Infantile (congenital) anaplastic intracranial solitary fibrous tumor/hemangiopericytoma—A case report with brief literature review

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a rare primary central nervous system (CNS) tumor, included in the World Health Organization (WHO) 2016 classification. Very few cases have been described in the literature so far, especially the infantile type. It is a mesenchymal tumor of the fibroblastic type, characterized by the fusion of NAB 2 and STAT 6 genes. A 10-month-old boy presented to our neurosurgery department with complaints of increasing head circumference since 1 month of age. The magnetic resonance imaging (MRI) showed a space-occupying lesion measuring 8.2 cm × 7 cm × 6.9 cm in the fronto-temporo-parietal region with a clinical diagnosis of glioma/atypical teratoid rhabdoid tumor (ATRT). The microscopy revealed a spindle cell tumor arranged in a patternless pattern with variable cellularity, increased mitosis, and areas of coagulative necrosis. The immunohistochemistry showed vimentin, CD 34, STAT6, CD99 positivity whereas Glial fibrillary acidic protein, Epithelial membrane antigen, and S-100 negativity. Hence, a diagnosis of anaplastic SFT/HPC (grade-III) was rendered. The patient improved after gross total resection (GTR). The primary intracranial congenital SFT/HPC are extremely rare, often a clinico-radiologically misdiagnosed entity. Thus, the immunohistochemistry/molecular study in addition to histology is mandatory for accurate diagnosis.

A study of retrospective analysis of the outcome of parasagittal meningioma

Background: Meningioma is a common benign tumours treated by neurosurgeons. They develop from arachnoid cap cells within the thin spider web-like membrane covering the brain and spinal cord. The arachnoid is one of the three protective layers of the central nervous system collectively known as the meninges surrounding the brain and spinal cord. The aim of our study is to analyze the outcome of parasagittal meningioma.Methods: The details of the patients diagnosed with parasagittal meningioma and operated upon during the study period of 2009-2013 were retrospectively collected from the medical records kept in the department of neurosurgery and different study parameters were documented for analysis.Results: Out of the total 40 patients with meningioma 15 (37.5%) patients had parasagittal meningioma in anterior 1/3rd of sagittal sinus, 21 (52.5%) patients had parasagittal meningioma in middle 1/3rd of sagittal sinus and 4 (10%) patients had parasagittal meningioma in posterior 1/3rd of the sagittal sinus and 28 (70%) patients  are treated by gross total resection (GTR), 7 (17.5%) patients were treated by subtotal resection (STR) and 5 (12.5%) patients were treated by dendritic cell (DC) and postoperatively 22 patients (55%) did not develop any complications. Weakness develops for 12 patients (30%), followed by recurrence in 3 patients (7.5%) and 3 patients (7.5%) died.Conclusions: Conservative resection of the tumour with residual lesion within the sagittal sinus followed by adjuvant treatment with modalities like radiotherapy, chemotherapy or targeted therapy for the residual lesion is an accepted mode of treatment.

Chordoid Glioma of the Third Ventricle, a Rare Tumor with an Unexpected Outcome

Chordoid glioma is a rare tumor of the third ventricle whose imaging features are difficult to distinguish from other more common lesions in this location. There are only 83 cases described so far in the literature. Although gross total resection (GTR) is the treatment of choice, immediate postoperative mortality with this approach can be as high as 29%, and morbidity among survivors can reach 67%. We report a case of a male patient of advanced age, with a third ventricle mass lesion, who presented with a progressive right temporal hemianopia. Imaging was compatible with craniopharyngioma, meningioma or even metastasis. Chordoid glioma was not considered in the differential diagnosis. The patient underwent surgery and GTR was achieved. There were no postoperative complications, and the patient was discharged from the hospital three weeks later. Unexpectedly, two days afterwards, he suffered a major brainstem hemorrhagic stroke and, unfortunately, died.

O glioma cordoide é um tumor raro do terceiro ventrículo, e as suas características imagiológicas são difíceis de distinguir de outras lesões mais comuns nesta localização. Até a data presente, existem apenas 83 casos de gliomas cordoides descritos na literatura. A remoção macroscópica total destes tumores deve ser o tratamento de escolha; no entanto, a mortalidade pós-operatória imediata pode chegar aos 29%, e a morbilidade pode atingir os 67% entre os sobreviventes. Nós descrevemos o caso de um homem idoso com uma lesão tumoral no terceiro ventrículo, que se manifestou com uma hemianopsia temporal direita progressiva. Os exames de imagem eram compatíveis com craniofaringioma, meningioma ou até metástase. O glioma cordoide não foi considerado como uma das hipóteses no diagnóstico diferencial inicial. O paciente foi submetido a cirurgia, tendo-se obtido a remoção macroscópica total. Não houve qualquer complicação no período pós-operatório, e o paciente teve alta hospitalar após três semanas. Inesperadamente, dois dias após a alta clínica, o paciente sofreu um AVC hemorrágico do tronco cerebral, e acabou por falecer.

A Long-Term Survival Case of a Primary Malignant Intracerebral Nerve Sheath Tumor

We report a long-term survival case of a primary malignant intracerebral nerve sheath tumor (MINST) occurring in the right frontal lobe of a 13-year old boy. After the gross total resection (GTR), we have performed radiation therapy but it recurred 50 months after the surgery, so the second GTR was performed. Later, second tumor recurrence was found 4 months after the second surgery. Subsequently the third GTR, radiotherapy, and chemotherapy were carried out. At present, the patient has been remaining alive for 77 months without evidence of tumor recurrence. According to the previous reports, the primary MINST is very rare : there are only 8 cases reported. It is also a fast-growing, invasive tumor with poor outcome. This is the first case that had no recurrence for 50 months after the surgery among the reported cases that had been followed up for more than 5 years. It is supposed that a period of recurrence free survival after GTR and low mitotic activity are associated with the patient's prognosis. A GTR followed by adjuvant radiation therapy and chemotherapy will be recommended to patients of MINST.

Clinical Outcome of the Mixed Glioma: Preliminary Analysis

The authors analyzed preliminary outcome of 10 patients with mixed glioma(3 patients with low-grade oligoastrocytoma and 7 patients with anaplastic oligoastrocytoma) who underwent surgical resection and were treated or are in treatment with or without adjuvant therapy at our institute since May 1993. In the low-grade oligoastrocytoma group, gross total tumor resection was done in all three cases and postoperative radiation therapy was performed in 2 cases. In the anaplastic oligoastrocytoma group, all seven cases were divided as to the amount of tumor removal(gross total resection; 4 cases, subtotal resection; 1 case, partial resection; 2 cases) and clinical course and prognosis were analyzed as to performing postoperative radiation therapy with or without chemotherapy. In the low-grade oligoastrocytoma group, one patient who didn't undergo postoperative radiation therapy suffered from tumor recurrence that showed histopathologically malignant transformation. In the anaplastic oligoastrocytoma group, 2 patients who underwent subtotal tumor resection and partial tumor resection with postoperative radiation therapy showed tumor progression and histopathologically more malignant transformation. The authors propose that gross total tumor resection with postoperative radiation therapy in low-grade oligoastrocytoma and adding chemotherapy(especially with procarbazine, lomustine, vincristine; PCV regimen) in anaplastic oligoastrocytoma appear to be associated with more prolongation of patient's survival.

A Case of Choroid Plexus Carcinoma with Rapid Dissemination after Gross Total Resection

One to three percent of childhood brain tumors are choroid plexus tumors. Of these, 30-40% are considered to be choroid plexus carcinoma. We report one case of choroid plexus carcinoma without parenchymal invasion, which showed rapid dissemination along whole CSF pathway and developed postoperative hydrocephalus in spite of gross total resection.

Posterior Transpetrosal Transtentorial Approach for Tumors of the Petrous and Clival Regions: Experience with 25 Consecutive Cases

Because of their proximity to adjacent vital structures, and deep and narrow operative field in the surrounding compact bony structures, tumors located in the clival or petroclival regions represent a formidable technical challenge to neurosurgeons. Between April 1990 and May 1996, 25 patients(eight males and 17 females with a mean age of 44 years), harboring clival or petroclival tumors underwent surgery in our department involving the posterior transpetrosal transtentorial approach. The median follow-up period was 24 months. A total of 28 operations were performed ; in three cases, these involved two stages, in which different approaches were used. The surgical pathology included 15 meningiomas, seven neurinomas(four acoustic and three trigeminal), and one case each of myxoid chondrosarcoma, chondroid chordoma, and paratrigeminal epidermoid. Tumor size ranged from 2 to 8cm ; 16 were larger than 4cm. According to the extent of petrous resection, three different approaches were used ; retrolabyrinthine in ten cases, translabyrinthine in four, and transcochlear in eleven. The superior petrosal sinus was always sacrificed and the tentorium completely cut. Gross total removal was accomplished in 13 cases, and subtotal removal in 12. The causes of incomplete removal included cavernous sinus invasion in four cases, severe adhesion to the brain stem in two, extremely high consistency and vascularity of the tumor in two, insufficient exposure to the tumor in two, and unexpected residual tumors seen in two cases on postoperative MRI. There was no operative mortality in this series and the most common complication was cranial nerve palsy, which occurred in 15 cases. Other complications included hemiparesis in two cases, CSF leakage in two and an epidural hematoma in one. The authors conclude that the posterior transpetrosal transtentorial approach is advantageous for resecting petroclival tumors because, by avoiding unnecessary brain retraction, it significantly reduces the risk of postoperative complications, and helps provide shorter access and wider exposure of the lesion.

A Case of Desmoplastic Infantile Ganglioglioma / 영남의대학술지

The desmoplastic infantile ganglioglioma is very rare cerebral tumor. It has been known to be characterized by its voluminious size, intense desmoplasia and the frequent presence of astrocytic and ganglioglionic differentiation. Also, It is usually presented in infantile period and predilected in the frontal and parietal lobes. We treated a huge desmoplastic infantile ganglioglioma(8?7?6cm) on the right frontotemporo-parietal area with only gross total resection. It includes solid mass & several cysts and shows the areas of the proliferation of spindle cells exhibiting storiform pattern in dense desmoplastic stroma and the areas composed of spindle shaped glial component.

Surgical Outcome of Choroid Plexus Tumors in Adults : Experience of 5 Cases

A retrospective analysis of five adult patients with choroid plexus tumor(four choroids plexus papillomas, one choroids plexus carcinoma who underwent surgical resection at our institute between February 1991 and October 1995 was performed. The study group included two males and three females with a mean age of 34.0 years(range 21 to 24 years). Two of four choroids plexus papillomas were removed gross totally and one of these was irradiated postoperatively. The remaining two patients with choroids plexus papilloma underwent a subtotal surgical resection and one of them received radiation therapy. During follow-up, the authors could not find any difference in the size of the residual tumor or in the patient's KPS between the irradiated groups. There was also no response to postoperative radiation therapy in a patient with choroids plexus carcinoma who underwent a subtotal resection. The author's clinical experiences suggest that the use of radiation therapy in adult patients with choroids plexus tumor is not effective, and thus gross total resection should be attempted during the primary operation of such tumors.