Pulsatile giant sacrococcygeal teratoma appearing like 2nd head.

Sacrococcygeal teratoma is a congenital tumor of early infancy. Eighty percent are present in the first month of life. It is considered to be an extragonadal germ cell tumor that can be diagnosed in utero. It is considered to be a rare tumor in infancy and childhood. Incidences in girls are more common as compared to boys. Association with other congenital abnormalities of the gastrointestinal system, cardiovascular system, and urological manifestations may be present. Association with spinal dysraphism may be present. A complete systemic evaluation needs to be done before any surgical intervention. We hereby present you a case of a 5-day-old male child, born from a non-consanguineous marriage, who presented to us with a large swelling at the coccygeal region. The mass had a variegated consistency with an ear-like appendage over it with associated hair, bones, and necrotic tissue in it. The patient was evaluated with imaging (MRI) which revealed a heterogeneously enhancing mass at the sacrococcygeal region with mild extension into the pre-sacral space suggesting sacrococcygeal teratoma. After a complete evaluation for congenital abnormal abnormalities of other sites, gross total excision of the mass was performed. The post-operative condition of the baby was uneventful. A biopsy of the mass revealed a mature cystic teratoma. The baby is under follow-up at present.

Variability in Surveillance Strategies Following Resection of Sacrococcygeal Teratoma.

INTRODUCTION: Surveillance following sacrococcygeal teratoma (SCT) resection varies. The purpose of this study was to describe the clinical characteristics and outcomes of patients undergoing SCT resection and examine current institutional practices to detect recurrence. METHODS: A single-institution retrospective review of children who underwent resection of an SCT from January 1, 2010 to December 31, 2020 was performed. Data were summarized and surveillance strategies compared between histopathologic subtypes using nonparametric methods. RESULTS: Thirty six patients (75.0% female) underwent SCT removal at a median age of 8 d. Histopathology revealed 27 mature teratomas (75.0%), eight immature teratomas (22.2%), and one malignant germ cell tumor (2.8%). Median postoperative follow-up was 3.17 y (interquartile range [IQR]: 2.31-4.38 y). Patients had a median of 2.32 clinic visits per year (IQR: 2.00-2.70), alpha-fetoprotein levels were obtained at a median of 2.01 times per year (IQR: 0-1.66), and surveillance imaging was performed at a median of 2.31 times per year (IQR: 0-2.84). Patients with immature teratomas had alpha-fetoprotein laboratories obtained more frequently than patients with mature teratomas (3.10 times/year versus 0.93 times/year, P = 0.001). There was no significant difference in the number of imaging studies obtained between groups. Two patients (5.6%) developed recurrence, which were identified on magnetic resonance imaging at 191 and 104 d postresection, respectively. CONCLUSIONS: Postoperative surveillance practices varied widely. Recurrence was noted in a single malignant case in the first year following resection. Multi-institutional studies are needed to determine the optimal surveillance strategy to detect recurrence of SCT.

Long-Term Outcomes of Infantile Sacrococcygeal Teratoma: Results from a Multi-Institutional Retrospective Observational Study in Japan.

BACKGROUND: Tumor recurrence, anorectal and urinary dysfunction, and lower limb dysfunction after surgery are observed in infantile sacrococcygeal teratoma (SCT). In this paper, a multi-institutional retrospective observational study was conducted to clarify the long-term functional prognosis in Japan. METHODS: This study was conducted using a paper-based questionnaire distributed to 192 facilities accredited by the Japanese Society of Pediatric Surgeons, covering patients who underwent radical surgery at less than 1 year old and who survived for at least 180 days after birth from 2000 to 2019. RESULTS: A total of 355 patients were included in this analysis. Altman type was I-II in 248 and type III-IV in 107, and the median maximum tumor diameter was 6.1 (range: 0.6-36.0) cm. There were 269 mature teratomas, 69 immature teratomas, and 10 malignant tumors. Total resection was performed in 325, subtotal or partial resection in 27, and surgical complications were noted in 54. The median postoperative follow-up was 6.6 (0.5-21.7) years. Eighty-three patients (23.4 %) had functional sequelae, including 62 (17.5 %) with anorectal dysfunction, 56 (13.0 %) with urinary dysfunction, and 15 (4.2 %) with lower limb motor dysfunction. Recurrence occurred in 42 (11.8 %) at a median age of 16.8 (1.7-145.1) months old. Risk factors for dysfunction included preterm delivery, a large tumor diameter, Altman type III-IV, incomplete resection, and surgical complications. Risk factors for recurrence included immature teratoma or malignancy, incomplete resection, and surgical complications. CONCLUSIONS: Postoperative dysfunction was not low at 23.4 %, and 11.8 % of the patients experienced recurrence occurring more than 10 years after surgery, suggesting the need for periodic imaging and tumor markers evaluations in patients with risk factors. It is necessary to establish treatment guidelines for best practice monitoring of the long-term quality of life. LEVEL OF EVIDENCE: Level II Retrospective Study.

Critical elements of pediatrics sacrococcygeal germ cell tumor surgery.

Sacrococcygeal teratoma is the most common extragonadal germ cell tumor in the pediatric population, and accounts for approximately 70% of all teratomas in childhood.1,2 They present in two distinct phases, with most cases seen in neonates with large predominately exophytic tumors, often detected in utero on prenatal sonography or at birth. A smaller cohort presents in older infants and children with primarily hidden tumors in the pelvis which have a much higher rate of malignancy. The primary surgical objective is complete tumor resection without compromise to critical structures or function. Herein we outline the critical elements of tumor resection and management of sacrococcygeal germ cell tumors with a focus on the technical aspects of this tumor across a range of presentations.

Surgical Neuropelveology: Laparoscopic Nerve-sparing Excision of a Pelvic Schwannoma.

STUDY OBJECTIVE: To show how advanced pelvic Schwannoma can be safely managed with a laparoscopic approach. DESIGN: Demonstration of the laparoscopic technique with narrated video footage. SETTING: Schwannomas are benign tumors that arise from well-differentiated Schwann cells (glial cells) of peripheral nerve sheaths. Schwannomas are nonaggressive, slow-growing, solitary masses with a low rate of malignant transformation and a low risk of recurrence after resection. They rarely occur in the pelvis, with a reported incidence of 1% to 3%. Tumors involving spinal nerve roots commonly present with radicular pain and nerve compression syndromes (Supplemental Video 1-3). This video shows the management of pelvic Schwannoma originating from the left sacral root S1 by a minimally invasive approach. INTERVENTIONS: Laparoscopic nerve-sparing excision of a pelvic Schwannoma. CONCLUSION: Historically, pelvic schwannomas have been managed mainly by laparotomy. Here, we demonstrate the feasibility and safety of a large pelvic Schwannoma excision by a minimally invasive approach.

Sequential teratoma in pediatric patients: causation or coincidence? / Teratoma de aparición secuencial en edad pediátrica: ¿casualidad o causalidad?

We describe the unusual case of a female patient with a history of two mature teratomas non-correlated in terms of location and occurrence. A 12-year-old girl presented at our consultation as a result of a growing tumor in the hypogastric region, with no further clinical signs. She had undergone surgery neonatally due to a mature cystic sacrococcygeal teratoma, which was fully removed. No clinical sequelae were noted and no additional treatment was required over a 10-year follow-up. Radiological examination showed a large 20 × 12 × 18 cm cystic mass extending from the pelvic region to the lower hemiabdomen, associated with two similar small formations adjacent to the right ovary. Tumor markers were negative, and a laparoscopic right salpingoophorectomy was carried out, with an excellent postoperative progression. Pathological examination revealed it was, again, a mature cystic teratoma. The genetic study ruled out causation in this respect.

Describimos el inusual caso de una paciente con antecedente de dos teratomas maduros no relacionados en cuanto a su localización y debut. Una niña de 12 años consultó por la aparición de una tumoración en la región hipogástrica de crecimiento progresivo sin otra clínica asociada. Había sido intervenida por un teratoma quístico maduro sacrococcígeo en el periodo neonatal con su extirpación completa y, ausencia secuelas clínicas y tratamiento adicional durante diez años de seguimiento posterior. Los exámenes radiológicos mostraron una gran masa quística de 20 × 12 × 18 cm que se extendía entre la región pélvica y el hemiabdomen inferior, acompañada por otras dos pequeñas formaciones similares adyacentes al ovario derecho. Los marcadores tumorales resultaron negativos y se llevó a cabo una salpingooforectomía derecha laparoscópica con una excelente evolución postoperatoria. El examen histopatológico, de nuevo, informó la lesión como teratoma quístico maduro. El estudio genético descartó una posible causalidad en este ámbito.

Primary immature teratoma of the thigh: a review.

Teratomas are a subtype of germ cell tumors composed of a variety of somatic tissues derived from more than one of the three germinal layers (ectoderm, endoderm and mesoderm). They can be classified as mature tumors and immature tumors. Teratomas most commonly arise at the sacrococcygeal region and the gonads. The occurrence of a teratoma outside the common gonadal and midline locations is exceptional. This review article lists the reported primary and metastatic malignant teratomas in extragonadal locations and discusses the possible explanation for the atypical location, their treatment and prognosis.

Sacrococcygeal teratomas: a case-based review from the perspective of individual multidisciplinary experts.

PURPOSE: Sacrococcygeal teratoma (SCT) is a rare congenital tumor originating from a variant of extragonadal germ cell neoplasm. Herein, we present three cases of neonatal SCTs undergoing surgical resection and reconstruction. We also review the literature to discuss the importance of proper perinatal management and timely surgical intervention depending on the tumor type and maturity to prevent malignant transformation and recurrence and ensure functional outcomes. METHODS AND RESULTS: Three cases of SCT were retrospectively analyzed. All infants underwent complete surgical resection of the SCT and coccygectomy during their neonatal/infancy period, followed by pelvic floor and buttock reconstruction while minimizing buttock contour deformity and undesirable skin scar. Two of the cases were histopathologically diagnosed as predominantly cystic mature teratomas of Altman types I and IV, and the third was a mature teratoma of Altman type II. There were no complications or tumor recurrence during the average follow-up period of 5.3 years. CONCLUSIONS: This case-based review highlights the role of multidisciplinary team approaches, including prenatal monitoring, oncologic resection, and adequate reconstruction according to the type of tumor and anomaly. Optimal perinatal evaluation allows promising oncologic and functional outcomes in terms of timely intervention to eliminate tumor recurrence and malignant transformation. Complete oncologic surgical resection of SCTs should also include proper functional preservation strategies, such as the reconstruction of the pelvic floor, cosmetic buttock contouring, and preservation of bladder and bowel sphincter function.

Sacrococcygeal teratoma: Long-term outcomes. A UK CCLG Surgeons Group Nationwide Study.

AIM: Sacrococcygeal teratoma (SCT) is a rare paediatric germ cell tumour (1:40,000). Long-term data regarding urinary tract and bowel function after SCT resection is limited to few studies. A UK Children's Cancer and Leukaemia Group (CCLG) Surgeons multicentre study aimed to critically analyse long-term functional outcomes in patients following resection of SCT. METHODS: Nationwide study of UK paediatric surgical oncology centres using a standardised data collection form. All index cases of newborn infants and children <16 years with SCT diagnosis during 2005-2015 were included. RESULTS: 165 SCT patients treated at 14 UK paediatric surgical oncology centres were included. Median age at presentation was 1 day [interquartile range, IQR: 0-25]; median age at surgery was 10 days [IQR: 4-150]. One hundred seventeen (70%) were female and 48 (30%) male. Antenatal diagnosis was made in 44% index cases. Total 59% of patients were Altman Stage I or II lesions. Follow-up data were available in 83% cases. Tumour recurrence occurred in 13 (7%) patients at median age 13 months [IQR: 8.75-30 months]. Fifty-nine (36%) of 165 patients had documented adverse bladder or bowel dysfunction. Twenty-two (37%) cases required urinary clean intermittent catheterisation (CIC) urology health care, with eight patients (14%) needing operative intervention to control management of bowel dysfunction. CONCLUSION: This UK CCLG study showed 36% of SCT patients develop bladder or bowel dysfunction after primary tumour resection. Functional assessment of bladder and bowel function is mandatory during after-care follow-up of all SCT patients. A multidisciplinary care pathway, with surgeon speciality groups including surgical oncology, paediatric urology and paediatric colorectal specialists, is strongly advised to facilitate 'best practice' monitoring of long-term health and improve patient quality of life (QoL) into adulthood.

Treatment of filum terminale pial arteriovenous fistulas in the sacral region: A case report and systematic review.

Spinal filum terminale pial arteriovenous fistulas (FT PAVFs) are uncommon. Most FT PAVFs are located in the lumbar region; far fewer are located in the sacral region. Due to the rarity of sacral FT PAVFs, the precise surgical dissection and removal of these lesions are challenging. Here, we report an FT PAVF in the sacral region. The patient was a 45-year-old male who suffered from progressive weakness and numbness of the bilateral lower limbs; his symptoms gradually worsened. Digital subtraction angiography (DSA) showed an AVF at the sacral canal at the S3-4 level. Microsurgical treatment with intraoperative DSA was performed, and the FT PAVF was resected. After the operation, the patient gradually recovered. Follow-up magnetic resonance imaging revealed a recession in the dilation of the spinal cord venous plexuses. A literature review was also performed, and a total of 14 FT PAVFs of the sacral region were identified. The patients identified in the literature review had an average age of 58.9 ± 12.9 years, and 92.9% of the patients were male. Spinal cord edema was present in 85.7% of the FT PAVF patients. Regarding treatment, 64.3% of the FT PAVF patients underwent microsurgical resection, 28.6% patients underwent endovascular treatment, and 7.1% patients underwent a hybrid operation; good outcomes were achieved with all three methods. Therefore, FT PAVF of the sacral region is a unique lesion whose angioarchitecture needs to be identified carefully; prompt treatment is necessary, and microsurgery can yield good outcomes.

Giant Sacrococcygeal Teratoma in a Neonate: A Case Report.

Sacrococcygeal teratomas are common tumours in neonates and infants, primarily affecting females. A 35-year-old primigravida presented with a large sacrococcygeal teratoma that was detected during the 30th week of gestation in the fetus. The baby was delivered via elective caesarean section at 36+3 weeks, and surgical excision of the 10x10x5 cm³ mass was performed successfully on the third day of life. Despite a surgical site infection, the patient had a favourable outcome with normal vital signs, bowel, bladder, and lower extremity functions upon discharge. Early diagnosis and prompt management of sacrococcygeal teratoma in newborns is vital for optimal outcomes, providing valuable insights and guidance to medical practitioners. Keywords: anaesthesia; case reports; neonate; teratoma.

A single-center experience of magnetic resonance imaging findings of fetal sacrococcygeal teratomas.

BACKGROUND: Sacrococcygeal teratomas (SCT) are known as rare tumors, but they are the most common tumor in fetuses and newborns. This study aims to present fetal magnetic resonance imaging (MRI) findings of SCT diagnosed prenatally and compare them with that of the prenatal Ultrasound (US) findings. METHODS: Eleven patients diagnosed as SCT prenatally by US and further assessed by MRI are included. MRI was performed via a 1.5-T magnet with a body coil. The presence, size, content extension, and compressive effects of each mass were determined and correlated with US findings and with postnatal examinations, including surgery and pathology. As complications, the presence of ascites and skin edema or pleural or pericardial effusion was diagnosed as hydrops. The amniotic fluid index was calculated for the assessment of oligo- or polyhydramnios. RESULTS: US findings are found strongly correlated with MRI findings. An agreement on the extent of each mass was observed in eight patients, disagreement in one fetus was an extension of the tumor within the spinal canal recognized only at MR and assessment of intrapelvic-abdominal extension was superior in MRI. There were n = 6 type I, n = 2 type II, n = 1 type III and, n = 2 type IV tumors. MRI was superior to US for detecting displacement of the colon (n = 3), intrapelvic-intraabdominal extension (n = 1), urinary tract complication (n = 2), and intraspinal extension (n = 1). DISCUSSION: MRI is found to be superior to US especially in the assessment of intrapelvic and intraspinal extension of the tumor, colonic displacement, and complications.

Laparoscopic Approach to Presacral Recurrence of Sacrococcygeal Teratoma.

A 6-month-old girl was presented to our hospital due to a presacral mass found 5 months after surgery of sacrococcygeal teratoma. The original tumor was a 63 x 50 mm sized round cyst connecting to the coccyx, observed with computed tomography. The initial operation was performed with en bloc removal of the tumor along with the coccyx in the prone position. During a routine follow up, ultrasonography indicated a possible local recurrence, 5 months after the initial operation. The magnetic resonance imaging revealed a polycystic formation with a diameter of 20 x 11 x 17 mm in the presacral space. The laparoscopic operation was conducted with the patient in the lithotomy and Trendelenburg position. The broad ligament of uterus was fixed to the abdominal skin and the rectum was mobilized to identify the tumor, which was resected laparoscopically. A histopathological examination showed the tumor to be a mature cystic teratoma. We observed her without any additional treatment and no recurrence is seen after 6 months.

Sacrococcygeal teratoma: evaluation of its approach, treatment and follow-up in two reference children cancer centers in Brazil / Rio de Janeiro.

INTRODUCTION: sacrococcygeal teratoma (TSC) is the most common tumor of the neonatal period. Alphafetoprotein is an important tumor marker and is used in the follow-up period as a marker of malignancy. The complete surgical resection of the tumor associated with coccygectomy is the standard treatment and chemotherapy in different stages are necessary. Follow-up consists of serial exam: tumor markers, imaging searching to possible metastasis sites, in addition to a complete physical examination. METHODOLOGY: a descriptive, retrospective, study was carried out by analyzing a chart of 25 patients of two different reference children cancer center; with TSC in the State of Rio de Janeiro from 2004 to 2019. The clinical and epidemiological data collected were described and a comparison was made between these two centers studied. RESULTS: the sociodemographic characteristics found were similar to those described in the medical literature. Data related to treatment and follow-up, such as the use of chemotherapy, use of specific imaging tests, digital rectal examination, and outpatient follow-up, differed between the two centers studied. There was a 25% loss of follow-up. CONCLUSION: the characteristic of being a non-cancer center can interfere with the full application of the current protocol for the treatment of sacrococcygeal teratoma. The knowledge of the data of the studied cases can allow the optimization of the approach of the patients with this pathology and generate discussions about the integral application of the specific therapeutic protocol in the medical centers that are qualified for such treatment.

Fetal sacrococcygeal immature teratoma - report of two cases and review of the literature.

Sacrococcygeal teratomas (SCTs) are rare congenital tumors. With the improvement of diagnostic imaging methods and follow-up protocols in pregnancies, in utero detection of these tumors has increased. Despite these progresses, SCTs may present difficulties in establishing in utero diagnosis and subsequent management. We present two cases of SCT in 18 weeks, respectively 22 weeks pregnancy, diagnosed using ultrasound imaging and pathologically confirmed. Also, the article aims to recapitulate clinicopathological aspects and prognosis of these lesions, following the review of the literature.

Surgical View of Morphological and Pathogenetic Identity of Pilonidal Cysts and Acne Inversa.

<b>Introduction:</b> As a rule, the treatment of pilonidal cysts is based on a surgical approach. Surgical treatment depends on the form of the disease and surgeon's preferences regarding the method. The treatment process does not consider preoperative treatment that would affect the structural components of the skin involved in the pathological process. The results of surgical treatment remain unsatisfactory, leading to a significant number of recurrences and long-term healing of the postoperative wound. </br></br> <b> Materials and methods:</b> Morphological examination of the pieces of skin with altered sacrococcygeal tissues after radical surgical treatment of 46 patients with pilonidal cyst of the sacrococcygeal region was performed, and 46 patients with acne inversa of the intergluteal cleft, groin and scalp were selected. The sex distribution of patients with pilonidal cysts was as follows: 43 (91.3%) male patients, 3 (6.5%) female patients. Among 46 patients with acne inversa and dissecting cellulitis, the distribution was as follows: 32 (69.6%) male patients and 14 (30.4%) female patients. Patients with pilonidal cysts of the sacrococcygeal region underwent surgical treatment according to the developed method of economical median resection using sutures with internal fixation. </br> </br> <b> Results:</b> Considering and comparing the morphological picture observed in the pathomorphological examination of histological specimens in patients with pilonidal cysts, acne inversa, dissecting cellulitis, the similarity of changes in most patients is noteworthy. "Acne inversa" was first described in 1839 by Velpeau, who originally called the disease "hydradenitis suppurativa", believing that inflammatory changes occur in the sweat glands [1-3]. In 1854, surgeon Verneuil described this disease, and later it was named after him. We observed chronic proliferative inflammation in different layers of the dermis and subcutaneous tissue (the main focus is in the subcutaneous tissue, but closely related to the dermis and epidermis). </br></br> <b>Discussions:</b> Pilonidal cyst of the sacrococcygeal region, especially without hair in the cavity of the cyst, can be considered as a type of acne inversa with appropriate location. We believe that the use of local and systemic retinoids, namely isotretinoin, in the treatment of patients with this disease other than surgery, together with other groups of drugs traditionally used in the treatment of pilonidal cysts, may be promising in the treatment of pilonidal cysts.

[Prenatal diagnosis and prognostic factors analysis of fetal sacrococcygeal teratoma].

Objective: To investigate the prenatal diagnosis and prognostic factors of fetal sacrococcygeal teratoma (SCT). Methods: A retrospective analysis was performed on 41 pregnant women who were diagnosed with fetal SCT by prenatal ultrasound at the Women's Hospital, Zhejiang University School of Medicine from January 2014 to September 2021. The prenatal imaging features and pregnancy outcomes, including tumor volume to fetal weight ratio (TFR), proportion of solid tumor, tumor growth rate (TGR), fetal hydrops, placentomegaly and polyhydramnios were analyzed. Receiver operating characteristic (ROC) curve was used to determine the critical values of TFR and TGR for predicting adverse fetal outcomes. Results: (1) Among the 41 pregnant women with fetal SCT, the diagnostic gestational week of ultrasound was (24.2±2.9) weeks (range: 18-28 weeks). Among them, 1 case progressed to fetal hydrops and induced labor at 22 weeks of gestation, 1 case developed intrauterine death and induced labor at 29 weeks of gestation, and 39 pregnancies continued until delivery. Among the 39 cases of continued pregnancy, 1 case underwent cesarean section at 31 weeks of gestation due to malignant polyhydramnios and increased fetal cardiothoracic ratio in the third trimester, 1 case underwent cesarean section at 32 weeks of gestation due to fetal heart failure, and 1 case underwent cesarean section at 32 weeks of gestation due to fetal heart failure and hydrops. The other 36 cases underwent surgical resection of tumor within 3 weeks after birth with good prognosis. (2) TFR>0.12 before 28 weeks of gestation could predict poor fetal prognosis, with a sensitivity of 100.0%, a specificity of 86.1% and an area under curve (AUC) of 0.922 (P<0.01). Among the fetuses with TFR>0.12, 5/10 had poor prognosis, while the fetuses with TFR≤0.12 all had good prognosis (100%,31/31), and the difference between the two groups was statistically significant (P<0.001). (3) TGR>48 cm3/week could predict poor fetal prognosis with a sensitivity of 100.0%, a specificity of 78.3% and an AUC of 0.880 (P<0.05). (4) Among the 28 SCT fetuses delivered in our hospital, the incidence rate of poor fetal prognosis was 0 (0/20) in those with solid tumor component<50%, and 5/8 in those with solid tumor component ≥50%, and the difference between the two groups was statistically significant (P<0.01). The incidence rate of poor fetal prognosis was 2/2 in those with placentomegaly (all with fetal hydrops), and 12% (3/26) in those without placentomegaly. The risk of poor fetal prognosis was 8.67 times higher in those with placentomegaly than those without placentomegaly, and the difference between the two groups was statistically significant (P<0.05). The incidence rate of poor fetal prognosis in those with polyhydramnios was 3/7, and 10% (2/21) in those without polyhydramnios, but there was no statistically significant difference between the two groups (P>0.05). Conclusion: TFR combined with solid tumor morphology, TGR, and presence of placentomegaly could predict the adverse pregnancy outcomes of fetal SCT.