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Sacrococcygeal teratoma

Heron D Baumgarten, Juliana S Gebb, Nahla Khalek, Julie S Moldenhauer, Mark P Johnson, William H Peranteau, Holly L Hedrick, N Scott Adzick, Alan W Flake
INTRODUCTION: Fetuses with "high-risk" sacrococcygeal teratoma (SCT) have a mortality rate of 40-50%. While fetal surgery may benefit select fetuses prior to 27 weeks' gestation, many fetuses die due to consequences of rapid tumor growth after 27 weeks. Here we report our experience applying "preemptive" delivery to fetuses who manifest signs of decompensation between 27 and 32 weeks. METHODS: A retrospective review of SCT fetuses delivered between 2010 and 2016 at ≤32 weeks' gestation was performed...
May 7, 2018: Fetal Diagnosis and Therapy
P Meignan, A Binet, A R Cook, H Lardy, G Captier
PURPOSE: The median sacral artery (MSA) is the termination of the dorsal aorta, which undergoes a complex regression and remodeling process during embryo and fetal development. The MSA contributes to the pelvic vascularization and may be injured during pelvic surgery. The embryological steps of MSA development, anastomosis formation and anatomical variations are linked, but not fully understood. METHODS: The pelvic vascularization and more precisely the MSA of a human fetus at 22 weeks of gestation (GW) were studied using micro-CT imaging...
April 30, 2018: Surgical and Radiologic Anatomy: SRA
Mette Hambraeus, Lars Hagander, Pernilla Stenström, Einar Arnbjörnsson, Anna Börjesson
OBJECTIVE: To evaluate urinary tract and bowel function in children with sacrococcygeal teratoma, compare the findings with healthy children, and assess predictors of poor outcome. STUDY DESIGN: This was a controlled cohort study of all patients operated for sacrococcygeal teratoma at a tertiary pediatric surgery center, 2000-2013. Urinary and bowel function were compared with healthy control patients matched for age and sex. Perioperative and histopathologic risk factors were analyzed...
April 12, 2018: Journal of Pediatrics
Juliana S Gebb, Nahla Khalek, Huma Qamar, Mark P Johnson, Edward R Oliver, Beverly G Coleman, William H Peranteau, Holly L Hedrick, Alan W Flake, N Scott Adzick, Julie S Moldenhauer
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...
March 1, 2018: Fetal Diagnosis and Therapy
Hrushikesh Umakant Kharosekar, Harish Naik, Vernon Velho
Teratomas are a type of multipotential cell tumor that contain a mixture of multiple germinal layers formed by normal organogenesis and reproductive tissues the incidence of intracranial teratomas is low, approximately 0.5-2.2% of all intracranial tumors. The occurrence of teratomas in the spine is extremely rare. Except for in the sacrococcygeal region, teratomas constitute <0.5% of all intraspinal tumors. According to the literature this is only 8th case of dorsal spinal mature cystic teratoma reported till date...
January 2018: Asian Journal of Neurosurgery
Marijke E B Kremer, Jessica F Althof, Joep P M Derikx, Robertine van Baren, Hugo A Heij, Marc H W A Wijnen, René M H Wijnen, David C van der Zee, L W Ernest van Heurn
BACKGROUND: Gross genetic causes for SCT are unknown; however, it might be associated with other abnormalities. We assessed the incidence of associated abnormalities in a large national cohort of neonates with SCT and aimed to identify predictive risk factors. PROCEDURE: The medical records were reviewed of 235 consecutive neonates with SCT treated at the six pediatric surgical centers in the Netherlands from 1970 to 2010. Potential risk factors for associated abnormalities analyzed included sex, gestational age, tumor-volume/histology and Altman-classification...
January 31, 2018: Journal of Pediatric Surgery
Jessica D Wharton, Shaina Johnson, James A Connelly, Tracy Hills, Lauren Gingles, Margaret Wood, Gabriella L Crane, Howard M Katzenstein
Aicardi syndrome (AS) is a rare neurodevelopmental disorder, predominantly seen in female individuals, which appears to have an increased risk of both benign and malignant neoplasia. We report the case of a child with AS who presented with metastatic malignant sacrococcygeal tumor (with yolk sac elements) which recurred and then was treated with 3 cycles of high-dose chemotherapy with autologous stem cell rescue. The patient tolerated therapy with acceptable toxicity and remains in clinical remission 3 months after the completion of therapy...
February 7, 2018: Journal of Pediatric Hematology/oncology
Yangyang Ma, Jicui Zheng, Jiayan Feng, Haitao Zhu, Xianmin Xiao, Lian Chen
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...
June 2018: Pediatric Blood & Cancer
Seung Mi Lee, Dong Hoon Suh, So Yeon Kim, Min Kyoung Kim, Sohee Oh, Sang Hoon Song, Hyun-Young Kim, Chan-Wook Park, Joong Shin Park, Jong Kwan Jun
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...
February 5, 2018: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Fengtian Zhang, Xiaolong Yu, Jin Zeng, Min Dai
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...
November 2017: Medicine (Baltimore)
Aaron Wessell, David S Hersh, Cheng-Ying Ho, Kimberly M Lumpkins, Mari L A Groves
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...
January 24, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Daniel Diaz-Aguilar, Sergei Terterov, Rudi Scharnweber, Catherine Merna, Stephanie Wang, Shayan Rahman
Background: Saccrococcygeal teratomas (SCT) are derived from embryonic germ cell layers. They frequently present at the base of the coccyx within the pelvis. While these tumors are common in children, they are exceedingly rare in adults. In adults, a majority of these tumors are intrapelvic and associated with a low risk of malignant transformation. Therefore, this contributes to a good prognosis following resection of mostly benign lesions. Case Description: An adult female with chronic pelvic pain presented with a sacral teratoma...
2017: Surgical Neurology International
Tarig Babiker, Efstathia Kyrodimou, Daniel M Berney, Mark Gurnell, William M Drake, Antonia Brooke
A 60-year-old man with a pre-existing stable sacrococcygeal teratoma developed acromegaly, ectopic Cushing's syndrome, and 5HIAA secretion. To our knowledge, this represents the first reported case of ACTH and serotonin secretion, and likely GHRH or GH cosecretion, from a sacrococcygeal teratoma in an adult.
November 2017: Clinical Case Reports
Ruchi Amin, Elizabeth Berdan, Jeffrey Knipstein, Jason Jarzembowski, Sabina Siddiqui
No abstract text is available yet for this article.
January 2018: Pediatric Surgery International
Muhammad S Ahmed, Sheeza Imtiaz, Hina Pathan, Rahila Usman
Unilateral renal agenesis (URA) is a rare condition with a reported incidence of 0.93-1.8 per 1000 autopsies. It is commonly diagnosed as an incidental finding on imaging. URA is frequently associated with other genitourinary anomalies. Different associations have been described in both males and females, however, to our knowledge, it has not been reported with subseptate uterus (SSU) and sacrococcygeal teratoma (SCT) in the same individual. Here, we present a unique case of URA with SSU and SCT.
July 2017: Indian Journal of Radiology & Imaging
Barkha Gupta, Surekha Yadav, Nita Khurana, Prince Raj
Sacrococcygeal Teratoma (SCT) showing pulmonary differentiation has been rarely reported in the literature. Till date, only eight cases have been reported, out of which six belong to teratomas arising from female genital tract. Spinal teratomas showing pulmonary differentiation are rarer and only two cases have been reported so far in the literature both of which had associated congenital spinal anomaly. We present a rare case of seven day old infant with sacrococcygeal teratoma showing mature lung differentiation as one of its components...
August 2017: Journal of Clinical and Diagnostic Research: JCDR
Zhen Shen, Shan Zheng, Rui Dong, Gong Chen
PURPOSE: The purpose of this study was to study whether color difference in facial color truly exists between neonates with obstructive and nonobstructive jaundice, and whether the color difference could be objectified by spectrophotometer. METHODS: Twelve biliary atresia patients were enrolled in an obstructive jaundice group and 15 neonates admitted for non-conjugated hyperbilirubinemia in a nonobstructive group. Nine patients with syphilis (n=6) and sacrococcygeal teratoma (n=3) were studied as control...
December 2017: Journal of Pediatric Surgery
Suyash Singh, Jayesh Sardhara, Pradeep Sharma, Arun Kumar Srivastava, Kuntal Kanti Das, Kamlesh S Bhaisora, Anant Mehrotra, Awadhesh Kumar Jaiswal, Sanjay Behari, Raj Kumar
Teratomas are one of the most common tumors in newborn with excellent prognosis arises from totipotent primordial germ cells harboring two or three germ cell layers. The tumor has been titled "Great masquerade." The teratomas of sacrococcygeal region present with lower limb weakness, urinary or bowel obstruction, and swelling at lower back or intrauterine mass in ultrasound or complicated delivery. A 2-month-old male child presented with complaints of swelling over lumbosacral region with discharging punctum since birth...
April 2017: Journal of Pediatric Neurosciences
Benjamin E Padilla, Lan Vu, Hanmin Lee, Tippi MacKenzie, Barbara Bratton, Maura O'Day, Sarkis Derderian
BACKGROUND: Sacrococcygeal teratoma (SCT) is a rare childhood malignancy. Although overall survival is favorable, recurrent tumors are associated with poor outcomes. As most recurrences occur within 3 years of presentation, the utility of long-term surveillance is uncertain. METHODS: Patients with SCTs evaluated and managed by our pediatric surgery department between 1986 and 2013 were included. Details pertaining to laboratory values, operative findings, tumor histology, management, recurrence, and outcomes were recorded and analyzed...
November 2017: Pediatric Surgery International
Muataz A Alani
Most sacrococcygeal teratomas present between the 22nd and the 34th week of gestation. The diagnosis of sacrococcygeal teratoma on routine antenatal sonograms is associated with a greater than expected incidence of prenatal and perinatal complications. We report a premature baby with intrauterine spontaneous rupture of giant sacrococcygeal teratoma which was managed successfully.
April 2017: Journal of Neonatal Surgery
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