Unfolding the Art of Methodical Approach for Total Sacrectomy.

BACKGROUND: Total sacrectomy is a technically demanding surgery with substantial risks, including high morbidity and mortality due to the likelihood of exsanguination.1-3 Despite the evolution of surgical techniques,4,5 the incidence of postoperative complications remains significant.1 This study presents a systematic approach to total sacrectomy, with a particular focus on a modified technique for isolating the iliac vessels, aimed at effective management of complex sacrococcygeal masses and the reduction of operative complications. PATIENTS AND METHODS: Employing our approach, a 45-year-old male patient presenting with a sacrococcygeal mass involving the lower S1 bone and sacroiliac joint underwent total sacrectomy. A meticulous preoperative workup, including magnetic resonance imaging (MRI), was followed by precise surgical steps: sigmoid colon and rectal mobilization, isolation of the iliac vessels,2,6 lumbosacral nerve trunk preservation, and strategic anterior and posterior osteotomies. The procedure concluded with reconstruction using mesorectal fat and bilateral gluteus maximus flaps.5-7 RESULTS: The patient's operation was conducted successfully without any perioperative complications, culminating in a chordoma resection with clear margins. Postoperative recovery was swift, allowing for discharge on the seventh day. CONCLUSIONS: The application of our systematic sacrectomy method, with particular emphasis on the isolation of the external iliac veins, significantly minimized intraoperative bleeding risks and other perioperative complications. Our technique offers a reproducible and effective strategy for the surgical management of sacrococcygeal masses.

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.

Impact of microscopic deposits of yolk sac tumor on recurrence of mature sacrococcygeal teratoma.

INTRODUCTION: Sacrococcygeal teratomas (SCT) with malignant histology frequently recur and are treated aggressively, but risk factors and surveillance protocols are less established for mature tumors. In particular, prior studies have not investigated whether microscopic deposits of yolk sac tumor (YST) in otherwise mature teratomas lead to higher recurrence rates. METHODS: We reviewed patients with mature SCTs resected at our institution from 2011 to 2021 and analyzed tumor characteristics, treatment, and outcomes. RESULTS: We identified 56 patients with mature SCT, of which 9 (16%) demonstrated microscopic YST. Following surgery, 7/56 (13%) patients developed local recurrence at a mean of 1.2 ± 0.7 years, while no patients developed metastases. Recurrence was more likely in patients with microscopic YST [5/9 (56%) vs. 2/47 (4%), p = 0.021] and positive margins [6/24 (35%) vs. 1/32 (3.1%), p = 0.030]. A solid tumor component tended to increase recurrence risk as well [6/29 (21%) vs. 1/27 (4%), p = 0.053]. Five patients demonstrated malignant recurrence and were all detected by a rising alpha-fetoprotein (AFP), while two patients demonstrated recurrence of mature teratoma and were detected on surveillance magnetic resonance imaging (MRI). CONCLUSIONS: Microscopic foci of YST may increase recurrence risk for patients with mature SCT. Such patients might benefit from closer postoperative surveillance with serial AFP measurements and MRI.

Current surgical approach: Extracranial malignant germ cell tumors.

Germ cell tumors (GCT) are a complex, heterogeneous collection of tumors that may present in either gonadal or extragonadal sites. They consist of a variety of benign and malignant histologies that can occur at several locations throughout the body. An important component of treatment is surgical resection, and while the key components of resection are site specific, the universal goals of GCT resection include the complete resection of tumor without violating the tumor capsule, while preserving function of surrounding organs, minimizing morbidity, and assessing for regional spread.

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.

Management of a complicated colonexposed sacrococcygeal wound after pelvic exenteration: a case report.

DECLARATION OF INTEREST: This work was supported by the Naval Medical University and the University of Shanghai for Science and Technology Joint Projects (2020-RZ04), the Innovative Clinical Research Program of Shanghai Changzheng Hospital (2020YLCYJ-Y16), and the academic project of Naval Medical University (2022QN073). The authors have no conflicts of interest to declare.

Reconstruction for extensive sacrococcygeal defects in complex tumor patients with personalized customized gluteus maximus myocutaneous flaps.

AIM: To evaluate the clinical effects of personalized customized gluteus maximus myocutaneous flaps (GMMF) for reconstruction of extensive sacrococcygeal soft tissue defects in complex tumor patients. METHODS: A retrospective chart review was conducted on 8 patients who underwent personalized customized GMMF reconstruction for large sacrococcygeal defect from December 2021 to August 2023. The personalized customized GMMF were designed based on the variations of tissue defect in location, shape and volume of different dead spaces. The principle of the personalized GMMF is to ensure that the rotation point of the flap can reach the farthest end of the defect. Patient demographics, operative characteristics, and perioperative risk factors were analyzed. Clinical outcomes were assessed, focusing on complications such as flap necrosis, wound dehiscence, infection, seroma, and hematoma. RESULTS: Six patients with rectal cancer and two with sacral tumors underwent personalized customized GMMF reconstruction for extensive sacrococcygeal defects. The average volume of the wound cavity was 104 mL, with a mean vertical depth was 10.8 cm. Six patients had low serum albumin (<35 g/L). After a mean follow-up of 15.5 months, no major complications occurred, except for one seroma that resolved within 2 weeks. CONCLUSION: The personalized customized GMMF described in this study is an effective method for reconstructing large sacrococcygeal wounds with significant depth in complex tumor patients. It allows for greater rotation of the muscle flap into the sacrococcygeal wound defect and provides adequate blood supply by utilizing the bulk of muscle tissue to obliterate dead space.

Prenatal diagnosis in estimating the prognosis of sacrococcygeal teratoma.

: Sacrococcygeal teratoma is a rare congenital malformation, the prognosis depends on factors affecting foetal development. The diagnosis is based on ultrasound examination, especially the evaluation of the detailed morphology of the foetus in the 20th week of pregnancy. Therefore, it is crucial to keep looking for ultrasound markers that would prenatally determine the most accurate prognosis for the foetus. Now, we rely on a small number of studies with a predominance of case reports. We offer a literature review of the essential information concerning sacrococcygeal teratoma diagnostics, therapy, and complications of sacrococcygeal teratomas in connection with prenatal diagnosis. It turns out that in cases with a favourable prognosis according to prenatal ultrasound examination and adequate surgical treatment after childbirth, the prognosis of this congenital malformation is excellent.

Isolated Sacrococcygeal Plexiform Neurofibroma in a Child.

This case report describes a 4-year-old girl with an isolated neurofibroma in the sacrococcygeal region. Although initially resembling sacrococcygeal teratoma, histopathology revealed a benign nerve sheath tumor. Wide local excision was performed, and the final diagnosis was plexiform neurofibroma. Diagnostic challenges in rare childhood tumors require stepwise evaluation and multidisciplinary team discussions.

Approach and Technique for Cesarean Section to Immediate Resection for High-Risk Sacrococcygeal Teratomas.

INTRODUCTION: Ex-utero intrapartum treatment has been established as an option for fetal and perinatal surgeons to deliver patients with sacrococcygeal teratomas (SCTs) which are causing significant fetal distress and possible in-utero fetal demise. However, ex-utero intrapartum treatment procedures carry significant maternal risk and morbidity. Herein, we report an alternative technique of Cesarean section to immediate resection (CSIR) for managing high-risk SCTs. METHODS: A retrospective institutional review board-approved review was performed on all SCTs evaluated at our fetal center from May 2014 to September 2020. Demographics; prenatal imaging characteristics; prenatal interventions; and postnatal surgery data including operative time, estimated blood loss, pathology, and outcomes were collected. Outcomes of interest included surveillance serum alpha-fetoprotein levels, imaging surveillance, developmental milestones, and the presence or absence of constipation or fecal incontinence. RESULTS: A total of 20 patients with prenatal diagnosis of SCT were evaluated. Mothers who transferred their care to another institution after diagnosis were excluded from this study. Twelve neonates underwent standard postnatal resection. Three neonates underwent emergent CSIR for high output cardiac failure, fetal anemia, or concerns for in-utero hemorrhagic rupture. The median (interquartile range) operative time was 231.5 (113) minutes for the standard operative group versus 156 min in the CSIR group. We present three patients who underwent immediate resection after emergent Cesarean section. We report 100% survival for the three consecutive cases. CONCLUSIONS: CSIR is a safe and feasible approach for managing appropriately selected high-risk SCTs with signs of hydrops, fetal distress, or fetal anemia. Despite patient prematurity, we demonstrated 100% survival of three consecutive cases. We suggest that CSIR be considered an option in the management algorithm for high-risk SCTs.

Imaging of pediatric extragonadal pelvic soft tissue tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee White Paper.

The most common pediatric extragonadal pelvic cancers include germ cell tumors, sacrococcygeal teratomas, and rhabdomyosarcomas (arising from the urinary bladder, prostate, paratesticular tissues, vagina, uterus, and perineum). This paper describes the radiological and nuclear medicine features of these entities and provides consensus-based recommendations for the assessment at diagnosis, during, and after treatment.

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.

Tumor lysis syndrome in premature infant prompting early resection of a large sacrococcygeal teratoma: a case report.

BACKGROUND: Sacrococcygeal teratomas (SCTs) are the most common congenital neoplasm and often require resection soon after birth. There are rare reports of cardiac arrest during surgery due to manipulation of the tumor triggering secondary necrosis and hyperkalemia. CASE PRESENTATION: This case describes a very preterm infant with a SCT who develops spontaneous preoperative tumor lysis syndrome (TLS). The medical team utilized rasburicase and the patient underwent total gross resection at 40 h of life. CONCLUSIONS: We emphasize the importance of the early recognition and management of tumor lysis syndrome in SCT with rasburicase, aggressive management of hyperkalemia and consideration of early resection of SCTs even in the case of a very premature infant.

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.

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.

Management strategy and outcomes of sacrococcygeal teratoma - an Egyptian multicenter experience.

OBJECTIVES: Nationwide criteria regarding patients with sacrococcygeal teratoma (SCT) are still lacking in Egypt. We aimed to present a multicenter study regarding the management and outcomes of this tumor to evaluate our national treatment strategy. METHODS: A retrospective analysis including all patients with SCT who were managed at four major Egyptian centers between 2013 and 2023. Clinical data, surgical approaches, and short- and long-term outcomes were discussed. RESULTS: The study included 95 patients (74 were females). Antenatal diagnosis was reported in 25% of patients. Seventy-one patients (74.7%) were classified as Altman type I/II. Surgery was performed via a perineal approach in 75 patients, whereas the remaining 20 underwent a combined abdominoperineal approach. Vertical elliptical incision with midline closure was conducted in 51.5% of patients, followed by classic or modified chevron incisions. Benign mature teratoma was detected in 82% of patients. At a median follow-up of 57 months, eight patients (8.5%) had relapsed. The 5-year overall survival (OS) and event-free survival (EFS) of all patients were 94% and 91%, respectively. In the after-care monitoring, 19 patients (20%) had urinary or bowel dysfunctions. Nine of them were managed using medications. Clean intermittent catheterization was practiced in another five patients. The remaining five underwent further surgical interventions. CONCLUSION: Favorable outcomes were achieved in our country during the last decade. Diverse perineal incisions were performed for resection, and vertical elliptical with midline closure was the commonest. During follow-up, 20% of patients developed urological or bowel dysfunctions that required medical and surgical treatment modalities to improve their quality of life.

An effective and considerable treatment of pilonidal sinus disease by laser ablation.

The treatment of sacrococcygeal pilonidal disease (SPD) is still challenging. Although many non-surgical and surgical methods exist, no consensus has been reached on the best treatment. This study aimed to evaluate the efficacy of laser ablation using 1470-nm radial diode laser fiber in treating SPD. We retrospectively studied the data of our 48 patients who operated on this technique between March 2019 and July 2022. All patients were treated with laser ablation using 1470-nm radial diode laser fiber. The healing rate and recurrence rate, demographic and surgical data, postoperative pain, complications (wound infection, wound bleeding), the time of returning to regular work and life, and the time of wound healing were recorded. Postoperative pain was measured based on the visual analog scale (VAS) score. Postoperative follow-up was performed in the outpatient clinic every 1 week for 1 month. Among the 48 patients, 41 males and 7 females, with a mean age of 27.7 years (range 14-42), the healing rate was 100%, and the average healing time was 28.3 ± 5.5 days. Mean operative time was 15.5 ± 3.3 min. The recurrence rate was 2.1%. One patient relapsed 3 months after the operation. The patient underwent laser ablation again, and the sinus tract was closed. The median visual analog scale (VAS) score on the day of operation was 0(0,2). The median VAS score on the first, third, seventh, and fourteenth day after operation was 0(0,2), 0(0,1), 0(0,1), and 0(0,0), respectively. There was no wound infection or bleeding after the operation. The mean time to normal work/life was 7.1 ± 3.2 days. Almost all the patients felt very satisfied with the operation. Laser ablation using 1470-nm radial diode laser fiber is effective in SPD treatment. It is associated with minor wounds and mild postoperative pain. It is a simple, safe, and minimally invasive technique and its clinical application for acute and chronic SPD in the absence of abscess is promising.

It is all about form and function: a new approach to giant neonatal sacrococcygeal teratomas.

INTRODUCTION: Giant sacrococcygeal teratomas (GSCTs) involve severe deformation of the buttock region in addition to potential functional impacts. Little interest has been given to improving the aesthetic post-operative appearance in children with these tumours. METHODS: We describe a new technique for immediate reconstruction of GSCTs using buried dermal-fat flaps and a low transverse scar in the infragluteal fold. RESULTS: Our technique allows wide exposure for tumour resection and functional restoration of the pelvic floor while placing the scars in anatomical locations and restoring buttock aesthetics including gluteal projection and infragluteal fold definition. CONCLUSION: Reestablishment of function and form should be kept in mind at initial surgery in GSCT surgery to maximize results and enhance post-operative outcomes. LEVEL OF EVIDENCE: IV.

Percutaneous versus Open Fetal Surgical Intervention for Sacrococcygeal Teratomas: Is Less Really More?

INTRODUCTION: Sacrococcygeal teratomas (SCTs) may require in utero intervention for survival. Open surgical intervention (OSI) was first described, but increasing reports of percutaneous intervention (PI) with variable indications and outcomes exist. We reviewed the literature for all published cases of fetal SCT intervention and compared OSI to PI cohorts. METHODS: A keyword search of PubMed was conducted. Inclusion criteria were as follows: data available per individual fetus including gestational age at intervention, type of intervention, primary indication, survival, gestational age at birth, and complications. Complications were grouped into categories: placenta/membrane, procedural, or hemorrhagic. Failure was defined as little/no improvement or recurrence of the primary indication. χ2 analysis was performed for solid tumor PI versus OSI to assess significant trends in these intervention groups. A meta-analysis was not feasible due to small numbers and heterogeneity. RESULTS: Twenty-seven articles met inclusion criteria. In the PI group, 38 fetuses underwent intervention for solid tumors, 21 for cystic tumors, and 3 for solid and cystic tumor components. Among fetuses with solid tumors, OSI was associated with lower need for multiple interventions (0% vs. 31.6%, p = 0.01) and higher survival to discharge (50% vs. 39.5%, p = 0.02). A fetal intervention was performed in the absence of hydrops/early hydrops in 45% of fetuses receiving PI, compared to 21% receiving OSI. Failure to resolve the primary indication was higher in the PI group (55.9% vs. 11.1% OSI, p = 0.02). The overall complication rates were high in both groups (90% OSI, 87% PI), though bleeding was unique to the PI group (26.5%). Preemptive cyst drainage, for purely cystic tumors, was universally successful and associated with a low complication risk (18.2%). CONCLUSIONS: For solid tumors, OSI appears to be superior with regard to survival to discharge, fewer interventions, and lower failure rates. PIs to drain a cyst may facilitate delivery or preempt future complications, though consideration should be given to long-term oncologic outcomes.

Effect of local oxygen therapy combined with vacuum sealing drainage on the healing of stage IV sacrococcygeal pressure ulcers.

The present study aimed to investigate the effect of local oxygen therapy combined with vacuum sealing drainage (VSD) on the healing of stage IV pressure ulcers sacrococcygeal. In this prospective study, we included a total of 98 patients with stage IV sacrococcygeal pressure ulcers in our hospital between February 2021 and June 2022. The patients enrolled were randomly and equally divided into two groups: the study group (undergoing local oxygen therapy combined with VSD treatment) and the control group (receiving conventional treatment). The wound healing time and hospital stay were compared between the two groups. Additionally, the wound area, tissue type, wound exudation and pain intensity were assessed before treatment, 10, 20, 30 and 40 days after treatment. The incidence of complications was also calculated. The study group demonstrated significantly shorter wound healing time and hospital stays compared to the control group (p < 0.05). Before treatment, there were no significant differences in terms of wound area, tissue type and wound exudation between the two groups (p > 0.05); after 10, 20, 30 and 40 days of treatment, however, evidently smaller wound areas, improved tissue types and reduced wound exudation were observed in the study group compared to the control group (p < 0.05). Furthermore, the study group exhibited increased microvascular count compared to the control group (p < 0.05). Before treatment, there was no significant difference in pain intensity between the two groups (p > 0.05), whereas markedly lower pain intensity was seen in the study group than in the control group after 10, 20, 30 and 40 days of treatment (p < 0.05). The incidence of complications did not significantly differ between the two groups after 40 days of treatment (p > 0.05). Local oxygen therapy combined with VSD was found to effectively accelerate the healing process of stage IV sacrococcygeal pressure ulcers, leading to shorter hospital stays and improved patient prognosis. This combined therapy shows promise for widespread application in clinical practice.