Solitary intraventricular Hodgkin lymphoma post-transplant lymphoproliferative disease (HL-PTLD): Case report.

Lymphomas affecting the central nervous system (CNS), both primarily and secondarily, are uncommon malignancies. Immunosuppressed states, including iatrogenic immunosuppression following organ transplantation, are the most significant risk factors for developing primary CNS lymphoma (PCNSL). Post-transplant lymphoproliferative disease (PTLD) is a well described complication following bone marrow or solid organ transplantation. PTLD is usually a systemic disease with occasional CNS involvement. The incidence of CNS involvement in PTLD is low, and the majority of these cases tend to be PCNSL. Hodgkin lymphoma PTLD (HL-PTLD) constitutes only a very small percentage of PTLD. We report a rare case of a primary intraventricular CNS classical HL-PTLD in a male patient, 18 years following renal transplantation. The location allowed for safe neurosurgical intervention which resolved the symptom of elevated intracranial pressure and allowed for induction of a Rituximab-based chemotherapy regimen. Both the ventricular location of the PTLD and Hodgkin Lymphoma PTLD are themselves individually quite rare and have not previously been reported together. The unique location allowed safe neurosurgical intervention which quickly resolved the symptom of elevated intracranial pressure and allowed for induction of a Rituximab-based chemotherapy regimen.

Rosette-Forming Glioneuronal Tumor Originating From the Spinal Cord: Report of 2 Cases and Literature Review.

Rosette-forming glioneuronal tumor (RGNT) is a recently recognized and rarely encountered tumor occurring in the fourth ventricle. RGNT was first described as a new entity for the distinct clinicopathologic features by Komori et.al. in 2002. Histologically, it is composed of 2 distinct features: a glial component, resembling pilocytic astrocytoma, and a neurocytic component forming neurocytic rosettes and/or perivascular rosettes. We report 2 extremely rare cases of RGNT arising from the spinal cord, which were misdiagnosed as ependymoma and astrocytoma preoperatively. Symptoms included dissociated sensory disturbances and episodic pain and fatigue of 2 years' duration in case 1, as well as motor disturbance for 2 months' duration in case 2. Magnetic resonance imaging (MRI) revealed these masses in the thoracolumbar (T7-L1) and cervicothoracic (C3-C7) spinal cord. The solid component appeared hypointense in T1-weighted MRI sequences, hyperintense in the T2-weighted MRI sequences, and heterogeneous in MRI images enhanced with gadolinium contrast medium in both cases. Gross total resection was performed via a median laminectomy. Postoperative pathological examination confirmed the diagnosis of RGNT. In addition, extensive analysis of genetic mutations was performed to explore the relationship with glioma, including telomerase reverse transcriptase promoter, isocitrate dehydrogenase 1/2, BRAF-V600E, and O(6)-methylguanine-DNA methyltransferase promoter. No radiotherapy or chemotherapy were performed in these two cases. As of the latest follow-up, both patients had a good prognosis. Given the widely varying clinical characteristics of, prognosis of, and treatments for spinal tumors, differential diagnosis is of great importance before surgery. Consideration of the tumor location and the patient's age and sex, in combination with the imaging features, may be the best approach to narrowing the differential diagnosis. Surgery is the preferred treatment for RGNT. We do not recommend to implement adjuvant radiotherapy and chemotherapy in these patients except the invasive or recurrent tumors. Further examination and routine follow-up should be recommended to estimate the long-term prognosis.

Primary Intraventricular Leiomyoma in an Immunocompetent Patient: First Case Report and Review of the Literature.

BACKGROUND: Primary intracranial leiomyoma is an extremely rare occurrence of a low-grade mesenchymal tumor characterized by a proliferation of smooth muscle cells. When present, these lesions predominantly occur in immunocompromised patients in the setting of infection or transplant and have not been known to involve the ventricular system of the brain. In this report, we describe a case of primary leiomyoma of the lateral ventricle in an immunocompetent patient. CASE DESCRIPTION: A 30-year-old man with no medical history presented with progressive diplopia and occipital headaches. Magnetic resonance imaging of the brain revealed a homogenously enhancing mass of the left lateral ventricle with associated cerebral edema. The patient underwent interhemispheric transcallosal craniotomy for resection for symptom alleviation and surgical diagnosis. Histopathology and immunohistochemistry was subsequently consistent with that of leiomyoma. Genetic probing for Epstein-Barr virus was negative. Computed tomography of the chest and abdomen failed to uncover a primary tumor. The patient did well postoperatively and was discharged 3 days after resection. At a two-and-a-half year follow-up, there continued to be no radiologic or clinical evidence of recurrence. CONCLUSIONS: To date and to our knowledge, there are fewer than 25 reported cases of primary intracranial leiomyoma, with only 13 occurring in immunocompetent individuals. We believe this is the first report of this tumor type occurring within the ventricular system of the brain. As such, leiomyoma should be considered as a rare etiology in the differential diagnosis of intraventricular lesions.

Coexpression of glial and neuronal markers in the neurocytic rosettes of rosette-forming glioneuronal tumors.

Rosette-forming glioneuronal tumor of the fourth ventricle (RGNT) is a new entity in the WHO 2007 Classification of Tumors of the Central Nervous System. RGNT has two components: neurocytic rosettes and low-grade gliomas. Neurocytic rosettes are conventionally described as consisting of uniform neurocytes. However, some studies have reported rosette-forming tumor cells that expressed glial markers such as Olig2. We indicated the expression of glial markers including Olig2, cyclinD1, glial fibrillary acidic protein (GFAP), and platelet-derived growth factor receptor alpha (PDGFRα) in the neurocytic rosettes in our previous study, and we suggested that these tumor cells had a heterogeneous nature. In this study, we used double and triple immunostaining to demonstrate that these tumor cells have both glial and neuronal characteristics. We found that rosette-forming tumor cells coexpressed Olig2/cyclinD1 and synaptophysin. Furthermore, the cores of the rosettes coexpressed GFAP/PDGFRα in the peripheral zone and synaptophysin in the central zone. These findings imply that rosette-forming tumor cells have a similar nature to neuronal-glial progenitor cells, and we believe that the nomination "neurocytic rosette" may be unsuitable given their heterogeneous nature. Our study appears to clarify some of the properties of RGNT tumor cells and may help elucidate the histogenesis of RGNT.

Rosette-forming glioneuronal tumor of the fourth ventricle with bilateral olivary degeneration.

Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle has been recognized as a new type of glioneuronal tumor. RGNTs are typically located in the infratentorial midline with involvement of the fourth ventricle. They occasionally involve the aqueduct and/or vermis. RGNTs of unusual anatomical sites or those with unusual findings have been reported. The present case reports describe RGNT of the fourth ventricle with bilateral olivary degeneration. It is important to accumulate imaging findings and biological behaviors of RGNTs given the limited number of cases.

Uncomplicated neurosurgical resection of a malignant glioneuronal tumour under haemostatic cover of rFVIIa in a severe haemophilia patient with a high-titre inhibitor: a case report and literature review of rFVIIa use in major surgeries.

The development of inhibitors following factor VIII replacement therapy is a serious complication in severe inherited haemophilia. Whereas significant experience, notably in orthopaedic surgery, is now obtained with the use of bypassing agents in haemophilia with high-titre inhibitor, new surgical challenges might occur due to patients' increasing life expectancy. A 56-year-old severe haemophilia A patient with a high-titre inhibitor was diagnosed for probable right temporoparietal malignant glioneuronal tumour on cerebral magnetic resonance imaging (MRI) (4 cm x 3 cm cerebromeningeal tumour with perilesional oedema and transfalcial herniation) requiring total resection. Then recombinant activated FVII (rFVIIa) was chosen as the haemostatic agent: bolus of 270 microg kg(-1) every 2 h during the first 24 h, 180 microg kg(-1) every 3, 4 and 6 h, respectively, at days 2-3, from days 4-10 and finally from days 11-15. Tranexamic acid was associated. Pre- and postoperative courses were uneventful, the surgical procedure being assessed at optimal haemostatic condition without any unusual haemorrhage on MRI controls, diffuse intravascular coagulation criteria or thromboembolic event. Intensive rFVIIa therapy has shown to be safe and effective in this first reported neurosurgery about a malignant tumour exhibiting to a high-bleeding risk notably in haemophilia with high-titre inhibitor. The use of lower doses of rFVIIa might have been possible; however, in the absence of accurate test for monitoring rFVIIa therapy, the potentially life-threatening complications of this procedure required maximum haemostasis with high rFVIIa doses.

A rosette-forming glioneuronal tumor of the spinal cord: the first case of a rosette-forming glioneuronal tumor originating from the spinal cord.

Rosette-forming glioneuronal tumors of the fourth ventricle are rare brain tumors, and only 19 such lesions have been previously reported. This report presents the first case of a rosette-forming glioneuronal tumors arising from the spinal cord. A 44-year-old woman presented with a 15-year history of dissociated sensory disturbance of the lower extremities that gradually spread through her upper extremities. She also experienced continuing motor disturbance. Magnetic resonance imaging demonstrated a mass in the cervicothoracic spinal cord that suggested an intramedullary spinal tumor. A total gross resection of the tumor was performed. As is typical of rosette-forming glioneuronal tumors of the fourth ventricle, this spinal cord example manifested neurocytic and astrocytic components. Neurocytic rosettes were detected in the neurocytic component, and the center of rosettes showed positive immunostaining for synaptophysin. The astrocytic component showed characteristic features of a pilocytic astrocytoma, as is often the case in the fourth ventricle examples.

Intraventricular dysembryoplastic neuroepithelial tumor: case report.

OBJECTIVE: The most common localization of dysembryoplastic neuroepithelial tumors (DNTs) is the supratentorial cortex, often in the temporal lobe. However, intraventricular localization of a DNT is extremely rare. CLINICAL PRESENTATION: A 30-year-old woman presented with a 1-year history of epileptic seizures. The seizures had not been controlled despite standard doses of antiepileptics. INTERVENTION: Neuroimaging results demonstrated a lesion located in the occipital horn of the right lateral ventricle. The lesion was totally removed. Based on histopathological and immunohistochemical evaluation, a DNT was diagnosed. Over the course of the next 8 months, the patient's epileptic seizures were under control. The most recent neuroimaging examinations revealed neither residual nor recurrent tumor. CONCLUSION: Because DNTs are surgically curable and neither radiotherapy nor chemotherapy is required after surgery, recognition of an intraventricular DNT in this location is extremely important.

Rosette-forming glioneuronal tumour of the fourth ventricle: report of a case with clinical and surgical implications.

A 32-year-old woman presented with a 2-month history of episodic headache, cervical pain and neck rigidity. Neurological examination showed a moderate dysmetria. Magnetic resonance imaging (MRI) revealed a mass occupying the fourth ventricle. The patient underwent median sub-occipital craniotomy with total excision of the lesion well demarcated except for a portion infiltrating the right side of the IV ventricle wall. In the post-operative course the patient developed VI and VII right cranial nerves palsy and worsening of dysmetria. MRI confirmed the complete removal of the tumour without signs of recurrence. The pathological diagnosis was rosette forming glio-neuronal tumour (RGNT). At present this is the 13th RGNT reported in literature. These lesions are considered low-grade tumours (WHO I). Nevertheless, the case here reported, like in 6 of the 12 cases in literature, developed disabling post-operative deficits. To establish the therapeutic choice long-term follow-up studies are needed.

Epidemiology and pathology of intraventricular tumors.

Tumors that primarily or exclusively involve the ventricular system constitute a rare and heterogeneous group. Certain histologic tumor types predominantly occur in children, whereas others are more common in adults. Tumor location provides additional clues to correct diagnosis. When used in conjunction with clinical and radiologic data, histopathologic features can distinguish among this wide range of possibilities to provide the correct diagnosis for optimal patient management.

[Acute hepatitis B virus after chemotherapy for a case with germinoma].

A 28-year old man with HCG-producing germinoma had undergone chemotherapy and radiotherapy. On admission for the fifth session of maintenance chemotherapy, he was found to be positive for hepatitis B (HB)s antigen, but negative for HBs antibody. HBs antigen had been negative during previous admissions. Since liver function was normal, the patient underwent chemotherapy. During myelosuppression after chemotherapy, liver dysfunction developed and acute HB was diagnosed. He fortunately showed seroconversion 2 months after onset. Serum immunological examinations are required for patients receiving chemotherapy.

[Antigenic expression in human choroid plexus carcinoma: report of 2 cases]. / Expressão antigênica em carcinomas do plexo coróide humano: relato de dois casos.

Primary neoplasms of choroid plexus are rare. Six morphological variants have been described: papillary, cystic, acinar, mucus-secreting, oncocytic, and anaplastic. The anaplastic variant, the so-called choroid plexus carcinoma, is the rarest of all and can metastasize. The differential diagnosis of the anaplastic variant of choroid plexus neoplasms with adenocarcinomas, melanomas and undifferentiated neoplasms can be troublesome chiefly in adults. The now large use of immunocytochemical techniques in tissue section has become a powerful tool in the analysis of cell lineages, tumoral and non-tumoral. Nevertheless, the choroid plexus neoplasms have shown a complex and a somewhat confusing pattern of antigenic expression. In two choroid plexus carcinomas (one localized in the right lateral ventricle from a boy of 1 year and 9 months old, and the other localized in the left lateral ventricle from a girl of 3 years old) the following antigens were searched (using the avidin-biotin-peroxidase complex): glial fibrillary acidic protein (GFAP) with monoclonal and polyclonal antibodies; cytokeratins of 40-50kDa, cytokeratins of 60-70kDA (callus cytokeratin), neuronal specific enolase (NSE) and S-100 protein with monoclonal antibodies. The two neoplasms showed immunoreactivity against NSE, S-100 protein and cytokeratin of 40-50kDA. The neoplasm of the boy exhibited glial differentiation having immunoreactivity against GFAP with monoclonal and polyclonal antibodies.

Expressäo antigênica em carcinomas do plexo coróide humano: relato de dois casos / Antigenic expression in human choroid plexus carcinoma: report of 2 cases

Neoplasias provenientes do epitélio de revestimento do plexo coróide säo incomuns, tendo sido descritos 6 padröes morfológicos. O padräo anaplásico, também denominado carcinoma do plexo coróide, é o de menor freqüência e pode dar metástase fora do SNC. A distinçäo histológica desses tumores, particularmente da variedade anaplásica, com outras neoplasias primárias e metastáticas no SNC pode ser fidifícil. O uso de técnicas imunocitoquímicas em parafina tem-se mostrado útil no esclarecimento das linhagens tumorais. Os papilomas do plexo coróide têm, no entanto, sido objeto de controvérsia, por sua complexa expressäo antigênica. Usando a técnica de imunoperoxidase (sistema avidina-biotina-peroxidase) pesquisaram-se, em dois casos da variedade anaplásica, os seguintes marcadores: proteína glial fibrilar ácida (GFAP) com anticorpo monoclonal e policlonal; ceratinas de 40-50kDa, ceratinas de 60-70kDa (callus ceratina), enolase neuronal específica (NSE) e proteína S-100, com anticorpos monoclonais. Os dois tumores mostraram positividade para NSE, proteína S-100 e ceratina de 40-50kDa: uma das duas neoplasias mostrou diferenciaçäo glial, revelando positividade para GFAP tanto como anticorpo monoclona quanto policlonal

Choroid plexus papillomas: an immunohistological study of 16 cases.

Eleven benign and five malignant choroid plexus papillomas in children and adults were studied immunohistologically with a panel of antibodies against glial fibrillary acidic protein, S-100 protein, vimentin, desmin, epithelial membrane antigen and two different cytokeratins (LP34 and CAM 5.2). Glial fibrillary acidic protein was focally present in epithelial tumour cells, in cells within solid areas and in clusters of cells within the stroma. S-100 protein was diffusely present in tumour cells with focal accentuation. Vimentin was present in all cases, the epithelial tumour cells demonstrating strong and diffuse positivity with perinuclear accentuation; malignant tumours, however, showed stronger positivity than benign ones. Desmin was negative in all tumours. Epithelial membrane antigen and cytokeratin (LP34) were demonstrated in four of five malignant tumours but were absent in the benign ones; CAM 5.2 reacted with four of five malignant tumours and also reacted with eight of the 11 benign ones. The significance of these findings is discussed in respect of the ontogeny of these tumours.

Distribution of epithelial membrane antigen in normal and neoplastic human ependyma.

The ependyma and choroid plexus of 23 normal brains and 20 ependymal tumors were examined immunohistochemically for expression of epithelial membrane antigen (EMA) using a specific monoclonal antibody. The ependyma of normal brains showed three patterns of immunoreactivity: membrane immunoreactivity confined to the luminal surface; irregular punctate intracytoplasmic immunoreactivity in the subependymal layer; and spherical and ring-like intracytoplasmic immunoreactivity in the subependymal layer. Of 13 differentiated ependymomas 11 reflected the immunoreactive patterns of normal ependyma. The anaplastic ependymomas and ependymoblastomas had no immunoreactivity. Our results indicate that EMA has a highly selective distribution in the ependyma, and is a marker for differentiated ependymoma.

Relationship between simian virus 40 large tumor antigen expression and tumor formation in transgenic mice.

A line of transgenic mice containing the simian virus 40 (SV40) large tumor antigen gene under the control of the viral enhancer-promoter expressed this viral protein in the brains of these mice within the first 2 weeks after birth. Multiple foci of anaplastic cells formed in the choroid plexuses of these mice at 36 to 41 days after birth, and normal tissue coexisted with these transformed foci. Immunoperoxidase staining to detect the SV40 T antigen showed tumor-specific expression of nuclear T antigen at late times in tumor development, approximately 90 to 100 days and thereafter. The level of SV40 T antigen, on a per cell basis, appeared to be lower in the great majority of choroid plexus cells at earlier times in tumor development. These results suggest that low levels of tumor antigen (14 to 36 days) are present before detectable pathology (36 to 41 days) and the level of T antigen per cell is higher in rapidly growing late-stage tumors (older than 90 days).

[Light microscopic studies of concanavalin A binding in the choroid plexus epithelium and ependymal cells of the cerebral ventricle and human tumors derived from those tissues]. / Lichtmikroskopische Untersuchungen zur Concanavalin-A-Bindung an den Epithelien der Plexus chorioidei und den Ependymzellen der Hirnventrikel und davon abgeleitete Tumoren des Menschen.

Histochemical investigations of human choroid plexus with lectins revealed strong cytoplasmic binding of concanavalin A to plexus epithelium, whereas ependymal cells were unstained or only weakly reactive. A similar but less clear-cut staining pattern by concanavalin A was observed with plexus papillomas and ependymomas. A clear discrimination however, between the two tumor types using this method is not possible.

Regulation of natural killer cell function by glass-adherent cells in patients with primary intracranial malignancies.

Natural killer (NK) cell function against the NK cell-sensitive myeloid leukemia cell line, K562, was measured in the peripheral blood mononuclear cells (PBMCs) of 17 patients with primary brain tumors (4 diagnosed as having low grade tumors and 13 diagnosed as having high grade (malignant) tumors). The ability of monocytes to control the levels of NK cell function in PBMCs from these patients was assessed in glass-adherent cell depletion studies. Most patient assessments were performed before surgical biopsy and diagnosis; most but not all patients were receiving dexamethasone at the time of immunity assessment. The results demonstrate that patients with primary malignant brain tumors have depressed levels of NK cell function in their PBMCs due to the suppressive actions of glass-adherent monocytes, whereas patients with low grade tumors have normal levels of function shown by this assay.

Screening of human brain tumors for SV40-related T antigen.

A series of 39 human brain tumors has been screened for the presence or absence of SV40-related T antigen by the direct and indirect immunoperoxidase methods. Two tumors of ependymal origin (malignant ependymoma, choroid plexus papilloma) revealed markedly positive nuclear staining for T antigen both in vivo and in vitro. The relationship of these tumors to their experimental counterparts inducible by recent human papovavirus isolates is discussed.