Atypical rhabdoid tumor of lateral ventricle: Report of an unusual tumor

Singh, Jasmit; Kharosekar, Hrushikesh; Velho, Vernon; Survashe, Praveen

doi:10.4103/1817-1745.181257

Cited by 2 publications

(4 citation statements)

References 4 publications

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“…Compared to other CNS sites of tumor development, lateral ventricle AT/RT is more frequently associated to clinical manifestations related to increased intracranial pressure. Neurological symptoms, such as ataxia, vertigo, strabismus, and seizures, seem to be less frequent or evident than in children with lateral ventricle AT/RT compared to the other CNS locations or, in general, other CNS tumors ( 6 ). Notably, patients with AT/RT usually have a short clinical history ranging from days to weeks with an average duration of 0.75 months ( 18 ), whereas our patient had at least a 2-month clinical history before the CNS mass was investigated and detected, which may be explained by the ventricular location of the tumor.…”

Section: Discussionmentioning

confidence: 95%

“…This observation is consistent with the previous clinical experiences, as described by (study period: not specified). Here, we described a pediatric case affected with supra-tentorial AT/RT of the lateral ventricle: only 8 cases were previously described in the medical literature as originating from this specific supra-tentorial site, as shown in Table 2, which summarizes our case-based literature review of lateral ventricle AT/RT (6,(8)(9)(10)(11)(12)(13)(14). Notably, all the previous cases of lateral ventricle AT/RT were 4 years old or younger, and our case report is the first description of AT/RT arising from the lateral ventricle in an adolescent.…”

Section: Discussionmentioning

confidence: 99%

“…AT/RT is predominantly seen in males, with a male-to-female ratio of 2.7:1 ( 3 , 4 ). This tumor is characterized by rapid growth and spread in cerebrospinal fluid; therefore, it is characterized by a poor prognosis ( 5 , 6 ). Most cases of AT/RT arise from the posterior fossa or, much less frequently, from the spinal cord; the occurrence of AT/RT in the lateral ventricle is extremely rare in children ( 6 , 7 ).…”

Section: Introductionmentioning

confidence: 99%

“…Literature review of cases of pediatric lateral ventricle Atypical Teratoid/Rhabdoid Tumor(6,(8)(9)(10)(11)(12)(13)(14).…”

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confidence: 99%

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Case report: Atypical teratoid/rhabdoid tumor of the lateral ventricle in a male adolescent (case-based review and diagnostic challenges in developing countries)

Karim

Shaikhyzada²,

Suleimenova³

et al. 2022

Front. Oncol.

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Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and highly malignant central nervous system (CNS) embryonal neoplasm: it accounts for <2% of all pediatric CNS tumors and occurs mainly in infants and young children. The primary site of this tumor is usually the posterior cranial fossa. Supratentorial and, in detail, latero-ventricular location is extremely uncommon, especially in adolescents. This tumor is characterized by rapid growth and spread in cerebrospinal fluid and, therefore, it is characterized by a poor prognosis. Neurological signs and symptoms are related the location of the tumor. The radiological features of AT/RT are nonspecific. Immunohistochemical staining for loss of nuclear integrase interactor 1 (INI1) expression is considered a reliable criterion for the diagnosis of this type of tumor. AT/RT has been linked to mutations of SMARCB1 or, rarely, SMARCA4 genes, which function as tumor suppressor genes. Currently, there is no validated protocol of treatment for children with AT/RT, and multimodality treatment (consisting of surgery, chemotherapy, and radiation therapy) is considered. In this case report, we describe a 15-year-old adolescent with an AT/RT of the left lateral ventricle. Despite the late diagnosis, the multimodal therapeutic approach provided a good outcome for our patient at 21 months’ follow-up. Based on our case-based review, early diagnosis and a multimodal approach to treatment play a key role in improving the survival of patients with this diagnosis. Implementing a system supporting pathological and molecular analyses for developing countries and, in general, for non-academic centers is of primary importance to timely diagnose and treat rare tumors, such as AT/RT.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Literature review of cases of pediatric lateral ventricle Atypical Teratoid/Rhabdoid Tumor(6,(8)(9)(10)(11)(12)(13)(14).…”

mentioning

confidence: 99%

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Case report: Atypical teratoid/rhabdoid tumor of the lateral ventricle in a male adolescent (case-based review and diagnostic challenges in developing countries)

Karim

Shaikhyzada²,

Suleimenova³

et al. 2022

Front. Oncol.

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Atypical teratoid/rhabdoid tumor of the lateral ventricle presenting with metastasis: A case report

Mohan¹,

Viswanathan²,

Chowdhury³

et al. 2023

Journal of Pediatric Neurosciences

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Atypical teratoid/rhabdoid tumor (AT/RT) is a biologically aggressive tumor of the central nervous system, usually occurring in the posterior fossa in infants and young children. Supratentorial location is relatively rare, especially tumors arising primarily within the lateral ventricles. Prognosis remains poor, despite multiple treatment modalities consisting of surgical excision, radiotherapy, and chemotherapy. We present the case of a 3-year-old girl with an AT/RT of the lateral ventricle with diffuse leptomeningeal and spinal metastasis on magnetic resonance imaging. She underwent a left frontoparietal craniotomy and near-total excision of the tumor. Immunohistochemistry confirmed the diagnosis and the child died within 3 months of surgery. There are isolated case reports in the literature regarding primary lateral ventricular AT/RTs, although secondary involvement of the ventricle from an adjacent tumor is commoner. Ours is the first case that demonstrates AT/RT arising within the body of the lateral ventricle causing extensive metastasis, both within the brain and spine. Given the dismal prognosis, the choice between further adjuvant treatment and palliative care is a matter of debate that necessitates a truthful and meaningful discussion with the parents.

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Atypical rhabdoid tumor of lateral ventricle: Report of an unusual tumor

Cited by 2 publications

References 4 publications

Case report: Atypical teratoid/rhabdoid tumor of the lateral ventricle in a male adolescent (case-based review and diagnostic challenges in developing countries)

Case report: Atypical teratoid/rhabdoid tumor of the lateral ventricle in a male adolescent (case-based review and diagnostic challenges in developing countries)

Atypical teratoid/rhabdoid tumor of the lateral ventricle presenting with metastasis: A case report

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