Epithelioid glioblastoma presenting as multicentric glioma: A case report and review of the literature

Background: Epithelioid glioblastoma is a rare aggressive variant of glioblastoma multiforme (GBM), which was formally recognized by the World Health Organization classification of the central nervous system in 2016. Clinically, epithelioid GBMs are characterized by aggressive features, such as metastases and cerebrospinal fluid dissemination, and an extremely poor prognosis. A rare case of epithelioid GBM that was discovered as a multicentric glioma with different histopathology is reported. Case Description: A 78-year-old man was admitted to our hospital with mild motor weakness of the right leg. Neuroimaging showed small masses in the left frontal and parietal lobes on magnetic resonance imaging. The abnormal lesion had been increasing rapidly for 3 weeks, and a new lesion appeared in the frontal lobe. 11C-methionine positron emission tomography (PET) showed abnormal uptake corresponding to the lesion. To reach a definitive diagnosis, surgical excision of the right frontal mass lesion was performed. Histological findings showed diffuse astrocytoma. Only radiotherapy was planned, but the left frontal and parietal tumors progressed further within a short period. Therefore, it was thought that these tumors were GBM, and a biopsy of the left parietal tumor was performed. The histological diagnosis was epithelioid GBM. Immunohistochemistry showed that most tumor cells were negatively stained for p53 and isocitrate dehydrogenase 1. BRAF V600E mutations were not identified, but TERT promoter mutations were identified. Immediately after surgery, the patient was given chemotherapy using temozolomide, extended local radiotherapy and then bevacizumab. After 6 months, he showed no signs of recurrence. Conclusion: Epithelioid GBM is one of the rarest morphologic subtypes of GBM and has a strongly infiltrative and aggressive nature. Therefore, careful identification of preoperative imaging studies and detailed evaluation of genetic studies are necessary to select the appropriate treatment for epithelioid GBM.

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Imaging and Histopathologic Nuances of Epithelioid Glioblastoma

Case Reports in Surgery ◽

10.1155/2018/1285729 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Brian H. Le ◽

Richard A. Close

Keyword(s):

Mass Effect ◽

Clonic Seizure ◽

Cystic Mass ◽

Braf V600e ◽

World Health ◽

Significant Past Medical History ◽

Who Grade ◽

Epithelioid Glioblastoma ◽

Health Organization ◽

The Right

A 27-year-old male without significant past medical history presented following collapse resulting from a syncopal episode at work. There was an episode of vomiting, and one of tonic-clonic seizure activity, which was spontaneously resolved after approximately one minute. His neurologic exam was nonfocal, with full strength in the bilateral upper and lower extremities, and no sensory deficits were elicited. MRI studies demonstrated a 4.7 cm rim-enhancing cystic mass in the right temporal-parietal region, with resultant mass effect and edema. At surgery, intraoperative pathologic consultation favoured a primary glial neoplasm. Subsequent complete histologic examination on permanent sections confirmed the presence of glioblastoma, with a morphologic pattern and immunohistochemical profile most consistent with epithelioid glioblastoma (WHO grade IV). Epithelioid glioblastoma is a rare, especially aggressive variant of IDH-wildtype glioblastoma, recognized in the 2016 World Health Organization classification. Approximately 50% of such tumors harbour the BRAF V600E mutation, which has also been observed in some melanomas where selective inhibitors have demonstrated a therapeutic role. The especially aggressive behaviour and poor clinical outcome typically observed for this variant of glioblastoma demonstrate the importance of emerging areas relevant to neurooncology, specifically those of proteomic characterization and therapeutic nanomedicine.

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A Simple Panel of IDH1 and P53 in Differential Diagnosis Between Low-Grade Astrocytoma and Reactive Gliosis

Clinical Pathology ◽

10.1177/2632010x20986168 ◽

2021 ◽

Vol 14 ◽

pp. 2632010X2098616

Author(s):

Bita Geramizadeh ◽

Mahsa Kohandel-Shirazi ◽

Ahmad Soltani

Keyword(s):

Differential Diagnosis ◽

Isocitrate Dehydrogenase ◽

P53 Mutation ◽

Reactive Gliosis ◽

World Health ◽

Low Grade ◽

Diffuse Astrocytoma ◽

Glial Tumor ◽

Isocitrate Dehydrogenase 1 ◽

Who Grade

Background: Reactive gliosis is a response of glial tissue to different types of injury such as brain abscess, trauma, hemorrhage, or even neoplastic process. In some circumstances, especially when the tissue biopsy is small, there may be difficulty to discriminate this reactive condition with low-grade diffuse astrocytoma (World Health Organization [WHO] grade II) by conventional hematoxylin and eosin (H&E) slides, so some immunohistochemical and molecular markers have been introduced for this differential diagnosis. One of the important aspects of updated WHO classification in 2016 has been dividing some of the glial tumor according to IDH1 (isocitrate dehydrogenase 1) mutation. Objectives: In this study, we tried to evaluate IDH1 and P53 mutation by immunohistochemistry as a simple and highly specific and sensitive method to differentiate low-grade astrocytoma and reactive gliosis. Material and methods: For 5 years (2013-2018), 50 cases of clinically documented reactive gliosis and 50 cases of low-grade astrocytoma were evaluated for the presence or absence of IDH1 and P53 mutation by immunohistochemistry. Results: Isocitrate dehydrogenase 1 was positive in 92% and 4% of the astrocytoma and reactive gliosis cases and P53 was positive in 90% and 4% of the cases with the final diagnosis of astrocytoma and reactive gliosis, respectively. Discussion and conclusion: Combination of P53 and IDH1 as an immunohistochemical panel showed specificity of 96% and sensitivity of 91% for differential diagnosis of reactive gliosis and low-grade astrocytoma. These 2 markers can be extremely helpful for this differential diagnosis.

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A Lip Lump: An Unexpected Histological Diagnosis of a Lip Schwannoma

Case Reports in Otolaryngology ◽

10.1155/2017/3107362 ◽

2017 ◽

Vol 2017 ◽

pp. 1-2 ◽

Cited By ~ 2

Author(s):

Thomas Haigh ◽

John Raad Glore ◽

David Gouldesbrough ◽

Winson Wong

Keyword(s):

Surgical Excision ◽

World Health Organisation ◽

World Health ◽

Clinical Impression ◽

Upper Lip ◽

Benign Schwannoma ◽

History Of ◽

Benign Tumours ◽

The Right

Schwannomas are benign nerve sheath tumours arising from Schwann cells. They comprise 1% of all benign tumours. In the 2016 World Health Organisation Classification of Central Nervous System, they are classified as a tumour of the cranial and paraspinal nerves, Schwannoma 9560/0. A 23-year-old Caucasian lady presented with a seven-month history of a painless right upper lip lump. Examination revealed a small cystic 0.5 cm diameter lesion within the right upper lip. The clinical impression was that of a mucocele. Excision of the lip lesion was performed under local anaesthetic. Histological examination of the excised lesion demonstrated a circumscribed nodule consisting of spindle cells mixed with vascular spaces containing red blood cells and fibrin. Immunohistochemistry for S100 was strongly positive. The findings were consistent with that of a small benign schwannoma. The current consensus is for surgical excision of a conservative nature with no need for margins. If recurrence does occur one needs to consider whether complete enucleation was achieved or whether malignant transformation has occurred.

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Astrocytic tumours: diffuse astrocytoma, anaplastic astrocytoma, glioblastoma, and gliomatosis cerebri

10.1093/med/9780199651870.003.0003 ◽

2017 ◽

Author(s):

Michael Weller ◽

Michael Brada ◽

Tai-Tong Wong ◽

Michael A. Vogelbaum

Keyword(s):

Dna Methyltransferase ◽

Clinical Decision Making ◽

Methylation Status ◽

Progression Free Survival ◽

Clinical Decision ◽

World Health ◽

Diffuse Astrocytoma ◽

Isocitrate Dehydrogenase 1 ◽

Curative Radiotherapy ◽

Mgmt Promoter

Astrocytic gliomas are primary brain tumours thought to originate from neural stem or progenitor cells. They are assigned grades II, III, or IV by the World Health Organization according to degree of malignancy as defined by histology. The following molecular markers are increasingly used for diagnostic subclassification or clinical decision-making: 1p/19q co-deletion status, O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation status, and isocitrate dehydrogenase 1 and 2 mutation status. Extent of resection is a favourable prognostic factor, but surgery is never curative. Radiotherapy prolongs progression-free survival across all astrocytic glioma entities. Alkylating agent chemotherapy is an active treatment in particular for patients with MGMT promoter-methylated tumours. Anti-angiogenic therapies have failed to improve survival, and the current focus of major clinical trials is on novel targeted agents or on immunotherapy.

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Obstacles to Claiming Permanence and Injury-Relatedness for “Posttraumatic” Headache

Guides Newsletter ◽

10.1001/amaguidesnewsletters.2009.mayjun02 ◽

2009 ◽

Vol 14 (3) ◽

pp. 3-6

Author(s):

Robert J. Barth

Keyword(s):

Complete Recovery ◽

World Health ◽

Related Factors ◽

Posttraumatic Headache ◽

Sixth Edition ◽

Causative Relationship ◽

Short Period ◽

The One ◽

Health Organization ◽

Subjective Reports

Abstract “Posttraumatic” headaches claims are controversial because they are subjective reports often provided in the complex of litigation, and the underlying pathogenesis is not defined. This article reviews principles and scientific considerations in the AMAGuides to the Evaluation of Permanent Impairment (AMA Guides) that should be noted by evaluators who examine such cases. Some examples in the AMA Guides, Sixth Edition, may seem to imply that mild head trauma can cause permanent impairment due to headache. The author examines scientific findings that present obstacles to claiming that concussion or mild traumatic brain injury is a cause of permanent headache. The World Health Organization, for example, found a favorable prognosis for posttraumatic headache, and complete recovery over a short period of time was the norm. Other studies have highlighted the lack of a dose-response correlation between trauma and prolonged headache complaints, both in terms of the frequency and the severity of trauma. On the one hand, scientific studies have failed to support the hypothesis of a causative relationship between trauma and permanent or prolonged headaches; on the other hand, non–trauma-related factors are strongly associated with complaints of prolonged headache.

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Multidisciplinary Approach of a Very Rare Infected Verrucous Carcinoma of the Scrotum

Revista de Chimie ◽

10.37358/rc.19.4.7153 ◽

2019 ◽

Vol 70 (4) ◽

pp. 1476-1478

Author(s):

Laura Raducu ◽

Adelaida Avino ◽

Cristina-Nicoleta Cozma ◽

Sorin Nedelea ◽

Andra-Elena Balcangiu-Stroescu ◽

...

Keyword(s):

Adjuvant Therapy ◽

Rare Disease ◽

Chronic Inflammation ◽

Male Patient ◽

Multidisciplinary Approach ◽

Verrucous Carcinoma ◽

Surgical Excision ◽

Diagnosis And Treatment ◽

Wide Surgical Excision ◽

The Right

Verrucous carcinoma of the scrotum is an extremely rare disease and most cases are thought to result from poor hygiene and chronic inflammation. Currently, it has not been well characterized, the etiology, diagnosis and treatment remaining poorly understood. We present the case of a 50-year-old male patient diagnosed with verrucous carcinoma of the right hemiscrotum. Wide surgical excision was performed. Favorable outcomes can be achieved by surgery, even without any adjuvant therapy, but patients should be carefully followed up.

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Hypertriglyceridemia: An Infrequent, Difficult-to-predict, Severe Metabolic and Vascular Problem Associated with Estrogen Administration

Current Vascular Pharmacology ◽

10.2174/1570161117666190306102322 ◽

2020 ◽

Vol 18 (3) ◽

pp. 254-261

Author(s):

Thomas F. Whayne

Keyword(s):

Acute Pancreatitis ◽

Severe Side Effect ◽

Recurrent Acute Pancreatitis ◽

Postmenopausal Symptoms ◽

Severe Hypertriglyceridemia ◽

Short Period ◽

Cv Disease ◽

The Right ◽

And Control ◽

Transdermal Route

Supplementary estrogen plays important roles for female patients as convenient birth control, relief of postmenopausal symptoms, and in the management of other selected problems. However, as is the case for essentially all medications, there are side effects. Short of a major pulmonary embolus, the most severe side effect of estrogen would appear to be sporadic, rare, and severe hypertriglyceridemia associated with acute pancreatitis. The occurrence of this fortunately rare problem usually happens in the presence of some preexisting and usually mild increase in triglycerides (TG). A case of chronic and severe recurrent acute pancreatitis is described in the introduction and the management was complete estrogen avoidance. Started close to menopause and continued for a relatively short period, estrogens may have some cardiovascular (CV) benefit but the general recommendation is not to prescribe them for CV disease prevention. Estrogens may contribute to decreased diabetes mellitus (DM) risk and control. Administration of estrogens by the transdermal route may decrease some problems such as venous thromboembolism (VTE) and elevation of TG. Administration of estrogen in the right situation brings significant benefit to the female patient but skillful, careful, and knowledgeable use is essential.

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Hepatic brucelloma: a rare complication of a common zoonotic disease

BMJ Case Reports ◽

10.1136/bcr-2020-237076 ◽

2020 ◽

Vol 13 (12) ◽

pp. e237076

Author(s):

George Vatidis ◽

Eirini I Rigopoulou ◽

Konstantinos Tepetes ◽

George N Dalekos

Keyword(s):

Rare Complication ◽

Surgical Excision ◽

Coombs Test ◽

Inflammation Markers ◽

Laboratory Findings ◽

Ct Guided ◽

Rare Manifestation ◽

Bone Marrow Cultures ◽

History Of ◽

The Right

Hepatic brucelloma (HB), a rare manifestation of brucellosis, refers to liver involvement in the form of abscess. A 35-year-old woman stockbreeder was admitted due to 1-month history of evening fever, sweating and weight loss, while she was on 3-week course of rifampicin/doxycycline for suspected brucellosis. On admission, she had hepatosplenomegaly and a systolic murmur, while cholestasis, increased inflammation markers and a strong-positive Wright-Coombs test were the main laboratory findings. As blood and bone marrow cultures were unrevealing, further investigation with CT imaging showed a central liver calcification surrounded by heterogeneous hypodense area being compatible with HB. Material from CT-guided drainage tested negative for Brucella spp. After failure to improve on a 10-week triple regiment, surgical excision was decided and Brucella spp were identified by PCR. Our case highlights challenges in establishing HB diagnosis, which should be considered on the right epidemiological context and when serological and radiological evidence favour its diagnosis.

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Intrascrotal lipoblastoma in adulthood

BMJ Case Reports ◽

10.1136/bcr-2019-231320 ◽

2019 ◽

Vol 12 (12) ◽

pp. e231320

Author(s):

Mário José Pereira-Lourenço ◽

Duarte Vieira-Brito ◽

João Pedro Peralta ◽

Noémia Castelo-Branco

Keyword(s):

Case Report ◽

Magnetic Resonance ◽

S100 Protein ◽

Surgical Excision ◽

Large Mass ◽

Histological Diagnosis ◽

Physical Exam ◽

Nodular Lesion ◽

Inferior Portion ◽

The Right

This case report describes the case of a 37-year-old man that noticed an intrascrotal right mass with 1 month of evolution. During physical exam presented with a large mass at the inferior portion of the right testicle, clearly separated from the testicle, with a tender consistency and mobile. An ultrasound was performed that showed a solid and subcutaneous nodular lesion, extra testicular, heterogeneous, measuring 7.2 cm. Pelvic magnetic resonance imageMRI showed a lesion compatible with a lipoma. The patient was subjected to surgical excision of the lesion by scrotal access, having histology revealed a lipoblastoma (LB) of the scrotum. Histological diagnosis was obtained by microscopic characteristics (well-circumscribed fatty neoplasm) and immunohistochemistry (stains for CD34, S100 protein and PLAG1 were positive; stains for MDM2 and CDK4 were negative). LB is extremely rare after adolescence in any location, being this first described case of intrascrotal LB described in adulthood.

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Oncocytic Lipoadenoma: Report of 3 Rare Cases Involving the Parotid Gland, Including a Synchronous Presentation With Paraganglioma of the Right Carotid Bifurcation and Literature Review

International Journal of Surgical Pathology ◽

10.1177/10668969211021966 ◽

2021 ◽

pp. 106689692110219

Author(s):

John L.S. Cunha ◽

Marco A. Peñalonzo ◽

Ciro D. Soares ◽

Bruno A.B. de Andrade ◽

Mário J. Romañach ◽

...

Keyword(s):

English Language ◽

Smooth Muscle Actin ◽

Salivary Gland Tumor ◽

Surgical Excision ◽

Carotid Bifurcation ◽

Alpha Smooth Muscle Actin ◽

S 100 ◽

The Right ◽

Adequate Management ◽

Adipose Cells

Oncocytic lipoadenoma (OL) is a rare salivary gland tumor characterized by the presence of oncocytic cells and mature adipose tissue. To date, only 30 cases of OL have been reported in the English-language literature. We present 3 additional OL cases involving the parotid, including a synchronous presentation with paraganglioma of the right carotid bifurcation. Microscopically, both the OLs were composed of a mixed population of oncocytes and adipocytes in varying proportions surrounded by a thin, connective tissue fibrous capsule. Oncocytes were positive for pan-cytokeratins (CKs) AE1/AE3, epithelial membrane antigen, CK5, CK7, CK14, CK18, and CK19. Calponin, p63, alpha-smooth muscle actin, and carcinoembryonic antigen were negative. Vimentin and S-100 protein were positive only in adipose cells. Despite distinctive morphologic features, OL is often misdiagnosed, given its rarity. We hope to contribute to surgeons’ and pathologists’ awareness and knowledge regarding the existence of this tumor and provide adequate management through conservative surgical excision.

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