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

Singh J, Kharosekar H, Velho V, Survashe P - J Pediatr Neurosci (2016 Jan-Mar)

Bottom Line: Supratentorial atypical teratoid rhabdoid tumors (AT/RTs) of infancy and childhood are rare, highly malignant neoplasms, most common in the first 2 years of life.The majority of these tumors are located in the cerebellum, cerebellopontine angle, pineal gland, spinal cord, and the suprasellar region; supratentorial location is relatively uncommon, and the intraventricular location is extremely rare.We report a rare case of AT/RT arising in the lateral ventricle in a 4-year-old patient.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, Maharashtra, India.

ABSTRACT
Supratentorial atypical teratoid rhabdoid tumors (AT/RTs) of infancy and childhood are rare, highly malignant neoplasms, most common in the first 2 years of life. In spite of multiple treatment regimens consisting of surgical resection, radiation therapy, and multi-agent chemotherapy, the prognosis is very poor. The majority of these tumors are located in the cerebellum, cerebellopontine angle, pineal gland, spinal cord, and the suprasellar region; supratentorial location is relatively uncommon, and the intraventricular location is extremely rare. We report a rare case of AT/RT arising in the lateral ventricle in a 4-year-old patient.

No MeSH data available.


Related in: MedlinePlus

Preoperative computed tomography brain with contrast showing the lesion
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Figure 1: Preoperative computed tomography brain with contrast showing the lesion

Mentions: A 4-year-old girl presented to us with headache and vomiting. She also has a single episode of generalized tonic-clonic convulsion. On neurological examination, she was conscious, oriented. Her motor examination revealed left hemiparesis (Medical Research Council grade 4 in both upper and lower limb); rest of the examination was normal. Her computed tomography (CT) scan showed a well-defined contrast enhancing lesion in right temporal lobe extending into the temporal horn with significant perilesional edema and mass effect. Magnetic resonance imaging (MRI) brain with gadolinium contrast was done which revealed contrast enhancing bright lesion in right temporal lobe with intraventricular extension, with significant edema and mass effect [Figures 1–3]. The patient underwent right frontotemporal craniotomy with gross total tumor resection through transcortical approach. Intraoperatively tumor was located in the temporal horn of right lateral ventricle with no definite tumor - brain interface; it was grayish pink, vascular and suckable with cavitron ultrasonic aspirator. Gross total resection was achieved. The postoperatively patient recovered well and her power improved in both left upper and lower limbs. Postoperative CT brain showed gross total resection with postoperative changes [Figure 4]. Histopathology revealed large and pleomorphic rhabdoid cells with abundant eosinophilic cytoplasm, with filamentous cytoplasmic inclusions and vacuoles; along with eccentric round nuclei and prominent nucleolus. IHC revealed tumor cells to be positive for vimentin, epithelial membrane antigen (EMA), smooth muscle antigen (SMA), with patchy positivity for glial fibrillary acidic protein and synaptophysin, thus confirming the diagnosis of AT/RT. The patient was referred further planned for chemotherapy and radiotherapy at a specialist center [Figure 5].


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

Singh J, Kharosekar H, Velho V, Survashe P - J Pediatr Neurosci (2016 Jan-Mar)

Preoperative computed tomography brain with contrast showing the lesion
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4862293&req=5

Figure 1: Preoperative computed tomography brain with contrast showing the lesion
Mentions: A 4-year-old girl presented to us with headache and vomiting. She also has a single episode of generalized tonic-clonic convulsion. On neurological examination, she was conscious, oriented. Her motor examination revealed left hemiparesis (Medical Research Council grade 4 in both upper and lower limb); rest of the examination was normal. Her computed tomography (CT) scan showed a well-defined contrast enhancing lesion in right temporal lobe extending into the temporal horn with significant perilesional edema and mass effect. Magnetic resonance imaging (MRI) brain with gadolinium contrast was done which revealed contrast enhancing bright lesion in right temporal lobe with intraventricular extension, with significant edema and mass effect [Figures 1–3]. The patient underwent right frontotemporal craniotomy with gross total tumor resection through transcortical approach. Intraoperatively tumor was located in the temporal horn of right lateral ventricle with no definite tumor - brain interface; it was grayish pink, vascular and suckable with cavitron ultrasonic aspirator. Gross total resection was achieved. The postoperatively patient recovered well and her power improved in both left upper and lower limbs. Postoperative CT brain showed gross total resection with postoperative changes [Figure 4]. Histopathology revealed large and pleomorphic rhabdoid cells with abundant eosinophilic cytoplasm, with filamentous cytoplasmic inclusions and vacuoles; along with eccentric round nuclei and prominent nucleolus. IHC revealed tumor cells to be positive for vimentin, epithelial membrane antigen (EMA), smooth muscle antigen (SMA), with patchy positivity for glial fibrillary acidic protein and synaptophysin, thus confirming the diagnosis of AT/RT. The patient was referred further planned for chemotherapy and radiotherapy at a specialist center [Figure 5].

Bottom Line: Supratentorial atypical teratoid rhabdoid tumors (AT/RTs) of infancy and childhood are rare, highly malignant neoplasms, most common in the first 2 years of life.The majority of these tumors are located in the cerebellum, cerebellopontine angle, pineal gland, spinal cord, and the suprasellar region; supratentorial location is relatively uncommon, and the intraventricular location is extremely rare.We report a rare case of AT/RT arising in the lateral ventricle in a 4-year-old patient.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, Maharashtra, India.

ABSTRACT
Supratentorial atypical teratoid rhabdoid tumors (AT/RTs) of infancy and childhood are rare, highly malignant neoplasms, most common in the first 2 years of life. In spite of multiple treatment regimens consisting of surgical resection, radiation therapy, and multi-agent chemotherapy, the prognosis is very poor. The majority of these tumors are located in the cerebellum, cerebellopontine angle, pineal gland, spinal cord, and the suprasellar region; supratentorial location is relatively uncommon, and the intraventricular location is extremely rare. We report a rare case of AT/RT arising in the lateral ventricle in a 4-year-old patient.

No MeSH data available.


Related in: MedlinePlus