Extraventricular subependymal giant cell tumor in a child with tuberous sclerosis complex

2009 ◽  
Vol 4 (1) ◽  
pp. 85-90 ◽  
Author(s):  
Robert J. Bollo ◽  
Jonathan L. Berliner ◽  
Ingeborg Fischer ◽  
Daniel K. Miles ◽  
Elizabeth A. Thiele ◽  
...  

Subependymal giant cell tumors (SGCTs) are observed in 5–20% of patients with tuberous sclerosis complex (TSC) but account for ~ 25% of neurological morbidity. The authors report the case of a 7-year-old girl with TSC and multiple cortical tubers who presented with worsening seizures in the context of the rapid growth of a cystic, calcified, extraventricular SGCT in the right frontal lobe, initially thought to represent a cortical tuber. The tumor and surrounding tubers were excised, and clinical seizures resolved. This is the first report of an extraventricular SGCT in a child with TSC outside the neonatal period.

2008 ◽  
Vol 78 (1) ◽  
pp. 7-21 ◽  
Author(s):  
K. Boer ◽  
F. Jansen ◽  
M. Nellist ◽  
S. Redeker ◽  
A.M.W. van den Ouweland ◽  
...  

2020 ◽  
Vol 77 ◽  
pp. 85-88 ◽  
Author(s):  
James R. Barnett ◽  
Reid A. Grinspoon ◽  
Mukesh Harisinghani ◽  
Paul A. Caruso ◽  
Elizabeth A. Thiele

2010 ◽  
Vol 6 (4) ◽  
pp. 381-384 ◽  
Author(s):  
Ala Birca ◽  
Claude Mercier ◽  
Philippe Major

Tuberous sclerosis complex (TSC) is associated with the potential development of benign hamartomas, including subependymal giant cell astrocytomas (SEGAs). Intracranial hypertension can be caused by SEGAs due to their propensity to block the foramen of Monro. The traditional management approach is to monitor SEGAs with periodic neuroimaging and to resect those that exhibit serial growth and/or cause clinical signs of intracranial hypertension. Recent observations suggest that rapamycin therapy may induce partial regression of SEGAs, therefore providing a potential alternative to resection. The authors present the case of an 8-year-old girl with bilateral SEGAs that led to progressive hydrocephaly and incipient signs of papilledema. Three months after initiating rapamycin therapy, the SEGAs exhibited significant reduction in size (82.6% on the left and 46.7% on the right), and the lesions remained stable 5 months later. Compared with previous case reports, similar or even greater antitumor efficacy was achieved with much lower trough levels of rapamycin (10–15 compared with 3.3–4.5 ng/ml, respectively). The authors discuss various aspects of rapamycin therapy and address unresolved issues that highlight the need for further prospective clinical trials.


Neurology ◽  
2004 ◽  
Vol 63 (8) ◽  
pp. 1457-1461 ◽  
Author(s):  
S. Goh ◽  
W. Butler ◽  
E. A. Thiele

2021 ◽  
Vol 749 ◽  
pp. 135714
Author(s):  
Kefu Wu ◽  
Jiong Yue ◽  
Kaifeng Shen ◽  
Jiaojiang He ◽  
Gang Zhu ◽  
...  

2007 ◽  
Vol 97 (3) ◽  
pp. 225-228 ◽  
Author(s):  
Hakan Selek ◽  
Hamza Özer ◽  
Sacit Turanli ◽  
Özlem Erdem

We describe a patient with a giant cell tumor in the talar head and neck of the left foot who was diagnosed as having osteochondritis dissecans and treated with arthroscopic drilling in this same location 3 years earlier. Giant cell tumors can be confused with several conditions, including giant cell reparative granulomas, brown tumors, and aneurysmal bone cysts. Giant cell tumors of bone typically occur in the epiphysis of long bones, including the distal femur and proximal tibia. They are uncommonly found in the small bones of the foot or ankle, and talar involvement is rare. Despite this rarity, the radiographic appearance and clinical signs of talar lesions should be considered in the differential diagnosis of nontraumatic conditions in the foot. (J Am Podiatr Med Assoc 97(3): 225–228, 2007)


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