Cases reported "Teratoma"

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1/11. Familial presacral masses: Screening pitfalls.

    BACKGROUND/PURPOSE: Anterior sacral meningocele (ASM) and presacral teratoma (PT) are related malformations. Both can have familial occurrence. Surgical treatment of occult cases is essential. Hence, it is important to screen the asymptomatic relatives. Plain radiograph of the sacrum to look for any bony abnormalities is the usual accepted screening modality. The authors report ASM and PT in siblings (with positive family history) with no sacrococcygeal bony abnormality on imaging. Both the siblings had presacral mass on imaging. methods: The medical records of 2 siblings with ASM and PT were reviewed for the clinical presentation, diagnostic workup, and family history. RESULTS: One sibling was symptomatic, whereas the other was completely asymptomatic. A family history of sacral bony abnormalities and anterior meningoceles was present in father, 2 paternal cousins, paternal uncle, paternal aunt, paternal grandmother, and paternal grand uncle. CONCLUSIONS: These case reports suggest that imaging for screening for ASM and PT should be directed at identifying the presacral mass rather than sacral bony defect. Hence, computed tomography or magnetic resonance imaging looking for presacral mass rather than plain radiograph should be the screening modality. J Pediatr Surg 36:1841-1844.
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2/11. prenatal diagnosis of sacrococcygeal teratomas by two- and three-dimensional ultrasound.

    We suspected two fetuses of having sacrococcygeal teratomas following initial two-dimensional ultrasound scanning at 18 and 22 weeks' gestation. The fetuses were then scanned with three-dimensional multiplanar surface and three-dimensional orthogonal planar ultrasound to establish a definitive diagnosis. Although we made a presumptive diagnosis in both cases after initial two-dimensional scanning, with three-dimensional ultrasound we were better able to define the degree of involvement of the sacrum and other pelvic structures of prognostic importance.
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3/11. Newborn with an open posterior hip dislocation and sciatic nerve injury after intrauterine radiofrequency ablation of a sacrococcygeal teratoma.

    Advanced prenatal ultrasonography techniques have allowed for better understanding of the natural history, treatment, and prognosis of sacrococcygeal teratomas. Several intrauterine surgical techniques to debulk the tumor when fetal and maternal life are in jeopardy have been described. Orthopaedic impairment, such as lower extremity weakness and swelling, also has been described in association with sacrococcygeal teratomas. The authors report on a newborn in whom a large soft tissue defect overlying the posterior hip region with direct exposure of the disarticulated hip joint existed at the time of birth, which resulted from intrauterine radiofrequency ablation of a sacrococcygeal teratoma. This unexpected complication has resulted in a loss of sciatic nerve function, malformation of the acetabulum and femoral head, and loss of the left ischium, coccyx, inferior sacrum, gluteal, adductor and piriformis muscles, and posterior hip capsule. At 16 months of age, the patient has a flaccid left lower extremity with a hypoplastic hip joint.
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4/11. Anterior sacral meningomyelocele with sacrococcygeal teratoma.

    Anterior sacral meningocoele with sacrococcygeal teratoma is a rare entity. The cystic mass arising from anterior sacral and coccygeal defect, lies in the retrorectal space between the rectum and sacrum. It produces a variety of symptoms depending on its size and contents and constitutes a diagnostic problem. Such a rare association of two pathologies is presented, with review of literature, in an infant who had an anterior meningocoele with sacrococcygeal teratoma. Both the pathologies were surgically corrected individually, about a month apart.
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5/11. Currarino syndrome: proposal of a diagnostic and therapeutic protocol.

    BACKGROUND/PURPOSE: The Currarino syndrome (CS) is a peculiar form of caudal regression syndrome (CRS) characterized by the association of hemisacrum, anorectal malformation (arm), and presacral mass. The authors analyzed retrospectively their series, and they propose a multidisciplinary diagnostic and therapuetic protocol that until now has not been introduced. methods: A series of 6 patients with CS is presented. Five of them were treated initially in other centers. None of them had an early diagnosis. All presented associated anomalies; in 50%, Hirschsprung's disease (HD) and other dysganglionoses were present. One patient died of a presacral ectopic nephroblastoma. RESULTS: Depending on the expressivity, 3 types of CS can be identified, complete, mild, and minimal. Dysganglionoses and HD can be considered part of CS. A multidisciplinary diagnostic and therapeutic protocol is presented. Main points are sacrum x-Ray, molecular genetic diagnosis, radiologic evaluation of every member of CS families, magnetic resonance (MR) evaluation of patient spine and pelvis, suction rectal biopsies, and search for associated anomalies. CONCLUSIONS: This protocol could give a valid contribution to the treatment of CS, allowing an early diagnosis and proposing a rational timing of multidisciplinary surgical procedures. early diagnosis and treatment are essential to avoid morbidity and mortality from an undiagnosed presacral mass.
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6/11. A case of type III cystic sacrococcygeal teratoma.

    We report a rare case of congenital type III cystic teratoma that may be falsely diagnosed as an anterior sacral meningocele. A female newborn baby presented with a long, white-colored 4 x 1 cm coccygeal cyst posterior to the anus. magnetic resonance imaging (MRI) demonstrated that a 10 x 4 cm elliptical intrapelvic and -abdominal mass anterior to the sacrum and coccyx extended inferiorly to the retrorectal space. We intraoperatively found that the cyst consisted of a mostly thin, white wall, but also with small posterior narrow nodules. We suggest that all cysts in the sacrococcygeal area should be excluded from teratoma carefully, with MRI.
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7/11. Triad of anorectal stenosis, sacral anomaly and presacral mass: a remediable cause of severe constipation.

    Two neonates with intestinal obstruction and two children (aged 1 and 4 years) with severe constipation since birth are reported in whom stenosis of the distal rectum was found. In association with the rectal anomaly, three of them had a presacral tumour (teratoma in two, hamartoma in one) and all had a deformed sacrum. An embryological hypothesis to explain this association has been postulated by Currarino, after whom this triad has been named. Two patients were related (father and daughter). The role of hereditary factors in the occurrence of the syndrome has been reported before. Operative treatment of the rectal stenosis was necessary in all patients. Preoperative diverting colostomy was performed in three cases, followed by a posterior sagittal approach to excise the rectal stenosis and the presacral mass. In one case, persistent cerebrospinal fluid leakage required re-exploration for closure of a tear in a congenitally abnormal dural sac. The fourth patient had undergone a low anterior resection in the past via the abdominal route and needed rectal dilatation afterwards for some time. The final result in all patients appears satisfactory, although follow-up is short. Most cases of this triad have been reported in children but a number of patients have been diagnosed only as adults. Recognition of this triad should imply a careful search for neural crest malformations. Operative treatment to correct all soft tissue anomalies leads to good results.
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8/11. Neonatal diagnosis of a presacral mass in the presence of congenital anal stenosis and partial sacral agenesis.

    The simultaneous presentation of clinically symptomatic anal anomalies and roentgenographically demonstrated sacral dysgenesis should alert the pediatric surgeon to investigate for the presence of a presacral malformation. We report on such a case to illustrate a new radiographic technique that facilitates diagnosis and management of complex congenital malformations. A 1-day-old white boy presented with anal stenosis, a scimitar-shaped sacrum, and large anterior and posterior meningoceles. In addition, a distinct presacral tumor--a teratoma--was identified. These malformations were identified utilizing metrizamide myelography and three-dimensional reconstruction computed tomography (CT) scanning. The meningoceles and a tethered cord were successfully corrected utilizing a posterior approach. A diverting colostomy was performed and subsequently taken down. Two years postoperatively, the patient continues to do well. This case demonstrates that this triad of anomalies (presacral mass, sacral dysgenesis, and anorectal malformation), once considered, can be safely detected with modern radiologic techniques and can be expeditiously corrected during infancy before further deterioration occurs.
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9/11. Sacrococcygeal teratoma.

    Eight cases of sacrococcygeal teratoma are described. Seven patients were diagnosed at birth, and one presented at 3 1/2 months of age with urinary retention. Two patients had associated congenital anomalies (arachnoid cyst, external angular dermoid and bifid sacrum), while one had a family history of sacrococcygeal teratoma. All had the tumour removed together with the coccyx as soon as the diagnosis was made. None had recurrence of the tumour.
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10/11. Intradural extension of a sacrococcygeal teratoma.

    Intradural extension of a sacrococcygeal teratoma (SCT) is extremely rare and only well-documented in presacral tumors that have been associated with a familial history, anorectal stenosis, and sacral dysraphism. This case documents the extension of a type I SCT into the dural sac with attachment to the filum terminale. A full-term female was transferred to our tertiary newborn intensive care unit with a sacral mass measuring 12 x 13 cm. It protruded from the buttocks and displaced the anus anteriorly. Rectal examination showed no presacral component. Radiographs demonstrated calcification in the soft tissue mass and a normal-appearing sacrum with the last sacral segment not visualized. At operation during dissection of the cephalad component, the SCT extended into the spinal canal. Neurosurgical consultation resulted in a sacral laminectomy which revealed the tumor to be attached to the tip of the filum terminale. The tumor was removed in toto with all sacral roots preserved. The infant required a second operation to revise a wound dehiscence and suspected cerebrospinal fluid leak. The final pathology report was benign SCT. Follow-up at 2 years showed no recurrence, normal sphincter tone, and a normal computed tomography scan. This represents the first well-documented intradural extension of a Type I SCT with attachment to the spinal cord. This extremely rare occurrence requires awareness with the availability of neurosurgical support to expedite operative management.
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