scholarly journals Primary Gliosarcoma of the Cerebellum in a Young Pregnant Woman: Management Challenges and Immunohistochemical Features

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Marco Meloni ◽  
Salvatore Serra ◽  
Giulia Bellisano ◽  
Nikolaos Syrmos ◽  
Sanjeeva Jeyaretna ◽  
...  

Background. Gliosarcoma (GS) represents a rare, high-grade (WHO Grade IV), central nervous system neoplasm, characterized by a very poor prognosis. Similar to other high-grade gliomas, GS affects mainly adults in the 5th-7th decade of life and presents a higher incidence in males. The most reported locations of GS are the temporal lobe and the frontal lobe, while only eight cases of GS originating from the posterior cranial fossa are reported in the literature. Case Description. We report the first case occurring during pregnancy in a 33-year-old patient. Diagnosis was obtained on the 15th week of gestation when patient presented with signs and symptoms of life-threatening raised intracranial pressure. Surgical excision was followed by early recurrence and eventually disease progression because the patient refused adjuvant treatment to save her fetus. Conclusions. GS should be considered in the differential diagnosis of posterior cranial fossa tumors with radiological features of meningioma or glioblastoma, even in young patients. To this regard, sarcomas, solitary fibrous tumors, and even metastases should be considered, especially in light of the tendency of GS to give rise to extracranial localizations. Whenever an aggressive management with radical excision and adjuvant treatment is not safely achievable, disease progression is likely to be unavoidable.

2012 ◽  
Vol 03 (01) ◽  
pp. 60-64 ◽  
Author(s):  
Srikant Balasubramaniam ◽  
Devendra K Tyagi ◽  
Hemant V Sawant ◽  
Sridhar Epari

ABSTRACTGliosarcoma (GSM) is a WHO grade 4 tumor and a variant of glioblastoma multiforme with predilection for the temporal lobe. We record, perhaps the first case in literature, of a temporal lobe GSM with recurrence involving the posterior fossa. A 50-year-old man presented to us with headache, vomiting, and lethargy of relatively recent onset. Magnetic resonance imaging revealed a well-circumscribed lesion in the left temporal lobe for which left temporal craniotomy with radical excision of the tumor was performed. Histopathology was suggestive of GSM. He presented to us within a month of the first surgery with a large recurrence involving the temporal lobe. He underwent a second surgery with radical excision of the tumor. Histopathology was confirmatory of GSM. He was administered concomitant chemotherapy and radiotherapy. Within a fortnight of starting adjuvant therapy, the bone flap started bulging and a repeat computed tomography scan revealed a large recurrence extending into the posterior fossa. The patient′s relatives refused consent for third surgery and he finally succumbed on postoperative day 21. GSMs are aggressive tumors that have a temporal lobe predilection, but they may present anywhere in the brain. Detailed studies on larger cohort of cases are needed to understand the true nature of these biphasic tumors.


2020 ◽  
Vol 11 ◽  
pp. 367
Author(s):  
Sarah Bin Abdulqader ◽  
Nasser Almujaiwel ◽  
Wafa Alshakweer ◽  
Gmaan Alzhrani

Background: Spheno-orbital meningiomas (SOMs) are often benign. The association of meningioma and systemic lupus erythematosus (SLE) is rarely discussed in the literature. Here, we report two patients with high-grade, SOMs with a prolonged history of SLE and review the literature. Case Description: The first case is a 52-year-old female patient with a 15-year history of SLE diagnosis who was referred to our center with a 1-year history of proptosis and excessive tearing of the left eye. This patient was operated for the left SOM with histopathological diagnosis of the World Health Organization (WHO) Grade III rhabdoid meningioma. The second case is a 36-year-old female patient with a 12-year history of SLE diagnosis who presented to our clinic with a 5-year-history of progressive right eye proptosis and occasional headaches. She was operated for the right SOM with histopathological diagnosis of the WHO Grade II chordoid meningioma. Conclusion: Rhabdoid and chordoid SOMs are uncommon and no previous report discussed their occurrence in patients with SLE. The association of high-grade meningiomas and SLE deserves further exploration.


2015 ◽  
Vol 38 (1) ◽  
pp. E6 ◽  
Author(s):  
Elizabeth B. Claus ◽  
Kyle M. Walsh ◽  
John K. Wiencke ◽  
Annette M. Molinaro ◽  
Joseph L. Wiemels ◽  
...  

Significant gaps exist in our understanding of the causes and clinical management of glioma. One of the biggest gaps is how best to manage low-grade (World Health Organization [WHO] Grade II) glioma. Low-grade glioma (LGG) is a uniformly fatal disease of young adults (mean age 41 years), with survival averaging approximately 7 years. Although LGG patients have better survival than patients with high-grade (WHO Grade III or IV) glioma, all LGGs eventually progress to high-grade glioma and death. Data from the Surveillance, Epidemiology and End Results (SEER) program of the National Cancer Institute suggest that for the majority of LGG patients, overall survival has not significantly improved over the past 3 decades, highlighting the need for intensified study of this tumor. Recently published research suggests that historically used clinical variables are not sufficient (and are likely inferior) prognostic and predictive indicators relative to information provided by recently discovered tumor markers (e.g., 1p/19q deletion and IDH1 or IDH2 mutation status), tumor expression profiles (e.g., the proneural profile) and/or constitutive genotype (e.g., rs55705857 on 8q24.21). Discovery of such tumor and constitutive variation may identify variables needed to improve randomization in clinical trials as well as identify patients more sensitive to current treatments and targets for improved treatment in the future. This article reports on survival trends for patients diagnosed with LGG within the United States from 1973 through 2011 and reviews the emerging role of tumor and constitutive genetics in refining risk stratification, defining targeted therapy, and improving survival for this group of relatively young patients.


2011 ◽  
Vol 15 (5) ◽  
pp. 502-506 ◽  
Author(s):  
Damien Petit ◽  
Philippe Menei ◽  
Henri-Dominique Fournier

The authors describe the first case of spindle cell lipoma of the posterior neck invading the upper cervical spinal canal and the posterior cranial fossa. Spindle cell lipoma is an extremely rare variant of benign lipoma. It usually occurs as a solitary subcutaneous well-circumscribed lesion in the posterior neck or shoulders of adult men. Local aggressiveness is unusual. This 61-year-old man presented with an increased left cerebellar syndrome and headaches. He also had a posterior neck tumefaction, which had been known about for a long time. Computed tomography and MR imaging studies revealed a voluminous mass extending to the upper cervical canal and posterior cranial fossa and eroding the neighboring bones. The lesion was well delimited, and contrast enhancement was intense and heterogeneous. The tumor, which had initially developed under the muscles of the posterior neck, was totally resected. Histological assessment revealed numerous fat cells with spindle cells secreting collagen. The large size of the tumor and the submuscular location, bone erosion, and compression of the CNS were unusual in this rare subtype of benign adipose tumor. Its presentation could simulate a sarcoma.


Skull Base ◽  
2009 ◽  
Vol 19 (03) ◽  
Author(s):  
Shinya Ishimura ◽  
Takayuki Ohira ◽  
Masahito Kobayashi ◽  
Tadashige Kano ◽  
Maaya Orii ◽  
...  

Skull Base ◽  
2009 ◽  
Vol 19 (S 02) ◽  
Author(s):  
N. Syrmos ◽  
Ch. Iliadis ◽  
J. Marakomichelakis ◽  
G. Gavridakis ◽  
V. Valadakis ◽  
...  

2021 ◽  
pp. 1-7
Author(s):  
Mohammad Al-Wiswasy ◽  
Mahmoud Al-Balas ◽  
Raith Al-Saffar ◽  
Hamzeh Al-Balas

INTRODUCTION: Primary breast sarcoma (PBS) was first described in 1887 as a rare heterogeneous neoplasm arising from the mesenchymal tissue of the mammary gland accounting for less than 5% of all body soft-tissue sarcomas and less than 0.1% of all malignant tumors of the breast. CASE PRESENTATION: A 31-year old lady presented with left breast mass which she felt four years before during which the mass increased in size from 1 to 6 cm in the largest diameter, diagnosed clinically as a benign fibroadenoma without any further cytological or histopathological confirmation. Histopathological examination of the excised breast mass reveals undifferentiated, primary stromal sarcoma of the breast (PSSB), which was followed by mastectomy three weeks later with reconstructive breast surgery with a total duration of follow-up of 3 years thereafter. This is the first case of PSSB reported in Jordan. DISCUSSION/CONCLUSION: PSSB is the generic term given to malignant breast tumors thought to arise from the specialized mesenchymal stroma of the breast but lacking an epithelial component with a phylloides pattern. PSSB is difficult to diagnose preoperatively due to its rarity and inadequate imaging methods to establish an exact diagnosis. The histology of the patient mass may be the leading factor for the management of these tumors. Even in very young patients, a progressively growing breast mass should alert the clinician to investigate for malignancy and verify the results by biopsy. Surgery with adequate resection margins represents the only potentially curative modality with prognostic significance. Adjuvant chemotherapy and radiotherapy are not very beneficial. The prognosis is dismal for patients with lymph node involvement and the size of the tumor has a lesser bearing on the outcome.


2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii33-ii34
Author(s):  
Macarena De La Fuente ◽  
Tulay Koru-Sengul ◽  
Deborah Heros ◽  
Feng Miao ◽  
Alain Fernandez Marrero ◽  
...  

Abstract BACKGROUND Glioblastoma is the most common primary malignant brain tumor. Despite multimodality treatment approach, median progression-free survival (PFS) is only 8 months, median overall-survival (OS) 14 months and 5-year survival rate of under 10%. Dendritic cells (DCs) are the professional antigen presenting cells of the immune system. The rationale for sensitizing dendritic cells to a pool of non-selected tumor antigens is based on the marked heterogeneity present within glioblastoma tumor cells. METHODS Phase 1/feasibility study of DC vaccine for recurrent high-grade glioma was conducted. Pooled, non-selected tumor antigens collected via tumor cell lysate were used for DC sensitization. RNA sequencing analysis was performed on all tumor samples. Cytokine levels in serum were detected using a Luminex cytokine panel. RESULTS A total of 20 patients were enrolled onto this study (median age 58yrs, range: 39–74, 65% male). Pathology showed WHO grade IV glioblastoma in 14 (70%) and grade III anaplastic astrocytoma in 6 (30%) patients. IDH wild type in 19 (95%) patients. Treatment emergent adverse events (all grades, regardless of attribution) occurred in more than 15% of the patients (20% fatigue, 15% dizziness, 15% headache, none leading to treatment discontinuation). There were five grade 3–4 and none grade 5 events. One grade 4 event (seizure) probable related to investigational treatment leading to treatment discontinuation. Four grade 3 events (dysphasia, possible related; intracranial hemorrhage unrelated; muscle weakness, unlikely related and hematoma, unrelated). Median PFS was 3.8 months. Median OS was 11 months. RNA sequencing in tumor samples and correlation with cytokine levels in serum is currently been analyzed. CONCLUSION Tumor lysate pulsed DC vaccination demonstrates acceptable safety and tolerability in high-grade glioma patients. Evaluations of integrating molecular profiling RNA sequencing information and cytokine levels to identify potential subset of patients with significant clinical benefit will be provided.


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