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Neonatal tumors occur infrequently; sacrococcygeal teratoma (SCT) is a rare and abnormal mass often diagnosed on antenatal ultrasound. An SCT may cause serious antenatal complications, requires surgery in the neonatal period, and can lead to various long-term sequelae including fecal incontinence or constipation, urinary incontinence, and lower extremity mobility impairment. Even rarer are SCTs that include intraspinal extension necessitating complex neurosurgical intervention to relieve possible spinal cord compression or tumor tissue resection. A comprehensive understanding of the natural history of SCT provides frontline neonatal nurses and nurse practitioners with the expertise and language to support families during an infant's NICU admission. A glossary of key terms accompanied by a case review of a premature infant born with a large external SCT with intrapelvic and intraspinal components aids in enhancing knowledge related to the potential impact of an SCT on the central nervous system., (© Copyright 2020 Springer Publishing Company, LLC.)

Study Objective: Our main purpose was to describe the surgical technique and short-term outcomes of single-incision laparoscopic sacrocolpopexy (S-LSC) for the treatment of pelvic organ prolapse (POP)., Design: This study consisted of a retrospective analysis of 49 consecutive cases., Setting: This study was set at the Third Affiliated Hospital of Guangzhou Medical University from October 2016 to November 2017., Patients: The population for this study consisted of women with stage II to IV POP who met eligibility criteria for laparoscopic surgery., Interventions: S-LSC included the use of V-loc barbed suture and retroperitoneal tunneling, in addition to standard single-incision laparoscopic surgery techniques. All 49 cases were successfully completed. All cases included concomitant procedures; 42 (85.7%) had removal of the uterus and adnexa. The main measured outcomes include patient characteristics, perioperative outcomes, and change in pelvic floor support (Pelvic Organ Prolapse Quantification System), and quality of life (Pelvic Floor Impact Questionnaire)., Measurements and Main Results: All patients were parous, and 42.9% had a history of previous abdominal surgery. The mean operative duration from skin to skin was 201.20 ± 46.53 minutes. The mean estimated blood loss was 27.0 ± 16.6 mL. The mean pre- and post-operative Pelvic Organ Prolapse Quantification System scores were 2.2 ± 1.1 cm versus -2.6 ± 0.5 cm for the Aa point and 3.2 ± 2.8 cm versus -4.6 ± 0.8 cm for the C point (p <.05 for both). The mean pre- and post-operative Pelvic Floor Impact Questionnaire scores were 106.4 ± 18.9 versus 8.9 ± 4.26 (p <.05), suggesting that S-LSC significantly improved physical prolapse and quality of life. Four patients suffered from postoperative complications (3 mesh exposure and 1 lumbosacral pain). Six patients complained of new onset of stress urinary incontinence., Conclusions: Single-incision laparoscopic sacrocolpopexy is a feasible method to manage POP. However, the long-term effects and complications need to be further investigated., (Copyright © 2019 AAGL. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate the efficiency of percutaneous intratumor laser ablation for fetal solid sacrococcygeal teratoma (SCT)., Subjects and Methods: We carried out percutaneous ultrasound-guided intratumor laser ablation through a 17-gauge needle using an output of 40 W in 7 fetuses with large solid SCT and reviewed the literature for minimally invasive therapy for this condition., Results: Laser ablation was carried out at a median gestational age of 20 (range 19-23) weeks, and in all cases there was elimination of obvious vascularization within the tumor and improvement in cardiac function. Three (43%) babies survived and had surgical excision of the tumor within 2 days of birth, 3 liveborn babies died within 5 days of birth and before surgery, and 1 fetus died within 2 weeks after the procedure. In previous series of various percutaneous interventions for predominantly solid SCT the survival rate was 33% (2/6) (95% CI 9.7-70%) for endoscopic laser to superficial vessels, 57% (4/7) (95% CI 25-84%) for intratumor laser, 67% (8/12) (95% CI 39-86%) for intratumor radiofrequency ablation, and 20% (1/5) (95% CI 3.6-62%) for intratumor injection of alcohol., Conclusions: In solid SCT, the reported survival from intratumor laser or radiofrequency ablation is about 50%, but survival does not mean success, and it remains uncertain whether such interventions are beneficial or not because the number of fetuses is small and there were no controls that were managed expectantly., (© 2019 S. Karger AG, Basel.)

Objective: To evaluate the magnetic resonance (MR) signal intensity changes in the sacrococcygeal region of patients with uterine fibroids treated with high intensity focused ultrasound (HIFU). Materials and Methods: Two hundred and sixty-seven patients with uterine fibroids treated with HIFU between January and December 2016 were retrospectively reviewed. All patients underwent enhanced pre- and post-HIFU MRI. Multivariate analysis was used to assess the relationship between the factors and the signal intensity changes in the sacrum and the soft tissue adjacent to the sacrum. Results: Among the 267 patients, 122 (46%) had MR signal intensity changes in the sacrum and/or the soft tissue adjacent to the sacrum after HIFU. Multivariate analysis showed that the position of the uterus, the distance from the dorsal side of the fibroid to the sacrum, and the ablation efficiency were significantly correlated with MR signal intensity changes in the sacrum and the soft tissue adjacent to the sacrum. Further analysis showed a significant relationship between the location of the MR signal intensity changes and uterine size, the enhancement degree of the uterus. Leg pain was only seen in patients with MR signal intensity changes both in the sacrum and the soft tissue adjacent to the sacrum. Conclusions: The location of the uterus, the distance between the dorsal side of the fibroids to the sacrum, and ablation efficiency have a significant relationship with the MR signal intensity changes. The size of the uterus and the degree of enhancement are related to the locations of MR signal changes.

Background: Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups., Methods: A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017. Data captured included age at surgical resection, type of ARM, tumor location within the pelvis, tumor histopathology, tumor size, adjuvant chemotherapy, recurrence, and follow-up., Results: Forty-six patients comprised our cohort, of whom 12 had an ARM. The median age was older at resection for those with an ARM (1.4 years; range 1 day to 29.4 years) compared to those without an ARM (9 days; range 0 days to 6.9 years) (p = 0.01). The mean tumor size was 2.5 cm in patients with an ARM compared to 6.0 cm in patients without an ARM (p = 0.036). All patients with ARM had exclusively intrapelvic tumors, and histopathology included mature teratoma (8), yolk sac tumor (1), lipoma (1), and unknown (2). Tumor location for patients with sacral and presacral masses without ARM included exclusively extrapelvic (10), primarily extrapelvic with large intrapelvic component (7), primarily intrapelvic with extrapelvic component (1), exclusively intrapelvic (8), and unknown (8). Histopathology for patients with presacral masses without ARM included mature teratoma (20), immature teratoma (7), yolk sac tumor (3), ganglioneuroma (1), neuroblastoma (1), benign epithelial cyst (1), and unknown (1). Tumor recurrence rate was similar between patients with ARM (n = 3, 25%) and those without an ARM (n = 5, 15%) (p = 0.41). The 5-year event free survival was 65% (95% CI: 25%-87%) in the group with ARM and 81% (95% CI: 60%-92%) in the group without ARM (p = 0.44)., Conclusion: Sacral and presacral masses in patients with ARM are resected at a later age and are more likely to be intrapelvic. They appear histologically similar and have similar rates of recurrence and malignancy when compared to patients without ARM., Level of Evidence: III TYPE OF STUDY: Retrospective comparative study., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Skin tags are fairly common lesions usually seen in adults on the neck and in body folds. The sacrococcygeal region is an unusual location for skin tags in children and may represent a congenital malformation of the spine or an isolated skin lesion. In this review, we summarize the clinical presentation, histopathologic changes, and differential diagnosis of coccygeal polypoid eccrine nevus as a rare cause of sacrococcygeal papules in children.

Objective: We aimed to explore the efficacy of keystone flap combined with vacuum-assisted closure (VAC) in the repair of sacrococcygeal wounds., Methods: This study is a retrospective review of patients undergoing keystone flap reconstruction between January 2014 and January 2018. A standardized data collection template was used to collect related variables. The detailed process of the reconstructive surgery is carefully described in this study. The postoperative healing process was closely observed., Results: Twelve patients underwent keystone flap repair between January 2014 and January 2018. The average wound size before closure measured 7.83 ± 1.93 × 5.28 ± 0.91 cm. All the patients achieved primary wound healing and the flaps survived during the follow-up period, which ranged from 1 to 24 months. No severe complications and obvious scar appeared, and the patients were satisfied with both appearance and function., Conclusions: The application of keystone flap combined with VAC is a promising way to repair wounds in the sacrococcygeal region with little postoperative complication and similar soft-tissue thickness to the surrounding tissue., (© 2019 Wiley Periodicals, Inc.)

The main, but not sole, indication for an Ex-utero Intrapartum Treatment (EXIT) delivery is an airway obstruction due to either laryngeal atresia or tumors in the head and neck region. Here we present our Institution's experience with eleven cases: three teratomas, four lymphatic malformations, two laryngeal atresias and two dermoid cysts. The EXIT procedure was used to secure the fetal airway while maintaining uteroplacental gas exchange and fetal hemodynamic stability through the umbilical circulation. Five fetuses required tracheostomy. Only one fetal death occurred due to extensive growth of a teratoma preventing us from establishing an airway. No other fetal or major maternal complication occurred. The EXIT procedure is a complex procedure and these rare cases should be referred to a center with a dedicated and experienced multidisciplinary team.

We report here a rare case of adult sacrococcygeal teratoma (SCT) that was pathologically diagnosed as signet ring cell carcinoma (SRCC). A 26-year-old man complained of lower abdominal distension and discomfort and difficulty in urinating, and he was admitted to our hospital. Pelvic magnetic resonance imaging showed multiple oval, solid-cystic masses around the anterior sacrococcygeal region that measured approximately 96 × 114 × 89 mm. Magnetic resonance imaging also showed irregular cysts around the posterior sacrococcygeal region that measured approximately 34 × 72 × 60 mm. The preliminary diagnosis was cystic SCT. The patient then underwent surgery, during which we incised the cysts. A large amount of viscous, jelly-like liquid was present in the anterior sacrococcygeal mass. Furthermore, a large amount of light yellow, porridge-like secretion was present in the posterior sacrococcygeal mass. A pathological examination and immunohistochemistry confirmed teratoma, specifically SRCC.

A tailgut cyst (retrorectal cystic hamartoma) is an uncommon lesion that develops in the presacral (retrorectal) space. Malignant change in a tailgut cyst is extremely rare and presents as a soft tissue (presacral) or bone (sacral) neoplasm. We report a case of tailgut cyst in which a neuroendocrine tumor developed in a 25-year-old female. Computed tomography and magnetic resonance imaging scans revealed a sacrococcygeal malformation with absent left S4 and S5 and a partly cystic lesion within the right presacral space. Histologically, the lesion contained cystic and solid elements. The cysts were lined by columnar and stratified squamous epithelial cells with underlying patchy smooth muscle. The solid element was a partly necrotic neuroendocrine tumor composed mainly of ribbons of tumor cells, which showed mitotic activity and expressed cytokeratin, chromogranin, and synaptophysin. Histologically, tailgut cysts are lined by epithelium and contain scattered smooth muscle bundles in the cyst wall. Although rare, the possibility of tailgut cyst with neuroendocrine tumor should be included in the differential diagnosis of an enlarging presacral tumor.

Autorzy przedstawiają u 73 letniej kobiety torbiel ogonowo-jelitową przestrzeni pozaodbytniczej, która uległą zezłośłiwieniu. Już w trzecim roku życia była leczona, jako przetoka ropna pośladka prawego, która się okresowo uaktywniała. W 2016roku zgłosiła się do Poardni Chirurgicznej w Gorlicach z olbrzymim ropniakiem pośladka prawego z przetoką ropną . Na oddziale chirurgicznym w Gorlicach operacyjnie usunieto ropniak o ścianach dobrze ukształtowanych razem z przetoką. Torbiel zawierała ponad litr ropy. Po wygojeniu w bliźnie pojawiłą się przetoka śluzowa .Badaniem histologicznym rozpoznano retrocecal cyst hamartoma z komórkami mucinos adenocarcinoma. W czasie następonego pobytu chorej w szpitalu po wykonaniu rezonansu magnetycznego stwierdzono resztkowa zmianę w okolicy przedkrzyżowej sięgająca do trzeciego kręgu krzyżowego. Zmianę usunięto radykalnie potwierdzoną badaniem histopatologicznym. U chorej nawrotu nie zaobserwowano.

Acquired urethrovaginal fistulae and urethral atresia are rare findings in pediatric patients, but have been described in adult patients related to trauma or iatrogenic injury. Little exists in the published literature to guide management of such conditions in children, but lessons learned from congenital causes can help. Herein we discuss the preoperative evaluation and management of a child with an acquired urethrovaginal fistula and urethral atresia likely related to in utero compression from an intrapelvic sacrococcygeal teratoma and provide several images detailing the complex anatomy., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Rationale: Tailgut cysts are a type of very rare congenital polycystic lesions that are always located posterior to the rectum and anterior to the sacrum. The symptoms of tailgut cysts are nonspecific and misleading. Therefore, a full understanding of the characteristics of tailgut cysts is important for their diagnosis and treatment., Patient Concerns: We report a 46-year-old female with a sacrococcygeal mass that had been present for about 2 years., Diagnoses: Pelvic magnetic resonance imaging (MRI) indicated several cystic masses, the largest of which had a diameter of about 2.8 cm, near the ligaments of the sacrospinous and rectum. Pathological examination confirmed the tailgut cysts, with no signs of malignant transformation., Interventions: The patient received complete surgical cysts resection, which was performed through the sacrococcygeal region., Outcomes: The patient recovered well with no obvious complication and was discharged on the sixth-day post-surgery. Follow-up 6 months later revealed no signs of cyst recurrence., Lessons: The symptoms and diagnostic characteristics of tailgut cysts should be fully understood. Complete surgical resection through the sacrococcygeal region is vital to treat tailgut cysts.

Aims: Retrorectal tumors are rare, mostly benign tumors and named due to their localization. Diagnoses of these tumors are usually delayed because of nonspecific complaints and symptoms. Magnetic resonance imaging has beneficial uses both for diagnosis and treatment. In this study, we reviewed a case series of retrorectal tumors., Subjects and Methods: The patients who were diagnosed with retrorectal tumors between 2008 and 2015 were analyzed. This investigation was conducted at a Tertiary Education and Research Hospital. Sixteen patients were included in this study. Patients' demographic data, imaging workups, surgical operation reports, pathologic examination results, postoperative complications, and follow-up results were examined. Descriptive statistics, median, and standard deviation for continuous variables were used. The primary outcomes measured were diagnostic conflict, knowledge, and preference for surgery., Statistical Analysis Used: Definitive statistical methods (mean, standard deviation, median, frequency, and percentage) were used to evaluate the study data., Results: One patient refused operation and one was in preoperative preparation period. Fourteen of sixteen patients were operated. Two (14.3%) of operated patients have malignant histopathological result (one gastrointestinal stromal tumor, one ganglioneuroblastoma). Rest of the operated patients' histopathological reports was as follows: Four schwannomas, three epidermoid cysts, two tailgut cyst, one dermoid cyst, one teratoma, and one angiomyolipoma. Eight patients were operated by posterior incision, five patients with transabdominal approach, and one patient with combined approach., Conclusions: Retrorectal tumors are rare cases, and treatment of retrorectal tumors is surgery and should be operated in referenced hospitals to avoid diagnostic and therapeutic problems., Competing Interests: None

An 8-y-old, intact female degu ( Octodon degus) was presented with a slow-growing mass on the tail tip. The mass was completely removed by partial caudectomy. Histologically, the last coccygeal vertebra was replaced by a lobulated neoplasm composed of large clear polygonal cells embedded in a myxoid alcian blue-positive matrix with highly vacuolated cytoplasm (physaliferous cells) and intracytoplasmic periodic acid-Schiff-positive granules. The neoplasm exhibited the morphologic features of a "classic" chordoma of humans, which is 1 of 3 distinct chordoma subtypes. Immunohistochemistry revealed dual expression of cytokeratin AE1/AE3 and vimentin, consistent with a diagnosis of chordoma. Chordomas are uncommon slow-growing neoplasms in humans and animals, arising from notochordal remnants. Depending on their subtype and location, they can have a high local recurrence rate and metastatic risk. Chordoma should be included in the differential diagnosis of a soft tissue mass on the tail of a degu, similar to the clinical situation in ferrets.

Objective: Tumor volume to fetal weight ratio (TFR) > 0.12 before 24 weeks has been associated with poor outcome in fetuses with sacrococcygeal teratoma (SCT). We evaluated TFR in predicting poor fetal outcome and increased maternal operative risk in our cohort of SCT pregnancies., Methods: This is a retrospective, single-center review of fetuses seen with SCT from 1997 to 2015. Patients who chose termination of pregnancy (TOP), delivered elsewhere, or had initial evaluation at > 24 weeks were excluded. Receiver operating characteristic (ROC) analysis determined the optimal TFR to predict poor fetal outcome and increased maternal operative risk. Poor fetal outcome included fetal demise, neonatal demise, or fetal deterioration warranting open fetal surgery or delivery < 32 weeks. Increased maternal operative risk included cases necessitating open fetal surgery, classical cesarean delivery, or ex utero intrapartum treatment (EXIT)., Results: Of 139 pregnancies with SCT, 27 chose TOP, 14 delivered elsewhere, and 40 had initial evaluation at > 24 weeks. Thus, 58 fetuses were reviewed. ROC analysis revealed that at ≤24 weeks, TFR > 0.095 was predictive of poor fetal outcome and TFR > 0.12 was predictive of increased maternal operative risk., Conclusion: This study supports the use of TFR at ≤24 weeks for risk stratification of pregnancies with SCT., (© 2018 S. Karger AG, Basel.)

Background: Alpha-fetoprotein (AFP) is useful as a tumor marker for sacrococcygeal teratoma (SCT). We investigated the half-life of AFP in SCT., Methods: Neonates who underwent surgical treatment for SCT between 1997 and 2016 were included in the study, whereas patients who died before or after surgery or had malignant germ cell tumors were excluded., Results: Fifty-five non-recurrent SCT patients (M:F = 18:37) were enrolled. They underwent surgery on average 7.4 ± 4.1 days after birth. Serum AFP was measured an average 4.25 ± 2.07 times per patient. We obtained 165 half-lives following the formula (M = Mo * (1/2) Δt/T ). A positive correlation was observed between half-life and patient age using the formula T 1/2  = 0.0597 × days +6.1643 (p < 0.001). It was different from recurrent SCT (T 1/2  = 0.1196 × days -0.0633) (p < 0.05). Half-life was different between mature SCT (T 1/2  = 0.0671 × days +4.3912) and immature SCT (T 1/2  = 0.0433 × days +8.9339) (p < 0.05)., Conclusion: The half-life of AFP in neonatal patients with SCT was prolonged in proportion to the age, and it was getting longer in recurrent tumor than non-recurrent tumor. The half-life of AFP was longer in immature teratoma than in mature teratoma., Level of Evidence: IV., (Copyright © 2018 Elsevier Inc. All rights reserved.)

With limited information about the coccygeal glomus found in classic textbooks, we deemed it necessary to review the subject. The illustrations presented in this article derive from four coccygeal glomera incidentally encountered during examination of pilonidal disease specimens. Familiarization with its microanatomical features may help to avoid inappropriate interpretation of this enigmatic structure., (Copyright © 2018 Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.)

Competing Interests: Competing interests:The BMJ has judged that there are no disqualifying financial ties to commercial companies. The authors declare the following other interests: Navin Pinto has received grants from Baldrick’s Foundation, a cancer research foundation for his work on cyclophosphamide pharmacogenomics.

Neuroendocrine tumors (NETs) arising from tailgut cysts are a rare but increasingly reported entity with gene expression profiles that may be indicative of the gastrointestinal cell of origin. We present a case report describing the unique pathological and genomic characteristics of a tailgut cyst NET that metastasized to liver. The histologic and immunohistochemical findings were consistent with a well-differentiated NET. Genomic testing indicates a germline frameshift in BRCA1 and a few somatic mutations of unknown significance. Transcriptomic analysis suggests an enteroendocrine L cell in the tailgut as a putative cell of origin. Genomic profiling of a rare NET and metastasis provides insight into its origin, development, and potential therapeutic options., (© 2018 Erdrich et al.; Published by Cold Spring Harbor Laboratory Press.)

Study Design: Systematic review and meta-analysis of observational studies., Objective: The aim of the study is to evaluate different treatment modalities in the management of sacrococcygeal chordoma., Summary of Background Data: Chordomas are primary malignant bone tumors associated with considerable morbidity and mortality., Methods: We searched MEDLINE, EMBASE, Cochrane Central-Register of Controlled Trials, and Scopus from inception to July 2015. Eligible studies included patients with sacrococcygeal chordoma treated exclusively with surgery, radiotherapy, or both. Two reviewers independently assessed the eligibility of potential studies, risk of bias, and extracted data. Outcomes of interest were all-cause mortality, progression-free survival, and metastases. We analyzed further surgical outcomes by resection margin. All outcomes were assessed at 60 months and more than 60 months following intervention., Results: We included 33 noncomparative studies reporting on 501 patients (mean age 57 years). Overall mortality rate was (16%) after surgical resection with adjuvant radiotherapy and (28%) after surgical resection, and (43%) after radiotherapy (P = 0.28). All-cause mortality following wide surgical resection was (32%) compared to (40%) after marginal resection (P = 0.51). Overall progression-free survival rate was (58%) after surgical resection with adjuvant radiotherapy and (55%) after surgery (P = 0.92). However, at more than 60 months follow-up, progression-free survival rates were significantly higher (P = 0.024) following surgical resection with adjuvant radiotherapy (74%) in comparison to surgery (55%) and radiotherapy (36%). Overall progression-free survival rates were nonsignificantly higher after wide surgical resection (66%) than marginal resection (33%) (P = 0.16). However, at 60 months follow-up, progression-free survival rates were significantly higher following wide surgical resection (73%) than marginal resection (33%) (P = 0.047)., Conclusion: Sacrococcygeal chordoma is a difficult to treat disease entity. Until comparative studies become available, wide surgical resection and multidisciplinary management are the recommended approaches to improve patient outcomes., Level of Evidence: 3.

Introduction: Neuroendocrine tumors (NET) within tailgut cysts (TC) are usually seen in middle-aged females. A 14 year-old boy who underwent surgical excision of NET within TC is presented to discuss the clinical features, diagnosis and treatment options., Case Report: A 14-year-old boy with rectal bleeding and constipation had a 5 × 4 cm mass detected upon rectal examination. The cystic mass was totally excised. The histopathological evaluation revealed a low-grade NET within mucularis layer of the cyst showing a positive immunhistochemical staining for cytokeratin and synaptophysin., Conclusion: Although, the malignant transition of TC is commonly observed in middle age females, males and children can also be affected. This is first report of a pediatric TC with malignant transformation. Total surgical excision of cysts is mandatory for local control of the disease and chemotherapy should be reserved for the patients who have positive surgical margins.

Objectives: In sacrococcygeal teratoma, the presence of high-output cardiac failure resulting from arteriovenous shunting through the large tumor has been associated with an adverse outcome. The objective of this study was to determine whether the tumor size and cardiac biomarkers in cord blood can predict neonatal survival in sacrococcygeal teratoma., Methods: The study population consisted of 25 neonates with sacrococcygeal teratoma. Tumor size was calculated by the ellipsoid formula using dimensions measured by antenatal ultrasound ([length × width × depth in cm] × 0.52= volume in cm 3 ). To adjust the gestational age, the tumor volume index (tumor volume/biparietal diameter) was adopted in the analysis. Cardiac biomarkers for heart failure (N-terminal pro-B-type natriuretic peptide [NT-pro-BNP] and cardiac troponin T [cTnT]) were measured in cord blood taken at the time of delivery., Results: The rate of neonatal death was 24% (6 of 25). The cases that resulted in neonatal death had a higher tumor volume index and higher concentrations of NT-pro-BNP and cTnT than those with survival. A tumor volume index of greater than 60 cm 3 /cm, elevated NT-pro-BNP (>2000 pg/mL), and elevated cTnT (>0.08 ng/mL) had sensitivity of 100% for prediction of neonatal death., Conclusions: The tumor volume index and cord blood biomarkers for heart failure can be promising prognostic markers for neonatal survival in sacrococcygeal teratoma., (© 2018 by the American Institute of Ultrasound in Medicine.)

Purpose: Ectopic nephrogenic rests (ENR) are extremely rare, and their diagnosis and treatment have not yet been standardized. Our study aimed to analyze the clinical and pathological features of ENR in children and explore the optimal methods for diagnosis and treatment., Methods: A retrospective, single-center, case series study of 13 children with ENR was performed. Clinical manifestations, work-up, pathological features, and treatment methods were analyzed, and the postoperative status was evaluated at follow-up., Results: The study cohort included seven males and six females aged 1/4-19 months, who were divided into two subgroups: group 1 (pure ENR, cases 1-3) and group 2 (ENR associated with teratoma, cases 4-13). The patients commonly presented with a painless mass or an incidental finding in surgery. The ENR were detected in the lumbosacral and sacrococcygeal region (five cases), retroperitoneal area (five cases), testis (one case), gubernaculum testis (one case), and stomach (one case). Radiological findings showed a mixed-density mass or a mixed-signal-intensity lesion in group 2. All the patients underwent surgical resection, and all except two were event-free in a follow-up period of 2-154 months. Case 8 showed recurrence, and extended surgery was performed. Case 7 developed metastases and received chemotherapy., Conclusions: ENR are rare among children and have no specific clinical manifestations, lab tests, and radiological features. Different treatment strategies may be adopted based on the diagnosis. Complete resection can generally be achieved, and the prognosis is excellent in most cases. However, relapse or metastasis may occur in a small subset of patients. Close follow-up is mandatory to detect recurrence and metastasis early., (© 2018 Wiley Periodicals, Inc.)

Background: Sacrococcygeal teratoma accounts for the most common solid tumor in neonates. Because of improved technology, 50%-70% of cases can be diagnosed antenatally during routine ultrasound screenings. If not diagnosed antenatally, clinical findings at birth are distinct in most cases including a palpable or visible mass., Case: We report an unusual case of a 1-year-old girl who presented with persistent vaginal discharge leading to diagnosis of a mucosal polypoid lesion of the vagina, ultimately revealing a hidden sacrococcygeal teratoma., Summary and Conclusion: We suggest thorough investigation of all infants who present with purulent discharge and recurrent vaginal mass; sacrococcygeal teratoma should routinely be considered as a differential diagnosis., (Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.)

Type IV sacrococcygeal teratoma with intraspinal involvement is rare and to our knowledge has not been reported previously in the literature. The authors present the case of a 2-month-old infant with a type IV sacrococcygeal teratoma diagnosed on prenatal ultrasound. Postnatal MRI revealed intraspinal extension through an enlarged sacral neuroforamina on the right side. On surgical exploration, the authors discovered a dorsal cystic tumor involving the sacral spine that extended through an enlarged S4 foramen to a large presacral component. The tumor was successfully removed to achieve a complete en bloc surgical resection. The authors review the epidemiology, pathophysiology, and treatment of sacrococcygeal teratomas with intraspinal extension.

Aim: Neonatal tumours represents less than 2% of all childhood cancers. The biological behaviour of this tumours will differ in older children. The tumours's biological differences and the immature physiological characteristics of newborns represent a great therapeutically challenge making newborns vulnerable. The aim of this study is to describe the clinical characteristics, associated malformations, diagnostic methods, treatment and the outcomes of neonatal tumours., Methods: Retrospective review of patients ≤ 28 days-old with diagnosis of neonatal tumour between 2000-2016. Statistical analysis of clinical characteristics, histology, diagnostic methods, treatment and morbimortality., Results: A total of 26 tumours were diagnosed in newborns with a mean age of 4.85 ± 8.9 days and 69.2% of boys. Prenatal diagnosis was achieved in 38.5% (n = 10) and 38.5% (n = 10) in the first week of age. Associated malformations were found in 30.6% (n = 8). The most frequent tumours were hepatic hemangioma 23.1% (n = 6), neuroblastoma 15.4% (n = 4) and sacrococcygeal teratoma 11.5% (n = 3). Medical treatment was indicated in 7.7% (n = 5), surgical 57.7% (n = 15) and observation 30.8% (n = 7). Global mortality was 19.23% (n = 5) of which 42.9% (n = 3/7) were perioperatively., Conclusions: The management of neonatal tumours require a multidisciplinary approach to minimize the consequences and assure the best outcome. Global mortality is low and depends primarily of the physiologic and association of other malformations of the newborn.

Introduction: The aim of this study is to evaluate late side effects that affect quality of life in children with sacrococcygeal teratoma (SCT)., Patients and Methods: The patients with SCT were evaluated retrospectively. The data were expressed by percentage and the subgroups were compared statistically., Results: A total of 40 children with SCT were identified with median age 12 days (range: 1 day-14.6 years), 27 of whom were analyzed in this study with urodynamic data available for 24 and anal manometric evaluations for 20. Chronic constipation with need for laxative was reported in (7/27) 25.9%, fecal incontinence was present in (1/27) 3.7%, and urodynamic abnormalities were reported in (16/24) 66%. Among those with urodynamic abnormalities, low bladder capacity, dyssyergia and neurogenic bladder were observed in (21/24) 87.5% and anticholinergic treatment was applied. Urinary incontinence was present in (2/27) 7%, with clean intermittent catheterization utilized in (7/27) 25.9%. While defecation was observed more in the patients with Altman types II, III, and IV, micturation problems were observed more in the patients with Altman types II and IV. It was found that urodynamic dysfunctions were more frequent in the patients with increased number of operations., Discussion: Although the rate of symptomatic patients was low, abnormalities determined by radiological and urodynamic evaluations were high.

Introduction: R0 resection is achieved by high sacrectomy for local recurrence of colorectal cancer, but significant rates of perioperative complications and long-term patient morbidity are associated with this procedure. In this report, we outline our unique experience of using an expandable cage for vertebral body reconstruction following S1 sacrectomy in a 66-year-old patient with re-recurrent rectal cancer. We aim to highlight several key steps, with a view to improving postoperative outcomes., Technique: A midline laparotomy was performed with the patient in supine Lloyd-Davies position, demonstrating recurrence of tumor at the S1 vertebral body. Subtotal vertebral body excision of S1 with sparing of the posterior wall and ventral foramina was completed by using an ultrasonic bone aspirator. Reconstruction was performed using an expandable corpectomy spacer system. The system was assembled and expanded in situ to optimally bridge the corpectomy. The device was secured into the L5 and S2 vertebrae by means of angled end plate screws superiorly and inferiorly. Bone grafts were positioned adjacent to the implant after this., Results: Total operating time was 266 minutes with 350 mL of intraoperative blood loss. There were no immediate postoperative complications. The patient did not report any back pain at the time of discharge, and no neurological deficit was reported or identified. Postoperative CT scan showed excellent vertebral alignment and preservation of S1 height., Conclusion: We conclude that high sacrectomy with an expandable metal cage is feasible in the context of re-recurrent rectal cancer when consideration is given to the method of osteotomy and vertebral body replacement.

Sacrum is a triangular bone placed in the base of the spine and formed by the synostosis of five sacral vertebrae (S1-S5). Its upper part is connected with the inferior surface of the body of L5 vertebra forming the lumbosacral joint, while its lower part is connected with the base of the coccyx forming the sacrococcygeal symphysis, an amphiarthrodial joint. The existence of four pairs of sacral fora-mina in both anterior and posterior surface of the sacrum is the most common anatomy. Nevertheless, supernumerary sacral foramina are possible to be created by the synostosis of lumbosacral joint or sacrococcygeal symphysis. We present a case of an osseous cadaveric specimen of the sacrum belonging to a 79-year-old Caucasian woman. A rare variation of the anatomy of the sacrum is reported; in which, the simultaneous fusion of the sacrum with both the L5 vertebra and the coccyx has created six pairs of sacral foramina. This variation should be taken into serious consideration, especially in the domain of radiology, neurosurgery, orthopaedics and spine surgery, because low back pain, coccygodynia and other neurological symptoms may emerge due to mechanical compression. (Folia Morphol 2018; 77, 2: 397-399).

Introduction: Fournier's gangrene (FG) is a type of necrotizing fasciitis of the perineum and scrotum that is characterized by very rapid progression. Sacral pressure ulcers are one of the causes of FG., Case Report: An 85-year-old man was referred to the National Hospital Organization Nagasaki Medical Center (Ohmura City, Japan) with a diagnosis of FG extending to the retroperitoneum caused by a sacral pressure ulcer. Immediate debridement was performed; however, it was not possible to remove all necrotic tissue from the pelvis. The wound was cleansed with continuous irrigation combined with negative pressure wound therapy, which brought the infection under control. The exposed rectum was resurfaced with a gracilis musculocutaneous flap, and the remaining wound in the sacral region was covered with a gluteal artery perforator flap., Conclusions: The authors consider continuous irrigation combined with negative pressure wound therapy as extremely useful for patients with FG in whom sufficient debridement cannot be performed.

Rationale: Teratomas, which most frequently affect adult females, are the most common type of germ cell tumor, it always comprises derivatives of at least 2 germ layers. The most common site of primary teratomas is the ovary. Sacrococcygeal teratomas (SCTs), which are exceedingly rare in adults, are generally found in newborns or children., Patient Concerns: A 39-year-old woman presented to our clinic with a 1-year history of gradually aggravated difficulty in micturition and defecation, and a tumor in her right buttock present since birth. Appropriate preoperative examinations showed a large (15.6 cm × 12.2 cm × 30.0 cm) multicystic SCT., Diagnoses: Histologic examination confirmed a mucinous tumor arising in a giant SCT., Interventions: Abdominoperineal rectal resection was performed., Outcomes: The patient recovered well and was discharged on day 33 of admission., Lessons: We report the first case of a mucinous tumor arising in an SCT, in which the teratoma presented mature tissue elements derived only from the endodermal germ layer (keratinous debris)., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Primary and secondary neurulation are the two known processes that form the central neuraxis of vertebrates. Human phenotypes of neural tube defects (NTDs) mostly fall into two corresponding categories consistent with the two types of developmental sequence: primary NTD features an open skin defect, an exposed, unclosed neural plate (hence an open neural tube defect, or ONTD), and an unformed or poorly formed secondary neural tube, and secondary NTD with no skin abnormality (hence a closed NTD) and a malformed conus caudal to a well-developed primary neural tube., Methods and Results: We encountered three cases of a previously unrecorded form of spinal dysraphism in which the primary and secondary neural tubes are individually formed but are physically separated far apart and functionally disconnected from each other. One patient was operated on, in whom both the lumbosacral spinal cord from primary neurulation and the conus from secondary neurulation are each anatomically complete and endowed with functioning segmental motor roots tested by intraoperative triggered electromyography and direct spinal cord stimulation. The remarkable feature is that the two neural tubes are unjoined except by a functionally inert, probably non-neural band., Conclusion: The developmental error of this peculiar malformation probably occurs during the critical transition between the end of primary and the beginning of secondary neurulation, in a stage aptly called junctional neurulation. We describe the current knowledge concerning junctional neurulation and speculate on the embryogenesis of this new class of spinal dysraphism, which we call junctional neural tube defect.

OBJECTIVE Sacrococcygeal dimples in neonates and infants are of uncertain pathological import. Previously they were believed to be rarely associated with intraspinal anomalies. Recent studies using MRI, however, revealed that 6%-7% of pediatric cases of sacrococcygeal dimples were associated with anatomical tethered spinal cord (TSC). Because the prevalence of tethered cord syndrome is still unclear, there is no consensus among pediatric neurosurgeons on the management of children with sacrococcygeal dimples. The authors performed an analysis of MRI and urodynamic studies to validate their management strategy for pediatric cases of sacrococcygeal dimples. METHODS A total of 103 Japanese children (49 male and 54 female, median age 4 months, range 8 days-83 months) with sacrococcygeal dimples who were referred to the Division of Pediatric Neurosurgery between 2013 and 2015 were included in this study. The lumbosacral region of all the patients was investigated using MRI. Anatomical TSC was defined as a condition in which the caudal end of the conus medullaris is lower than the inferior border of the L2-3 intervertebral disc. Patients with minor spinal anomalies (e.g., anatomical TSC, filum lipoma, thickened filum, or filar cyst) underwent further urodynamic studies to ascertain the presence of neurogenic bladder (NGB). In this study, the presence of NGB without anatomical TSC but with other minor spinal anomalies was defined as "functional TSC." The prevalence of anatomical and functional TSC was investigated. The association of the following cutaneous findings with spinal anomalies was also assessed: 1) depth of the dimple, 2) deviation of the gluteal fold, and 3) other skin abnormalities (e.g., discoloration, angioma, or abnormal hair). RESULTS The children were classified into 4 groups: Group 1, patients with anatomical TSC; Group 2, patients with functional TSC; Group 3, patients without anatomical or functional TSC but with other minor spinal anomalies; and Group 4, patients with no spinal anomaly. There were 6 patients (5.8%) in Group 1, 8 patients (7.8%) in Group 2, 10 patients (9.7%) in Group 3, and 79 patients (76.7%) in Group 4. Twenty-four patients (23.3%; Groups 1, 2, and 3) showed MRI abnormalities, including filum lipoma (14 cases), filar cysts (5 cases), thickened filum (2 cases), and anatomical TSC without other spinal anomalies (3 cases). Untethering of the spinal cord was indicated for 14 patients (13.6%; Groups 1 and 2) with anatomical and functional TSCs. Preoperative NGB was found in 12 patients and improved postoperatively in 7 (58.3%). None of the associated lumbosacral skin findings predicted the presence of underlying spinal anomalies. CONCLUSIONS The prevalence of tethered cord syndrome among children with sacrococcygeal dimples was, for the first time, revealed to be higher than previously thought. MRI and supplemental urodynamic studies may be indicated for children with sacrococcygeal dimples to identify patients with symptomatic TSC.

Objectives: The purpose of this study was to describe selected sacral tissue characteristics in a convenience sample of healthy volunteer subjects., Design: Descriptive., Sample and Setting: Fifty healthy volunteers in a clinical learning center in a school of nursing., Methods: Sacral scans were obtained using a 20-MHz ultrasound scanning system in 3 positions: prone and 60° and 90° side-lying from the back. The images were analyzed by software in the ultrasound program using quantitative parameters of dermal thickness and density (dermal median intensity and derived number of low-echogenic pixels to total pixels [LEP:TP] ratio)., Results: In general, average values were as follows: dermal thickness between 2.32 and 2.65 mm; median pixel intensity between 102 and 112; and the LEP:TP ratio between 0.39 and 0.56. There were significant differences in sacral tissue characteristics between measures of thickness and dermal density (median intensity and LEP:TP ratio) by subject side-lying position (60° and 90°) versus prone position, with all P values less than .0001., Conclusions: Overall, the ranges were consistent across measures of thickness and dermal density except for systematic differences between side-lying and prone positions. When comparing thickness, median intensity, or LEP:TP ratio, it is important to report subject position. To best recognize tissue inflammation indicative of pressure injuries before surface changes are seen, it is useful to understand healthy high-frequency ultrasound sacral tissue characteristics. It is anticipated that quantitative assessment of dermal thickness, density, and LEP:TP ratio could help identify individuals with incipient pressure injury.

Study Design: Twenty-six patients were treated surgically for sacral osteosarcomas in our center. The oncological and functional results are analyzed., Objective: To describe the general characteristics and evaluate the outcome of surgical treatment of primary osteosarcoma of the sacrum., Summary of Background Data: A very few reports have addressed the technique and outcome of surgical treatment of primary sacral osteosarcoma. The survival rate of such cases is low., Methods: The clinical data of 26 patients treated surgically for sacral osteosarcomas from June 2000 to December 2013 at our hospital were retrospectively reviewed., Results: There were 15 males and 11 females with a median age of 28 years (range, 12-68 years). Distal metastasis occurred in 13 patients (50%), and local recurrence occurred in 10 patients (38.5%, including 6 patients with additional distal metastasis). The 1-year and 5-year survival rates were 92.3% and 38.7%, respectively. Adequate and inadequate surgical margins were obtained in 16 and 10 cases, respectively. Patients with adequate margins had a significantly lower recurrence rate (P = 0.015) and higher event-free survival rate (P = 0.04) than those with inadequate margins. However, the overall survival rate did not differ significantly between patients with adequate and inadequate margins (P = 0.22). Only seven cases showed a good response based on necrosis rate (>90%). Patients with a better response to chemotherapy had better event-free and overall survival rates., Conclusion: Adequate surgical margins are achievable in most cases of sacral osteosarcomas. Adequate margins can significantly improve the recurrence rate and event-free survival rate compared with inadequate margins. However, sacrectomy is still a challenging surgical technique. Adequate margins are also difficult to obtain in sacrectomy. In addition, only a few patients had a good response to chemotherapy. These reasons lead to a low 5-year survival rate in primary sacral osteosarcoma patients., Level of Evidence: 4.

Background: Teratomas are tumors that contain tissues derived from three different germ cell layers and having a wider range of differentiation with different site and age at presentation. The aim of the present study was to know the frequency of teratomas in various sites and to analyze their clinicomorphological features., Materials and Methods: The present study is a retrospective study conducted in tertiary care hospital of Central India. All the cases diagnosed as teratoma in the period of 8 years were included and studied with reference to age, sex, site, size, gross, and microscopic features., Results: A total of 92 cases were retrieved. The most common teratoma was ovarian (78.26%) followed by intracranial/intraspinal and sacrococcygeal in frequency of 7.61% each. Out of 92 cases, 89 were mature and benign, 2 cases were immature teratoma each in ovary and in sacrococcyx, and 1 case of teratocarcinoma in testis., Conclusion: Teratomas have much diversity in their age at presentation, location, gross features, and in degree of differentiation. The prognosis and treatment also depends on the histological grading of teratomas. Thus, pathologists have an important role in establishing a reliable prognostic profile., Competing Interests: There are no conflicts of interest

Case: A 31-year-old immunocompetent woman presented with a large sacral mass on the left side, concerning for a neoplastic process. A biopsy specimen demonstrated fungal osteomyelitis. Intraoperatively, the left S1 sacral nerve root was found to be necrotic, consistent with the symptoms of numbness and weakness. The infection was resolved with aggressive surgical debridement and long-term therapy with antifungal medication., Conclusion: Fungal osteomyelitis of the sacrum is rare, especially in an immunocompetent patient, and untreated infections can cause nerve root necrosis. We recommend aggressive surgical and antifungal management to avoid neurologic compromise.

Objective This video is a step-by-step illustration of the laparoscopic-assisted mobilisation and resection of a sacrococcygeal teratoma Altman type III. Indication Laparoscopic supralevatory tumour mobilisation is an established method in selected centres and can be the initial step in approaching sacrococcygeal teratomas with an intrapelvic portion, facilitating a complete (R0) resection. Method Laparoscopic surgery is performed in supine position with a 5 mm umbilical trocar, a 5 mm trocar in the right upper abdomen, and two 3 mm trocars in the left middle and lower abdomen. We use a 5 mm 45° optic and 3 mm laparoscopic grasping forceps as well as Overholt clamps. For coagulation, a laparoscopic 5 mm diathermy Sealer (LigaSure ™ , Covidien, Neustadt) is used. Conclusion In selected tumours, laparoscopic assisted tumour mobilisation enables surgeons to clearly identify and ligate the primary tumour-supplying vascular structures at the beginning of the operation, thereby avoiding the risk of major bleeding throughout the subsequent tumour mobilisation. The laparoscopic approach also allows an accurate dissection and mobilisation of the supralevatory tumour parts under direct vision. After repositioning the patient into the prone position, infralevatory preparation can be carried out safely and fast in the conventional sacral approach without any major risk of bleeding., Competing Interests: Interessenkonflikt: Nein., (Georg Thieme Verlag KG Stuttgart · New York.)