Chemotherapy for malignant gliomas of the brain: a review of ten-years experience

Summary In recent years, there has been a great improvement in the knowledge of the biological aspects of malignant gliomas of the brain. Conversely, there has been an increase of interest in the multimodal treatment of these tumours.In this review, we have analyzed the results of the several reports which have appeared in the literature that deal with the chemotherapeutic treatment of malignant gliomas. Furthermore, some areas of biological investigation that could have an impact on pharmacological therapy are discussed.Abbreviations AA anaplastic astrocytoma - ACNU (l-4-amino-2methil-5pyrimidinyl)-methyl-3-(2-chloroethyl)-3-nitrosourea - AraC cytosine arabinoside - AZQ aziridinylbenzoquinone - BCNU 1,3-bis(2-chloroetyl)-1-nitrosourea - BTSG Brain Tumor Study Group (USA) - BTCG Brain Tumor Cooperative Group (USA) - BUdR 5-bromodeoxyuridine - CCNU 1-(2-chloroethyl)-3cyclohexyl-1 -nitrosourea - CDDP cisplatin - DAG dianhydrogalacticol - DBD dibromodulcitol - DTIC imidazolcarboxamide - EORTC European Organization for Research on Treatment of Cancer - 5-FU fluorouracil - GBM glioblastoma - HU hydroxyurea - MeCCNU methyl-CCNU - Miso misonidazole - MP 6-mercaptopurine - MST median survival time - MTTP median time to tumor progression - PCNU 1-(2-chloroethyl)-3-(2,6-dioxo-3-piperidyl)-1-nitrosourea - PCZ procarbazine - RT radiotherapy - VCR vincristine - VM26 teniposide - VP16 etoposide     

Malignant hemangiopericytoma of the liver: Report of a case

A 30 year old female was admitted with right upper abdominal pain and fever. Ultrasonography and computed tomography revealed a large cystic mass in the right lobe of the liver, and aspiration bacteriology was negative. A right hepatic lobectomy was performed for a suspected cystadenocarcinoma, however, the tumor was histologically diagnosed as a hemangiopericytoma with prominent venous invasion. The patient died within a short time of multiple pulmonary metastases. Primary hepatic hemangiopericytoma is extremely rare, and according to our research, this is only the 4th case reported in the literature.     

Primary malignant melanoma: a rare cause of mediastinal mass

A case of primary malignant melanoma in the mediastinum presenting as recurrent laryngeal nerve palsy is reported. Tissue biopsy at mediastinotomy yielded a diagnosis of malignant melanoma. The mass was fixed to the left aspect of the trachea and to the upper border of the left main bronchus and could not be removed surgically. Further extensive clinical and radiological investigations revealed no evidence of tumor elsewhere in the body.     

Chordoid glioma: report of two rare examples with unusual features

Summary Background. Chordoid glioma, a rare tumour of the third ventricle, represents a distinct clinico-pathologic entity. Thirty nine examples have been described in the literature, mostly in females and in the third ventricle. The clinical presentation is variable but they tend to occur mostly in adults. There is only one report of a chordoid glioma in a 12 year old child. Finding. This paper describes two examples of chordoid glioma in a seven year old female child and a 70 year old male respectively. Radiologically, the paediatric chordoid glioma was located in the juxtaventricular region in the occipital horn of the lateral ventricle and was of mixed density whereas the adult patient had a typical third ventricle location with homogenous contrast enhancement. Gross total surgical removal was achieved in both but the adult patient died post-operatively due to intra ventricular bleeding and bacterial meningitis. The younger patient is doing well at the last follow up two years post-operatively. Microscopically, both showed the classic morphology of chordoid glioma. Ultrastructural examination was suggestive of ependymal differentiation. Conclusion. The younger age and unusual location are some of the rare features which need documentation and have not been described earlier. We propose that chordoid glioma is a variant of an ependymoma (WHO grade II) which arises from tanycytes and should be included in the WHO classification of brain tumors. Correspondence: Dr. Mehar Chand Sharma, M.D., Associate Professor, Department of Pathology, AIIMS, New Delhi 110029, India.     

Surgical treatment of meningiomas of the orbit and optic canal: A retrospective study with particular attention to the visual outcome

Summary A series of 57 patients affected either by optic nerve sheath meningiomas (15 patients) or by secondary meningiomas extending into the orbital apex or optic canal (42 patients) has been reviewed. Proptosis (80%), progressive visual loss (76%), and visual fields defects (63%) were the most common pre-operative complaints. All patients underwent transcranial surgery. Radical tumour resection was possible in 9 out of 15 primary and 22 out of 32 secondary tumours. Overall, 27% of the patients obtained improved postoperative visual acuity, 62% remained unchanged, and 11% were worse. The postoperative visual outcome was related to the degree of pre-operative visual impairment. In a period between 38 and 68 months, 5 meningiomas recurred. The time to progression of partially resected lesions ranged between 9 and 68 months.Dedicated to Prof. Dr. H.-D. Herrmann on the occasion of his 60th birthday.     

Safety of Gliadel Implant for Malignant Glioma: Report of Postmarketing Surveillance in Japan

Clinical trial data of Carmustine implant (Gliadel Wafer) in Japanese patients with malignant glioma are limited; thus, we conducted a postmarketing surveillance study to evaluate the safety of Gliadel in real-world clinical practice in Japan. In this postmarketing surveillance study, all patients who received Gliadel placement for malignant glioma surgeries from its market launch (January 9, 2013) to July 10, 2013 were enrolled from 229 institutions using a central registration system. Up to eight wafers of Gliadel (containing 61.6 mg of carmustine) were used to cover the site of brain tumor resection intraoperatively according to the size and shape of the tumor resection cavity. The observation period lasted 3 months after Gliadel placement. Patients were followed up for 1 year postoperatively. Safety was assessed by the incidence of adverse events (AEs) and adverse drug reactions (ADRs). In total, 558 patients were included. Most patients (66.7%) received eight Gliadel wafers. The percentage of patients with ADRs was 35.7% (365 ADR episodes in 199 patients). Of the AEs of special interest, the most common were cerebral edema (22.2%, 124/558 patients), convulsion (9.9%, 55/558 patients), impaired healing (4.8%, 27/558 patients), and infection (3.4%, 19/558 patients). This first all-case postmarketing surveillance report of the safety of Gliadel in real-world clinical practice in Japan suggests that the risk of toxicity with Gliadel placement is relatively tolerable. The survival benefits of Gliadel placement should be evaluated and considered carefully by the clinician taking into account possible toxicities.     

Renovascular hypertension: a rare cause of neonatal salt loss

We report a case of severe hypertension in the newborn period due to obstruction of the right renal artery. The baby presented with polyuria leading to dehydration and was found to have hyponatraemia and severe renal salt loss. When sudden malignant hypertension is induced in experimental conditions, a high pressure diuresis and increased angiotensin II production are found. These findings could explain the renal salt loss, notwithstanding the effects of secondary hyperaldosteronism and hyper-reninaemia.     

Malignant fibrous histiocytoma originating in the mediastinum: Report of a case

Malignant fibrous histiocytoma (MFH) is a tumor which most often develops in the soft tissues of the extremities and retroperitoneum, but very rarely originates in the mediastinum. We report herein the case of a 63-year-old man who underwent surgical resection of a rapidly growing tumor in the right thoracic cavity which was diagnosed as an MFH of mediastinal origin on the basis of histological findings, the definitive diagnosis ultimately being made by specific immunostaining.     

Malignant schwannoma of the intrathoracic vagus nerve: Report of a case

We report herein the case of a 48-year-old man with malignant schwannoma of the intrathoracic vagus nerve associated with von Recklinghausen's disease. Malignant intrathoracic vagal tumors are extremely rare and to our knowledge, only four other cases have been documented in the literature, none of which were associated with von Recklinghausen's disease.     

Malignant fibrous histiocytoma of the stomach: Report of two cases

(Received for publication on July 8, 1996; accepted on May 12, 1997)     

Cerebro-vasculopathy and malignancy: catastrophic complications of radiotherapy for optic nerve glioma in a von Recklinghausen neurofibromatosis patient

The authors report a unique case of a patient with intraorbital optic nerve glioma and von Recklinghausen neurofibromatosis who developed cerebro-vasculopathy and malignant transformation in the orbit 18 months after radiotherapy treatment. The case is an important reminder of the possibly increased susceptibility of von Recklinghausen neurofibromatosis patients to the complications of radiotherapy.     

Current Status of Palliative and Terminal Care for Patients with Primary Malignant Brain Tumors in Japan

Palliative care and advance care planning (ACP) from the first diagnosis of glioblastoma are important. This questionnaire survey was conducted to understand the current status of palliative care for brain tumors in Japan. Representative characteristics of Japan in comparison with Western countries (P <0.01) are described below: (1) Gender ratio of male in physicians who treat brain tumors in Europe and the United States/Canada are about 70%, but 94% in Japan. (2) The specialty is predominantly neurosurgeon (93%) in Japan. The ratio of neurologists is predominantly 40% in Europe. In the United States/Canada, neurologist (27%) and neurosurgeon (29%) are main parts. (3) Years of medical experience over 15 in physicians is 73% in Japan. Proportions of those with over 15 years are 45% in Europe and 30% in the United States/Canada. (4) In practicing setting, the rate of academic medical centers is about 80% in Europe and the United States/Canada, and ~60% in Japan. Representative differences compared with past domestic data (2007) (P <0.01): (1) In glioblastoma, the rate of explaining about median survival time increases from 39% (2007) to 80% (2018). Explanation about medical conditions to the patient himself with his family increases from 20% (2007) to 39% (2018). (2) Place of death: The rate at hospital is decreasing from 96% (2007) to 79% (2018) and at home is increasing from 3% (2007) to 10% (2018) (3) The rate of ventilator in adult has decreased from 74% (2007) to 54% (2018), but nasal tube feeding has remained unchanged from 62% (2007) to 60% (2018). These results will be shared with physicians to make better care systems for patients with brain tumors.     

A rare lesion of the central nervous system: inflammatory pseudotumor

Inflammatory pseudotumor is an uncommon tumor, initially described in the lung, but which can involve various organs. It is a controversial entity. We report the case of a 19-year-old-man with an inflammatory pseudotumor localized in the central nervous system, revealed by epilepsy. Characteristically, the inflammatory pseudotumor is an inflammatory mass leading to manifestations related to its localization. Relatively ubiquitous, this tumor is seldom described in the central nervous system. This uncommon lesion is part of a heterogeneous group of entities which are difficult to diagnose for both surgical pathologists and clinicians.     

Malignant Progression of Diffuse Low-grade Gliomas: A Systematic Review and Meta-analysis on Incidence and Related Factors

Malignant progression of diffuse low-grade glioma (LGG) is a critical event affecting patient survival; however, the incidence and related factors have been inconsistent in literature. According to the PRISMA guidelines, we systematically reviewed articles from 2009, meta-analyzed the incidence of malignant progression, and clarified factors related to the transformation. Forty-one articles were included in this study (n = 7,122; n, number of patients). We identified two definitions of malignant progression: histologically proven (Htrans) and clinically defined (Ctrans). The malignant progression rate curves of Htrans and Ctrans were almost in parallel when constructed from the results of meta-regression by the mean follow-up time. The true transformation rate was supposed to lie between the two curves, approximately 40% at the 10-year mean follow-up. Risk of malignant progression was evaluated using hazard ratio (HR). Pooled HRs were significantly higher in tumors with a larger pre- and postoperative tumor volume, lower degree of resection, and notable preoperative contrast enhancement on magnetic resonance imaging than in others. Oligodendroglial histology and IDH mutation (IDHm) with 1p/19q codeletion (Codel) also significantly reduced the HRs. Using Kaplan-Meier curves from eight studies with molecular data, we extracted data and calculated the 10-year malignant progression-free survival (10yMPFS). The 10yMPFS in patients with IDHm without Codel was 30.4% (95% confidence interval [95% CI]: 22.2-39.0) in Htrans and 38.3% (95% CI: 32.3-44.3) in Ctrans, and that with IDHm with Codel was 71.7% (95% CI: 61.7-79.5) in Htrans and 62.5% (95% CI: 55.9-68.5) in Ctrans. The effect of adjuvant radiotherapy or chemotherapy could not be determined.     

Radionecrosis of malignant glioma and cerebral metastasis: A diagnostic challenge in MRI

Detecting a new area of contrast-enhancement at MRI after irradiation of malignant brain tumor arises the problem of differential diagnosis between tumor recurrence and radiation necrosis induced by the treatment. The challenge for imaging is to distinguish the two diagnoses given: the prognostic and therapeutic issues. Various criteria have been proposed in the literature based on morphological, functional or metabolic MRI. The purpose of this study was to perform an analysis of these tools to identify MRI best criteria to differentiate radiation necrosis lesions from malignant gliomas and brain metastases recurrence. For gliomas, the morphology of the contrast-enhancement cannot guide the diagnosis and the use of perfusion techniques and spectroscopy (multivoxels if possible) are necessary. In the follow-up of metastasis, a transient increase and moderate lesion volume is possible with a good prognosis. Morphological characteristics (volume ratio T2/T1Gd) and perfusion analysis provide valuable tools for approaching the diagnosis of radionecrosis.     

前列腺恶性纤维组织细胞瘤1例报告

目的 :探讨前列腺恶性纤维组织细胞瘤 (MFH)的诊断和治疗。　方法 :诊治 1例前列腺MFH病人。行前列腺肿瘤根治术 +直肠切除术 +乙状结肠造瘘术。并结合文献进行诊治讨论。　结果 :术后 3周复发 ,遂行放疗 ,2个月后因肿瘤转移而死亡。　结论 :前列腺MFH预后差 ,早期原发病灶切除并术后辅助治疗是关键     

A rare case of diminished visual acuity: neurohypophyseal granular cell tumor

A neurohypophyseal granular cell tumor was found in a 60-year-old man who presented diminished visual acuity. Neuronavigation resection via a fronto-pteronial approach removed split up material. The diagnosis was established by histology and immunochemical studies. Granulous cell tumors, which are common, are rarely located in the neurohypophysis. Their origin remains a subject of debate, but unlike the other localizations, these tumors in the sellar and/or supra-sellar area are always considered benign. The clinical course depends on the quality of the surgical resection; there is no consensus on appropriate management.     

Calvarium mass as the first presentation of glioblastoma multiforme: A very rare manifestation of high-grade glioma

Glioblastoma multiforme (GBM) is a high grade glial tumor, primarily located in cerebral hemispheres. The most common clinical presentations are slowly progressive neurological deficit such as motor weakness, seizure, and headaches that last less than three months. Calvarium and extra-axial invasion are very rare and generally occur after a brain biopsy or surgery, or secondary to radiotherapy of primary intra-axial glial tumors. We report a case of GBM with calvarium involvement in a 60-year-old man who presented with a frontal bump and left-sided clumsiness. Imaging studies revealed a tumoral lesion that destroyed the frontal bone with white matter involvement of the frontal lobe and extension into the corpus callosum. Histopathological examination of intra-axial and extra-axial lesions revealed pleomorphic high-grade tumor with large areas of necrosis and hemorrhage. Immunohistochemical (IHC) studies confirmed GBM that spread directly into the dura, galea, and calvarium (positive reaction for GFAP, S-100, CD68, OLIG2, and p53). The patient was treated with radiotherapy (60 Gy/30 fractions) and concomitant temozolomide. Unfortunately, the patient died seven months after the initial diagnosis.     

A rare cause of vaginal bleeding in a 7-month-old female infant

Ovarian sex cord stromal tumor (OSCST), sclerosing type, is an extremely rare ovarian tumor. Sex hormone production by OSCST can result in isosexual or heterosexual precocious puberty in younger patients. We present a case of a 7-month-old female infant found to have a sclerosing-type OSCST that presented with vaginal bleeding and very prominent vellus hair at the mons pubis. This represents the youngest patient reported in the literature with this subset of OSCST.