Abstract
Desmoplastic gangliogliomas (DIG) are rare primary neoplasms that comprise 0.5–1.0% of all intracranial tumors. Clinically, there are two forms of DIG, the infantile and the non-infantile. These tumors invariably arise in the supratentorial region and commonly involve more than one lobe, preferentially the temporal and frontal. On neuroimaging are seen as large hypodense cystic masses with a solid isodense or slightly hyperdense superficial portion. The histologic diagnosis is characterized by the presence of three different cell lines: astrocytic, neuronal, and primitive neuroectodermal marker sites, which were demonstrable. The treatment of choice is radical surgical excision, and if this is done, achieved complete healing of the patient does not require additional treatment. A literature review of DIG was compiled through Medline/Ovid using the keywords “desmoplastic infantile ganglioglioma”, “desmoplastic non-infantile ganglioglioma” covering the years 1984–2009. We present a review of a total of 113 cases of infantile (94) and non-infantile gangliogliomas (19) published to date, examining the clinical, radiologic, surgical, and pathological aspects, as well as the outcome. Desmoplastic gangliogliomas represent a rare tumor group with two well-defined age groups, the children and non-children. Desmoplastic infantile gangliogliomas are the most common and occur in children below 5 years of age, and the large majority of them present within the first year of life. Surgery is the treatment of choice and no complementary treatment is needed in cases of complete tumor resection.
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Hamit Selim Karabekir, Afyon, Turkey
Gelabert-Gonzalez et al. defined desmoplastic gangliogliomas (infantile or non-infantile) clearly and summarized the literature for 29 years from 1980 to 2009. They looked at the age, gender, presenting symptoms, clinical findings, localization, size, diagnostic and surgical procedures, and survival of the cases. Also, they included the pathological findings and immunohistochemical assays. In the data of the literature, they commend that the end surgical treatment is the first and best choice if complete resection can be realized. This review is very useful for looking at desmoplastic gangliomas for the last 30 years and can show the way how to approach the cases to researchers. I congratulated the authors for catching the readers' interest in an actual way.
Kaoru Kurisu, Hiroshima, Japan
Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature submitted by Gelabert-Gonzales Miguel et al. is well discussed and precisely described in almost all aspects of the “special tumor” on etiology, histological character, image diagnosis, natural course, clinical presentation, treatment strategy, outcome, and prognosis, except only one issue. The authors emphasized the benign prognosis after a total or gross total removal of the tumor. But very less intraoperative findings during surgery we could find. Generally speaking, if the patient with some disease can be cured by surgery, the surgeons are eager to know how to remove the lesion without additional impairment. I think these kinds of tumor, DIG/DNIG, can be easily removed by the different “consistency” between tumor and surrounding brain tissue. The consistency of DIG/DNIG is relatively firm or of high consistency, so the operators can “relatively” easily remove the tumor in total or gross total fashion (Sugiyama et al, Good clinical course in infant with desmoplastic cerebral neuroepithelial tumor treated by surgery alone, J Neurooncol 59;63–69, 2002). This is just only one issue that I have to add to this wonderful review article.
Dattatraya Muzumdar, Mumbai, India
Gelabert-Gonzalez et al. have presented a comprehensive review of 119 cases of desmoplastic infantile and noninfantile ganglioglioma described in the literature. They have compiled the salient clinical, diagnostic and therapeutic features of these relatively benign tumors into various segments, which provide a quick overview.
Desmoplastic infantile ganglioglioma was first described in 1987. The term is self-explanatory. These tumors are usually seen in infancy, have prominent desmoplasia, and have neuronal differentiation. It is surprising to note that the most common presenting symptoms were macrocephaly in 40.7%, suggesting that it forms an entity to be included in the differential diagnosis of a large head in infancy. The electroencephalogram is normal in most cases inspite of seizures noted in 23 (23.7%) of patients, which is unusual. In addition, there is no higher incidence of developmental delay at presentation or at follow-up, which denotes that the integrity of brain substance is maintained in utero. CT and MR imaging features are not specific for DIG. They are heterogenous tumors, having solid–cystic components but show homogenous enhancement. Purely solid tumor is rare. Calcification is seen in few cases.There is no specific preference for hemispheric predominance, although it is most commonly found in the temporal lobe. The volume of the tumors or cysts ranged from 5 to 1000 cc, which suggests a differential growth pattern, which does not necessarily correlate with an oncologic progression. Although they can attain a voluminous size, they are relatively benign and long-term prognosis is usually good. Total excision is curative. Due to the large size seen at presentation, as well as diffuse vascularization of DIGs, blood loss during surgery poses increased risk for hemodynamic instability. Deep and inaccessible locations of the tumor preclude a radical excision. There is clear demarcation between the cortical surface and the tumor tends to fill the Virchow–Robin spaces. Adherence to the duramater is not seen in about 40% of the cases. The histological features are essentially benign, and proliferative index of these tumors is low. These characteristics suggest that the DIG have a low invasive potential and have a predictable behavior. Surgery is the mainstay of the treatment protocol. Residual or recurrent tumors can be considered for repeat surgery. There is a tendency for spontaneous regression in few cases due to induction of apoptosis. Radiation and chemotherapy is unlikely to be overwhelmingly responsive. Inspite of a benign nature of the DIGs, only seven reported cases survived beyond 10 years. The median survival is 36.7 months. There is a need for further research into the genetics of these tumors for better understanding of the biology and further evolution of therapeutics.
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Gelabert-Gonzalez, M., Serramito-García, R. & Arcos-Algaba, A. Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature. Neurosurg Rev 34, 151–158 (2011). https://doi.org/10.1007/s10143-010-0303-4
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DOI: https://doi.org/10.1007/s10143-010-0303-4