Congenital inclusion tumours in spinal dysraphism

Shubha, Attibele Mahadevaiah; Mohanty, Sriloy; Das, Kanishka; Garg, Isha

doi:10.1007/s12098-009-0290-z

Cited by 17 publications

(14 citation statements)

References 10 publications

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“…Given the infiltrative margin and overall appearances, somewhat concerned with low grade malignant peripheral nerve sheath tumor. Congenital inclusion tumor exist at birth but become overtly symptomatic later in life because of a characteristic slow growth14). But in present case tumor mass was rapidly growing.…”

Section: Discussionmentioning

confidence: 52%

Intradural Extramedullary and Subcutaneous Tumors in Neonate : Atypical Myxoid Spindle Cell Neoplasm

Choi

Lee

et al. 2012

J Korean Neurosurg Soc

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Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.

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Section: Discussionmentioning

confidence: 52%

Intradural Extramedullary and Subcutaneous Tumors in Neonate : Atypical Myxoid Spindle Cell Neoplasm

Choi

Lee

et al. 2012

J Korean Neurosurg Soc

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“…The reported incidence of IC after postnatal spina bifida repair ranges from 2 to 16% [5][6][7][8], with the age at presentation between 1 and 12 years. However, these numbers depict a population of patients with tethered cord syndrome, thus the true incidence could be higher, since imaging and surgery were only performed in patients with functional deterioration.…”

Section: Incidencementioning

confidence: 99%

“…Before the era of fetal surgery, inclusion cysts (IC) already represented a significant problem, occurring in up to 16% of patients with spinal dysraphism [5][6][7][8], and causing severe functional deterioration. However, there is only limited literature on the occurrence of IC after fSBR.…”

Section: Introductionmentioning

confidence: 99%

Inclusion Cysts after Fetal Spina Bifida Repair: A Third Hit?

et al. 2018

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Introduction: Fetal spina bifida repair (fSBR) has proven effective in the reversibility of hindbrain herniation, lower rate of shunt-dependent hydrocephalus, and independent ambulation. Besides distinct advantages, there are also concerns related to fSBR. One of these is the postnatal occurrence of inclusion cysts (IC). Methods: In a prospective study, 48 children who underwent fSBR were followed up. Postnatal assessment included clinical examination, cystometry, and spinal MRI. Indication for IC resection was the evidence of a spinal mass on MRI in the presence of deteriorating motor or bladder function, pain, or considerable growth of the IC. Results: Fourteen children (30%) developed IC, all within the first 2 years of life. Six children underwent IC resection; 4 children due to deteriorating function, 2 children due to doubling of the mass on MRI within 1 year. Following IC resection, 4/6 children (67%) demonstrated altered motor function and 6 children (100%) were diagnosed with neurogenic bladder dysfunction. Conclusions: Systematic follow-up of patients with a history of fSBR revealed a high incidence of IC. Whether these are of dysembryogenic or iatrogenic origin, remains unclear. Since both IC per se and IC resection may lead to loss of neurologic function, IC can be considered a “third hit”.

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“…When located in the spine, they are usually found in the lumbar region and are often associated with congenital spinal dysraphism and/or dermal sinus tracts [1,2]. Arising from an embryologic miss-development during the ectodermal folds, dermoid cysts often present themselves in the 2nd or 3 rd decade [3].…”

Section: Introductionmentioning

confidence: 99%

Unruptured Spinal Dermoid Cyst with Progressive Paraplegia

Hj¹,

Henker²,

Erbersdobler³

et al. 2017

Med Case Rep

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Intramedullary dermoid cysts without spinal dysraphism are uncommon tumors. Only a few cases have been reported in the literature. We report a case of a 63-yearold woman with a spinal intramedullary dermoid cyst without rupture, but with progressive paraparesis and bladder dysfunction. Lumbar magnetic resonance imaging showed a large cystic lesion with inhomogeneous contrast enhancement and compression of the conus medullaris. Operation and histopathological examination confirmed a dermoid cyst. A follow up MRI imaging and examination was performed 4, 8 and 20 months after the operation. This case report adds to the few previous reported cases of intramedullary unruptured dermoid cysts and summarizes the latest literature.

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Congenital inclusion tumours in spinal dysraphism

Abstract: Early detection and comprehensive management of CIT with spinal dysraphism ensures social fecourinary continence, preserves renal function, achieves ambulation and enables patients to lead an acceptable quality of life.

Cited by 17 publications

References 10 publications

Intradural Extramedullary and Subcutaneous Tumors in Neonate : Atypical Myxoid Spindle Cell Neoplasm

Intradural Extramedullary and Subcutaneous Tumors in Neonate : Atypical Myxoid Spindle Cell Neoplasm

Inclusion Cysts after Fetal Spina Bifida Repair: A Third Hit?

Unruptured Spinal Dermoid Cyst with Progressive Paraplegia

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