely serious problems, and that appeared untreatable, although, it was believed that they could have been treated at an earlier stage of development. After the natural history of several correctable lesions had been determined and the selection criteria for intervention developed, fetal surgery emerged as a means of improving the overall morbidity and mortality rates.

A Case of Schinzel-Giedion Syndrome

Schinzel-Giedion syndrome is a rare autosomal recessive condition characterized by a typical face including midface retraction, urogenital anomalies, and skeletal manifestations. So far 30 patients have been reported. This is the first report of this syndrome demonstrated in Korea. We describe a male infant with the typical findings of Schinzel-Giedion syndrome, also showing sacrococcygeal teratoma.