Intracranial Chondrosarcoma: A Case Report and Review of the Literature

OBJECTIVE AND IMPORTANCE: Chondrosarcomas rarely occur intracranially away from the skull base. We present a case of a supratentorial dural-based, low-grade classic chondrosarcoma and emphasize salient imaging findings that assist in its diagnosis. CLINICAL PRESENTATION: A 23-year-old female presented with new onset seizures. Multiple radiographic imaging studies identified a large left frontal mass with radiological findings that suggested the correct diagnosis. INTERVENTION: The patient underwent a left frontal craniotomy and gross total resection of the lesion and associated dura. CONCLUSION: Intracranial chondrosarcomas away from the skull based are extremely rare malignancies. As with other extra-axial masses, they often grow to a relatively large size before generating symptoms. Careful analysis of CT, MR, and angiographic imaging characteristics should suggest the correct diagnosis.     

An Indwelling Brachytherapy Balloon Catheter: Potential Use as an Intracranial Light Applicator for Photodynamic Therapy

An indwelling balloon applicator developed for postoperative intracavity afterloading brachytherapy was evaluated for photodynamic therapy (PDT). Following tumor resection, the applicator is positioned in the center of the resultant cavity and the balloon is inflated with a scattering solution. The liquid-filled balloon stabilizes the resection cavity ensuring a constant and simple geometric shape during treatment. The catheter is brought out through the skin and adjusted so that 1–2 cm projects above the scalp surface.Measurements of light distribution in a phantom model surrounding the balloon catheter, show that it may be used to deliver sufficiently uniform light doses during PDT. The light distribution is uniform to within 5% when the balloon is filled with a scattering medium. Based on simple assumptions, it is shown that the applicator can be used to deliver a sufficient optical dose to brain tissue at a depth of 1 cm in less than 1 h.The applicator has already been used for brachytherapy in 72 patients with few complications. A new treatment protocol employing the combination of both fractionated intracavity brachytherapy and PDT is currently being planned.     

Gastric leiomyosarcoma: clinical and pathological review of fifty patients

Fifty patients with gastric leiomyosarcoma seen at M.D. Anderson Hospital between 1957 and 1978 were reviewed. Symptoms included weakness, gastrointestinal bleeding, and epigastric pain. An upper abdominal mass or tenderness was the most frequent physical finding. Laparotomy was performed in all patients, with gastric resection in 86%, and resection for cure in 68%. Survival after distal subtotal gastric resection (mean: 62 months) was longer than after proximal subtotal (30 months) or wedge resection (46 months). The five-year survival was 19% for all patients, and 32% for those operated on for cure. A favorable prognosis was predicted by mild atypia or few mitoses (grade 1). No differences in survival were demonstrated between patients with tumors of different cell types (epithelioid, spindle, or pleomorphic). No lymph node in any patient contained metastatic tumor, suggesting a lymph node dissection need not be performed. Commonly, tumor spread was to liver or lungs or by direct invasion of contiguous tissue or organ. Radiotherapy and chemotherapy are of limited value in treating unresectable disease. If possible, both the primary lesion and recurrent tumor should be widely resected.     

Kaposi's sarcoma and the acquired immunodeficiency syndrome: treatment with high and low doses of recombinant leukocyte A interferon

The efficacy of recombinant leukocyte A interferon (rIFN-alpha A [Roferon-A, Hoffman-La Roche, Nutley, NJ]) treatment of Kaposi's sarcoma in patients with acquired immunodeficiency syndrome was evaluated in sequential trials using high doses (36 X 10(6) units) and low doses (3 X 10(6) units) of interferon. A major response was seen in 38% of patients treated at the high dose, with a median response duration of 18 months. At the low dose, the major response rate was 3%; dose escalation to 36 X 10(6) units resulted in an additional major response rate of 17% in low-dose nonresponders, with a median response duration of 10 months. Four of 11 patients who achieved a complete response remain free of disease, whereas all partial responders have shown disease progression. Unacceptable toxicity occurred in 27% of patients initially treated at the high dose and only in 10% of those who had progressive dose escalation up to 36 X 10(6) units. Prior opportunistic infections correlated negatively with therapeutic response, whereas large tumor burden and gastrointestinal involvement did not. Responding patients showed a significantly longer survival and a lower incidence of subsequent opportunistic infections than nonresponders. However, from our study we cannot determine whether rIFN-alpha A has an effect on the natural history of Kaposi's sarcoma in patients with the acquired immunodeficiency syndrome.     

颅内上皮样血管内皮瘤一例报告并文献复习

上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)是一种罕见的血管源性肿瘤,其组织学及生物学特性介于血管瘤和血管肉瘤之间,具有潜在复发和转移能力[1]。1982年Weiss等[2]在软组织中发现了这种独特组织类型的肿物,将其命名为EHE。EHE可发生于全身多个部位,最常见于肝、肺和骨,发生于颅内者非常罕见,文献报道其发病率不到颅内肿瘤的0.02%[3-4]。     

Intracranial therapy of glioblastoma with the fusion protein DTIL13 in immunodeficient mice

A fusion protein consisting of human interleukin-13 and the first 389 amino acids of diphtheria toxin was assembled in order to target human glioblastoma cell lines in a murine intracranial model. In vitro studies to determine specificity indicated that the protein called DTIL13 was highly selective for human glioblastoma. In vivo, the maximum tolerated dose of DTIL13 was 1 microg/injection given every other day and repeated for 3 days. Doses that exceeded this amount resulted in weight loss and liver damage as determined by histology and enzyme assay. Experiments in IL-4 receptor knockout mice revealed that liver toxicity was receptor-related. This same dose given to nude mice with established U373 MG brain tumors resulted in significant reductions in tumor volume and significantly prolonged survival (p<0.0001). Magnetic resonance imaging (MRI) proved to be extremely useful in (i) determining the ability of DTIL13 to reduce tumor size and (ii) for studying toxicity since diffusion-weighted and gradient echo-weighted MRI revealed that vascular leak syndrome was not a limiting toxicity at this dose. These results suggest that DTIL13 is as effective in an intracranial rodent model as it was in a flank model in previous studies and that DTIL13 might be an effective treatment for glioblastoma multiforme.     

手术治疗原发性颅内外沟通平滑肌肉瘤#br#并复发1例#br#

目的分析总结原发性颅内外沟通平滑肌肉瘤的临床特点和诊治体会,提高临床医师对该罕见病的认识。方法回顾性分析昆明市第一人民医院神经外科收治的1例原发性颅内外沟通平滑肌肉瘤患者的临床资料及复发后的诊治情况。结果患者于2015年5月第1次入院明确诊断后,首次扩大切除病灶（范围2 cm）,术后2年4个月出现复发;于2017年9月第2次入院再次扩大切除病灶（范围3～4 cm）,至今随访2年8月余,未出现复发,继续随访中。结论外科手术切除是目前原发性颅内外沟通平滑肌肉瘤最主要和最有效的治疗手段。     

Chondromas of the falx cerebri and dural convexity: report of two cases and review of the literature

Summary Intracranial chondromas usually arise from the base of the skull. They rarely originate from the convexity dura and falx. Here we describe two cases of intracranial chondroma located at the convexity dura and falx, discuss the genesis, radiologic, histologic features and review the literature.     

Cytogenetic and histopathologic studies of congenital supratentorial primitive neuroectodermal tumors: A case report

Primitive neuroectodermal tumors (PNET) represent about 25% of primary central nervous system tumors in childhood, but congenital PNETs are rare. Cytogenetic studies and studies on molecular pathology have identified several genetic alterations in medulloblastoma, but molecular investigations on supratentorial PNETs are infrequent. We present a male newborn with a large congenital PNET of the right cerebral hemisphere and the molecular analysis of the tumor. Tumor tissue was investigated by routine histology and immunohistochemistry. Fluorescence in-situ hybridization was carried out on native tumor tissue to investigate deletions on chromosome 17p and to analyze c-Myc or N-Myc amplifications. Histologic examination revealed a primitive neuroectodermal tumor with massive extension covering almost the entire right hemisphere. Genetic analysis of the native tumor tissue of our patient excluded a deletion of chromosome 17p. An amplification of the c-Myc or N-Myc oncogene was absent using fluorescence in-situ hybridization. Despite unremarkable genetic analysis in our case prognosis was poor, suggesting that there are additional, yet unknown constitutional genetic aberrations in the pathogenesis of congenital supratentorial PNET.     

Intracranial aspergillosis producing cavernous sinus syndrome and rupture of internal carotid artery

A case of invasive, intracranial aspergillosis in a non-immunocompromised patient, complicated by rupture of the internal carotid artery due to fungal arteritis, is presented. The computed tomography and magnetic resonance imaging findings are discussed. This is a rare condition with a poor prognosis and requires a high level of suspicion when paranasal sinus disease is detected.     

Radiation therapy for Kaposi's sarcoma associated with acquired immunodeficiency syndrome: Tokyo Metropolitan Komagome Hospital experience

Background. Kaposi's sarcoma is frequently found in association with acquired immunodeficiency syndrome (AIDS). We report on radiotherapy for patients with AIDS-related Kaposi's sarcoma at Tokyo Metropolitan Komagome Hospital. Methods. Between April 1991 and May 1997, radiotherapy was given to 11 lesions in eight men with AIDS-related Kaposi's sarcoma to relieve their symptoms. The lesions involved the head and neck region, the legs, and the gastrointestinal tract. Radiotherapy was carried out with 4-MV photon through parallel opposed fields or high energy electrons. Total doses ranged from 20 to 38 Gy, with a median of 30 Gy, delivered in 2- to 3-Gy fractions. Four patients were given other treatments prior to the radiotherapy. Acute reaction was evaluated according to the modified acute radiation morbidity scoring criteria of the Radiation Therapy Oncology Group (RTOG). Results. Radiotherapy had relieved the symptoms in all patients at completion of this therapy. Lesions that involved the hard palate and vocal cords had completely disappeared. The lesions that received radiotherapy were controlled without symptoms until the patients died. Patients who had the head and neck region treated exhibited severe acute mucosal reaction (at a dose of 30 Gy, there was grade 2 morbidity by modified RTOG criteria, in two patients, and grade 3 in three patients) although the radiation therapy was completed for these patients. Conclusion. Radiotherapy promises a favorable outcome for symptom relief in AIDS-related Kaposi's sarcoma. Received: April 24, 2000 / Accepted: August 18, 2000     

Intracranial therapy of glioblastoma with the fusion protein DTAT in immunodeficient mice

A gene splicing technique was used to create a hybrid fusion protein DTAT encoding the 390 amino acid portion of diphtheria toxin (DT(390)), a linker, and the downstream 135-amino terminal fragment portion of human urokinase plasminogen activator. DTAT was assembled to target human glioblastoma cell lines in a murine intracranial model. Previously published in vitro studies demonstrated that DTAT was highly selective and toxic to human glioblastoma cell lines in a flank tumor model. The purpose of this study was to determine the toxicity, specificity and possible therapeutic efficacy of DTAT in an intracranial model. Convection enhanced delivery of DTAT resulted in about a 16-fold increase in maximum tolerated dose. Intracranial administration of DTAT on an every-other-day basis in nude mice with established U87 MG brain tumors resulted in significant reductions in tumor volume and significantly prolonged survival (p < 0.0001). Magnetic resonance imaging proved to be a powerful tool in mice and rats for demonstrating tumor growth in a xenograft intracranial model, assessing the efficacy of DTAT in tumor volume reduction and detecting DTAT-associated intracranial toxicity and vascular damage. These results suggest that the DTAT recombinant fusion protein is highly effective in an intracranial model and DTAT might be an effective treatment for glioblastoma.     

Intracranial astrocytoma with diffuse bone marrow metastasis: a case report and review of the literature

A 41 year old male presented with headache, lethargy, and ataxia and found to have a left temporal lobe mass and a leukoerythroblastic peripheral blood smear. The latter prompted an iliac crest bone marrow biopsy on which a diagnosis of metastatic glioma was made and verified by immunohistologic characterization. The patient was treated with cranial irradiation and simultaneous systemic BCNU (bis-dichloroethylnitrosurea) with complete response. This case with diffuse bone marrow involvement demonstrates that a glioblastoma is capable of extracranial metastases without previous intervention. From a review of reported cases of gliomas of extraneural metastasis, it is concluded that untreated gliomas are capable of vascular spread although less frequently than previously manipulated tumors.     

Signet cell carcinoma of the stomach in a patient with acquired immunodeficiency syndrome: a case report

A case of signet cell carcinoma associated with AIDS is presented. A 50-year old Japanese man with hemophilia A was suffering from human immunodeficiency virus (HIV) infection, the result of multiple injections of clotting factor concentrates. A diagnosis of signet cell carcinoma of the stomach was reached upon endoscopic and histological examinations. Opportunistic infections of esophageal candidiasis and candida septicemia occurred. The patient died of repeated gastrointestinal bleeding and cachexia. Although there is a possibility of the patient having a coincidential carcinoma along with AIDS, the HIV infection, perhaps, had a role in causing signet cell cercinoma.     

Cumulative incidence of cancer among individuals with acquired immunodeficiency syndrome in the United States

BACKGROUND:

The overall burden of cancer may increase as individuals with acquired immunodeficiency syndrome (AIDS) live longer because of highly active antiretroviral therapy (HAART), which has been widely available since 1996.

METHODS:

A population‐based, record‐linkage study identified cancers in 472,378 individuals with AIDS from 1980 to 2006. By using nonparametric competing‐risk methods, the cumulative incidence of cancer was estimated across 3 calendar periods (AIDS onset in 1980‐1989, 1990‐1995, and 1996‐2006).

RESULTS:

Measured at 5 years after AIDS onset, the cumulative incidence of AIDS‐defining cancer (ADC) declined sharply across the 3 AIDS calendar periods (from 18% in 1980‐1989, to 11% in 1990‐1995, to 4.2% in 1996‐2006 [ie, the HAART era]). The cumulative incidence of Kaposi sarcoma declined from 14.3% during 1980 to 1989, to 6.7% during 1990 to 1995, and to 1.8% during 1996 to 2006. The cumulative incidence of non‐Hodgkin lymphoma (NHL) declined from 3.8% during 1990 through 1995 to 2.2% during 1996 through 2006; during the HAART era, NHL was the most common ADC (53%). The cumulative incidence of non‐AIDS‐defining cancer (NADC) increased from 1.1% to 1.5% with no change thereafter (1%; 1996‐2006), in part because of declines in competing mortality. However, cumulative incidence increased steadily over time for specific NADCs (anal cancer, Hodgkin lymphoma, and liver cancer). The cumulative incidence of lung cancer increased from 0.14% during 1980 to 1989 to 0.32% during 1990 to 1995, and no change was observed thereafter.

CONCLUSIONS:

Dramatically declining cumulative incidence was noted in 2 major ADCs (Kaposi sarcoma and NHL), and increases were observed in some NADCs (specifically, cancers of the anus, liver, and lung and Hodgkin lymphoma). As HIV/AIDS is increasingly managed as a chronic disease, greater attention should be focused on cancer screening and prevention. Cancer 2011. Published 2010 by American Cancer Society.     

Characteristics and survival of patients with non-Hodgkin's lymphoma with and without acquired immunodeficiency syndrome

Our objective was to determine the characteristics and survival of patients with non-Hodgkin's lymphoma (NHL) with and without acquired immunodeficiency syndrome (AIDS). A cancer registry and AIDS registry linkage for San Diego County was performed in October 1998 as part of a national multicentre study. We performed Kaplan-Meier analysis to compare survival in NHL patients with and without AIDS, after matching for age, sex, and race/ethnicity. We performed logistic regression to determine which patient and tumour characteristics were significantly associated with 1-year survival. Of the 4361 cases of NHL, 324 (7%) had AIDS and 4037 (93%) were not known to have AIDS. Patients with AIDS were more likely to have extranodal, high-grade, and disseminated NHL diagnosed by non-histologic means and were less likely to have received chemotherapy. Patients with AIDS and NHL who survived at least 1 year had less advanced disease stage and received chemotherapy. The median survival in patients with AIDS was 4 months (95% confidence interval (CI): 4-5) and 95 months (95% CI: 58-157) in patients without AIDS (P<0.001). Although these patients with AIDS-related NHL were unlikely to survive, the highly active antiretroviral agents currently used may improve outcomes in future patients.     

Radiotherapy for Pediatric Central Nervous System Tumors: A Regional Cancer Centre Experience

INTRODUCTION: The purpose of this review was to analyze outcomes for pediatric patients treated for more common (non-low grade glioma) primary central nervous system (CNS) tumors at a Regional (tertiary) Cancer Center. Comparison to reported results from other regional centres and results from the contemporary literature were made. MATERIAL AND METHODS: The records of pediatric patients treated with radiotherapy at the London Regional Cancer Center (LRCC) for more common (non-low grade glioma) primary CNS tumors between 1980 and 2001 were reviewed. Details regarding tumor presentation, treatment and outcome were analyzed. Results: Eighty-eight patients were eligible for the review. Twenty-nine patients with malignant glioma, 37 patients with medulloblastoma or primitive neuroectodermal tumor (PNET), 15 patients with brainstem glioma, 4 with ependymoma and 3 with germ cell tumors were treated during this time period. Average follow-up for the group was 5 years (range 4 months to 19 years). Five-year overall, progression free and cause specific survival were 45, 42 and 50%, respectively. For patients with malignant glioma median progression free and overall survival was 20 and 29 months. For patients with brainstem glioma median progression free and overall survival was 9 and 13 months. For medulloblastoma, 5-year progression free, and overall survival was 60 and 59%. CONCLUSIONS: Results of this retrospective review of pediatric patients treated at a regional cancer center for primary CNS tumors (other than low grade glioma) were comparable to contemporary results reported by other Canadian centers and North American co-operative group trials.     

High risk of Kaposi's sarcoma and central nervous system lymphoma in the same individuals: A finding related to acquired immunodeficiency syndrome

Kaposi's sarcoma and central nervous system (CNS) lymphoma are the 2 most common malignancies related to HIV infection. To investigate the association between Kaposi's sarcoma and CNS lymphoma, a population-based retrospective cohort study was conducted. Using U.S. Surveillance, Epidemiology and End Results Program data, the gender-specific age- and calendar year-adjusted standardized incidence ratios were calculated for the pre-AIDS (1973–1980) and AIDS (1981–1990) eras, as an estimate of the relative risk (RR) of developing one condition following another. For the AIDS era in men, the RR of CNS lymphoma following Kaposi's sarcoma was 979.7 and that of Kaposi's sarcoma following CNS lymphoma was 231.1. There were no instances of the co-occurrence of these malignancies in women in either era or in men for the pre-AIDS era. The extremely high RRs for the co-occurrence of Kaposi's sarcoma and CNS lymphoma in men during the AIDS era suggests that the association of these malignancies occurs within the same HIV-infected individuals. © 1996 Wiley-Liss, Inc.