Malignant Sacrococcygeal Teratoma Research Articles

Pregnancy and Long-Term Postnatal Outcomes of Congenital Sacrococcygeal Teratoma: A Single Institution's 18-Year Experience.

The objective of this study is to evaluate outcomes of fetal sacrococcygeal teratoma (SCT) from an academic tertiary center. This is a retrospective study evaluating pregnancy and postnatal outcomes of fetal SCT management at a single institution between 2006 and 2023. Results are reported as median (range). Fourteen patients with fetal SCT were studied. Hydrops fetalis occurred in 2 (14.3%) cases. Pregnancy course included expectant management in 12 (85.7%) and palliative preterm induction in 1 patient (7.1%). Indications for delivering timing included fetal deterioration in 7 patients (50%), preeclampsia in 3 (21.4%), elective induction of delivery in 3 (21.4%), and preterm labor in 1 (7.1%). Delivery GA was 36.4 weeks (26.4-40.1 weeks), with 11 (78.5%) cesareans and 3 (21.4%) vaginal deliveries. There were 13 (92.9%) live births and 11 (78.6%) long-term survivors. Postnatal resection pathology demonstrated 7 (53.8%) mature, 3 (23.1%) immature, and 3 (23.1%) malignant SCTs. Of the 11 long-term survivors, 3 (27.3%) had teratoma recurrences, including 2 (18.2%) with metastatic disease requiring chemotherapy. Notable long-term complications involved gastrointestinal (n = 7, 63.6%), genitourinary (n = 4, 36.4%), and musculoskeletal (n = 2, 18.2%) systems. SCT confers significant morbidity to both the pregnant patient and neonate. Multidisciplinary prenatal and postnatal care is needed to comprehensively manage this complex condition.

Pediatric presacral tumors with intraspinal extension: a rare entity with diagnostic challenges.

The presacral space is a potential space located between the rectum and the lumbosacral spine. It contains various primitive germ cell types that serve as the origin for a range of tumors. Imaging is crucial in characterizing, assessing the extent of and evaluating the treatment response to these tumors. We report a series of six cases of pediatric presacral tumors with intraspinal extension, including an immature sacrococcygeal teratoma (Altman type II), a malignant sacrococcygeal teratoma (Altman type IV), a neuroblastoma, a rhabdomyosarcoma, a clear cell sarcoma and an Ewing's sarcoma of the ilium. These tumors can be broadly categorized as tumors of germ cell, neuroblastic, mesenchymal and osteogenic origin. Despite overlapping imaging features, a review of the existing literature and careful retrospective observation revealed several distinctive features that aid in the optimal characterization of tumors. These include the tumor's epicenter, the pattern and degree of bone involvement, the status of sacral foramina and neural elements, and internal tumor characteristics such as the presence of fat, calcification, hemorrhage and necrosis.

A fetal case of a large sacrococcygeal teratoma

Rationale: Sacrococcygeal teratoma (SCT), derived from early embryonic pluripotent stem cells, is one of the most common neoplasms in fetuses. We report a case of a large mass in the fetal sacrococcygeal region detected by ultrasonography at a gestational age of 16+ weeks. Patient concerns: A 26-year-old woman, gravida 2, para 1, was referred to our hospital with a mixed echo protrusion (3.6 × 2.8 × 2.8 cm in the fetal sacrococcygeal region. Diagnoses: Ultrasound scan showed that the sacrococcygeal mass had grown to 7.1 × 5.8 cm. Magnetic resonance imaging revealed a large exophytic mass about 7.7 × 4.5 × 6.5 cm in the perineum of the fetus. Pathological examination revealed malignant SCT. Interventions: The patient was closely monitored and followed-up through prenatal examinations to assess the condition of the mother and child. Outcomes: The fetus was found dead in utero at 20-week gestation. The pregnant woman then induced labor. Lessons: SCT is a common germ cell tumor in fetuses. Attention should be paid to maternal and fetal conditions due to serious complications. Appropriate interventions during pregnancy and timely surgery after delivery are recommended.

Adult sacrococcygeal teratoma: a retrospective study over eight years at a single institution.

To determine the clinical, imaging, and histological features, and surgical resection modalities and outcomes of adult sacrococcygeal teratoma (SCT). 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. 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. 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.

Malignant sacro-coccygeal teratoma with growing teratoma syndrome

Abstract A 13-month-old female with sacro-coccygeal tumor type II by Altman, was proven by biopsy to be a malignant germ cell tumor. Serum tumor markers were normal. During neo-adjuvant chemotherapy visible tumor progression was confirmed radiologically and progressed despite changing chemotherapy. The external growing mass forced its way out of the skin. More importantly, CT showed resolution of the pelvic component. Complete tumor resection with coccygectomy was performed. Histopathology showed mature teratoma, immature cells in the coccyx and no malignant germ cells. Three months after ending her therapy she remains in remission. We report the first case of growing teratoma syndrome (GTS) in the coccygeal region.

Malignant transformation of sacrococcygeal teratoma with voluminous mesentery cyst: a case study conducted in Bamako

Malignant sacrococcygeal teratoma is a rare congenital embryonal tumor at high risk of malignant transformation. We report the case of a 20-month old female infant. She was the couple's fourth child. Her mother was 29 years old, she had four pregnancies with vaginal deliveries. All the infants were born alive. The woman was not properly followed during pregnancy. She had vaginal delivery at term and had correctly attended expanded programme on immunization (EPI) with positive reaction to BCG vaccine. The child walked autonomously at 13 months. She had painful fluctuating inguinal and left labium major swelling (A), distended abdomen with oozing wound measuring 4cm/3cm on the right buttock (A). Frontal x-rays of abdomen without preparation showed distended flaccid abdomen with opacity in the periumbilical region as well as in the hypogastric region (B). Ultrasound objectified voluminous calcified sub-vescical tissue mass measuring 87*54 mm (C). Thoracoabdominal CT scan showed calcified sacrococcygeal tissue mass measuring 83*59 mm, with modest fissure in the left pubis and abdominal cyst without wall (D,E). Moreover, there were nodules of variable size in the lungs and the liver (F,G). The infant was treated by an oncopediatric team.

Malignant Sacrococcygeal Teratoma with Yolk Sac Differentiation in a Child—A Case Presentation

Sacrococcygeal teratoma (SCT) is a common congenital neoplasm, contains derivatives of more than one of the three embryonic germ cell layers. However, malignant Sacrococcygeal Yolk Sac tumor (YST) is an extremely rare extra-gonadal germ cell tumor. This case describes a two and half years old female child presenting with history of swelling at sacrococcygeal region for nine months. Case was evaluated clinically. Patient’s serum alpha fetoprotein (AFP) level was elevated abnormally. FNAC of the swelling was done which shows suspicious cell for immature teratoma. Swelling excised and histopathological examination was carried out, the report of which shows malignant sacrococcygeal teratoma with yolk sac tumor.

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.

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.

Solid Cancers in the Premature and the Newborn: Report of Three National Referral Centers

Advances in multidisciplinary care for pediatric cancer have resulted in significant improvement in cure rates over the last decades; however, these advances have not been uniform across all age groups. Cancer is an important cause of perinatal mortality, yet the full spectrum of malignant neoplasms in newborns is not well defined. The authors have reviewed the clinical features and outcomes of 37 newborns with congenital malignant tumors treated at three referral centers in North, Central, and South Poland between 1980 and 2014. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier methods and compared using long-rank test and Cox models. Twenty-two patients were diagnosed prenatally. The most common diagnoses were neuroblastoma (48.7%), followed by malignant germ-cell tumor (16.2%), and Wilms' tumor (8.1%). Neuroblastoma was the most common malignancy among full-term infants, and malignant sacrococcygeal teratoma was the most common malignancy in premature infants. Thirty patients (81%) are alive with a median follow-up of 4.8years from diagnosis. Patients with Wilms' tumor and malignant germ-cell tumors had the best outcomes (5-year OS 100% for both), whereas the worst prognosis was observed for sarcoma patients (5-year OS 72.92%). Premature infants had better outcome than full-term infants (5-year OS 92.8% vs. 72.58%, respectively). Although rare, neonatal cancers can present with an aggressive clinical behavior, but they have a generally good outcome. Early diagnosis and management by expert multidisciplinary teams that integrate perinatal medicine experts with pediatric and surgical oncologists are critical. Centralized care with clear referral pathways that facilitate early initiation of specialized treatment should be prioritized.

An interesting presentation of a sacrococcygel teratoma with an associated neuroendocrine tumor: A case report

Abstract Teratomas are the most frequently occurring germ cell tumors, with 45–65 percent occurring in the sacrococcygeal region [1]. The case presented below involves a newborn male with a malignant sacrococcygeal teratoma and a number of congenital deformities including a left lower extremity amputation, an absent left kidney, hypoplastic and absent left hemipelvis musculature. After excision of the teratoma and subsequent chemotherapy treatment, the patient returned with a persistent abnormal soft tissue, containing persistent malignant elements around the left femur, suspicious for a neuroendocrine tumor. This case shows a rare association between two exceedingly uncommon malignancies.

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.

Surgical Outcomes in Adults With Benign and Malignant Sacrococcygeal Teratoma

Adult sacrococcygeal teratomas are rare, and limited data exist on their management and outcomes following surgery. The aim of this study was to review the outcomes in adult patients undergoing surgery for sacrococcygeal teratomas. A retrospective review of our prospectively maintained surgical pathology and tumor registries was conducted. This study was conducted at the Mayo Clinic in Rochester. Information was collected on patients treated between 1980 and 2013. A total of 26 patients with sacrococcygeal teratoma were identified (19 female), with a median age of 37.5 years. Malignancy was seen in 5 patients. Data on demographics, clinical presentation, tumor pathology, adjuvant therapy, surgical approach, surgical margins, use of preoperative biopsy, radiological investigations, morbidity, mortality, and local recurrence was collected. Complications were assessed by using the Clavien-Dindo system of classification. Patients most commonly presented with pelvic pain (n = 16) and/or a palpable mass (n = 15). On radiology, 8 lesions were purely cystic, 14 were mixed, and 4 were solid; teratoma was suspected as a diagnosis in 8 patients. Preoperative biopsy (13 patients) had 100% concordance with final pathology. Median tumor size was 6 cm, and the surgical approach was posterior only (n = 15), anterior only (n = 5), and combined anterior-posterior (n = 6). Of 5 patients with malignancy, 3 died of recurrent disease. Limitations of this study include the small number of patients, the long study period, and the heterogeneous nature of these tumors. Presacral teratomas require multidisciplinary management and have a risk of malignant transformation. They are more common in females, and the majority are intrapelvic in location in adults. We recommend clinical evaluation, radiological investigation, and image-guided biopsy in all suspicious presacral lesions. A treatment algorithm has been designed to improve the management of these rare tumors.

Pattern of Childhood Malignant Tumour in the Paediatric Surgery Department of Bangabandhu Sheikh Mujib Medical University

Background: Childhood malignancy is fast becoming an important paediatric problem in Bangladesh. They differ markedly from adult malignancy in their nature and distribution. This is a 5 years retrospective study of childhood malignant tumour as seen at the paediatric surgery department of Bangabandhu Sheikh Mujib Medical University (BSMMU). Objectives: The objective of this study is to determine the types of malignant patients admitted in the paediatric surgery department of BSMMU and also to know their age and sex distribution. Methods: This is a 5 years retrospective study from January 2005 to December 2009 in the paediatric surgery department of BSMMU, Dhaka, Bangladesh. All admitted malignant patients, aged up to 15 years diagnosed by means of histological or cytological examination included in this study. Results: Seventy ( 70) malignant patients were admitted during the study period, 65.7% patients were male and 34.3% patients were female with a male female ratio of 1.9:1. Fifty two (74.3%) patients were below 5 years and eighteen (25.7%) patients were between 5 to 15 years. The Wilms’ tumour was the most common malignant tumour which accounted for 42.8%. Hepatoblastoma was the second most common (24.2%). The other malignant tumours were neuroblastoma (15.7%), Non-Hodgkins lymphoma (5.7%), Rhabdomyosarcoma (4.2%), malignant fibrous histocytoma (1.4%), Fibroscarcoma (1.4%), Osteosarcoma (1.4%), malignant sacrococcygeal teratoma (1.4%), carcinoma of the rectum (1.4%0. Conclusion: Based on the result of this study, Wilms’ tumour is the commonest paediatric solid malignancy in our country. Carcinoma of the rectum may rarely occur in paediatric age group. Key words: Frequency; malignant solid tumor; children. DOI: http://dx.doi.org/10.3329/bsmmuj.v4i2.8638 BSMMU J 2011; 4(2):99-101

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

The timing of surgery for malignant sacrococcygeal teratoma is controversial. The long-term outcomes and complications of surgery for this rare tumour are presented. 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. 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. Surgery for malignant sacrococcygeal teratoma is safe and has a low complication rate. The long-term outcomes are favourable with minimal side effects.

Extradural ependymal tumor with myxopapillary and ependymoblastic differentiation in a case of Schinzel–Giedion syndrome

Primary extradural ependymomas are rare neoplasms usually of the myxopapillary type. Reports on malignant primary extradural ependymal tumors are exceptionally rare. We here report on a 3-year-old boy with Schinzel-Giedion syndrome (SGS), who presented with lumbar spina bifida occulta and a progressive extraspinal lesion in the subcutaneous sacrococcygeal region. Microscopic examinations revealed an uncommon ependymal tumor with well-differentiated regions reflecting myxopapillary ependymoma and highly anaplastic regions with numerous mitoses, necroses, ependymal rosettes and ependymoblastic rosettes. Final neuropathologic diagnosis was an extraspinal anaplastic ependymal tumor with myxopapillary and ependymoblastic differentiation, corresponding to WHO grade IV. SGS is a very rare disorder with a likely autosomal recessive pattern of inheritance. So far, 42 cases have been reported, among them 7 were diagnosed to have malignant neoplasms, including three malignant sacrococcygeal teratomas, two sacrococcygeal primitive neuroectodermal tumors (PNET), one hepatoblastoma and one malignant kidney tumor. The present case is the first report on an ependymal tumor with a mixture of myxopapillary, anaplastic and ependymoblastic features and the first report on an ependymal tumor arising on the genetic background of SGS.

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.

A Massive Pulmonary Tumor Embolism After Surgical Manipulation and Biopsy of a Pelvic Mass

A Massive Pulmonary Tumor Embolism After Surgical Manipulation and Biopsy of a Pelvic Mass

Near-Haploidy in a Malignant Sacrococcygeal Teratoma

Cytogenetic analysis of a malignant sacrococcygeal teratoma in an adult patient revealed near-haploid (77%), near-diploid (19%), and polyploid (4%) cells. The near-haploid cells had a karyotype of 25,XX,der(5)t(5;7)(p15;p13),+7,der(9)t(6;9)(p21;q34),r(17)(p13q25). In the near-diploid and polyploid cells identical copies of the structural chromosomal changes were found. Although some of the anomalies observed appear unique to this case, a common breakpoint in chromosome 6 was previously reported as specific in a subgroup of extragonadal germ cell tumors of adults.