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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. [ABSTRACT FROM AUTHOR]

Aims: The study aimed to evaluate the presentation, management, and outcome of patients of sacrococcygeal malignant germ cell tumors (SC-MGCTs) with intraspinal extension. Materials and Methods: Case records of all cases of SC-MGCT were reviewed to identify cases with intraspinal extension. They were evaluated in terms of their presentation, response to therapy, extent of surgical resection, recovery of neurological symptoms if any, and outcome. Results: Of the three cases of SC-MGT, two had intraspinal extension. One had Altman Type 3 disease and another had Altman Type 4 disease; both had Stage 4 disease. The intraspinal extension in both patients was detected on contrast computed tomography scan and magnetic resonance imaging. One patient had bladder and bowel incontinence. All the tumors responded to preoperative chemotherapy. Gross complete local resection could be achieved in all patients. Neurological recovery was complete. All were alive with no recurrence on follow-up. Conclusions: Malignant sacrococcygeal GCTs with intraspinal extension are rare and can be managed with neoadjuvant chemotherapy which obviates the need for extensive exploration for excision and has got good neurological and oncological prognosis.

Aims: The study aimed to evaluate the presentation, management, and outcome of patients of sacrococcygeal malignant germ cell tumors (SC-MGCTs) with intraspinal extension. Materials and Methods: Case records of all cases of SC-MGCT were reviewed to identify cases with intraspinal extension. They were evaluated in terms of their presentation, response to therapy, extent of surgical resection, recovery of neurological symptoms if any, and outcome. Results: Of the three cases of SC-MGT, two had intraspinal extension. One had Altman Type 3 disease and another had Altman Type 4 disease; both had Stage 4 disease. The intraspinal extension in both patients was detected on contrast computed tomography scan and magnetic resonance imaging. One patient had bladder and bowel incontinence. All the tumors responded to preoperative chemotherapy. Gross complete local resection could be achieved in all patients. Neurological recovery was complete. All were alive with no recurrence on follow-up. Conclusions: Malignant sacrococcygeal GCTs with intraspinal extension are rare and can be managed with neoadjuvant chemotherapy which obviates the need for extensive exploration for excision and has got good neurological and oncological prognosis. [ABSTRACT FROM AUTHOR]

Sacrococcygeal teratoma (SCT) with intraspinal extension is rare. There is a risk of paraplegia associated with prolonged spinal cord compression. We present the case of an infant with a prenatal diagnosis of an SCT with a large intraspinal component that was causing compression of the lower spinal cord. Ultrasound at 33 weeks showed bilateral lower extremity and foot movement without hydrops or cardiac failure. Multidisciplinary decision was made to administer betamethasone and proceed with Cesarean delivery at 34 weeks. A vigorous live-born female was delivered and a multilevel laminectomy was performed at day of life 4. The pelvic resection was performed at 4 months. Pathology revealed mature teratoma. She had an uncomplicated postoperative course, is ambulatory, continent of stool, and has no evidence of recurrence. We conclude that intraspinal extension of SCT should be evaluated prenatally with ultrasound and fetal MRI. If there is concern for spinal cord compression, early delivery and urgent decompressive laminectomy may diminish the neurologic sequelae of prolonged spinal cord compression. Since these cases are rare, risks of prematurity need to be weighed against the neurologic risks. These infants should be treated with a multidisciplinary approach. [ABSTRACT FROM AUTHOR]

Abstract Retroperitoneum is a relatively uncommon site for pediatric teratomas. Rarely, such tumors can have an intraspinal extension and few cases of retroperitoneal teratomas associated with spinal dysraphism have been reported. Teratomas consist of tissues arising from all three embryonic layers. However, mature renal tissues in the form of glomeruli and tubules are sparingly found in teratomas. A 15-day-old female presented with spina bifida occulta and on evaluation a cystic presacral mass was detected. Intraoperatively the cyst was found densely adherent to the hemivertebrae but not entering the spinal canal. Histopathological examination confirmed a mature cystic teratoma but also demonstrated presence of mature renal elements in the cyst wall. The teratomas lying in proximity to spine and associated with spinal dysraphism are likely to contain mature renal tissues or even nephroblastic elements. It supports the dysembryogenic model of origin of intradural teratomas from native progenitor cells rather than aberrantly migrated germ cells.

Abstract: Background: Neurological involvement due to intraspinal extension in sacrococcygeal malignant germ cell tumors (SC-MGCTs) has rarely been reported. Aim: To evaluate the incidence, presentation, management and the outcome of patients of SC-MGCT with intraspinal extension. Materials and Methods: Case records of all cases of SC-MGCT from 2001 to 2008, were reviewed to identify cases with vertebral involvement and intraspinal extension. They were evaluated in terms of their presentation, response to therapy, extent of surgical resection, recovery of neurological symptoms and outcome. Results: Of the 31 cases of SC-MGCT, 5 (16%) had intraspinal extension. Age ranged from 12 to 84months (median 24months). Four patients had Altman type 4 disease (stage 4) and 1 had Altman type 3 (stage 3) disease. The intraspinal extension in all patients was detected on contrast CT scan. Patients presented with neurological symptoms in the form of lower limb paresis (80%), bowel and bladder (20%) incontinence. All the tumors responded to pre-operative chemotherapy. Gross complete local resection could be achieved in 4(80%). Neurological recovery was complete in all except for persisting neurogenic bladder in one. During follow up of 3–32months, all were alive with no recurrence. Conclusions: SC-MGCT presenting with neurological deficits due to intraspinal extension is usually advanced disease. These patients respond to chemotherapy and surgical resection and most have complete neurological improvement. [Copyright &y& Elsevier]

We performed a systematic review to define the long-term health problems and optimal treatment strategy for patients with neuroblastoma with intraspinal extension. Of 685 identified studies, 28 were included in this review. The burden of long-term health problems is high; a median of 50% of patients suffered from neurological motor deficit, 34% from sphincter dysfunction, and 30% from spinal deformity. The currently available literature remains suboptimal as a guide for treatment of NBL with intraspinal extension. More well-designed, prospective studies are needed to determine the optimal treatment strategy., (© 2017 Wiley Periodicals, Inc.)

Aim: To evaluate the prevalence of health problems in 5-year survivors treated for neuroblastoma (NBL) with intraspinal extension., Patients and Methods: Retrospective, single center cohort study (using data from Childhood Cancer Registry and medical records) of patients treated for NBL with intraspinal extension (between 1980 and 2007) who survived ≥ 5 years after diagnosis. Health problems were graded according to the Common Terminology Criteria for Adverse Events (CTCAEv.3.0)., Results: All eligible patients (n = 19) were included (n = 7 no neurological symptoms at diagnosis), median age at diagnosis was 1.2 years (0.6-10.8 years), and median follow-up time was 15.6 years (6.3-29.5 years). Ninety-five percent of survivors had ≥1 health problem and 48% of survivors had ≥4 health problem with a mean of 3.8 per survivor. Fifty-three percent of survivors had at least one severe (grade 3) or life-threatening/disabling (grade 4) health problem. The three most prevalent health problems were kyphosis and/or scoliosis (68% of patients), motor neuropathy (32% of patients), and sensory neuropathy (26% of patients). Of the 13 patients who underwent a laminectomy, 54% (seven of 13) developed a grade 3 and 23% (three of 13) developed a grade 4 health problem. Among six patients, without laminectomy, 17% developed (one of six) a grade 3 and in 17% developed (one of six) a grade 4 health problem., Conclusions: Ninety-five percent of 5-year survivors treated for a childhood intraspinal NBL have health problems. The high prevalence of grade 3 and 4 health problems (especially in the laminectomy group) emphasizes the importance of specialized long-term multidisciplinary follow-up and identifies optimal treatment with limited morbidity and maximal efficacy., (© 2016 Wiley Periodicals, Inc.)