scholarly journals Subependymal giant cell astrocytoma associated with hyperproteinorrhachia, presented with vp shunt malfunction: a rare clinical entity

2020 ◽  
Vol 10 (3) ◽  
pp. 97-99
Author(s):  
Nazmin Ahmed ◽  
Shamsul Alam ◽  
KM Taikul Islam ◽  
Moududul Haque ◽  
Md Ashik Ahsan ◽  
...  

Subependymal giant cell astrocytomas (SEGAs) are WHO grade I tumors, most frequently associated with tuberous sclerosis complex (TSC). TSC sometimes also called Bourneville’s disease is a neurocutaneous disorder characterized by hamartoma of many organs like skin, brain, eye and kidneys. In this report, we illustrate the case of SEGA with hydrocephalus, who presented with features of raised intracranial pressure, along with shunt malfunction due to shunt valve blockage with proteinaceous material. Patient underwent interhemispheric transcallosal approach and complete removal of tumor followed by ventriculo- atrial shunt and achieved favourable outcome.

2016 ◽  
Vol 8 (01) ◽  
pp. 058-061 ◽  
Author(s):  
Jitendra Nasit ◽  
Viren Vaghsiya ◽  
Srilaxmi Hiryur ◽  
Smita Patel

ABSTRACTSubependymal giant cell astrocytoma (SEGA) is a low grade (WHO Grade I) tumor, usually seen in patients with tuberous sclerosis complex and commonly occurs at a lateral ventricular location. Intraoperative squash cytologic features can help in differentiating SEGA from gemistocytic astrocytoma (GA), giant cell glioblastoma and ependymoma, in proper clinical context and radiological findings, which may alter the surgical management. Here, we present a case of SEGA with squash cytologic findings and a review of cytology findings of SEGA presently available in the literature. Loose cohesive clusters of large polygonal cells containing an eccentric nucleus, evenly distributed granular chromatin, distinct to prominent nucleoli, and moderate to the abundant eosinophilic cytoplasm in a hair-like fibrillar background are the key cytologic features of SEGA. Other important features are moderate anisonucleosis and frequent binucleation and multinucleation. The absence of mitoses, necrosis, and vascular endothelial proliferation are important negative features. Other consistent features are cellular smears, few dispersed cells, few spindly strap-like cells, rare intranuclear cytoplasmic inclusion, and perivascular pseudorosettes.


Author(s):  
Brian P O’Neill ◽  
Jeffrey Allen ◽  
Mitchell S. Berger ◽  
Rolf-Dieter Kortmann

Pilocytic astrocytoma (PA) (World Health Organization (WHO) grade I). A relatively circumscribed, slow-growing, often cystic astrocytoma occurring in children and young adults, histologically characterized by a biphasic pattern with varying proportions of compacted bipolar cells associated with Rosenthal fibres and loose-textured multipolar cells associated with microcysts and eosinophilic granular bodies. Most PAs are localized, macrocystic, and only marginally infiltrative. However some PAs, such as those arising in the optic pathways, are rarely cystic and may have an extensive infiltrative pattern but within a neuroanatomic pathway. Pleomorphic xanthoastrocytoma (PXA) (WHO grade II). An astrocytic neoplasm with a relatively favourable prognosis, typically encountered in children and young adults, with superficial location in the cerebral hemispheres and involvement of the meninges; characteristic histological features include pleomorphic and lipidized cells expressing glial fibrillary acidic protein and often surrounded by a reticulin network as well as eosinophilic granular bodies. Subependymal giant cell astrocytoma (SEGA) (WHO grade I). A benign, slow-growing tumour typically arising in the wall of the lateral ventricles and composed of large ganglioid astrocytes. It is the most common CNS neoplasm in patients with tuberous sclerosis.


Neurosurgery ◽  
1994 ◽  
Vol 35 (4) ◽  
pp. 748-750 ◽  
Author(s):  
Susumu Oikawa ◽  
Keizo Sakamoto ◽  
Norio Kobayashi

2005 ◽  
Vol 7 (4) ◽  
pp. 544-549 ◽  
Author(s):  
Tomotsugu Ichikawa ◽  
Akiko Wakisaka ◽  
Shigeru Daido ◽  
Soichiro Takao ◽  
Takashi Tamiya ◽  
...  

2016 ◽  
Vol 41 (4) ◽  
pp. E9 ◽  
Author(s):  
David Y. A. Dadey ◽  
Ashwin A. Kamath ◽  
Eric C. Leuthardt ◽  
Matthew D. Smyth

Subependymal giant cell astrocytoma (SEGA) is a rare tumor occurring almost exclusively in patients with tuberous sclerosis complex. Although open resection remains the standard therapy, complication rates remain high. To minimize morbidity, less invasive approaches, such as endoscope-assisted resection, radiosurgery, and chemotherapy with mTOR pathway inhibitors, are also used to treat these lesions. Laser interstitial thermal therapy (LITT) is a relatively new modality that is increasingly used to treat a variety of intracranial lesions. In this report, the authors describe two pediatric cases of SEGA that were treated with LITT. In both patients the lesion responded well to this treatment modality, with tumor shrinkage observed on follow-up MRI. These cases highlight the potential of LITT to serve as a viable minimally invasive therapeutic approach to the management of SEGAs in the pediatric population.


Neurosurgery ◽  
1991 ◽  
pp. 864 ◽  
Author(s):  
C W Shepherd ◽  
B W Scheithauer ◽  
M R Gomez ◽  
H J Altermatt ◽  
J A Katzmann

1979 ◽  
Vol 50 (1) ◽  
pp. 106-109 ◽  
Author(s):  
G. Michael Halmagyi ◽  
Leon P. Bignold ◽  
John L. Allsop

✓ A case is described of a subependymal giant-cell astrocytoma that occurred as a mural nodule within a cyst in the parietal lobe. The tumor recurred twice over a period of 47 years despite two extensive surgical resections. Neither the patient nor any of his children suffered tuberous sclerosis, a disease with which this type of astrocytoma is associated.


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