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.

Abordaje interhemisférico contralateral transfalcino para resección de empendimoma supratentorial extraventricular: presentación de caso y análisis de literatura / Contralateral Transfalcine Interhemispheric Approach for Resection of Extraventricular Supratentorial Empendymoma: Case Presentation and Literature Analysis

Introducción: los ependimomas supratentoriales extraventriculares son una entidad sumamente infrecuente, solo 45 casos han sido reportados en la literatura. La mayoría de los ependimomas se localizan a nivel infratentorial e intraventricular, solo en un grupo pequeño de casos no presentan continuidad con el sistema ventricular. El objetivo de este trabajo es describir y presentar el caso de un tumor inusual, cuya importancia radica en la baja prevalencia de casos reportados en la literatura y en la particularidad del abordaje quirúrgico seleccionado. Descripción del caso: paciente de sexo masculino, de 16 años de edad, con diagnóstico de lesión ocupante de espacio sólida-quística, a nivel frontal izquierdo, entre el giro frontal superior y giro del cíngulo. Se optó por realizar un abordaje interhemisférico contralateral transfalcino, logrando la resección total de la lesión tumoral. Se obtuvo el diagnóstico histopatológico de ependimoma Grado II según clasificación de la OMS. Discusión: en base a la información analizada en los diferentes artículos, los hallazgos imagenológicos y anatomopatológicos del caso presentado coinciden con lo relatado en la literatura acerca de los ependimomas supratentoriales extraventricualres. Es imprescindible la utilización de técnicas de inmunohistoquímica para la correcta tipificación del tumor ya que las características del mismo son fácilmente confundibles con otras entidades y su correcta graduación tiene implicancias pronósticas y terapéuticas. Conclusión: los ependimomas supratentoriales extraventricualares son neoplasias sumamente inusuales. La resección quirúrgica es considerada el tratamiento de primera línea para mejorar el pronóstico y la sobrevida. El abordaje interhemisférico contralateral transfalcino nos permitió lograr la exéresis total de la lesión tumoral, favoreciéndonos un adecuado ángulo de trabajo y reduciendo así la transgresión del parénquima cerebral

ntroduction: extraventricular supratentorial ependymomas are an extremely rare entity, only 45 cases have been reported in the literature. Most ependymomas are located at the infratentorial and intraventricular level, only in a small group of cases don ́t present continuity with the ventricular system. The aim of this paper is to describe and to present the case of an unusual tumor, the importance lies in the low prevalence of cases reported in the literature and in the particularity of the selected surgical approach. Case description: a 16-year-old male patient with a diagnosis of a solid-cystic space-occupying lesion, at the left frontal level, between the superior frontal gyrus and the cingulate gyrus, measuring 40mm x 50mm x 60mm. A contralateral transfalcine interhemispheric approach was chosen, achieving total resection of the lesion. The histopathological diagnosis of Grade II ependymoma was obtained according to WHO. Discussion: based on the information analyzed in the different articles, the imaging and pathological findings of the case presented coincide with what is reported in the literature about supratentorial extraventricular ependymomas. The use of immunohistochemical techniques is essential for the correct typing of the tumor since its characteristics are easily confused with other entities and its correct graduation has prognostic and therapeutic implications Conclusion: extraventricular supratentorial ependymomas are extremely rare neoplasms. Surgical resection is considered the first-line treatment to improve prognosis and survival. The contralateral transfalcine interhemispheric approach allowed us to achieve a total resection of the lesion, favoring an adequate working angle and thus reducing the transgression of the brain parenchyma

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.

Comparison of Clinical Efficacy between Spleen Total Resection and Spleen Partial Splenectomy in Treatment of Traumatic Spleen Rupture / 现代生物医学进展

Objective:To compare the clinical efficacy and safety between spleen total resection splenectomy and spleen partial splenectomy in the treatment of patients with traumatic spleen rupture.Methods:84 cases of patients with traumatic spleen rupture from March 2013 to March 2016 were selected and divided into two groups.42 cases in the spleen total resection group were treated with spleen total resection,while the other 42 cases in the spleen partial nephrectomy group were treated with spleen partial splenectomy.The operative effect,the levels of platelet count,serum IgA,IgG,IgM,CD3 +,CD4 +,CD8 +,CD4 + / CD8 + levels before and after treatment,the incidence of complications were compared between two groups.Results:The intraoperative blood loss,exhaust time,length of hospital stay of spleen partial nephrectomy group were shorter than those of the spleen total resection group,but the operation time of research group was longer than that of the spleen total resection group (P＜0.05).The platelet count,CD8+ of spleen partial nephrectomy group were lower than those of the spleen total resection group (P＜0.05).The IgA,IgG,IgM,CD3+,CD4+,CD8+,CD4+/CD8+ of spleen partial nephrectomy group were significantly higher than those of the spleen total resection group (P＜0.05).The incidence rate of complications in the spleen partial nephrectomy group was lower than that of the spleen total resection group (P＜0.05).Conclusion:Spleen partial splenectomy was more effective than spleen total resection in the treatment of traumatic splenic rupture,which had little effect on the platelet and immune function.

Presentación de un paciente con fascitis nodular pseudosarcomatosa del cuero cabelludo / Presentation of a Patient with Pseudosarcomatous Nodular Fasciitis of the Scalp

La fascitis nodular es una enfermedad infrecuente caracterizada por una tumoración de crecimiento rápido, curso benigno y características pseudosarcomatosas, descrita por Konwaler como fibromatosis pseudosarcomatosa subcutánea, frecuente en las partes blandas en extremidades superiores y tronco; en el cráneo es rara. El pronóstico es bueno y si la exéresis es completa se logra la curación total. Se presentó un paciente de 35 años, con lesiones multinodulares de seis años de evolución, única al inicio, con aumento de tamaño, número de lesiones y cambios de coloración luego, que ocuparon ambas regiones frontales y parietales, firmes, rosáceas, poco movibles. La tomografía axial computarizada de cráneo reveló lesiones multinodulares frontales en partes blandas con erosión de tabla externa. Se realizó tratamiento quirúrgico con exéresis total de la lesión e injerto en un segundo tiempo. La biopsia informó fascitis nodular pseudosarcomatosa del cuero cabelludo. Luego de dos años la lesión no ha recidivado.

The nodular fasciitis is an uncommon fast growth disease, benign and pseudosarcomatous, described by Konwaler as subcutaneous pseudosarcomatous fibromatosis, frequent in the upper extremities and the trunk, and rare in the scalp. The outcome is good if the resection is completely performed, and the cure is total. A 35- year- old patient with multinodular lesions six years ago of progress in the time was presented, which was in the frontal and parietal zone of the skull. The CT scan showed a little erosion of the bone. A total resection of the lesion was done. The biopsy showed pseudosarcomatous nodular fasciitis totally. No relapse in two years.

Application of Keyhole-style Total Removal of the Glands for Male Gynecomastia / 中国微创外科杂志

Objective To evaluate the effects and cosmetic results of total removal of the glands for male gynecomastia . Methods A total of 13 male patients with gynaecomastia were operated by using the method of keyhole-style total removal of the glands from March 2012 to March 2013.The fat lysate was injected into the subcutaneous area of the breast enlargement .Then an incision about 1.0 cm in length was made by the side of areola , through which a vacuum aspiration was carried out to suck the fat tissues.Also, the hypertrophical breast tissues were excised gradually .Finally, the incision was closed with medical biological adhesive or absorbable suture . Results The time of unilateral operation was 40-100 min ( average , 70 min) .The amount of blood loss was 20-50 ml (average, 30 ml).Subcutaneous effusion occurred in 2 cases (accompanying local hematoma in 1 case), which was cured by repeated aspiration for 3 days.The postoperative hospitalization was 2-5 days (mean, 2.8 days).All the patients were cured with incision healed by first intention and no obvious scars left .There was no necrosis on the nipple or areola .The breast curve was flat and natural .A total of 8 cases were followed up for 3 months, with satisfactory appearance .Three patients felt numbness of nipples and areola of breast after surgery and the symptom disappeared 3 months later . Conclusion Keyhole-style total removal of the glands for the male ynaecomastia has advantages of easy operation ,safety,and good cosmetic results .

Totally Ossified Metaplastic Spinal Meningioma

A 61-year-old woman with a very rare case of totally ossified large thoracic spinal metaplastic meningioma, showing progressing myelopathy is presented. Computed tomographic images showed a large totally ossfied intradural round mass occupying the spinal canal on T9-10 level. Magnetic resonance imaging revealed a large T9-10 intradural extramedullary mass that was hypointense to spinal cord on T1- and T2-weighted sequences, partial enhancement was apparent after Gadolinium administration. The spinal cord was severely compressed and displaced toward the right at the level of T9-10. Surgical removal of the tumor was successfully accomplished via the posterior midline approach and the histological diagnosis verified an ossified metaplastic meningioma. The clinical neurological symptoms of patient were improved postoperatively. In this article we discuss the surgical and pathological aspects of rare case of spinal totally ossified metaplastic meningioma.

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.

Efficacy of Preoperative Chemotherapy in a Choroid Plexus Carcinoma

Choroid plexus carcinomas(CPCs) are rare neuroectodermal malginancies occuring mostly in young children of less than two years old, of which prognosis have been reported extremely grave. Generally, the extent of surgical resection has been regarded the most significant prognostic factor. However, the inherent hypervascularity and softness of these tumors make the total removal very difficult. We present our experience in a 18-months-old child with a large intraventricular CPC, of which total resection was achieved by the use of preoperative neoadjuvant chemotherapy with ICE regimen(Ifosfamide, Cisplatinum, Etoposide).

Quality of Life in Patients with Vestibular Schwannoma after Treatment: Short Form-36 Based Study

OBJECTIVE: To evaluate the quality of life(QOL) of patients with vestibular schwannoma(VS) and to compare QOL among subtotal resection(STR) group, radical resection(RR) group, and gamma knife surgery only (GKS) group, we report a retrospective analysis of consecutive 104 patients with VS who were treated between 1997 and 2000. METHODS: The STR group included 31 patients, RR group did 26, and GKS only did 47. Various approaches such as retrosigmoid transmeatal approach(45), translabyrinthine approach(4), transotic approach(2), middle fossa approach(3), and combined approach(3) were used. A questionnaire concerning postoperative symptoms and the Short Form 36(SF-36) QOL instrument were mailed to 104 patients with VS. Follow-up imaging studies, Karnofsky performance scale(KPS) scores, and SF-36 scores at the last follow-up were assessed. The mean duration of follow-up was 33.4 months. RESULTS: The survey response rate was 51%(53 patients). The postoperative QOL in VS patients, as quantified by the eight SF-36 health scales was less than the appropriate matched healthy standard. Furthermore there were differences between the doctor's assessment(KPS) and the patient's assessment(SF-36). Facial nerve outcome had little influence on QOL in VS patients. CONCLUSION: Patients with VS have a significant impairment of their QOL. In the selection of treatment options for VS, the QOL of patient should be considered seriously.

A Huge Neurilemmoma of the Cauda Equina

We are report a case of surgically treated a huge neurilemmoma in the lower thoracic and lumbosacral areas. A 57-year-old women presented with low back pain, motor weakness of the both lower extremities and urination difficulty. Magnetic resonance imaging revealed a huge enhancing lesion at the L3-S1 levels and isolated two small enhancing lesions at the T12 and L1 level respectively. This case was treated with extensive laminectomy from T12 through S1 level and total resection. The pathological findings were consistent with neurilemmoma. The patient's preoperative neurologic deficit has been recovered and no postoperative complications developed.

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.