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1

Anaplastic oligodendroglioma with ganglioglioma-like maturation.  

UK PubMed Central (United Kingdom)

Neuronal differentiation of oligodendroglioma has been demonstrated by immunohistochemical and ultrastructural examinations in recent studies. However, oligodendrogliomas displaying a complete neurocytic morphology or even gangliocytic differentiation are rare. We describe a case of anaplastic oligodendroglioma that was characterized by the presence of ganglion cells in a 40-year-old-male. Histologically, the tumor was mainly composed of classical oligodendroglioma cells. The most exceptional finding of this tumor was the presence of ganglion cells and intermediate-sized ganglioid cells. Immunohistochemical analysis revealed that these cells were positive for Olig2 and negative for glial fibrillary acid protein (GFAP). Synaptophysin and microtubule-associated protein 2 (MAP2) were mainly detected in the ganglion cells. Fluorescence in situ hybridization analysis (FISH) revealed the deletion of the 1p and 19q chromosome arms in both the oligodendroglioma cells and ganglion cells. The R132H mutated isocitrate dehydrogenase 1 (IDH1) protein was detected by immunohistochemistry and direct DNA sequencing. The morphological, immunohistochemical, and genetic features of the tumor suggested a diagnosis of anaplastic oligodendroglioma, and this tumor was considered to be a rare form of oligodendroglioma displaying ganglioglioma-like maturation. FISH and mutant IDH1 examinations are useful diagnostic tools for the differential diagnosis of this tumor, i.e., ganglioglioma with anaplastic oligodendroglial features.

Tanaka Y; Nobusawa S; Yagi S; Ikota H; Yokoo H; Nakazato Y

2012-10-01

2

Anaplastic oligodendroglioma with ganglioglioma-like maturation.  

Science.gov (United States)

Neuronal differentiation of oligodendroglioma has been demonstrated by immunohistochemical and ultrastructural examinations in recent studies. However, oligodendrogliomas displaying a complete neurocytic morphology or even gangliocytic differentiation are rare. We describe a case of anaplastic oligodendroglioma that was characterized by the presence of ganglion cells in a 40-year-old-male. Histologically, the tumor was mainly composed of classical oligodendroglioma cells. The most exceptional finding of this tumor was the presence of ganglion cells and intermediate-sized ganglioid cells. Immunohistochemical analysis revealed that these cells were positive for Olig2 and negative for glial fibrillary acid protein (GFAP). Synaptophysin and microtubule-associated protein 2 (MAP2) were mainly detected in the ganglion cells. Fluorescence in situ hybridization analysis (FISH) revealed the deletion of the 1p and 19q chromosome arms in both the oligodendroglioma cells and ganglion cells. The R132H mutated isocitrate dehydrogenase 1 (IDH1) protein was detected by immunohistochemistry and direct DNA sequencing. The morphological, immunohistochemical, and genetic features of the tumor suggested a diagnosis of anaplastic oligodendroglioma, and this tumor was considered to be a rare form of oligodendroglioma displaying ganglioglioma-like maturation. FISH and mutant IDH1 examinations are useful diagnostic tools for the differential diagnosis of this tumor, i.e., ganglioglioma with anaplastic oligodendroglial features. PMID:22231405

Tanaka, Yuko; Nobusawa, Sumihito; Yagi, Shinichi; Ikota, Hayato; Yokoo, Hideaki; Nakazato, Yoichi

2012-01-11

3

Anaplastic oligodendroglioma: advances and treatment options.  

UK PubMed Central (United Kingdom)

OPINION STATEMENT: The optimal treatment strategy for anaplastic oligodendroglial (AO) tumors is evolving. Molecular profiling of oligodendrogliomas have shown distinctive genetic patterns characterized by combined deletions of chromosome arms 1p and 19q, O(6)-methylguanine methyltransferase (MGMT) methylation, and isocitrate dehydrogenase 1 (IDH1) mutations; they are all prognostic factors for patients with AO. In addition, a strong association has also been found between the CpG island hypermethylation phenotype (CIMP) status and MGMT promoter methylation. Long term follow up data of the Radiation Therapy Oncology Group (RTOG) 9402 and the European Organisation for Research and Treatment of Cancer (EORTC) 26951 studies demonstrate clear evidence that for patients with codeleted 1p19q AO, early chemotherapy with radiation offers a significant improvement in overall survival compared with early radiation, even with salvage chemotherapy at tumor relapse, and thus establishes the 1p19q allelic loss as a predictive marker distinct from tumors without the chromosome change. Radiotherapy alone is no longer considered an adequate treatment for this patient population. In cases with no 1p19q deletion, most neuro-oncologists recommend incorporating radiotherapy into the upfront treatment strategy. However, there are still unanswered questions regarding whether upfront chemotherapy, omitting/deferring radiotherapy, in the desire to avoid late neurocognitive toxicity of radiotherapy should be the initial therapy for AO tumors with codeleted 1p19q, or whether temozolomide, an oral agent with a better toxicity profile, can be substituted for procarbazine, lomustine, and vincristine (PCV). Further studies are warranted and the increasing understanding of molecular pathways involved may lead to more selective therapeutic targets in the future.

McNamara MG; Sahebjam S; Mason WP

2013-06-01

4

Paraplegia due to drop metastases from anaplastic oligodendroglioma.  

DEFF Research Database (Denmark)

In this article, we report on a rare case of spinal seeding from a cerebral anaplastic oligodendroglioma presenting with signs of medullar compression. We discuss the prevalence, mechanisms and imaging findings of spinal seeding in various gliomas. A suitable clinical treatment and follow up for these patients is suggested.

Carlsen, Jakob; Tietze, Anna

2012-01-01

5

Anaplastic oligodendroglioma in an adolescent with Lynch syndrome.  

UK PubMed Central (United Kingdom)

Lynch syndrome (hereditary non-polyposis colorectal cancer; HNPCC) is an autosomal dominant cancer predisposition syndrome with high penetrance. It is caused by heterozygous germline mutations in one of the DNA mismatch repair (MMR) genes MLH1, MSH2, MSH6, and PMS2. Carriers are at high-risk for developing colorectal carcinomas, as well as various extracolonic malignancies. This case report describes a 15 year-old male with a confirmed germline mutation of MSH2 and early onset anaplastic oligodendroglioma. The patient's tumor showed loss of expression of MSH2 and MSH6 proteins with normal microsatellite stability. The immunohistochemical staining pattern provided strong evidence to support the inclusion of anaplastic oligodendroglioma as part of the spectrum of tumors found in Lynch syndrome.

Heath JA; Ng J; Beshay V; Coleman L; Lo P; Amor DJ

2013-06-01

6

Synchronous anaplastic oligodendroglioma and carcinoma tongue: A rare association  

Directory of Open Access Journals (Sweden)

Full Text Available We present the case of a 45-year-old female patient who harbored two synchronous primary malignant neoplasms-an anaplastic oligodendroglioma of the right frontal lobe and a squamous cell carcinoma of the tongue. Both neoplasms were in advanced stage and carried a dismal prognosis. To the best of our knowledge, this is the first documentation in the english literature of such a presentation. The purpose of this article is to alert clinicians to this possibility and to outline the management approach in a different manner in patients presenting with multiple primary neoplasms.

Kumar Vikash; Singh K; Tatke Medha; Rathi A; Shekhar Shashank; Bahadur A

2010-01-01

7

Anaplastic Oligodendroglioma: A New Treatment Paradigm and Current Controversies.  

UK PubMed Central (United Kingdom)

OPINION STATEMENT: Anaplastic oligodendroglial tumors have gained increasing interest with the emerging role of molecular markers and systemic chemotherapy during the past years. The long-term results of two landmark trials, RTOG 9402 and EORTC 26961, have resulted in a reconsideration of the appropriate therapeutic approaches for patients with these tumors. Both trials indicate that patients whose tumors harbor a 1p/19q co-deletion benefit particularly from the addition of procarbazine/lomustine (CCNU)/vincristine (PCV) chemotherapy to radiation therapy (RT). The median survival of patients with co-deleted tumors treated within the RTOG trial with PCV before irradiation was 14.7 years compared with 7.3 years of patients who received RT alone. Median overall survival has not been reached in the RT plus PCV arm of the EORTC trial, but a similar difference can be anticipated after a follow-up of more than 12 years. In contrast, no such benefit was observed for patients with tumors lacking 1p/19q co-deletion. Outside clinical trials, patients with anaplastic oligodendroglial tumors, and 1p/19q co-deletion therefore should be offered a combined treatment modality regimen, including radio- and chemotherapy. PCV, however, is associated with significant hematological toxicity and also nonhematological side effects, which probably translate into reduced quality of life for long-term survivors. Therefore, it might be warranted to replace PCV by temozolomide, which displays a more favorable side effect profile. Data from the NOA-04 study suggest that PCV and temozolomide have similar effects. However, long-term data on the benefit from temozolomide are lacking, making a definite answer on the equivalence of temozolomide and PCV in anaplastic oligodendroglioma (AO) impossible. The current evidence precludes RT alone for AO patients. Neither the RTOG nor the EORTC trial defined the role of chemotherapy alone. A comparison of combined modality treatment with chemotherapy alone followed by RT at progression is pending. Long-term follow-up of NOA-04 patients and results from future trials may help to clarify these questions. With more and more AO patients living 10 years or more, particular attention must be paid to late side effects, such as neurotoxicity, and careful monitoring is required for all treated patients.

Roth P; Wick W; Weller M

2013-08-01

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Anaplastic oligodendroglioma responding favorably to intranasal delivery of perillyl alcohol: a case report and literature review.  

UK PubMed Central (United Kingdom)

BACKGROUND: In recent years, molecular genetics and biology are exerting significant influence on the practice of neuro-oncology, with oligodendrogliomas being the most prominent example. To explore therapeutic strategies and evaluate the clinical results, we report a case of a patient with anaplastic oligodendroglioma managed with intranasal delivery of POH. CASE DESCRIPTION: A 62 year-old white woman presented with complaints of seizures and frontal headache in June 1999. Nervous system examination was normal. Her Karnofsky performance score was 90. A contrast-enhanced MRI scan of the brain revealed a regular space-occupying lesion in the right frontal lobe that enhanced with gadolinium. A radical surgical excision of the tumor was carried out, and the histopathological diagnosis was an anaplastic oligodendroglioma. Subsequently, there were 2 recurrent/progressive lesions, in July 2002 and October 2004, despite combination treatment using surgery, radiotherapy, and chemotherapy. Intranasal delivery of 0.3% concentration of POH 4 times daily was performed. A follow-up MRI scan after 5 months of treatment revealed reduction in size of the enhancing lesion. CONCLUSION: Whereas surgery continues to be the primary treatment for oligodendroglioma, the scheme for postoperative therapy has shifted primarily because of the lesion's relative chemosensitivity. This article evaluates the effects of intranasal delivery of POH in a case of regression of anaplastic oligodendroglioma.

Da Fonseca CO; Masini M; Futuro D; Caetano R; Gattass CR; Quirico-Santos T

2006-12-01

9

Anaplastic oligodendroglioma arising from the brain stem and featuring 1p/19q co-deletion.  

UK PubMed Central (United Kingdom)

With respect to localization, oligodendrogliomas are characterized by a marked preponderance of the cerebral hemispheres. Outside these typical sites, any tumor histopathologically reminiscent of oligodendroglioma a priori is likely to represent one of its morphological mimics, including clear cell ependymoma, neurocytoma, pilocytic astrocytoma or glioneuronal tumors. This is particularly relevant as several of the latter are in principle curable by surgery. Among extrahemispherical sites, bona fide oligodendroglioma - as characterized by loss of heterozygosity (LOH) of chromosome arms 1p and 19q - so far has not been documented to occur in the brain stem. Here, we report the case of a 55-year-old female patient with an anaplastic oligodendroglioma (WHO grade III) of the brain stem and cerebellum diagnosed by stereotactic biopsy and featuring combined LOH of 1p and 19q. A morphological peculiarity was a population of interspersed tumor giant cells, a phenomenon that has been referred to as polymorphous oligodendroglioma. Our findings confirm the notion that - although very infrequently - true oligodendrogliomas do occur in the infratentorial compartment.

Hewer E; Beck J; Vassella E; Vajtai I

2013-05-01

10

Differential diagnosis of small cell glioblastoma and anaplastic oligodendroglioma: a case report of an elderly man.  

UK PubMed Central (United Kingdom)

Small cell glioblastoma is a histological subtype of glioblastoma with characteristic features of highly proliferative, monotonous small glial cells with high nuclear cytoplasm ratio. Morphologically, malignant lymphoma or small cell metastatic carcinoma should be carefully discriminated. Some cases are difficult to differentiate from anaplastic oligodendroglioma. In this report, we present a case of small cell glioblastoma of an elderly man. The lack of IDH1/2 mutation was confirmed by immunohistochemistry and direct sequencing. Fluorescence in situ hybridization revealed the lower rates of chromosome 1p and 19q deletion. Microsatellite analysis disclosed partial 10q alteration near the PTEN locus. Not only morphological and immunohistochemical examinations, but also cytogenetical investigations for IDH1/2 mutation, 1p/19q loss, and PTEN alteration, are strongly supportive methods for the differential diagnosis of small cell glioblastoma and anaplastic oligodendroglioma.

Takahashi K; Tsuda M; Kanno H; Murata J; Mahabir R; Ishida Y; Kimura T; Tanino M; Nishihara H; Nagashima K; Tanaka S

2013-08-01

11

Bone marrow metastases from anaplastic oligodendroglioma presenting with pancytopenia and hypogammaglobulinemia: a case report.  

UK PubMed Central (United Kingdom)

We report the case of a 40-year-old man whose bone marrow metastases occurred 57 months after the initial diagnosis and 9 months after completing radiotherapy for an anaplastic oligodendroglioma. Four months before the demonstration of visceral metastases was obtained by bone marrow biopsy, the patient developed diffuse bone pain, pancytopenia, hypercalcemia, and panhypogammaglobulinemia. These abnormalities and other clinical signs of extracranial dissemination of the primary brain tumor were initially unrecognized until the patient was admitted with the suspicion of a nonsecretory multiple myeloma. We also briefly review the factors predisposing these tumors to spread outside the CNS, albeit rarely, and discuss the clinical implications of a misdiagnosis of extracranial invasion by anaplastic oligodendroglioma, whose chemosensitivity has been definitively demonstrated.

Cordiano V; Miserocchi F; Storti M

2011-11-01

12

Oligodendroglioma  

Science.gov (United States)

... Neuronal-Glial Tumors Oligoastrocytoma Oligodendroglioma Pineal Region Pituitary PNET Schwannoma Risk Factors Brain Tumor Facts Webinars Anytime ... Neuronal-Glial Tumors Oligoastrocytoma Oligodendroglioma Pineal Region Pituitary PNET Schwannoma Risk Factors Brain Tumor Facts Webinars Anytime ...

13

Low-grade and anaplastic oligodendrogliomas: differences in tumour microvascular permeability evaluated with dynamic contrast-enhanced magnetic resonance imaging.  

Science.gov (United States)

This study was designed to quantitatively assess the microvascular permeability of oligodendroglioma using the volume transfer constant (K(trans)) and the volume of the extravascular extracellular space per unit volume of tissue (V(e)) with dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). We aimed to evaluate the effectiveness of K(trans) and V(e) in distinguishing between low-grade and anaplastic oligodendroglioma. The maximal values of K(trans) and V(e) for 65 patients with oligodendroglioma (27 grade II, 38 grade III) were obtained. Differences in K(trans) and V(e) between the two groups were analysed using the Mann-Whitney rank-sum test. Receiver operating characteristic (ROC) curve analyses were performed to determine the cut-off values for the K(trans) and Ve that could differentiate between low-grade and anaplastic oligodendrogliomas. Values for K(trans) and Ve in low-grade oligodendrogliomas were significantly lower than those in anaplastic oligodendrogliomas (p DCE-MRI can distinguish the differences in microvascular permeability between low-grade and anaplastic oligodendrogliomas. PMID:23673143

Jia, Zhongzheng; Geng, Daoying; Liu, Ying; Chen, Xingrong; Zhang, Jun

2013-05-11

14

Increased 99mTc TRODAT-1 Uptake in Anaplastic Oligodendroglioma.  

UK PubMed Central (United Kingdom)

Tc TRODAT-1, a selective dopamine transporter SPECT imaging agent, has demonstrated its efficacy in identifying patients with Parkinson disease. Primary or metastatic brain neoplasm uptake of TRODAT-1 is rarely reported in literatures. A 51-year-old female patient underwent TRODAT-1 study for bradykinesia and altered cognitive function; the images showed abnormal extrastriatal uptake in the right frontal lobe subsequent to operation, and pathological examination confirmed anaplastic oligodendroglioma. Care should be taken in interpreting TRODAT-1 image; any focus on abnormal accumulation of radiotracer should not be overlooked because it can be brain neoplasm as demonstrated in this case.

Chen YR; Hsieh TC; Yen KY; Kao CH

2013-04-01

15

Long-term survival in a dog with anaplastic oligodendroglioma treated with radiation therapy and CCNU.  

UK PubMed Central (United Kingdom)

A 9 year-old, neutered, male French Bulldog showing cluster seizures was diagnosed with a glioma in the right piriform cortex by MRI. Hypofractionated radiation therapy (RT) was performed using a linear accelerator. Although the lesion had involuted significantly at 2 months after RT, recurrence was observed at 4 months after RT. Chemotherapy was started using CCNU (60 mg/m(2) every 6-9 weeks) and was continued for one year. Follow-up MRI revealed involution of the lesion and the intervals of CCNU were increased to every 9-14 weeks. Two years after the first presentation, the dog suffered status epilepticus, followed by deficits of left sided postural reaction with cognitive dysfunction. The dog died on day 910, and histopathological diagnosis confirmed anaplastic oligodendroglioma.

Hasegawa D; Uchida K; Kuwabara T; Mizoguchi S; Yayoshi N; Fujita M

2012-11-01

16

Long-term survival in a dog with anaplastic oligodendroglioma treated with radiation therapy and CCNU.  

Science.gov (United States)

A 9 year-old, neutered, male French Bulldog showing cluster seizures was diagnosed with a glioma in the right piriform cortex by MRI. Hypofractionated radiation therapy (RT) was performed using a linear accelerator. Although the lesion had involuted significantly at 2 months after RT, recurrence was observed at 4 months after RT. Chemotherapy was started using CCNU (60 mg/m(2) every 6-9 weeks) and was continued for one year. Follow-up MRI revealed involution of the lesion and the intervals of CCNU were increased to every 9-14 weeks. Two years after the first presentation, the dog suffered status epilepticus, followed by deficits of left sided postural reaction with cognitive dysfunction. The dog died on day 910, and histopathological diagnosis confirmed anaplastic oligodendroglioma. PMID:22785244

Hasegawa, Daisuke; Uchida, Kazuyuki; Kuwabara, Takayuki; Mizoguchi, Shunta; Yayoshi, Naoko; Fujita, Michio

2012-06-20

17

Feline anaplastic oligodendroglioma: long-term remission through radiation therapy and chemotherapy.  

UK PubMed Central (United Kingdom)

A 10-year-old spayed female Abyssinian cat was presented with cluster limbic focal seizures with secondarily generalisation. From magnetic resonance imaging (MRI) findings, the cat was diagnosed clinically as having a glioma in the left piriform lobe, and hypofractionated radiation therapy (RT) was performed using a linear accelerator. Although the tumour size had reduced significantly at 4 months after RT, recurrence was observed at 11 months after RT. Additional RT was performed and was effective; however, recurrence was observed at 11 months after the additional RT. Chemotherapy was started using nimustine (ACNU; 30 mg/m(2), every 6 weeks). Tumour regression was confirmed by follow-up MRIs from 2 to 5 months after starting chemotherapy. Four years and 2 months after the first presentation the cat died as a result of tumour lysis syndrome following treatment of a high-grade lymphoma. Histopathological diagnosis of the brain tumour confirmed anaplastic oligodendroglioma.

Tamura M; Hasegawa D; Uchida K; Kuwabara T; Mizoguchi S; Ochi N; Fujita M

2013-05-01

18

Feline anaplastic oligodendroglioma: long-term remission through radiation therapy and chemotherapy.  

Science.gov (United States)

A 10-year-old spayed female Abyssinian cat was presented with cluster limbic focal seizures with secondarily generalisation. From magnetic resonance imaging (MRI) findings, the cat was diagnosed clinically as having a glioma in the left piriform lobe, and hypofractionated radiation therapy (RT) was performed using a linear accelerator. Although the tumour size had reduced significantly at 4 months after RT, recurrence was observed at 11 months after RT. Additional RT was performed and was effective; however, recurrence was observed at 11 months after the additional RT. Chemotherapy was started using nimustine (ACNU; 30 mg/m(2), every 6 weeks). Tumour regression was confirmed by follow-up MRIs from 2 to 5 months after starting chemotherapy. Four years and 2 months after the first presentation the cat died as a result of tumour lysis syndrome following treatment of a high-grade lymphoma. Histopathological diagnosis of the brain tumour confirmed anaplastic oligodendroglioma. PMID:23651604

Tamura, Masahiro; Hasegawa, Daisuke; Uchida, Kazuyuki; Kuwabara, Takayuki; Mizoguchi, Shunta; Ochi, Naoko; Fujita, Michio

2013-05-01

19

Pleomorphic xanthoastrocytoma and oligodendroglioma: collision of 2 morphologically and genetically distinct anaplastic components.  

Science.gov (United States)

With the exception of oligoastrocytoma, mixed gliomas are rarely encountered, and the astrocytic component of mixed oligoastrocytoma is almost always fibrillary and diffusely infiltrative. Pleomorphic xanthoastrocytoma (PXA) has occasionally been described in conjunction with ganglioglioma, as well as in 1 case of oligodendroglioma. In this latter case, described by Perry et al., 1p/19q codeletions were not detected. The authors report on a 25-year-old woman with a combined PXA/oligodendroglioma in which concurrent 1p/19q codeletions were detected in the oligodendroglial component only. The patient presented with a 1-month history of headaches. Neuroimaging revealed a heterogeneous left temporal mass with focal enhancement, cystic changes, hemorrhage, and left-to-right midline shift. The patient underwent a craniotomy and gross-total resection. Pathological examination revealed a glial tumor composed of 2 apparently distinct components. The largest component exhibited a prominent fascicular, reticulin-rich, spindle cell arrangement admixed with areas of highly pleomorphic cells, with bizarre cytological features reminiscent of PXA. A smaller component was composed of cellular sheets and lobules of oligodendroglial cells. Both components were characterized by anaplastic features. Dual-color fluorescence in situ hybridization for 1p/19q codeletions was performed. Only the oligodendroglial component showed the combined 1p/19q deletions. This case represents the first instance in which PXA has been reported in conjunction with an oligodendroglioma exhibiting the "molecular signature" characteristic of oligodendroglial neoplasms. The different genetic alterations seen in the 2 components of this neoplasm argue in favor of a "collision tumor" rather than a mixed glioma of the same genotype. PMID:21214337

Hattab, Eyas M; Martin, Sarah E; Shapiro, Scott A; Cheng, Liang

2011-01-07

20

Pleomorphic xanthoastrocytoma and oligodendroglioma: collision of 2 morphologically and genetically distinct anaplastic components.  

UK PubMed Central (United Kingdom)

With the exception of oligoastrocytoma, mixed gliomas are rarely encountered, and the astrocytic component of mixed oligoastrocytoma is almost always fibrillary and diffusely infiltrative. Pleomorphic xanthoastrocytoma (PXA) has occasionally been described in conjunction with ganglioglioma, as well as in 1 case of oligodendroglioma. In this latter case, described by Perry et al., 1p/19q codeletions were not detected. The authors report on a 25-year-old woman with a combined PXA/oligodendroglioma in which concurrent 1p/19q codeletions were detected in the oligodendroglial component only. The patient presented with a 1-month history of headaches. Neuroimaging revealed a heterogeneous left temporal mass with focal enhancement, cystic changes, hemorrhage, and left-to-right midline shift. The patient underwent a craniotomy and gross-total resection. Pathological examination revealed a glial tumor composed of 2 apparently distinct components. The largest component exhibited a prominent fascicular, reticulin-rich, spindle cell arrangement admixed with areas of highly pleomorphic cells, with bizarre cytological features reminiscent of PXA. A smaller component was composed of cellular sheets and lobules of oligodendroglial cells. Both components were characterized by anaplastic features. Dual-color fluorescence in situ hybridization for 1p/19q codeletions was performed. Only the oligodendroglial component showed the combined 1p/19q deletions. This case represents the first instance in which PXA has been reported in conjunction with an oligodendroglioma exhibiting the "molecular signature" characteristic of oligodendroglial neoplasms. The different genetic alterations seen in the 2 components of this neoplasm argue in favor of a "collision tumor" rather than a mixed glioma of the same genotype.

Hattab EM; Martin SE; Shapiro SA; Cheng L

2011-06-01

 
 
 
 
21

Leukemia-like onset of bone marrow metastasis from anaplastic oligodendroglioma after 17 years of dormancy: an autopsy case report.  

UK PubMed Central (United Kingdom)

Extraneural metastases from primary brain tumors are extremely rare. We present an autopsy case that displayed a very late and unique pattern of metastasis from an anaplastic oligodendroglioma. The patient was a 74-year-old woman who was disease free for 17 years after resection of the primary oligodendroglioma. She was subsequently admitted to a hospital for heart failure where her bone marrow was found to be completely infiltrated with tumor cells, eventually resulting in disseminated intravascular coagulation. The onset was like leukemia, but the "blast-like" cells were different from leukemic cells, and the diagnosis was difficult until autopsy. After her death, a review of her past medical history and comprehensive analysis of her primary brain tumor and aspiration biopsy/autopsy bone marrow samples with glial immunohistochemical markers, fluorescence in situ hybridization examination, and immunohistochemical/sequencing analyses of mutant IDH1 revealed the accurate diagnosis. The metastatic tumor in her bone marrow was finally diagnosed as bone metastasis from the primary anaplastic oligodendroglioma. Although metastatic oligodendroglioma is very rare, it should be noted that this condition displays a propensity for bone and bone marrow and can present with features similar to those of leukemia after a long latency period.

Tanaka Y; Nobusawa S; Ikota H; Yokoo H; Hirato J; Ito H; Saito T; Ogura H; Nakazato Y

2013-07-01

22

MicroRNA expression profiling in recurrent anaplastic oligodendroglioma treated with postoperative radiotherapy  

Directory of Open Access Journals (Sweden)

Full Text Available In anaplastic oligodendroglioma (AO), genetic alternation was associated with clinical outcome. We explored radiation-associated up or downregulation of microRNAs (miRNAs) in AO by comparing miRNA expression profiles in newly-diagnosed AO to recurrent AO treated with postoperative radiotherapy. We identified that 23 miRNAs were upregulated and 42 miRNAs were downregulated in recurrent AO compared with newly-diagnosed AO. Especially, the expression of MiR-124, miR-128, miR-139-5p, miR-153, miR-210, miR-582-5p, and miR-96 were highly increased in the patient with recurrent AO. In contrast, MiR-1, miR-1180, miR-133b, miR-135b, miR-1539, miR-193a-5p, miR-196a, miR-196b, miR-200b, miR-21*, miR-221*, miR-224, miR-24-1*, miR-31, miR-32*, miR-34a*, miR-34c-5p, miR-455-5p, miR-503, and miR-631 showed extremely decreased expression in the patient with recurrent AO. Our results provide meaningful data for miRNAs as molecular diagnosis and novel therapeutic targets in recurrent AO treated with radiotherapy.

Giwon Kim; Edmond Changkyun Park; Chung Heon Ryu; Sin-Soo Jeon; Seung Il Kim; Hong-Seok Jang; Gun-Hwa Kim; Byung-Ock Choi

2011-01-01

23

Anaplastic oligodendrogliomas with 1p19q codeletion have a proneural gene expression profile  

Directory of Open Access Journals (Sweden)

Full Text Available Abstract Background In high grade gliomas, 1p19q codeletion and EGFR amplification are mutually exclusive and predictive of dramatically different outcomes. We performed a microarray gene expression study of four high grade gliomas with 1p19q codeletion and nine with EGFR amplification, identified by CGH-array. Results The two groups of gliomas exhibited very different gene expression profiles and were consistently distinguished by unsupervised clustering analysis. One of the most striking differences was the expression of normal brain genes by oligodendrogliomas with 1p19q codeletion. These gliomas harbored a gene expression profile that partially resembled the gene expression of normal brain samples, whereas gliomas with EGFR amplification expressed many genes in common with glioblastoma cancer stem cells. The differences between the two types of gliomas and the expression of neuronal genes in gliomas with 1p19q codeletion were both validated in an independent series of 16 gliomas using real-time RT-PCR with a set of 22 genes differentiating the two groups of gliomas (AKR1C3, ATOH8, BMP2, C20orf42, CCNB1, CDK2, CHI3L1, CTTNBP2, DCX, EGFR, GALNT13, GBP1, IGFBP2, IQGAP1, L1CAM, NCAM1, NOG, OLIG2, PDPN, PLAT, POSTN, RNF135). Immunohistochemical study of the most differentially expressed neuronal gene, alpha-internexin, clearly differentiated the two groups of gliomas, with 1p19q codeletion gliomas showing specific staining in tumor cells. Conclusion These findings provide evidence for neuronal differentiation in oligodendrogliomas with 1p19q codeletion and support the hypothesis that the cell of origin for gliomas with 1p19q codeletion could be a bi-potential progenitor cell, able to give rise to both neurons and oligodendrocytes.

Ducray François; Idbaih Ahmed; de Reyniès Aurélien; Bièche Ivan; Thillet Joëlle; Mokhtari Karima; Lair Séverine; Marie Yannick; Paris Sophie; Vidaud Michel; Hoang-Xuan Khê; Delattre Olivier; Delattre Jean-Yves; Sanson Marc

2008-01-01

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Phase III trial of chemoradiotherapy for anaplastic oligodendroglioma: long-term results of RTOG 9402.  

UK PubMed Central (United Kingdom)

PURPOSE: Anaplastic oligodendrogliomas, pure (AO) and mixed (anaplastic oligoastrocytoma [AOA]), are chemosensitive, especially if codeleted for 1p/19q, but whether patients live longer after chemoradiotherapy is unknown. PATIENTS AND METHODS: Eligible patients with AO/AOA were randomly assigned to procarbazine, lomustine, and vincristine (PCV) plus radiotherapy (RT) versus RT alone. The primary end point was overall survival (OS). RESULTS: Two hundred ninety-one eligible patients were randomly assigned: 148 to PCV plus RT and 143 to RT. For the entire cohort, there was no difference in median survival by treatment (4.6 years for PCV plus RT v 4.7 years for RT; hazard ratio [HR] = 0.79; 95% CI, 0.60 to 1.04; P = .1). Patients with codeleted tumors lived longer than those with noncodeleted tumors (PCV plus RT: 14.7 v 2.6 years, HR = 0.36, 95% CI, 0.23 to 0.57, P < .001; RT: 7.3 v 2.7 years, HR = 0.40, 95% CI, 0.27 to 0.60, P < .001), and the median survival of those with codeleted tumors treated with PCV plus RT was twice that of patients receiving RT (14.7 v 7.3 years; HR = 0.59; 95% CI, 0.37 to 0.95; P = .03). For those with noncodeleted tumors, there was no difference in median survival by treatment arm (2.6 v 2.7 years; HR = 0.85; 95% CI, 0.58 to 1.23; P = .39). In Cox models that included codeletion status, the adjusted OS for all patients was prolonged by PCV plus RT (HR = 0.67; 95% CI, 0.50 to 0.91; P = .01). CONCLUSION: For the subset of patients with 1p/19q codeleted AO/AOA, PCV plus RT may be an especially effective treatment, although this observation was derived from an unplanned analysis.

Cairncross G; Wang M; Shaw E; Jenkins R; Brachman D; Buckner J; Fink K; Souhami L; Laperriere N; Curran W; Mehta M

2013-01-01

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A novel, diffusely infiltrative xenograft model of human anaplastic oligodendroglioma with mutations in FUBP1, CIC, and IDH1.  

UK PubMed Central (United Kingdom)

Oligodendroglioma poses a biological conundrum for malignant adult human gliomas: it is a tumor type that is universally incurable for patients, and yet, only a few of the human tumors have been established as cell populations in vitro or as intracranial xenografts in vivo. Their survival, thus, may emerge only within a specific environmental context. To determine the fate of human oligodendroglioma in an experimental model, we studied the development of an anaplastic tumor after intracranial implantation into enhanced green fluorescent protein (eGFP) positive NOD/SCID mice. Remarkably after nearly nine months, the tumor not only engrafted, but it also retained classic histological and genetic features of human oligodendroglioma, in particular cells with a clear cytoplasm, showing an infiltrative growth pattern, and harboring mutations of IDH1 (R132H) and of the tumor suppressor genes, FUBP1 and CIC. The xenografts were highly invasive, exhibiting a distinct migration and growth pattern around neurons, especially in the hippocampus, and following white matter tracts of the corpus callosum with tumor cells accumulating around established vasculature. Although tumors exhibited a high growth fraction in vivo, neither cells from the original patient tumor nor the xenograft exhibited significant growth in vitro over a six-month period. This glioma xenograft is the first to display a pure oligodendroglioma histology and expression of R132H. The unexpected property, that the cells fail to grow in vitro even after passage through the mouse, allows us to uniquely investigate the relationship of this oligodendroglioma with the in vivo microenvironment.

Klink B; Miletic H; Stieber D; Huszthy PC; Valenzuela JA; Balss J; Wang J; Schubert M; Sakariassen PØ; Sundstrøm T; Torsvik A; Aarhus M; Mahesparan R; von Deimling A; Kaderali L; Niclou SP; Schröck E; Bjerkvig R; Nigro JM

2013-01-01

26

A Novel, Diffusely Infiltrative Xenograft Model of Human Anaplastic Oligodendroglioma with Mutations in FUBP1, CIC, and IDH1  

Science.gov (United States)

Oligodendroglioma poses a biological conundrum for malignant adult human gliomas: it is a tumor type that is universally incurable for patients, and yet, only a few of the human tumors have been established as cell populations in vitro or as intracranial xenografts in vivo. Their survival, thus, may emerge only within a specific environmental context. To determine the fate of human oligodendroglioma in an experimental model, we studied the development of an anaplastic tumor after intracranial implantation into enhanced green fluorescent protein (eGFP) positive NOD/SCID mice. Remarkably after nearly nine months, the tumor not only engrafted, but it also retained classic histological and genetic features of human oligodendroglioma, in particular cells with a clear cytoplasm, showing an infiltrative growth pattern, and harboring mutations of IDH1 (R132H) and of the tumor suppressor genes, FUBP1 and CIC. The xenografts were highly invasive, exhibiting a distinct migration and growth pattern around neurons, especially in the hippocampus, and following white matter tracts of the corpus callosum with tumor cells accumulating around established vasculature. Although tumors exhibited a high growth fraction in vivo, neither cells from the original patient tumor nor the xenograft exhibited significant growth in vitro over a six-month period. This glioma xenograft is the first to display a pure oligodendroglioma histology and expression of R132H. The unexpected property, that the cells fail to grow in vitro even after passage through the mouse, allows us to uniquely investigate the relationship of this oligodendroglioma with the in vivo microenvironment.

Valenzuela, Jaime Alberto Campos; Balss, Jorg; Wang, Jian; Schubert, Manja; Sakariassen, Per ?ystein; Sundstr?m, Terje; Torsvik, Anja; Aarhus, Mads; Mahesparan, Rupavathana; von Deimling, Andreas; Kaderali, Lars; Niclou, Simone P.; Schrock, Evelin; Bjerkvig, Rolf; Nigro, Janice M.

2013-01-01

27

1p/19q codeletion and IDH1/2 mutation identified a subtype of anaplastic oligoastrocytomas with prognosis as favorable as anaplastic oligodendrogliomas.  

UK PubMed Central (United Kingdom)

BACKGROUND: Anaplastic astrocytoma (AA), anaplastic oligoastrocytoma (AOA), and anaplastic oligodendroglioma (AO) are the major histological subtypes of World Health Organization grade III gliomas. More evidence suggests that AOA is unlikely to be a distinct entity, and re-evaluation of this issue has been recommended. In this study, we divided AOA into 2 subgroups, according to molecular biomarkers, and compared the survivals between them. METHODS: One hundred nine patients with histological diagnosis of anaplastic gliomas enrolled in the study. Molecular biomarkers evaluated included 1p/19q codeletion and IDH1/2 mutation. Kaplan-Meier plots were compared by log-rank method. RESULTS: There was no significant difference between AA and AOA with regard to the frequencies of biomarkers and survival plots. According to the status of biomarkers, AOA was classified into 2 subgroups (AOA1 and AOA2), for which Kaplan-Meier plots were significantly different (P = .001 for both progression-free survival [PFS] and overall survival [OS]). AOA1 with 1p/19q codeletion and/or IDH1/2 mutation showed similar Kaplan-Meier plots with AO (P = .169 for PFS and P = .523 for OS). AOA2 without either biomarker showed similar Kaplan-Meier plots with AA (P = .369 for PFS and P = .271 for OS). In addition, patients with AO and AOA1 had significantly longer PFS and OS than did patients with AA and AOA2 (P < .001 for both PFS and OS). CONCLUSIONS: AOA is a heterogeneous group and can be divided into 2 subgroups with significantly different prognoses according to the status of 1p/19q and IDH1/2. This will be helpful in estimating patients' prognosis and guiding reasonable therapy for patients with anaplastic gliomas.

Jiang H; Ren X; Cui X; Wang J; Jia W; Zhou Z; Lin S

2013-06-01

28

Brainstem oligodendroglioma in a puppy.  

UK PubMed Central (United Kingdom)

A 5 mo old male golden retriever presented for evaluation of an acute onset, progressive neurologic disease. Although computed tomography (CT) was unremarkable, MRI identified an ill-defined mass located in the medulla, which was considered likely responsible for the clinical signs. The imaging features closely resembled the classic features of human brainstem gliomas in the pediatric population. Histopathologic examination confirmed the lesion to be an anaplastic oligodendroglioma.

Mateo I; Orlandi R; Vazquez F; Muñoz A

2013-09-01

29

Cytologic characteristics of intracytoplasmic refractile eosinophilic granular bodies in anaplastic oligodendroglioma: a case report.  

UK PubMed Central (United Kingdom)

BACKGROUND: Oligodendrogliomas, which have a relatively better prognosis than tumors of the astrocytic lineage, have few morphologic clues for diagnosis. CASE: To address this problem, eosinophilic refractile inclusions were examined cytologically in the tumor of a 59-year-old man, using surgical materials for rapid diagnosis. Cytologic, histologic, and immunohistochemical findings were compatible with the refractile eosinophilic inclusions found in oligodendroglial tumors. The tumor cells presented a sheet-like epithelial pattern, forming no overlapping cell clusters, with an ill-defined cytoplasmic membrane, and nuclei that appeared to be naked, approximately 2 times the size of a red blood cell (approximately 7 microm) in diameter. It was easier to examine the cells and inclusions by cytologic preparations than by histology. CONCLUSION: The inclusions were thought to be a diagnostic clue for oligodendrogliomas, especially on cytology, and cytology was more useful than histology.

Kojima H; Mori K; Fukudome N; Iseki M; Shimizu S

2008-07-01

30

Phase II trial of preirradiation and concurrent temozolomide in patients with newly diagnosed anaplastic oligodendrogliomas and mixed anaplastic oligoastrocytomas: RTOG BR0131.  

Science.gov (United States)

The primary objectives of this phase II study were to evaluate the use of preirradiation temozolomide followed by concurrent temozolomide and radiotherapy (RT) in patients with newly diagnosed anaplastic oligodendroglioma (AO) and mixed anaplastic oligoastrocytoma (MOA). Preirradiation temozolomide (150 mg/m(2)/day) was given on a 7-day-on/7-day-off schedule for up to six cycles. The primary end point was the response rate during the 6-month, pre-RT chemotherapy. Tumor tissue was analyzed for the presence of chromosomal deletions on 1p and 19q and for MGMT-promoter methylation. Forty-two patients were enrolled; 39 were eligible. The objective response rate was 32% (6% [complete response, CR], 26% [partial response PR]), and the rate of progression during pre-RT chemotherapy was 10%. The worst nonhematological toxicity was grade 4 in three patients (8%). Twenty-two patients completed concurrent chemotherapy and RT. There were no grade 4 nonhematological toxicities during the concurrent chemotherapy and RT. Seventeen of 28 (60.7%) evaluable cases had codeletion of 1p/19q; all 17 were free from progression at 6 months. Sixteen of 20 (80%) evaluable cases had MGMT-promoter methylation; all 16 were free from progression at 6 months. In conclusion, the rate of progression of 10% during pre-RT temozolomide chemotherapy for newly diagnosed AO and MAO compared favorably with prior experience with pre-RT PCV chemotherapy (20% in RTOG 9402). The toxicity of the dose-intense pre-RT regimen used in this study may warrant evaluation of other, less intense dosing strategies. Future studies will need to prospectively stratify patients according to the presence of deletions of chromosomes 1p and 19q. PMID:18779504

Vogelbaum, Michael A; Berkey, Brian; Peereboom, David; Macdonald, David; Giannini, Caterina; Suh, John H; Jenkins, Robert; Herman, James; Brown, Paul; Blumenthal, Deborah T; Biggs, Christopher; Schultz, Christopher; Mehta, Minesh

2008-09-08

31

Bone marrow involvement in systemic alk+ anaplastic large cell lymphoma: morphological resemblance with hodgkin's lymphoma  

International Nuclear Information System (INIS)

[en] A 21 years old male presented with enlarged cervical lymph nodes. Diagnosis of anaplastic large cell lymphoma was made on lymph node biopsy and confirmed by immunohistochemistry using a panel of monoclonal antibodies including ALK-1. Bone marrow aspiration revealed the presence of large lymphoma cells and trephine biopsy showed interstitial involvement. In addition, there was presence of binucleate cells, lymphocytes, plasma cells and eosinophils. All these features resulted in a strong resemblance of the morphology with Hodgkin's lymphoma. (author)

2006-01-01

32

Up-front temozolomide in elderly patients with anaplastic oligodendroglioma and oligoastrocytoma.  

Science.gov (United States)

Optimal treatment of anaplastic oligodendroglial tumors (AOT) in elderly patients is debatable. We report a retrospective study of 44 consecutive patients aged 70 years or older [median age: 74 years; median Karnofsky performance status (KPS): 70] treated with up-front chemotherapy using temozolomide (TMZ) at conventional doses until tumor progression. O(6)-methylguanine-DNA methyltransferase promoter (MGMTP) methylation was assessed in 38 patients. Of the 41 evaluable patients, partial response (PR) was seen in 13 (32%) patients, 17 (41%) patients achieved stable disease, while the disease progressed in 11 (27%) patients. Median progression-free survival (PFS) and overall survival (OS) were 6.9 and 12.4 months, respectively. Hematotoxicity grades 3-4 occurred in nine patients (20%). MGMTP was methylated in 50% of patients and was associated with both longer PFS (8.7 versus 5.7 months, P = 0.01) and longer OS (16.1 versus 12.4 months, P = 0.05). The rate of responders to chemotherapy was similar in MGMTP-methylated (38%) and in MGMTP-unmethylated patients (31%), but duration of response was significantly longer in responders with methylated MGMTP than in responders with unmethylated MGMTP (16.1 versus 9.6 months, P = 0.0004). This study demonstrates that a substantial number of elderly patients with AOT can achieve prolonged survival with up-front chemotherapy using TMZ. Further investigation is needed to determine whether this treatment is preferable to initial radiation therapy. PMID:20556480

Ducray, François; del Rio, Monica Sierra; Carpentier, Catherine; Psimaras, Dimitri; Idbaih, Ahmed; Dehais, Caroline; Kaloshi, Gentian; Mokhtari, Karima; Taillibert, Sophie; Laigle-Donadey, Florence; Omuro, Antonio; Sanson, Marc; Delattre, Jean-Yves; Hoang-Xuan, Khê

2010-06-17

33

Up-front temozolomide in elderly patients with anaplastic oligodendroglioma and oligoastrocytoma.  

UK PubMed Central (United Kingdom)

Optimal treatment of anaplastic oligodendroglial tumors (AOT) in elderly patients is debatable. We report a retrospective study of 44 consecutive patients aged 70 years or older [median age: 74 years; median Karnofsky performance status (KPS): 70] treated with up-front chemotherapy using temozolomide (TMZ) at conventional doses until tumor progression. O(6)-methylguanine-DNA methyltransferase promoter (MGMTP) methylation was assessed in 38 patients. Of the 41 evaluable patients, partial response (PR) was seen in 13 (32%) patients, 17 (41%) patients achieved stable disease, while the disease progressed in 11 (27%) patients. Median progression-free survival (PFS) and overall survival (OS) were 6.9 and 12.4 months, respectively. Hematotoxicity grades 3-4 occurred in nine patients (20%). MGMTP was methylated in 50% of patients and was associated with both longer PFS (8.7 versus 5.7 months, P = 0.01) and longer OS (16.1 versus 12.4 months, P = 0.05). The rate of responders to chemotherapy was similar in MGMTP-methylated (38%) and in MGMTP-unmethylated patients (31%), but duration of response was significantly longer in responders with methylated MGMTP than in responders with unmethylated MGMTP (16.1 versus 9.6 months, P = 0.0004). This study demonstrates that a substantial number of elderly patients with AOT can achieve prolonged survival with up-front chemotherapy using TMZ. Further investigation is needed to determine whether this treatment is preferable to initial radiation therapy.

Ducray F; del Rio MS; Carpentier C; Psimaras D; Idbaih A; Dehais C; Kaloshi G; Mokhtari K; Taillibert S; Laigle-Donadey F; Omuro A; Sanson M; Delattre JY; Hoang-Xuan K

2011-02-01

34

Postoperative treatment of patients with anaplastic oligodendrogliomas. Thirty years experience of the Maria Sklodowska-Curie Memorial Centre in Cracow, 1975 - 2000  

International Nuclear Information System (INIS)

[en] Background: Anaplastic oligodendrogliomas (AO) are infiltrative, mostly supratentorial tumours, often bilaterally affecting the white matter. Radiotherapy alone or in combination with chemotherapy have a role in the adjuvant treatment of AO, but currently the efficacy of various treatment modalities could not be definitively determined because of the heterogeneity of the therapies used. Aim: Assessment of the efficacy of altered therapy schedules in postoperative treatment of patients with anaplastic oligodendrogliomas. Materials/Methods: Between 1975 and 2000, 101 adult patients with anaplastic oligodendrogliomas were postoperatively treated in our institution. During this period patients received conventional radiation therapy and chemotherapy (CRT/CH), conventional radiation therapy (CRT), and split course hypofractionated radiation therapy (SCHRT). Between 1975 and 1985, CRT/CH was applied in 42 patients. Whole brain irradiation was delivered; the tumour dose of 5 Gy in 25 fractions over 5 weeks was calculated at the midplane of the skull. Then treatment fields were reduced and a 10 Gy boost was given in 5 fractions over 5 days to the known tumour bearing area. On the last day of irradiation patients began the first of six planned series of chemotherapy with CCNU, given 100 mg/2, orally every 8 weeks. From 1986 to 1990, CRT was received by 27 patients. Irradiation was only as described above. Between 1991 and 2000, 32 patients were given SCHRT. There were 3 courses of irradiation separated by a one-month interval. In each of the two first series patients received 20 Gy in 5 fractions in five days to the whole brain, and in the third course a 20 Gy boost in 5 fractions over 5 days was given as in the CRT regimen. Results: Actuarial overall survival rates at two and five years were 38 % and 10 % respectively for patients treated with CRT/CH, 36 % and 11 % for the CRT group, and 23 % and 6 % for the SCHRT option. Multivariate analysis revealed that only age was a significant factor. Patients aged 45 years or less carried the best prognosis. Conclusions: The efficacy of different postoperative treatments administered to our patients with anaplastic oligodendrogliomas gave approximately comparable and unrewarding poor results. (authors)

2007-01-01

35

Endobronchial ALK+ anaplastic large-cell lymphoma resembling asthma in a 13-year-old girl.  

UK PubMed Central (United Kingdom)

Anaplastic large-cell lymphoma is a rare disease in children, and endobronchial localization is extremely rare in any age group. We report the case of a 13-year-old girl with endobronchial anaplastic lymphoma kinase-positive anaplastic large-cell lymphoma presenting as asthma, and discuss the diagnostic, therapeutic, and clinical implications.

Pavlov N; Pavlov V; Culi? S; Armanda V; Siebert R; Lozi? B; Forempoher G; Lukši? B; Peri? I; Goic-Barisic I

2013-01-01

36

Adjuvant procarbazine, lomustine, and vincristine chemotherapy in newly diagnosed anaplastic oligodendroglioma: long-term follow-up of EORTC brain tumor group study 26951.  

UK PubMed Central (United Kingdom)

PURPOSE: Anaplastic oligodendroglioma are chemotherapy-sensitive tumors. We now present the long-term follow-up findings of a randomized phase III study on the addition of six cycles of procarbazine, lomustine, and vincristine (PCV) chemotherapy to radiotherapy (RT). PATIENTS AND METHODS: Adult patients with newly diagnosed anaplastic oligodendroglial tumors were randomly assigned to either 59.4 Gy of RT or the same RT followed by six cycles of adjuvant PCV. An exploratory analysis of the correlation between 1p/19q status and survival was part of the study. Retrospectively, the methylation status of the methyl-guanine methyl transferase gene promoter and the mutational status of the isocitrate dehydrogenase (IDH) gene were determined. The primary end points were overall survival (OS) and progression-free survival based on intent-to-treat analysis. RESULTS: A total of 368 patients were enrolled. With a median follow-up of 140 months, OS in the RT/PCV arm was significantly longer (42.3 v 30.6 months in the RT arm, hazard ratio [HR], 0.75; 95% CI, 0.60 to 0.95). In the 80 patients with a 1p/19q codeletion, OS was increased, with a trend toward more benefit from adjuvant PCV (OS not reached in the RT/PCV group v 112 months in the RT group; HR, 0.56; 95% CI, 0.31 to 1.03). IDH mutational status was also of prognostic significance. CONCLUSION: The addition of six cycles of PCV after 59.4 Gy of RT increases both OS and PFS in anaplastic oligodendroglial tumors. 1p/19q-codeleted tumors derive more benefit from adjuvant PCV compared with non-1p/19q-deleted tumors.

van den Bent MJ; Brandes AA; Taphoorn MJ; Kros JM; Kouwenhoven MC; Delattre JY; Bernsen HJ; Frenay M; Tijssen CC; Grisold W; Sipos L; Enting RH; French PJ; Dinjens WN; Vecht CJ; Allgeier A; Lacombe D; Gorlia T; Hoang-Xuan K

2013-01-01

37

Predictive value of multimodality MRI using conventional, perfusion, and spectroscopy MR in anaplastic transformation of low-grade oligodendrogliomas.  

UK PubMed Central (United Kingdom)

The aim of our study was to evaluate the role of proton magnetic resonance (MR) spectroscopy and MR perfusion in the follow-up of low-grade gliomas, since conventional MR imaging (MRI) is not reliable in detecting the passage from a low- to high-grade tumor. Twenty-one patients with a World Health Organisation (WHO) grade II glioma were followed up using proton MR spectroscopy, perfusion, and conventional MRIs. Follow-up MRIs had been performed at the third month of evolution and then twice a year, with an average of five MR studies per patient. Five out of the 21 patients had an anaplastic transformation. A choline to creatine ratio (choline/creatine ratio) above 2.4 is associated with an 83% risk of a malignant transformation in an average delay of 15.4 months. The choline/creatine ratio at this threshold was more efficient than perfusion MR in detecting the anaplastic transformation, with sensitivity of 80% and specificity of 94%. An increased choline/creatine ratio seemed to occur an average 15 months before the elevation of relative cerebral blood volume (rCBV). The mean annual growth of low-grade glioma was 3.65 mm. A growth rate higher than 3 mm per year was also correlated with greater risk of anaplastic transformation. Proton magnetic resonance spectroscopy should be recommended in the follow-up of low-grade gliomas since the choline/creatine ratio can predict anaplastic transformation before perfusion abnormalities, with high positive predictive value of 83%.

Hlaihel C; Guilloton L; Guyotat J; Streichenberger N; Honnorat J; Cotton F

2010-03-01

38

Combining two biomarkers, IDH1/2 mutations and 1p/19q codeletion, to stratify anaplastic oligodendroglioma in three groups: a single-center experience.  

Science.gov (United States)

IDH1/2 mutations and 1p/19q codeletion occur frequently in anaplastic gliomas and are prognostic factors. We combined these two biomarkers to stratify patients treated for anaplastic oligodendroglioma (AO). 43 consecutive WHO AO were selected. We combined immunohistochemistry (IHC) with the monoclonal antibody mIDH1R132H and DNA sequencing of IDH1 and IDH2 genes. Fluorescence in situ hybridization was carried out to evaluate 1p/19q codeletion. These biomarkers were correlated with progression-free survival (PFS) and overall survival (OS). IDH1/IDH2 mutations occurred in 23/43 (54 %) patients: 20/43 IDH1-R132H mutation in IHC, 2/43 IDH1-R132G mutation and 1/43 IDH2-R172K mutation identified by DNA sequencing. 1p/19q codeletion was detected for 23/43 patients. With median follow-up of 19 months (range 1.4-128), median PFS and OS were 22 and 35 months respectively. IDH1/IDH2 mutations were strongly associated with improved PFS and OS: 5-year PFS was 86 versus 6 % and 5-year OS was 91 versus 9 % for patients with IDH1/IDH2 mutations versus wild-type IDH respectively. In multivariate analyses, IDH1/IDH2 mutations and 1p/19q loss were independent prognostic factors. Three groups with distinct prognostic features were identified: patients with IDH1/2 mutations and 1p/19q loss (median PFS, median OS not reached), patients with IDH1/2 mutations or 1p/19q loss (median PFS: 22 months, median OS: 30 months), and patients without IDH1/2 mutations nor 1p/19q loss with a bad prognosis (median PFS: 8.6 months, median OS: 9.9 months). Combining two biomarkers, IDH1/2 and 1p/19q codeletion, makes it possible to stratify AO in three groups with very distinct prognostic features. PMID:23681562

Frenel, J S; Leux, C; Loussouarn, D; Le Loupp, A-G; Leclair, F; Aumont, M; Mervoyer, A; Martin, S; Denis, M G; Campone, M

2013-05-17

39

MGMT-STP27 methylation status as Predictive Marker for Response to PCV in Anaplastic Oligodendrogliomas and Oligoastrocytomas. A report from EORTC study 26951.  

UK PubMed Central (United Kingdom)

PURPOSE: The long-term follow-up results from the EORTC-26951 trial showed that the addition of PCV after radiotherapy increases survival in anaplastic oligodendrogliomas/oligoastrocytomas (AOD/AOA). However, some patients appeared to benefit more from PCV treatment than others. EXPERIMENTAL DESIGN: We performed genome-wide methylation profiling of 115 samples included in the EORTC-26951 trial and extracted the CpG island hypermethylated phenotype (CIMP) and MGMT promoter-methylation (MGMT-STP27) status. RESULTS: We first demonstrate that methylation profiling can be performed on archival tissues with a performance that is similar to snap frozen tissue samples. We then performed methylation profiling on EORTC-26951 clinical trial samples. Univariate analysis indicated that CIMP+ or MGMT-STP27 methylated tumors had an improved survival compared to CIMP- and/or MGMT-STP27 unmethylated tumors (median overall survival (OS) 1.05 v. 6.46 years and 1.06 v. 3.8 years, both P<0.0001 for CIMP and MGMT-STP27 status respectively). Multivariable analysis indicates that CIMP and MGMT-STP27 are significant prognostic factors for survival in presence of age, sex performance score and review diagnosis in the model. CIMP+ and MGMT-STP27 methylated tumors showed a clear benefit from adjuvant PCV chemotherapy: the median OS of CIMP+ samples in the RT and RT-PCV arms was 3.27 and 9.51 years respectively P=0.0033; for MGMT-STP27 methylated samples it was 1.98 and 8.65 years. There was no such benefit for CIMP- or MGMT-STP27 unmethylated tumors. MGMT-STP27 status remained significant in an interaction test (P=0.003). Statistical analysis of microarrays (SAM) identified 259 novel CpGs associated with treatment response. CONCLUSIONS: MGMT-STP27 may be used to guide treatment decisions in this tumor type.

van den Bent MJ; Erdem Eraslan L; Idbaih A; de Rooi JJ; Eilers PH; Spliet W; den Dunnen WF; Tijssen C; Wesseling P; Sillevis Smitt PA; Kros JM; Gorlia T; French PJ

2013-08-01

40

New clinical, pathological and molecular prognostic models and calculators in patients with locally diagnosed anaplastic oligodendroglioma or oligoastrocytoma. A prognostic factor analysis of European Organisation for Research and Treatment of Cancer Brain Tumour Group Study 26951.  

UK PubMed Central (United Kingdom)

BACKGROUND: The prognosis of patients with anaplastic oligodendrogliomas (AOD) and oligoastrocytomas (AOA) is variable. Biomarkers might be helpful to identify more homogeneous disease subtypes and improve therapeutic index. The aim of this study is to develop new clinical, pathological and molecular prognostic models for locally diagnosed anaplastic gliomas with oligodendroglial features (AOD or AOA). METHODS: Data from 368 patients with AOD or AOA recruited in The European Organisation for Research and Treatment of Cancer (EORTC) trial 26951 on adjuvant PCV (Procarbazine, CCNU, Vincristine) chemotherapy in anaplastic oligodendroglial tumours were used to develop multifactor models to predict progression free survival (PFS) and overall survival (OS). Different models were compared by their percentage of explained variation (PEV). Prognostic calculators were derived from these new models. RESULTS: Treatment (for PFS only), younger age, confirmed absence of residual tumour on imaging, frontal location, good World Health Organisation (WHO) performance status, absence of endothelial abnormalities and/or necrosis, 1p/19q codeletion and Isocitrate dehydrogenase 1 (IDH1) mutation were independent factors that predicted better PFS and OS. CONCLUSIONS: We identified important prognostic factors for AOD and AOA and showed that molecular markers added a major contribution to clinical and pathological factors in explaining PFS and OS. With a positive predictive value of 92% for PFS and 94% for OS, our models allow physicians to precisely identify high risk patients and aid in making therapeutic decisions.

Gorlia T; Delattre JY; Brandes AA; Kros JM; Taphoorn MJ; Kouwenhoven MC; Bernsen HJ; Frénay M; Tijssen CC; Lacombe D; van den Bent MJ

2013-07-01

 
 
 
 
41

Prognosis and management of oligodendrogliomas  

International Nuclear Information System (INIS)

Purpose: To determine the prognostic significance of histologic grading in oligodendrogliomas. And to assess the role of postoperative radiotherapy in low-grade tumors and adjuvant chemotherapy in anaplastic tumors. Methods and Materials: The records of 38 consecutive patients with the diagnosis of oligodendroglioma were studied. Histologic grade was based on the Smith System and adjusted for the Kernohan's variables. Treatment of low-grade tumors consisted of surgery or surgery plus postoperative radiotherapy. High-grade tumors, anaplastic and malignant, had surgery and postoperative radiotherapy with or without adjuvant chemotherapy. Results: 23 patients with low-grade tumors showed improved 5-year progression-free survival compared to 15 patients with high-grade tumors (74% and 27% respectively), and improved overall 5-year survival (70% compared to 27%). Of the low-grade group, 16 patients receiving postoperative radiotherapy showed improved 5-year progression-free survival compared to 7 patients who did not receive adjuvant radiation (80% and 24% respectively). The overall 5-year survival was similar (86%). 10 patients of the high-grade group had anaplastic oligodendrogliomas. 5 patients receiving adjuvant chemotherapy showed improved 5-year progression-free survival compared to 5 patients not receiving adjuvant chemotherapy (60% and 20% respectively). The overall 5-year survival was similar (40%). The remaining 5 patients of the high-grade group had malignant oligodendrogliomas. These received adjuvant chemotherapy and had similar overall survival and progression-free survival, ie: 40% at 5 years. Conclusion: High-grade oligodendroglimas have worse prognosis than low-grade tumors, postoperative radiotherapy may offer some benefit prolonging time to progression of low-grade tumors. Similarly adjuvant chemotherapy may prolong time to progression of anaplastic oligodendrogliomas. Prospective randomized clinical trials are needed to more fully assess these issues.

1996-01-01

42

Oligodendroglioma of the cerebellum.  

Science.gov (United States)

Oligodendrogliomas make up about 3.9 to 8% of brain tumors and present commonly as lesions of the cerebral hemispheres. Such tumors are uncommon in the posterior fossa. We would like to record the case of a woman who presented with a cerebellar oligodendroglioma, has survived two operations and is well 13 years after the first operation. PMID:7368074

Gittens, W O; Huestis, W S; Sangalang, V E

1980-03-01

43

Oligodendroglioma of the cerebellum.  

UK PubMed Central (United Kingdom)

Oligodendrogliomas make up about 3.9 to 8% of brain tumors and present commonly as lesions of the cerebral hemispheres. Such tumors are uncommon in the posterior fossa. We would like to record the case of a woman who presented with a cerebellar oligodendroglioma, has survived two operations and is well 13 years after the first operation.

Gittens WO; Huestis WS; Sangalang VE

1980-03-01

44

Anaplastic glioma.  

UK PubMed Central (United Kingdom)

OPINION STATEMENT: The treatment of anaplastic glioma (AG) varies depending on histopathology of the tumor, molecular markers, and individual patient characteristics. Maximal surgical resection is desirable for all types of AG if technically feasible, with an acceptable level of risk, and with the goal of preserving neurologic function. As opposed to the standard treatment of glioblastoma, based on a large, randomized, phase 3 trial, there is no accepted standard treatment for AG. Anaplastic astrocytoma (AA) is most often treated with radiotherapy (RT), with or without concomitant temozolomide (TMZ) and with or without adjuvant temozolomide. Rarely is AA treated with chemotherapy alone, although different treatment modalities are being evaluated in ongoing trials. The treatment of anaplastic oligodendroglioma (AO) and anaplastic oligoastrocytoma (AOA) is influenced by the 1p/19q status, as allelic co-deletion of chromosomes 1p and 19q predicts increased sensitivity to chemotherapy and prolonged survival. In contrast to the treatment of AA, carefully selected patients with AO and AOA may be treated with chemotherapy alone. Temozolomide has largely replaced PCV (procarbazine, CCNU, vincristine) as the chemotherapeutic agent for AO and AOA, largely due to greater tolerability and less potential for toxicity. However, whether temozolomide has similar efficacy to PCV has not been fully evaluated. Patients with AO and AOA with significant residual tumor after surgery, intractable seizures, and/or non co-deleted 1p/19q status are often treated with RT with or without concomitant chemotherapy and with or without adjuvant chemotherapy. There is no standard postoperative care for anaplastic ependymoma (AE). The efficacy of upfront versus delayed RT has not been evaluated. Surgery may be indicated for patients with recurrent AG. There may be benefit on overall survival, although this has not been clearly proven. Reoperation may also provide symptomatic relief and confirm the pathology, including differentiation of radiation necrosis from recurrent tumor. Confirmation of tumor grade is often important for enrollment in clinical trials, a reasonable treatment choice for patients with recurrent tumor. Treatment of recurrent AG often depends on prior treatments. Patients who have progressed after RT alone may be treated with temozolomide or PCV. Patients treated previously with chemotherapy alone may be treated with RT at time of progression. Dose-intense temozolomide, bevacizumab alone, or bevacizumab in combination with a cytotoxic agent are other treatment options. Focused radiation such as stereotactic radiosurgery has no proven role in treating recurrent AG. A number of other treatment modalities are currently under active investigation, including targeted molecular inhibitors, immunotherapies, convection enhanced delivery, and viral gene therapies. There is no standard treatment for recurrent AE. Most patients undergo re-resection followed by RT if RT was not previously given. Chemotherapy may be given, but there is no standard chemotherapeutic regimen. Ongoing trials are evaluating the role of bevicizumab and targeted molecular agents in the treatment of AE.

Paleologos NA; Merrell RT

2012-08-01

45

Extraneural metastases of anaplastic oligodendroglial tumors.  

UK PubMed Central (United Kingdom)

Extraneural metastases of malignant gliomas are rare. According to the literature, they tend to appear in glioblastoma patients, but are exceptionally rare in anaplastic oligodendroglioma. We report on an anaplastic oligodendroglioma and an anaplastic oligoastrocytoma that metastasized to cervical lymph nodes and bones. Both patients were women aged 54 and 30 years, and the metastases appeared following craniotomy. In the first patient, metastases to cervical lymph nodes developed one year after surgery, and, despite adjuvant therapy, recurred in the same location several times. Fine needle aspiration biopsy (FNAB) of the cervical lymph node prior to neck dissection suggested a possible metastatic primitive neuroepithelial tumor. In the second case, metastases to the sacrum and femur developed after surgery for a recurrent anaplastic oligoastrocytoma. Our two cases reconfirm a rare but definite ability not only of glioblastoma but also of anaplastic oligodendroglioma, namely to metastasize to extraneural sites. It is important to bear this in mind, particularly in cases when the history of primary brain tumor is unavailable. In such instances, the correct diagnosis of the metastatic lesion may be extremely difficult if not impossible.

Volavsek M; Lamovec J; Popovi? M

2009-01-01

46

Supratentorial astrocytomas and oligodendrogliomas treated in the MRI era  

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There is at present no consensus on the policy for the treatment of patients with low-grade gliomas (LGGs). This report is a retrospective multi-institutional study of 100 patients (ages 16-65 years) with astrocytoma (grade II), oligodendroglioma, anaplastic oligodendroglioma and anaplastic oligoastrocytoma of the supratentorial areas which were treated with surgery and postoperative radiotherapy at five university hospitals in northern Japan between 1990 and 1997 when MRI was routinely used to determine the target volume. Most patients were irradiated with 50-60 Gy. The target volume usually covered the areas with T2 prolongation of MRI with a margin of 2 cm. The disease-specific 5-year survival rate was 87.4% for patients with oligodendroglioma and 75.3% for patients with astrocytoma. Survival for patients with astrocytoma in the MRI era appears to be improved compared with historical controls in the literature. Patients with astrocytoma aged 40 years and under had a significantly better disease-specific survival rate than those over 40 years (P<0.05) and patients with oligodendroglioma and oligoastrocytoma showed a similar tendency. Patients with astrocytoma who had over 50% of their tumor removed had a significantly better survival rate than those who had less than 50% removed (P<0.05). Chemotherapy appeared to improve the disease-specific survival rate of patients with oligodendroglioma but not that of patients with astrocytoma. Oligodendroglioma has a more protracted course of disease progression than astrocytoma. This particular feature and the sensitivity of LGGs to chemotherapy as well as their relevant prognostic factors, such as age, histopathology and amount of tumor removal, should be taken into account before any decision on treatment methods for LGGs is made. (author)

Sakata, Koh-ichi; Hareyama, Masato [Sapporo Medical Univ. (Japan). School of Medicine; Komae, Takashi (and others)

2001-06-01

47

Age-related differences in 1p and 19q deletions in oligodendrogliomas  

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Full Text Available Abstract Background Recent reports indicate that anaplastic oligodendrogliomas frequently show allelic losses on chromosome arms 1p and 19q, and that these deletions are associated with better chemotherapeutic response and overall patient survival. Because of the diversified genetic makeup of the population and the centralized provincial referral system for brain tumor patients in Manitoba, the epidemiological features of such tumors sometimes differ from the published data acquired from non-community based settings. In this study, we assessed the prevalence of allelic deletions for chromosome arms 1p and 19q in anaplastic and in low-grade oligodendrogliomas in the Manitoba population. Methods Loss of heterozygosity (LOH) analysis of brain tumors was carried out using 4 microsatellite markers (D1S508, D1S2734, D19S219 and D19S412) and a PCR based assay. The tumors were consecutively acquired during the period September 1999–March 2001 and a total of 63 tumors were assessed. Results We found that allelic loss of chromosome 1p and 19q was higher in oligodendrogliomas than in other diffuse gliomas and that for anaplastic oligodendrogliomas, younger patients exhibited significantly more deletions than older patients (>60 years of age). Conclusions These studies suggest that age may be a factor in the genetic alterations of oligodendrogliomas. In addition, these studies demonstrate that this assay can easily be carried out in a cost-effective manner in a small tertiary center.

Myal Yvonne; Del Bigio Marc R; Rhodes Roy H

2003-01-01

48

Radiation and chemotherapy improve outcome in oligodendroglioma.  

UK PubMed Central (United Kingdom)

PURPOSE: Oligodendroglioma is a rare central nervous system tumor which may occur in pure or mixed histology. This scarcity results in difficulty in defining optimal management, mainly due to a lack of outcome analysis. Results are further complicated because the reported series include patients treated before megavoltage radiation or computed tomographic (CT) scan development. This makes extrapolation of outcome difficult to apply to modern-era patients. We report results obtained by current treatment and evaluation. METHODS AND MATERIALS: Outcome of all 38 patients (age 5-70 years) pathologically diagnosed between 1975 and 1993 were reviewed. Pure lesions were seen in 14 cases, of which three were anaplastic. The remainder had mixed tumors, of which six contained anaplastic astrocytic elements. Each patient had undergone maximal debulking surgery, but all remained with residual disease on postoperative CT. RESULTS: For nonanaplastic lesions, no local failure was seen in any patient receiving postoperative radiation (60 Gy) and chemotherapy (vincristine, procarbazine, and carmustine). Follow-up ranged from 28 to 240 months (median 125; N = 6). No postoperative therapy or chemotherapy alone resulted in local failure in all nine patients at risk [time to failure (TTF): 2-40 months; median 25]. Radiation alone in doses of 50 Gy at 2 Gy per day resulted in all six patients failing (TTF: 12-52 months; median 36). Achieving 60 Gy at 2 Gy a day allowed five of eight patients to remain disease free (disease-free survival: 24-160 months; median 66), with three failing at 26, 40, and 60 months. All lesions containing anaplastic elements underwent postoperative radiation therapy (60 Gy) and chemotherapy, with five of nine patients alive and well (median disease-free survival: 48 months; range 28-120). CONCLUSION: The combination of radiation and chemotherapy increases local control of oligodendroglioma whether they contain pure, mixed, or anaplastic histology. Radiation alone may offer good local control as long as 60 Gy is delivered. Chemotherapy alone appears to delay TTF and may be useful for pediatric patients.

Allison RR; Schulsinger A; Vongtama V; Barry T; Shin KH

1997-01-01

49

Radiation and chemotherapy improve outcome in oligodendroglioma  

International Nuclear Information System (INIS)

Purpose: Oligodendroglioma is a rare central nervous system tumor which may occur in pure or mixed histology. This scarcity results in difficulty in defining optimal management, mainly due to a lack of outcome analysis. Results are further complicated because the reported series include patients treated before megavoltage radiation or computed tomographic (CT) scan development. This makes extrapolation of outcome difficult to apply to modern-era patients. We report results obtained by current treatment and evaluation. Methods and Materials: Outcome of all 38 patients (age 5-70 years) pathologically diagnosed between 1975 and 1993 were reviewed. Pure lesions were seen in 14 cases, of which three were anaplastic. The remainder had mixed tumors, of which sic contained anaplastic astrocytic elements. Each patient had undergone maximal debulking surgery, but all remained with residual disease on postoperative CT. Results: For nonanaplastic lesions, no local failure was seen in any patient receiving postoperative radiation (60 Gy) and chemotherapy (vincristine, procarbazine, and carmustine). Follow-up ranged from 28 to 240 months (median 125; N = 6). No postoperative therapy or chemotherapy alone resulted in local failure in all nine patients at risk [time to failure (TTF): 2-40 months; median 25]. Radiation alone in doses of 50 Gy at 2 Gy per day resulted in all six patients failing (TTF: 12-52 months; median 36). Achieving 60 Gy at 2 Gy a day allowed five of eight patients to remain disease free (disease-free survival: 24-160 months; median 66), with three failing at 26, 40, and 60 months. All lesions containing anaplastic elements underwent post-operative radiation therapy (60 Gy) and chemotherapy, with five of nine patients alive and well (median disease-free survival: 48 months; range 28-120). Conclusion: The combination of radiation and chemotherapy increases local control of oligodendroglioma whether they contain pure, mixed, or anaplastic histology. Radiation alone may offer good local control as long as 60 Gy is delivered. Chemotherapy alone appears to delay TTF and may be useful for pediatric patients

1997-01-15

50

Supratentorial astrocytomas and oligodendrogliomas treated in the MRI era  

International Nuclear Information System (INIS)

There is at present no consensus on the policy for the treatment of patients with low-grade gliomas (LGGs). This report is a retrospective multi-institutional study of 100 patients (ages 16-65 years) with astrocytoma (grade II), oligodendroglioma, anaplastic oligodendroglioma and anaplastic oligoastrocytoma of the supratentorial areas which were treated with surgery and postoperative radiotherapy at five university hospitals in northern Japan between 1990 and 1997 when MRI was routinely used to determine the target volume. Most patients were irradiated with 50-60 Gy. The target volume usually covered the areas with T2 prolongation of MRI with a margin of 2 cm. The disease-specific 5-year survival rate was 87.4% for patients with oligodendroglioma and 75.3% for patients with astrocytoma. Survival for patients with astrocytoma in the MRI era appears to be improved compared with historical controls in the literature. Patients with astrocytoma aged 40 years and under had a significantly better disease-specific survival rate than those over 40 years (P

2001-01-01

51

Breast metastases from oligodendroglioma: An unusual extraneural spread in two young women and a review of the literature.  

UK PubMed Central (United Kingdom)

BACKGROUND: Extraneural dissemination of oligodendroglioma is rare. Cases of breast metastases have never been described in the literature. CASE REPORTS: We report the first two cases of young women with initial diagnosis of anaplastic oligodendroglioma who experienced mammary gland metastases and a review of the literature. RESULTS: Immunohistochemical analysis performed on material from both primary and metastatic sites did not allow to draw any conclusion on possible etiopathogenetic hypothesis. A review of literature yielded 35 cases of extracranial metastatic oligodendroglioma from 1989 to 2012. CONCLUSION: Though rare, extracranial dissemination from oligodendroglioma may occur not only in long surviving heavily pre-treated patients. The review of literature and these two cases suggest that spread is primarily to bone and then from bone to other organs through hematogenous route mostly due to leptomeningeal or dura mater invasion. Chemotherapy regimens similar to those commonly used for non metastatic oligodendroglioma are recommended for patients with good performance status.

Mazza E; Belli C; Terreni M; Doglioni C; Losio C; Cantore M; Mambrini A; Reni M

2013-08-01

52

Multiple oligodendroglioma: case report.  

Science.gov (United States)

An 18-year-old female patient was hospitalized with headache and disturbance of consciousness. Magnetic resonance imaging (MRI) revealed a tumor in the left parieto-occipital lobe. The tumor was totally removed, and postoperative radiation therapy was administered locally at 50 Gy. Ten months later, she experienced sudden onset of unconsciousness and headache. Computed tomography (CT) and MRI demonstrated multiple mass lesions in the whole brain. Following the systemic chemotherapy, removal of the largest tumor was performed. Histological examination proved all excised tumors to be oligodendroglioma without evidence of malignant change. PMID:10794567

Daneyemez, M; Baysefer, A; Can, C; Timurkaynak, E

2000-03-01

53

Multiple oligodendroglioma: case report.  

UK PubMed Central (United Kingdom)

An 18-year-old female patient was hospitalized with headache and disturbance of consciousness. Magnetic resonance imaging (MRI) revealed a tumor in the left parieto-occipital lobe. The tumor was totally removed, and postoperative radiation therapy was administered locally at 50 Gy. Ten months later, she experienced sudden onset of unconsciousness and headache. Computed tomography (CT) and MRI demonstrated multiple mass lesions in the whole brain. Following the systemic chemotherapy, removal of the largest tumor was performed. Histological examination proved all excised tumors to be oligodendroglioma without evidence of malignant change.

Daneyemez M; Baysefer A; Can C; Timurkaynak E

2000-03-01

54

Intraventricular oligodendroglioma: a case report  

International Nuclear Information System (INIS)

Oligodendrogliomas constitute approximately 5% off all cerebral gliomas and are usually found in the cerebral hemispheres (frontal lobe). They may invade the ventricles and spread to the ventricular system and leptomeninges. Oligodendrogliomas very rarely occur in the ventricles. This paper presents a case of 26-year-old woman with raised intracranial pressure and which computed tomographic findings of an intraventricular hyperdense mass occupying both lateral ventricles. Correlation with angiography and details of surgical treatment are presented. (author)

1992-01-01

55

Computed tomography and magnetic resonance imaging findings in patients with oligodendrogliomas: clinical and pathological correlation  

International Nuclear Information System (INIS)

Oligodendrogliomas are neuro epithelial neoplasms that originate from oligodendrocytes. These tumors account for 4% to 7% of all primary brain tumors and are most supratentorial. The purpose of this study was to evaluate the findings of computed tomography and magnetic resonance imaging examinations of patients with oligodendrogliomas and correlate these findings with clinical and pathological data. Oligodendrogliomas may be pure or mixed, present astrocytic elements and vary from well differentiated to anaplastic. The most common finding observed was a lesion that appeared hypodense on CT or had low-intensity signal on T 1-weighted and high-intensity signal on T 2-weighted magnetic resonance images. Cystic elements, mild surrounding edema and calcifications (usually coarse) in 2/3 of the cases were also observed. Tumor enhancement was generally mild and was observed in 80% of the cases. (author)

2001-01-01

56

p53 protein alterations in adult astrocytic tumors and oligodendrogliomas  

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Full Text Available BACKGROUND: p53 is a tumor suppressor gene implicated in the genesis of a variety of malignancies including brain tumors. Overexpression of the p53 protein is often used as a surrogate indicator of alterations in the p53 gene. AIMS: In this study, data is presented on p53 protein expression in adult cases (>15 years of age) of astrocytic (n=152) and oligodendroglial (n=28) tumors of all grades. Of the astrocytic tumors, 86% were supratentorial in location while remaining 14% were located infratentorially - 8 in the the cerebellum and 13 in the brainstem. All the oligodendrogliomas were supratentorial. MATERIALS AND METHODS: p53 protein expression was evaluated on formalin-fixed paraffin-embedded sections using streptavidin biotin immunoperoxidase technique after high temperature antigen retrieval. RESULTS: Overall 52% of supratentorial astrocytic tumors showed p53 immunopositivity with no correlation to the histological grade. Thus, 58.8% of diffuse astrocytomas (WHO Grade II), 53.8% of anaplastic astrocytomas (WHO Grade III) and 50% of glioblastomas (WHO Grade IV) were p53 protein positive. In contrast, all the infratentorial tumors were p53 negative except for one brainstem glioblastoma. Similarly, pilocytic astrocytomas were uniformly p53 negative irrespective of the location. Among oligodendroglial tumors, the overall frequency of p53 immunopositivity was lower (only 28%), though a trend of positive correlation with the tumor grade was noted - 25% in Grade II and 31.5% in grade III (anaplastic oligodendroglioma). Interestingly, p53 labeling index (p53 LI) did not correlate with the histopathological grade in both astrocytic and oligodendroglial tumors. CONCLUSIONS: Thus, this study gives an insight into the genetic and hence biological heterogeneity of gliomas, not only between astrocytic tumors vs. oligodendrogliomas but also within astrocytic tumors with regard to their grade and location. With p53 gene therapy trials in progress, this will possibly have future therapeutic implications.

Nayak Anupma; Ralte Angela; Sharma Mehar; Singh Varinder; Mahapatra Ashok; Mehta Veer; Sarkar Chitra

2004-01-01

57

Survival and prognostic factors of anaplastic gliomas.  

UK PubMed Central (United Kingdom)

BACKGROUND: The prognosis of patients with anaplastic glioma tumors is relatively favorable compared with patients with glioblastoma multiforme. OBJECTIVE: To estimate survival differences between anaplastic astrocytoma (AA) and anaplastic oligodendroglioma (AO) patients and factors associated with survival prognosis. METHODS: A nationwide cohort of grade III glioma patients diagnosed between 1990 and 2008 was studied using the Surveillance, Epidemiology, and End Results registry. Multivariate Cox proportional hazard models evaluated the role of patient and clinical characteristics on overall survival. RESULTS: A total of 1766 patients with AA and 570 patients with AO were studied. The median overall survival was 15 and 42 months among AA and AO patients, respectively. Age increments of 10 years implicated a 50% increase in mortality hazards among AA (hazard ratio [HR], 1.49; P < .001) and AO (HR, 1.51; P < .001) patients. Among AA patients, radiation (HR, 0.62; P < .001), surgery (vs biopsy; HR, 0.73; P < .001), female sex (HR, 0.87; P = .02), and married status (HR, 0.87; P = .02) were associated with a reduction in the hazard of mortality. Longer survival if diagnosed in 2000 relative to 1990 was observed (HR, 0.84; P = .004) in AA patients. Although surgery did not significantly improve survival among AO patients, gross total resection increased the median survival from 40 to 61 months (P = .001) in this cohort. CONCLUSION: First-course radiation, younger age, female sex, treatment in recent years, and surgery were associated with improved survival in AA patients. In contrast, age was the most prominent predictor of survival in AO patients. Surgery alone did not seem to benefit AO patients, and gross total resection improved survival by 21 months. ABBREVIATIONS: AA, anaplastic astrocytomaAO, anaplastic oligodendrogliomaGTR, gross total resectionKSP, Karnofsky Performance ScoreOS, overall survivalRT, radiation therapySEER, Surveillance, Epidemiology, and End Results.

Nuño M; Birch K; Mukherjee D; Sarmiento JM; Black KL; Patil CG

2013-09-01

58

Primary spinal cord oligodendroglioma: Case illustration Oligodendroglioma primario de la médula espinal: Caso clínico  

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Full Text Available Spinal cord oligodendrogliomas are rare pathologies of the spinal cord, and their location at conus and/or filum terminale is even rarer. There are only 7 spinal cord oligodendrogliomas reported in the literature. Our case is the eighth spinal cord oligodendrogliomas at this location.Los oligodendrogliomas de la médula espinal son raros y su localización en el cono medular o del filum terminale son aún menos frecuentes. Sólo hay siete oligodendrogliomas de la médula encontrados en la literatura. Nuestro caso es el octavo oligodendroglioma medular con esta localización

D. Gürkanlar; H. Koçak; A. Aciduman; E. Yucel; Ö. Ekinci

2006-01-01

59

[A rare case of dissemination of anaplastic oligodendroma outside the central nervous system].  

Science.gov (United States)

A rare case is reported of primary brain tumour with histological features of anaplastic oligodendroglioma, with metastases outside the central nervous system. Of interest is a short remission after treatment with cyclophosphamide and vepesid which shows that this type of tumour is sensitive in a way to chemotherapy. PMID:8502365

Rolski, J; Rzepecki, W; Ka?uza, J; Zeme?ka, T; Zuchowska-Vogelgesang, B

60

[A rare case of dissemination of anaplastic oligodendroma outside the central nervous system  

UK PubMed Central (United Kingdom)

A rare case is reported of primary brain tumour with histological features of anaplastic oligodendroglioma, with metastases outside the central nervous system. Of interest is a short remission after treatment with cyclophosphamide and vepesid which shows that this type of tumour is sensitive in a way to chemotherapy.

Rolski J; Rzepecki W; Ka?uza J; Zeme?ka T; Zuchowska-Vogelgesang B

1993-01-01

 
 
 
 
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Tomografia computadorizada e ressonância magnética nos oligodendrogliomas: correlação clínica e patológica Computed tomography and magnetic resonance imaging findings in patients with oligodendrogliomas: clinical and pathological correlation  

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Full Text Available Oligodendrogliomas são neoplasias do tecido neuroepitelial glial originárias de oligodendrócitos. São tumores infreqüentes, responsáveis por cerca de 4% a 7% das neoplasias primárias do cérebro, predominantemente supratentoriais. O presente trabalho consistiu na avaliação dos achados de imagem pré-operatória em tomografia computadorizada e ressonância magnética e correlação clínica e patológica, levando-se em consideração a presença de tumores puros ou mistos, com componente astrocitário e o seu grau de anaplasia. O aspecto mais freqüente foi o de lesão hipodensa na tomografia computadorizada ou com hipossinal em T1 e hipersinal em T2 na ressonância magnética, podendo ter componente cístico, com pouco edema ao redor, apresentando calcificações, quase sempre grosseiras, em dois terços dos casos. Reforço após contraste ocorre em 80% dos casos, na maioria discreto.Oligodendrogliomas are neuroepithelial neoplasms that originate from oligodendrocytes. These tumors account for 4% to 7% of all primary brain tumors and are most supratentorial. The purpose of this study was to evaluate the findings of computed tomography and magnetic resonance imaging examinations of patients with oligodendrogliomas and correlate these findings with clinical and pathological data. Oligodendrogliomas may be pure or mixed, present astrocytic elements and vary from well differentiated to anaplastic. The most common finding observed was a lesion that appeared hypodense on CT or had low-intensity signal on T1-weighted and high-intensity signal on T2-weighted magnetic resonance images. Cystic elements, mild surrounding edema and calcifications (usually coarse) in 2/3 of the cases were also observed. Tumor enhancement was generally mild and was observed in 80% of the cases.

Marcelo Novelino Simão; Gustavo Novelino Simão; Antônio Carlos dos Santos; Clóvis Simão Trad

2001-01-01

62

First report of oligodendroglioma in a sheep.  

UK PubMed Central (United Kingdom)

Oligodendrogliomas occur most commonly in the dog, but have also been reported in cattle, horses and cats. A 1-year-old sheep with neurological disturbances, including blindness, ataxia, circling and incoordination was referred to the veterinary clinic of Shahid Bahonar University of Kerman. Following euthanasia and necropsy, a soft, relatively well-demarcated mass was observed in the white and grey matter of the right cerebral hemisphere, close to the sylvian fissure in the right cerebral hemisphere. Microscopic examination revealed a sheet of densely packed tumour cells with hyperchromatic nuclei, lightly staining cytoplasm and characteristic perinuclear halo effect which is consistent with a diagnosis of oligodendroglioma. This is the 1st report of oligodendroglioma in sheep.

Derakhshanfar A; Mozaffari AA

2010-06-01

63

First report of oligodendroglioma in a sheep.  

Science.gov (United States)

Oligodendrogliomas occur most commonly in the dog, but have also been reported in cattle, horses and cats. A 1-year-old sheep with neurological disturbances, including blindness, ataxia, circling and incoordination was referred to the veterinary clinic of Shahid Bahonar University of Kerman. Following euthanasia and necropsy, a soft, relatively well-demarcated mass was observed in the white and grey matter of the right cerebral hemisphere, close to the sylvian fissure in the right cerebral hemisphere. Microscopic examination revealed a sheet of densely packed tumour cells with hyperchromatic nuclei, lightly staining cytoplasm and characteristic perinuclear halo effect which is consistent with a diagnosis of oligodendroglioma. This is the 1st report of oligodendroglioma in sheep. PMID:21247018

Derakhshanfar, A; Mozaffari, A A

2010-06-01

64

Anaplastic thyroid carcinoma  

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Full Text Available The aim of the present paper was to study some characteristics and posibility of surgery of anaplastic thyroid cancer. During five years period in Center for endocrine surgery, we found anaplastic thyroid cancer in 65 patienst (44 female and 21 male), median age 63 years (from 37 to 88 years). Surgical treatment was peerformed in one half (32) anaplastic thyroid cancer patients, at majority of them operative biopsy or tumor reduction only. Radical syrgery was performed in about 10% patients. Posibility of surgery in anaplastic thyroid cancer are very limited. In one third patients there were longstanding goter or thyroid nodul or histological verified dediferentiation of papillary thyroid cancer. This patienst should be operated formerly, before anaplastic transformation.

Živaljevi? Vladan R.; Dikli? Aleksandar ?.; Paunovi? Ivan R.; Krgovi? Ksenija Lj.; Živi? Rastko; Kaži? Milena; Kalezi? Nevena K.; Boži? Vesna; Tati? Svetislav B.; Havelka Marija J.

2003-01-01

65

Oligodendrogliomas: Impact of molecular genetics on treatment  

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Full Text Available The interest in oligodendrogliomas has increased since it became evident that a subset of these tumors respond to chemotherapy or radiation. This interest was augmented when the combined loss of the short arm of chromosome 1 and the long arm of chromosome 19 was identified as a powerful prediction factor for response. Lack of stringent morphological criteria allow high-interobserver variation with regard to classification and grading of oligodendroglial tumors. The prospect of beneficial chemotherapy prompted neuropathologists to diagnose more ?oligodendroglioma? than before. Therefore, there is great demand for unambiguous classification of oligodendroglial tumors. Supplementary analysis of the integrity of chromosomal arms 1p and 19q may greatly assist diagnostic characterization of tumors with oligodendroglial phenotype. The underlying mechanisms for these deletions are not known. Tumor suppressor genes on 1p and 19q relevant for oligodendroglioma have not yet been identified. Knowledge of these genes and the mechanisms of their inactivation might help to understand why oligodendroglial tumors do respond better to chemotherapy and radiotherapy than astrocytomas. This review compiles clinical, pathological and molecular genetic findings on oligodendrogliomas and oligoastrocytomas of WHO Grades II and III to present a brief overview on recent developments.

Hartmann C; von Deimling A

2005-01-01

66

ADAM 12: a putative marker of oligodendrogliomas?  

UK PubMed Central (United Kingdom)

ADAM 12 (meltrin alpha) belongs to a large family of molecules, consisting of members with both disintegrin and metalloproteinase properties. ADAMs have been implicated in several cell physiological processes including cell adhesion, cell fusion, proteolysis and signalling. ADAM 12 is widely expressed, including skeletal muscle, testis, bone, intestine, heart and kidney. In addition, a variety of tumours show elevated expression of ADAM12; among them being breast-, colon-, gastric- and lung-carcinoma. As to the brain, ADAM 12 has been shown previously to be expressed in rat and human oligodendrocytes. However, little is known about the expression of this protease in brain tumours. This study demonstrates the presence of ADAM 12 in non-neoplastic oligodendroglial cells of normal human brain as well as in neoplastic oligodendroglia and minigemistocytes arising from four pure oligodendrogliomas and three mixed oligoastrocytomas. Double stainings revealed a notable preference of ADAM 12 for the oligodendroglial over astroglial components. The results of immunohistochemistry are in accordance with the results obtained from the RT-PCR, which further demonstrated a mild difference concerning the mRNA concentration of ADAM 12 between similar grades of eight astrocytomas and eight oligodendrogliomas (namely four astrocytomas grade II versus four oligodendrogliomas grade II and four astrocytomas grade III versus four oligodendrogliomas grade III). Both cellular immunostaining for ADAM 12 and ADAM 12 mRNA content decrease with higher histologic grade of the tumour. Surprisingly, the latter parameter (ADAM12 mRNA) showed a significant opposite correlation to the degree of histologic tumour malignancy. From our data showing that ADAM 12 is highly expressed in, but not restricted to, oligodendrogliomas, we conclude that ADAM 12 immunohistochemistry may be a helpful tool in the diagnosis of brain tumours.

Kanakis D; Lendeckel U; Theodosiou P; Dobrowolny H; Mawrin C; Keilhoff G; Bukowska A; Dietzmann K; Bogerts B; Bernstein HG

2013-01-01

67

Purely cortical anaplastic ependymoma.  

UK PubMed Central (United Kingdom)

Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. It may occur outside the ventricular structures, representing the extraventicular form, or without any relationship of ventricular system, called ectopic ependymona. Less than fifteen cases of ectopic ependymomas were reported and less than five were anaplastic. We report a rare case of pure cortical ectopic anaplastic ependymoma.

Romero FR; Zanini MA; Ducati LG; Vital RB; de Lima Neto NM; Gabarra RC

2012-01-01

68

Purely Cortical Anaplastic Ependymoma  

Science.gov (United States)

Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. It may occur outside the ventricular structures, representing the extraventicular form, or without any relationship of ventricular system, called ectopic ependymona. Less than fifteen cases of ectopic ependymomas were reported and less than five were anaplastic. We report a rare case of pure cortical ectopic anaplastic ependymoma.

Romero, Flavio Ramalho; Zanini, Marco Antonio; Ducati, Luis Gustavo; Vital, Roberto Bezerra; de Lima Neto, Newton Moreira; Gabarra, Roberto Colichio

2012-01-01

69

Anaplastic ependymoma with ependymoblastic multilayered rosettes.  

Science.gov (United States)

Anaplastic ependymoma, World Health Organization grade III, is a malignant glioma with ependymal differentiation characterized by high mitotic activity often accompanied by microvascular proliferation and necrosis, where, generally, much fewer ependymal rosettes are found than in ependymoma, World Health Organization grade II. Ependymal rosettes, forming a single layer of tumor cells, differ from ependymoblastic multilayered rosettes, which are characteristic histologic features of ependymoblastoma, a variant of central nervous system primitive neuroectodermal tumor. Here, we report an autopsy case involving a 24-year-old woman with a frontal lobe tumor, which showed the aggregation of true rosettes with multilayering of tumor cells resembling the ependymoblastoma histology. Molecular and cytogenetic analyses revealed the absence of 19q13.42 amplification, a specific molecular hallmark of ependymoblastoma and embryonal tumor with abundant neuropil and true rosettes, supporting the diagnosis of anaplastic ependymoma. PMID:23791209

Nobusawa, Sumihito; Suzuki, Aya; Nagaishi, Masaya; Isoda, Koji; Ikota, Hayato; Yokoo, Hideaki; Hirato, Junko; Nakazato, Yoichi

2013-06-18

70

The effect of WHO reclassification of necrotic anaplastic oligoastrocytomas on incidence and survival in glioblastoma.  

Science.gov (United States)

In 2007 the WHO Classification of Tumours of the Central Nervous System introduced the entity "glioblastoma with oligodendroglioma component" (GBM/OLIGO). This entity accounts for approximately one in every ten new glioblastomas (GBM). In practice, those mixed gliomas with necrosis which were previously diagnosed as anaplastic oligoastrocytoma (AOA) are now designated glioblastoma with oligodendroglioma component. In this way we diagnose as glioblastomas some tumours we already know for which survival is longer than for "classic" glioblastomas. Oncologists should be aware that some newly diagnosed cases of GBM, specifically those called GBM/OLIGO, may behave as AOA. In the next few years we could observe two consequences of this: first, if anaplastic oligoastrocytomas with necrosis are now to be diagnosed as glioblastomas with oligodendroglioma component, obviously it must be expected that the incidence of glioblastoma will increase in these years; second, because these tumours with known longer median OS are now included in the group of glioblastomas, improvement in the survival of patients with glioblastoma in the next few years should be ascribed not only to advances in diagnosis and treatment but also to a semantic factor. PMID:21229290

Marucci, Gianluca

2011-01-13

71

The effect of WHO reclassification of necrotic anaplastic oligoastrocytomas on incidence and survival in glioblastoma.  

UK PubMed Central (United Kingdom)

In 2007 the WHO Classification of Tumours of the Central Nervous System introduced the entity "glioblastoma with oligodendroglioma component" (GBM/OLIGO). This entity accounts for approximately one in every ten new glioblastomas (GBM). In practice, those mixed gliomas with necrosis which were previously diagnosed as anaplastic oligoastrocytoma (AOA) are now designated glioblastoma with oligodendroglioma component. In this way we diagnose as glioblastomas some tumours we already know for which survival is longer than for "classic" glioblastomas. Oncologists should be aware that some newly diagnosed cases of GBM, specifically those called GBM/OLIGO, may behave as AOA. In the next few years we could observe two consequences of this: first, if anaplastic oligoastrocytomas with necrosis are now to be diagnosed as glioblastomas with oligodendroglioma component, obviously it must be expected that the incidence of glioblastoma will increase in these years; second, because these tumours with known longer median OS are now included in the group of glioblastomas, improvement in the survival of patients with glioblastoma in the next few years should be ascribed not only to advances in diagnosis and treatment but also to a semantic factor.

Marucci G

2011-09-01

72

Mutation-specific IDH1 antibody differentiates oligodendrogliomas and oligoastrocytomas from other brain tumors with oligodendroglioma-like morphology.  

Science.gov (United States)

Isocitrate dehydrogenase 1 (IDH1) mutations are frequent in astrocytomas, oligoastrocytomas and oligodendrogliomas. We previously reported the generation of a mutation-specific antibody that specifically detects R132H mutated IDH1 protein (clone H09). Here, we investigate the feasibility of H09 immunohistochemistry to differentiate between oligodendrogliomas/oligoastrocytomas and other tumors with similar morphology. A total of 274 brain tumors presenting with focal or extensive clear cell morphology were investigated. High numbers of H09-positive cases were observed in adult grade II oligodendrogliomas (67 of 74, 91%), grade III oligodendrogliomas (65 of 69, 94%), grade II oligoastrocytomas (11 of 14, 79%) and grade III oligoastrocytomas (10 of 11, 91%). All cases of pediatric oligodendrogliomas (n = 7), neurocytomas (n = 41, 35 central, 4 extraventricular, 2 cerebellar liponeurocytomas), dysembryoplastic neuroepithelial tumors (n = 21), clear cell ependymomas (n = 8), clear cell meningiomas (n = 9) as well as 12 primary glioblastomas with oligodendroglial differentiation and 5 pilocytic astrocytomas with oligodendroglial-like differentiation were negative for H09 immunohistochemistry. Three oligodendrogliomas with neurocytic differentiation had evidence of IDH1/IDH2 mutations either by H09 immunohistochemistry or direct sequencing. We conclude that in tumors with an oligodendroglioma-like morphology, binding of H09 is highly specific for oligodendrogliomas or oligoastrocytomas and substantially helps in the discrimination from other clear cell tumors. Negative H09 immunohistochemistry of an adult oligodendroglioma or oligoastrocytoma should prompt the consideration of other clear cell neoplasms. Further, our observations firmly assign oligodendrogliomas with neurocytic differentiation to the group of oligodendrogliomas and demonstrate that H09 is especially helpful for the difficult discrimination of such lesions from extraventricular neurocytomas. PMID:21069360

Capper, David; Reuss, David; Schittenhelm, Jens; Hartmann, Christian; Bremer, Juliane; Sahm, Felix; Harter, Patrick N; Jeibmann, Astrid; von Deimling, Andreas

2010-11-11

73

Mutation-specific IDH1 antibody differentiates oligodendrogliomas and oligoastrocytomas from other brain tumors with oligodendroglioma-like morphology.  

UK PubMed Central (United Kingdom)

Isocitrate dehydrogenase 1 (IDH1) mutations are frequent in astrocytomas, oligoastrocytomas and oligodendrogliomas. We previously reported the generation of a mutation-specific antibody that specifically detects R132H mutated IDH1 protein (clone H09). Here, we investigate the feasibility of H09 immunohistochemistry to differentiate between oligodendrogliomas/oligoastrocytomas and other tumors with similar morphology. A total of 274 brain tumors presenting with focal or extensive clear cell morphology were investigated. High numbers of H09-positive cases were observed in adult grade II oligodendrogliomas (67 of 74, 91%), grade III oligodendrogliomas (65 of 69, 94%), grade II oligoastrocytomas (11 of 14, 79%) and grade III oligoastrocytomas (10 of 11, 91%). All cases of pediatric oligodendrogliomas (n = 7), neurocytomas (n = 41, 35 central, 4 extraventricular, 2 cerebellar liponeurocytomas), dysembryoplastic neuroepithelial tumors (n = 21), clear cell ependymomas (n = 8), clear cell meningiomas (n = 9) as well as 12 primary glioblastomas with oligodendroglial differentiation and 5 pilocytic astrocytomas with oligodendroglial-like differentiation were negative for H09 immunohistochemistry. Three oligodendrogliomas with neurocytic differentiation had evidence of IDH1/IDH2 mutations either by H09 immunohistochemistry or direct sequencing. We conclude that in tumors with an oligodendroglioma-like morphology, binding of H09 is highly specific for oligodendrogliomas or oligoastrocytomas and substantially helps in the discrimination from other clear cell tumors. Negative H09 immunohistochemistry of an adult oligodendroglioma or oligoastrocytoma should prompt the consideration of other clear cell neoplasms. Further, our observations firmly assign oligodendrogliomas with neurocytic differentiation to the group of oligodendrogliomas and demonstrate that H09 is especially helpful for the difficult discrimination of such lesions from extraventricular neurocytomas.

Capper D; Reuss D; Schittenhelm J; Hartmann C; Bremer J; Sahm F; Harter PN; Jeibmann A; von Deimling A

2011-02-01

74

Extraventricular cerebral anaplastic ependymomas.  

UK PubMed Central (United Kingdom)

BACKGROUND: Anaplastic ependymomas are considered to be uncommon cerebral tumors by most authors. We have had the opportunity to study 34 cases of such lesions in 13 years. METHODS: 34 cases of anaplastic ependymoma operated in different hospitals of Maracaibo, Venezuela, during the period of 1983-1995 were analyzed. Cases of ependymoblastoma were excluded. RESULTS: Adult patients made up most of the present series. All patients harbored supratentorial growths in locations distant from the ventricular system. The microscopic pattern was of limited value to establish prognosis, for there was no constant correlation between the histologic features and tumor relapse; only in sporadic cases in which high cell density and conspicuous mitotic activity were maximally expressed, did tumor relapse occur shortly after removal of the lesion. CONCLUSION: This type of paradoxical behavior being the rule makes all attempts at predicting prognosis in these entities a disappointing task.

Molina OM; Colina JL; Luzardo GD; Mendez OE; Cardozo D; Velasquez HS; Cardozo JJ

1999-06-01

75

Chemotherapy plus radiotherapy versus radiotherapy alone in patients with anaplastic glioma: a systematic review and meta-analysis.  

UK PubMed Central (United Kingdom)

OBJECT: The aim of this study was to answer whether overall survival and progression-free survival are increased in patients with anaplastic glioma who are treated with radiotherapy plus chemotherapy as compared with those who treated with radiotherapy alone. METHODS: Searches of the MEDLINE (PubMed), Embase and Cochrane Library from January 2001 to May 2012 for relevant trials were undertaken. Selected studies based on the following criteria: First, only randomized clinical trial of adjuvant radiotherapy plus chemotherapy versus radiotherapy alone was permitted. Second, overall survival and/or progression-free survival was compared with patients in the studies. Third, cases were medically confirmed of anaplastic glioma. RESULTS: 4 randomized clinical trials were found eligible for this article (involving a total of 963 patients). The meta-analysis showed a significant increase in overall survival in patients treated with radiotherapy plus chemotherapy (HR = 0.84, 95 % CI 0.72-0.98, P = 0.031). CONCLUSIONS: Results of our meta-analysis indicated that adjuvant chemotherapy played a beneficial role in the treatment for anaplastic gliomas. But the role of adjuvant chemotherapy in the treatment for anaplastic astrocytoma or anaplastic oligodendroglioma/anaplastic oligoastrocytoma still needs further large randomized trials to demonstrate.

Zhang L; Wu X; Xu T; Luo C; Qian J; Lu Y

2013-05-01

76

Acantholytic anaplastic Paget's disease.  

UK PubMed Central (United Kingdom)

Classic Paget's disease (PD) can be diagnosed relatively easily by histopathologic examination. 'Anaplastic' variant of this disease is a less-recognized subset that may pose as a diagnostic challenge and pitfall. We describe two cases who presented with scaly erythematous plaques on their nipple/areola. In the first patient, there was no palpable mass and imaging studies were negative. The second case presented with a lesion 5 years after a lumpectomy. Initial shave biopsies revealed histopathologic changes indistinguishable from Bowen's disease with no readily identifiable classic Paget's cells, associated with prominent superficial acantholysis. The neoplastic cells were negative for mucin, GCDFP-15, negative/minimally positive for CEA and strongly positive for CK7 markers. A high-grade ductal carcinoma in situ in the underlying breast was ultimately found in both cases. Anaplastic PD is a rare variant of this disease that histologically mimics Bowen's disease with an associated prominent superficial acantholysis. There is mucin, CEA and GCDFP-15 negativity with positive CK7 reaction. A high index of suspicion along with a complete immunohistochemical panel should be considered in evaluating any Bowenoid neoplasm of the breast skin, particularly in superficial skin shave biopsies along with negative imaging studies and no palpable mass clinically.

Mobini N

2009-03-01

77

Acantholytic anaplastic Paget's disease.  

Science.gov (United States)

Classic Paget's disease (PD) can be diagnosed relatively easily by histopathologic examination. 'Anaplastic' variant of this disease is a less-recognized subset that may pose as a diagnostic challenge and pitfall. We describe two cases who presented with scaly erythematous plaques on their nipple/areola. In the first patient, there was no palpable mass and imaging studies were negative. The second case presented with a lesion 5 years after a lumpectomy. Initial shave biopsies revealed histopathologic changes indistinguishable from Bowen's disease with no readily identifiable classic Paget's cells, associated with prominent superficial acantholysis. The neoplastic cells were negative for mucin, GCDFP-15, negative/minimally positive for CEA and strongly positive for CK7 markers. A high-grade ductal carcinoma in situ in the underlying breast was ultimately found in both cases. Anaplastic PD is a rare variant of this disease that histologically mimics Bowen's disease with an associated prominent superficial acantholysis. There is mucin, CEA and GCDFP-15 negativity with positive CK7 reaction. A high index of suspicion along with a complete immunohistochemical panel should be considered in evaluating any Bowenoid neoplasm of the breast skin, particularly in superficial skin shave biopsies along with negative imaging studies and no palpable mass clinically. PMID:19220635

Mobini, Narciss

2009-03-01

78

Phase II study of accelerated fractionation radiation therapy with carboplatin followed by PCV chemotherapy for the treatment of anaplastic gliomas  

International Nuclear Information System (INIS)

Purpose: To conduct a Phase II one-arm study to evaluate the long-term efficacy and safety of accelerated fractionated radiotherapy combined with i.v. carboplatin for patients with previously untreated anaplastic gliomas. Methods and Materials: Between 1988 and 1992, 90 patients received 1.9-2.0-Gy radiation 3 times a day with 2-h infusions of 33 g/m2 carboplatin for two 5-day cycles separated by 2 weeks. After radiotherapy, patients received procarbazine, lomustine (CCNU), and vincristine (PCV) for 1 year or until the tumor progressed. Results: Ninety patients were evaluable for analysis. Histologically, 69 had anaplastic astrocytoma; 14, anaplastic oligoastrocytoma; and 7, anaplastic oligodendroglioma. Gross total resection was performed in 20 (22%), subtotal resection in 45 (50%), and biopsy in 25 (28%); reoperation (total or subtotal resection) was performed in 50 (56%) patients. A multivariate analysis showed that a younger age (p=0.026), Karnofsky performance score (KPS; p=0.009), and brain necrosis (p=0.0002) were predictive of a better survival. Results from analysis of extent of surgery (biopsy, subtotal resection, gross total resection) approached significance (p=0.058). Radiation dose, irradiated tumor volume, and techniques used (boost and fields) were not significant variables. The median survival (MS) of all anaplastic glioma patients was 28.1 months; for anaplastic astrocytoma patients, MS was 28.7 months and 40.8 months for the combined anaplastic oligodendroglioma/oligoastrocytoma patients. Long-term survival occurred in 25% of anaplastic glioma patients who were alive 8.6 years after treatment was initiated. Treatment-induced necrosis was documented by surgery or autopsy in 19 (21%) patients; 21 (23%) had a mixed pattern of necrosis and tumor; and an additional 13 (14%) patients who did not have surgical or autopsy demonstration of predominant radiation necrosis had magnetic resonance imaging (MRI) evidence of radiation necrosis. Serious clinical neurologic deterioration and/or dementia requiring full-time caregiver attention were observed in 9 (10%) patients. Conclusion: When comparable selection criteria are applied, the rate of MS in this study is inferior to results attainable with current radiation and chemotherapy approaches, although the rates of long-term survival are comparable. Theoretically, patients failing therapy and dying earlier than anticipated may be because of excessive central nervous system (CNS) toxicity resulting from the combination of accelerated fractionated irradiation, intensive carboplatin chemotherapy before each radiation fraction, and postirradiation PCV chemotherapy. On the other hand, patients with treatment-induced necrosis survived significantly longer than patients who did not demonstrate MRI or histologic evidence of necrosis (MS, 106 months vs. 18-33 months)

2002-05-01

79

Anaplastic large cell lymphoma  

Directory of Open Access Journals (Sweden)

Full Text Available Anaplastic large cell lymphoma is defined by a proliferation of large pleomorphic blasts and a constant expression of the CD30 molecule on all neoplastic cells, and has been clinically subdivided into a primary form and a secondary form. We are presenting a case of 14-year-old boy with reddish-livid papules on the skin of right half of trunk that were confluent in the lower end into solid plaque with diameter 20 x 15 cm, and enlarged lymph nodes in axilla and on neck. Lesioned skin biopsy, biopsy of lymph node and bone marrow showed pleomorphic cells, strongly CD30, and ALK positive. In our patient diagnosis was reached on the basis of morphological and immunohistochemical characteristics.

Tiodorovi? Jelica; Lazarevi? Viktor; Stanojevi? Milenko; Jovanovi? Dragan; Tiodorovi?-Živkovi? Danica

2006-01-01

80

Oligodendrogliomas en el Instituto Nacional de Neurología y Neurocirugía: comportamiento biológico en una población definida/ Oligodebdrogliomas the National Institute of Neurology and neurosurgery mvs: biologic behavior in a defined population  

Scientific Electronic Library Online (English)

Full Text Available Abstract in spanish Los oligodendrogliomas corresponden entre el 2 y 5% de los tumores intracranerales siendo diagnosticados en la 4º ó 5º décadas de la vida. La localización más frecuente es el lóbulo frontal. La cirugía es la piedra angular del tratamiento utilizado. Material y métodos: entre enero de 1990 y enero del 2000, 28 pacientes tuvieron diagnóstico de oligodendroglioma. Se revisaron los expedientes en busca de localización, signos y síntomas de presentación, diagnóst (more) ico preoperatorio, estirpe histológica, tipo de tratamiento recibido y complicaciones. Resultados: el sitio de localización más frecuente fue el lóbulo frontal (43%), mientras que el síntoma de presentación principal fue epilepsia (61%). El diagnóstico preoperatorio fue glioma de bajo grado en 39% de los casos. Se realizó resección parcial en 36% de los casos. La cirugía fue realizada por un neurocirujano recibido en la mitad de los casos y por un residente de último año en la otra mitad. Recibió radioterapia fraccionada el 71% de los pacientes, mientras que 21% recibieron quimioterapia. El diagnóstico histopatológico fue oligodendroglioma en 71% de los casos y oligodendroglioma anaplásico en el 29%. La mortalidad operatoria fue del 8%. Conclusiones: los resultados obtenidos en el estudio son similares a los descritos en la literatura. Abstract in english Oligodendrogliomas represent 2 to 5% of the intracraneal tumors. Usually these tumors are diagnosed between the 4th and the 5th decades of life. The most commonly site is the frontal lobe. Surgery is the milestone of the treatment approach. Material and methods: between January 1990 and January 2000, 28 patients had a diagnosis of oligodendroglioma. We looked for localization, signs and symtoms of presentation, peroperative diagnosis, histopathology, type of treatment rec (more) eived and complications. Results: the most frecuent site was the frontal lobe (43%), while the main symptom of presentation were seizures (61%). The preoperative diagnosis with clinical data and imaging was low-grade glioma in 39% of the cases. Partial resection was performed in 36% of the cases being the most prevalent of all treatments. The surgery was performed by neurosurgeons in half the cases and by residents of the last year in the other half. Seventy one porcent of patients were treated with radiotherapy, and 21% with chemotherapy. The hystopathologic diagnosis was oligodendroglioma in 71% and anaplastic oligodendroglioma in 29%. The operative mortality was 8%. Conclusion: our results are similar to the ones described

Moreno-Jiménez, Sergio; Vanegas, Mario Alonso; Bramasco Aviléz, Antonio; García-Pastor, Cuauhtémoc; Terrazo-Lluch, Javier; Tena, Martha; Aguirre, Lucinda

2005-07-01

 
 
 
 
81

Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma Tumor neuroepitelial disembrioplásico diagnosticado originalmente como astrocitoma ou oligodendroglioma  

Directory of Open Access Journals (Sweden)

Full Text Available Dysembryoplastic neuroepithelial tumor (DNT), described in 1988 and introduced in the WHO classification in 1993, affects predominantly children or young adults causing intractable complex partial seizures. Since it is benign and treated with surgical resection, its recognition is important. It has similarities with low-grade gliomas and gangliogliomas, which may recur and become malignant. OBJECTIVES: To investigate whether DNT was previously diagnosed as astrocytoma, oligodendroglioma, or ganglioglioma and to determine its frequency in a series of low-grade glial/glio-neuronal tumors. METHODS: Clinical, radiological, and histological aspects of 58 tumors operated from 1978 to 2008, classified as astrocytomas (32, including 8 pilocytic), oligodendrogliomas (12), gangliogliomas (7), and DNT (7), were reviewed. RESULTS: Four new DNT, one operated before 1993, previously classified as astrocytoma (3) and oligodendroglioma (1), were identified. One DNT diagnosed in 2002 was classified once more as angiocentric glioma. Therefore, 10 DNT (17.2%) were identified. CONCLUSIONS: Clinical-radiological and histopathological correlations have contributed to diagnose the DNT.O tumor neuroepitelial disembrioplásico (DNT), descrito em 1988 e incorporado na classificação da OMS em 1993, acomete predominantemente crianças ou adultos jovens, causando crises convulsivas parciais complexas farmacorresistentes. Como é benigno e tratável com ressecção cirúrgica, seu reconhecimento é importante. Tem semelhanças com gliomas de baixo grau e gangliogliomas, que podem recidivar e malignizar. OBJETIVOS: Investigar se o DNT foi originalmente diagnosticado como astrocitoma, oligodendroglioma ou ganglioglioma e determinar sua frequência numa série de neoplasias gliais/glioneuronais de baixo grau. MÉTODOS: Foram revistos aspectos clínicos, radiológicos e histológicos de 58 neoplasias operadas entre 1978 e 2008, classificadas como astrocitomas (32, sendo 8 pilocíticas), oligodendrogliomas (12), gangliogliomas (7) e DNT (7). RESULTADOS: Foram identificados quatro novos DNT, um operado antes de 1993, originalmente diagnosticado como astrocitoma (3) e oligodendroglioma (1). Um DNT diagnosticado em 2002 foi reclassificado como glioma angiocêntrico. Portanto, 10 DNT (17,2%) foram identificados. CONCLUSÕES: Correlações clínico-radiológicas e histopatológicas contribuíram para o diagnóstico do DNT.

Diego Cassol Dozza; Flávio Freinkel Rodrigues; Leila Chimelli

2012-01-01

82

Meningioma anaplásico/ Anaplastic meningioma  

Scientific Electronic Library Online (English)

Full Text Available Abstract in spanish Se presenta el caso clínico de un paciente que comenzó a presentar cefalea, vértigos, trastornos visuales y pérdida del equilibrio. Mediante la resonancia magnética se visualizó una imagen tumoral parietal izquierda de 3 cm diámetro, de localización extraaxial y contornos lobulados bien definidos, con gran captación no homogénea de contraste, rodeada de extenso edema perilesional. Se realizó angiotomografía, previa a la cirugía, en busca de irrigación y dañ (more) o vascular. Se logró la resección de 95% de la lesión (grado II de Simpson), que incluyó duramadre adyacente infiltrada y respetó el seno longitudinal superior. Los resultados anatomopatológicos confirmaron que se trataba de un meningioma anaplásico de grado III, con criterio de tratamiento coadyuvante. Abstract in english The case report of a patient who began presenting headache, vertigos, visual disorders and loss of balance is presented. By means of the magnetic resonance a left tumoral parietal image of 3 cm diameter was visualized, of extra-axial localization and well defined lobulated contours, with great non-homogeneous zones of contrast, surrounded by extensive perilesional edema. An angiotomography was carried out, previous to the surgery, looking for irrigation and vascular compr (more) omise. The resection of 95% of the lesion was achieved (grade II of Simpson) which included adjacent infiltrated dura madre and preserving the superior longitudinal sinus. Pathological results confirmed that it was an anaplastic meningioma grade III, with criterium for adyuvant treatment.

Piña Tornés, Arlines Alina; Mendoza Montero, Fernando; Oliva Pontón, Francisco José

2013-03-01

83

Meningioma anaplásico Anaplastic meningioma  

Directory of Open Access Journals (Sweden)

Full Text Available Se presenta el caso clínico de un paciente que comenzó a presentar cefalea, vértigos, trastornos visuales y pérdida del equilibrio. Mediante la resonancia magnética se visualizó una imagen tumoral parietal izquierda de 3 cm diámetro, de localización extraaxial y contornos lobulados bien definidos, con gran captación no homogénea de contraste, rodeada de extenso edema perilesional. Se realizó angiotomografía, previa a la cirugía, en busca de irrigación y daño vascular. Se logró la resección de 95% de la lesión (grado II de Simpson), que incluyó duramadre adyacente infiltrada y respetó el seno longitudinal superior. Los resultados anatomopatológicos confirmaron que se trataba de un meningioma anaplásico de grado III, con criterio de tratamiento coadyuvante.The case report of a patient who began presenting headache, vertigos, visual disorders and loss of balance is presented. By means of the magnetic resonance a left tumoral parietal image of 3 cm diameter was visualized, of extra-axial localization and well defined lobulated contours, with great non-homogeneous zones of contrast, surrounded by extensive perilesional edema. An angiotomography was carried out, previous to the surgery, looking for irrigation and vascular compromise. The resection of 95% of the lesion was achieved (grade II of Simpson) which included adjacent infiltrated dura madre and preserving the superior longitudinal sinus. Pathological results confirmed that it was an anaplastic meningioma grade III, with criterium for adyuvant treatment.

Arlines Alina Piña Tornés; Fernando Mendoza Montero; Francisco José Oliva Pontón

2013-01-01

84

Anaplastic thyroid carcinoma.  

Science.gov (United States)

Thyroid cancers represent about 1% of all human cancers. Differentiate thyroid carcinomas (DTCs), papillary and follicular cancers, are the most frequent forms, instead Anaplastic Thyroid Carcinoma (ATC) is estimated to comprise 1-2% of thyroid malignancies and it accounts for 14-39% of thyroid cancer deaths. The annual incidence of ATC is about one to two cases/million, with the overall incidence being higher in Europe (and area of endemic goiter) than in USA. ATC has a more complex genotype than DTCs, with chromosomal aberrations present in 85-100% of cases. A small number of gene mutations have been identified, and there appears to be a progression in mutations acquired during dedifferentiation. The mean survival time is around 6?months from diagnosis an outcome that is frequently not altered by treatment. ATC presents with a rapidly growing fixed and hard neck mass, often metastatic local lymph nodes appreciable on examination and/or vocal paralysis. Symptoms may reflect rapid growth of tumor with local invasion and/or compression. The majority of patients with ATC die from aggressive local regional disease, primarily from upper airway respiratory failure. For this reason, aggressive local therapy is indicated in all patients who can tolerate it. Although rarely possible, complete surgical resection gives the best chance of long-term control and improved survival. Therapy options include surgery, external beam radiation therapy, tracheostomy, chemotherapy, and investigational clinical trials. Multimodal or combination therapy should be useful. In fact, surgical debulking of local tumor, combined with external beam radiation therapy and chemotherapy as neoadjuvant (before surgery) or adjuvant (after surgery) therapy, may prevent death from local airway obstruction and as best may slight prolong survival. Investigational clinical trials in phase I or in phase II are actually in running and they include anti-angiogenetic drugs, multi-kinase inhibitor drugs. PMID:22783225

Taccaliti, Augusto; Silvetti, Francesca; Palmonella, Gioia; Boscaro, Marco

2012-07-05

85

Anaplastic thyroid carcinoma.  

UK PubMed Central (United Kingdom)

Thyroid cancers represent about 1% of all human cancers. Differentiate thyroid carcinomas (DTCs), papillary and follicular cancers, are the most frequent forms, instead Anaplastic Thyroid Carcinoma (ATC) is estimated to comprise 1-2% of thyroid malignancies and it accounts for 14-39% of thyroid cancer deaths. The annual incidence of ATC is about one to two cases/million, with the overall incidence being higher in Europe (and area of endemic goiter) than in USA. ATC has a more complex genotype than DTCs, with chromosomal aberrations present in 85-100% of cases. A small number of gene mutations have been identified, and there appears to be a progression in mutations acquired during dedifferentiation. The mean survival time is around 6?months from diagnosis an outcome that is frequently not altered by treatment. ATC presents with a rapidly growing fixed and hard neck mass, often metastatic local lymph nodes appreciable on examination and/or vocal paralysis. Symptoms may reflect rapid growth of tumor with local invasion and/or compression. The majority of patients with ATC die from aggressive local regional disease, primarily from upper airway respiratory failure. For this reason, aggressive local therapy is indicated in all patients who can tolerate it. Although rarely possible, complete surgical resection gives the best chance of long-term control and improved survival. Therapy options include surgery, external beam radiation therapy, tracheostomy, chemotherapy, and investigational clinical trials. Multimodal or combination therapy should be useful. In fact, surgical debulking of local tumor, combined with external beam radiation therapy and chemotherapy as neoadjuvant (before surgery) or adjuvant (after surgery) therapy, may prevent death from local airway obstruction and as best may slight prolong survival. Investigational clinical trials in phase I or in phase II are actually in running and they include anti-angiogenetic drugs, multi-kinase inhibitor drugs.

Taccaliti A; Silvetti F; Palmonella G; Boscaro M

2012-01-01

86

ATRX loss refines the classification of anaplastic gliomas and identifies a subgroup of IDH mutant astrocytic tumors with better prognosis.  

Science.gov (United States)

Mutation/loss of alpha-thalassemia/mental retardation syndrome X-linked (ATRX) expression has been described in anaplastic gliomas. The present study explored the role of ATRX status in the molecular classification of anaplastic gliomas and its impact on survival in the biomarker cohort of the NOA-04 anaplastic glioma trial. Patients (n = 133) of the NOA-04 trial were analyzed for ATRX expression using immunohistochemistry. ATRX status was correlated with age, histology, isocitrate dehydrogenase (IDH), 1p/19q, alternative lengthening of telomeres (ALT) and O6-methylguanine-DNA methyltransferase (MGMT) status, and the trial efficacy endpoints. Loss of ATRX expression was detected in 45 % of anaplastic astrocytomas (AA), 27 % of anaplastic oligoastrocytomas (AOA) and 10 % of anaplastic oligodendrogliomas (AO). It was mostly restricted to IDH mutant tumors and almost mutually exclusive with 1p/19q co-deletion. The ALT phenotype was significantly correlated with ATRX loss. ATRX and 1p/19q status were used to re-classify AOA: AOA harboring ATRX loss shared a similar clinical course with AA, whereas AOA carrying 1p/19q co-deletion shared a similar course with AO. Accordingly, in a Cox regression model including ATRX and 1p/19q status, histology was no longer significantly associated with time to treatment failure. Survival analysis showed a marked separation of IDH mutant astrocytic tumors into two groups based on ATRX status: tumors with ATRX loss had a significantly better prognosis (median time to treatment failure 55.6 vs. 31.8 months, p = 0.0168, log rank test). ATRX status helps better define the clinically and morphologically mixed group of AOA, since ATRX loss is a hallmark of astrocytic tumors. Furthermore, ATRX loss defines a subgroup of astrocytic tumors with a favorable prognosis. PMID:23904111

Wiestler, Benedikt; Capper, David; Holland-Letz, Tim; Korshunov, Andrey; von Deimling, Andreas; Pfister, Stefan Michael; Platten, Michael; Weller, Michael; Wick, Wolfgang

2013-08-01

87

ATRX loss refines the classification of anaplastic gliomas and identifies a subgroup of IDH mutant astrocytic tumors with better prognosis.  

UK PubMed Central (United Kingdom)

Mutation/loss of alpha-thalassemia/mental retardation syndrome X-linked (ATRX) expression has been described in anaplastic gliomas. The present study explored the role of ATRX status in the molecular classification of anaplastic gliomas and its impact on survival in the biomarker cohort of the NOA-04 anaplastic glioma trial. Patients (n = 133) of the NOA-04 trial were analyzed for ATRX expression using immunohistochemistry. ATRX status was correlated with age, histology, isocitrate dehydrogenase (IDH), 1p/19q, alternative lengthening of telomeres (ALT) and O6-methylguanine-DNA methyltransferase (MGMT) status, and the trial efficacy endpoints. Loss of ATRX expression was detected in 45 % of anaplastic astrocytomas (AA), 27 % of anaplastic oligoastrocytomas (AOA) and 10 % of anaplastic oligodendrogliomas (AO). It was mostly restricted to IDH mutant tumors and almost mutually exclusive with 1p/19q co-deletion. The ALT phenotype was significantly correlated with ATRX loss. ATRX and 1p/19q status were used to re-classify AOA: AOA harboring ATRX loss shared a similar clinical course with AA, whereas AOA carrying 1p/19q co-deletion shared a similar course with AO. Accordingly, in a Cox regression model including ATRX and 1p/19q status, histology was no longer significantly associated with time to treatment failure. Survival analysis showed a marked separation of IDH mutant astrocytic tumors into two groups based on ATRX status: tumors with ATRX loss had a significantly better prognosis (median time to treatment failure 55.6 vs. 31.8 months, p = 0.0168, log rank test). ATRX status helps better define the clinically and morphologically mixed group of AOA, since ATRX loss is a hallmark of astrocytic tumors. Furthermore, ATRX loss defines a subgroup of astrocytic tumors with a favorable prognosis.

Wiestler B; Capper D; Holland-Letz T; Korshunov A; von Deimling A; Pfister SM; Platten M; Weller M; Wick W

2013-09-01

88

Artefacts and Family Resemblance.  

UK PubMed Central (United Kingdom)

I develop in this paper a conception of artefacts based on L. Wittgenstein's idea of family resemblance. My approach peruses the notion of frame, which was invented in cognitive psychology as an operationisable extension of this philosophical idea. Following the metaphor of life-cycle I show how this schematic notion of frame may be filled with the content relevant for artefacts if we consider them from the point of view of their histories. The resulting conception of artefacts provides a new insight into the current debate on artefact categorisation.

Garbacz P

2013-01-01

89

Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma.  

UK PubMed Central (United Kingdom)

UNLABELLED: Dysembryoplastic neuroepithelial tumor (DNT), described in 1988 and introduced in the WHO classification in 1993, affects predominantly children or young adults causing intractable complex partial seizures. Since it is benign and treated with surgical resection, its recognition is important. It has similarities with low-grade gliomas and gangliogliomas, which may recur and become malignant. OBJECTIVES: To investigate whether DNT was previously diagnosed as astrocytoma, oligodendroglioma, or ganglioglioma and to determine its frequency in a series of low-grade glial/glio-neuronal tumors. METHODS: Clinical, radiological, and histological aspects of 58 tumors operated from 1978 to 2008, classified as astrocytomas (32, including 8 pilocytic), oligodendrogliomas (12), gangliogliomas (7), and DNT (7), were reviewed. RESULTS: Four new DNT, one operated before 1993, previously classified as astrocytoma (3) and oligodendroglioma (1), were identified. One DNT diagnosed in 2002 was classified once more as angiocentric glioma. Therefore, 10 DNT (17.2%) were identified. CONCLUSIONS: Clinical-radiological and histopathological correlations have contributed to diagnose the DNT.

Dozza DC; Rodrigues FF; Chimelli L

2012-09-01

90

Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma/ Tumor neuroepitelial disembrioplásico diagnosticado originalmente como astrocitoma ou oligodendroglioma  

Scientific Electronic Library Online (English)

Full Text Available Abstract in portuguese O tumor neuroepitelial disembrioplásico (DNT), descrito em 1988 e incorporado na classificação da OMS em 1993, acomete predominantemente crianças ou adultos jovens, causando crises convulsivas parciais complexas farmacorresistentes. Como é benigno e tratável com ressecção cirúrgica, seu reconhecimento é importante. Tem semelhanças com gliomas de baixo grau e gangliogliomas, que podem recidivar e malignizar. OBJETIVOS: Investigar se o DNT foi originalmente diagn (more) osticado como astrocitoma, oligodendroglioma ou ganglioglioma e determinar sua frequência numa série de neoplasias gliais/glioneuronais de baixo grau. MÉTODOS: Foram revistos aspectos clínicos, radiológicos e histológicos de 58 neoplasias operadas entre 1978 e 2008, classificadas como astrocitomas (32, sendo 8 pilocíticas), oligodendrogliomas (12), gangliogliomas (7) e DNT (7). RESULTADOS: Foram identificados quatro novos DNT, um operado antes de 1993, originalmente diagnosticado como astrocitoma (3) e oligodendroglioma (1). Um DNT diagnosticado em 2002 foi reclassificado como glioma angiocêntrico. Portanto, 10 DNT (17,2%) foram identificados. CONCLUSÕES: Correlações clínico-radiológicas e histopatológicas contribuíram para o diagnóstico do DNT. Abstract in english Dysembryoplastic neuroepithelial tumor (DNT), described in 1988 and introduced in the WHO classification in 1993, affects predominantly children or young adults causing intractable complex partial seizures. Since it is benign and treated with surgical resection, its recognition is important. It has similarities with low-grade gliomas and gangliogliomas, which may recur and become malignant. OBJECTIVES: To investigate whether DNT was previously diagnosed as astrocytoma, ol (more) igodendroglioma, or ganglioglioma and to determine its frequency in a series of low-grade glial/glio-neuronal tumors. METHODS: Clinical, radiological, and histological aspects of 58 tumors operated from 1978 to 2008, classified as astrocytomas (32, including 8 pilocytic), oligodendrogliomas (12), gangliogliomas (7), and DNT (7), were reviewed. RESULTS: Four new DNT, one operated before 1993, previously classified as astrocytoma (3) and oligodendroglioma (1), were identified. One DNT diagnosed in 2002 was classified once more as angiocentric glioma. Therefore, 10 DNT (17.2%) were identified. CONCLUSIONS: Clinical-radiological and histopathological correlations have contributed to diagnose the DNT.

Dozza, Diego Cassol; Rodrigues, Flávio Freinkel; Chimelli, Leila

2012-09-01

91

Investigating the function of Anaplastic Lymphoma Kinase  

Digital Repository Infrastructure Vision for European Research (DRIVER)

Anaplastic Lymphoma Kinase (ALK) was discovered in 1994, as a chromosomal translocation, t(2;5)(p23;q35), often seen in Anaplastic Large Cell Lymphomas (ALCL). Since then ALK has been extensively studied in this disease as well as in different model organisms. Due to its expression pattern within th...

Vernersson Lindahl, Emma

92

Surgical treatment for anaplastic thyroid cancer  

Directory of Open Access Journals (Sweden)

Full Text Available Background: Anaplastic thyroid cancer is relatively rare but extremely aggressive neoplasm. The aim of the present paper was to study the possibility of surgery for anaplastic thyroid cancer. Methods: During 5-year period (from 1998 to 2002) in the Center for endocrine surgery, we found anaplastic thyroid cancer in 65 patients (44 female and 21 male patients) of median age 63 years (range: 37-88 years). Diagnosis was determined on the basis of histological analysis in operated patients or on cytology findings in case of patients who were not operated. Histological analysis confirmed anaplastic transformation of papillary thyroid cancer in 18 cases. Results In 50% patients we performed only fine needle biopsy, and in 37% patients operative biopsy or tumor reduction. We performed radical surgery hemithyroidectomy or total thyroidectomy, in 13% patients with anaplastic thyroid cancer. Thyroid goiter was present in 35% patients longer than a year before diagnosis of anaplastic cancer was made. Conclusion: Possibility of surgery for anaplastic thyroid cancer is very limited. In about one third of patients there were longstanding goiter or histological verified dedifferentiation of papillary thyroid cancer. These patients should have been operated before anaplastic transformation.

Živaljevi? Vladan R.; Dikli? Aleksandar ?.; Krgovi? Ksenija Lj.; Kaži? Milena; Kalezi? Nevena K.; Paunovi? Ivan R.

2003-01-01

93

Anaplastic oligoastrocytoma in Turcot syndrome.  

Science.gov (United States)

Turcot syndrome (TS), a rare variant of hereditary non-polyposis colorectal cancer (HNPCC), is characterized by familial clustering of cancer of the large bowel, extracolonic body sites and brain. It is caused by germline mutations in genes encoding for components of the DNA mismatch repair system. We report a 72 year old woman with anaplastic oligoastrocytoma in the setting of TS. Careful analysis of tumor DNA is required to exclude the chance occurrence of a brain tumor in HNPCC kindreds and increase our understanding of the pathogenesis of the disease. Our case adds to the handful of cases published with detailed molecular data previously. PMID:19495563

Baehring, Joachim; Hui, Pei; Piepmeier, Joseph; Bannykh, Serguei I

2009-06-04

94

Anaplastic oligoastrocytoma in Turcot syndrome.  

UK PubMed Central (United Kingdom)

Turcot syndrome (TS), a rare variant of hereditary non-polyposis colorectal cancer (HNPCC), is characterized by familial clustering of cancer of the large bowel, extracolonic body sites and brain. It is caused by germline mutations in genes encoding for components of the DNA mismatch repair system. We report a 72 year old woman with anaplastic oligoastrocytoma in the setting of TS. Careful analysis of tumor DNA is required to exclude the chance occurrence of a brain tumor in HNPCC kindreds and increase our understanding of the pathogenesis of the disease. Our case adds to the handful of cases published with detailed molecular data previously.

Baehring J; Hui P; Piepmeier J; Bannykh SI

2009-11-01

95

Oligodendrogliomas: estudo anatomopatológico e clínico de 15 casos  

Directory of Open Access Journals (Sweden)

Full Text Available Oligodendrogliomas correspondem a 4-5% dos tumores primários do sistema nervoso central apresentando crescimento infiltrativo e lento. Relatamos os achados anatomopatológicos e clínicos de 15 casos de oligodendrogliomas. Oito pacientes eram do sexo masculino e 7 do feminino. As idades oscilaram entre 17 e 66 anos, apresentando média de 39,73 anos. A sintomatologia apresentada correspondeu ao crescimento expansivo, sendo cefaléia (60%) e crises convulsivas (60%) os sintomas mais frequentes. O lobo frontal (n=6) foi o sítio anatômico mais acometido, seguido pelo parietal (n=2), temporal (n=1) e occipital (n=1). Cinco pacientes foram submetidos a ressecção total do tumor e 10 pacientes a exerese parcial; dentre estes, 3 foram submetidos a radioterapia adjuvante, 1 a quimioterapia e 1 a quimio e radioterapia. Evidenciou-se taxa de recidiva tumoral total de 60% em período médio de 32 meses de acompanhamento. Cinco recidivas tumorais ocorreram nos pacientes submetidos apenas ao tratamento cirúrgico e quatro nos pacientes submetidos a quimio ou radioterapia adjuvante. Estes achados aproximam-se dos encontrados na literatura, auxiliando na compreensão do comportamento biológico deste raro tumor cerebral.

REIS FILHO JORGE SERGIO; MONTEMÓR NETTO MÁRIO RODRIGUES; SLUMINSKY BEATRIZ GARCIA; DELLÉ LINEI AUGUSTA BROLINI; ANTONIUK AFONSO; RAMINA RICARDO; TORRES LUIZ FERNANDO BLEGGI

1999-01-01

96

Molecular analysis of anaplastic oligodendroglial tumors in a prospective randomized study: A report from EORTC study 26951.  

Science.gov (United States)

Recent studies have shown that the clinical outcome of anaplastic oligodendroglial tumors is variable, but also that the histological diagnosis is subject to interobserver variation. We investigated whether the assessment of 1p/19q codeletion, polysomy of chromosome 7, epidermal growth factor receptor (EGFR) gene amplification (EGFR(amp)), and loss of chromosome 10 or 10q offers additional prognostic information to the histological diagnosis and would allow molecular subtyping. For this study, we used the clinical data and tumor samples of the patients included in multicenter prospective phase III European Organisation for Research and Treatment of Cancer (EORTC) study 26951 on the effects of adjuvant procarbazine, chloroethyl cyclohexylnitrosourea (lomustine), and vincristine chemotherapy in anaplastic oligodendroglial tumors. Fluorescence in situ hybridization was used to assess copy number aberrations of chromosome 1p, 19q, 7, 10, and 10q and EGFR. Three different analyses were performed: on all included patients based on local pathology diagnosis, on the patients with confirmed anaplastic oligodendroglial tumors on central pathology review, and on this latter group but after excluding anaplastic oligoastrocytoma (AOA) with necrosis. As a reference set for glioblastoma multiforme (GBM), patients from the prospective randomized phase III study on GBM (EORTC 26981) were used as a benchmark. In 257 of 368 patients, central pathology review confirmed the presence of an anaplastic oligodendroglial tumor. Tumors with combined 1p and 19q loss (1p(loss)19q(loss)) were histopathologically diagnosed as anaplastic oligodendroglioma, were more frequently located in the frontal lobe, and had a better outcome. Anaplastic oligodendroglial tumors with EGFR(amp) were more frequently AOA, were more often localized outside the frontal lobe, and had a survival similar to that for GBM. Survival of patients with AOA harboring necrosis was in a similar range as for GBM, while patients with AOA with only endothelial proliferation had better overall survival. In univariate analyses, all molecular factors except loss of 10q were of prognostic significance, but on multivariate analysis a histopathological diagnosis of AOA, necrosis, and 1p(loss)19q(loss) remained independent prognostic factors. AOA tumors with necrosis are to be considered WHO grade IV tumors (GBM). Of all molecular markers analyzed in this study, especially loss of 1p/19q carried prognostic significance, while the others contributed little prognostic value to classical histology. PMID:19224764

Kouwenhoven, Mathilde C M; Gorlia, Thierry; Kros, Johan M; Ibdaih, Ahmed; Brandes, Alba A; Bromberg, Jacolien E C; Mokhtari, Karima; van Duinen, Sjoerd G; Teepen, Johannes L; Wesseling, Pieter; Vandenbos, Fanny; Grisold, Wolfgang; Sipos, László; Mirimanoff, Rene; Vecht, Charles J; Allgeier, Anouk; Lacombe, Denis; van den Bent, Martin J

2009-12-01

97

Molecular analysis of anaplastic oligodendroglial tumors in a prospective randomized study: A report from EORTC study 26951.  

UK PubMed Central (United Kingdom)

Recent studies have shown that the clinical outcome of anaplastic oligodendroglial tumors is variable, but also that the histological diagnosis is subject to interobserver variation. We investigated whether the assessment of 1p/19q codeletion, polysomy of chromosome 7, epidermal growth factor receptor (EGFR) gene amplification (EGFR(amp)), and loss of chromosome 10 or 10q offers additional prognostic information to the histological diagnosis and would allow molecular subtyping. For this study, we used the clinical data and tumor samples of the patients included in multicenter prospective phase III European Organisation for Research and Treatment of Cancer (EORTC) study 26951 on the effects of adjuvant procarbazine, chloroethyl cyclohexylnitrosourea (lomustine), and vincristine chemotherapy in anaplastic oligodendroglial tumors. Fluorescence in situ hybridization was used to assess copy number aberrations of chromosome 1p, 19q, 7, 10, and 10q and EGFR. Three different analyses were performed: on all included patients based on local pathology diagnosis, on the patients with confirmed anaplastic oligodendroglial tumors on central pathology review, and on this latter group but after excluding anaplastic oligoastrocytoma (AOA) with necrosis. As a reference set for glioblastoma multiforme (GBM), patients from the prospective randomized phase III study on GBM (EORTC 26981) were used as a benchmark. In 257 of 368 patients, central pathology review confirmed the presence of an anaplastic oligodendroglial tumor. Tumors with combined 1p and 19q loss (1p(loss)19q(loss)) were histopathologically diagnosed as anaplastic oligodendroglioma, were more frequently located in the frontal lobe, and had a better outcome. Anaplastic oligodendroglial tumors with EGFR(amp) were more frequently AOA, were more often localized outside the frontal lobe, and had a survival similar to that for GBM. Survival of patients with AOA harboring necrosis was in a similar range as for GBM, while patients with AOA with only endothelial proliferation had better overall survival. In univariate analyses, all molecular factors except loss of 10q were of prognostic significance, but on multivariate analysis a histopathological diagnosis of AOA, necrosis, and 1p(loss)19q(loss) remained independent prognostic factors. AOA tumors with necrosis are to be considered WHO grade IV tumors (GBM). Of all molecular markers analyzed in this study, especially loss of 1p/19q carried prognostic significance, while the others contributed little prognostic value to classical histology.

Kouwenhoven MC; Gorlia T; Kros JM; Ibdaih A; Brandes AA; Bromberg JE; Mokhtari K; van Duinen SG; Teepen JL; Wesseling P; Vandenbos F; Grisold W; Sipos L; Mirimanoff R; Vecht CJ; Allgeier A; Lacombe D; van den Bent MJ

2009-12-01

98

Outcome of oligodendroglioma treatment in the era of modern neuroimaging  

International Nuclear Information System (INIS)

Purpose/Objective: The benefit of routine postoperative radiotherapy for low grade oligodendroglioma remains controversial. Most published series include many patients treated before the availability of CT or MRI scans which allow early diagnosis, guide surgery, detect residual disease, improve radiotherapy, and detect asymptomatic recurrences. The purpose of this analysis is to determine whether observation rather than radiation continues to be an appropriate option for selected patients with the availability of modern neuroimaging. Materials and Methods: 58 patients (age 2-67 years, 6 pts. =2 poor prognostic factor (p=.04). Results: Two and five year actuarial freedom from local progression was 93 +/- 4% and 75% +/- 8% whereas 2 and 5 year overall survival was 94% +/- 3% and 80% +/- 7%. Despite the imbalance of prognostic factors, there was no significant difference whether or not postoperative RT was given. With RT, 2 and 4 year actuarial freedom from progression was 94% +/- 4% and 78% +/- 8%, whereas without RT it was 94% +/- 6% at 2 and 4 years. Similarly, 2 and 4 year actuarial survival was 94% +/- 4% and 78% +/- 8% with RT and was 91% +/- 8% without RT. (5(10)) recurrences were detected radiologically without new or progressive clinical symptoms. Conclusion: These data support the hypothesis that, in the era of modern neuroimaging, the initial observation of good risk patients and immediate irradiation of poor risk patients is an appropriate treatment approach which results in good medium term control and survival for low grade oligodendroglioma patients. A policy of treatment vs. observation based on selected prognostic factors will be tested prospectively in an intergroup trial for low grade glioma histologies

1997-01-01

99

Leptomeningeal metastases from anaplastic thyroid carcinoma  

International Nuclear Information System (INIS)

Anaplastic thyroid carcinoma is an extremely aggressive neoplasm that accounts for 1-3% of all thyroid cancers. ' Most patients have metastatic disease at presentation and die in a short period of time, often with uncontrolled local disease. We report a case of anaplastic thyroid cancer characterised by good response to initial treatment both locally and in distant metastases, and the subsequent development of refractory metastatic disease in an unusual site, the leptomeninges

2000-01-01

100

Resemblance and investment in children.  

UK PubMed Central (United Kingdom)

According to evolutionary explanations men hardly ever are absolutely certain about their biological fatherhood therefore they must seek various sources of information to subjectively establish whether they are the genetic fathers of the children they raise. Apicella and Marlowe (2004) showed that fathers who perceived greater similarity between their children and themselves were willing to invest more resources (e.g., time, money, care) in their offspring presumably because the perceived resemblance indicated to the fathers their genetic relatedness with their children. The present study extended the design of Apicella and Marlowe's original study and included both fathers and mothers as participants. Parents were recruited by a female confederate at the airport and at the railway station in Wroclaw (Poland). Multiple regression analyses showed that perceived resemblance predicted parental investment in the child for both men and women. The fact that mothers' declarations of investment in their children also depended on the perceived resemblance factor is not consistent with evolutionary formulations delineated by Apicella and Marlowe (2004; 2007). Future studies must resolve the issue of whether the resemblance-investment relation in fathers results from men relaying on child's resemblance to themselves as an indicator of their own biological paternity, or whether it results from the more parsimonious phenomenon that people in general are attracted more to other people who are similar to them.

Dolinska B

2013-01-01

 
 
 
 
101

Unexpected expression of intermediate filament protein genes in human oligodendroglioma cell lines  

Energy Technology Data Exchange (ETDEWEB)

From a human oligodendroglioma cell line cDNA library, ten intermediate filament (IF) cDNA clones were isolated. Five clones corresponded to vimentin mRNA, two corresponded to cytokeratin K7 mRNA, and two corresponded to cytokeratin K8 mRNA. One clone encoded a novel IF mRNA. The expression of these and other IF protein genes was examined in five cell lines derived from human oligodendroglioma, astrocytoma and neuroblastoma tumors. Vimentin mRNA and K18 mRNA were expressed in all the cell lines. The K7 and K8 genes were expressed only in the oligodendroglioma cell lines. Surprisingly, nestin mRNA was expressed in the astrocytoma lines and the neuroblastoma line, but was not expressed in the oligodendroglioma lines. These results indicate that oligodendroglioma cell lines express Types I and II cytokeratin genes. This pattern of IF gene expression was different from that of the astrocytoma and neuroblastoma cell lines, which expressed IF genes usually associated with the mature cell types or with differentiating fetal neural precursor cells, i.e. GFAP and neurofilament-L. The results also suggest that the oligodendroglioma cell lines are more epithelial in character and do not reflect the gene expression of mature oligodendrocytes. 46 refs., 8 figs., 2 tabs.

Kashima, Tsuyoshi; Vinters, H.V.; Campagnoni, A.T. [Univ. of California, Los Angeles, CA (United States)

1995-01-01

102

Primary Cutaneous Anaplastic Large-cell Lymphoma.  

UK PubMed Central (United Kingdom)

Since the recognition of the anaplastic large-cell lymphomas in the 1980s, much has been learned about the diagnosis, clinical presentation, and treatment of these malignant conditions. The systemic and primary cutaneous types of anaplastic large cell lymphomas have been differentiated on clinical and immunophenotypical findings, but further research is required to elucidate their exact etiologies and pathogeneses. Primary cutaneous anaplastic large-cell lymphoma has a 95% disease-specific 5-year survival, owing partly to the relatively benign course of the disease and partly to the variety of effective treatments that are available. As with many other oncological diseases, new drugs are continually being tested and developed, with immunotherapy and biological response modifiers showing promise.

Perry E; Karajgikar J; Tabbara IA

2013-10-01

103

BILATERAL THALAMIC ANAPLASTIC GLIOMA : CASE REPORT  

Directory of Open Access Journals (Sweden)

Full Text Available The authors report on patient with bilateral thalamic anaplastic glioma. A 54-year-old man was presented with headache and gradually increasing personality changes. Computed tomography and Magnetic Resonance (MR) of the brain demonstrated bilateral thalamic lesions. MR Spectroscopy of the thalamic lesions showed an increased Choline and creatinin peak and glial tumor was diagnosed radiologically. Stereotactic brain biopsy was performed. Pathological examination revealed anaplastic astrosytoma grade III (World Health Organisation Classification 1993). Patient was referred to radiation therapy. Gliomas of the thalamus are rare and Bilateral Thalamic Anaplastic Gliomas are less defined. Surgical treatment is limited since eloquency of the region and stereotactic biopsy is necessary. The choice of the treatment is radiotherapy.

Halil Ibrahim Sun; Celal Salsini; Ayca Sun; Baran Y?lmaz; Kadriye Agan

2008-01-01

104

Molecular prognostic factors of anaplastic oligodendroglial tumors and its relationship: a single institutional review of 77 patients from China.  

UK PubMed Central (United Kingdom)

The increased chemosensitivity of oligodendroglial tumors has been associated with loss of heterozygosity (LOH) on chromosomes 1p and 19q. Other clinical and molecular factors have also been identified as being prognostic and predictive for treatment outcome. Seventy-seven patients with anaplastic oligodendroglioma (AO) or anaplastic oligoastrocytoma (AOA), treated in Beijing Tiantan Hospital from 2006 through 2008, were reviewed. LOH 1p, LOH 19q, IDH1 mutation, O(6)-methylguanine-DNA methyltransferase (MGMT) promoter methylation, and protein expression level of MGMT, P53, EGFR, and Ki-67 were evaluated. Age at diagnosis, LOH 1p and 19q, IDH1 mutation, P53 expression level, reoperation when progression, and adjuvant chemotherapy were statistically significant factors for overall survival (OS) in univariate analysis. Further multivariate analysis showed that age at diagnosis (P = .010), LOH 1p and 19q (P = .016), IDH1 mutation (P = .011), and reoperation after progression (P = .048) were independent predictors for longer survival in these patients. Nonrandom associations were found between LOH 1p and LOH 19q, MGMT promoter methylation and LOH 1p or 19q, IDH1 mutation and LOH 1p and 19q, IDH1 mutation and MGMT promoter methylation, whereas mutual exclusion was found between MGMT promoter methylation and MGMT expression level. The present study confirmed that age at diagnosis, LOH 1p and 19q, IDH1 mutation, and reoperation after progression were independent significant prognostic factors for patients with anaplastic oligodendroglial tumors. Inter-relationship between LOH 1p, LOH 19q, IDH1 mutation, MGMT promoter methylation, and MGMT expression level were also revealed. Future clinical trials for AO and AOA should consider the molecular alterations of patients.

Li S; Yan C; Huang L; Qiu X; Wang Z; Jiang T

2012-01-01

105

Molecular prognostic factors of anaplastic oligodendroglial tumors and its relationship: a single institutional review of 77 patients from China.  

Science.gov (United States)

The increased chemosensitivity of oligodendroglial tumors has been associated with loss of heterozygosity (LOH) on chromosomes 1p and 19q. Other clinical and molecular factors have also been identified as being prognostic and predictive for treatment outcome. Seventy-seven patients with anaplastic oligodendroglioma (AO) or anaplastic oligoastrocytoma (AOA), treated in Beijing Tiantan Hospital from 2006 through 2008, were reviewed. LOH 1p, LOH 19q, IDH1 mutation, O(6)-methylguanine-DNA methyltransferase (MGMT) promoter methylation, and protein expression level of MGMT, P53, EGFR, and Ki-67 were evaluated. Age at diagnosis, LOH 1p and 19q, IDH1 mutation, P53 expression level, reoperation when progression, and adjuvant chemotherapy were statistically significant factors for overall survival (OS) in univariate analysis. Further multivariate analysis showed that age at diagnosis (P = .010), LOH 1p and 19q (P = .016), IDH1 mutation (P = .011), and reoperation after progression (P = .048) were independent predictors for longer survival in these patients. Nonrandom associations were found between LOH 1p and LOH 19q, MGMT promoter methylation and LOH 1p or 19q, IDH1 mutation and LOH 1p and 19q, IDH1 mutation and MGMT promoter methylation, whereas mutual exclusion was found between MGMT promoter methylation and MGMT expression level. The present study confirmed that age at diagnosis, LOH 1p and 19q, IDH1 mutation, and reoperation after progression were independent significant prognostic factors for patients with anaplastic oligodendroglial tumors. Inter-relationship between LOH 1p, LOH 19q, IDH1 mutation, MGMT promoter methylation, and MGMT expression level were also revealed. Future clinical trials for AO and AOA should consider the molecular alterations of patients. PMID:22039037

Li, Shouwei; Yan, Changxiang; Huang, Lei; Qiu, Xiaoguang; Wang, Zhongcheng; Jiang, Tao

2011-10-27

106

Bevacizumab With or Without Anti-Endoglin Monoclonal Antibody TRC105 in Treating Patients With Recurrent Glioblastoma Multiforme  

Science.gov (United States)

Adult Anaplastic Astrocytoma; Adult Anaplastic Oligodendroglioma; Adult Diffuse Astrocytoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Oligodendroglioma; Recurrent Adult Brain Tumor

2013-09-03

107

Erlotinib Hydrochloride and Isotretinoin in Treating Patients With Recurrent Malignant Glioma  

Science.gov (United States)

Adult Anaplastic Astrocytoma; Adult Anaplastic Oligodendroglioma; Adult Diffuse Astrocytoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Mixed Glioma; Adult Oligodendroglioma; Recurrent Adult Brain Tumor

2013-02-27

108

Pediatric glioblastoma with oligodendroglioma component: Aggressive clinical phenotype with distinct molecular characteristics.  

UK PubMed Central (United Kingdom)

The 2007 World Health Organization classification defined a new variant of glioblastoma (GBM) containing oligodendroglioma foci as GBM with an oligodendroglioma component (GBMO), which shows a favorable clinical outcome compared with "classic" GBM. However, all of the reported cases of GBMO have been adult cases, with no previous reports of pediatric cases. In this report, we demonstrated molecular characteristics of a pediatric GBMO case, showing aggressive clinical behavior with 8-month overall survival. The case showed neither isocitrate dehydrogenase 1/2 genes (IDH1/2) mutation nor 1p/19q co-deletion, a hallmark of oligodendroglioal tumors. In addition, microsatellite instability, leading to the putative mechanism of temozolomide (TMZ) resistance, was frequently detected. Molecular genetic analysis may provide critical prognostic and therapeutic insights, especially for the pediatric glioma containing oligodendroglioma components.

Mizoguchi M; Hata N; Suzuki SO; Fujioka Y; Murata H; Amano T; Nakamizo A; Yoshimoto K; Iwaki T; Sasaki T

2013-03-01

109

Cutis marmorata resemblance after liposuction.  

UK PubMed Central (United Kingdom)

Liposuction is a safe method for the treatment of lipodystrophy. It gives good results in relation to body contours, especially when the superficial and deep layers of the superficial fascia are aspirated. The authors present clinical cases of female patients who underwent liposuction of the abdomen, flanks, and back in which superficial and deep liposuction was used. In the immediate postoperative period, these patients presented a skin pattern of marbled appearance, involving rosy-purplish stains intermingled with other whitish stains on the skin in the areas subjected to surgery and resembling the cutis marmorata described in the literature. Even 1 year after the operation, the stains had not receded. The literature mentions cases of cutaneous necrosis provoked by a temperature increase induced by liposuction cannulas. This trauma said to be the determining factor for local lesions of the subdermal plexus. However, no cases involving lesions of this plexus attributable to mechanical trauma from cannulas are cited. According to several authors, it is important during superficial liposuction to maintain a strip about 1 cm thick under the deep dermis for the preservation of the arterial plexus of the skin. This would avoid the formation of a skin pattern resembling cutis marmorata.

Porto da Rocha R; Porto da Rocha EL; de Souza Pinto EB; Sementilli A; Nakanishi CP

2005-07-01

110

Diagnosis and Treatment of Anaplastic Thyroid Carcinoma  

Digital Repository Infrastructure Vision for European Research (DRIVER)

Anaplastic thyroid carcinoma (ATC) is a poorly differentiated thyroid cancer. It cannot uptake iodine or synthesis thyroglobulin. The incidence is low; about 2% - 5% of thyroid cancer. The peak age incidence is 60 - 70 years and it is more common in females (55% - 77% of all patients). In recent yea...

Patorn Piromchai; Teeraporn Ratanaanekchai; Pornthep Kasemsiri

111

Comparative cytomorphology of pituitary adenomas and oligodendrogliomas in intraoperative crush preparations.  

Science.gov (United States)

Minute fresh tissue fragments from 20 pituitary adenomas and 18 oligodendrogliomas were crushed between two glass slides and stained with hematoxylin and eosin for cytologic examination. These two tumor types displayed distinctive cytologic features that may permit their correct identification. Pituitary adenomas were characterized by single and clustered tumor cells with monomorphic, round or vesicular nuclei that were commonly denuded of cytoplasm. Rare well-preserved tumor cells showed well- or ill-defined, variable and granular cytoplasm. Oligodendrogliomas showed cells with monomorphic or slightly pleomorphic nuclei and scant, fibrillary, wispy cytoplasm, commonly arranged in clusters or around circular and empty spaces. PMID:1523922

Nguyen, G K; Johnson, E S; Mielke, B W

112

Comparative cytomorphology of pituitary adenomas and oligodendrogliomas in intraoperative crush preparations.  

UK PubMed Central (United Kingdom)

Minute fresh tissue fragments from 20 pituitary adenomas and 18 oligodendrogliomas were crushed between two glass slides and stained with hematoxylin and eosin for cytologic examination. These two tumor types displayed distinctive cytologic features that may permit their correct identification. Pituitary adenomas were characterized by single and clustered tumor cells with monomorphic, round or vesicular nuclei that were commonly denuded of cytoplasm. Rare well-preserved tumor cells showed well- or ill-defined, variable and granular cytoplasm. Oligodendrogliomas showed cells with monomorphic or slightly pleomorphic nuclei and scant, fibrillary, wispy cytoplasm, commonly arranged in clusters or around circular and empty spaces.

Nguyen GK; Johnson ES; Mielke BW

1992-09-01

113

Marginal treatment benefit in anaplastic thyroid cancer.  

UK PubMed Central (United Kingdom)

BACKGROUND: Because anaplastic thyroid cancer is a rare malignancy with a high mortality rate, the benefit of multimodality treatment was evaluated. METHODS: Overall survival was determined in the 2742 patients captured by the National Cancer Database who were diagnosed with anaplastic thyroid cancer between 1998 and 2008. Kaplan-Meier analysis and then Cox proportional hazard regression was performed, controlling for patient characteristics and treatment. RESULTS: Only older age (adjusted hazard ratio [AHR] for ?? ?85 years?=?3.43, 95% confidence interval [CI]?=?2.34-5.03; for 75-84 years, AHR?=?2.85, 95% CI?=?1.97-4.11; for 65-74 years, AHR?=?2.20, 95% CI?=?1.53-3.15; for 45-64 years, AHR?=?2.08, 95% CI?=?1.47-2.95) and omission of treatment were associated with greater mortality (omission of surgery: AHR?=?1.79, 95% CI?=?1.61-1.99; omission of radiation therapy: AHR?=?1.56; 95% CI?=?1.41-1.73; and omission of chemotherapy: AHR?=?1.28, 95% CI?=?1.15-1.43). In subgroup analysis of patients with American Joint Committee on Cancer stage IVA, IVB, and IVC anaplastic thyroid cancer, combination therapy with surgery, radiation, and chemotherapy was associated a difference in median survival of months. CONCLUSIONS: Multimodality management of anaplastic thyroid cancer results in a marginal treatment benefit. The poor overall survival of all anaplastic thyroid cancer patients, regardless of treatment, emphasizes the need for informed patients whose preferences are incorporated into treatment decision-making.

Haymart MR; Banerjee M; Yin H; Worden F; Griggs JJ

2013-09-01

114

The Characteristics of Astrocytomas and Oligodendrogliomas Are Caused by Two Distinct and Interchangeable Signaling Formats1  

Digital Repository Infrastructure Vision for European Research (DRIVER)

Chronic platelet-derived growth factor (PDGF) signaling in glial progenitors leads to the formation of oligodendrogliomas in mice, whereas chronic combined Ras and Akt signaling leads to astrocytomas. Different histologies of these tumors imply that the pathways activated by these two oncogenic stim...

Dai, Chengkai; Lyustikman, Yelena; Shih, Alan; Hu, Xiaoyi; Fuller, Gregory N; Rosenblum, Marc; Holland, Eric C

115

GFAP-positive neoplastic astrocytes in spontaneous oligodendrogliomas and mixed gliomas of rats.  

UK PubMed Central (United Kingdom)

It is generally said that neoplastic cells are immunohistochemically negative for glial fibrillary acidic protein (GFAP) in rat spontaneous astrocytomas, and there are no reports describing the existence of GFAP-positive neoplastic astrocytes in rat spontaneous oligodendrogliomas and mixed gliomas which contain neoplastic astrocytes. In the present study, to clarify whether GFAP-positive neoplastic astrocytes exist in rat spontaneous oligodendrogliomas and mixed gliomas or not, immunohistochemical examination was performed on spontaneous oligodendrogliomas (26 cases) and mixed gliomas (5 cases) collected from the carcinogenicity studies and short-term toxicity studies. The neoplastic cells that constitute oligodendrogliomas and mixed gliomas were morphologically classified into five types: round A, round B, round C, spindle, and bizarre. The cells of round A, B, and C types were thought to be neoplastic oligodendrocytes because of their positive immunostainability for Olig2.  The origin of bizarre cells was obscure because they were negative for Olig2, GFAP, and nestin. The spindle cells were considered to be neoplastic astrocytes, because some of them were positive for GFAP or nestin, and GFAP-positive spindle cells could be morphologically distinguished from reactive astrocytes.  In conclusion, the present study clarified for the first time that GFAP-positive neoplastic astrocytes exist in rat spontaneous gliomas.

Nagatani M; Yamakawa S; Saito T; Ando R; Hoshiya T; Tamura K; Uchida K

2013-01-01

116

GFAP-positive neoplastic astrocytes in spontaneous oligodendrogliomas and mixed gliomas of rats.  

Science.gov (United States)

It is generally said that neoplastic cells are immunohistochemically negative for glial fibrillary acidic protein (GFAP) in rat spontaneous astrocytomas, and there are no reports describing the existence of GFAP-positive neoplastic astrocytes in rat spontaneous oligodendrogliomas and mixed gliomas which contain neoplastic astrocytes. In the present study, to clarify whether GFAP-positive neoplastic astrocytes exist in rat spontaneous oligodendrogliomas and mixed gliomas or not, immunohistochemical examination was performed on spontaneous oligodendrogliomas (26 cases) and mixed gliomas (5 cases) collected from the carcinogenicity studies and short-term toxicity studies. The neoplastic cells that constitute oligodendrogliomas and mixed gliomas were morphologically classified into five types: round A, round B, round C, spindle, and bizarre. The cells of round A, B, and C types were thought to be neoplastic oligodendrocytes because of their positive immunostainability for Olig2.  The origin of bizarre cells was obscure because they were negative for Olig2, GFAP, and nestin. The spindle cells were considered to be neoplastic astrocytes, because some of them were positive for GFAP or nestin, and GFAP-positive spindle cells could be morphologically distinguished from reactive astrocytes.  In conclusion, the present study clarified for the first time that GFAP-positive neoplastic astrocytes exist in rat spontaneous gliomas. PMID:23076037

Nagatani, Mariko; Yamakawa, Seiki; Saito, Tsubasa; Ando, Ryo; Hoshiya, Toru; Tamura, Kazutoshi; Uchida, Kazuyuki

2012-10-17

117

Expression of anaplastic lymphoma kinase in Merkel cell carcinomas.  

UK PubMed Central (United Kingdom)

This study examines the presence of anaplastic lymphoma kinase protein and anaplastic lymphoma kinase gene rearrangements in Merkel cell carcinomas. A total of 32 cases of Merkel cell carcinomas and 12 cases of small cell lung carcinomas were analyzed. Immunohistochemistry was performed using 3 different anaplastic lymphoma kinase antibody clones (D5F3, 5A4, and anaplastic lymphoma kinase 1). Tumors were divided into high (intensity score 2-3+ in ?25% of the tumor cells) and low expressors (all other positive expression patterns). Anaplastic lymphoma kinase reactivity in Merkel cell carcinoma was observed in 93.8% (30/32) with clone D5F3, 87.5% (28/32) with clone 5A4, and 12.5% (4/32) with clone anaplastic lymphoma kinase 1. One small cell lung carcinoma (1/12; 8.3%) showed anaplastic lymphoma kinase low expression with clone D5F3. Anaplastic lymphoma kinase high expression was observed in 81.3% (26/32) of the Merkel cell carcinomas with clone D5F3, 71.9% (23/32) with clone 5A4, and none with clone anaplastic lymphoma kinase 1. The specificity of anaplastic lymphoma kinase expression in Merkel cell carcinoma versus small cell lung carcinoma was 91.7% with clone D5F3 and 100% with the clones 5A4 and anaplastic lymphoma kinase 1. Interphase fluorescence in situ hybridization with the anaplastic lymphoma kinase dual-color, break-apart rearrangement probe was performed on 10 randomly selected Merkel cell carcinoma anaplastic lymphoma kinase high expressors. No rearrangement or other cytogenetic aberration of the anaplastic lymphoma kinase gene locus was identified. In conclusion, the anaplastic lymphoma kinase protein was detected with high frequency in Merkel cell carcinomas and was useful in distinguishing Merkel cell carcinoma from small cell lung carcinoma. No correlation with anaplastic lymphoma kinase rearrangement was found. Our findings could have important therapeutic consequences for patients, but the role of anaplastic lymphoma kinase in the pathogenesis of Merkel cell carcinoma needs to be further elucidated.

Filtenborg-Barnkob BE; Bzorek M

2013-08-01

118

Youngest case of third ventricular anaplastic neurocytoma  

Directory of Open Access Journals (Sweden)

Full Text Available A 6-year-old child presented to us with on and off headache and vomiting for 4 months. On examination, there was bilateral papilledema with mild intracranial hypertension but with no neurological deficits. Magnetic resonance imaging (MRI) showed third ventricular mass with obstructive hydrocephalus with possibility of glioma. The patient underwent gross tumor excision and histopathology confirmed anaplastic neurocytoma. The postoperative MRI showed residual disease. The patient treated with adjuvant radiotherapy and temozolamide chemotherapy.

Chinnikatti Shravan; Sharma D; Sharma Kuldeep; Haresh K; Rath G

2010-01-01

119

Anaplastic lymphoma kinase-positive primary cutaneous anaplastic large cell lymphoma--is it a new variant?  

UK PubMed Central (United Kingdom)

Anaplastic large cell cutaneous lymphomas are clinically and pathologically heterogeneous, CD30 + (Ki-1) lymphoproliferative disorders. The importance of anaplastic lymphoma kinase (ALK) positivity is well known in the prognosis of primary systemic anaplastic large cell cutaneous lymphomas; however, the same in primary cutaneous anaplastic large cell cutaneous lymphomas is not much clear. Herein we report a 65-year-old male with an 18-month history of minimally pruritic localized nodulo-plaque lesion over lower back. Histology revealed cutaneous large cell lymphoma and immunohistochemical staining showed positivity for CD30, CD3 and ALK. The role of ALK positivity in pcALCL is discussed in this article.

Kumaran MS; Jithendriya M; Nagaraj P; Tirumalae R; Jayaseelan E

2012-05-01

120

Id4 and FABP7 are preferentially expressed in cells with astrocytic features in oligodendrogliomas and oligoastrocytomas  

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Abstract Background Oligodendroglioma (ODG) and oligoastrocytoma (OAC) are diffusely infiltrating primary brain tumors whose pathogenesis remains unclear. We previously identified a group of genes whose expression was inversely correlated with survival in a cohort of patients with g...

Liang Yu; Bollen Andrew W; Nicholas M Kelly; Gupta Nalin

 
 
 
 
121

Histone 3 lysine 9 trimethylation is differentially associated with isocitrate dehydrogenase mutations in oligodendrogliomas and high-grade astrocytomas.  

Science.gov (United States)

Trimethylation of histone 3 lysine 9 (H3K9me3) is a marker of repressed transcription. Cells transfected with mutant isocitrate dehydrogenase (IDH) show increased methylation of histone lysine residues, including H3K9me3, because of inhibition of histone demethylases by 2-hydroxyglutarate. Here, we evaluated H3K9me3 and its association with IDH mutations in 284 gliomas. Trimethylation of H3K9 was significantly associated with IDH mutations in oligodendrogliomas. Moreover, 72% of World Health Organization grade II and 65% of grade III oligodendrogliomas showed combined H3K9me3 positivity and 1p19q codeletion. In astrocytic tumors, H3K9me3 positivity was found in all grades of tumors; it showed a significant relationship with IDH mutational status in grade II astrocytomas but not in grade III astrocytomas or glioblastomas. Finally, H3K9me3-positive grade II oligodendrogliomas, but not other tumor subtypes, showed improved overall survival compared with H3K9me3-negative cases. These results suggest that repressive trimethylation of H3K9 in gliomas may occur in a context-dependent manner and is associated with IDH mutations in oligodendrogliomas but may be differently regulated in high-grade astrocytic tumors. Furthermore, H3K9me3 may define a subset of grade II oligodendrogliomas with better overall survival. Our results suggest variable roles for IDH mutations in the pathogenesis of oligodendrogliomas versus astrocytic tumors. PMID:23481705

Venneti, Sriram; Felicella, Michelle Madden; Coyne, Thomas; Phillips, Joanna J; Gorovets, Daniel; Huse, Jason T; Kofler, Julia; Lu, Chao; Tihan, Tarik; Sullivan, Lisa M; Santi, Mariarita; Judkins, Alexander R; Perry, Arie; Thompson, Craig B

2013-04-01

122

Histone 3 lysine 9 trimethylation is differentially associated with isocitrate dehydrogenase mutations in oligodendrogliomas and high-grade astrocytomas.  

UK PubMed Central (United Kingdom)

Trimethylation of histone 3 lysine 9 (H3K9me3) is a marker of repressed transcription. Cells transfected with mutant isocitrate dehydrogenase (IDH) show increased methylation of histone lysine residues, including H3K9me3, because of inhibition of histone demethylases by 2-hydroxyglutarate. Here, we evaluated H3K9me3 and its association with IDH mutations in 284 gliomas. Trimethylation of H3K9 was significantly associated with IDH mutations in oligodendrogliomas. Moreover, 72% of World Health Organization grade II and 65% of grade III oligodendrogliomas showed combined H3K9me3 positivity and 1p19q codeletion. In astrocytic tumors, H3K9me3 positivity was found in all grades of tumors; it showed a significant relationship with IDH mutational status in grade II astrocytomas but not in grade III astrocytomas or glioblastomas. Finally, H3K9me3-positive grade II oligodendrogliomas, but not other tumor subtypes, showed improved overall survival compared with H3K9me3-negative cases. These results suggest that repressive trimethylation of H3K9 in gliomas may occur in a context-dependent manner and is associated with IDH mutations in oligodendrogliomas but may be differently regulated in high-grade astrocytic tumors. Furthermore, H3K9me3 may define a subset of grade II oligodendrogliomas with better overall survival. Our results suggest variable roles for IDH mutations in the pathogenesis of oligodendrogliomas versus astrocytic tumors.

Venneti S; Felicella MM; Coyne T; Phillips JJ; Gorovets D; Huse JT; Kofler J; Lu C; Tihan T; Sullivan LM; Santi M; Judkins AR; Perry A; Thompson CB

2013-04-01

123

Proliferating cell nuclear antigen and Ki-67 immunohistochemistry of oligodendrogliomas with special reference to prognosis  

DEFF Research Database (Denmark)

Background. The biologic behavior of oligodendrogliomas is somewhat unpredictable. A supplementary prognostic factor is, therefore, desirable. Methods. Thirty-two pure supratentorial oligodendrogliomas were investigated using proliferating cell nuclear antigen (PCNA) and Ki-67 immunohistochemical analyses. The correlation of PCNA and Ki-67 labeling index (LI) with prognosis were studied, and the correlation of LI with clinical data was evaluated. Results. The PCNA LI had a range of 0-17% (mean, 5.27%; standard deviation [SD] = 4.65), and the Ki-67 LI had a range of 0-29% (mean, 4.19%; SD = 5.66). In general, the PCNA LI seemed to be higher than the Ki-67 LI. The mean survival time was 4.4 years, and 5- and 10-year survival rates were 38% and 19%, respectively. Ki-67 and PCNA staining indicated that patients with a high LI (>3% and >4%, respectively) had a significantly higher mortality, with mean survival time of 23.5 months and 26.2 months, respectively. No significant correlation between LI (or survival) and tumor size, cerebral localization, radiation, resection/biopsy, sex, age, or cytologic atypia was found. Conclusions. The use of Ki-67 and PCNA LI higher than 3% and 4%, respectively, appears reliable as prognostic factors when investigating pure supratentorial oligodendrogliomas.

HEEGAARD, S.; Sommer, Helle MØlgaard

1995-01-01

124

Microvesicles shed by oligodendroglioma cells and rheumatoid synovial fibroblasts contain aggrecanase activity.  

UK PubMed Central (United Kingdom)

Membrane microvesicle shedding is an active process and occurs in viable cells with no signs of apoptosis or necrosis. We report here that microvesicles shed by oligodendroglioma cells contain an 'aggrecanase' activity, cleaving aggrecan at sites previously identified as targets for adamalysin metalloproteinases with disintegrin and thrombospondin domains (ADAMTSs). Degradation was inhibited by EDTA, the metalloproteinase inhibitor GM6001 and by tissue inhibitor of metalloproteinases (TIMP)-3, but not by TIMP-1 or TIMP-2. This inhibitor profile indicates that the shed microvesicles contain aggrecanolytic ADAMTS(s) or related TIMP-3-sensitive metalloproteinase(s). The oligodendroglioma cells were shown to express the three most active aggrecanases, namely Adamts1, Adamts4 and Adamts5, suggesting that one or more of these enzymes may be responsible for the microvesicle activity. Microvesicles shed by rheumatoid synovial fibroblasts similarly degraded aggrecan in a TIMP-3-sensitive manner. Our findings raise the novel possibility that microvesicles may assist oligodendroglioma and rheumatoid synovial fibroblasts to invade through aggrecan-rich extracellular matrices.

Lo Cicero A; Majkowska I; Nagase H; Di Liegro I; Troeberg L

2012-05-01

125

Microvesicles shed by oligodendroglioma cells and rheumatoid synovial fibroblasts contain aggrecanase activity.  

Science.gov (United States)

Membrane microvesicle shedding is an active process and occurs in viable cells with no signs of apoptosis or necrosis. We report here that microvesicles shed by oligodendroglioma cells contain an 'aggrecanase' activity, cleaving aggrecan at sites previously identified as targets for adamalysin metalloproteinases with disintegrin and thrombospondin domains (ADAMTSs). Degradation was inhibited by EDTA, the metalloproteinase inhibitor GM6001 and by tissue inhibitor of metalloproteinases (TIMP)-3, but not by TIMP-1 or TIMP-2. This inhibitor profile indicates that the shed microvesicles contain aggrecanolytic ADAMTS(s) or related TIMP-3-sensitive metalloproteinase(s). The oligodendroglioma cells were shown to express the three most active aggrecanases, namely Adamts1, Adamts4 and Adamts5, suggesting that one or more of these enzymes may be responsible for the microvesicle activity. Microvesicles shed by rheumatoid synovial fibroblasts similarly degraded aggrecan in a TIMP-3-sensitive manner. Our findings raise the novel possibility that microvesicles may assist oligodendroglioma and rheumatoid synovial fibroblasts to invade through aggrecan-rich extracellular matrices. PMID:22406378

Lo Cicero, Alessandra; Majkowska, Iwona; Nagase, Hideaki; Di Liegro, Italia; Troeberg, Linda

2012-03-03

126

Cutaneous intravascular anaplastic large cell lymphoma.  

UK PubMed Central (United Kingdom)

Intravascular lymphoma (IL) is a rare variant of non-Hodgkin lymphoma with a predilection for skin. Most reported cases are large B cell lymphomas. Intravascular anaplastic large cell lymphoma (IALCL) is extremely rare. Retrospective analysis of a case of cutaneous IALCL was performed. Hematoxylin and eosin stained sections and immunohistochemical staining results were analyzed. The patient was a 47-year-old woman who had developed multiple erythematous patches and plaques on her back. The lesions responded well to CHOP (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone) chemotherapy, but relapsed shortly after therapy. The patient was surviving with the disease for eight years but was ultimately lost to follow up. Histopathologically, the neoplasm evolved from IL to extravascular lymphoma. This was showed in biopsies obtained at different stages of the disease. The lymphoma cells stained positively for CD30, CD45, CD3, CD4, CD5 and Ki67, and lacked expression of anaplastic lymphoma kinase (ALK), CD8, CD45RA, CD45RO, CD20, CD79, CD56, perforin and granzyme B. Our results suggest that IALCL represents a distinct subtype of IL and is histopathologically and biologically different from IL with B, NK or T cell phenotype.

Wang L; Li C; Gao T

2011-02-01

127

Cutaneous intravascular anaplastic large cell lymphoma.  

Science.gov (United States)

Intravascular lymphoma (IL) is a rare variant of non-Hodgkin lymphoma with a predilection for skin. Most reported cases are large B cell lymphomas. Intravascular anaplastic large cell lymphoma (IALCL) is extremely rare. Retrospective analysis of a case of cutaneous IALCL was performed. Hematoxylin and eosin stained sections and immunohistochemical staining results were analyzed. The patient was a 47-year-old woman who had developed multiple erythematous patches and plaques on her back. The lesions responded well to CHOP (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone) chemotherapy, but relapsed shortly after therapy. The patient was surviving with the disease for eight years but was ultimately lost to follow up. Histopathologically, the neoplasm evolved from IL to extravascular lymphoma. This was showed in biopsies obtained at different stages of the disease. The lymphoma cells stained positively for CD30, CD45, CD3, CD4, CD5 and Ki67, and lacked expression of anaplastic lymphoma kinase (ALK), CD8, CD45RA, CD45RO, CD20, CD79, CD56, perforin and granzyme B. Our results suggest that IALCL represents a distinct subtype of IL and is histopathologically and biologically different from IL with B, NK or T cell phenotype. PMID:20337769

Wang, Lei; Li, Chengxin; Gao, Tianwen

2011-02-01

128

Brentuximab vedotin in anaplastic large cell lymphoma.  

UK PubMed Central (United Kingdom)

INTRODUCTION: Brentuximab vedotin , a novel anti-CD30 antibody-drug conjugate, delivers a cytotoxic agent into CD30(+) cells. CD30 expression is characteristic of anaplastic large cell lymphoma (ALCL) and Hodgkin lymphoma (HL). AREAS COVERED: We reviewed data on brentuximab vedotin, focusing on ALCL and discuss pharmacology, clinical trials leading to approval and future research directions. Systemic ALCL, 3% of adult NHL, is characterized by large anaplastic CD30(+) cells. The fusion protein NPM-ALK, when present in systemic ALCL, confers better prognosis, although even ALK- patients with IPI score ? 3 are high-risk. For patients with systemic ALCL, 25 - 45% relapse after frontline therapy, and > 50% of patients will relapse following high-dose chemotherapy with autologous stem-cell support. There has been no standard therapy for relapsed/refractory systemic ALCL. Brentuximab vedotin, combines a monoclonal antibody targeted to CD30 with a microtubule disrupting agent and was recently approved for treatment of patients with systemic ALCL that is refractory or relapsed after at least one multiagent chemotherapy regimen. EXPERT OPINION: Brentuximab vedotin provides targeted therapy to CD30(+) lymphomas, including ALCL and HL, with high response rates and manageable toxicity, predominantly myelosuppression and peripheral neuropathy.

Skarbnik AP; Smith MR

2012-05-01

129

Valor pronóstico de los marcadores tumorales de proliferación celular y angiogénesis en los oligodendrogliomas/ The prognostic importance of cell proliferation and angiogenesis tumor markers in oligodendroglioma  

Scientific Electronic Library Online (English)

Full Text Available Abstract in spanish PROPÓSITO: la evolución clínica de los oligodendrogliomas (ODG) es impredecible por los actuales criterios clínicos e histológicos. La presencia de marcadores tumorales del ciclo celular o de la angiogénesis podría predecir el curso clínico de los ODG. El propósito de este estudio ha sido la cuantificación de las expresiones ki67 y CD34 en ODG y su correlación con el grado histológico y la supervivencia. MATERIAL Y MÉTODOS: hemos estudiado 25 ODG. Las inmunoe (more) xpresiones ki67 y CD34 fueron determinadas por técnicas inmunohistoquímicas. Posteriormente se realizó un análisis cuantitativo con un programa de análisis de imagen sistema Qwin. Finalmente, se realizaron las cuantificaciones del ki67 y del CD34, y el análisis estadístico de los resultados. RESULTADOS: el número de núcleos Ki67 positivos se correlacionó con la malignidad de la lesión. El número de núcleos ki67 también se correlacionó con la supervivencia de los pacientes. Por el contrario, el número de microvasos (estructuras CD34 positivas) no se correlacionaba ni con el grado histológico ni con la supervivencia en los ODG. CONCLUSIONES: estos resultados sugieren que el ki67, fundamentalmente el número de núcleos marcados, es un importante indicador diagnóstico y puede ser usado como un parámetro de mal pronóstico en los ODG. Por el contrario, la angiogénesis no es un parámetro pronóstico en los ODG. Abstract in english PURPOSE: The clinical behavior of oligodendroglioma (ODG) is unpredictable using clinical and histological criteria. The present tumor markers related to cell cycle regulation and angiogenesis might improve the possibility of predicting the clinical course of ODG. The aim of this study is the quantification of ki67 and CD34 expression in oligodendrogliomas and its correlation with histological grade and survival. MATERIAL AND METHODS: We studied 25 ODG. The ki67 and CD34 (more) immunoexpression was determined by immunohistochemical techniques. Later, the quantitative study was carried out with a Qwin system image analysis program. Finally, ki67 and CD34 expression was quantified and a statistical analysis of the results was performed. RESULTS: The number of ki67 positive nuclei correlated with the malignancy of the lesion, and with the overall survival. Contrarily, the number of CD34-positive microvessels of OGD showed no significant correlation with neither histological grade nor survival. CONCLUSIONS: The obtained results suggest that ki67, as shown here by the number of immunomarked nuclei, is an important diagnostic indicator, and can be used as a poor prognostic parameter of OGD. On the contrary, angiogenesis, considered here by the CD-34-positive microvessels, is not a prognostic indicator of ODG.

López-Muñiz, A.; Gutiérrez, J. C.; García-Fernández, C.; Herrero, A.

2004-05-01

130

Anaplastic thyroid carcinoma: outcome and prognostic factors  

International Nuclear Information System (INIS)

Purpose: Anaplastic carcinoma of the thyroid has been described as a rapidly progressive disease. We assessed the outcome and prognostic factors in patients with anaplastic thyroid carcinoma at our institution. Materials and Methods: Between 1975 and 1995, 37 patients were seen and treated at our institution with pathologically proven anaplastic carcinoma of the thyroid gland. Patients ranged in age from 49 to 97 years old (median 73 years) and females were represented in a 2:1 ratio. Many patients had history of prior benign thyroid disease (17) or low grade malignancy (6). Other medical illnesses were frequently present in these patients, including 5 with diabetes, 1 scleroderma, 1 sarcoidosis and 1 polycythemia vera. 12 patients had metastatic disease at presentation. 26 patients had locally advanced (T4) disease. The time from diagnosis to treatment was never longer than 1 month. Management was most often with biopsy only (22 patients) and local irradiation (34 patients, median dose 52.5 Gy). 15 patients had primary surgical resection, one of which had negative surgical margins. 11 patients received chemotherapy, 9 with Adriamycin-based regimens. Follow-up ranged from 4 months to 11 years, with a mean of 11 months. Results: 26 patients had a local response, either partial or complete, to their treatment regimen. However, systemic disease was an important cause of failure. 9 patients (24%) survived at least one year from diagnosis; 3 (8%) survived beyond two years. The development of metastases occurred quickly in originally localized disease, at a median of 2 months. Metastases occurred most commonly in the lung (11 of 14 cases), but also occured in brain (2), liver (1), bone (1) and pericardium (1). Performance status, sex, metastatic disease, hyperfractionation, treatment modalities, RT dose, age and response to treatment were assessed as prognostic factors for survival. On univariate analysis, age over 70 (p=.004) and failure to attain a complete response to therapy (p=.013) were significant predictors of poor survival. There was also a trend (p=.07) to worse outcome with dose of 50 Gy or less. Treatments incorporating chemotherapy offered the best median survival (18 months) when compared to no chemotherapy (4 months). There was no significant survival difference between patients treated with primary vs. postoperative chemoirradiation. The only long term survivor (>5 years) was treated with radical surgery with attainment of negative surgical margins followed by radiation and chemotherapy. Conclusions: Anaplastic carcinoma of the thyroid tends to present in a locally advanced or metastatic state in patients who are elderly and have concurrent illness. Its natural history is one of rapid systemic dissemination and short median survivals. Patients who are younger and have better performance status at presentation have a marginally longer median survival, as do those attaining a clinical complete response. Our experience with this disease suggests that adjuvant chemotherapy may confer a survival benefit in these patients. Our current approach in these patients is preoperative chemo-radiation (60 Gy/30 fractions/6 weeks) and surgery

1997-01-01

131

Anaplastic medullary ependymoma presenting as subarachnoid hemorrhage.  

UK PubMed Central (United Kingdom)

A-41-year old man presented with violent thunderclap headache and a bilateral proprioceptive sensibility deficit of the upper limbs. Cerebral CT scan and MRI were negative. Lumbar puncture confirmed subarachnoid hemorrhage (SAH), but cerebral angiography was negative. Three months later, the patient presented with paraparesis, and a thorough work-up revealed a diffuse, anaplastic extramedullary C7-D10 ependymoma with meningeal carcinomatosis considered the source of hemorrhage. The patient went through a D5-D8 laminectomy, temozolomide chemotherapy, and radiotherapy. The situation remained stable for a few months. In this paper, we would like to emphasize that spinal masses should be considered in cases of SAH with negative diagnostic findings for aneurysms or arteriovenous malformation.

Nicastro N; Schnider A; Leemann B

2013-01-01

132

Diagnosis and Treatment of Anaplastic Thyroid Carcinoma  

Directory of Open Access Journals (Sweden)

Full Text Available Anaplastic thyroid carcinoma (ATC) is a poorly differentiated thyroid cancer. It cannot uptake iodine or synthesis thyroglobulin. The incidence is low; about 2% - 5% of thyroid cancer. The peak age incidence is 60 - 70 years and it is more common in females (55% - 77% of all patients). In recent years, the incidence has declined; however, it may be higher in areas of endemic goiter. ATC may occur with a coexisting carcinoma and may represent transformation of a well-differentiated thyroid cancer. Patients typically present with a rapidly growing anterior neck mass and aggressive symptoms. The most reliable tool in detecting thyroid malignancies is fine-needle aspiration cytology (FNAC). Sensitivity of FNAC for thyroid malignancy ranged from 61% to 97.7%. Fine-needle aspiration can diagnose ATC by the demonstration of spindled or giant cells, bizarre neoplastic cells that may be multinucleated, or atypical cells with high mitotic activity. A syncytial pattern is the predominant cellular pattern of anaplastic thyroid carcinoma. Other laboratory tests, including tumor markers (cytokeratin, vimentin, and carcinoembryogenic antigen) are helpful in diagnosis and follow-up of the patients. Multimodality therapy (surgery, external beam radiation, and chemotherapy) is the recommended treatment and it seems to have slightly improved outcomes. The prognosis is not as bad in younger patients with smaller tumors. The most common cause of death is lung metastasis. The mean survival time is less than 6 months from the time of diagnosis. The prompt diagnosis and aggressive treatment are essential modality to achieve optimal outcomes.

Patorn Piromchai; Teeraporn Ratanaanekchai; Pornthep Kasemsiri

2012-01-01

133

Resemblance Argument” and Controversies over Mimesis  

Directory of Open Access Journals (Sweden)

Full Text Available Contrary to the common interpretation of Platonic art that supports the view that it is ontologically and gnoseologically irrelevant because it is mainly defined by mimetic concept as a mere imitation of the material world, and is therefore banished from the Republic, we will offer some different interpretations. Namely, it is possible to show that mimetic principle is not the reason why Plato condemns art, and that the notion of artistic mimesis in fact stems from the metaphysical notion of mimesis as approximation or gradual resemblance to the paradigm. In this case artistic mimesis achieves higher ontological authenticity and imitates the ideal by means of the sensory. Parmenides’ “resemblance argument” may constitute a serious obstacle for the acceptance of, on the one hand, the idea of the relation of resemblance (homoiotes) between Forms and particulars, that is between the paradigm and its image, and on the other hand it may question the idea of approximation in which mimetic principle has metaphysical foundation. However, when Form is seen as a synthetic unity of many things (hen epi pollon), it then represents the right standpoint for the explanation of the phenomena, and it becomes questionable when it is placed on the same level with its exemplars. If the relation of resemblance between the paradigm and its image is determined by the “dynamic”, and not by “symmetric resemblance” in which both parts are on the same level of ontological authenticity, then the view of philosophical mimesis as approximation on which relies artistic mimetic concept is legitimate.

Nives Delija

2004-01-01

134

Prognostic value of perfusion MR imaging in patients with oligodendroglioma: A survival study.  

UK PubMed Central (United Kingdom)

OBJECTIVE: The purpose of this study was to evaluate retrospectively whether cerebral blood volume measurement based on pretreatment perfusion MRI is a prognostic biomarker for survival in patients with oligodendroglioma or mixed oligoastrocytoma. PATIENTS AND METHODS: Between 1998 and 2004, 54 patients (23 females and 31 males), aged 21-73 years, with oligodendroglioma (or mixed tumour) were examined prior to beginning treatment with dynamic susceptibility-weighted contrast (DSC) perfusion MRI during gadolinium first-pass. The relative cerebral blood volume (rCBV) was calculated by dividing the measurement within the tumour by the measurement of the normal-appearing contralateral region. Patients were classified in two groups, grade A and grade B, according to the Saint-Anne Hospital classification and followed-up clinically and by means of MRI until their death or for a minimum of 5 years. Patients were also classified in grade II and grade III-IV, according to the World Health Organisation (WHO) classification, and were analysed with the same methods. Age, sex, treatment, tumour grade, contrast agent uptake, and rCBV were tested using survival curves with Kaplan-Meier's method, and their differences were analysed using the log-rank test. RESULTS: In this population, median survival was 3 years. A rCBV threshold value of 2.2 was validated as a prognostic factor, for survival in these patients with oligodendrogliomas. Age, sex, contrast uptake, and maximum rCBV were found to be prognostic factors in univariate analysis. Multivariate analysis revealed that tumour grade (grade A/grade B), rCBV, age, and sex were prognostic factors independent of the other factors. The tumour grade according to the WHO classification (II versus III-IV) was also detected as an independent prognostic factor. CONCLUSION: Pretreatment rCBV measured by DSC perfusion MRI was found to be a prognostic factor for survival in patients with oligodendroglioma or mixed tumour, by using the Saint-Anne Hospital classification, which separate the IIB from the IIA.

Jiang Z; Le Bas JF; Grand S; Salon C; Pasteris C; Hoffmann D; Bing F; Berger F; Chabardes S; Liu C; Krainik A

2011-03-01

135

Influence of an oligodendrogliomal component on the survival of patients with anaplastic astrocytomas: a report of radiation therapy oncology group 83-02  

International Nuclear Information System (INIS)

Purpose: Seven percent of patients with high grade gliomas enrolled in RTOG 8302 had mixed glial/oligodendroglial elements on central pathology review. It has often been assumed that the most aggressive histologic component of a tumor determines biologic behavior; however in this trial, the survival of patients who had mixed glioblastomas/oligodendrogliomas was significantly longer than that of patients with pure glioblastomas (GBM). We, therefore, evaluated the effect of an oligodendroglial component on the survival of patients who had anaplastic astrocytomas (AAF) treated in the same trial. Material and Methods: 109 patients who had AAF and 24 patients with mixed AAF/oligodendrogliomas (AAF/OL) were enrolled in a Phase I/II trial of randomized dose-escalation hyperfractionated radiotherapy plus BCNU. AAF/OL patients were older and more likely to have more aggressive surgery than AAF patients. Other pretreatment characteristics were balanced between groups as was assigned treatment. Results: The median survival time for AAF was 3.0 years vs 7.3 for AAF/OL (p=0.019). In a multivariate analysis, adjusting for extent of surgical resection and age, an oligodendrogliomal component was an independent prognostic factor for survival. Conclusion: The results support the concept that AAF's with an oligodendroglial component have a better prognosis than pure AAF tumors, similar to the effect seen in patients who have glioblastoma multiforme. This prolonged survival must be taken into consideration in the design and stratification of future trials. Additionally, in contrast to patients who have GBMs, patients who have AAF/OL have the potential for prolonged survival, and, therefore, late sequelae of treatment (both radiation and chemotherapy) must be weighed more heavily in the benefits to risks analysis.

1996-01-01

136

Antagonistic modulation of gliomagenesis by Pax6 and Olig2 in PDGF-induced oligodendroglioma.  

UK PubMed Central (United Kingdom)

Gliomas are aggressive tumors of the central nervous system originating from proliferating neural cells. Regulators of neural stem or progenitor cells biology may thus influence aspects of brain tumorigenesis, such as the maintenance of tumor-propagating potential. We investigated the role of Pax6, a neurogenic transcription factor already suggested as a positive prognostic marker for human gliomas, in a well-characterized in vivo model of PDGF-B-driven oligodendroglioma. In this system, the expression of Pax6 severely impairs tumor propagation by inducing a reduction of cell proliferation and the acquisition of differentiation traits in tumor-initiating cells. The overexpression of Pax6 correlates with a downregulation of Olig2, a bHLH transcription factor that normally antagonizes Pax6 in adult neurogenic niches and that plays a key role in the maintenance of neural stem and progenitor cells. Furthermore, we found that Olig2 is strictly required to maintain the malignancy of oligodendroglioma cells, since its silencing by interfering RNA abrogates tumor propagation. We finally show evidence that this function depends, at least in part, on the silencing of ID4, a dominant negative bHLH protein, whose upregulation follows Olig2 loss. In our model, the upregulation of ID4 mimics the loss of Olig2 in impairing the tumor-propagating potential of glioma cells. Our data, therefore, establish the relevance of physiological regulators of neural stem cell biology in regulating glial tumor malignancy and provide support for their functional interactions in this context.

Appolloni I; Calzolari F; Barilari M; Terrile M; Daga A; Malatesta P

2012-10-01

137

Antagonistic modulation of gliomagenesis by Pax6 and Olig2 in PDGF-induced oligodendroglioma.  

Science.gov (United States)

Gliomas are aggressive tumors of the central nervous system originating from proliferating neural cells. Regulators of neural stem or progenitor cells biology may thus influence aspects of brain tumorigenesis, such as the maintenance of tumor-propagating potential. We investigated the role of Pax6, a neurogenic transcription factor already suggested as a positive prognostic marker for human gliomas, in a well-characterized in vivo model of PDGF-B-driven oligodendroglioma. In this system, the expression of Pax6 severely impairs tumor propagation by inducing a reduction of cell proliferation and the acquisition of differentiation traits in tumor-initiating cells. The overexpression of Pax6 correlates with a downregulation of Olig2, a bHLH transcription factor that normally antagonizes Pax6 in adult neurogenic niches and that plays a key role in the maintenance of neural stem and progenitor cells. Furthermore, we found that Olig2 is strictly required to maintain the malignancy of oligodendroglioma cells, since its silencing by interfering RNA abrogates tumor propagation. We finally show evidence that this function depends, at least in part, on the silencing of ID4, a dominant negative bHLH protein, whose upregulation follows Olig2 loss. In our model, the upregulation of ID4 mimics the loss of Olig2 in impairing the tumor-propagating potential of glioma cells. Our data, therefore, establish the relevance of physiological regulators of neural stem cell biology in regulating glial tumor malignancy and provide support for their functional interactions in this context. PMID:22514120

Appolloni, Irene; Calzolari, Filippo; Barilari, Manuela; Terrile, Marta; Daga, Antonio; Malatesta, Paolo

2012-05-17

138

Oligodendroglioma in a patient with AIDS: case report and review of the literature Oligodendroglioma en un paciente con sida: reporte de caso y revisión de la literatura  

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Full Text Available In the last years, new techniques of neuroimages and histopathological methods have been added to the management of cerebral mass lesions in patients with AIDS. Stereotactic biopsy is necessary when after 14 days of empirical treatment for Toxoplasma gondii encephalitis there is no clinical or neuroradiologic improvement. We report a woman with AIDS who developed a single focal brain lesion on the right frontal lobe. She presented a long history of headache and seizures. After two weeks of empirical treatment for toxoplasma encephalitis without response, a magnetic resonance image with spectroscopy was performed and showed a tumoral pattern with a choline peak, diminished of N-acetyl-aspartate and presence of lactate. A stereotactic biopsy was performed. Histopathological diagnosis was a diffuse oligodendroglioma type A. A microsurgical resection of the tumor was carried out and antiretroviral treatment was started. To date she is in good clinical condition, with undetectable plasma viral load and CD4 T cell count > 200 cell/uL.En los últimos años, las nuevas técnicas de neuroimágenes y diversos métodos de diagnóstico histopatológico se han agregado al manejo clínico de las lesiones de masa cerebral ocupante en los pacientes con sida. La biopsia estereotáxica es necesaria cuando, luego de dos semanas de tratamiento empírico para toxoplasmosis cerebral, no se comprueba mejoría clínica ni neurorradiológica. Presentamos una paciente con sida que desarrolló una lesión cerebral a nivel del lóbulo frontal derecho. Como antecedente refirió una larga historia de cefalea y convulsiones. La resonancia nuclear magnética con espectroscopia de voxel único ubicado a nivel de la lesión mostró un patrón de lesión tumoral con pico de colina, déficit de N-acetil-aspartato y presencia de ácido láctico. La biopsia estereotáxica y el estudio histopatológico permitieron arribar al diagnóstico de oligodendroglioma difuso de tipo A. Se le efectuó resección por microcirugía y tratamiento antirretroviral de alta eficacia. Actualmente la paciente se encuentra en buen estado clínico, con carga viral indetectable y recuento de linfocitos T CD4 + > de 200 cél/uL.

Marcelo E. Corti; Claudio Yampolsky; Humberto Metta; Mario Valerga; Gustavo Sevlever; Andrés Capizzano

2004-01-01

139

Histopathological and immunohistochemical profile in anaplastic gangliogliomas.  

UK PubMed Central (United Kingdom)

BACKGROUND: The anaplastic ganglioglioma (AG) is the high-grade counterpart of ganglioglioma, a rare mixed tumor composed of neuronal/ganglion and glial cells. MATERIALS AND METHODS: We describe the histopathology and immunohistochemistry in 7 cases of AG and correlate them with the clinical and radiological features. RESULTS: Our AG patients correspond to 2.5% of the central nervous system tumor patients evaluated in our institution. The mean age at presentation was 25.7 years, with a male predominance. The most common clinical presentation was generalized tonic-clonic seizures (3/7 cases), in correlation with frequent cortical/subcortical location (6/7 cases). Histopathologically, all our cases showed high-grade features in glial (glial fibrillary acid protein-positive) and neuron-ganglion cells (synaptophysin, PGP-9.5, neurofilament, NSE and CD56-positive), as well as moderate cellularity, frequent mitotic figures and a Ki-67 labeling index >5%. All our patients had poor survival. CONCLUSION: We found that a typical histopathological and immunohistochemical profile is constant and can be useful in early diagnosis of these aggressive neoplasms.

Romero-Rojas AE; Diaz-Perez JA; Chinchilla-Olaya SI; Amaro D; Lozano-Castillo A; Restrepo-Escobar LI

2013-09-01

140

Carcinoma indiferenciado de tireóide Anaplastic thyroid carcinoma  

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Full Text Available O carcinoma diferenciado de tireóide, papilífero ou folicular, usualmente tem um curso relativo benigno após a tireoidectomia total e ablação de remanescentes tireoideanos com 131I. Em contraste, o carcinoma anaplásico de tireóide ou carcinoma indiferenciado de tireóide, também derivado do epitélio folicular tireoideano, é uma das neoplasias humanas mais agressivas, que perdeu a maioria ou todas as características do tecido de origem. Crescimento tumoral rápido é um presságio de mortalidade precoce a menos que se institua tratamento combinado agressivo. Não dispomos ainda de um tratamento que leva à cura definitiva para a maioria dos pacientes. A melhor conduta se constitui de um tratamento cirúrgico agressivo associado com a combinação de novos agentes quimioterápicos e radioterapia externa.Well-differentiated thyroid carcinoma (TC), as papillary and follicular carcinoma, usually follows a relatively benign course after total thyroidectomy and thyroid remnant ablation with 131I. In contrast, anaplastic TC or undiferentiated TC, also derived from the thyroid follicular epithelium, refers to one of the more aggressive human malignancies, which have lost most or all characteristics of the tissue from which it originated. Rapid tumor growth presages early mortality unless combined therapy is aggressively pursued. Definitive curative approach does not exist for most patients. The best approach is still aggressive surgery combined with the associated use of new chemotherapies associated with local external beam radiotherapy.

Gisah A. de Carvalho; Hans Graf

2005-01-01

 
 
 
 
141

Shared allelic losses on chromosomes 1p and 19q suggest a common origin of oligodendroglioma and oligoastrocytoma  

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Loss of heterozygosity (LOH) in specific chromosomal regions, which are likely to harbor tumor suppressor genes, has been associated with human gliomas. In this study we have analyzed astrocytic and oligodendroglial tumors for LOH on chromosomes 1 and 19. By microsatellite analysis LOH was found on chromosome arm 1p in 6/15 oligodendrogliomas WHO grade II and III, 12/25 oligoastrocytomas WHO grade II and III, 6/79 glioblastomas WHO grade IV, 5/44 astrocytomas WHO grade II and III and 0/23 pilocystic astrocytomas WHO grade I. The high incidence of LOH on chromosome arm 1p in oligodendrogliomas and oligoastrocytomas indicates that a putative tumor suppressor gene in this region is involved in the formation of gliomas with oligodendroglial features. Furthermore, the frequent involvement of chromosome arm 1p in oligodendrogliomas and oligoastrocytomas, but not in astrocytomas, suggests that genetically oligoastrocytoma is more similar to oligodendroglioma than to astrocytoma. In order to support this hypothesis, oligodendroglial and astrocytic areas in three mixed oligoastrocytomas were examined differentially for LOH 1p and for LOH 19q, the second genetic region believed to be affected in these tumors. All three tumors had LOH of 1p and LOH of 19q in both areas of oligodendroglial and of astrocytic differentiation. These findings show that the astrocytic and oligodendroglial portions of oligoastrocytoma share molecular genetic features and probably are of monoclonal origin. 32 refs., 2 figs., 1 tab.

Kraus, J.A.; Koopmann, J.; Kaskel, P. [Universitaetskliniken Bonn (Germany)] [and others

1995-01-01

142

Fractionated stereotactic radiation therapy in the management of primary oligodendroglioma and oligoastrocytoma  

International Nuclear Information System (INIS)

Purpose: To retrospectively analyze the outcomes and benefits from radiation therapy (RT) as a component of multimodal treatment for oligodendroglioma and oligoastrocytoma, assessing local control and survival rates and evaluating prognostic factors. Methods and Materials: We retrospectively reviewed 56 adult patients with supratentorial oligodendroglioma or oligoastrocytoma treated at our institution from January 1990 to December 2003 with fractionated stereotactic RT (FSRT). Results: Fractionated stereotactic RT was well tolerated in all patients, without side effects. Median survival and progression-free survival calculated from the initiation of radiotherapy were 48 months (range, 2-133 months) and 38 months (range, 2-132 months), respectively. Progression-free survival rates after radiation were 89% at 1 year and 52% at 5 years. Of 26 recurrences, 92% developed in field. With regard to histology, overall survival rates in the World Health Organization (WHO) Grade II group were 89% and 74% at 5 and 10 years, respectively. In patients with WHO Grade III tumors, overall survival rates at 5 and 10 years were 69% and 46%, respectively. No prognosticators could be identified for median survival and progression-free survival after radiotherapy. Median overall survival calculated from primary diagnosis was 77.5 months (range, 3-214 months). The Cox regression multivariate analysis for age and neurologic symptoms showed a significance of p = 0.003 for age and p = 0.037 for the presence of neurologic symptoms on overall survival since primary diagnosis. Conclusions: Commonly, conventional conformal RT is applied in the treatment of brain tumors. In FSRT, the tumor volume can be irradiated with high doses, sparing volume of normal brain tissue. Our data are in accordance with survival times found in the literature. Ninety-two percent of all recurrences occurred within the defined target volume, confirming that reduction of the RT portals by the use of FSRT does not lead to an increased rate of recurrences at the field border or out of field. Fractionated stereotactic RT can therefore be implemented as an effective and safe modality in the therapy of primary oligodendroglioma and oligoastrocytoma

2005-07-01

143

Uterine cervical tubulosquamous polyp resembling a penis.  

UK PubMed Central (United Kingdom)

This case report describes a tubulosquamous polyp resembling a penis in the uterine cervix. A 34-yr-old, gravida 0, para 0, woman showed an 18 × 8 × 5 mm polypoid lesion in the uterine ectocervix. The polyp had a penis-like appearance; the tip looked like glans penis and the middle portion resembled the shaft of the penis. Its surface was covered by squamous epithelium, and tissues resembling those of a urethra, corpus spongiosum penis, and external orifice urethra were observed. Foreskin-like tissues were also observed, although a corpus cavernosum penis was not seen. Skene glands and Cowper glands were also observed. Immunohistochemically, Skene glands and the urethra-like epithelium were focally positive for prostate-specific antigen and/or prostatic acid proteins. Histologically and immunohistochemically, the polypoid lesion overlapped with a tubulosquamous polyp of the vagina and ectopic prostatic tissue of the uterine cervix and encompassed these lesions in the lower female genital tract. The most likely theory of histogenesis is a developmental anomaly and misplacement of Skene glands.

Fukunaga M

2013-07-01

144

Uterine cervical tubulosquamous polyp resembling a penis.  

Science.gov (United States)

This case report describes a tubulosquamous polyp resembling a penis in the uterine cervix. A 34-yr-old, gravida 0, para 0, woman showed an 18 × 8 × 5 mm polypoid lesion in the uterine ectocervix. The polyp had a penis-like appearance; the tip looked like glans penis and the middle portion resembled the shaft of the penis. Its surface was covered by squamous epithelium, and tissues resembling those of a urethra, corpus spongiosum penis, and external orifice urethra were observed. Foreskin-like tissues were also observed, although a corpus cavernosum penis was not seen. Skene glands and Cowper glands were also observed. Immunohistochemically, Skene glands and the urethra-like epithelium were focally positive for prostate-specific antigen and/or prostatic acid proteins. Histologically and immunohistochemically, the polypoid lesion overlapped with a tubulosquamous polyp of the vagina and ectopic prostatic tissue of the uterine cervix and encompassed these lesions in the lower female genital tract. The most likely theory of histogenesis is a developmental anomaly and misplacement of Skene glands. PMID:23722517

Fukunaga, Masaharu

2013-07-01

145

Anaplastic large cell lymphoma, ALK-negative.  

Science.gov (United States)

Anaplastic large cell lymphoma (ALCL), anaplastic lymphoma kinase (ALK)-negative (ALCL-ALK-) is a provisional entity in the WHO 2008 Classification that represents 2-3% of NHL and 12% of T-cell NHL. No particular risk factor has been clearly identified for ALCL, but a recent study showed an odds ratio of 18 for ALCL associated with breast implants. Usually, the architecture of involved organs is eroded by solid, cohesive sheets of neoplastic cells, with peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) and classical Hodgkin lymphoma being the main differential diagnoses. In this regard, staining for PAX5 and CD30 is useful. Translocations involving ALK are absent, TCR genes are clonally rearranged. CGH and GEP studies suggest a tendency of ALCL-ALK- to differ both from PTCL-NOS and from ALCL-ALK+. Patients with ALCL-ALK- are usually adults with a median age of 54-61 years, and a male-to-female ratio of 0.9. At presentation, ALCL-ALK- is often in III-IV stage, with B symptoms, high International Prognostic Index score, high lactate dehydrogenase serum levels, and an aggressive course. ALCL-ALK- presents with lymph node involvement in ?50% of cases; extranodal spread (20%) is less common. Staging work-up for ALCL-ALK- is similar to that routinely used for nodal NHL. Overall prognosis is poor, with a 5-year OS of 30-49%, which is significantly worse when compared to OS reported in patients with ALCL-ALK+ (5-year: 70-86%). Patients with systemic ALCL exhibit a significantly better survival compared with patients with PTCL-NOS, with a 5-year OS of 51% and 32%, respectively. Age, PIT scoring system, ?2-microglobulin, and bone marrow infiltration are the main prognostic factors. The expression of proteins involved in the regulation of apoptosis (caspase 3, Bcl-2, PI9) and of CD56 is related to clinical outcome. ALCL-ALK- is generally responsive to doxorubicin-containing chemotherapy, but relapses are frequent. CHOP is the most commonly used regimen to treat systemic ALCL with complete remission rates of 56%, and a 10-year DFS of 28%. Encouraging results have been reported with more intensive chemotherapy regimens. The addition of etoposide improved outcome. Alemtuxumab-CHOP regimen was associated with excellent remission rate but increased toxicity. The role of high-dose chemotherapy supported by ASCT has not been investigated in a trial of exclusively ALCL patients. When used in first remission, it was associated with a 5-year PFS of 64%. High-dose chemotherapy with ASCT is the standard therapeutic option for patients with relapsed or refractory disease. The role of allogeneic transplantation in patients with relapsed/refractory ALCL remains to be defined but there are data to support the contention that a graft-versus-lymphoma effect does exist. Myeloablative conditioning has been associated with 5-year PFS and OS of 40% and 41%, respectively, but a 5-year TRM of 33% was reported. Allo-SCT can be an option for relapsed/refractory ALCL in younger patients, preferably in the setting of a clinical trial. Pralatrexate, anti-CD30 monoclonal antibodies, brentuximab vedotin (SGN-35) in particular, (131)I-anti-CD45 radioantibody, yttrium-anti-CD25 radioimmunoconjugates, histone deacetylase inhibitors, bortezomib, gemcitabine, vorinostat, lenalidomide, and their combinations represent the most appealing chemotherapy and/or targeted agents to be investigated in future trials. PMID:22789917

Ferreri, Andrés J M; Govi, Silvia; Pileri, Stefano A; Savage, Kerry J

2012-07-11

146

Anaplastic Pancreatic Carcinoma. A Case Report and Review of Literature  

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Full Text Available CONTEXT: Anaplastic pancreatic carcinoma is an aggressive neoplasm with survival measurable in weeks. It presents as a large cystic mass with loco-regional and distant spread. Three histological types have been described: pleomorphic, spindle cell and sarcomatoid. CASE REPORT: We describe the case of a 74-year-old woman with pleomorphic anaplastic carcinoma of the pancreas diagnosed after laparoscopic biopsy. The patient had a rapid downhill course with progression of the disease and demise within 4 weeks after diagnostic laparoscopy. CONCLUSION: Due to the rapid spread of the disease, no effective cure exists for these tumors. A brief review of the histological and radiological findings and the possible mechanisms of the pathogenesis of anaplastic tumors is included in the discussion.

Chadha MK; LeVea C; Javle M; Kuvshinoff B; Vijaykumar R; Iyer R

2004-01-01

147

Anaplastic lymphoma kinase status in rhabdomyosarcomas.  

UK PubMed Central (United Kingdom)

Rhabdomyosarcoma is a rare soft tissue sarcoma that typically affects children, adolescents, and young adults. Despite treatment via a multidisciplinary approach, the prognosis of advance-stage rhabdomyosarcomas remains poor, and a new treatment strategy is needed. Anaplastic lymphoma kinase (ALK) is a receptor tyrosine kinase that is a potential target for specific inhibitors. In this study, we investigated 116 rhabdomyosarcomas using a polymer-based ALK immunostaining method and correlated the results with clinicopathological parameters. In addition, we examined ALK status using dual-color fluorescence in situ hybridization, PCR, and sequencing. In immunohistochemical analysis, ALK was detected in 2 (6%) of 33 embryonal rhabdomyosarcomas, 42 (69%) of 61 alveolar rhabdomyosarcomas, and 0 (0%) of 22 other subtypes, including pleomorphic, adult-spindle-cell/sclerosing, and epithelioid variants. Compared with ALK-negative alveolar rhabdomyosarcomas, ALK-positive ones are presented with metastatic spread more frequently and showed a greater extent of myogenin reactivity. Overall survival was not associated with ALK expression. FOXO1 rearrangement was significantly associated with ALK immunoreactivity. The median ALK copy number was greater in ALK-positive tumors than in ALK-negative tumors. Most (93%) cases tested showed no selective increase in the ALK gene dosage. ALK selective amplification and low-level selective gain were noted in one and three cases, respectively. Further, a high-polysomy pattern (?4 ALK copies in ?40% of cells) was observed in seven cases. A significant increase in the ALK copy number was exclusive to the ALK-immunopositive cohort, but it was uncommon, accounting for only 30% of the 37 ALK-positive rhabdomyosarcomas. ALK gene rearrangement was not observed in either cohort, while an ALK somatic mutation (I1277T) was found in one ALK-negative embryonal case. Although it remains controversial whether ALK expression without gene rearrangement is therapeutically relevant, this comprehensive analysis may help future studies on the utility of ALK-targeted therapy for patients with rhabdomyosarcoma.

Yoshida A; Shibata T; Wakai S; Ushiku T; Tsuta K; Fukayama M; Makimoto A; Furuta K; Tsuda H

2013-06-01

148

New targeted therapies for anaplastic thyroid cancer.  

UK PubMed Central (United Kingdom)

Anaplastic thyroid cancer (ATC) is often incurable because it doesn't respond to radioiodine, radiotherapy or chemotherapy, and new therapeutic approaches are needed. Peroxisome proliferator-activated receptor-gamma (PPARg) gene and protein are present in ATC cells, and PPARg ligands inhibit cell proliferation, induce apoptosis, and also down regulate the invasive potential of ATC cells. Also, inhibitors of the Aurora serine/threonine kinases have antineoplastic effect on ATC cells in vitro and on ATC xenografts. Tyrosine kinases inhibitors are actually under evaluation for the treatment of ATC, for example imanitib or sorafenib. Other studies have focused on evaluating antiangiogenic agents for treatment of ATC. These agents include: combretastatin A4 phosphate, aplidin, PTK787/ZK222584, and human VEGF monoclonal antibodies (bevacizumab, cetuximab). Small-molecule adenosine triphosphate (ATP) competitive inhibitors directed intracellularly at epidermal growth factor receptor (EGFR)'s tyrosine kinase, such as erlotinib, or gefitinib are also under evaluation. The development of drugs that have multiple therapeutic targets and the utilization of multiple cancer-targeting agents are both emerging strategies for ATC treatment. For example, a preclinical study evaluated the activity of a dual inhibitor of EGFR and vascular endothelial growth factor (VEGF), NVP-AEE788, alone and in combination with paclitaxel for the treatment of ATC. Even if new therapeutic approaches against ATC are under development, more research is needed to finally identify therapies able to control and to cure this disease. The possibility of testing the sensitivity of primary ATC cells from each subject to different drugs could increase the effectiveness of the treatment in the next future.

Antonelli A; Fallahi P; Ulisse S; Ferrari SM; Minuto M; Saraceno G; Santini F; Mazzi V; D'Armiento M; Miccoli P

2012-01-01

149

Anaplastic carcinoma of cervix; rationale of para-aortic irradiation  

Energy Technology Data Exchange (ETDEWEB)

Carcinoma of cervix is the most common malignancy seen in the Department of Radiotherapy at Christian Medical College and Hospital, Vellore, in Southern India, and 12% are of anaplastic type. Eighty-three patients between 1968 and 1970 with anaplastic carcinoma of cervix were evaluated for the effect of pelvic irradiation as well as for pelvic and prophylactic para-aortic irradiation. The early reactions were slightly higher in para-aortic group which could be controlled with simple medication. A favourable long-term survival in the para-aortic group probably suggests the advantage of nodal irradiation.

Roul, R.K.; Singh, S.P.; Jayachandran, C.A. (Christian Medical Coll., Vellore (India))

1981-02-01

150

Multiple courses of stereotactic re-irradiation in recurrent oligodendroglioma: a case report  

Directory of Open Access Journals (Sweden)

Full Text Available Abstract Introduction High grade gliomas are an insidious disease associated with an extremely poor prognosis. The role of re-irradiation for recurrent gliomas is unclear but several retrospective studies have indicated mild toxicity and modest outcomes with this regimen. With subsequent progression, it is unclear what options remain and more radiotherapy is rarely offered for fear of surpassing normal central nervous system tissue tolerance and causing significant side effects without significant benefit. Case presentation In this report, we describe a 37-year-old Caucasian male initially diagnosed with a grade IV oligodendroglioma, who received multiple courses of re-irradiation and experienced a survival of 10 years with minimal cognitive or neurologic deficits. Conclusion Significant toxicity with multiple courses of radiation does not always occur. Re-irradiation should be considered in a salvage setting.

Fogh Shannon; Glass Charles; Andrews David W; Werner-Wasik Maria

2011-01-01

151

Tomografia computadorizada e ressonância magnética nos oligodendrogliomas: correlação clínica e patológica  

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Full Text Available Oligodendrogliomas são neoplasias do tecido neuroepitelial glial originárias de oligodendrócitos. São tumores infreqüentes, responsáveis por cerca de 4% a 7% das neoplasias primárias do cérebro, predominantemente supratentoriais. O presente trabalho consistiu na avaliação dos achados de imagem pré-operatória em tomografia computadorizada e ressonância magnética e correlação clínica e patológica, levando-se em consideração a presença de tumores puros ou mistos, com componente astrocitário e o seu grau de anaplasia. O aspecto mais freqüente foi o de lesão hipodensa na tomografia computadorizada ou com hipossinal em T1 e hipersinal em T2 na ressonância magnética, podendo ter componente cístico, com pouco edema ao redor, apresentando calcificações, quase sempre grosseiras, em dois terços dos casos. Reforço após contraste ocorre em 80% dos casos, na maioria discreto.

Simão Marcelo Novelino; Simão Gustavo Novelino; Santos Antônio Carlos dos; Trad Clóvis Simão

2001-01-01

152

Valproic acid induces the glutamate transporter excitatory amino acid transporter-3 in human oligodendroglioma cells.  

UK PubMed Central (United Kingdom)

Glutamate transport in early, undifferentiated oligodendrocytic precursors has not been characterized thus far. Here we show that human oligodendroglioma Hs683 cells are not endowed with EAAT-dependent anionic amino acid transport. However, in these cells, but not in U373 human glioblastoma cells, valproic acid (VPA), an inhibitor of histone deacetylases, markedly induces SLC1A1 mRNA, which encodes for the glutamate transporter EAAT3. The effect is detectable after 8h and persists up to 120h of treatment. EAAT3 protein increase becomes detectable after 24h of treatment and reaches its maximum after 72-96h, when it is eightfold more abundant than control. The initial influx of d-aspartate increases in parallel, exhibiting the typical features of an EAAT3-mediated process. SLC1A1 mRNA induction is associated with the increased expression of PDGFRA mRNA (+150%), a marker of early oligodendrocyte precursor cells, while the expression of GFAP, CNP and TUBB3 remains unchanged. Short term experiments have indicated that the VPA effect is shared by trichostatin A, another inhibitor of histone deacetylases. On the contrary, EAAT3 induction is neither prevented by inhibitors of mitogen-activated protein kinases nor triggered by a prolonged incubation with lithium, thus excluding a role for the GSK3?/?-catenin pathway. Thus, the VPA-dependent induction of the glutamate transporter EAAT3 in human oligodendroglioma cells likely occurs through an epigenetic mechanism and may represent an early indicator of commitment to oligodendrocytic differentiation.

Bianchi MG; Franchi-Gazzola R; Reia L; Allegri M; Uggeri J; Chiu M; Sala R; Bussolati O

2012-12-01

153

Valproic acid induces the glutamate transporter excitatory amino acid transporter-3 in human oligodendroglioma cells.  

Science.gov (United States)

Glutamate transport in early, undifferentiated oligodendrocytic precursors has not been characterized thus far. Here we show that human oligodendroglioma Hs683 cells are not endowed with EAAT-dependent anionic amino acid transport. However, in these cells, but not in U373 human glioblastoma cells, valproic acid (VPA), an inhibitor of histone deacetylases, markedly induces SLC1A1 mRNA, which encodes for the glutamate transporter EAAT3. The effect is detectable after 8h and persists up to 120h of treatment. EAAT3 protein increase becomes detectable after 24h of treatment and reaches its maximum after 72-96h, when it is eightfold more abundant than control. The initial influx of d-aspartate increases in parallel, exhibiting the typical features of an EAAT3-mediated process. SLC1A1 mRNA induction is associated with the increased expression of PDGFRA mRNA (+150%), a marker of early oligodendrocyte precursor cells, while the expression of GFAP, CNP and TUBB3 remains unchanged. Short term experiments have indicated that the VPA effect is shared by trichostatin A, another inhibitor of histone deacetylases. On the contrary, EAAT3 induction is neither prevented by inhibitors of mitogen-activated protein kinases nor triggered by a prolonged incubation with lithium, thus excluding a role for the GSK3?/?-catenin pathway. Thus, the VPA-dependent induction of the glutamate transporter EAAT3 in human oligodendroglioma cells likely occurs through an epigenetic mechanism and may represent an early indicator of commitment to oligodendrocytic differentiation. PMID:23041758

Bianchi, M G; Franchi-Gazzola, R; Reia, L; Allegri, M; Uggeri, J; Chiu, M; Sala, R; Bussolati, O

2012-10-03

154

Familial Resemblance for Serum Metabolite Concentrations.  

UK PubMed Central (United Kingdom)

Metabolomics is the comprehensive study of metabolites, which are the substrates, intermediate, and end products of cellular metabolism. The heritability of the concentrations of circulating metabolites bears relevance for evaluating their suitability as biomarkers for disease. We report aspects of familial resemblance for the concentrations in human serum of more than 100 metabolites, measured using a targeted metabolomics platform. Age- and sex-corrected monozygotic twin correlations, midparent-offspring regression coefficients, and spouse correlations in subjects from two independent cohorts (Netherlands Twin Register and Leiden Longevity Study) were estimated for each metabolite. In the Netherlands Twin Register subjects, who were largely fasting, we found significant monozygotic twin correlations for 121 out of 123 metabolites. Heritability was confirmed by midparent-offspring regression. For most detected metabolites, the correlations between spouses were considerably lower than those between twins, indicating a contribution of genetic effects to familial resemblance. Remarkably high heritability was observed for free carnitine (monozygotic twin correlation 0.66), for the amino acids serine (monozygotic twin correlation 0.77) and threonine (monozygotic twin correlation 0.64), and for phosphatidylcholine acyl-alkyl C40:3 (monozygotic twin correlation 0.77). For octenoylcarnitine, a consistent point estimate of approximately 0.50 was found for the spouse correlations in the two cohorts as well as for the monozygotic twin correlation, suggesting that familiality for this metabolite is explained by shared environment. We conclude that for the majority of metabolites targeted by the used metabolomics platform, the familial resemblance of serum concentrations is largely genetic. Our results contribute to the knowledge of the heritability of fasting serum metabolite concentrations, which is relevant for biomarker research.

Draisma HH; Beekman M; Pool R; van Ommen GJ; Vaarhorst AA; de Craen AJ; Willemsen G; Slagboom PE; Boomsma DI

2013-08-01

155

Expression of CD8 is associated with non-common type morphology and outcome in pediatric anaplastic lymphoma kinase-positive anaplastic large cell lymphoma.  

Science.gov (United States)

Anaplastic lymphoma kinase-positive anaplastic large T-cell lymphoma is characterized by morphological variability. Morphological variants (non-common subtype) are associated with a poor outcome. They display abundant reactive bystander cells admixed with the lymphoma cells. So far, the difficulty in distinguishing lymphoma cells from bystander cells by visual inspection has prevented detailed and reliable immunophenotypic analysis using conventional immunohistochemistry. To overcome these limitations, we analyzed 124 cases of pediatric anaplastic lymphoma kinase-positive anaplastic large cell lymphoma treated within clinical trials using immunofluorescence multi-staining and digital image analysis combining antibodies against anaplastic lymphoma kinase to specifically identify lymphoma cells with antibodies against CD30, CD3, CD5, CD8, Ki67 and phosphorylated STAT3. Non-common type anaplastic lymphoma kinase-positive anaplastic large cell lymphomas express CD8 more frequently than common type anaplastic lymphoma kinase-positive anaplastic large cell lymphomas (35.4% and 5.6%, respectively; P=0.0002). CD8 expression was associated with a poorer outcome. Importantly, in a multivariate analysis including clinical risk factors, histological subtype and CD8 expression, CD8-positivity proved to be an independent prognostic predictor of worse outcome (hazard ratio for survival 3.38, P=0.042). PMID:23716548

Abramov, Dmitriy; Oschlies, Ilske; Zimmermann, Martin; Konovalov, Dmitriy; Damm-Welk, Christine; Wössmann, Wilhelm; Klapper, Wolfram

2013-05-28

156

Sweet's syndrome with panuveitis resembling Behcet's disease.  

UK PubMed Central (United Kingdom)

PURPOSE: Sweet's syndrome (SS) is a skin disorder clinically characterized by fever, neutrophilia, and painful edematous plaques that occasionally causes posterior uveitis. We present two cases of SS with panuveitis resembling Behçet's disease (BD). SUBJECTS: Two patients with panuveitis associated with SS. OBSERVATIONS: The patient in case 1 was a 57-year-old Japanese man who developed acute severe iritis with hypopyon in the left eye. Fluorescein angiography (FA) performed 1 month after treatment showed findings observed in posterior uveitis: dye leakage from the optic disc and a petaloid pattern of hyperfluorescence in the macular region. The patient in case 2 was a 64-year-old Japanese man who complained of blurred vision in his left eye. Faint flare and occasional cells were present in the left anterior chamber, 2+ cells in the anterior vitreous, and 2+ vitreous opacification in the left eye. FA demonstrated dye leakage from the optic disc and peripheral capillary vessels in both eyes. Both patients were diagnosed as having SS on the basis of fever, neutrophilia, elevated C-reactive protein, and skin biopsy results of neutrophilic infiltration without vasculitis. CONCLUSIONS: Differentiation of SS from BD based on the ocular manifestations is difficult. Ophthalmologists should bear in mind that SS can exhibit panuveitis resembling BD.

Matsumiya W; Kusuhara S; Yamada Y; Azumi A; Negi A

2012-05-01

157

Identificación de alteraciones genéticas en oligodendrogliomas mediante amplificación dependiente de ligasa de múltiples sondas (MLPA) (multiple ligation-dependent probe amplification)/ Identification of genetic alterations by multiple ligation-dependent probe amplification (MLPA) analysis in oligodendrogliomas  

Scientific Electronic Library Online (English)

Full Text Available Abstract in spanish La alteración genética más frecuente en oligodendro gliomas es la pérdida conjunta de lp/19q. Este evento ya acontece en etapas primarias del desarrollo de estos tumores. Es de gran valor clínico conocer si dichos tumores poseen esta deleción ya que se ha correlacionado con un mejor pronóstico de los pacientes. Además de esta alteración. también se ha observado la deleción de CDKN2A y PTEN y la amplificación de EGFR; estos cambios parecen asociarse a una mayor (more) agresividad tumoral. Mediante la técnica de MLPA en una misma reacción podemos determinar si existe pérdida de lp/19q y deleciones/amplificaciones de los genes anteriormente mencionados en el ADN procedente de muestras tumorales. En este trabajo hemos analizado 40 oligodendrogliomas y el kit MLPA P088 para determinar el estado alélico de lp/19q, así como el kit MLPA P105 para observar la amplificación/ deleción de los genes CDKN2A, PTEN, ERBB2, TP53 y EGFR. Mostraron pérdida de 1p el 45% de los tumores (18/40) y el 65% (26/40) de los oligodendrogliomas presentaron deleción de las sondas que hibridan en las regiones de 19q. Para el kit MLPA P105, mostraron duplicación/deleción de EGFR en el 7,5% (3/41) y 35% (14/40) de las muestras, respectivamente. El 60% de los casos (24/40) mostraron deleción de CDKN2 y ninguna muestra presentó duplicación de las sondas para este gen. El gen ERBB2 se presentó duplicado en el 12,5% de los tumores (5/40) y un único tumor mostró pérdidas de dicho gen. El 30% (12/40) de las muestras presentó deleción para PTEN y el 12,5% (5/40) mostró duplicación de dicho gen y, por último, 12,5% de los casos (5/40) presentaron duplicaciones de TP53. Estos resultados indican que la técnica de MLPA es idónea para la identificación de las alteraciones moleculares características de oligodendrogliomas. Estas alteraciones estarían contribuyendo a la formación del tumor, siendo la anomalía más significativa en oligodendrogliomas la pérdida de 1p/19q. Abstract in english Concurrent deletion at 1p/19q is a common signature of oligodendrogliomas, and it may be identified in low-grade tumours (grade II) suggesting it represents an early event in the development of these brain neoplasms. Additional non-random changes primarily involve CDKN2A, PTEN and EGFR. Identification of all of these genetic changes has become an additional parameter in the evaluation of the clinical patients' prognosis, including good response to conventional che mothera (more) py. Multiple ligation-dependent probe amplification (MLPA) analysis is a new methodology that allows an easy identification of the oligodendrogliomas' abnormalities in a single step. No need of the respective constitutional DNA from each patient is another advantage of this method. We used MLPA kits P088 and P105 to determine the molecular characteristics of a series of 40 oligodendrogliomas. Deletions at l p and 19q were identified in 45% and 65% of cases, respectively. Alterations of EGFR, CDKN2A, ERBB2, PTEN and TP53 were also identified in variable frequencies among 7% to 35% of tumours. These findings demonstrate that MLPA is a reliable technique to the detection of molecular genetic changes in oligodendrogliomas.

Franco-Hernández, C.; Martínez-Glez, V.; Torres-Martín, M.; Campos, J.M. de; Isla, A.; Vaquero, J.; Casartelli, C.; Rey, J.A.

2009-04-01

158

Identificación de alteraciones genéticas en oligodendrogliomas mediante amplificación dependiente de ligasa de múltiples sondas (MLPA) (multiple ligation-dependent probe amplification) Identification of genetic alterations by multiple ligation-dependent probe amplification (MLPA) analysis in oligodendrogliomas  

Directory of Open Access Journals (Sweden)

Full Text Available La alteración genética más frecuente en oligodendro gliomas es la pérdida conjunta de lp/19q. Este evento ya acontece en etapas primarias del desarrollo de estos tumores. Es de gran valor clínico conocer si dichos tumores poseen esta deleción ya que se ha correlacionado con un mejor pronóstico de los pacientes. Además de esta alteración. también se ha observado la deleción de CDKN2A y PTEN y la amplificación de EGFR; estos cambios parecen asociarse a una mayor agresividad tumoral. Mediante la técnica de MLPA en una misma reacción podemos determinar si existe pérdida de lp/19q y deleciones/amplificaciones de los genes anteriormente mencionados en el ADN procedente de muestras tumorales. En este trabajo hemos analizado 40 oligodendrogliomas y el kit MLPA P088 para determinar el estado alélico de lp/19q, así como el kit MLPA P105 para observar la amplificación/ deleción de los genes CDKN2A, PTEN, ERBB2, TP53 y EGFR. Mostraron pérdida de 1p el 45% de los tumores (18/40) y el 65% (26/40) de los oligodendrogliomas presentaron deleción de las sondas que hibridan en las regiones de 19q. Para el kit MLPA P105, mostraron duplicación/deleción de EGFR en el 7,5% (3/41) y 35% (14/40) de las muestras, respectivamente. El 60% de los casos (24/40) mostraron deleción de CDKN2 y ninguna muestra presentó duplicación de las sondas para este gen. El gen ERBB2 se presentó duplicado en el 12,5% de los tumores (5/40) y un único tumor mostró pérdidas de dicho gen. El 30% (12/40) de las muestras presentó deleción para PTEN y el 12,5% (5/40) mostró duplicación de dicho gen y, por último, 12,5% de los casos (5/40) presentaron duplicaciones de TP53. Estos resultados indican que la técnica de MLPA es idónea para la identificación de las alteraciones moleculares características de oligodendrogliomas. Estas alteraciones estarían contribuyendo a la formación del tumor, siendo la anomalía más significativa en oligodendrogliomas la pérdida de 1p/19q.Concurrent deletion at 1p/19q is a common signature of oligodendrogliomas, and it may be identified in low-grade tumours (grade II) suggesting it represents an early event in the development of these brain neoplasms. Additional non-random changes primarily involve CDKN2A, PTEN and EGFR. Identification of all of these genetic changes has become an additional parameter in the evaluation of the clinical patients' prognosis, including good response to conventional che motherapy. Multiple ligation-dependent probe amplification (MLPA) analysis is a new methodology that allows an easy identification of the oligodendrogliomas' abnormalities in a single step. No need of the respective constitutional DNA from each patient is another advantage of this method. We used MLPA kits P088 and P105 to determine the molecular characteristics of a series of 40 oligodendrogliomas. Deletions at l p and 19q were identified in 45% and 65% of cases, respectively. Alterations of EGFR, CDKN2A, ERBB2, PTEN and TP53 were also identified in variable frequencies among 7% to 35% of tumours. These findings demonstrate that MLPA is a reliable technique to the detection of molecular genetic changes in oligodendrogliomas.

C. Franco-Hernández; V. Martínez-Glez; M. Torres-Martín; J.M. de Campos; A. Isla; J. Vaquero; C. Casartelli; J.A. Rey

2009-01-01

159

Pathobiology of ALK+ anaplastic large-cell lymphoma  

Digital Repository Infrastructure Vision for European Research (DRIVER)

Anaplastic large-cell lymphoma (ALCL) was initially recognized on the basis of morphologic features and the consistent expression of CD30. It then became evident that the majority of these tumors are derived from lymphoid cells of T or null immunophenotype. The subsequent finding that t(2;5)(p23;q35...

Amin, Hesham M.; Lai, Raymond

160

Periocular cutaneous anaplastic large cell lymphoma clearance with brentuximab vedotin.  

Science.gov (United States)

The treatment options for primary cutaneous anaplastic large cell lymphoma are numerous, including excision, external beam radiation, methotrexate, and chemotherapy. In patients with recalcitrant tumors, alternative options may be necessary. The authors report a 60-year-old man with a 6cm primary cutaneous anaplastic large cell lymphoma located on the right cheek, near the eye. After failing four months of methotrexate, and due to concern for ocular radiation toxicity, the patient started brentuximab vedotin, an anti-CD30 antibody-drug conjugate. With two cycles of brentuximab vedotin, he had a complete response that was histologically confirmed. Six months after a total of four cycles, he remains clear. He experienced no side effects other than a mild infusion reaction. Brentuximab vedotin may be an effective option for primary cutaneous anaplastic large cell lymphoma in patients with large tumors in cosmetically sensitive areas, those who have not responded to conventional therapy, or those who have contraindications to radiation therapy. Optimal dosing for primary cutaneous anaplastic large cell lymphoma and long-term outcomes are not currently known. PMID:24003348

Kaffenberger, Benjamin H; Kartono Winardi, Francisca; Frederickson, Julie; Porcu, Pierluigi; Wong, Henry K

2013-08-01

 
 
 
 
161

Periocular cutaneous anaplastic large cell lymphoma clearance with brentuximab vedotin.  

UK PubMed Central (United Kingdom)

The treatment options for primary cutaneous anaplastic large cell lymphoma are numerous, including excision, external beam radiation, methotrexate, and chemotherapy. In patients with recalcitrant tumors, alternative options may be necessary. The authors report a 60-year-old man with a 6cm primary cutaneous anaplastic large cell lymphoma located on the right cheek, near the eye. After failing four months of methotrexate, and due to concern for ocular radiation toxicity, the patient started brentuximab vedotin, an anti-CD30 antibody-drug conjugate. With two cycles of brentuximab vedotin, he had a complete response that was histologically confirmed. Six months after a total of four cycles, he remains clear. He experienced no side effects other than a mild infusion reaction. Brentuximab vedotin may be an effective option for primary cutaneous anaplastic large cell lymphoma in patients with large tumors in cosmetically sensitive areas, those who have not responded to conventional therapy, or those who have contraindications to radiation therapy. Optimal dosing for primary cutaneous anaplastic large cell lymphoma and long-term outcomes are not currently known.

Kaffenberger BH; Kartono Winardi F; Frederickson J; Porcu P; Wong HK

2013-08-01

162

A Rare Case of Anaplastic Variant of Diffuse Large B-Cell Lymphoma Presenting as a Lung Primary.  

UK PubMed Central (United Kingdom)

Primary pulmonary lymphoma is an uncommon neoplastic disorder representing approximately 0.5% to 1% of primary pulmonary malignancies. The vast majority are of low-grade, mucosa-associated lymphoid tissue type. Primary diffuse large B-cell lymphoma of the lung is rare, though cases of the centroblastic and immunoblastic variants have been described. We present herein an interesting case of an 80-year-old man who presented with both respiratory and constitutional symptoms and was found to have a 4.5 cm left hilar mass with bilateral hilar and mediastinal lymphadenopathy on imaging. Endobronchial biopsy revealed an aggressive large cell lymphoma, with scattered large, bizarre-shaped nuclei resembling Reed-Sternberg cells, positive for CD20, PAX5, CD30, and MUM-1, consistent with an anaplastic variant of diffuse large B-cell lymphoma. Imaging showed no evidence of extrathoracic disease. Standard treatment with cyclophosphamide/vincristine/prednisone and rituximab resulted in significant clinical and radiological response and the patient remains in remission 21 months later. To the best of our knowledge, this modified Ann Arbor stage II2E primary pulmonary lymphoma, is the first description in the English literature of anaplastic variant of diffuse large B-cell lymphoma presenting as a lung primary.

Kos Z; Burns BF; Gomes MM; Sekhon H

2013-06-01

163

Bafetinib in Treating Patients With Recurrent High-Grade Glioma or Brain Metastases  

Science.gov (United States)

Adult Anaplastic Astrocytoma; Adult Anaplastic Ependymoma; Adult Anaplastic Oligodendroglioma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Mixed Glioma; Recurrent Adult Brain Tumor; Tumors Metastatic to Brain; Adult Anaplastic Oligoastrocytoma

2013-03-18

164

Body elimination attitude family resemblance in Kuwait.  

UK PubMed Central (United Kingdom)

The purpose of the present study was to determine the family resemblance of attitude toward body elimination in Kuwaiti participants. This study was conceptualized in the context of the theories of moral development, importance of cleanliness in the Muslim religion, cross-cultural differences in personal hygiene practices, previous research reporting an association between family attitudes and body elimination attitude, and health implications. The 24-item Likert-type format Body Elimination Attitude Scale-Revised was administered to 277 Kuwaiti high school students and 437 of their parents. Females scored higher, indicating greater disgust, than the males. Moreover, sons' body elimination attitude correlated more strongly with fathers' attitude (r = .85) than with that of the mothers (r = .64). Daughters' attitude was similarly associated with the fathers' (r = .89) and the mothers' attitude (r = .86). The high correlations were discussed within the context of Kuwait having a collectivistic culture with authoritarian parenting style. The higher adolescent correlations, and in particular the boys' correlation with fathers than with mothers, was explained in terms of the more dominant role of the Muslim father in the family. Public health and future research implications were suggested. A theoretical formulation was advanced in which "ideal" body elimination attitude is relative rather than absolute, and is a function of one's life circumstances, one's occupation, one's culture and subculture, and the society that one lives in.

Al-Fayez G; Awadalla A; Arikawa H; Templer DI; Hutton S

2009-12-01

165

Giant sublingual epidermoid cyst resembling plunging ranula.  

Science.gov (United States)

Epidermoid and dermoid cysts represent less than 0.01% of all oral cavity cysts. We describe a rare case of large epidermoid cyst in floor of mouth, with an oral as well as submental component resembling plunging ranula reported in the literature from India. We present a case of a 16-year-old girl with complaints of a mass in sublingual region, difficulty chewing, and dysphagia for about 5 months. Fine-needle aspiration cytology showed keratin flakes and proteinaceous material. Contrast-enhanced CT oral cavity was done and showed 7.0 × 5 × 4.5 cm well-circumscribed non-enhancing cystic mass extending into the floor of the mouth. On examination, a firm swelling was noticed in the submental area, extending down to the thyroid notch. The patient underwent surgical removal of the mass. On histopathology, acidophilic stratum corneum and basophilic dot like staining of stratum granulosum, which is the hallmark of an epidermoid cyst, were seen. PMID:23833501

Verma, Sandeep; Kushwaha, Jitendra Kumar; Sonkar, A A; Kumar, Rahul; Gupta, Rajni

2012-07-01

166

Giant sublingual epidermoid cyst resembling plunging ranula.  

UK PubMed Central (United Kingdom)

Epidermoid and dermoid cysts represent less than 0.01% of all oral cavity cysts. We describe a rare case of large epidermoid cyst in floor of mouth, with an oral as well as submental component resembling plunging ranula reported in the literature from India. We present a case of a 16-year-old girl with complaints of a mass in sublingual region, difficulty chewing, and dysphagia for about 5 months. Fine-needle aspiration cytology showed keratin flakes and proteinaceous material. Contrast-enhanced CT oral cavity was done and showed 7.0 × 5 × 4.5 cm well-circumscribed non-enhancing cystic mass extending into the floor of the mouth. On examination, a firm swelling was noticed in the submental area, extending down to the thyroid notch. The patient underwent surgical removal of the mass. On histopathology, acidophilic stratum corneum and basophilic dot like staining of stratum granulosum, which is the hallmark of an epidermoid cyst, were seen.

Verma S; Kushwaha JK; Sonkar AA; Kumar R; Gupta R

2012-07-01

167

Novel genomic alterations and mechanisms associated with tumor progression in oligodendroglioma and mixed oligoastrocytoma.  

UK PubMed Central (United Kingdom)

Combined 1p/19q deletions are very prevalent in oligodendrogliomas (OGs) and, to a lesser extent, in oligoastrocytomas (OAs). These losses are associated with responsiveness to therapy. Using array-based comparative genomic hybridization, we screened for recurrent genomic alterations in OG and oligoastrocytoma subtypes on chromosome 19. Concomitant 1p/19q loss was detected in most of the tumors with allelic loss, but array-based comparative genomic hybridization revealed some tumors to have unrelated 1p/19q arm losses, suggesting alternative mechanisms of loss to that related to the reported t(1;19) translocation. Analyses of 1p/19q loss by fluorescence in situ hybridization and loss of heterozygosity assays and correlations of genomic data with the Ki-67 proliferation marker were also performed. Four 1q (or 19p) and 2 1p (or 19q) fluorescence in situ hybridization probe signals together with homozygosity of the 1p/19q microsatellites suggested a hypothetical mechanism of genome duplication consecutive to the loss of the derivative chromosome der(1p;19q) from the t(1;19)(1q;19p) translocation. This genome duplication was frequent in high-grade OGs and was strongly correlated with Ki-67 expression; thus, it could be related to tumor progression. Finally, in addition to the frequent 1p/19q loss, we report a novel 17q amplified region in OGs with BIRC5 as one of the possible candidate target genes of the amplicon.

Blesa D; Mollejo M; Ruano Y; de Lope AR; Fiaño C; Ribalta T; García JF; Campos-Martín Y; Hernández-Moneo JL; Cigudosa JC; Meléndez B

2009-03-01

168

Novel genomic alterations and mechanisms associated with tumor progression in oligodendroglioma and mixed oligoastrocytoma.  

Science.gov (United States)

Combined 1p/19q deletions are very prevalent in oligodendrogliomas (OGs) and, to a lesser extent, in oligoastrocytomas (OAs). These losses are associated with responsiveness to therapy. Using array-based comparative genomic hybridization, we screened for recurrent genomic alterations in OG and oligoastrocytoma subtypes on chromosome 19. Concomitant 1p/19q loss was detected in most of the tumors with allelic loss, but array-based comparative genomic hybridization revealed some tumors to have unrelated 1p/19q arm losses, suggesting alternative mechanisms of loss to that related to the reported t(1;19) translocation. Analyses of 1p/19q loss by fluorescence in situ hybridization and loss of heterozygosity assays and correlations of genomic data with the Ki-67 proliferation marker were also performed. Four 1q (or 19p) and 2 1p (or 19q) fluorescence in situ hybridization probe signals together with homozygosity of the 1p/19q microsatellites suggested a hypothetical mechanism of genome duplication consecutive to the loss of the derivative chromosome der(1p;19q) from the t(1;19)(1q;19p) translocation. This genome duplication was frequent in high-grade OGs and was strongly correlated with Ki-67 expression; thus, it could be related to tumor progression. Finally, in addition to the frequent 1p/19q loss, we report a novel 17q amplified region in OGs with BIRC5 as one of the possible candidate target genes of the amplicon. PMID:19225409

Blesa, David; Mollejo, Manuela; Ruano, Yolanda; de Lope, Angel Rodríguez; Fiaño, Concepción; Ribalta, Teresa; García, Juan Fernando; Campos-Martín, Yolanda; Hernández-Moneo, Jose-Luis; Cigudosa, Juan Cruz; Meléndez, Bárbara

2009-03-01

169

Glioblastoma with oligodendroglioma component (GBM-O): molecular genetic and clinical characteristics.  

UK PubMed Central (United Kingdom)

Glioblastoma (GBM) is an aggressive primary brain tumor with an average survival of approximately 1 year. A recently recognized subtype, glioblastoma with oligodendroglioma component (GBM-O), was designated by the World Health Organization (WHO) in 2007. We investigated GBM-Os for their clinical and molecular characteristics as compared to other forms of GBM. Tissue samples were used to determine EGFR, PTEN, and 1p and 19q status by fluorescence in situ hybridization (FISH); p53 and mutant IDH1 protein expression by immunohistochemistry (IHC); and MGMT promoter status by methylation-specific polymerase chain reaction (PCR). GBM-Os accounted for 11.9% of all GBMs. GBM-Os arose in younger patients compared to other forms of GBMs (50.7 years vs. 58.7 years, respectively), were more frequently secondary neoplasms, had a higher frequency of IDH1 mutations and had a lower frequency of PTEN deletions. Survival was longer in patients with GBM-Os compared to those with other GBMs, with median survivals of 16.2 and 8.1 months, respectively. Most of the survival advantage for GBM-O appeared to be associated with a younger age at presentation. Among patients with GBM-O, younger age at presentation and 1p deletion were most significant in conferring prolonged survival. Thus, GBM-O represents a subset of GBMs with distinctive morphologic, clinical and molecular characteristics.

Appin CL; Gao J; Chisolm C; Torian M; Alexis D; Vincentelli C; Schniederjan MJ; Hadjipanayis C; Olson JJ; Hunter S; Hao C; Brat DJ

2013-07-01

170

Glioblastoma with oligodendroglioma component (GBM-O): molecular genetic and clinical characteristics.  

Science.gov (United States)

Glioblastoma (GBM) is an aggressive primary brain tumor with an average survival of approximately 1 year. A recently recognized subtype, glioblastoma with oligodendroglioma component (GBM-O), was designated by the World Health Organization (WHO) in 2007. We investigated GBM-Os for their clinical and molecular characteristics as compared to other forms of GBM. Tissue samples were used to determine EGFR, PTEN, and 1p and 19q status by fluorescence in situ hybridization (FISH); p53 and mutant IDH1 protein expression by immunohistochemistry (IHC); and MGMT promoter status by methylation-specific polymerase chain reaction (PCR). GBM-Os accounted for 11.9% of all GBMs. GBM-Os arose in younger patients compared to other forms of GBMs (50.7 years vs. 58.7 years, respectively), were more frequently secondary neoplasms, had a higher frequency of IDH1 mutations and had a lower frequency of PTEN deletions. Survival was longer in patients with GBM-Os compared to those with other GBMs, with median survivals of 16.2 and 8.1 months, respectively. Most of the survival advantage for GBM-O appeared to be associated with a younger age at presentation. Among patients with GBM-O, younger age at presentation and 1p deletion were most significant in conferring prolonged survival. Thus, GBM-O represents a subset of GBMs with distinctive morphologic, clinical and molecular characteristics. PMID:23289977

Appin, Christina L; Gao, Jingjing; Chisolm, Candace; Torian, Mike; Alexis, Dianne; Vincentelli, Cristina; Schniederjan, Matthew J; Hadjipanayis, Costas; Olson, Jeffrey J; Hunter, Stephen; Hao, Chunhai; Brat, Daniel J

2013-01-30

171

Anaplastic sarcoma of the kidney: Case report and literature review.  

Science.gov (United States)

Anaplastic sarcoma of the kidney (ASK) is a relatively newly recognized pediatric renal tumor. The present patient, a 13-year-old boy with a large renal mass, underwent surgery. Pathological findings showed proliferation of short spindle-shaped cells with anaplastic features including multiple foci in hyaline cartilage. Complex chromosomal abnormalities were detected in the tumor cells. Postoperative chemotherapy with the regimen for Ewing's sarcoma achieved complete remission but the tumor recurred and the patient died during re-induction chemotherapy. Autopsy indicated the cause of death as duodenal hemorrhage. Because there were no viable tumor cells, the recurrent tumor was considered to have been completely cured by chemotherapy. ASK is a very rare tumor, of unknown pathogenesis, and no standard treatment has yet been established, but the tumor cells may be responsive to chemotherapy. Further study is needed to establish the optimal treatment strategy. PMID:24134767

Watanabe, Noriko; Omagari, Daisuke; Yamada, Tsutomu; Nemoto, Norimichi; Furuya, Takeshi; Sugito, Kiminobu; Koshinaga, Tsugumichi; Yagasaki, Hiroshi; Sugitani, Masahiko

2013-10-01

172

MK-1775 and Local Radiation Therapy in Treating Younger Patients With Newly Diagnosed Diffuse Intrinsic Pontine Gliomas  

Science.gov (United States)

Untreated Childhood Anaplastic Astrocytoma; Untreated Childhood Anaplastic Oligodendroglioma; Untreated Childhood Brain Stem Glioma; Untreated Childhood Giant Cell Glioblastoma; Untreated Childhood Gliosarcoma

2013-09-16

173

Pathobiology of ALK-negative anaplastic large cell lymphoma  

Directory of Open Access Journals (Sweden)

Full Text Available The authors revise the concept of ALK-negative anaplastic large cell lymphoma (ALCL) in the light of the recently updated WHO classification of Tumors of Hematopoietic and Lymphoid Tissues both on biological and clinical grounds. The main histological findings are illustrated as well as the phenotypic, molecular and clinical characteristics. Finally, the biological rationale for possible innovative targeted therapies is presented.

Stefano A. Pileri; Claudio Agostinelli; Francesco Bacci; Elena Sabattini; Carlo Sagramoso; Brunangelo Falini; Pier Paolo Piccaluga

2011-01-01

174

Anaplastic thyroid cancer in young patients: A contemporary review.  

UK PubMed Central (United Kingdom)

PURPOSE: Little is known about prognostic factors and treatment outcomes in young patients with anaplastic thyroid cancer (ATC). The goal of this study is to define the clinical features of this subgroup. MATERIAL AND METHODS: Patients age 55 or younger with either ATC or well-differentiated thyroid cancer (WDTC) with anaplastic changes were identified using electronic medical record at the Cleveland Clinic. The same number of patients older than 55 was randomly selected to serve as control. Progression-free survival (PFS), overall survival time (OST) and cause-specific mortality (CSM) were measured against age, tumor histology, extent of disease, and treatment modalities. RESULTS: Twelve patients age 55 or younger were identified. The median age was 51years. Four patients had WDTC with anaplastic components - mixed tumor group (MTG). Their median PFS, OST, and CSM at 24months were 21.5months, 51months, and 25%, respectively. For the other 8 patients who had pure ATC, their median PFS, OST, and CSM were 3.5months, 6months, and 100%, respectively. Patients in the MTG had better survival compared to the pure ATC and control group in terms of PFS (p=0.0047 and p=0.0053), OST (p=0.0028 and p=0.0029) and the CSM at 24months (p=0.0339 and p=0.0096). In the pure ATC group, patients with positive cervical lymph node and distant metastases had similar overall survival outcomes (6 vs. 8months, p=0.4995). CONCLUSION: Prognostic factors favoring survival in young patients with ATC include ATC arising within WDTC. Once full anaplastic transformation occurs, age was not a significant factor in survival.

Li M; Milas M; Nasr C; Brainard JA; Khan MJ; Burkey BB; Scharpf J

2013-08-01

175

Anaplastic ganglioglioma of the brainstem in an adult.  

Science.gov (United States)

Gangliogliomas are neoplasms with neuronal and glial components. The most common location is the temporal lobe and for that reason those patients have seizures as the major complaint. Gangliogliomas with anaplastic features are uncommon. A 33-year-old man presented with a two-year history of progressively worsening right-sided weakness and contractures. Physical examination demonstrated right-sided weakness and contractures involving the upper and lower extremities. Magnetic resonance demonstrated multiple nodules involving the tegmental pons with a small projection into the prepontine cistern on the left, midbrain tegmentum on the left in the subthalamic region. The patient was studied by MRI on T1WI, T2WI, FLAIR, DWI, and magnetic resonance spectroscopy. He underwent a craniotomy and biopsy of the mass. Histological examination of the specimen revealed glial proliferation. Based on these findings the pathologic diagnosis was anaplastic ganglioglioma. Only one previous report of an anaplastic astrocytoma in the cerebello-pontine angle in an adult has been published. In children three cases were reported, only one with magnetic resonance. Our case showed multiple nodular structures hypointense on T1 and hyperintense on T2 and FLAIR with enhancement on T1 after injection of paramagnetic contrast. Only in this contribution T2 value were diffusion-weighted and ADC characteristics and (1)H spectroscopy analyzed. PMID:24028985

González Toledo, E; Nader, M; Thomas-Ogunniyi, J; Wilson, J

2012-06-26

176

Anaplastic ganglioglioma of the brainstem in an adult.  

UK PubMed Central (United Kingdom)

Gangliogliomas are neoplasms with neuronal and glial components. The most common location is the temporal lobe and for that reason those patients have seizures as the major complaint. Gangliogliomas with anaplastic features are uncommon. A 33-year-old man presented with a two-year history of progressively worsening right-sided weakness and contractures. Physical examination demonstrated right-sided weakness and contractures involving the upper and lower extremities. Magnetic resonance demonstrated multiple nodules involving the tegmental pons with a small projection into the prepontine cistern on the left, midbrain tegmentum on the left in the subthalamic region. The patient was studied by MRI on T1WI, T2WI, FLAIR, DWI, and magnetic resonance spectroscopy. He underwent a craniotomy and biopsy of the mass. Histological examination of the specimen revealed glial proliferation. Based on these findings the pathologic diagnosis was anaplastic ganglioglioma. Only one previous report of an anaplastic astrocytoma in the cerebello-pontine angle in an adult has been published. In children three cases were reported, only one with magnetic resonance. Our case showed multiple nodular structures hypointense on T1 and hyperintense on T2 and FLAIR with enhancement on T1 after injection of paramagnetic contrast. Only in this contribution T2 value were diffusion-weighted and ADC characteristics and (1)H spectroscopy analyzed.

González Toledo E; Nader M; Thomas-Ogunniyi J; Wilson J

2012-07-01

177

Brentuximab vedotin and crizotinib in anaplastic large-cell lymphoma.  

Science.gov (United States)

Systemic anaplastic large-cell lymphoma (ALCL) is a rare, mature T-cell non-Hodgkin lymphoma. Anaplastic large-cell lymphoma cells express the surface antigen CD30, and more than half express the anaplastic lymphoma kinase (ALK) protein. These 2 proteins provide unique therapeutic targets in ALCL. Remission rates in ALCL with combination chemotherapy are approximately 80%, but relapse after first-line therapy is common. Brentuximab vedotin is a US Food and Drug Administration-approved, antibody-drug conjugate that combines an anti-CD30 antibody with monomethylauristatin E, a potent antimicrotubule agent. Response rates to brentuximab vedotin in patients with relapsed/refractory ALK and ALK ALCL have exceeded 80% with frequent complete responses and a median duration of response greater than 1 year. Brentuximab vedotin in combination with chemotherapy is being explored as a first-line therapy in ALCL. Crizotinib is an inhibitor of ALK tyrosine kinase that has been approved for the treatment of ALK non-small cell lung cancer. Successful treatment of ALK ALCL with crizotinib has been reported in pediatric patients and small case series leading to ongoing trials in relapsed/refractory ALCL. Brentuximab vedotin and crizotinib represent major advances in the treatment of ALK and ALK ALCL and will likely result in marked improvement in prognosis for this subset of aggressive lymphomas. PMID:23006951

Foyil, Kelley V; Bartlett, Nancy L

178

Brentuximab vedotin and crizotinib in anaplastic large-cell lymphoma.  

UK PubMed Central (United Kingdom)

Systemic anaplastic large-cell lymphoma (ALCL) is a rare, mature T-cell non-Hodgkin lymphoma. Anaplastic large-cell lymphoma cells express the surface antigen CD30, and more than half express the anaplastic lymphoma kinase (ALK) protein. These 2 proteins provide unique therapeutic targets in ALCL. Remission rates in ALCL with combination chemotherapy are approximately 80%, but relapse after first-line therapy is common. Brentuximab vedotin is a US Food and Drug Administration-approved, antibody-drug conjugate that combines an anti-CD30 antibody with monomethylauristatin E, a potent antimicrotubule agent. Response rates to brentuximab vedotin in patients with relapsed/refractory ALK and ALK ALCL have exceeded 80% with frequent complete responses and a median duration of response greater than 1 year. Brentuximab vedotin in combination with chemotherapy is being explored as a first-line therapy in ALCL. Crizotinib is an inhibitor of ALK tyrosine kinase that has been approved for the treatment of ALK non-small cell lung cancer. Successful treatment of ALK ALCL with crizotinib has been reported in pediatric patients and small case series leading to ongoing trials in relapsed/refractory ALCL. Brentuximab vedotin and crizotinib represent major advances in the treatment of ALK and ALK ALCL and will likely result in marked improvement in prognosis for this subset of aggressive lymphomas.

Foyil KV; Bartlett NL

2012-09-01

179

The efficiency of radiation therapy in the treatment of intracranial oligodendrogliomas: factors influencing the prognosis  

International Nuclear Information System (INIS)

Oligodendrogliomas (ODG) are a rare, slow growing, tumor in the brain, which can be cured by complete surgical resection, but as yet it is not known if postoperative adjuvant radiation therapy (RT) is essential. We analyzed the treatment results of patients with irradiated ODG to investigate the efficacy of RT in terms of survival rates and other influencing prognostic factors. Between March 1983 and December 1997, 42 patients with ODG were treated with RT at our hospital. The RT was performed daily at a dose of 1.8 ? 2.0 Gy, at 5 fractions per week, to a total dose of between 39.6 Gy and 64.8 Gy (mean 53.3 Gy). The ages of the patients ranged between 5 and 62 years, with a median age of 39 years. The mean follow-up period was 63.4 months (8-152 months). The Kaplan-Meier method was used to assess the survival, and 5 year survival rates (5-YSR). Log rank tests and Cox regression analyses were used to define the significance of prognostic factors. The majority of ODG in this study were located in the cerebral hemisphere (83.3%). ODG are slightly more common in men than women, and commonly occurs in middle age, between the 3rd and 4th decades. It has been recommended that RT is commenced within 4 weeks following surgery (5-YSR; 86% vs. 49%; ? 0.05). A local involved field irradiation with conventional fractionation, commencing within 4 weeks following surgical excision of the tumor, was beneficial for the 5-YSR, but a total radiation dose exceeding 60 Gy did not improve the 5-YSR.

2002-01-01

180

Expression of hedgehog signalling pathway in anaplastic thyroid cancer.  

UK PubMed Central (United Kingdom)

The purpose of this work is to study the activation of the hedgehog signalling pathway is associated with tumour progression in various types of cancer, hence the development of specific antagonists raises hope for new therapeutic strategies. Therefore, the expression of hedgehog pathway components in anaplastic thyroid cancer (ATC) and effects of the hedgehog inhibitor Cyclopamine on ATC cells were investigated in this study. Expression of the ligand Sonic Hedgehog (SHh), the transmembrane protein Smoothened (Smo), the receptor Patched (Ptc) and the target gene Gli-1 was evaluated in two ATC cell lines (Hth 74, C643) by RT-PCR and in tumour specimens by immunohistochemistry. The corresponding gene products were examined by western blotting analysis. After treatment with different concentrations of Cyclopamine the time-dependent course of cell viability in ATC cell lines was evaluated by MTT assay. SHh, Smo, Ptc and Gli were clearly expressed on mRNA and protein levels in both cell lines and in tumour samples (41 %SHh, 65 %Smo, 65 %Ptc and 65 %Gli). Treatment with Cyclopamine showed a time- and dose-dependent inhibition of cell numbers with IC50 values between 1 and 4 ?M in both cell lines, comparable to other types of cancer. In conclusion, we believe that the hedgehog pathway is expressed in anaplastic thyroid carcinoma specimens and proliferation of ATC cell lines can be influenced by the Hh inhibitor Cyclopamine. Aberrant activation of this pathway might be involved in the aggressive biology of anaplastic cancer and further evaluation regarding a possible clinical impact of pathway inhibition is warranted.

Hinterseher U; Wunderlich A; Roth S; Ramaswamy A; Bartsch DK; Hauptmann S; Greene BH; Fendrich V; Hoffmann S

2013-07-01

 
 
 
 
181

Loss of heterozygosity on chromosome 16p and 18q in anaplastic thyroid carcinoma.  

UK PubMed Central (United Kingdom)

The aim of this study was to clarify the genetic alterations in anaplastic transformation of the thyroid cancer. A total of 17 thyroid cancers including 7 anaplastic and 10 papillary cancers were analyzed for loss of heterozygosity (LOH) on chromosome 16p and 18q. All the samples from anaplastic cancer showed LOH at one or more loci out of ten markers on 16p, and only two showed one LOH at two loci out of five markers on 18q. No LOH was found on either 16p or 18q in papillary cancers. D16S423, D16S418 and D16s406 on 16p13.3 were the most frequently deleted loci in anaplastic cancers, and the region around these may harbor the putative tumor suppressor gene related to anaplastic transformation of thyroid cancer.

Kadota M; Tamaki Y; Sekimoto M; Fujiwara Y; Aritake N; Hasegawa S; Kobayashi T; Ikeda T; Horii A; Monden M

2003-01-01

182

Restoration of p53 function in anaplastic Wilms' tumor.  

UK PubMed Central (United Kingdom)

BACKGROUND/PURPOSE: Recent studies have reported a high incidence of p53 mutations in anaplastic Wilms' tumors (WT). Restoration of the normal p53 state by current gene therapy techniques is thus an attractive potential mode of therapy for this tumor, which is poorly responsive to standard therapy. The purpose of this study is to determine whether gene delivery of normal p53 is possible and to characterize the subsequent effect of restoring the wild-type p53 state. METHODS: Anaplastic WT RM1 cells (mutant p53) were transduced with replication-deficient adenoviral vectors containing either the wild-type p53 gene (rAd-p53) or the gene encoding a green fluorescent protein (rAd-GFP). The transduction efficiency of adenovirus for RM1 cells was determined by flow cytometric analysis of rAd-GFP-transduced cells. The effect of p53 transduction on cell viability was evaluated using a colorimetric proliferation assay. Apoptosis was evaluated by labeling DNA breaks using a TUNEL assay (Apo-Direct kit). RESULTS: Cells treated with increasing concentrations of viral particles relative to tumor cells (multiplicity of infection-MOI) showed a dose-dependent increase in the number of cells transduced. Twenty-four hours after viral treatment, the percentage of cells transduced for MOIs of 10, 50, 100, and 500 was 29.5, 60.9, 74.6, and 92.4, respectively; at 48 hours the percentage of cells transduced increased to 70.8, 90.7, 93. 7, and 96.3, respectively. Viral treatment at an MOI of 50 reduced cell proliferation by 10% at 17 hours and 97% at 5 days; at an MOI of 100, the relative reduction in proliferation was 15% and 99.8%, respectively. When assayed, 30% of cells became apoptotic at an MOI of 50, and 48% at an MOI of 100. CONCLUSIONS: Highly efficient delivery of the p53 tumor suppressor gene by adenoviral vector to anaplastic WT is possible. Subsequent restoration of the normal p53 state results in reduced viability and increased apoptosis. Gene replacement of p53 may represent a novel therapeutic agent for anaplastic Wilms' tumors.

Delatte SJ; Hazen-Martin DJ; Re GG; Kelly JR; Sutphin A; Tagge EP

2001-01-01

183

Influence of an oligodendroglial component on the survival of patients with anaplastic astrocytomas: a report of radiation therapy oncology group 83-02  

International Nuclear Information System (INIS)

Purpose: Seven percent of patients with high grade gliomas enrolled in RTOG 83-02 had mixed astrocytoma/oligodenroglial elements on central pathology review. It has often been assumed that the most aggressive histologic component of a tumor determines biologic behavior; however in this trial, the survival of patients who had mixed glioblastomas/oligodenrogliomas was significantly longer than that of patients with pure glioblastomas (GBM). We therefore evaluated the effect of an oligodendroglial component on the survival of patients who had anaplastic astrocytomas (AAF) treated in the same trial. Methods and Materials: One hundred nine patients who had AAF and 24 patients with mixed AAF/oligodendrogliomas (AAF/OL) were enrolled in a Phase I/II trial of randomized dose-escalation hyper fractioned radiotherapy plus BCNU. AAF/OL patients were older and more likely to have had more aggressive surgery than AAF patients. Other pretreatment characteristics were balanced between groups, as was assigned treatment. Results: The median survival time for AAF was 3.0 years versus 7.3 years for AAF/OL (p = 0.019). In a multivariate analysis, adjusting for extent of surgical resection and age, an oligodendroglial component was an independent prognostic factor for survival. Conclusion: The results support the concept that AAFs with an oligodendroglial component have a better prognosis than pure AAF tumors, similar to the effect seen among patients with glioblastoma multiforme tumors. This better survival outcome should be taken into consideration in the design and stratification of future trials. Additionally, in contrast to patients with GBMs, patients who have AAF/OL have the potential for prolonged survival; therefore, late sequelae of treatment (both radiation and chemotherapy) must be weighed more heavily in the benefits to risks analysis.

1997-07-15

184

Modulation of the Wnt/beta-catenin pathway in human oligodendroglioma cells by Sox17 regulates proliferation and differentiation.  

Science.gov (United States)

Oligodendrogliomas originate from oligodendrocyte progenitor cells (OPCs), whose development is regulated by the Sonic hedgehog and Wnt/beta-catenin pathways. We investigated the contribution of these pathways in the proliferation and differentiation of human oligodendroglioma cells (HOG). Inhibition of Hedgehog signaling with cyclopamine decreased cell survival and increased phosphorylated beta-catenin without altering myelin protein levels. Conversely, treatment of HOG with the Wnt antagonist secreted frizzled related protein (SFRP1), led to increased myelin protein levels and reduced cell proliferation, suggesting cell cycle arrest and differentiation. Unlike normal primary human OPCs, beta-catenin in HOG cells is not associated with endogenous Sox17 protein despite high levels of both proteins. Retroviral overexpression of recombinant Sox17 increased HOG cell cycle exit and apoptosis, and raised myelin protein levels and the percentage of O4(+) cells, indicating increased differentiation. Recombinant Sox17 also increased beta-catenin-TCF4-Sox17 complex formation and decreased total cellular levels of beta-catenin. These changes were associated with increased SFRP1, and reduced expression of Wnt-1 and Frizzled-1, -3 and -7 RNA, indicating that Sox17 induced a Hedgehog target, and regulated Wnt signaling at multiple levels. Our studies indicate that Wnt signaling regulates HOG cell cycle arrest and differentiation, and that recombinant Sox17 mediates modulation of the Wnt pathway through changes in beta-catenin, SFRP1 and Wnt/Frizzled expression. Our results thus identify Sox17 as a potential molecular target to include in HOG therapeutic strategies. PMID:23474492

Chen, Hui-Ling; Chew, Li-Jin; Packer, Roger J; Gallo, Vittorio

2013-03-06

185

Modulation of the Wnt/beta-catenin pathway in human oligodendroglioma cells by Sox17 regulates proliferation and differentiation.  

UK PubMed Central (United Kingdom)

Oligodendrogliomas originate from oligodendrocyte progenitor cells (OPCs), whose development is regulated by the Sonic hedgehog and Wnt/beta-catenin pathways. We investigated the contribution of these pathways in the proliferation and differentiation of human oligodendroglioma cells (HOG). Inhibition of Hedgehog signaling with cyclopamine decreased cell survival and increased phosphorylated beta-catenin without altering myelin protein levels. Conversely, treatment of HOG with the Wnt antagonist secreted frizzled related protein (SFRP1), led to increased myelin protein levels and reduced cell proliferation, suggesting cell cycle arrest and differentiation. Unlike normal primary human OPCs, beta-catenin in HOG cells is not associated with endogenous Sox17 protein despite high levels of both proteins. Retroviral overexpression of recombinant Sox17 increased HOG cell cycle exit and apoptosis, and raised myelin protein levels and the percentage of O4(+) cells, indicating increased differentiation. Recombinant Sox17 also increased beta-catenin-TCF4-Sox17 complex formation and decreased total cellular levels of beta-catenin. These changes were associated with increased SFRP1, and reduced expression of Wnt-1 and Frizzled-1, -3 and -7 RNA, indicating that Sox17 induced a Hedgehog target, and regulated Wnt signaling at multiple levels. Our studies indicate that Wnt signaling regulates HOG cell cycle arrest and differentiation, and that recombinant Sox17 mediates modulation of the Wnt pathway through changes in beta-catenin, SFRP1 and Wnt/Frizzled expression. Our results thus identify Sox17 as a potential molecular target to include in HOG therapeutic strategies.

Chen HL; Chew LJ; Packer RJ; Gallo V

2013-07-01

186

Anaplastic carcinoma of the thyroid: a 24-year experience.  

UK PubMed Central (United Kingdom)

BACKGROUND: Anaplastic carcinoma of the thyroid gland is a lethal entity; few patients live more than 12 months following diagnosis. We retrospectively reviewed the experience with this entity at our cancer institute and identified a subgroup of patients with complete resection who have a 60% 5-year survival. METHODS: Twenty-one cases of anaplastic carcinoma of the thyroid gland were analyzed retrospectively with respect to prognostic factors influencing survival. This represents 2.7% of 771 cases of thyroid cancer seen at our institution from 1968 to 1992. The median age at presentation was 65.1 years; male/female ratio was 1:1.1; and the most common symptom was a rapidly enlarging neck mass (76%). RESULTS: Estimated 5-year survival was 10% (median: 4.5 months). Tumor size less than 6.0 cm (p = .004) and female gender (p = .02) were significant prognostic factors. Five patients who underwent complete resection had an estimated 5-year survival of 60% (median: 131 months). Four of these patients had postoperative radiotherapy with or without sequential chemotherapy. Two of these patients survived more than 10 years, and a third remains alive without disease at 26 months.

Tan RK; Finley RK 3rd; Driscoll D; Bakamjian V; Hicks WL Jr; Shedd DP

1995-01-01

187

Absence of Epstein-Barr virus in anaplastic large cell lymphoma: a study of 64 cases classified according to World Health Organization criteria.  

UK PubMed Central (United Kingdom)

The frequency of Epstein-Barr virus (EBV) in anaplastic large cell lymphoma (ALCL) has been controversial. The interpretation of previous studies is complicated by the use of nonuniform EBV detection methods and the inclusion of cases of CD30-positive diffuse large B-cell lymphoma and so-called "ALCL, Hodgkin-like," as defined in the Revised European-American Lymphoma classification scheme. In the current World Health Organization (WHO) classification system, both of these tumors are excluded from the ALCL category. Also, recently developed antibodies (eg, the antibody specific for PAX-5/B-cell-specific activator protein [BSAP]) provide new, sensitive tools for identifying neoplasms of B-cell lineage that can morphologically resemble ALCL. In this study we evaluated 64 cases of ALCL of T- or null-cell lineage, defined according to the WHO classification system, for the presence of EBV. All tumors were negative for B-cell antigens, including PAX-5/BSAP and CD20 or CD79a. The study group included 27 (42%) anaplastic lymphoma kinase (ALK)-positive (18 T-cell and 9 null-cell) and 37 (58%) ALK-negative (30 T-cell and 7 null-cell) tumors analyzed by in situ hybridization for EBV-encoded RNA (EBER) or immunohistochemistry for EBV-latent membrane protein type 1. All 64 cases were negative for EBV. We conclude, based on the current definition of ALCL in the WHO classification, there is no role for EBV in ALCL arising in Western patients. We suggest that published reports of EBV in a small proportion of ALCL cases in Western patients can be explained by the inclusion of tumors no longer considered to be in the current classification of ALCL, such as CD30-positive anaplastic tumors of B-cell origin.

Herling M; Rassidakis GZ; Jones D; Schmitt-Graeff A; Sarris AH; Medeiros LJ

2004-04-01

188

Anaplastic lymphoma kinase (ALK): structure, oncogenic activation, and pharmacological inhibition.  

UK PubMed Central (United Kingdom)

Anaplastic lymphoma kinase was first described in 1994 as the NPM-ALK fusion protein that is expressed in the majority of anaplastic large-cell lymphomas. ALK is a receptor protein-tyrosine kinase that was more fully characterized in 1997. Physiological ALK participates in embryonic nervous system development, but its expression decreases after birth. ALK is a member of the insulin receptor superfamily and is most closely related to leukocyte tyrosine kinase (Ltk), which is a receptor protein-tyrosine kinase. Twenty different ALK-fusion proteins have been described that result from various chromosomal rearrangements, and they have been implicated in the pathogenesis of several diseases including anaplastic large-cell lymphoma, diffuse large B-cell lymphoma, and inflammatory myofibroblastic tumors. The EML4-ALK fusion protein and four other ALK-fusion proteins play a fundamental role in the development in about 5% of non-small cell lung cancers. The formation of dimers by the amino-terminal portion of the ALK fusion proteins results in the activation of the ALK protein kinase domain that plays a key role in the tumorigenic process. Downstream signaling from ALK fusion proteins involves the Ras/Raf/MEK/ERK1/2 cell proliferation module and the JAK/STAT cell survival pathway. Furthermore, nearly two dozen ALK activating mutations participate in the pathogenesis of childhood neuroblastomas along with ALK overexpression. The occurrence of oncogenic ALK, particularly in non-small cell lung cancer, has generated considerable interest and effort in developing ALK inhibitors. Currently, crizotinib has been approved by the US Food and Drug Administration for the treatment of ALK-positive non-small cell lung cancer along with an approved fluorescence in situ hybridization kit used for the diagnosis of the disease. The emergence of crizotinib drug resistance with a median occurrence at approximately 10 months after the initiation of therapy has stimulated the development of second-generation drugs for the treatment of non-small cell lung cancer and other disorders. About 28% of the cases of crizotinib resistance are related to nearly a dozen different mutations of ALK in the EML4-ALK fusion protein; the other cases of resistance are related to the upregulation of alternative signaling pathways or to undefined mechanisms. It is remarkable that the EML4-ALK fusion protein was discovered in 2007 and crizotinib was approved for the treatment of ALK-positive non-small cell lung cancer in 2011, which is a remarkably short timeframe in the overall scheme of drug discovery.

Roskoski R Jr

2013-02-01

189

Recent progress of genome study for anaplastic thyroid cancer.  

UK PubMed Central (United Kingdom)

Anaplastic thyroid cancer (ATC) belongs to the most malignant and rapidly progressive human thyroid cancers and its prognosis is very poor. Also, it shows high resistance to cancer treatments, so that effective treatment for ATC has not been found to date, and virtually all patients terminate their life rapidly after diagnosis. Although targeted treatment of genetic alterations has emerged as an extremely promising approach to human cancers, such as BRAF in metastatic melanoma, it remains unclear that how commonly genomic alterations are influenced in ATC tumorigenesis. In recent years, genome wide approaches have been exploited to find genetic alterations associated with complex diseases, including cancer. Here, we reviewed the comprehensive genetic alterations in ATC and recent approaches in the context of identifying genomic alterations associated with ATC. Since surprisingly few reports have been published on the genome wide study of ATC, this review puts emphasis on the urgent needs of genomic research for the prevention and treatment of ATC.

Lee J; Hwang JA; Lee EK

2013-06-01

190

Anaplastic astrocytoma following radiation for a glomus jugular tumor.  

UK PubMed Central (United Kingdom)

Evaluation of radiation therapy for a given neoplasm includes consideration of possible treatment complications as well as potential benefit. A 43-year-old male with a glomus jugular tumor or the right middle ear had received 4480 rad to the right middle and inner ear and temporal bone. Eight years later, he developed an anaplastic astrocytoma of the right cerebellar hemisphere. At this time a third neoplasm, a left carotid body tumor, was demonstrated angiographically. Although radiation can be implicated in the genesis of the glial neoplasm, the presence of two neural crest derived tumors suggests that a lowered threshold for neoplastic transformation in neuroectodermal cells may have been an additional factor. Long-term follow-up of large numbers of patients with glomus jugulare tumors will be necessary to determine if multiple paragangliomas predispose to radiation-associated gliomas.

Preissig SH; Bohmfalk GL; Reichel GW; Smith MT

1979-06-01

191

Anaplastic astrocytoma following radiation for a glomus jugular tumor.  

Science.gov (United States)

Evaluation of radiation therapy for a given neoplasm includes consideration of possible treatment complications as well as potential benefit. A 43-year-old male with a glomus jugular tumor or the right middle ear had received 4480 rad to the right middle and inner ear and temporal bone. Eight years later, he developed an anaplastic astrocytoma of the right cerebellar hemisphere. At this time a third neoplasm, a left carotid body tumor, was demonstrated angiographically. Although radiation can be implicated in the genesis of the glial neoplasm, the presence of two neural crest derived tumors suggests that a lowered threshold for neoplastic transformation in neuroectodermal cells may have been an additional factor. Long-term follow-up of large numbers of patients with glomus jugulare tumors will be necessary to determine if multiple paragangliomas predispose to radiation-associated gliomas. PMID:222421

Preissig, S H; Bohmfalk, G L; Reichel, G W; Smith, M T

1979-06-01

192

Targeting CD30 in anaplastic large cell lymphoma.  

UK PubMed Central (United Kingdom)

Anaplastic large cell lymphoma (ALCL) is a lymphoid neoplasm characterized by strong and uniform expression of the CD30 antigen on the cell surface. Current standard frontline therapy of ALCL is anthracycline-based combination chemotherapy, usually CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) or CHOP-like regimens. Despite aggressive chemotherapy a significant number of patients relapse. Newer agents and strategies are needed in the management of this challenging disease especially in ALK-negative and high-risk ALK-positive patients who tend to have a poor prognosis. In this review we discuss the different approaches to targeting CD30 including naked antibodies, "enhanced antibodies", antibody drug-toxin conjugates, radioimmunoconjugates, CD30-ligand-toxin conjugates, bispecific antibodies and T cell-based immune therapies.

Vadakara J; Pro B

2012-12-01

193

Anaplastic ganglioglioma: a very rare intramedullary spinal cord tumor.  

Science.gov (United States)

Gangliogliomas (GGs) are a small subset of intramedullary spinal cord tumors in children. The anaplastic variant (WHO grade III) appears to be an extreme rarity. A literature research revealed only 15 case reports of intramedullary anaplastic GGs (aGGs) and only 4 pediatric patients. The course of an 18-month-old boy with sudden onset of paraparesis is presented. Spinal MRI revealed a contrast-enhancing intramedullary tumor ranging from T6 to T12. The patient underwent a standard laminectomy/laminoplasty and gross total resection of the lesion. His neurological status remained unchanged postoperatively and he recovered very well during outpatient neurorehabilitation. Neuropathologic examination revealed an aGG of WHO grade III. Because of the high-grade histology, adjuvant radiotherapy and chemotherapy with temozolomide were administered. The patient subsequently recovered to a normal functional status. Clinical and radiographic progression-free survival is now 4 years. Based on an extensive literature review, this is only the fifth pediatric patient with a primary intramedullary aGG and the second with documented progression-free survival of over 4 years. Another 4 primary intramedullary aGGs in adults and 7 patients with spinal dissemination from a cerebral aGG or malignant transformation of a low-grade GG have been reported. In comparison to the published case reports, which often indicate significant neurological dysfunction and rather short survival, the neurological recovery in this patient was favorable, and the oncologic outcome even more so. This is an argument for the use of the aggressive treatment regimen of complete resection followed by radio- and chemotherapy applied here. PMID:22922809

Schneider, Christian; Vosbeck, Jürg; Grotzer, Michael A; Boltshauser, Eugen; Kothbauer, Karl F

2012-08-21

194

Anaplastic ganglioglioma: a very rare intramedullary spinal cord tumor.  

UK PubMed Central (United Kingdom)

Gangliogliomas (GGs) are a small subset of intramedullary spinal cord tumors in children. The anaplastic variant (WHO grade III) appears to be an extreme rarity. A literature research revealed only 15 case reports of intramedullary anaplastic GGs (aGGs) and only 4 pediatric patients. The course of an 18-month-old boy with sudden onset of paraparesis is presented. Spinal MRI revealed a contrast-enhancing intramedullary tumor ranging from T6 to T12. The patient underwent a standard laminectomy/laminoplasty and gross total resection of the lesion. His neurological status remained unchanged postoperatively and he recovered very well during outpatient neurorehabilitation. Neuropathologic examination revealed an aGG of WHO grade III. Because of the high-grade histology, adjuvant radiotherapy and chemotherapy with temozolomide were administered. The patient subsequently recovered to a normal functional status. Clinical and radiographic progression-free survival is now 4 years. Based on an extensive literature review, this is only the fifth pediatric patient with a primary intramedullary aGG and the second with documented progression-free survival of over 4 years. Another 4 primary intramedullary aGGs in adults and 7 patients with spinal dissemination from a cerebral aGG or malignant transformation of a low-grade GG have been reported. In comparison to the published case reports, which often indicate significant neurological dysfunction and rather short survival, the neurological recovery in this patient was favorable, and the oncologic outcome even more so. This is an argument for the use of the aggressive treatment regimen of complete resection followed by radio- and chemotherapy applied here.

Schneider C; Vosbeck J; Grotzer MA; Boltshauser E; Kothbauer KF

2012-01-01

195

Anaplastic large cell lymphoma and breast implants: a systematic review.  

UK PubMed Central (United Kingdom)

BACKGROUND: In recent years, there have been growing concerns about a possible association of non-Hodgkin's lymphoma--in particular, anaplastic large cell lymphoma (ALCL)--and breast implants. The purpose of this study was to identify and analyze all reported cases of non-Hodgkin's lymphoma occurring in patients with breast implants. METHODS: The authors conducted a systematic literature review of reported cases of non-Hodgkin's lymphoma in patients with breast implants. Publications were identified with a search algorithm, forward searches, and expert nominations. After references were reviewed and assessed for inclusion or exclusion, case-based data were independently abstracted, reconciled, and adjudicated by multiple investigators. The data were then synthesized and analyzed. RESULTS: Of 884 identified articles, only 83 were relevant to non-Hodgkin's lymphoma involving the breast, and 34 were included in our study. Thirty-six cases of non-Hodgkin's lymphoma in patients with implants were found, of which 29 (81 percent) were ALCLs. Although detailed clinical information was lacking in many cases, ALCL often involved the capsule and/or presented as an unexplained seroma or mass, was negative for anaplastic lymphoma kinase (ALK) expression, and had a relatively indolent clinical course when it developed adjacent to a breast implant. CONCLUSIONS: A form of ALCL, which clinically behaves more like the less aggressive primary cutaneous form of ALK-negative ALCL rather than the more aggressive systemic form, may be associated with breast implants. Future research on the epidemiology and biology of this rare disease is clearly needed to better understand its nature.

Kim B; Roth C; Chung KC; Young VL; van Busum K; Schnyer C; Mattke S

2011-06-01

196

Synchronous occurrence of anaplastic, follicular and papillary carcinomas with follicular adenoma in thyroid gland  

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Full Text Available Various combinations of thyroid carcinomas have been reported including those between different cancers of follicular cell origin and those between follicular and C-cell histogenesis. Accordingly, anaplastic carcinomas have been seen to coincide with simultaneous papillary and follicular cancers. We report a case of composite anaplastic and papillary cancer on one thyroid lobe with a follicular carcinoma in the other lobe in a female patient aged 64 years. The patient also had a separate and independent follicular adenoma in the same lobe as the composite anaplastic and papillary carcinoma. The papillary carcinoma was continuous with the anaplastic carcinoma. The findings were supported by immunohistochemistry. The patient was managed by a total thyroidectomy with bilateral modified radical neck dissection followed by chemotherapy. However, she died two months after surgery. The common follicular cell origin will explain the concurrent presence of all these cancers. This could result from the dedifferentiation of a pre-existing differentiated carcinoma.

Ganguly R; Mitra S; Datta A

2010-01-01

197

Pediatric primary anaplastic ganglioglioma: a case report and review of the literature.  

Science.gov (United States)

Gangliogliomas with anaplastic features are classified as grade III tumors by the World Health Organization. The clinical course and optimal treatment of anaplastic gangliogliomas have not been well understood to date. We report a case of a primary pure anaplastic ganglioglioma in a 14-year-old male treated with surgery and radiotherapy, who is disease-free 6 years after the diagnosis. A review of primary pure anaplastic gangliogliomas in children (between 3 and 21 years of age) is presented. Gross total removal and focal radiotherapy with a total dose of 54 Gy are recommended. The addition of chemotherapy should be evaluated. Prospective studies are needed to identify an appropriate chemotherapy schedule and to define biological factors in order to select those patients with a poor prognosis, who are to be treated with a more aggressive therapy. PMID:22922381

Scoccianti, Silvia; Giordano, Flavio; Agresti, Benedetta; Detti, Beatrice; Cipressi, Samantha; Franceschini, Davide; Greto, Daniela; Mussa, Federico; Sardi, Iacopo; Buccoliero, Annamaria; Aricò, Maurizio; Genitori, Lorenzo; Biti, Giampaolo

2012-08-21

198

Pediatric primary anaplastic ganglioglioma: a case report and review of the literature.  

UK PubMed Central (United Kingdom)

Gangliogliomas with anaplastic features are classified as grade III tumors by the World Health Organization. The clinical course and optimal treatment of anaplastic gangliogliomas have not been well understood to date. We report a case of a primary pure anaplastic ganglioglioma in a 14-year-old male treated with surgery and radiotherapy, who is disease-free 6 years after the diagnosis. A review of primary pure anaplastic gangliogliomas in children (between 3 and 21 years of age) is presented. Gross total removal and focal radiotherapy with a total dose of 54 Gy are recommended. The addition of chemotherapy should be evaluated. Prospective studies are needed to identify an appropriate chemotherapy schedule and to define biological factors in order to select those patients with a poor prognosis, who are to be treated with a more aggressive therapy.

Scoccianti S; Giordano F; Agresti B; Detti B; Cipressi S; Franceschini D; Greto D; Mussa F; Sardi I; Buccoliero A; Aricò M; Genitori L; Biti G

2012-01-01

199

A Pilot Feasibility Study of Oral 5-Fluorocytosine and Genetically-Modified Neural Stem Cells Expressing E.Coli Cytosine Deaminase for Treatment of Recurrent High Grade Gliomas  

Science.gov (United States)

Adult Anaplastic Astrocytoma; Recurrent Grade III Glioma; Recurrent Grade IV Glioma; Adult Anaplastic Oligodendroglioma; Adult Brain Tumor; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Mixed Glioma; Recurrent Adult Brain Tumor; Adult Anaplastic Oligoastrocytoma; Recurrent High Grade Glioma

2013-03-06

200

Anaplastic large T-cell lymphoma of the external ear in childhood.  

UK PubMed Central (United Kingdom)

Anaplastic large T-cell lymphoma is a very rare disease in childhood. The most common locations are lymph nodes and skin, while the external ear location is uncommon. We present the case of a 6-year-old child with an earlobe tumour. Surgical treatment was performed and the anatomopathological results showed anaplastic large cell lymphoma. Radiological tests were negative and there was no systemic involvement.

Rodríguez V; Perolada JM; Ibañez I; Fernández A

2013-05-01

 
 
 
 
201

Anaplastic large T-cell lymphoma of the external ear in childhood.  

Science.gov (United States)

Anaplastic large T-cell lymphoma is a very rare disease in childhood. The most common locations are lymph nodes and skin, while the external ear location is uncommon. We present the case of a 6-year-old child with an earlobe tumour. Surgical treatment was performed and the anatomopathological results showed anaplastic large cell lymphoma. Radiological tests were negative and there was no systemic involvement. PMID:22483233

Rodríguez, Verónica; Perolada, José María; Ibañez, Isabel; Fernández, Amets

2012-04-05

202

Primary Anaplastic Large Cell Lymphoma of the Nasal Cavity: A Case Report  

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Full Text Available Introduction: Anaplastic large-cell lymphoma occurring in the nasal cavity is a rare disease. The latest World Health Organization (WHO) Classification recognizes three distinct subtypes: primary systemic anaplastic lymphoma kinase positive been our case, primary systemic anaplastic lymphoma kinase negative and primary cutaneous types. Through this case study, we focus on the clinical presentation, treatment and prognostic characteristics of this pathology.Case Presentation: We report the case of a patient aged 32 years, who presented for seven months a runny nose associated with swelling of the face on the left side, without peripheral lymphadenopathy or general signs. A Blondeau scanner objectified a total filling of the frontal and left maxillary sinus, and a filling of the left nasal cavity. Complete resection of the tumor was performed. Histological examination was in favor of anaplastic large T-cell lymphoma anaplastic lymphoma kinase positive. The patient was stage IE according to Ann Arbor classification, with an International Prognostic Index estimated at one. Thus, the patient received six cycles of CHOP chemotherapy. Currently, he is in good loco-regional control with a decline of three months.Conclusion: The rarity of this case lies partly in the lymphomatous localization in the nasal cavity, and secondly in the anaplastic histology. It poses a diagnostic problem. So, we conclude that in case of any symptom of nasal cavities, it is necessary to explore and possibly biopsy if tumor, before surgery, because lymphomas are chemosensitive disease.

Imane Ouafki; Tanae Sghiri; Saber Boutayeb; Mohamed Mouanis; Mustapha Maher; Hind Mrabti; Hassan Errihani

2013-01-01

203

Primary anaplastic large T cell lymphoma of central nervous system  

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Full Text Available Background Primary anaplastic large T cell lymphoma (ALCL) of central nervous system (CNS) can occur in people of all ages, and is usually unrelated with immunodeficiency. It is often misdiagnosed as meningitis, especially tuberculous meningitis, on clinical practice and imaging examination. In pathological diagnosis, the morphological changes of primary ALCL of CNS are similar to the systemic ALCL and the anaplastic lymphoma kinase-1 (ALK-1) can be positive or negative. Being misdiagnosed as meningitis, hormone therapy with glucocorticoid before biopsy is always used, and massive necrosis and a lot of histocyte proliferation and phagocytosis can be found under histological findings. Therefore, when the material is not enough, primary ALCL of CNS is often misdiagnosed as cerebral infarction or malignant histocytosis and so on. This paper reports a case of primary ALCL of CNS and makes a review of relevant literature, so as to summarize the clinical manifestations and elevate the recognition of clinicians and pathologists on this disease. Methods and Results A 12-year-old boy was admitted because of fever, worsening headache, numbness and weakness of right limbs. MRI showed local gyri swelling and abnormal enhancement of pia mater in the right parietal lobe, expanding to the right temporal lobe, and pia mater enhancement in the left parietal lobe. The right temporo-parietal lobe lesion biopsy revealed irregularly shaped tumor cells of large size, rich and eosinophilic cytoplasm and horseshoe-shaped or kidney-shaped nuclei. Immunohistochemical examination showed tumor cells positive for CD3, CD45RO, CD30, ALK-1 and epithelial membrane antigen (EMA), and negative for CD20 and CD79a. Conclusion Primary ALCL of CNS is an extremely rare tumor which is usually misdiagnosed as meningitis according to clinical and imaging examinations. Therefore, for those patients who are considered as meningitis but with poor treatment effect and replase of illness, brain tissue biopsy, which is an important means for diagnosis, or cerebrospinal fluid cytologic examination, should be carried out as soon as possible.

LIU Teng-fei; HAN Hui-xia; LI Xiang-zhao; ZHANG Yan

2013-01-01

204

Reported sleep: needed minus obtained sleep, family resemblance, and age.  

UK PubMed Central (United Kingdom)

For a normal population from 13 to 89 yr. of age there was a correlation of .02 between age and sleep obtained, -.07 between age and sleep needed, and -.33 (p less than .01) between age and sleep needed minus sleep obtained. Also, significant family resemblance was found for all three of the above sleep variables.

Ayers JL; Ruff CF; Templer DI

1979-08-01

205

Canine sterile neutrophilic dermatitis (resembling Sweet's syndrome) in a Dachshund.  

UK PubMed Central (United Kingdom)

A 6-year-old Dachshund was presented with a 2-day history of lethargy, anorexia and cutaneous erythema, edema, and multifocal erythematous papules affecting the ventral abdomen, axillae, and groin. Microscopic examination revealed a sterile neutrophilic dermatitis resembling Sweet's syndrome; however, extracutaneous lesions were not present. The condition responded rapidly to corticosteroid therapy.

Gains MJ; Morency A; Sauvé F; Blais MC; Bongrand Y

2010-12-01

206

Canine sterile neutrophilic dermatitis (resembling Sweet's syndrome) in a Dachshund.  

Science.gov (United States)

A 6-year-old Dachshund was presented with a 2-day history of lethargy, anorexia and cutaneous erythema, edema, and multifocal erythematous papules affecting the ventral abdomen, axillae, and groin. Microscopic examination revealed a sterile neutrophilic dermatitis resembling Sweet's syndrome; however, extracutaneous lesions were not present. The condition responded rapidly to corticosteroid therapy. PMID:21358936

Gains, Malcolm J; Morency, Andréanne; Sauvé, Frédéric; Blais, Marie-Claude; Bongrand, Yannick

2010-12-01

207

Conodont bioapatite resembles vertebrate enamel by XRD properties  

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Full Text Available XRD properties of Phanerozoic conodont apatite material were studied. It was found out that in terms of crystallinity the apatite resembles the enamel tissue of modern vertebrates. In terms of crystal lattice, apatite of conodonts is independent of taxa on the one hand and of chemistry of the surrounding rock type on the other hand.

Jüri Nemliher; Toivo Kallaste

2012-01-01

208

Conodont bioapatite resembles vertebrate enamel by XRD properties  

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XRD properties of Phanerozoic conodont apatite material were studied. It was found out that in terms of crystallinity the apatite resembles the enamel tissue of modern vertebrates. In terms of crystal lattice, apatite of conodonts is independent of taxa on the one hand and of chemistry of the surrou...

Jüri Nemliher; Toivo Kallaste

209

Anaplastic astrocytoma 14 years after radiotherapy for pituitary adenoma  

International Nuclear Information System (INIS)

[en] A case of anaplastic astrocytoma following radiotherapy for growth hormone secreting pituitary adenoma is presented with a review of the literature. A 43 year old female was admitted with signs of acromegaly and hypertension. An eosinophilic pituitary adenoma was subtotally removed by transsphenoidal approach, followed by 60 Gy irradiation using a 2x2 cm lateral field. Fourteen years later at the age of 57, she suffered from headache, recent-memory disturbance and uncinate fits. CT scan and MRI disclosed ring-like enhanced mass lesion in the left temporal lobe, corresponding to the previous irradiated field. 18F-FDG PET showed hypermetabolism at the lesion. Left frontotemporal craniotomy was performed, and a reddish gray gelatinous tumor containing necrotic center and cyst was partially removed. Histologically, the tumor consisted of hypercellular astrocytic cells with perivascular pseudorosette. Coagulation necrosis at the center of the tumor, and hyalinosis and fibrosis of the blood vessels in and around the tumor, which might have been caused by the antecedent radiotherapy, were recognized. Postoperative radiotherapy and chemotherapy, were given, however, she expired 13 months after the operation. Seven cases, including ours, of malignant glioma following radiotherapy for pituitary adenoma were reported in the literature. A total dose of irradiation varies from 45 to 95 Gy with a mean of 50 Gy. The period of latency before tumor occurrence ranges from 5 to 22 years with a mean of 10 years. The differentiation of radiation-induced gliomas from radionecrosis of the brain is also discussed. (author)

1992-01-01

210

Anaplastic astrocytoma 14 years after radiotherapy for pituitary adenoma  

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A case of anaplastic astrocytoma following radiotherapy for growth hormone secreting pituitary adenoma is presented with a review of the literature. A 43 year old female was admitted with signs of acromegaly and hypertension. An eosinophilic pituitary adenoma was subtotally removed by transsphenoidal approach, followed by 60 Gy irradiation using a 2x2 cm lateral field. Fourteen years later at the age of 57, she suffered from headache, recent-memory disturbance and uncinate fits. CT scan and MRI disclosed ring-like enhanced mass lesion in the left temporal lobe, corresponding to the previous irradiated field. {sup 18}F-FDG PET showed hypermetabolism at the lesion. Left frontotemporal craniotomy was performed, and a reddish gray gelatinous tumor containing necrotic center and cyst was partially removed. Histologically, the tumor consisted of hypercellular astrocytic cells with perivascular pseudorosette. Coagulation necrosis at the center of the tumor, and hyalinosis and fibrosis of the blood vessels in and around the tumor, which might have been caused by the antecedent radiotherapy, were recognized. Postoperative radiotherapy and chemotherapy, were given, however, she expired 13 months after the operation. Seven cases, including ours, of malignant glioma following radiotherapy for pituitary adenoma were reported in the literature. A total dose of irradiation varies from 45 to 95 Gy with a mean of 50 Gy. The period of latency before tumor occurrence ranges from 5 to 22 years with a mean of 10 years. The differentiation of radiation-induced gliomas from radionecrosis of the brain is also discussed. (author).

Tamura, Masaru; Misumi, Syuuzou; Kurosaki, Syuuhei; Shibasaki, Takashi; Ohye, Chihiro (Gunma Univ., Maebashi (Japan). School of Medicine)

1992-04-01

211

NGAL controls the metastatic potential of anaplastic thyroid carcinoma cells.  

UK PubMed Central (United Kingdom)

CONTEXT: We have previously identified neutrophil gelatinase-associated lipocalin (NGAL) as one of the genes mediating the oncogenic activity of nuclear factor-?B in human anaplastic thyroid carcinomas (ATCs). OBJECTIVES: To further investigate the role of NGAL in thyroid cancer, we established NGAL knocked-down and NGAL overexpressing ATC cell lines. RESULTS: We found that the ability of NGAL knocked-down cells to degrade Matrigel in a transwell invasion assay and to form lung metastasis in nude mice was decreased. Because NGAL binds matrix metalloproteinase-9 (MMP-9), to form a macromolecular complex involved in the regulation of metastatic spread of cancer cells and given the strong expression of both genes in tissue specimens from human ATCs, we analyzed the MMP-9 enzymatic activity in NGAL-null ATC cells. Enzymatic immunoassays show that MMP-9 activity is reduced in NGAL-null ATC cells, even if its expression is not affected by NGAL inhibition. Ectopic expression of NGAL in an ATC cell line not expressing NGAL determines an increase of its metastatic property. The use of a mutated form of NGAL, unable to bind MMP-9, has no positive effect on the invasive potential of ATC cells and does not improve the MMP-9 enzymatic activity. CONCLUSIONS: Our results indicate NGAL as a novel target of nuclear factor-?B prometastatic activity in thyroid cancer through enhancement of MMP-9 enzymatic activity.

Volpe V; Raia Z; Sanguigno L; Somma D; Mastrovito P; Moscato F; Mellone S; Leonardi A; Pacifico F

2013-01-01

212

Increased p53 immunopositivity in anaplastic medulloblastoma and supratentorial PNET is not caused by JC virus  

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Full Text Available Abstract Background p53 mutations are relatively uncommon in medulloblastoma, but abnormalities in this cell cycle pathway have been associated with anaplasia and worse clinical outcomes. We correlated p53 protein expression with pathological subtype and clinical outcome in 75 embryonal brain tumors. The presence of JC virus, which results in p53 protein accumulation, was also examined. Methods p53 protein levels were evaluated semi-quantitatively in 64 medulloblastomas, 3 atypical teratoid rhabdoid tumors (ATRT), and 8 supratentorial primitive neuroectodermal tumors (sPNET) using immunohistochemistry. JC viral sequences were analyzed in DNA extracted from 33 frozen medulloblastoma and PNET samples using quantitative polymerase chain reaction. Results p53 expression was detected in 18% of non-anaplastic medulloblastomas, 45% of anaplastic medulloblastomas, 67% of ATRT, and 88% of sPNET. The increased p53 immunoreactivity in anaplastic medulloblastoma, ATRT, and sPNET was statistically significant. Log rank analysis of clinical outcome revealed significantly shorter survival in patients with p53 immunopositive embryonal tumors. No JC virus was identified in the embryonal brain tumor samples, while an endogenous human retrovirus (ERV-3) was readily detected. Conclusion Immunoreactivity for p53 protein is more common in anaplastic medulloblastomas, ATRT and sPNET than in non-anaplastic tumors, and is associated with worse clinical outcomes. However, JC virus infection is not responsible for increased levels of p53 protein.

Eberhart Charles G; Chaudhry Aneeka; Daniel Richard W; Khaki Leila; Shah Keerti V; Gravitt Patti E

2005-01-01

213

Mitotic count in seminomas--an unreliable criterion for distinguishing between classical and anaplastic types.  

UK PubMed Central (United Kingdom)

Testicular seminomas occur in various forms of which the classical, the spermatocytic, and that with syncytiotrophoblastic giant cells are distinctly defined. Anaplastic seminomas, however, are less clearly distinguished. Forty-five seminomas, 39 pure and 6 combined tumors, were examined from the perspective of the current definition that 3 or more mitoses per high power field (m/hpf) distinguish the anaplastic from the classical form. Our resultant yield of over 80% of "anaplastic" seminomas is clearly incompatible with general clinical experience, indicating that the arbitrary criterion of 3 m/hpf does not segregate anaplastic forms from neoplasms that are mitotically active but relatively non-aggressive. Moreover, the evidence indicates that mitotic activity does not adequately define a tumor form that is near the undifferentiated end of the spectrum which extends from "embryonal carcinoma" to the spermatocytic type. If it should prove, however, that the mitotic rate must be used as an arbitrary watershed criterion until a more reliable one is found, it should then be set at more than 5 m/hpf, yielding a percentage of anaplastic seminomas (about 9%) that is compatible with clinical experience.

von Hochstetter AR

1981-01-01

214

Studies of Phosphoproteomic Changes Induced by Nucleophosmin-Anaplastic Lymphoma Kinase (ALK) Highlight Deregulation of Tumor Necrosis Factor (TNF)/Fas/TNF-related Apoptosis-induced Ligand Signaling Pathway in ALK-positive Anaplastic Large Cell Lymphoma*  

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The oncogenic fusion protein nucleophosmin-anaplastic lymphoma kinase (NPM-ALK), found exclusively in a subset of ALK-positive anaplastic large cell lymphoma, promotes tumorigenesis by exerting its constitutively active tyrosine kinase activity. Thus, characterization of the NPM-ALK-induced changes ...

Wu, Fang; Wang, Peng; Zhang, Jingdong; Young, Leah C.; Lai, Raymond; Li, Liang

215

Twin resemblances in creativity and in esthetic and emotional expression.  

UK PubMed Central (United Kingdom)

Three sources of observation relevant to the measurement of individual differences in emotional and esthetic expressiveness were employed to study their heritability by application to a sample of some 60 pairs of young adult like-sexed twins, approximately evenly divided between male and female and MZ and DZ pairs. The sources of observation were objective test performances, trait ascription using a standard list of adjectives, and videotaped enactments of mood and esthetic performances. Perceptual and esthetic abilities do appear to have substantial heritability, although esthetic preferences do not. Heritability is also indicated for such adjectives as artistic, inventive, original, and independent. Ratings of the videotape performances yielded somewhat ambiguous results, due to the presence of a marked halo effect; the most likely interpretation congruent with earlier results is that greater MZ twin resemblances in social extroversion generated greater resemblances in the videotape situation on such other trait-rating variables as creativity, naturalness, and dominance.

Barron F; Parisi P

1976-01-01

216

Facial resemblances between heterosexual, gay, and lesbian couples.  

UK PubMed Central (United Kingdom)

Researchers have noted a physical resemblance (homophily) between human sex partners. To date, these studies and their related interpretations have been based on heterosexual couples. The present study compared physical resemblances between gay, lesbian, and heterosexual couples, using 40 photographs of each from national newspapers, which were rated by 34 men and 56 women (M age = 53 yr., SD = 12.1). Half the photographs were of actual couples and half were randomly mixed within each group. Actual couples were rated as significantly more similar in appearance than random pairings of people. Ratings of similarity were significantly higher (indicating greater perceived homophily) for gay couples than heterosexual couples, while there was no statistically significant difference in similarity ratings between lesbian couples versus gay and heterosexual couples. The results were interpreted in terms of evolutionary and parental imprinting hypotheses.

Abel EL; Kruger ML

2011-06-01

217

Facial resemblances between heterosexual, gay, and lesbian couples.  

Science.gov (United States)

Researchers have noted a physical resemblance (homophily) between human sex partners. To date, these studies and their related interpretations have been based on heterosexual couples. The present study compared physical resemblances between gay, lesbian, and heterosexual couples, using 40 photographs of each from national newspapers, which were rated by 34 men and 56 women (M age = 53 yr., SD = 12.1). Half the photographs were of actual couples and half were randomly mixed within each group. Actual couples were rated as significantly more similar in appearance than random pairings of people. Ratings of similarity were significantly higher (indicating greater perceived homophily) for gay couples than heterosexual couples, while there was no statistically significant difference in similarity ratings between lesbian couples versus gay and heterosexual couples. The results were interpreted in terms of evolutionary and parental imprinting hypotheses. PMID:21879614

Abel, Ernest L; Kruger, Michael L

2011-06-01

218

Subacute Combined Degeneration Resembling Radiculopathy: Two Case Reports  

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Full Text Available The neurologic manifestations of vitamin B12 deficiency are the result of its effects on the brain, optic nerves, peripheral nerves and spinal cord. Subacute combined degeneration of the spinal cord is a complication of vitamin B12 deficiency, which is reversible if diagnosed and treated early. We present two cases of subacute combined degeneration who resemble radiculopathy. Turk J Phys Med Rehab 2008;54:174-6.

Murat TERZ?; Tülay TERZ?; Berna TANDER; Ferhan CANTÜRK; Musa ONAR

2008-01-01

219

Lingual Tuberculosis Clinically Resembling as a Neoplasm - A Case Report  

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Full Text Available Lingual tuberculosis is a very rare case in theareas where tuberculosis is endemic. There arediagnostic difficulties in patients presentingwith non healing ulcer over the tongue due tovariety of clinical appearances, most of whichmay clinically resemble malignant lingual neo-plasm. Here we report a case of lingual nonhealing ulcer in a 40 years male which was clini-cally diagnosed as malignant ulcer but histopa-thology and ZN staining confirmed the diagno-sis of lingual tuberculosis.

Smita S. Shete; Jayashri A. Khiste; Nandkumar M. Deshpande; Gopal A. Pandit

2013-01-01

220

Giant lymph node hyperplasia resembling abdominal abscess on gallium scan  

International Nuclear Information System (INIS)

[en] A case of giant lymph node hyperplasia with systemic symptoms resembling chronic infection is described which showed intense gallium uptake and indistinguishable from uptake seen in an abscess. This rare syndrome may mimic an abdominal abscess and should be considered in the differential diagnosis of patients without prior history of abdominal surgery and in whom an abdominal abscess is suspected. The condition is relatively benign

1978-01-01

 
 
 
 
221

Viral Plasmacytosis (Aleutian Disease) of Mink Resembling Human Collagen Disease  

Science.gov (United States)

A disease in mink has been discovered that has many of the features of collagen diseases in man. Affected animals suffer from wasting with leukopenia and thrombocytopenia as well as plasma cell infiltration, hypergammaglobulinemia, glomerulonephritis, arteritis and amyloidosis. Cell-free filtrates and ultracentrifugates from diseased animals induced the disease in normal mink, and aleutian genotypes were unusually susceptible to infection. This genotype was characterized by abnormal lysosomal structures in all the granule-forming cells, resembling the Chediak-Higashi syndrome of man. Anti-?-globulin factors similar to human rheumatoid factors were reported, although tests for antibodies such as ANF and LE factors have been negative. Arteritis and glomerulonephritis lesions stained positively for ?-globulin, and Coombs-type sensitized red cells have been detected in the majority of affected mink. Some mink develop a monodispersion of hypergammaglobulinemia resembling the serum protein changes in human myeloma. These studies highlight genetic, immunological and microbiological causative factors in a mink disorder resembling human collagen disease. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5Fig. 6

Gordon, Duncan A.; Franklin, Arthur E.; Karstad, Lars

1967-01-01

222

Viral plasmacytosis (Aleutian disease) of mink resembling human collagen disease.  

UK PubMed Central (United Kingdom)

A disease in mink has been discovered that has many of the features of collagen diseases in man. Affected animals suffer from wasting with leukopenia and thrombocytopenia as well as plasma cell infiltration, hypergammaglobulinemia, glomerulonephritis, arteritis and amyloidosis. Cell-free filtrates and ultracentrifugates from diseased animals induced the disease in normal mink, and aleutian genotypes were unusually susceptible to infection. This genotype was characterized by abnormal lysosomal structures in all the granule-forming cells, resembling the Chediak-Higashi syndrome of man. Anti-gamma-globulin factors similar to human rheumatoid factors were reported, although tests for antibodies such as ANF and LE factors have been negative. Arteritis and glomerulonephritis lesions stained positively for gamma-globulin, and Coombs-type sensitized red cells have been detected in the majority of affected mink. Some mink develop a monodispersion of hypergammaglobulinemia resembling the serum protein changes in human myeloma. These studies highlight genetic, immunological and microbiological causative factors in a mink disorder resembling human collagen disease.

Gordon DA; Franklin AE; Karstad L

1967-05-01

223

Supratentorial extraventricular anaplastic ependymoma in an adult with repeated intratumoral hemorrhage.  

UK PubMed Central (United Kingdom)

We report the case of a 61-year-old man with supratentorial extraventricular anaplastic ependymoma who presented with repeated intratumoral hemorrhage. The patient was admitted with headache. Computed tomography and magnetic resonance imaging showed an enhancing mass with intratumoral hemorrhage in the right temporal lobe. Gross total resection was performed. The tumor was well demarcated from the brain tissue, and showed no continuity with the ventricular system. Histopathological examination revealed the features of anaplastic ependymoma. Therefore, additional radiation therapy and adjuvant chemotherapy were administered. Ten months later, the tumor recurred with hemorrhage in the spinal canal. This case showed rapid malignant progression and repeated intratumoral hemorrhage within a short period of time, both of which are characteristics of anaplastic ependymomas. Close observation of the central nervous system and adjuvant radiotherapy are mandatory, even if the ependymoma presents with repeated intratumoral hemorrhage.

Iwamoto N; Murai Y; Yamamoto Y; Adachi K; Teramoto A

2013-04-01

224

A case of pleomorphic xanthoastrocytoma with anaplastic features in the pineal gland.  

UK PubMed Central (United Kingdom)

BACKGROUND AND IMPORTANCE: Different types of tumor have been reported in the pineal gland, but pleomorphic xanthoastrocytoma (PXA) in this region is extremely rare. CLINICAL PRESENTATIONS: A 61-year-old man had gait disturbance and dementia for 1 month. Radiological examination revealed a 22 × 26 × 22-mm-diameter mass in the pineal gland and remarkable hydrocephalus. Biopsy of the tumor was performed and histological examination confirmed diagnosis of PXA with anaplastic features. Radiation therapy with concomitant temozolomide was performed, and tumor reduction was achieved. CONCLUSION: We report the first case of PXA with anaplastic features in the pineal gland. This case indicates that temozolomide and radiation therapy are effective for treating PXA with anaplastic features.

Katayama K; Asano K; Shimamura N; Ogasawara Y; Naraoka M; Ohkuma H; Kurose A

2013-03-01

225

ALK negative anaplastic large cell lymphoma of the oral cavity showing spontaneous regression  

Directory of Open Access Journals (Sweden)

Full Text Available Anaplastic large cell lymphoma is a subset of T-cell lymphoma which is rarely seen in the oral cavity. This entity may be either primary cutaneous or systemic and the prognosis varies significantly by subtype. In addition, several similar entities have been reported which may mimic this lesion both clinically and histologically. We present a case of anaplastic large cell lymphoma, ALK negative, on the mucosal aspect of the upper lip of an 88 year-old female with a history of cutaneous T-cell lymphoma which demonstrated spontaneous regression after biopsy. A brief review of the literature along with an overview of clinical behavior, histologic presentation, immunohistochemical analysis, genetics, and differential diagnosis of this subtype of anaplastic large cell lymphoma are presented.

Sarah G Fitzpatrick; Leah M Bowers; Samer Z Al-Quran; Eric G Fox; Donald M Cohen; Indraneel Bhattacharyya

2012-01-01

226

A case of pleomorphic xanthoastrocytoma with anaplastic features in the pineal gland.  

Science.gov (United States)

BACKGROUND AND IMPORTANCE: Different types of tumor have been reported in the pineal gland, but pleomorphic xanthoastrocytoma (PXA) in this region is extremely rare. CLINICAL PRESENTATIONS: A 61-year-old man had gait disturbance and dementia for 1 month. Radiological examination revealed a 22 × 26 × 22-mm-diameter mass in the pineal gland and remarkable hydrocephalus. Biopsy of the tumor was performed and histological examination confirmed diagnosis of PXA with anaplastic features. Radiation therapy with concomitant temozolomide was performed, and tumor reduction was achieved. CONCLUSION: We report the first case of PXA with anaplastic features in the pineal gland. This case indicates that temozolomide and radiation therapy are effective for treating PXA with anaplastic features. PMID:23460303

Katayama, Kosuke; Asano, Kenichiro; Shimamura, Norihito; Ogasawara, Yukari; Naraoka, Masato; Ohkuma, Hiroki; Kurose, Akira

2013-03-01

227

Occult multifocal papillary thyroid microcarcinoma presenting as a supraclavicular mass containing anaplastic thyroid carcinoma.  

UK PubMed Central (United Kingdom)

IMPORTANCE: There are reports in the literature of anaplastic thyroid carcinoma in cervical lymph nodes with evidence of only papillary carcinoma in the thyroid gland. There have been no cases of this clinical scenario with only papillary microcarcinoma in the thyroid gland. OBSERVATIONS: We describe the case of a 60-year-old man who initially presented with an enlarged right, level 5, supraclavicular lymph node. Initial fine-needle aspiration demonstrated evidence of papillary thyroid carcinoma. The final pathologic finding in the thyroid gland showed only multiple foci of papillary thyroid microcarcinoma. The index neck mass showed evidence of anaplastic thyroid carcinoma. CONCLUSIONS AND RELEVANCE: This is the first instance in the literature in which anaplastic thyroid carcinoma has appeared in metastatic cervical lymph nodes with only a focus of papillary microcarcinoma in the thyroid gland. With this case, we hope to build awareness of this rare finding.

Deutschmann M; Khalil M; Bhayana S; Chandarana S

2013-04-01

228

The prognosis of anaplastic astrocytoma with radiologic necrosis mimicking glioblastoma.  

UK PubMed Central (United Kingdom)

Anaplastic astrocytoma (AA) sometimes shows a rapid poor course like glioblastoma. In this study, we investigated the prognosis of AA with radiologic necrosis which is the representative radiologic finding of glioblastoma. From 1995 to 2010, we operated on 26 patients who were confirmed to have AA. The male:female ratio was 13:13, and the median age was 47.23 years. The mean follow-up period was 3 years. We analyzed the prognostic significance of radiologic necrosis with age, sex, KPS, tumour location, radiologic findings, extent of removal and radiation therapy oncology group recursive partitioning analysis (RTOG-RPA) classification. The median progression-free survival (PFS) was 0.5 (± 0.17) years and the median overall survival (OS) was 1.6 (± 0.40) years. In univariate analysis, the clinical variables of younger age (p = 0.030) and RTOG-RPA class III (p = 0.043) correlated with longer PFS, and KPS (p = 0.038), radiologic necrosis (p = 0.013) and the extent of removal (p = 0.041) correlated with OS. The median OS was 1.0 (± 0.21) year in AA with radiologic necrosis compared to AA without radiologic necrosis, which showed 2.1 (± 0.29) years median OS. On multivariate analysis, there was no statistically significant prognostic factor. However, Cox's regression model revealed that gross total removal was associated with a longer OS (hazard ratio = 0.136; 95% CI, 0.018 to 1.046; p = 0.055) compared to partial removal or biopsy. Gross total resection was associated with good prognosis, and AA with radiologic necrosis had poor prognosis like glioblastoma.

Kim SD; Jung TY; Jung S; Kim IY; Jang WY; Moon KS; Jeong EH

2013-02-01

229

Anaplastic meningioma: case report Meningioma anaplásico: relato de caso  

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Full Text Available Intracranial meningiomas continue to challenge our best clinical efforts to eliminate them once discovered and deemed appropriate for treatment. Malignant meningiomas constitute 10% to 15% of all meningiomas and limited information exists regarding adjuvant treatment. The external whole brain irradiation is recommended. Traditional chemotherapy has proven ineffective; thus, new chemotherapeutic agents and new methods of delivery should be developed. Immunotherapy may be considered for patients with malignant meningiomas when all others previous treatment have failed. We report a case of anaplastic papillary meningioma. A 67-year-old man presented with partial complex seizures, headache and aggressiveness. A computerized tomography and magnetic resonance image demonstrated a large left temporo-occipital mass with difuse contrast enhancement and extensive surrounding edema. A left temporo-occipital flap was performed. The tumor and the infiltrated dura were radically removed. Postoperatively, the patient remained neurologically intact. The treatment was complemented by external whole brain radiation.O tratamento adequado para os pacientes com meningiomas intracranianos continua sendo um desafio, principalmente o de sua variante maligna, a qual tem incidência de 10% a 15%, sem uma certeza do melhor tratamento adjuvante. É indicado o uso da radioterapia externa holocraniana. O uso da quimioterapia tradicional se mostra ineficaz, havendo necessidade de estudos para desenvolver outros agentes quimioterápicos e novos métodos de administração desses agentes no tumor cerebral. A imunoterapia pode ser considerada para os casos de refratariedade aos outros tratamentos adjuvantes. Relatamos o caso de um paciente de 67 anos, com história progressiva de cefaléia, crises convulsivas parciais complexas e agressividade. A investigação radiológica com tomografia computadorizada e ressonância magnética evidenciaram um processo expansivo na região temporoccipital esquerda com contrastação difusa e edema peritumoral importante. Foi realizada craniotomia frontoparietotemporal esquerda com remoção radical da dura-máter infiltrada e do tumor. O paciente evoluiu sem déficit neurológico no pós-operatório. O exame anatomopatológico foi compatível com meningioma maligno do tipo papilar. Foi instituído tratamento complementar com radioterapia externa holocraniana.

Asdrubal Falavigna; José Augusto Nasser dos Santos; Leila Chimelli; Fernando Antonio Patriani Ferraz; Antonio de Padua Furquim Bonatelli

2001-01-01

230

Pretherapeutic drug evaluation by tumor xenografting in anaplastic thyroid cancer.  

UK PubMed Central (United Kingdom)

BACKGROUND: Despite various attempts at modifying usual treatment modalities, anaplastic thyroid cancer (ATC) is still associated with unfavorable prognosis. Results of preclinical investigations are often of limited transferability to clinical tumor biology. Individualized multimodal treatment regimens, including novel growth-inhibiting drugs, might be a future option. METHODS: Tumor tissue, freshly prepared from a patient operated for ATC, was xenotransplanted to nude mice. While the patient obtained a hyperfractionated external beam radiation, mice carrying xenotransplanted tumors were randomized (n = 6) and treated by multikinase inhibitors (sorafenib [S]: vascular endothelial growth factor receptor [VEGF-R], platelet derived growth factor receptor, RET; vandetanib [V]: VEGF-R, endothelial growth factor receptor [EGF-R]; and MLN8054 [M]: Aurora kinases [AK]). Antiproliferative, antiangiogenic, and proapoptotic effects were evaluated. RESULTS: Treatment of successfully xenotransplanted fresh ATC tumor tissue by multikinase inhibitors and aurora kinase inhibitor reduced the tumor volume up to 61% depending on the drug and time of application (3 wk of treatment: 46% [M], 34% [V], 30% [S]; 5 wk of treatment: 61% [S]). Tumor cell proliferation (BrdU) was reduced between 34% and 58% [S] and [V]. Reduction of tumor vascularity was between 67% [V] and 33% [S] and was accompanied by decreased EGF-R/VEGF-R2 receptor activity [V/V,S]. Tumor cell apoptosis (caspase 3 activity) increased up to 2.4-fold [S]. CONCLUSIONS: Successful in vivo evaluation of novel drugs in xenotransplanted fresh tumor tissue allows in-time (while patient receives standard treatment) prospective analysis for possible additional clinical application. However, technical specifications have to be taken into account to obtain stable in vivo tumor growth. Based on the individual results, a tailored clinical drug application seems possible.

Wunderlich A; Khoruzhyk M; Roth S; Ramaswamy A; Greene BH; Doll D; Bartsch DK; Hoffmann S

2013-06-01

231

Combined chemotherapy and irradiation in anaplastic thyroid carcinoma  

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Full Text Available Background: Anaplastic thyroid carcinoma (ATC) is a very rare and extremely aggressive cancer; patient's death usually occurs rapidly after diagnosis with a mean survival of six months in the majority of individual research series. Treatment of ATC ranges from surgery, radiotherapy, chemotherapy, or a combination of these regimes. Yet, the optimal sequence of treatment modalities has not been established. Methods: From 1997 to 2002 six consecutive patients with a histological diagnosis of ATC were treated with combined chemotherapy and irradiation at our Clinic for Oncology, Clinical Center Ni¹. Five of these patients were females and 1 male, aged between 28 and 71 years (mean age: 57 years). None of them had distant metastases at the time of diagnosis. Extrathyroidal extension was present in 3 patients with invasion into skin and hypoderm. Treatment consisted of doxorubicin 60 mg/m 2 plus cisplatin 60 mg/m 2 every three weeks. Total doses ranged between 158-375 mg/m 2 for doxorubicin and 183-380 mg/m 2 for cisplatin. External beam radiation to the neck was administered, at a daily dose of 1.2 Gy, up to total doses ranging between 45-60 Gy. Results: One patient achieved a complete response (CR) and one patient achieved a partial response (PR). Three patients had stable disease. One patient with CR progressed during follow-up and died 18 months from bone and brain metastases. The treatment was moderately well tolerated, although all patients experienced some mild form of toxicity; neutropenia occurred in all patients, but none of them required hospital admission. Median survival was 8 months (range: 4-18 months). Conclusion: We concluded that the present regimen produces meaningful responses for patients with localized ATC. A randomized study is needed to determine the effect on survival.

Pej?i? Ivica; Vrbi? Svetislav; Š?eki? Mirjana

2003-01-01

232

Critical appraisal of clinicopathological features of anaplastic carcinoma small intestine – A rare case report  

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Full Text Available Anaplastic carcinoma is an extremely rare variant of small intestine adenocarcinoma with only an occasional case having been reported in literature to date. We report a case of 46 year old male who presented with an endophytic tumor in ileum extending through full thickness of intestinal wall, with invasion into mesenteric fat. Microscopically, the tumor showed a biphasic growth pattern unlike conventional adenocarcinoma. The present case discusses the histomorphology of anaplastic carcinoma small intestine, its histochemical and immunohistochemical profile and the differential diagnosis of this rare but highly aggressive pathological entity. 

VANDANA PURI

2012-01-01

233

Dual ALK and MYC rearrangements leading to an aggressive variant of anaplastic large cell lymphoma.  

UK PubMed Central (United Kingdom)

Anaplastic lymphoma kinase (ALK) and MYC are oncogenes often dysregulated in pediatric lymphomas. NPM-ALK/t(2;5)(p23;q35) is a genetic hallmark of ALK anaplastic large cell lymphoma (ALCL). MYC gene translocations are frequently detected in high-grade B-cell lymphomas. ALKALCL cases with concurrent MYC translocation are exceedingly rare and are more aggressive and chemoresistent compared with other ALKALCL. We report a patient who presented with ALKALCL possessing coexistent MYC rearrangement, massive tumor dissemination, and early widespread relapse. This case underscores the importance of recognition of close correlation between dual ALK and MYC rearrangements and the characteristic clinical features in this unusual ALCL variant.

Liang X; Branchford B; Greffe B; McGavran L; Carstens B; Meltesen L; Albano EA; Quinones R; Cook B; Graham DK

2013-07-01

234

An infant with prenatally diagnosed congenital anaplastic astrocytoma who remains disease-free after proton therapy.  

UK PubMed Central (United Kingdom)

The authors present a rare of prenatally diagnosed congenital anaplastic astrocytoma. A 9-month-old boy had three recurrences despite two surgical resections and various chemotherapeutic regimens. He underwent the 3rd gross tumor removal at 11 months of age, followed by proton therapy, and now he remains disease-free for 3 yr without a significant neurocognitive dysfunction. This is the 1st case of a pediatric tumor treated by proton therapy in Korea, and proton therapy may be a treatment of choice for a congenital anaplastic astrocytoma in infants and young children, considering limitation of radiation therapy.

Shin HJ; Kwon YJ; Park HJ; Park BK; Shin SH; Kim JY; Lee SH; Kim HS; Kim DW

2013-09-01

235

Anaplastic large cell anaplastic lymphoma kinase + non-Hodgkin lymphoma in a 10-year-old male discovered during dental visit: a case report.  

Science.gov (United States)

Non-Hodgkin lymphoma is rare in children, even though it is the third most frequent type of tumour. Management of a child with non-Hodgkin lymphoma is complex and coordinate presence of haematologists, surgeons, radiotherapists, neurologists, psychologists and other expert personnel is required. Our patient presented with a bulky mass of approximately 5 cm in diameter, which grew from his upper-left maxillary bone, thereby causing gum bulge. Thus, the left side of his face from lip to eye appeared swollen. A whole body computed tomography-positron emission tomography examination revealed that the mass was growing in the mouth and maxillary bone and that many bulky nodes were present both in the right and left neck. Histological haematoxylin and eosin assessment revealed an anaplastic large cell proliferation underlying the epithelial tissue without epithelial infiltration. The patient underwent a first cycle of chemotherapy according to the 'International Protocol of anaplastic large cell lymphoma'. The Maxillofacial Surgery Unit and the Dentistry Unit of the same hospital took care of his dental situation to avoid the spread of infective foci to the entire body. After 1 year and 3 months from the first cycle of chemotherapy, a bulky splenic mass was discovered. Laparoscopic biopsy revealed a relapse of the anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in a splenic node. The patient is now alive in good conditions, and continuously followed-up by the Pediatric and Hemato-oncology Operative Unit of University Hospital of Parma. Rapidity of action and a correct multidisciplinary (oncology-maxillofacial surgery-dentistry) approach is the key to cure illness, promptly diagnose relapse and avoid the spread of infective foci from the patient's teeth during chemotherapy cycles. PMID:23296283

Guida, A; Meleti, M; Vescovi, P; Serpico, R; Lucchese, A; Lo Muzio, L; Bufo, P; Pannone, G

2012-12-07

236

Anaplastic large cell anaplastic lymphoma kinase + non-Hodgkin lymphoma in a 10-year-old male discovered during dental visit: a case report.  

UK PubMed Central (United Kingdom)

Non-Hodgkin lymphoma is rare in children, even though it is the third most frequent type of tumour. Management of a child with non-Hodgkin lymphoma is complex and coordinate presence of haematologists, surgeons, radiotherapists, neurologists, psychologists and other expert personnel is required. Our patient presented with a bulky mass of approximately 5 cm in diameter, which grew from his upper-left maxillary bone, thereby causing gum bulge. Thus, the left side of his face from lip to eye appeared swollen. A whole body computed tomography-positron emission tomography examination revealed that the mass was growing in the mouth and maxillary bone and that many bulky nodes were present both in the right and left neck. Histological haematoxylin and eosin assessment revealed an anaplastic large cell proliferation underlying the epithelial tissue without epithelial infiltration. The patient underwent a first cycle of chemotherapy according to the 'International Protocol of anaplastic large cell lymphoma'. The Maxillofacial Surgery Unit and the Dentistry Unit of the same hospital took care of his dental situation to avoid the spread of infective foci to the entire body. After 1 year and 3 months from the first cycle of chemotherapy, a bulky splenic mass was discovered. Laparoscopic biopsy revealed a relapse of the anaplastic lymphoma kinase-positive anaplastic large cell lymphoma in a splenic node. The patient is now alive in good conditions, and continuously followed-up by the Pediatric and Hemato-oncology Operative Unit of University Hospital of Parma. Rapidity of action and a correct multidisciplinary (oncology-maxillofacial surgery-dentistry) approach is the key to cure illness, promptly diagnose relapse and avoid the spread of infective foci from the patient's teeth during chemotherapy cycles.

Guida A; Meleti M; Vescovi P; Serpico R; Lucchese A; Lo Muzio L; Bufo P; Pannone G

2012-01-01

237

Fluorine F 18 Fluorodopa-Labeled PET Scan in Planning Surgery and Radiation Therapy in Treating Patients With Newly Diagnosed High- or Low-Grade Malignant Glioma  

Science.gov (United States)

Adult Anaplastic Astrocytoma; Adult Anaplastic Ependymoma; Adult Anaplastic Oligodendroglioma; Adult Brain Stem Glioma; Adult Diffuse Astrocytoma; Adult Ependymoma; Adult Giant Cell Glioblastoma; Adult Glioblastoma; Adult Gliosarcoma; Adult Mixed Glioma; Adult Myxopapillary Ependymoma; Adult Oligodendroglioma; Adult Pilocytic Astrocytoma; Adult Pineal Gland Astrocytoma; Adult Subependymal Giant Cell Astrocytoma; Adult Subependymoma

2012-10-16

238

An orthotopic mouse model of anaplastic thyroid carcinoma.  

UK PubMed Central (United Kingdom)

Several types of animal models of human thyroid carcinomas have been established, including subcutaneous xenograft and orthotopic implantation of cancer cells into immunodeficient mice. Subcutaneous xenograft models have been valuable for preclinical screening and evaluation of new therapeutic treatments. There are a number of advantages to using a subcutaneous model; 1) rapid, 2) reproducible, and 3) tumor establishment, growth, and response to therapeutic agents may be monitored by visual inspection. However, substantial evidence has shed light on the short-comings of subcutaneous xenograft models(1-3). For instance, medicinal treatments demonstrating curative properties in subcutaneous xenograft models often have no notable impact on the human disease. The microenvironment of the site of xenographic transplantation or injection lies at the heart of this dissimilarity. Orthotopic tumor xenograft models provide a more biologically relevant context in which to study the disease. The advantages of implanting diseased cells or tissue into their anatomical origin equivalent within a host animal includes a suitable site for tumor-host interactions, development of disease-related metastases and the ability to examine site-specific influence on investigational therapeutic remedies. Therefore, orthotopic xenograft models harbor far more clinical value because they closely reproduce human disease. For these reasons, a number of groups have taken advantage of an orthotopic thyroid cancer model as a research tool(4-7). Here, we describe an approach that establishes an orthotopic model for the study of anaplastic thyroid carcinoma (ATC), which is highly invasive, resists treatment, and is virtually fatal in all diagnosed patients. Cultured ATC cells are prepared as a dissociated cellular suspension in a solution containing a basement membrane matrix. A small volume is slowly injected into the right thyroid gland. Overall appearance and health of the mice are monitored to ensure minimal post-operative complications and to gauge pathological penetrance of the cancer. Mice are sacrificed at 4 weeks, and tissue is collected for histological analysis. Animals may be taken at later time-points to examine more advance progression of the disease. Production of this orthotopic mouse model establishes a platform that accomplishes two objectives: 1) further our understanding of ATC pathology, and 2) screen current and future therapeutic agents for efficacy in combating ATC.

Sewell W; Reeb A; Lin RY

2013-01-01

239

RESEMBLANCE OPERATIONS AND CONCEPTUAL COMPLEXY IN ANIMAL METAPHORS  

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Full Text Available For over thirty years cognitive linguists have devoted much effort to the study of metaphors based on the correlation of events in human experience to the detriment of the more traditional notion of resemblance metaphor, which exploits perceived similarities among objects. Grady (1999) draws attention to this problem and calls for a more serious study of the latter type of metaphor. The present paper takes up this challenge on the basis of a small corpus of ‘animal’ metaphors in English, which are essentially based on resemblance. Contrary to previous analyses by cognitive linguists (e.g. Lakoff & Turner 1989, Ruiz de Mendoza Ibáñez, 1998), who claim that such metaphors are based on a single mapping generally involving comparable behavioral attributes, I will argue that we have a more complex situation which involves different patterns of conceptual interaction. In this respect, I have identified cases of (i) animal metaphors interacting with high-level (i.e. grammatical) metaphors and metonymies, of (ii) (situational) animal metaphors whose source domains are constructed metonymically (cf. Goossens 1990; Ruiz de Mendoza Ibáñez & Díez Velasco 2002), and of (iii) animal metaphors interacting with other metaphors thereby giving rise to metaphoric amalgams (cf. Ruiz de Mendoza Ibáñez & Galera Masegosa 2011).

Aneider Iza Ervitia

2012-01-01

240

Resemblance operations and conceptual complexity in animal metaphors  

Directory of Open Access Journals (Sweden)

Full Text Available For over thirty years cognitive linguists have devoted much effort to the study of metaphors based on the correlation of events in human experience to the detriment of the more traditional notion of resemblance metaphor, which exploits perceived similarities among objects. Grady (1999) draws attention to this problem and calls for a more serious study of the latter type of metaphor. The present paper takes up this challenge on the basis of a small corpus of ‘animal’ metaphors in English, which are essentially based on resemblance. Contrary to previous analyses by cognitive linguists (e.g. Lakoff & Turner 1989, Ruiz de Mendoza Ibáñez, 1998), who claim that such metaphors are based on a single mapping generally involving comparable behavioral attributes, I will argue that we have a more complex situation which involves different patterns of conceptual interaction. In this respect, I have identified cases of (i) animal metaphors interacting with high-level (i.e. grammatical) metaphors and metonymies, of (ii) (situational) animal metaphors whose source domains are constructed metonymically (cf. Goossens 1990; Ruiz de Mendoza Ibáñez & Díez Velasco 2002), and of (iii) animal metaphors interacting with other metaphors thereby giving rise to metaphoric amalgams (cf. Ruiz de Mendoza Ibáñez & Galera Masegosa 2011).

Aneider Iza Ervitia

2012-01-01

 
 
 
 
241

Extending disorder: essentialism, family resemblance and secondary sense.  

UK PubMed Central (United Kingdom)

It is commonly thought that mental disorder is a valid concept only in so far as it is an extension of or continuous with the concept of physical disorder. A valid extension has to meet two criteria: determination and coherence. Essentialists meet these criteria through necessary and sufficient conditions for being a disorder. Two Wittgensteinian alternatives to essentialism are considered and assessed against the two criteria. These are the family resemblance approach and the secondary sense approach. Where the focus is solely on the characteristics or attributes of things, both these approaches seem to fail to meet the criteria for valid extension. However, this focus on attributes is mistaken. The criteria for valid extension are met in the case of family resemblance by the pattern of characteristics associated with a concept, and by the limits of intelligibility of applying a concept. Secondary sense, though it may have some claims to be a good account of the relation between physical and mental disorder, cannot claim to meet the two criteria of valid extension.

Pickering N

2013-05-01

242

A visual latent semantic approach for automatic analysis and interpretation of anaplastic medulloblastoma virtual slides.  

UK PubMed Central (United Kingdom)

A method for automatic analysis and interpretation of histopathology images is presented. The method uses a representation of the image data set based on bag of features histograms built from visual dictionary of Haar-based patches and a novel visual latent semantic strategy for characterizing the visual content of a set of images. One important contribution of the method is the provision of an interpretability layer, which is able to explain a particular classification by visually mapping the most important visual patterns associated with such classification. The method was evaluated on a challenging problem involving automated discrimination of medulloblastoma tumors based on image derived attributes from whole slide images as anaplastic or non-anaplastic. The data set comprised 10 labeled histopathological patient studies, 5 for anaplastic and 5 for non-anaplastic, where 750 square images cropped randomly from cancerous region from whole slide per study. The experimental results show that the new method is competitive in terms of classification accuracy achieving 0.87 in average.

Cruz-Roa A; González F; Galaro J; Judkins AR; Ellison D; Baccon J; Madabhushi A; Romero E

2012-01-01

243

Tumor Stroma in Anaplastic Thyroid Carcinoma : Interstitial Collagen and Tumor Interstitial Fluid Pressure  

Digital Repository Infrastructure Vision for European Research (DRIVER)

Anaplastic thyroid carcinoma (ATC) is an aggressive malignancy in man with stromal fibrosis as one of the main features. Carcinoma cells synthesized no or little collagen I protein. Pro-?1(I) collagen mRNA was expressed by stromal cells throughout the tumor, but expression of procollagen type I p...

Lammerts, Ellen

244

Overexpression of c-erbB2 is a negative prognostic factor in anaplastic astrocytomas  

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The epidermal growth factor receptor (EGFR) family, consisting of four tyrosine kinase receptors, c-erbB1-4, seems to be influential in gliomagenesis. The aim of this study was to investigate EGFR gene amplification and expression of c-erbB1-4 receptor proteins in human anaplastic astrocytomas. Form...

Gulati, Sasha; Ytterhus, Borgny; Granli, Unn S; Gulati, Michel; Lydersen, Stian; Torp, Sverre H

245

Anaplastic Lymphoma Kinase-Positive Large B-Cell Lymphoma: An Underrecognized Aggressive Lymphoma  

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Anaplastic lymphoma kinase-(ALK-) positive large B-cell lymphoma (ALK+ LBCL) is a rare, aggressive tumor characterized by an immunoblastic or plasmablastic morphologic appearance, expression of ALK, CD138, CD45, EMA, and often IgA by immunohistochemistry, and characteristic chromosomal translocation...

Morgan, Elizabeth A.; Nascimento, Alessandra F.

246

Anaplastic large-cell lymphoma with florid granulomatous reaction: A case report and review of literature  

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Granulomatous reactions have been reported in association with lymphomas, more often with Hodgkins disease than with Non-Hodgkins Lymphoma. Not many reports are available on the association of anaplastic large-cell lymphoma with sarcoid-type granuloma. Herein, we report a case of an elderly female w...

Balamurugan S; Rajasekar B; Rao R

247

Meta-Analysis of Glioblastoma Multiforme versus Anaplastic Astrocytoma Identifies Robust Gene Markers  

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Background: Anaplastic astrocytoma (AA) and its more aggressive counterpart, glioblastoma multiforme (GBM), are the most common intrinsic brain tumors in adults and are almost universally fatal. A deeper understanding of the molecular relationship of these tumor types is necessary to derive insights...

Dreyfuss, Jonathan M; Johnson, Mark Damone; Park, Peter J.

248

An integrated texton and bag of words classifier for identifying anaplastic medulloblastomas.  

Science.gov (United States)

In this paper we present a combined Bag of Words and texton based classifier for differentiating anaplastic and non-anaplastic medulloblastoma on digitized histopathology. The hypothesis behind this work is that histological image signatures may reflect different levels of aggressiveness of the disease and that texture based approaches can help discriminate between more aggressive and less aggressive phenotypes of medulloblastoma. The bag of words approach attempts to model the occurrence of differently expressed image features. In this work we choose to model the image features via textons which can quantitatively capture and model texture appearance in the images. The texton-based features, obtained via two methods, the Haar Wavelet responses and MR8 filter bank, provide spatial orientation and rotation invariant attributes. Applying these features to the bag of words framework yields textural representations that can be used in conjunction with a classifier (?-nearest neighbor) or a content based image retrieval system. Over multiple runs of randomized cross validation, a ?-NN classifier in conjunction with Haar wavelets and the texton, bag of words approach yielded a mean classification accuracy of 80, an area under the precision recall curve of 87 and an area under the ROC curve of 83 in distinguishing between anaplastic and non-anaplastic medulloblastomas on a cohort of 36 patient studies. PMID:22255080

Galaro, Joseph; Judkins, Alexander R; Ellison, David; Baccon, Jennifer; Madabhushi, Anant

2011-01-01

249

An integrated texton and bag of words classifier for identifying anaplastic medulloblastomas.  

UK PubMed Central (United Kingdom)

In this paper we present a combined Bag of Words and texton based classifier for differentiating anaplastic and non-anaplastic medulloblastoma on digitized histopathology. The hypothesis behind this work is that histological image signatures may reflect different levels of aggressiveness of the disease and that texture based approaches can help discriminate between more aggressive and less aggressive phenotypes of medulloblastoma. The bag of words approach attempts to model the occurrence of differently expressed image features. In this work we choose to model the image features via textons which can quantitatively capture and model texture appearance in the images. The texton-based features, obtained via two methods, the Haar Wavelet responses and MR8 filter bank, provide spatial orientation and rotation invariant attributes. Applying these features to the bag of words framework yields textural representations that can be used in conjunction with a classifier (?-nearest neighbor) or a content based image retrieval system. Over multiple runs of randomized cross validation, a ?-NN classifier in conjunction with Haar wavelets and the texton, bag of words approach yielded a mean classification accuracy of 80, an area under the precision recall curve of 87 and an area under the ROC curve of 83 in distinguishing between anaplastic and non-anaplastic medulloblastomas on a cohort of 36 patient studies.

Galaro J; Judkins AR; Ellison D; Baccon J; Madabhushi A

2011-01-01

250

[Hemophagocytic lymphohistiocytosis associated with a lymphohistiocytic pattern anaplastic large cell lymphoma: A case report].  

UK PubMed Central (United Kingdom)

We report the case of a 16-year-old girl with an anaplastic large cell lymphoma of lymphohistiocytic pattern revealed by a hemophagocytic syndrome. Histologically, the lymphomatous population was concealed by clusters of histiocytes. Immunohistochemical study allowed the diagnosis. The combination of these two entities is rarely described and may be a source of delay in diagnosis of a life-threatening condition.

Sizaret D; Lecointre C; Kerdraon R; Bléchet C; Bonneau C; Alexis M; Camus F; Michenet P

2013-08-01

251

Management of acute clinical presentation of anaplastic thyroid cancer. A difficult choice.  

UK PubMed Central (United Kingdom)

AIM: evaluate emergency treatment of patients presenting with anaplastic thyroid cancer with acute compressive sympthoms. MATERIAL OF STUDY: In the present report, we describe three patients with anaplastic thyroid cancer who presented clinically with acute compressive sympthoms. All patients underwent debulking surgery in order to relieve the sympthoms and perform a potentially curative resection. RESULTS: The first two patients with diagnosis of ATC couldn't undergo to radiotherapy and chemotherapy for the presence of infection of the wound and for the earlier disease relapse in the mediastinum and died after 1 month and 3 months. The third patient (with papillary thyroid cancer with focal areas of anaplastic cancer) after surgical treatment underwent external radiotherapy and is alive over 2 years. DISCUSSION: Compression, deviation, and infiltration of trachea have impeded the achievement of a safe respiratory access with tracheostomy and these three patients underwent debulking surgery to relieve compressive sympthoms. These three cases confirm the importance of acute local compressive symptoms of ATC and their difficult management. CONCLUSIONS: In such cases a debulking surgery can be considered as a valid option in the emergency management. Nevertheless debulking surgery (and even radical excision of the tumor), when performed alone, offer a minimal improved survival. KEY WORDS: Anaplastic thyroid cancer, Debulking surgery, Emergency treatment.

Biffoni M; Garritano S; Scipioni P; Colangelo M; De Meo D; Monti M

2013-03-01

252

Recurrent anaplastic medulloblastoma in cerebrospinal fluid after autologous hematopoietic stem cell transplant.  

Science.gov (United States)

Cerebrospinal fluid (CSF) dissemination can be seen relatively frequently in medulloblastomas and its presence at diagnosis is important for determining both treatment and prognosis. The anaplastic variant is an aggressive variant of medulloblastoma with characteristic histopathologic features and unfavorable prognosis that was included in the latest WHO classification. Herein, we report the CSF cytologic features of a case of recurrent anaplastic medulloblastoma in a 17-year-old male patient who had undergone autologous hematopoietic stem cell transplant as a part of the treatment protocol. The malignant cells were large, had high nucleocytoplasmic ratios, and highly pleomorphic, frequently polylobated nuclei with coarse chromatin and 1-3 visible nucleoli. These CSF cytologic features differed significantly from those of classic medulloblastoma, which usually shows small cells with rounded, rather uniform nuclei with fine ("blastic") chromatin. The differential diagnosis of anaplastic medulloblastoma is broader than that of classic medulloblastoma, as it includes metastatic carcinomas and large cell lymphoma, a differential diagnosis especially pertinent in this patient with a history of autologous hematopoietic stem cell transplant. Awareness of these unusual but distinctive cytologic features is important for the accurate diagnosis of anaplastic medulloblastomas in CSF specimens and to avoid possible diagnostic pitfalls. Diagn. Diagn. Cytopathol. 2013;41:980-985. © 2012 Wiley Periodicals, Inc. PMID:22550044

Nelson, Andrew C; Singh, Charanjeet; Brent Clark, H; Pambuccian, Stefan E

2012-04-30

253

Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database.  

UK PubMed Central (United Kingdom)

Anaplastic ganglioglioma (AGG) are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated potential clinicopathological factors that can affect outcome in patients with anaplastic ganglioglioma. Patients with anaplastic ganglioglioma diagnosed between 1973 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient age at diagnosis, gender, race, tumour location, uni-focal or multi-focal tumour, surgical resection and the use of adjuvant radiotherapy. Fifty-eight patients were identified, with a median age at diagnosis of 25.5 years. Ninety-three percent of patients underwent surgery and 36% received adjuvant radiotherapy. The median overall survival was 28.5 months. The most common tumour site was the temporal lobe (27%). Univariate and multivariate analysis identified surgery and uni-focal disease as important predictors of overall survival. Adjuvant radiotherapy did not influence overall survival. This study represents the largest analysis of anaplastic ganglioglioma to date. Furthermore it also emphasises the role of national tumour databases for furthering our understanding of rare brain tumours and determining management options.

Selvanathan SK; Hammouche S; Salminen HJ; Jenkinson MD

2011-12-01

254

Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database.  

Science.gov (United States)

Anaplastic ganglioglioma (AGG) are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated potential clinicopathological factors that can affect outcome in patients with anaplastic ganglioglioma. Patients with anaplastic ganglioglioma diagnosed between 1973 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient age at diagnosis, gender, race, tumour location, uni-focal or multi-focal tumour, surgical resection and the use of adjuvant radiotherapy. Fifty-eight patients were identified, with a median age at diagnosis of 25.5 years. Ninety-three percent of patients underwent surgery and 36% received adjuvant radiotherapy. The median overall survival was 28.5 months. The most common tumour site was the temporal lobe (27%). Univariate and multivariate analysis identified surgery and uni-focal disease as important predictors of overall survival. Adjuvant radiotherapy did not influence overall survival. This study represents the largest analysis of anaplastic ganglioglioma to date. Furthermore it also emphasises the role of national tumour databases for furthering our understanding of rare brain tumours and determining management options. PMID:21626070

Selvanathan, Senthil K; Hammouche, Salah; Salminen, Heidi J; Jenkinson, Michael D

2011-05-29

255

Anaplastic ganglioglioma in the spinal cord: case report and literature review.  

Science.gov (United States)

Anaplastic ganglioglioma (AGG) is a rare tumor. A PubMed database search yielded only a few case reports and fewer case series. An even rarer entity is AGG arising in the spinal cord. We present a case of a pediatric patient with a pathological diagnosis of spinal AGG. PMID:23689037

Kuten, Jonathan; Kaidar-Person, Orit; Vlodavsky, Eugene; Postovsky, Sergey; Billan, Salem; Kuten, Abraham; Bortnyak-Abdah, Roxolyana

2013-05-16

256

Anaplastic ganglioglioma in the spinal cord: case report and literature review.  

UK PubMed Central (United Kingdom)

Anaplastic ganglioglioma (AGG) is a rare tumor. A PubMed database search yielded only a few case reports and fewer case series. An even rarer entity is AGG arising in the spinal cord. We present a case of a pediatric patient with a pathological diagnosis of spinal AGG.

Kuten J; Kaidar-Person O; Vlodavsky E; Postovsky S; Billan S; Kuten A; Bortnyak-Abdah R

2012-01-01

257

Primary cutaneous anaplastic large cell lymphoma arising from lymphomatoid papulosis, responding to low dose methotrexate  

Directory of Open Access Journals (Sweden)

Full Text Available CD301 cutaneous lymphoproliferative disorders (CLPDs) present variable clinical and histological manifestations. We report here a case of an adult male patient who progressed from lymphomatoid papulosis to anaplastic large cell lymphoma. The patient responded satisfactorily to a low dose of methotrexate.

Nandini A; Mysore Venkatram; Sacchidanand S; Chandra Suresh

2009-01-01

258

Primary cutaneous anaplastic large cell lymphoma arising from lymphomatoid papulosis, responding to low dose methotrexate  

Digital Repository Infrastructure Vision for European Research (DRIVER)

CD301 cutaneous lymphoproliferative disorders (CLPDs) present variable clinical and histological manifestations. We report here a case of an adult male patient who progressed from lymphomatoid papulosis to anaplastic large cell lymphoma. The patient responded satisfactorily to a low dose of methotre...

Nandini A; Mysore Venkatram; Sacchidanand S; Chandra Suresh

259

Primary Cutaneous Anaplastic Large Cell Lymphoma Arising from Lymphomatoid Papulosis, Responding to Low Dose Methotrexate  

Digital Repository Infrastructure Vision for European Research (DRIVER)

CD30+ cutaneous lymphoproliferative disorders (CLPDs) present variable clinical and histological manifestations. We report here a case of an adult male patient who progressed from lymphomatoid papulosis to anaplastic large cell lymphoma. The patient responded satisfactorily to a low dose of methotre...

Nandini, AS; Mysore, Venkatram; Sacchidanand, S; Chandra, Suresh

260

Combined temozolomide and radiation as an initial treatment for anaplastic glioma.  

UK PubMed Central (United Kingdom)

AIM: Combined temozolomide (TMZ) and radiation therapy (RT) is often used as initial treatment for anaplastic glioma. However, there is no prospective randomized data available that proves the efficacy of the combination for anaplastic glioma. In this retrospective study we aimed to compare the outcome of patients who had combined TMZ and RT with those who had RT alone for the initial treatment of anaplastic glioma in our centers. METHODS: Patients with anaplastic astrocytoma or oligoastrocytoma treated at our centers between 2000 and 2010 were reviewed. Only patients who received initial RT or concurrent TMZ and RT (TMZ-RT) were included. RESULTS: Of 62 patients, 55 were less than 66-years old; 36 (58.1%) had a tumor resection and 26 had a biopsy only. An oligodendroglial component in their tumor histology was present in 21 patients (33.9%). At a median follow up of 20.7 months for all patients, median progression-free survival was similar for the two treatment groups (RT alone: 16.7 months (95% CI 9.4, 34.8 months) versus TMZ-RT: 14.8 months (95% CI 8.6, 28.6 months, P?=?NS). Median overall survival was 27.4 months (95% CI 10.6, not estimable [NE] months) for patients who had RT alone and 34.1 months (95% CI 19.8, 42.1 months) for those who had TMZ-RT. CONCLUSION: No significant benefit of combined TMZ with RT compared to RT alone was observed as the initial treatment of anaplastic glioma. Prospective randomized trials are needed to evaluate the optimal treatment for this disease.

Tham CK; See SJ; Tan SH; Lim KH; Ng WH; Thomas J; Chong DQ; Chua ET

2013-09-01

 
 
 
 
261

A case of cervical radiation radiculopathy resembling motor neuron disease  

International Nuclear Information System (INIS)

[en] A 67-year-old man developed slowly progressive muscular weakness in the bilateral upper extremities (C5-7 regions) without signs of sensory deficit following the cervical radiation therapy (70.5 Gy) for right laryngeal cancer 4 years before. These clinical signs resembled those of lower motor neuron disease. MRI with gadolinium-DTPA, however, showed enhancement in the bilateral C5 and C6 anterior roots, suggesting the cervical radiculopathy due to radiotherapy. It is known that radiation to the spinal cord can lead to ''selective anterior horn cell injury''. This is the first case report of the cervical radiation radiculopathy, which, if without MRI, might be classified into selective anterior horn cell injury. Suggestion is made for the hypothesis that the spinal motoneuron loss in radiation myelopathy would be caused by retrograde degeneration due to anterior root damages. (author)

1998-01-01

262

Paraspinal Gossypiboma Resembling an Abscess: A Case Report  

Directory of Open Access Journals (Sweden)

Full Text Available A 45-year-old man presented with complaints of low back pain, sciatalgia on the  left persisting for five months and fluid leakage from the wound. He had been operated for L4-5 disc herniation before seven months. Computed tomography (CT) and magnetic resonance imaging (MRI) detected a contrasted mass lesion resembling abscess formation localized in the left posterior paravertebral region. Paravertebral abscess was diagnosed and aspiration was planned under local anesthesia with ultrasonography guidance. During intervention, a needle was inserted into cavity but enough material was not aspirated. Thus, surgery was plannedand a retained sponge material was found within the left paraspinal region  and removed totally. The patient healed without complication.

Süleyman Coþkun

2013-01-01

263

Autosomal dominant syndrome resembling Coffin-Siris syndrome.  

UK PubMed Central (United Kingdom)

Coffin-Siris syndrome is a multiple congenital anomaly/mental retardation syndrome with phenotypic variability [OMIM 135900]. The diagnosis is based solely on clinical findings, as there is currently no molecular, biochemical, or cytogenetic analysis available to confirm a diagnosis. Although typically described as an autosomal recessive disorder, autosomal dominant inheritance has also been infrequently reported. We describe a mother and her two daughters who all have features that resemble Coffin-Siris syndrome. However, this is not a completely convincing diagnosis given that hypertelorism is not a feature of Coffin-Siris syndrome and the family is relatively mildly affected. Yet, this family provides further evidence of an autosomal dominant mode of inheritance for a likely variant of Coffin-Siris syndrome (at least in some families). In addition, Sibling 1 had premature thelarche. She is the second reported individual within the spectrum of Coffin-Siris syndrome to have premature thelarche, indicating that it may be a rare clinical feature.

Flynn MA; Milunsky JM

2006-06-01

264

Rare synchronous association of vestibular schwannoma and indolent insular oligodendroglioma in a patient without neurofibromatosis: controversial issue of timing for surgical treatment of asymptomatic low-grade gliomas  

Directory of Open Access Journals (Sweden)

Full Text Available Maurizio Iacoangeli,1 Alessandro Di Rienzo,1 Roberto Colasanti,1 Lorenzo Alvaro,1 Niccolò Nocchi,1 Gabriele Polonara,2 Lucia Giovanna Maria Di Somma,1 Antonio Zizzi,3 Marina Scarpelli,3 Massimo Scerrati11Department of Neurosurgery, 2Department of Radiology, Section of Neuroradiology, 3Department of Pathology, Università Politecnica delle Marche, Umberto I General Hospital, Ancona, ItalyAbstract: The co-occurrence of a vestibular schwannoma and a low-grade glioma is rare, and even rarer is the association with an oligodendroglioma. Although various authors have addressed the problem of treating patients with incidentally discovered indolent low-grade gliomas, an established protocol does not exist to date. The common approach is to reserve surgery until there is radiological evidence of tumor growth or high-grade transformation. However, because incidental low-grade glioma may represent the first stage of unavoidable pathological progression towards high-grade glioma, early and radical surgical resection should be advocated in order to increase the chance of a "cure" and prolonged survival. This case report supports this view, and suggests reflection on a possible change from a conservative philosophy to preventative surgical treatment.Keywords: multiple primary intracranial tumors, vestibular schwannoma, oligodendroglioma, indolent low grade gliomas, incidentaloma, surgery

Iacoangeli M; Di Rienzo A; Colasanti R; Alvaro L; Nocchi N; Polonara G; Di Somma LG; Zizzi A; Scarpelli M; Scerrati M

2012-01-01

265

Oligodendroglioma cells synthesize the differentiation-specific linker histone H1? and release it into the extracellular environment through shed vesicles.  

UK PubMed Central (United Kingdom)

Chromatin remodelling can be involved in some of the epigenetic modifications found in tumor cells. One of the mechanisms at the basis of chromatin dynamics is likely to be synthesis and incorporation of replacement histone variants, such as the H1? linker histone. Regulation of the expression of this protein can thus be critical in tumorigenesis. In developing brain, H1? expression is mainly regulated at the post-transcriptional level and RNA-binding proteins (RBPs) are involved. In the past, attention mainly focused on the whole brain or isolated neurons and little information is available on H1? expression in other brain cells. Even less is known relating to tumor glial cells. In this study we report that, like in maturing brain and isolated neurons, H1? synthesis sharply increases in differentiating astrocytes growing in a serum-free medium, while the corresponding mRNA decreases. Unexpectedly, in tumor glial cells both H1? RNA and protein are highly expressed, in spite of the fact that H1? is considered a differentiation-specific histone variant. Persistence of H1? mRNA in oligodendroglioma cells is accompanied by high levels of H1? RNA-binding activities which seem to be present, at least in part, also in actively proliferating, but not in differentiating, astrocytes. Finally, we report that oligodendroglioma cells, but not astrocytes, release H1? protein into the culture medium by shedding extracellular vesicles. These findings suggest that deregulation of H1? histone expression can be linked to tumorigenesis.

Schiera G; Di Liegro CM; Saladino P; Pitti R; Savettieri G; Proia P; Di Liegro I

2013-10-01

266

Id4 and FABP7 are preferentially expressed in cells with astrocytic features in oligodendrogliomas and oligoastrocytomas  

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Full Text Available Abstract Background Oligodendroglioma (ODG) and oligoastrocytoma (OAC) are diffusely infiltrating primary brain tumors whose pathogenesis remains unclear. We previously identified a group of genes whose expression was inversely correlated with survival in a cohort of patients with glioblastoma (GBM), and some of these genes are also reportedly expressed in ODG and OAC. We examined the expression patterns and localization of these survival-associated genes in ODG and OAC in order to analyze their possible roles in the oncogenesis of these two tumor types. Methods We used UniGene libraries derived from GBM and ODG specimens to examine the expression levels of the transcripts for each of the 50 GBM survival-associated genes. We used immunohistochemistry and cDNA microarrays to examine expression of selected survival-associated genes and Id4, a gene believed to control the timing of oligodendrocyte development. The expression of FABP7 and Id4 and the survival of patients with ODG and OAC were also analyzed. Results Transcripts of most survival-associated genes as well as Id4 were present in both GBM and ODG tumors, whereas protein expression of Id4 and one of the survival-associated genes, brain-type fatty acid-binding protein (FABP7), was present in cells with astrocytic features, including reactive and neoplastic astrocytes, but not in neoplastic oligodendrocytes. Id4 was co-expressed with FABP7 in microgemistocytes in ODG and in neoplastic astrocytes in OAC. Id4 and FABP7 expression, however, did not correlate with the clinical outcome of patients with ODG or OAC tumors. Conclusion Expression of Id4 and some of our previously identified GBM survival-associated genes is present in developing or mature oligodendrocytes. However, protein expression of Id4 and FABP7 in GBM, ODG, and OAC suggests that this group of functionally important genes might demonstrate two patterns of expression in these glioma subtypes: one group is universally expressed in glioma cells, and the other group of genes is expressed primarily in neoplastic astrocytes but not in neoplastic oligodendrocytes. Differential protein expression of these two groups of genes in ODG and OAC may be related to the cellular origins and the histological features of the neoplastic cells.

Liang Yu; Bollen Andrew W; Nicholas M Kelly; Gupta Nalin

2005-01-01

267

Phase I/II Trial Of Super-Selective Intraarterial Infusion Of Erbitux and Bevacizumab For Treatment Of Relapsed/Refractory Intracranial Glioma In Patients Under 22 Years Of Age  

Science.gov (United States)

Glioblastoma Multiforme; Fibrillary Astrocytoma of Brain; Glioma of Brainstem; Anaplastic Astrocytoma; Pilomyxoid Astrocytoma; Mixed Oligodendroglioma-Astrocytoma; Brain Stem Glioma; Diffuse Intrinsic Pontine Glioma; High Grade Glioma

2013-07-31

268

Extended retroperitoneal necrotizing fasciitis with genital involvement, resembling fournier gangrene.  

UK PubMed Central (United Kingdom)

BACKGROUND: Necrotizing fasciitis is a serious infection that originates in the subcutaneous tissues. Although many reports have been published about necrotizing infections of other anatomical sites, retroperitoneal necrotizing soft tissue infection is a rare entity that has been described in only a few case reports. The etiology and clinical course of retroperitoneal necrotizing fasciitis can be variable and it is often difficult to identify the etiology of the infective process. CASE REPORT: We report a 58-year-old man with rapidly progressive, gas-producing, necrotizing inflammation in the retroperitoneum, complicated with genital involvement resembling Fournier gangrene. The patient was managed successfully by aggressive drainage, debridement, and sequential laparotomies to track and control the extensive necrosis of the retroperitoneum and perineum, in addition to systemic care to control sepsis. After his general condition stabilized, early rectosigmoid adenocarcinoma was identified and resected curatively. He remained well at follow up, six months after discharge. RESULTS: In retrospect, the trigger of the disease process was unclear. Although it was believed possibly to be due to the colon lesion, adenocarcinoma of the rectosigmoid colon was identified and the patient was managed successfully. CONCLUSIONS: Similar to necrotizing infections at other anatomical sites, early diagnosis and timely surgical intervention and systemic antimicrobial therapy are mandatory for treating patients with retroperitoneal necrotizing fasciitis.

Sugimoto M; Matsuura K; Takayama H; Kayo M; Ie T

2010-10-01

269

Malignant myoepithelioma of the vulva resembling a rhabdoid tumour.  

UK PubMed Central (United Kingdom)

AIMS: We report an example of malignant myoepithelioma of the vulva, which has not been hitherto described. We discuss the differential diagnosis and briefly review the literature. METHODS AND RESULTS: The lesion was found in an 81-year-old woman as an indolent 40 mm tumour. The neoplastic cells showed a myoid, spindled, epithelioid and plasmacytoid phenotype. Hyalinization of extracellular material and myxoid changes were present. There was a partly solid and microcystic pattern and a tight cohesiveness of cells was lacking. The circumscribed multinodular tumour somewhat resembled an extrarenal rhabdoid tumour, having large tumour cells with prominent nucleoli and large amounts of acidophilic cytoplasm. Immunohistochemically, the tumour cells were immunoreactive for cytokeratin, vimentin, muscle-specific actin, alpha-smooth muscle actin, and S100 protein, but not for desmin, epithelial membrane antigen, factor VIII-related antigen, CD34 and CD31. CONCLUSIONS: The histological and cytomorphological appearance of the tumour well as the immunohistochemical findings suggest the diagnosis of malignant myoepithelioma, possibly derived from minor vestibulary glands or ectopic breast tissue. Differential diagnoses are, in particular, extrarenal rhabdoid tumour and 'proximal type' epithelioid sarcoma. Differentiation is important, because the tumours show a different behaviour and prognosis.

Hinze P; Feyler S; Berndt J; Knolle J; Katenkamp D

1999-07-01

270

Autosomal dominant syndrome resembling Coffin-Siris syndrome.  

Science.gov (United States)

Coffin-Siris syndrome is a multiple congenital anomaly/mental retardation syndrome with phenotypic variability [OMIM 135900]. The diagnosis is based solely on clinical findings, as there is currently no molecular, biochemical, or cytogenetic analysis available to confirm a diagnosis. Although typically described as an autosomal recessive disorder, autosomal dominant inheritance has also been infrequently reported. We describe a mother and her two daughters who all have features that resemble Coffin-Siris syndrome. However, this is not a completely convincing diagnosis given that hypertelorism is not a feature of Coffin-Siris syndrome and the family is relatively mildly affected. Yet, this family provides further evidence of an autosomal dominant mode of inheritance for a likely variant of Coffin-Siris syndrome (at least in some families). In addition, Sibling 1 had premature thelarche. She is the second reported individual within the spectrum of Coffin-Siris syndrome to have premature thelarche, indicating that it may be a rare clinical feature. PMID:16691594

Flynn, Maureen A; Milunsky, Jeff M

2006-06-15

271

Brainstem anaplastic glioma in patients with AIDS: a case report and review of the literature.  

UK PubMed Central (United Kingdom)

AIDS is an increasingly common diagnosis seen by neurologists and neurosurgeons alike. Although the more common brain lesions associated with AIDS are due to central nervous system lymphomas, toxoplasma encephalitis or progressive multifocal leukoencephalopathy, relatively recent clinical evidence has shown that AIDS-independent cerebral tumours can arise as well, albeit less commonly. Previous incidents have been reported with HIV and AIDS patients presenting with cerebral astrocytomas. To our knowledge, there has never been a report in the literature of a brainstem anaplastic glioma occurring in an AIDS or HIV patient. We report a 55-year-old patient with HIV and brainstem anaplastic glioma. Its presentation, diagnostic difficulty, scans, histology and subsequent treatment are discussed. We also review the relevant literature on gliomas in HIV/AIDS patients.

Chaudhry NS; Ahmad FU; Blieden C; Benveniste RJ

2013-01-01

272

The role of chemotherapy and latest emerging target therapies in anaplastic thyroid cancer.  

UK PubMed Central (United Kingdom)

Anaplastic thyroid cancer represents 1%-2% of thyroid cancers. For its aggressiveness, it is considered a systemic disease at the time of diagnosis. Surgery remains the cornerstone of therapy in resectable tumor. Traditional chemotherapy has little effect on metastatic disease. A multimodality approach, incorporating cytoreductive surgical resection, chemoradiation, either concurrently or sequentially, and new promising target therapies is advisable. Doxorubicin is the most commonly used agent, with a response rate of 22%. Recently, other chemotherapy agents have been used, such as paclitaxel and gemcitabine, with superimposable activity and response rates of 10%-20%. However, survival of patients with anaplastic thyroid cancer has changed little in the past 50 years, despite more aggressive systemic and radiotherapies. Several new agents are currently under investigation. Some of them, such as sorafenib, imatinib, and axitinib have been tested in small clinical trials, showing promising disease control rates ranging from 35%-75%. Referral of patients for participation in clinical trials is needed.

Denaro N; Nigro CL; Russi EG; Merlano MC

2013-01-01

273

The role of chemotherapy and latest emerging target therapies in anaplastic thyroid cancer  

Science.gov (United States)

Anaplastic thyroid cancer represents 1%–2% of thyroid cancers. For its aggressiveness, it is considered a systemic disease at the time of diagnosis. Surgery remains the cornerstone of therapy in resectable tumor. Traditional chemotherapy has little effect on metastatic disease. A multimodality approach, incorporating cytoreductive surgical resection, chemoradiation, either concurrently or sequentially, and new promising target therapies is advisable. Doxorubicin is the most commonly used agent, with a response rate of 22%. Recently, other chemotherapy agents have been used, such as paclitaxel and gemcitabine, with superimposable activity and response rates of 10%–20%. However, survival of patients with anaplastic thyroid cancer has changed little in the past 50 years, despite more aggressive systemic and radiotherapies. Several new agents are currently under investigation. Some of them, such as sorafenib, imatinib, and axitinib have been tested in small clinical trials, showing promising disease control rates ranging from 35%–75%. Referral of patients for participation in clinical trials is needed.

Denaro, Nerina; Nigro, Cristiana Lo; Russi, Elvio G; Merlano, Marco C

2013-01-01

274

Spinal intradural, extramedullary anaplastic ependymoma with an extradural component: Case report and review of the literature.  

UK PubMed Central (United Kingdom)

BACKGROUND: There have been 18 reported cases of primary spinal intradural, extramedullary ependymomas reported in the literature. One of the 18 cases had an extradural component and was benign. Our case is the second spinal intradural, extramedullary ependymoma with an extradural component and the first with its initial presentation as an anaplastic ependymoma. CASE DESCRIPTION: A 50-year-old male presented with bilateral upper thigh weakness and thoracic numbness. His exam showed the pin-prick level at T5. Magnetic resonance imaging (MRI) of the thoracic spine showed an enhancing lesion at T5-6 with severe compression of the spinal cord with a dumbbell shape extension of the tumor through the right T5-6 neural foramen. The patient had a laminectomy at T4-T6 with the resection of the tumor. Postoperatively, the patient regained full strength in his lower extremities. Intraoperatively, the tumor was found to be intradural, extramedullary with an extradural component. The tumor was found to be an anaplastic ependymoma. CONCLUSIONS: Even though spinal intradural extramedullary ependymomas are very rare, surgeons must be aware that on MRI, they can be mistaken for meningiomas or nerve sheath tumors especially if there is an extradural component. Our case report is the first intradural, extramedullary ependymoma that is anaplastic and has an extradural component. A review of the literature provides little information on the treatment and prognosis for these tumors especially if they are anaplastic. We propose that the treatment, as done in our case, should be complete resection of the tumor with spinal radiotherapy to the tumor level.

Guppy KH; Hou L; Moes GS; Sahrakar K

2011-01-01

275

Long-term follow-up of surgical treatment of spinal anaplastic astrocytoma in a cat.  

UK PubMed Central (United Kingdom)

A 10-year-old spayed female chinchilla feline presented with gradually progressive tetraparesis and cervical pain that had begun 1 month before the onset of a 4-day tetraplegic episode. Magnetic resonance imaging revealed a large elliptical intramedullary mass at the fourth cervical vertebrae. The mass was removed surgically and diagnosed as an anaplastic astrocytoma. No neurological abnormalities were observed 3 weeks postsurgery. Magnetic resonance at 3.5 year follow-up revealed neither mass regrowth nor recurrence of signs.

Tamura S; Hori Y; Tamura Y; Uchida K

2013-10-01

276

Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: A case report and review of literature  

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Full Text Available We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male. On initial presentation, the patient had a WHO Grade II tumor. However, at recurrence 1 year later, the tumor progressed to WHO Grade III tumor with more cellularity, necrosis and brisk mitotic activity. Chondroid metaplasia was present in both the initial and recurrent tumors.

Ghosal Nandita; Murthy Ganesh; Dadlani Ravi; Hegde Alangar; Singh Devendra

2010-01-01

277

Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma.  

Science.gov (United States)

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by third spacing of plasma into the extravascular compartment, leading to anasarca, hemoconcentration, and hypovolemic shock. It has been rarely associated with lymphomas, and reports usually indicate that it occurs after antineoplastic treatment. We present the case of a patient with ALK-negative anaplastic large cell lymphoma who presented with SCLS as the initial manifestation of her lymphoma. The SCLS resolved with treatment of the malignancy with steroids and chemotherapy. PMID:22953081

Lourdes, Laura S; Al-Quran, Samer Z; Dang, Nam H; Markham, Merry-Jennifer

2012-03-26

278

Systemic Capillary Leak Syndrome as an Initial Presentation of ALK-Negative Anaplastic Large Cell Lymphoma.  

UK PubMed Central (United Kingdom)

Systemic capillary leak syndrome (SCLS) is a rare disease characterized by third spacing of plasma into the extravascular compartment, leading to anasarca, hemoconcentration, and hypovolemic shock. It has been rarely associated with lymphomas, and reports usually indicate that it occurs after antineoplastic treatment. We present the case of a patient with ALK-negative anaplastic large cell lymphoma who presented with SCLS as the initial manifestation of her lymphoma. The SCLS resolved with treatment of the malignancy with steroids and chemotherapy.

Lourdes LS; Al-Quran SZ; Dang NH; Markham MJ

2012-01-01

279

Differential proteomic and phenotypic behaviour of papillary and anaplastic thyroid cell lines.  

UK PubMed Central (United Kingdom)

Thyroid carcinomas account for a minority of all malignant tumours but, after those of the gonads, they represent the most common forms of endocrine cancers. They include several types, among which the papillary thyroid cancer (PTC) and the anaplastic thyroid cancer (ATC) are the best known. The two hystotypes display significant biological and clinical differences: PTC is a well differentiated form of tumour with a high incidence and a good prognosis, while the ATC is less frequent but represents one of the most aggressive endocrine tumours with morphological features of an undifferentiated type. To date, as far as we know, no conclusive studies, useful to design arrays of molecular markers, have been published illustrating the phenotypic and proteomic differences between these two tumours. The aim of this work was to perform a comparative analysis of two thyroid cancer cell lines, derived respectively from papillary (BCPAP) and anaplastic (8505C) thyroid carcinomas. The comparative analysis included cell behaviour assays and proteomic analysis by 2D-PAGE and mass spectrometry. The results have highlighted a new proteomic signature for the anaplastic carcinoma-derived cells, consistent with their high proliferation rate, motility propensity and metabolic shift, in relation to the well-differentiated PTC cells. This article is part of a Special Issue entitled: From Genome to Proteome: Open Innovations.

Musso R; Di Cara G; Albanese NN; Marabeti MR; Cancemi P; Martini D; Orsini E; Giordano C; Pucci-Minafra I

2013-09-01

280

Differential proteomic and phenotypic behaviour of papillary and anaplastic thyroid cell lines.  

Science.gov (United States)

Thyroid carcinomas account for a minority of all malignant tumours but, after those of the gonads, they represent the most common forms of endocrine cancers. They include several types, among which the papillary thyroid cancer (PTC) and the anaplastic thyroid cancer (ATC) are the best known. The two hystotypes display significant biological and clinical differences: PTC is a well differentiated form of tumour with a high incidence and a good prognosis, while the ATC is less frequent but represents one of the most aggressive endocrine tumours with morphological features of an undifferentiated type. To date, as far as we know, no conclusive studies, useful to design arrays of molecular markers, have been published illustrating the phenotypic and proteomic differences between these two tumours. The aim of this work was to perform a comparative analysis of two thyroid cancer cell lines, derived respectively from papillary (BCPAP) and anaplastic (8505C) thyroid carcinomas. The comparative analysis included cell behaviour assays and proteomic analysis by 2D-PAGE and mass spectrometry. The results have highlighted a new proteomic signature for the anaplastic carcinoma-derived cells, consistent with their high proliferation rate, motility propensity and metabolic shift, in relation to the well-differentiated PTC cells. This article is part of a Special Issue entitled: From Genome to Proteome: Open Innovations. PMID:23385357

Musso, Rosa; Di Cara, Gianluca; Albanese, Nadia Ninfa; Marabeti, Maria Rita; Cancemi, Patrizia; Martini, Dèsirèe; Orsini, Ester; Giordano, Carla; Pucci-Minafra, Ida

2013-02-04

 
 
 
 
281

Identification of ALK germline mutation (3605delG) in pediatric anaplastic medulloblastoma.  

UK PubMed Central (United Kingdom)

The anaplastic lymphoma kinase (ALK) gene has been found either rearranged or mutated in several neoplasms such as anaplastic large-cell lymphoma, non-small-cell lung cancer, neuroblastoma and anaplastic thyroid cancer. Medulloblastoma (MB) is an embryonic pediatric cancer arising from nervous system, a tissue in which ALK is expressed during embryonic development. We performed an ALK mutation screening in 52 MBs and we found a novel heterozygous germline deletion of a single base in exon 23 (3605delG) in a case with marked anaplasia. This G deletion results in a frameshift mutation producing a premature stop codon in exon 25 of ALK tyrosine kinase domain. We also screened three human MB cell lines without finding any mutation of ALK gene. Quantitative expression analysis of 16 out of 52 samples showed overexpression of ALK mRNA in three MBs. In the present study, we report the first mutation of ALK found in MB. Moreover, a deletion of ALK gene producing a stop codon has not been detected in human tumors up to now. Further investigations are now required to elucidate whether the truncated form of ALK may have a role in signal transduction.

Coco S; De Mariano M; Valdora F; Servidei T; Ridola V; Andolfo I; Oberthuer A; Tonini GP; Longo L

2012-10-01

282

In vitro evaluation of the therapeutic potential of nevirapine in treatment of human thyroid anaplastic carcinoma.  

Science.gov (United States)

Anaplastic thyroid carcinoma (ATC) is a severe thyroid malignancy with poor prognosis, due to its early metastasis and unresponsiveness to both radiation and chemotherapy. Nevirapine, a non-nucleoside reverse transcriptase inhibitor, has been used as a re-differentiation agent to treat cancers in several human cancer models. So far, the effects of nevirapine on human thyroid anaplastic carcinoma cells have not been documented. The aim of this study was to evaluate the therapeutic potential of nevirapine in treatment of human thyroid anaplastic carcinoma. Cell proliferation was determined by methly thiazolyl tetrazolium (MTT) assay. Cell apoptosis was analyzed by Hoechst 33258 staining. The mRNA expression of NIS and TSHR was determined by real-time quantitative reverse transcription-polymerase chain reaction (real time RT-PCR). Iodine uptake was determined by (125)I radioactivity assay. At all doses (100, 200, 350, 500 ?mol/L) tested, nevirapine significantly inhibited cell proliferation after 48 h treatment. At high dose (500 ?mol/L), nevirapine significantly increased the percentage of apoptotic cells compared with control (PFRO cells pre-treated with nevirapine, the increase in NIS expression had no obvious effect on iodine uptake. These findings indicate that nevirapine has an anti-proliferative effect on FRO cells, which correlates with an induction of cell differentiation. PMID:23462194

Dong, J J; Zhou, Y; Liu, Y T; Zhang, Z W; Zhou, X J; Wang, H J; Liao, L

2013-02-24

283

Frequent expression of FAS/APO-1 in Hodgkin's disease and anaplastic large cell lymphomas.  

UK PubMed Central (United Kingdom)

FAS/APO-1 (CD95) is a membrane glycoprotein belonging to the tumour necrosis factor/nerve growth factor receptor family, and which can trigger apoptosis in some lymphoid cell lines. Immunohistochemistry combined with Northern blotting allowed determination of the pattern of FAS/APO-1 expression in a series of Ki-1 [CD30] positive lymphoid malignancies, including 27 Hodgkin's disease and eight anaplastic large cell lymphomas. CD30 negative tumours used as controls included 27 B-cell non-Hodgkin's lymphomas. 14 T-cell non-Hodgkin's lymphomas, four reactive lymphadenitis, and non-lymphoid tissues. Immunohistochemistry, performed on frozen sections, revealed a strong FAS/APO-1 expression in 25 out of 27 (92%) Hodgkin's disease cases, predominantly in Reed Sternberg cells; 50 to 100% of the neoplastic cells in eight out of (100%) anaplastic large cell lymphoma cases were positive. In contrast, positive FAS/APO-1 immunostaining was observed only in 22 out of 41 (53%) CD30 negative non-Hodgkin's lymphomas. Northern blot analysis detected variable amounts of the FAS/APO-1 transcript in the immunohistochemistry-positive samples. These results suggest possible hyper-expression of FAS/APO-1 (CD95) in Hodgkin's disease and anaplastic large cell lymphomas.

Xerri L; Carbuccia N; Parc P; Hassoun J; Birg F

1995-09-01

284

Predator-Resembling Aversive Conditioning for Managing Habituated Wildlife  

Directory of Open Access Journals (Sweden)

Full Text Available Wildlife habituation near urban centers can disrupt natural ecological processes, destroy habitat, and threaten public safety. Consequently, management of habituated animals is typically invasive and often includes translocation of these animals to remote areas and sometimes even their destruction. Techniques to prevent or reverse habituation and other forms of in situ management are necessary to balance ecological and social requirements, but they have received very little experimental attention to date. This study compared the efficacy of two aversive conditioning treatments that used either humans or dogs to create sequences resembling chases by predators, which, along with a control category, were repeatedly and individually applied to 24 moderately habituated, radio-collared elk in Banff National Park during the winter of 2001–2002. Three response variables were measured before and after treatment. Relative to untreated animals, the distance at which elk fled from approaching humans, i.e., the flight response distance, increased following both human and dog treatments, but there was no difference between the two treatments. The proportion of time spent in vigilance postures decreased for all treatment groups, without differences among groups, suggesting that this behavior responded mainly to seasonal effects. The average distance between elk locations and the town boundary, measured once daily by telemetry, significantly increased for human-conditioned elk. One of the co-variates we measured, wolf activity, exerted counteracting effects on conditioning effects; flight response distances and proximity to the town site were both lower when wolf activity was high. This research demonstrates that it is possible to temporarily modify aspects of the behavior of moderately habituated elk using aversive conditioning, suggests a method for reducing habituation in the first place, and provides a solution for Banff and other jurisdictions to manage hyperabundant and habituated urban wildlife.

Elsabé Louise Kloppers; Colleen Cassady St. Clair; Thomas Eric Hurd

2005-01-01

285

The monoclonal antibody ALK1 identifies a distinct morphological subtype of anaplastic large cell lymphoma associated with 2p23/ALK rearrangements.  

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Anaplastic large cell lymphoma (ALCL) is a heterogeneous group of diseases by morphology, phenotype, genotype, and clinical presentation. Using a new monoclonal antibody (ALK1) that recognizes the native anaplastic lymphoma kinase (ALK) protein as well as the fusion product of the t(2;5)(p23;q35), n...

Pittaluga, S.; Wlodarska, I.; Pulford, K.; Campo, E.; Morris, S. W.; Van den Berghe, H.; De Wolf-Peeters, C.

286

NPM-ALK and the JunB transcription factor regulate the expression of cytotoxic molecules in ALK-positive, anaplastic large cell lymphoma  

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Anaplastic lymphoma kinase-positive, anaplastic large cell lymphoma (ALK+ ALCL) is an aggressive non-Hodgkin lymphoma of T/null immunophenotype that is most prevalent in children and young adults. The normal cellular counterpart of this malignancy is presumed to be the cytotoxic T lymphocyte (CTL), ...

Pearson, Joel D; Lee, Jason KH; Bacani, Julinor TC; Lai, Raymond; Ingham, Robert J

287

Genomic Profiling of Peripheral T-Cell Lymphoma, Unspecified, and Anaplastic Large T-Cell Lymphoma Delineates Novel Recurrent Chromosomal Alterations  

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To characterize genetic alterations in peripheral T-cell lymphoma, not otherwise specified (PTCL NOS), and anaplastic large T-cell lymphoma (ALCL), 42 PTCL NOS and 37 ALCL [17 anaplastic large cell kinase (ALK)-negative ALCL, 9 ALK-positive ALCL, 11 cutaneous ALCL] were analyzed by comparative genom...

Zettl, Andreas; Rüdiger, Thomas; Konrad, Maria-Anette; Chott, Andreas; Simonitsch-Klupp, Ingrid; Sonnen, Ruth; Müller-Hermelink, Hans Konrad

288

Insular and anaplastic carcinoma of the thyroid: A 45-year comparative study at a single institution and a review of the significance of p53 and p21  

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Objective: To analyze the clinicopathologic features of a large cohort of patients with insular or anaplastic carcinomas treated at a single institution. Summary Background Data: Insular and anaplastic carcinomas of the thyroid, although uncommon, have more aggressive clinical behavior than well-dif...

Lam, KY; Lo, CY; Chan, KW; Wan, KY

289

EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma  

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Primary cutaneous CD30(+) lymphoproliferative disorders (CD30(+) LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressiv...

Kempf, W; Pfaltz, K; Vermeer, M H; Cozzio, A; Ortiz-Romero, P L; Bagot, M; Olsen, E; Kim, Y H; Dummer, R; Pimpinelli, N

290

EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma*  

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Primary cutaneous CD30+ lymphoproliferative disorders (CD30+ LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressive co...

Kempf, Werner; Pfaltz, Katrin; Vermeer, Maarten H.; Cozzio, Antonio; Ortiz-Romero, Pablo L.; Bagot, Martine; Olsen, Elise

291

Large cell/anaplastic medulloblastoma: outcome according to myc status, histopathological, and clinical risk factors.  

UK PubMed Central (United Kingdom)

PURPOSE: To evaluate the prognostic impact of large cell/anaplastic (LC/A) histology together with molecular and clinical risk factors in childhood medulloblastoma. METHODS: Three consecutive prospective medulloblastoma trials were screened for patients with the histological diagnosis of LC/A medulloblastoma. Tumors were considered as LC/A if they displayed areas of severe cytological anaplasia or a significant or predominant large cell component. Histology was centrally confirmed. Genomic DNA amplification of c-myc and n-myc, and mRNA expression of c-myc and trkC were analyzed. RESULTS: Twenty-eight patients with LC/A medulloblastoma with a median age of 6.1 years (1.4-16.5 years) and a median follow-up of 4.5 years were identified (5% of all medulloblastoma). Four-year event-free (EFS) and overall survival (OS) were 58% and 67%. Young age and metastases (n = 13, 4-year EFS 31% vs. 82% in 15 children >4 years and without metastases, P = 0.001), large cell histology (n = 9, 4-year EFS 22% vs. 75%, P = 0.005) and c-myc amplification (n = 9, 4-year EFS 22% vs. 89%, P < 0.0001) were negative prognostic factors. C-myc amplification was highly correlated with young age (P < 0.001), metastases (P = 0.002) and large cell histology (P = 0.007). Outcome of 12 patients with severely anaplastic tumors without these risk factors was not impaired (4-year EFS 86%). CONCLUSION: In a subgroup of patients without clinical and molecular risk factors outcome was favorable despite severely anaplastic histology. In contrast, c-myc amplification and large-cell histology were associated with an inferior outcome. Intensified treatment strategies should be considered for children with LC/A medulloblastoma and these characteristics.

von Hoff K; Hartmann W; von Bueren AO; Gerber NU; Grotzer MA; Pietsch T; Rutkowski S

2010-03-01

292

Effect of missense mutations on structure and interaction of anaplastic Lymphoma kinase (ALK) in neuroblastom.  

Science.gov (United States)

Neuroblastoma is a cancer of the sympathetic nervous system, accounting for upto 15% of childhood cancer mortality. It can occur in many areas but most of them begin in the abdomen in the adrenal gland and can spread to the bones and other areas. http://en.wikipedia.org/wiki/Neuroblastoma-cite_note-pmid19383347-3. Unfortunately, like other cancers, its causes are still poorly understood. Anaplastic lymphoma kinase (ALK), a membrane associated tyrosine kinase was recently found to be mutated in neuroblastoma. Protein sequence of ALK was retrieved from UniProt and the seven identified mutations were substituted in native sequence to get its mutant proteins. Significant changes were explored in the mutant secondary structures when compared with the native protein. Changes were also observed in the physiochemical properties and it can therefore be inferred that, these changes may be translated in the tertiary structures due to their effects on the folding pattern. Tertiary structure of the protein modeled after refinement and validation was submitted to Protein Model Database (PMDB) and was assigned with the PMDB ID P0077827. RMSD values of the mutant structures were observed deviated from the native structure when compared with probability < 0.05. It was observed that there are a total of 15 Disordered Regions in the protein having a total of 290 Disordered Residues. Protein-ligand interaction analysis was performed to investigate the effects of mutations damaging its interactions and it was observed that the mutations understudy affects its interactions with ATP which ultimately results in causing neuroblastoma. This study was based on the in silico mutation analysis of Seven missense mutations of anaplastic lymphoma kinase which can better explain why missense mutations in ALK protein cause neuroblastoma. Structure and sequence based computations were systematically and comprehensively evaluated applied to the mutants in anaplastic lymphoma kinase and on the basis of our observations a detailed structural explanations have been developed for the measured and predicted impact of these missense substitutions. PMID:23625438

Kanwal, Hafsah; Khan, Mohammad Haroon; Rashid, Hamid

2013-05-01

293

In vitro evaluation of the therapeutic potential of nevirapine in treatment of human thyroid anaplastic carcinoma.  

UK PubMed Central (United Kingdom)

Anaplastic thyroid carcinoma (ATC) is a severe thyroid malignancy with poor prognosis, due to its early metastasis and unresponsiveness to both radiation and chemotherapy. Nevirapine, a non-nucleoside reverse transcriptase inhibitor, has been used as a re-differentiation agent to treat cancers in several human cancer models. So far, the effects of nevirapine on human thyroid anaplastic carcinoma cells have not been documented. The aim of this study was to evaluate the therapeutic potential of nevirapine in treatment of human thyroid anaplastic carcinoma. Cell proliferation was determined by methly thiazolyl tetrazolium (MTT) assay. Cell apoptosis was analyzed by Hoechst 33258 staining. The mRNA expression of NIS and TSHR was determined by real-time quantitative reverse transcription-polymerase chain reaction (real time RT-PCR). Iodine uptake was determined by (125)I radioactivity assay. At all doses (100, 200, 350, 500 ?mol/L) tested, nevirapine significantly inhibited cell proliferation after 48 h treatment. At high dose (500 ?mol/L), nevirapine significantly increased the percentage of apoptotic cells compared with control (P<0.01). At lower doses (200 ?mol/L and 350 ?mol/L), nevirapine did not induce cell apoptosis, but up-regulated NIS and THSR mRNA expression in a dose-dependent manner. In FRO cells pre-treated with nevirapine, the increase in NIS expression had no obvious effect on iodine uptake. These findings indicate that nevirapine has an anti-proliferative effect on FRO cells, which correlates with an induction of cell differentiation.

Dong JJ; Zhou Y; Liu YT; Zhang ZW; Zhou XJ; Wang HJ; Liao L

2013-05-01

294

Anaplastic large cell lymphoma and breast implants: results from a structured expert consultation process.  

UK PubMed Central (United Kingdom)

BACKGROUND: There are increasing concerns about a possible association between anaplastic large cell lymphoma (ALCL) and breast implants. The authors conducted a structured expert consultation process to evaluate the evidence for the association, its clinical significance, and a potential biological model based on their interpretation of the published evidence. METHODS: A multidisciplinary panel of 10 experts was selected based on nominations from national specialty societies, academic department heads, and recognized researchers in the United States. RESULTS: Panelists agreed that (1) there is a positive association between breast implants and ALCL development but likely underrecognition of the true number of cases; (2) a recurrent, clinically evident seroma occurring 6 months or more after breast implantation should be aspirated and sent for cytologic analysis; (3) anaplastic lymphoma kinase-negative ALCL that develops around breast implants is a clinically indolent disease with a favorable prognosis that is distinct from systemic anaplastic lymphoma kinase-negative ALCL; (4) management should consist of removal of the involved implant and capsule, which is likely to prevent recurrence, and evaluation for other sites of disease; and (5) adjuvant radiation or chemotherapy should not be offered to women with capsule-confined disease. Little agreement, however, was found regarding etiologic risk factors for implant-associated ALCL. CONCLUSION: The authors' assessment yielded consistent results on a number of key issues regarding ALCL in women with breast implants, but substantial further research is needed to improve our understanding of the epidemiology, clinical aspects, and biology of this disease. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, V.

Kim B; Roth C; Young VL; Chung KC; van Busum K; Schnyer C; Mattke S

2011-09-01

295

Anaplastic lymphoma kinase (ALK1) immunohistochemistry in diagnostic dermatopathology; an update.  

UK PubMed Central (United Kingdom)

The use of anaplastic lymphoma kinase antibodies (ALK1) as a diagnostic aid has expanded since becoming a routinely available immunohistochemical stain. Because the skin may be the site of a wide variety of hematolymphoid and fibroblastic proliferations, dermatopathologists commonly use ALK1 as part of a broader staining panel in diagnosing soft tissue and cutaneous hematolymphoid neoplasms. Furthermore, new entities and differential diagnostic contexts are emerging, which broaden the utility of ALK1 immunohistochemistry. We review the expanding role of ALK1 immunohistochemistry in contemporary dermatopathology.

Papalas JA; Kulbacki E; Wang E

2013-06-01

296

Anaplastic lymphoma kinase (ALK1) immunohistochemistry in diagnostic dermatopathology; an update.  

Science.gov (United States)

The use of anaplastic lymphoma kinase antibodies (ALK1) as a diagnostic aid has expanded since becoming a routinely available immunohistochemical stain. Because the skin may be the site of a wide variety of hematolymphoid and fibroblastic proliferations, dermatopathologists commonly use ALK1 as part of a broader staining panel in diagnosing soft tissue and cutaneous hematolymphoid neoplasms. Furthermore, new entities and differential diagnostic contexts are emerging, which broaden the utility of ALK1 immunohistochemistry. We review the expanding role of ALK1 immunohistochemistry in contemporary dermatopathology. PMID:23689691

Papalas, John A; Kulbacki, Evan; Wang, Endi

2013-06-01

297

Anaplastic large-cell lymphoma with florid granulomatous reaction: A case report and review of literature  

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Full Text Available Granulomatous reactions have been reported in association with lymphomas, more often with Hodgkins disease than with Non-Hodgkins Lymphoma. Not many reports are available on the association of anaplastic large-cell lymphoma with sarcoid-type granuloma. Herein, we report a case of an elderly female with generalized lymphadenopathy who had a florid granulomatous reaction almost masking the lymphoma cells in the lymph node biopsy. A detailed clinical history, careful histological examination and immunohistochemistry helped in attaining the correct diagnosis.

Balamurugan S; Rajasekar B; Rao R

2009-01-01

298

Primary anaplastic large cell lymphoma of central nervous system-A case report  

Directory of Open Access Journals (Sweden)

Full Text Available Central nervous system (CNS) involvement is extremely rare in anaplastic large cell lymphoma (ALCL). Primary ALCL of CNS on radiology is often misdiagnosed as tuberculosis. We report a fatal case of primary ALCL of CNS in a 17 year old male. He came with history of headache and left partial seizures. MRI showed a well- circumscribed lesion in the right fronto-parietal lobe eroding the skull bone. Biopsy showed large pleomorphic cells. Immunohistochemical stains showed positivity for CD30, CD43, EMA and ALK-1. In spite of radiotherapy and steroids, patient expired. Hence a high level of suspicion is essential for early diagnosis and for instituting appropriate treatment.

Rupani A; Modi C; Desai S; Rege J

2005-01-01

299

Investigation of the results of therapy of anaplastic thyroid gland carcinomas  

International Nuclear Information System (INIS)

The results of the treatment of 28 patients with an anaplastic thyroid gland carcinoma are investigated, to see whether an optimal therapy is indicated. The execution of an operation before radiotherapy does not appear to improve the prognosis (statistically this conclusion is not wholly justified). The presence of metastases at the beginning of the therapy gave rise to a worse prognosis than the absence of metastases. The combination treatment of chemotherapy and either surgery or radiotherapy was only applied to two patients so no conclusions can be made about its benefit. (C.F.)

1979-01-01

300

Combined chemical and radiotherapy of the small cell anaplastic carcinoma of the lung  

International Nuclear Information System (INIS)

[en] In this thesis the therapeutic regimens for small cell anaplastic carcinoma of the lung (SCL) are reviewed. In addition, the results of a course of combined chemotherapy and radiotherapy in a series of patients with SCL are presented. The purpose of this study was to investigate, whether this regimen of combination therapy inproves survival and subjective well-being of the patients as compared both with previously used therapeutic regimens for SCL in the Department of Pulmonology, Academic Hospital, Groningen and elsewhere. (Auth.)

1979-01-01

 
 
 
 
301

Hypocellular/lymphohistiocytic variant of Anaplastic Large Cell Lymphoma of lymph node, mimicking granulation tissue.  

UK PubMed Central (United Kingdom)

Anaplastic Large Cell Lymphoma (ALCL) is a subgroup of T/null cell non-Hodgkin lymphoma (NHL) in WHO classification. Lymphohistiocytic (LH) variant constitutes about 10% of all ALCLs and characterized by presence of abundant reactive histiocytes that can mask the neoplastic nature of the lesion, leading to misdiagnose as "reactive lymphadenopathy". Here we introduce a 16-year-old female patient, diagnosed as hypocellular LH variant ALCL with unusual histologic feature including granulation tissue- like appearance. We emphasize that in young patients with unusual- looking reactive lymphadenopathy, ALCL should be considered as one of differential diagnoses. A brief review of the nature of the lesion and differential diagnoses is also included. 

Kosari F; Saffar H; Izadi B

2013-07-01

302

Inflammatory myofibroblastic tumor of the urinary bladder diagnosed by anaplastic lymphoma kinase immunostaining.  

Science.gov (United States)

Inflammatory myofibroblastic tumor (IMFT) of the urinary bladder is an unusual spindle cell lesion that exhibits cytologic atypia, infiltrative growth, and mitotic activity mimicking malignant tumors, such as leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid carcinoma. Recently, anaplastic lymphoma kinase (ALK) gene translocations or ALK protein expression in IMFT has been reported, especially in patients of children and young adults. This lesion has been described in numerous locations in addition to the urinary bladder. The detection of ALK protein and ALK gene rearrangements are useful in distinguishing IMFT from spindle cell malignancies in the urinary bladder. PMID:22629012

Rao, Ram Nawal; Ranjan, Priydarshi; Singla, Nidhi; Pandey, Rakesh

2012-05-01

303

Inflammatory myofibroblastic tumor of the urinary bladder diagnosed by anaplastic lymphoma kinase immunostaining.  

UK PubMed Central (United Kingdom)

Inflammatory myofibroblastic tumor (IMFT) of the urinary bladder is an unusual spindle cell lesion that exhibits cytologic atypia, infiltrative growth, and mitotic activity mimicking malignant tumors, such as leiomyosarcoma, rhabdomyosarcoma, and sarcomatoid carcinoma. Recently, anaplastic lymphoma kinase (ALK) gene translocations or ALK protein expression in IMFT has been reported, especially in patients of children and young adults. This lesion has been described in numerous locations in addition to the urinary bladder. The detection of ALK protein and ALK gene rearrangements are useful in distinguishing IMFT from spindle cell malignancies in the urinary bladder.

Rao RN; Ranjan P; Singla N; Pandey R

2012-05-01

304

Long-term follow-up of surgical treatment of spinal anaplastic astrocytoma in a cat.  

Science.gov (United States)

A 10-year-old spayed female chinchilla feline presented with gradually progressive tetraparesis and cervical pain that had begun 1 month before the onset of a 4-day tetraplegic episode. Magnetic resonance imaging revealed a large elliptical intramedullary mass at the fourth cervical vertebrae. The mass was removed surgically and diagnosed as an anaplastic astrocytoma. No neurological abnormalities were observed 3 weeks postsurgery. Magnetic resonance at 3.5 year follow-up revealed neither mass regrowth nor recurrence of signs. PMID:23428584

Tamura, Shinji; Hori, Yuko; Tamura, Yumiko; Uchida, Kazuyuki

2013-02-21

305

[Differential diagnosis and strategy for the treatment of anaplastic thyroid carcinoma].  

Science.gov (United States)

Anaplastic thyroid carcinoma (ATC) is most aggressive and poor prognosis among solid neoplasmas. Unfortunately, effective treatment for ATC is not established until now. We recommend new strategy for the treatment of ATC based on new UICC staging. Stage IV is recommended the surgical treatment, and Stage IVB, IVC are recommended systemic chemotherapy like as doxorubicin and cisplatin and external radiotherapy. If tumor become decreasing by these chemoradiation, salvage surgery will be preformed for complete resection. But survival period is not longer over 20 years, so the establishment of new treatment is desired. PMID:18018574

Suzuki, Shinichi

2007-11-01

306

[Differential diagnosis and strategy for the treatment of anaplastic thyroid carcinoma].  

UK PubMed Central (United Kingdom)

Anaplastic thyroid carcinoma (ATC) is most aggressive and poor prognosis among solid neoplasmas. Unfortunately, effective treatment for ATC is not established until now. We recommend new strategy for the treatment of ATC based on new UICC staging. Stage IV is recommended the surgical treatment, and Stage IVB, IVC are recommended systemic chemotherapy like as doxorubicin and cisplatin and external radiotherapy. If tumor become decreasing by these chemoradiation, salvage surgery will be preformed for complete resection. But survival period is not longer over 20 years, so the establishment of new treatment is desired.

Suzuki S

2007-11-01

307

Specificity of IRF4 Translocations for Primary Cutaneous Anaplastic Large Cell Lymphoma: a Multicenter Study of 204 Skin Biopsies  

Science.gov (United States)

Current pathologic criteria cannot reliably distinguish cutaneous anaplastic large cell lymphoma from other CD30-positive T-cell lymphoproliferative disorders (lymphomatoid papulosis, systemic anaplastic large cell lymphoma with skin involvement, and transformed mycosis fungoides). We previously reported IRF4 (interferon regulatory factor-4) translocations in cutaneous anaplastic large cell lymphomas. Here, we investigated the clinical utility of detecting IRF4 translocations in skin biopsies. We performed fluorescence in situ hybridization for IRF4 in 204 biopsies involved by T-cell lymphoproliferative disorders from 182 patients at three institutions. Nine of forty-five (20%) cutaneous anaplastic large cell lymphomas and 1 of 32 (3%) cases of lymphomatoid papulosis with informative results demonstrated an IRF4 translocation. Remaining informative cases were negative for a translocation (7 systemic anaplastic large cell lymphomas; 44 cases of mycosis fungoides/Sézary syndrome (13 transformed); 24 peripheral T-cell lymphomas, not otherwise specified; 12 CD4-positive small/medium-sized pleomorphic T-cell lymphomas; 5 extranodal NK/T-cell lymphomas, nasal type; 4 gamma-delta T-cell lymphomas; and 5 other uncommon T-cell lymphoproliferative disorders). Among all cutaneous T-cell lymphoproliferative disorders, fluorescence in situ hybridization for IRF4 had a specificity and positive predictive value for cutaneous anaplastic large cell lymphoma of 99% and 90%, respectively (p=0.00002, Fisher’s exact test). Among anaplastic large cell lymphomas, lymphomatoid papulosis, and transformed mycosis fungoides, specificity and positive predictive value were 98% and 90%, respectively (p=0.005). Fluorescence in situ hybridization abnormalities other than translocations and IRF4 protein expression were seen in 13% and 65% of cases, respectively, but were non-specific with regard to T-cell lymphoproliferative disorder subtype. Our findings support the clinical utility of fluorescence in situ hybridization for IRF4 in the differential diagnosis of T-cell lymphoproliferative disorders in skin biopsies, with detection of a translocation favoring cutaneous anaplastic large cell lymphoma. Like all fluorescence in situ hybridization studies, IRF4 testing must be interpreted in the context of morphology, phenotype, and clinical features.

Wada, David A.; Law, Mark E.; Hsi, Eric D.; DiCaudo, David J.; Ma, Linglei; Lim, Megan S.; de Souza, Aieska; Comfere, Nneka I.; Weenig, Roger H.; Macon, William R.; Erickson, Lori A.; Ozsan, Nazan; Ansell, Stephen M.; Dogan, Ahmet; Feldman, Andrew L.

2011-01-01

308

Specificity of IRF4 translocations for primary cutaneous anaplastic large cell lymphoma: a multicenter study of 204 skin biopsies.  

UK PubMed Central (United Kingdom)

Current pathologic criteria cannot reliably distinguish cutaneous anaplastic large cell lymphoma from other CD30-positive T-cell lymphoproliferative disorders (lymphomatoid papulosis, systemic anaplastic large cell lymphoma with skin involvement, and transformed mycosis fungoides). We previously reported IRF4 (interferon regulatory factor-4) translocations in cutaneous anaplastic large cell lymphomas. Here, we investigated the clinical utility of detecting IRF4 translocations in skin biopsies. We performed fluorescence in situ hybridization (FISH) for IRF4 in 204 biopsies involved by T-cell lymphoproliferative disorders from 182 patients at three institutions. In all, 9 of 45 (20%) cutaneous anaplastic large cell lymphomas and 1 of 32 (3%) cases of lymphomatoid papulosis with informative results demonstrated an IRF4 translocation. Remaining informative cases were negative for a translocation (7 systemic anaplastic large cell lymphomas; 44 cases of mycosis fungoides/Sézary syndrome (13 transformed); 24 peripheral T-cell lymphomas, not otherwise specified; 12 CD4-positive small/medium-sized pleomorphic T-cell lymphomas; 5 extranodal NK/T-cell lymphomas, nasal type; 4 gamma-delta T-cell lymphomas; and 5 other uncommon T-cell lymphoproliferative disorders). Among all cutaneous T-cell lymphoproliferative disorders, FISH for IRF4 had a specificity and positive predictive value for cutaneous anaplastic large cell lymphoma of 99 and 90%, respectively (P=0.00002, Fisher's exact test). Among anaplastic large cell lymphomas, lymphomatoid papulosis, and transformed mycosis fungoides, specificity and positive predictive value were 98 and 90%, respectively (P=0.005). FISH abnormalities other than translocations and IRF4 protein expression were seen in 13 and 65% of cases, respectively, but were nonspecific with regard to T-cell lymphoproliferative disorder subtype. Our findings support the clinical utility of FISH for IRF4 in the differential diagnosis of T-cell lymphoproliferative disorders in skin biopsies, with detection of a translocation favoring cutaneous anaplastic large cell lymphoma. Like all FISH studies, IRF4 testing must be interpreted in the context of morphology, phenotype, and clinical features.

Wada DA; Law ME; Hsi ED; Dicaudo DJ; Ma L; Lim MS; Souza Ad; Comfere NI; Weenig RH; Macon WR; Erickson LA; Ozsan N; Ansell SM; Dogan A; Feldman AL

2011-04-01

309

A new der(1;7)(q10;p10) leading to a singular 1p loss in a case of glioblastoma with oligodendroglioma component.  

UK PubMed Central (United Kingdom)

The combined 1p-/19q- deletions in oligodendrogliomas originate from translocation between both chromosomes. In the few cases of oligoastrocytomas and glioblastomas with an oligodendroglioma component (GBMO) where only 1p deletion was described, the origin remains unknown. We report the first case of GBMO, in which a single 1p deletion was detected and was linked to a translocation between chromosomes 1 and 7. Fresh surgical specimens were collected during surgery and the samples were used for cell culture, touch preparation smear slides (TP slides) and DNA extraction. Peripheral venous blood was also collected from the patient. G-banding using Trypsin and stained with Giemsa (GTG) banding and karyotyping were performed and 1p-/19q-, TP53, PTEN and c-MYC were analyzed by fluorescent in situ hybridization (FISH). Multicolor FISH (mFISH) and microsatellites analyses were also performed to complete the investigation. Three-dimensional quantitative FISH (3D-QFISH) of telomeres was performed on nuclei from TP slides and analyzed using TeloView(TM) to determine whether the 3D telomere profile as an assessment of telomere dysfunction and a characterization of genomic instability could predict the disease aggressiveness. An unbalanced chromosomal translocation was found in all metaphases and confirmed by mFISH. The karyotype of the case is: 50?99,XXX, +der(1;7)(q10;p10),inc[47] The derivative chromosome was found in all 47 analyzed cells, but the number of derivatives varied from one to four. There was neither imbalance in copy number for genes TP53 and PTEN, nor amplification of c-MYC gene. We did not find loss of heterozygosity with analysis of microsatellite markers for chromosomes 1p and 19q in tumor cells. The 3D-telomere profile predicted a very poor prognostic and short-term survival of the patient and highlights the potential clinical power of telomere signatures as a solid biomarker of GBMO. Furthermore, this translocation between chromosomes 1 and 7 led to a singular 1p deletion in this GBMO and may generate the 1p and 7q deletions.

Gadji M; Crous-Tsanaclis AM; Mathieu D; Mai S; Fortin D; Drouin R

2013-09-01

310

Elevated serum-soluble interleukin-2 receptor levels in patients with anaplastic large cell lymphoma.  

Science.gov (United States)

Levels of serum soluble interleukin 2 receptor (sIL-2R) provide a reliable marker of disease activity in patients with hairy cell leukemia and adult T-cell leukemia/lymphoma. The malignant cells in patients with anaplastic large cell lymphoma (ALCL) express CD30 and are usually positive for expression of CD25. We measured serum sIL-2R and soluble CD30 (sCD30) levels in patients with ALCL treated with EPOCH (etoposide, prednisone, Oncovin, Cytoxan, hydroxydaunorubicin) infusional chemotherapy. Serum sCD30 levels were elevated and decreased in response to therapy as previously reported. Serum sIL-2R levels were elevated in 7 of 9 patients with ALCL and decreased in response to treatment. Baseline serum sIL-2R levels varied but correlated well with serum sCD30 levels (r = 0.97). Patients positive for the anaplastic lymphoma kinase (ALK) gene showed elevated sIL-2R levels, whereas those negative for ALK had normal serum sIL-2R levels and their tumors lacked CD25 expression. Serum sIL-2R levels were elevated in both patients with recurrent disease. PMID:15205267

Janik, John E; Morris, John C; Pittaluga, Stefania; McDonald, Kristin; Raffeld, Mark; Jaffe, Elaine S; Grant, Nicole; Gutierrez, Martin; Waldmann, Thomas A; Wilson, Wyndham H

2004-06-17

311

Primary central nervous system anaplastic large-cell lymphoma mimicking lymphomatosis cerebri.  

UK PubMed Central (United Kingdom)

Primary central nervous system lymphoma (PCNSL) is usually diffuse large B-cell lymphoma. Anaplastic large-cell lymphoma (ALCL) rarely occurs in the central nervous system. PCNSL always presents as single or multiple nodular contrast-enhancing mass lesions within T2-hyperintense areas on magnetic resonance imaging (MRI). Infrequently, diffuse infiltrating change with little contrast enhancement called lymphomatosis cerebri can be seen in PCNSL. In this report, we describe a 75-year-old immunocompetent man who had progressive dementia. On MRI, diffuse white matter lesions with little contrast enhancement were observed to gradually progress, which was clinically consistent with his worsening condition. A biopsy specimen revealed non-destructive, diffusely infiltrating, anaplastic large CD30-positive lymphoma, indicating a diagnosis of ALCL. After the biopsy, he was treated by whole brain irradiation (total 46 Gy) and focal boost irradiation (total 14 Gy). However, his performance status worsened and there was no symptom improvement. The patient died 8 months after symptom onset. The clinical course, diagnostic workup, pathologic correlates, and treatment outcomes are described herein.

Sugino T; Mikami T; Akiyama Y; Wanibuchi M; Hasegawa T; Mikuni N

2013-01-01

312

Anaplastic lymphoma kinase is expressed in different subtypes of human breast cancer  

International Nuclear Information System (INIS)

[en] Pleiotrophin (PTN, Ptn) is an 18 kDa cytokine expressed in human breast cancers. Since inappropriate expression of Ptn stimulates progression of breast cancer in transgenic mice and a dominant negative PTN reverses the transformed phenotype of human breast cancer cells that inappropriately express Ptn, it is suggested that constitutive PTN signaling in breast cancer cells that inappropriately express Ptn activates pathways that promote a more aggressive breast cancer phenotype. Pleiotrophin signals by inactivating its receptor, the receptor protein tyrosine phosphatase (RPTP)?/?, and, recently, PTN was found to activate anaplastic lymphoma kinase (ALK) through the PTN/RPTP?/? signaling pathway in PTN-stimulated cells, not through a direct interaction of PTN with ALK and thus not through the PTN-enforced dimerization of ALK. Since full-length ALK is activated in different malignant cancers and activated ALK is a potent oncogenic protein, we examined human breast cancers to test the possibility that ALK may be expressed in breast cancers and potentially activated through the PTN/RPTP?/? signaling pathway; we now demonstrate that ALK is strongly expressed in different histological subtypes of human breast cancer; furthermore, ALK is expressed in both nuclei and cytoplasm and, in the 'dotted' pattern characteristic of ALK fusion proteins in anaplastic large cell lymphoma. This study thus supports the possibility that activated ALK may be important in human breast cancers and potentially activated either through the PTN/RPTP?/? signaling pathway, or, alternatively, as an activated fusion protein to stimulate progression of breast cancer in humans

2007-06-29

313

Novel TRAF1-ALK fusion identified by deep RNA sequencing of anaplastic large cell lymphoma.  

UK PubMed Central (United Kingdom)

Chromosomal translocations leading to expression of abnormal fusion proteins play a major role in the pathogenesis of various hematologic malignancies. The recent development of high-throughput, "deep" sequencing has allowed discovery of novel translocations leading to a rapid increase in understanding these diseases. Translocations involving the anaplastic lymphoma kinase (ALK) gene leading to ALK fusion proteins originally were discovered in anaplastic large cell lymphomas (ALCLs). Among ALCLs, NPM1-ALK fusions are most common and lead to nuclear localization of the fusion protein. Here, we present a 50-year-old male with ALCL demonstrating cytoplasmic ALK immunoreactivity only, suggesting the presence of a non-NPM1 fusion partner. We performed deep RNA sequencing of tumor tissue from this patient and identified a novel transcript fusing Exon 6 of TRAF1 to Exon 20 of ALK. The TRAF1-ALK fusion transcript was confirmed at the mRNA level by Sanger sequencing and the fusion protein was visualized by Western blot. The discovery of this TRAF1-ALK fusion expands the diversity of known ALK fusion partners and highlights the power of deep sequencing for fusion transcript discovery. © 2013 Wiley Periodicals, Inc.

Feldman AL; Vasmatzis G; Asmann YW; Davila J; Middha S; Eckloff BW; Johnson SH; Porcher JC; Ansell SM; Caride A

2013-11-01

314

PRDM1/BLIMP1 is commonly inactivated in anaplastic large T-cell lymphoma.  

UK PubMed Central (United Kingdom)

Anaplastic Large Cell Lymphoma (ALCL) is a mature T-cell lymphoma that can present as a systemic or primary cutaneous disease. Systemic ALCL represents 2-5% of adult lymphoma but up to 30% of all pediatric cases. Two subtypes of systemic ALCL are currently recognized on the basis of the presence of a translocation involving the Anaplastic Lymphoma Kinase ALK gene. Despite considerable progress, several questions remain open regarding the pathogenesis of both ALCL subtypes. To investogate the molecular pathogenesis and to assess the relationship between the ALK(+) and ALK(-)ALCL subtypes, we performed a genome-wide DNA profiling using high density, single nucleotide polymorphism (SNP) arrays (SNP-array) on a series of 64 cases and seven cell lines. The commonest lesions were losses at 17p13 and at 6q21, encompassing the TP53 and PRDM1 genes respectively. The latter gene, coding for BLIMP1, was inactivated by multiple mechanisms, more frequently, but not exclusively, in ALK(-)ALCL. In vitro and in vivo experiments showed that that PRDM1 is a tumor suppressor gene in ALCL models, likely acting as an anti-apoptotic agent. Losses of TP53 and/or PRDM1 were present in 52% of ALK(-)ALCL, and in 29% of all ALCL cases with a clinical implication.

Boi M; Rinaldi A; Kwee I; Bonetti P; Todaro M; Tabbò F; Piva R; Rancoita PM; Matolcsy A; Timar B; Tousseyn T; Rodríguez-Pinilla SM; Piris MA; Beà S; Campo E; Bhagat G; Swerdlow SH; Rosenwald A; Ponzoni M; Young KH; Piccaluga PP; Dummer R; Pileri S; Zucca E; Inghirami G; Bertoni F

2013-09-01

315

Primary anaplastic large cell lymphoma of the psoas muscle: a case report and literature review.  

UK PubMed Central (United Kingdom)

Primary anaplastic large cell lymphoma (ALCL) of skeletal muscle is very rare. We report a case of ALCL arising from the left psoas muscle. A 14-year-old girl presented with a large left inguinal tumor. She complained of a 2-month history of left leg pain, which had been exacerbated upon leg extension, and she had become aware of a rapidly growing left inguinal tumor 3 weeks before admission. CT scan and MRI revealed a large tumor arising from the left major psoas muscle and protruding into the inguinal region. In view of the tumor's location and the patient's age, soft tissue tumors such as rhabdomyosarcoma and primitive neuroectodermal tumor were initially considered. However, histopathological examination yielded a diagnosis of anaplastic lymphoma kinase-positive ALCL. The serum level of soluble interleukin-2 receptor was markedly elevated at 50,414 U/ml, and this also strongly suggested ALCL. Although rarely reported, ALCL is an important entity to consider in the differential diagnosis of skeletal muscle tumors in children and young adults.

Kounami S; Shibuta K; Yoshiyama M; Mitani Y; Watanabe T; Takifuji K; Yoshikawa N

2012-01-01

316

PRDM1/BLIMP1 is commonly inactivated in anaplastic large T-cell lymphoma.  

UK PubMed Central (United Kingdom)

Anaplastic large cell lymphoma (ALCL) is a mature T-cell lymphoma that can present as a systemic or primary cutaneous disease. Systemic ALCL represents 2% to 5% of adult lymphoma but up to 30% of all pediatric cases. Two subtypes of systemic ALCL are currently recognized on the basis of the presence of a translocation involving the anaplastic lymphoma kinase ALK gene. Despite considerable progress, several questions remain open regarding the pathogenesis of both ALCL subtypes. To investigate the molecular pathogenesis and to assess the relationship between the ALK(+) and ALK(-) ALCL subtypes, we performed a genome-wide DNA profiling using high-density, single nucleotide polymorphism arrays on a series of 64 cases and 7 cell lines. The commonest lesions were losses at 17p13 and at 6q21, encompassing the TP53 and PRDM1 genes, respectively. The latter gene, coding for BLIMP1, was inactivated by multiple mechanisms, more frequently, but not exclusively, in ALK(-)ALCL. In vitro and in vivo experiments showed that that PRDM1 is a tumor suppressor gene in ALCL models, likely acting as an antiapoptotic agent. Losses of TP53 and/or PRDM1 were present in 52% of ALK(-)ALCL, and in 29% of all ALCL cases with a clinical implication.

Boi M; Rinaldi A; Kwee I; Bonetti P; Todaro M; Tabbò F; Piva R; Rancoita PM; Matolcsy A; Timar B; Tousseyn T; Rodríguez-Pinilla SM; Piris MA; Beà S; Campo E; Bhagat G; Swerdlow SH; Rosenwald A; Ponzoni M; Young KH; Piccaluga PP; Dummer R; Pileri S; Zucca E; Inghirami G; Bertoni F

2013-10-01

317

PRDM1/BLIMP1 is commonly inactivated in anaplastic large T-cell lymphoma.  

Science.gov (United States)

Anaplastic large cell lymphoma (ALCL) is a mature T-cell lymphoma that can present as a systemic or primary cutaneous disease. Systemic ALCL represents 2% to 5% of adult lymphoma but up to 30% of all pediatric cases. Two subtypes of systemic ALCL are currently recognized on the basis of the presence of a translocation involving the anaplastic lymphoma kinase ALK gene. Despite considerable progress, several questions remain open regarding the pathogenesis of both ALCL subtypes. To investigate the molecular pathogenesis and to assess the relationship between the ALK(+) and ALK(-) ALCL subtypes, we performed a genome-wide DNA profiling using high-density, single nucleotide polymorphism arrays on a series of 64 cases and 7 cell lines. The commonest lesions were losses at 17p13 and at 6q21, encompassing the TP53 and PRDM1 genes, respectively. The latter gene, coding for BLIMP1, was inactivated by multiple mechanisms, more frequently, but not exclusively, in ALK(-)ALCL. In vitro and in vivo experiments showed that that PRDM1 is a tumor suppressor gene in ALCL models, likely acting as an antiapoptotic agent. Losses of TP53 and/or PRDM1 were present in 52% of ALK(-)ALCL, and in 29% of all ALCL cases with a clinical implication. PMID:24004669

Boi, Michela; Rinaldi, Andrea; Kwee, Ivo; Bonetti, Paola; Todaro, Maria; Tabbò, Fabrizio; Piva, Roberto; Rancoita, Paola M V; Matolcsy, András; Timar, Botond; Tousseyn, Thomas; Rodríguez-Pinilla, Socorro Maria; Piris, Miguel A; Beà, Sílvia; Campo, Elias; Bhagat, Govind; Swerdlow, Steven H; Rosenwald, Andreas; Ponzoni, Maurilio; Young, Ken H; Piccaluga, Pier Paolo; Dummer, Reinhard; Pileri, Stefano; Zucca, Emanuele; Inghirami, Giorgio; Bertoni, Francesco

2013-09-04

318

Proteomic Profile Modification of Anaplastic Medulloblastoma after in-Vivo Radiotherapy: A Case Study  

Directory of Open Access Journals (Sweden)

Full Text Available Medulloblastoma (MDB) is an aggressive tumor of Central Nervous System (CNS). Radiotherapy after radical surgery has an important role in treatment of standard and high risk patients and is followed by intensive chemotherapy. To explore modifications of protein expression induced by in vivo radiotherapy proteomic analysis was performed on a case of Anaplastic MDB. 2D-gel electrophoresis and MALDI-TOF mass spectrometry detected qualitative differences of protein expression in Anaplastic MDB at diagnosis and in relapse after radiotherapy. Relevant proteomic data were confirmed by western blot and Real-Time PCR analysis, validating the presence of Sthatmin 1 (STMN1), Heat shock protein 60 (HSP60), HSP27 and Disulfide Isomerase (ER60) among the six proteins present in both samples. The most relevant modification induced by radiotherapy was a drastic reduction of the total number of proteins (60.6%) and the appearance of few new proteins. The modifications and the striking simplification of proteins expressed by the tumor after radiotherapy may allow to tailor subsequent chemotherapy on a rational basis. A proteomic guided chemotherapy may be of great benefit to patients.

C. Zanini; G. Mandili; D. Baci; M. Leone; I. Morra; M. Forni

2010-01-01

319

FOXM1 is a molecular determinant of the mitogenic and invasive phenotype of anaplastic thyroid carcinoma.  

UK PubMed Central (United Kingdom)

Anaplastic thyroid carcinoma (ATC) is a very aggressive thyroid cancer. forkhead box protein M1 (FOXM1) is a member of the forkhead box family of transcription factors involved in control of cell proliferation, chromosomal stability, angiogenesis, and invasion. Here, we show that FOXM1 is significantly increased in ATCs compared with normal thyroid, well-differentiated thyroid carcinomas (papillary and/or follicular), and poorly differentiated thyroid carcinomas (P=0.000002). Upregulation of FOXM1 levels in ATC cells was mechanistically linked to loss-of-function of p53 and to the hyperactivation of the phosphatidylinositol-3-kinase/AKT/FOXO3a pathway. Knockdown of FOXM1 by RNA interference inhibited cell proliferation by arresting cells in G2/M and reduced cell invasion and motility. This phenotype was associated with decreased expression of FOXM1 target genes, like cyclin B1 (CCNB1), polo-like kinase 1 (PLK1), Aurora B (AURKB), S-phase kinase-associated protein 2 (SKP2), and plasminogen activator, urokinase: uPA (PLAU). Pharmacological inhibition of FOXM1 in an orthotopic mouse model of ATC reduced tumor burden and metastasization. All together, these findings suggest that FOXM1 represents an important player in thyroid cancer progression to the anaplastic phenotype and a potential therapeutic target for this fatal cancer.

Bellelli R; Castellone MD; Garcia-Rostan G; Ugolini C; Nucera C; Sadow PM; Nappi TC; Salerno P; Cantisani MC; Basolo F; Gago TA; Salvatore G; Santoro M

2012-10-01

320

FOXM1 is a molecular determinant of the mitogenic and invasive phenotype of anaplastic thyroid carcinoma.  

Science.gov (United States)

Anaplastic thyroid carcinoma (ATC) is a very aggressive thyroid cancer. forkhead box protein M1 (FOXM1) is a member of the forkhead box family of transcription factors involved in control of cell proliferation, chromosomal stability, angiogenesis, and invasion. Here, we show that FOXM1 is significantly increased in ATCs compared with normal thyroid, well-differentiated thyroid carcinomas (papillary and/or follicular), and poorly differentiated thyroid carcinomas (P=0.000002). Upregulation of FOXM1 levels in ATC cells was mechanistically linked to loss-of-function of p53 and to the hyperactivation of the phosphatidylinositol-3-kinase/AKT/FOXO3a pathway. Knockdown of FOXM1 by RNA interference inhibited cell proliferation by arresting cells in G2/M and reduced cell invasion and motility. This phenotype was associated with decreased expression of FOXM1 target genes, like cyclin B1 (CCNB1), polo-like kinase 1 (PLK1), Aurora B (AURKB), S-phase kinase-associated protein 2 (SKP2), and plasminogen activator, urokinase: uPA (PLAU). Pharmacological inhibition of FOXM1 in an orthotopic mouse model of ATC reduced tumor burden and metastasization. All together, these findings suggest that FOXM1 represents an important player in thyroid cancer progression to the anaplastic phenotype and a potential therapeutic target for this fatal cancer. PMID:22919068

Bellelli, Roberto; Castellone, Maria Domenica; Garcia-Rostan, Ginesa; Ugolini, Clara; Nucera, Carmelo; Sadow, Peter M; Nappi, Tito Claudio; Salerno, Paolo; Cantisani, Maria Carmela; Basolo, Fulvio; Gago, Tomas Alvarez; Salvatore, Giuliana; Santoro, Massimo

2012-09-21

 
 
 
 
321

Novel TRAF1-ALK fusion identified by deep RNA sequencing of anaplastic large cell lymphoma.  

Science.gov (United States)

Chromosomal translocations leading to expression of abnormal fusion proteins play a major role in the pathogenesis of various hematologic malignancies. The recent development of high-throughput, "deep" sequencing has allowed discovery of novel translocations leading to a rapid increase in understanding these diseases. Translocations involving the anaplastic lymphoma kinase (ALK) gene leading to ALK fusion proteins originally were discovered in anaplastic large cell lymphomas (ALCLs). Among ALCLs, NPM1-ALK fusions are most common and lead to nuclear localization of the fusion protein. Here, we present a 50-year-old male with ALCL demonstrating cytoplasmic ALK immunoreactivity only, suggesting the presence of a non-NPM1 fusion partner. We performed deep RNA sequencing of tumor tissue from this patient and identified a novel transcript fusing Exon 6 of TRAF1 to Exon 20 of ALK. The TRAF1-ALK fusion transcript was confirmed at the mRNA level by Sanger sequencing and the fusion protein was visualized by Western blot. The discovery of this TRAF1-ALK fusion expands the diversity of known ALK fusion partners and highlights the power of deep sequencing for fusion transcript discovery. © 2013 Wiley Periodicals, Inc. PMID:23999969

Feldman, Andrew L; Vasmatzis, George; Asmann, Yan W; Davila, Jaime; Middha, Sumit; Eckloff, Bruce W; Johnson, Sarah H; Porcher, Julie C; Ansell, Stephen M; Caride, Ariel

2013-09-02

322

Ki-1 anaplastic large-cell lymphoma in the differential diagnosis of unknown primary cancer.  

UK PubMed Central (United Kingdom)

The importance of immunohistochemical analysis of anaplastic or undifferentiated carcinoma, especially in the setting of an unusual clinical presentation, is illustrated by two cases of metastatic undifferentiated carcinoma. Re-evaluation of pathological material resulted in a diagnosis of Ki-1 lymphoma and led to substantial alteration in therapeutic strategy and long-term outlook. The response to therapy was unusually favorable in both cases, and survival was prolonged in both. Archival pathological material was retrieved in order to perform special studies that were not originally available at the time of diagnosis, consisting of immunoperoxidase detection of hematological markers that are preserved in formalin-fixed, paraffin-embedded tissues. Both tumors were found in retrospect to actually represent anaplastic, Ki-1-positive, large-cell lymphoma. This neoplastic entity has been introduced to the oncologic literature only within the past decade. Because this neoplasm shows chemosensitivity and responsiveness similar to that of other large-cell lymphomas, we alert practitioners to consider this diagnosis in any patient who presents with an apparently metastatic undifferentiated tumor.

Lokich J; Sherburne B

1998-01-01

323

Anaplastic large cell lymphoma: an unusual presentation in a 7-year-old girl.  

UK PubMed Central (United Kingdom)

Anaplastic large cell lymphoma (ALCL) accounts for 10% to 30% of all childhood lymphomas and approximately 5% of all non-Hodgkin's lymphoma. ALCL is considered to be a T-cell non-Hodgkin's lymphoma that can be divided into two major groups with distinct genetic, immunophenotypic, and clinical behaviors. The first group consists of a spectrum of CD30+ T-cell lymphoproliferative disorders that include primary cutaneous ALCL (C-ALCL) and lymphomatoid papulosis. The second group is systemic ALCL (S-ALCL), which is further divided into two subgroups: anaplastic lymphoma kinase positive (ALK+) and ALK-negative. Between 30% and 60% of S-ALCL express ALK, which is usually the result of a t(2;5) translocation that correlates with onset in the first three decades of life, male predominance, and good prognosis. Although morphologically similar, ALK- ALCL shows varied clinical behaviors and immunophenotypes; is commonly seen in older age groups, with a peak incidence in the sixth decade of life with no preference as to sex; and has an overall poorer prognosis. We present a case of CD30+, ALK- S-ALCL in a 7-year-old girl.

Ju E; Adigun C; Dunphy C; Gold S; Morrell DS

2012-07-01

324

Cutaneous anaplastic large cell lymphomas: a report of 9 cases from Thailand.  

UK PubMed Central (United Kingdom)

BACKGROUND: Anaplastic large cell lymphoma (ALCL) is one type of lymphoma, which is characterized by the proliferation of pleomorphic large atypical lymphoid cells expressing CD30 antigen. ALCL involving skin can be either primary cutaneous disease or cutaneous involvement secondary from systemic disease. Data of clinical manifestation of cutaneous ALCL in Thai patients is limited. ALCL in Thai patients may differ from other groups of patients. OBJECTIVE: To study the clinical manifestation of cutaneous ALCL in patients of Faculty of Medicine Siriraj Hospital, Thailand. MATERIAL AND METHOD: Medical records of nine patients with histopathologic diagnosis of ALCL from skin biopsy at Faculty of Medicine Siriraj Hospital were reviewed. RESULTS: Of nine patients, four patients were diagnosed as primary cutaneous ALCL, four patients as systemic ALCL with secondary skin involvement, and one patient as combined primary cutaneous ALCL and lymphomatoid papulosis. Three primary cutaneous ALCL patients had no recurrence of disease during 6-year follow-up. However all systemic ALCL patients died at one day to 1.5 years after diagnosis. CONCLUSION: Clinical manifestation and clinical course of Thai patients with anaplastic large cell lymphoma corresponded with the data from other patient population.

Eimpunth S; Sittinamsuwan P; Pattanaprichakul P; Silpa-archa N; Sethabutra P; Chularojanamontri L; Hanamornroongruang S; Mahaisavariya P

2012-03-01

325

Anaplastic large cell lymphoma: an unusual presentation in a 7-year-old girl.  

Science.gov (United States)

Anaplastic large cell lymphoma (ALCL) accounts for 10% to 30% of all childhood lymphomas and approximately 5% of all non-Hodgkin's lymphoma. ALCL is considered to be a T-cell non-Hodgkin's lymphoma that can be divided into two major groups with distinct genetic, immunophenotypic, and clinical behaviors. The first group consists of a spectrum of CD30+ T-cell lymphoproliferative disorders that include primary cutaneous ALCL (C-ALCL) and lymphomatoid papulosis. The second group is systemic ALCL (S-ALCL), which is further divided into two subgroups: anaplastic lymphoma kinase positive (ALK+) and ALK-negative. Between 30% and 60% of S-ALCL express ALK, which is usually the result of a t(2;5) translocation that correlates with onset in the first three decades of life, male predominance, and good prognosis. Although morphologically similar, ALK- ALCL shows varied clinical behaviors and immunophenotypes; is commonly seen in older age groups, with a peak incidence in the sixth decade of life with no preference as to sex; and has an overall poorer prognosis. We present a case of CD30+, ALK- S-ALCL in a 7-year-old girl. PMID:21967522

Ju, Elizabeth; Adigun, Chris; Dunphy, Cherie; Gold, Stuart; Morrell, Dean S

2011-10-04

326

A cell proliferation and chromosomal instability signature in anaplastic thyroid carcinoma.  

Science.gov (United States)

Here, we show that the anaplastic thyroid carcinoma (ATC) features the up-regulation of a set of genes involved in the control of cell cycle progression and chromosome segregation. This phenotype differentiates ATC from normal tissue and from well-differentiated papillary thyroid carcinoma. Transcriptional promoters of the ATC up-regulated genes are characterized by a modular organization featuring binding sites for E2F and NF-Y transcription factors and cell cycle-dependent element (CDE)/cell cycle gene homology region (CHR) cis-regulatory elements. Two protein kinases involved in cell cycle regulation, namely, Polo-like kinase 1 (PLK1) and T cell tyrosine kinase (TTK), are part of the gene set that is up-regulated in ATC. Adoptive overexpression of p53, p21 (CIP1/WAF1), and E2F4 down-regulated transcription from the PLK1 and TTK promoters in ATC cells, suggesting that these genes might be under the negative control of tumor suppressors of the p53 and pRB families. ATC, but not normal thyroid, cells depended on PLK1 for survival. RNAi-mediated PLK1 knockdown caused cell cycle arrest associated with 4N DNA content and massive mitotic cell death. Thus, thyroid cell anaplastic transformation is accompanied by the overexpression of a cell proliferation/genetic instability-related gene cluster that includes PLK1 kinase, which is a potential molecular target for ATC treatment. PMID:17981789

Salvatore, Giuliana; Nappi, Tito Claudio; Salerno, Paolo; Jiang, Yuan; Garbi, Corrado; Ugolini, Clara; Miccoli, Paolo; Basolo, Fulvio; Castellone, Maria Domenica; Cirafici, Anna Maria; Melillo, Rosa Marina; Fusco, Alfredo; Bittner, Michael L; Santoro, Massimo

2007-11-01

327

Recurrent ALK-negative anaplastic large T-cell lymphoma presenting as necrotizing vasculitis.  

UK PubMed Central (United Kingdom)

Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma histologically characterized by expression of CD30, a cell surface receptor present on activated T cells and B cells. ALCL may occur in a primary cutaneous form or as systemic ALCL with lymph node involvement. Anaplastic lymphoma kinase (ALK) is a tyrosine kinase that induces neoplastic transformation as a result of translocational fusion with an activating promoter. The presence of ALK can be used to distinguish between primary cutaneous ALCL and systemic nodal ALCL in certain cases. Primary cutaneous and systemic ALCL metastatic to the skin are histologically indistinguishable. "Leukemic vasculitis"--an uncommon finding in cases of cutaneous leukemia and even more exceptional in cutaneous lymphoma--refers to a pattern of vasculitis occurring as a direct result of infiltrating neoplastic cells. We report a fatal case of recurrent ALK-negative ALCL presenting as ulcerating skin lesions in a patient previously treated with the new anti-CD30 agent brentuximab vedotin. Biopsy revealed a necrotizing vasculitis resulting from the infiltration of neoplastic cells reminiscent of the patient's primary malignancy. We review the clinical and pathological findings of ALCL and present this case to highlight a subtle diagnostic clue in assessing recurrence of cutaneous lymphoma.

Nambudiri VE; Aboutalebi A; Granter SR; Saavedra A

2013-06-01

328

Recurrent ALK-negative anaplastic large T-cell lymphoma presenting as necrotizing vasculitis.  

Science.gov (United States)

Anaplastic large cell lymphoma (ALCL) is a T-cell lymphoma histologically characterized by expression of CD30, a cell surface receptor present on activated T cells and B cells. ALCL may occur in a primary cutaneous form or as systemic ALCL with lymph node involvement. Anaplastic lymphoma kinase (ALK) is a tyrosine kinase that induces neoplastic transformation as a result of translocational fusion with an activating promoter. The presence of ALK can be used to distinguish between primary cutaneous ALCL and systemic nodal ALCL in certain cases. Primary cutaneous and systemic ALCL metastatic to the skin are histologically indistinguishable. "Leukemic vasculitis"--an uncommon finding in cases of cutaneous leukemia and even more exceptional in cutaneous lymphoma--refers to a pattern of vasculitis occurring as a direct result of infiltrating neoplastic cells. We report a fatal case of recurrent ALK-negative ALCL presenting as ulcerating skin lesions in a patient previously treated with the new anti-CD30 agent brentuximab vedotin. Biopsy revealed a necrotizing vasculitis resulting from the infiltration of neoplastic cells reminiscent of the patient's primary malignancy. We review the clinical and pathological findings of ALCL and present this case to highlight a subtle diagnostic clue in assessing recurrence of cutaneous lymphoma. PMID:23291583

Nambudiri, Vinod E; Aboutalebi, Amir; Granter, Scott R; Saavedra, Arturo

2013-06-01

329

Detection of Epstein-Barr virus genome in Ki-1 (CD30)-positive, large-cell anaplastic lymphomas using the polymerase chain reaction.  

Science.gov (United States)

Ki-1 (CD30)-positive, large-cell anaplastic lymphoma (LCAL) is a distinctive subset of non-Hodgkin's lymphoma; morphologically, the neoplastic cells of LCAL may closely resemble Reed-Sternberg cell variants of Hodgkin's disease. The neoplastic cells in Hodgkin's disease are often CD30-positive, as are some of the transformed lymphocytes in infectious mononucleosis. Recent evidence suggests an etiologic role for the Epstein-Barr virus (EBV) in Hodgkin's disease. Because of the phenotypic similarities between Hodgkin's disease and LCAL, we used the polymerase chain reaction (PCR) to analyze eight specimens of LCAL for EBV genome. Diagnoses were established by paraffin section morphology and immunohistochemistry. For comparison, we also analyzed nine non-Hodgkin's lymphomas other than the LCAL type, three Hodgkin's disease specimens, and nine non-neoplastic lymph nodes. PCR was performed using DNA extracted from frozen tissue; DNA was amplified using two sets of oligonucleotide primers corresponding to the BamH1 W-fragment of the EBV genome. Amplified EBV genome was obtained from all specimens except for one mantle zone lymphoma, one diffuse mixed-cell lymphoma, and six non-neoplastic lymph nodes. EBV terminus region probing and in situ hybridization techniques, each less sensitive than PCR, were performed in selected cases in an attempt to corroborate our PCR results. Only 2 of 13 specimens contained EBV detectable by these other techniques, and neither specimen was a LCAL. In view of the high incidence of latent EBV infections in humans, the biologic significance of our PCR results is uncertain. Despite the detection of EBV genome by PCR in a high percentage of lymphomas, we were unable to substantiate an etiologic role for EBV in LCAL. The PCR technique may be too sensitive to provide meaningful data on the possible role of EBV in lymphomagenesis. Images Figure 1 Figure 2 Figure 3 Figure 4

Ross, C. W.; Schlegelmilch, J. A.; Grogan, T. M.; Weiss, L. M.; Schnitzer, B.; Hanson, C. A.

1992-01-01

330

Neoadjuvant temozolomide followed by complete resection of a 1p- and 19q-deleted anaplastic oligoastrocytoma: Case study1  

Digital Repository Infrastructure Vision for European Research (DRIVER)

A 38-year-old woman presented with an infiltrative tumor of the right frontal lobe and genu of the corpus callosum that was deemed only partially resectable. A stereotactic biopsy was performed, which revealed a right frontal oligoastrocytoma that had some anaplastic features as well as allelic loss...

Voloschin, Alfredo D.; Louis, David N.; Cosgrove, Garth R.; Batchelor, Tracy T.

331

A crucial role for the Anaplastic lymphoma kinase receptor tyrosine kinase in gut development in Drosophila melanogaster  

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The Drosophila melanogaster gene Anaplastic lymphoma kinase (Alk) is homologous to mammalian Alk, which encodes a member of the Alk/Ltk family of receptor tyrosine kinases (RTKs). In humans, the t(2;5) translocation, which involves the ALK locus, produces an active form of ALK, which is ...

Lorén, Christina E.; Englund, Camilla; Grabbe, Caroline; Hallberg, Bengt; Hunter, Tony; Palmer, Ruth H.

332

Autocrine release of interleukin-9 promotes Jak3-dependent survival of ALK+ anaplastic large-cell lymphoma cells  

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The aberrant fusion protein NPM-ALK plays an important pathogenetic role in ALK+ anaplastic large-cell lymphoma (ALCL). We previously demonstrated that Jak3 potentiates the activity of NPM-ALK. Jak3 activation is restricted to interleukins that recruit the common ? chain (?c) receptor, including IL-...

Qiu, Lin; Lai, Raymond; Lin, Quan; Lau, Esther; Thomazy, David M.; Calame, Daniel; Ford, Richard J.; Kwak, Larry W.

333

[CD30-positive anaplastic large cell T-cell lymphoma developing during immunosuppressive therapy of pityriasis rubra pilaris with ustekinumab].  

UK PubMed Central (United Kingdom)

The development of malignancies during therapy with biologics has been discussed controversially. A patient with extensive pityriasis rubra pilaris failed to respond to standard therapeutic approaches. While receiving immunomodulatory therapy, lastly with ustekinumab, the patient developed a CD30(+) anaplastic large cell lymphoma.

Humme D; Beyer M; Röwert-Huber HJ; Sterry W; Philipp S

2013-03-01

334

Anaplastic Ganglioglioma in a Middle-aged Woman: A Case Report with a Review of the Literature  

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We report a case of anaplastic ganglioglioma. A 45-yr-old woman was admitted with a 5-month history of headache and dizziness, both of which progressed slowly. Preoperative magnetic resonance imaging revealed a strong enhancing mass in the left frontal lobe extending to the cingulate gyrus. Adjuvant...

Kang, Dong-Ho; Lee, Chul-Hee; Hwang, Soo-Hyun; Park, In-Sung; Han, Jong-Woo; Jung, Jin-Myung

335

Epithelial Proliferation in Small Ducts of Salivary Cystadenoma Resembling Atypical Ductal Hyperplasia of Breast  

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Salivary gland cystadenomas are cystic neoplasms with diverse architecture and cytology. Cystadenomas may have a considerable intracystic epithelial component, but an epithelial proliferation in small ducts and cysts resembling atypical ductal hyperplasia of breast has not been documented. The patie...

Fahim, Lisa; Weinreb, Ilan; Alexander, Cherupushpam; Perez Ordoñez, Bayardo

336

Trypanosoma cruzi: extracellular amastigote-resembling forms induced by chicken and human plasma.  

Science.gov (United States)

Chicken and human plasma and some of their proteins, partially isolated by means of isoelectrofocusing and ion-exchange chromatography, induce changes in the epimastigotes of Trypanosoma (Schizotrypanum) cruzi into forms resembling extracellular amastigotes in synthetic media. PMID:3896837

Zaidenberg, A J

1985-10-01

337

18F-FDG PET/CT in the Diagnosis of Tumor Thrombus from Anaplastic Thyroid Carcinoma in a Young Boy  

Directory of Open Access Journals (Sweden)

Full Text Available Anaplastic thyroid carcinoma is an uncommon, highly aggressive malignancy usually presenting in the elderly. An eighteen year old boy was recently diagnosed as anaplastic carcinoma of the thyroid.  PET/CECT scan performed for staging, revealed a large FDG avid heterogeneously enhancing thyroid mass with bilateral jugular venous thrombosis, which also showed increased FDG uptake, thus pointing towards tumor thrombus. To our knowledge, this is the first case wherein the PET/CT diagnosis of tumor thrombosis from anaplastic thyroid carcinoma was made in a young patient.

Maria M D’Souza; Abhinav Jaimini; Rajnish Sharma; Madhavi Tripathi; Dinesh Singh; Santosh Pandey; Anupam Mondal

2010-01-01

338

Developing retroperitoneal anaplastic carcinoma with choriocarcinoma focus after ovarian non-gestastional choriocarcinoma: Case report  

Directory of Open Access Journals (Sweden)

Full Text Available Introduction. Choriocarcinoma is a malignant form of gestational trophoblastic neoplasm (GTN). It is a rare event but also a curable malignancy. In the majority of instancies it developes after any gestational event. In some cases it developes as non-gestational extrauterine malignancy. Prognosis of choriocarcinoma is poor when invasion and metastases appear early and spread fast. This form of choriocarcinoma can lead to incurable and letal outcome. Case report. We presented a 20-year-old patient with abdominal and retroperitoneal malignancy - anaplastic carcinoma combined with choriocarcinoma metastases in. Tumor developed three months after left adnexectomy which had been done because of adnexal tumor. Choriocarcinoma was immunohistochemicaly confirmed in adnexal masses. Two courses of chemotherapy, metotrexate + folic acid (MTX+FA) regimen, were administrated. The initial serum beta human chorionic gonadotropin level stayed unknown as well as the last one after the treatment. The patient came from the other country and was hospitalized because of pelvic and abdominal pain and palpable abdominal masses in hypogastrium with progressive anemia. The human chorionic gonadotropin level was 38 mIU/L. Tumor biopsy was done and choriocarcinoma metastases were immunohistochemicaly confirmed with predominant anaplastic carcinoma. Five day course of MTX + cyclophosphamide regimen was administrated and the patient was prepared for operative treatment. Relaparotomy was perforemed and tumor completely exceeded. Tumor mass mostly developed retroperitonely and partialy in abdominal cavity infiltrating intestinal wall with rupture of sigmoid colon. Anaplastic carcinoma, with large fields of necrosis and bleeding, was confirmed after histological examination. Immunohistochemical examination excluded choriocarcinoma in tumor mass. After 20 blood units transfusion, one course of chemotherapy and tumor excision, the patient left hospital on the 9th postoperative day. The patient rejected chemotherapy which was recommended according to the protocol and died one month after the operation. Conclusion. Non-gestational metastatic choriocarcinoma complicated with another type of malignancy with early spread of the disease and low responsiriness to chemotherapy has poor prognosis and leads to lethal outocome. [Acknowledgment. Projekat Ministarstva nauke Republike Srbije, br. 41021 and 175082

Nikoli? Branka; Ljubi? Aleksandar; Terzi? Milan; Aran?elovi? Aleksandra; Babi? Sr?an; Vu?i? Miloš

2012-01-01

339

New targeted therapies and other advances in the management of anaplastic thyroid cancer.  

UK PubMed Central (United Kingdom)

PURPOSE OF REVIEW: Anaplastic thyroid cancer is the most aggressive solid tumor known to humans. Even when found in a localized form, the prognosis is grave. For metastatic disease, there has been little effect on survival using traditional chemotherapy. RECENT FINDINGS: Over a five-decade interval, there has been little progress in the treatment of this malignancy. However, targeted agents represent a new mode of treatment, and some studies have shown encouraging preclinical results. Combretastatin has shown activity in phase 1 and phase II trials; although the registration phase III study failed to meet its accrual goals, it did appear to show some benefit, especially in younger patients. SUMMARY: Combinations of this compound and other targeted agents may prove to be a breakthrough in an otherwise untreatable cancer.

Deshpande HA; Roman S; Sosa JA

2013-01-01

340

Diagnostic assays for identification of anaplastic lymphoma kinase-positive non-small cell lung cancer.  

UK PubMed Central (United Kingdom)

In series dominated by adenocarcinoma histology, approximately 5% of non-small cell lung cancers (NSCLCs) harbor an anaplastic lymphoma kinase (ALK) gene rearrangement. Crizotinib, a tyrosine kinase inhibitor with significant activity against ALK, has demonstrated high response rates and prolonged progression-free survival in ALK-positive patients enrolled in phase 1/2 clinical trials. In 2011, crizotinib received accelerated approval from the US Food and Drug Administration (FDA) for the treatment of proven ALK-positive NSCLC using an FDA-approved diagnostic test. Currently, only break-apart fluorescence in situ hybridization testing is FDA approved as a companion diagnostic for crizotinib; however, many other assays are available or in development. In the current review, the authors summarize the diagnostic tests available, or likely to become available, that could be used to identify patients with ALK-positive NSCLC, highlighting the pros and cons of each.

Weickhardt AJ; Aisner DL; Franklin WA; Varella-Garcia M; Doebele RC; Camidge DR

2013-04-01

 
 
 
 
341

Anaplastic lymphoma kinase expression and prognosis in inflammatory myofibroblastic tumours of the maxillary sinus.  

Science.gov (United States)

Inflammatory myofibroblastic tumours (IMT) of the nasal cavity and nasal sinus are rare and, although over 50 cases have been reported in the English-language literature, their precise aetiology and biological behaviour have not been elucidated. Recent studies suggest that anaplastic lymphoma kinase (ALK)-positive tumours have a very low risk of metastasis, but ALK reactivity does not appear to correlate with recurrence. Between March 2002 and December 2008, we encountered three cases of maxillary sinus IMT and investigated them to determine the clinicopathological course, prognosis and immunohistochemical expression of ALK. Two of the patients died immunohisto chemically negative for ALK expression. IMT of the sino-nasal tract is rare and may undergo malignant transformation in a minority of cases. The three cases manifested progressive extension with bone destruction and multiple recurrences, and two cases had a fatal outcome and one case had high recurrence. PMID:20146901

Lu, Z-J; Zhou, S-H; Yan, S-X; Yao, H-T

342

Brentuximab vedotin: treatment role for relapsed refractory systemic anaplastic large-cell lymphoma.  

Science.gov (United States)

The identification of CD30 has been known since 1985. Trials exploring the targeted therapy focusing on this antigen have led to the successful development and approval of brentuximab vedotin (Adcetris®) for treatment of relapsed refractory systemic anaplastic large-cell lymphoma. Brentuximab vedotin has a high-level of response with generally durable remission. Common side effects of brentuximab vedotin include peripheral neuropathy, fatigue, nausea, arthralgia, and pyrexia. Grade 3-4 neutropenia, thrombocytopenia, and hyperglycemia have also been reported. Development of progressive multifocal leukoencephalopathy from John Cunningham virus is a very rare occurrence, but its seriousness has prompted the US FDA to mandate a black box warning. Brentuximab vedotin is currently being evaluated to be used in conjunction with other chemotherapy regimens, including in frontline therapies to induce potentially higher complete remission rate than chemotherapy alone and thus achieve potentially higher progression-free survival rates that might translate ultimately to improve overall survival. PMID:23991923

Lai, Chao-Ming; Horowitz, Sandra

2013-08-01

343

[Ki-1-positive anaplastic large-cell lymphoma with mediastinal involvement].  

Science.gov (United States)

A 30-year-old woman was admitted to our hospital because of an abnormal mass shadow in the right side of the mediastinum on a a chest X-ray film. The chest X-ray film and computed tomogram revealed a mass approximately 4.8 cm in diameter in the right side of the mediastinum. The mass had invaded the right supraclavicular fossa. Lymph-node biopsy was done. Microscopical examination of an HE-stained specimen of a supraclavicular lymph nodes showed prediferation of large atypical lymphocytes. These atypical lymphocytes were stained immunohistochemically with anti-Ki-1 antibody. No T cell or B cell markers were found on these cells. No other tumors were detected by radiological examination. Therefore, our diagnosis was anaplastic large-cell lymphoma, which is identical to Ki-1 lymphoma of the mediastinum. PMID:9294301

Makiguchi, N; Ohsaki, Y; Fujiuchi, S; Yamamoto, Y; Takahashi, T; Ide, H; Akiba, Y; Nakano, H; Miyokawa, N; Kikuchi, K

1997-06-01

344

Conduction Aphasia as a Result of Left Parietal-Temporal-Occipital Anaplastic Astrocytoma: A Case Study  

Directory of Open Access Journals (Sweden)

Full Text Available Conduction aphasia is a language disorder characterized by an impaired ability to repeat verbal material associated with phonological paraphasias but a relatively fluent spontaneous speech and preserved comprehension. It has been attributed to lesions of the arcuate fasciculus by disconnection between posterior temporal lobe and frontal lobe, however, this idea has been debated, because the integrity and function of the arcuate fasciculus does not seem to be essential in verbal repetition. We report a case of a 23 year old male, with conduction aphasia as a result of a recurrent anaplastic astrocytoma in parietal and temporo-occipital areas. We propose a reconceptualization of the aphasia, analyzing it in terms of clinical neuropsychological and neural networks between ipsilateral and contralateral posterior brain areas

Oscar Mauricio Aguilar Mejía; Beatriz Ramírez Bermejo; Juan Carlos Acevedo González; Miguel Enrique Berbeo Calderón

2011-01-01

345

Primary cutaneous CD30-positive anaplastic large-cell lymphoma of the external auditory canal.  

Science.gov (United States)

Primary cutaneous T-cell lymphoma is rare. Cutaneous lymphoma is defined as primary when there is an absence of nodal or systemic disease during the first 6 months following diagnosis. We report what we believe to be the first documented case of a primary cutaneous CD30-positive anaplastic large-cell lymphoma of the external auditory canal. The patient was an elderly woman who presented with progressively worsening right otalgia and hypoacusis. Otoscopy revealed an erythematic, ulcerative, nonbleeding, localized lesion in the anterosuperior area of the external auditory canal. The patient underwent an excisional biopsy, and after the diagnosis was established, she underwent 22 sessions of radiotherapy. During follow-up, she exhibited no evidence of recurrence. PMID:23288823

Marçal, Nuno; Campelos, Sofia; Dias, Luís; Gonçalves, Matos; Pereira, Gabriel; Godinho, Tiago

2012-12-01

346

Primary cutaneous CD30-positive anaplastic large-cell lymphoma of the external auditory canal.  

UK PubMed Central (United Kingdom)

Primary cutaneous T-cell lymphoma is rare. Cutaneous lymphoma is defined as primary when there is an absence of nodal or systemic disease during the first 6 months following diagnosis. We report what we believe to be the first documented case of a primary cutaneous CD30-positive anaplastic large-cell lymphoma of the external auditory canal. The patient was an elderly woman who presented with progressively worsening right otalgia and hypoacusis. Otoscopy revealed an erythematic, ulcerative, nonbleeding, localized lesion in the anterosuperior area of the external auditory canal. The patient underwent an excisional biopsy, and after the diagnosis was established, she underwent 22 sessions of radiotherapy. During follow-up, she exhibited no evidence of recurrence.

Marçal N; Campelos S; Dias L; Gonçalves M; Pereira G; Godinho T

2012-12-01

347

Small Cell Anaplastic Carcinoma of Primary Lung Tumor in a Miniature Schnauzer Dog  

Directory of Open Access Journals (Sweden)

Full Text Available A seven-year-old male, an intact miniature Schnauzer dog with history of vomiting, abdominal distention, anorexia, and dyspnea was referred for further evaluation and treatment. Thoracic radiographs showed the well marginated solitary mass with soft density in the right caudal lung field, and abdominal radiographs showed signs of ascites, such as abdominal distention and moderate serosal detail loss. On ultrasonograph and computed tomograph, it was observed that the mass compressed the caudal vena cava (CVC) and adhered to the heart. Exploratory thoracotomy was performed, and then it was showed that mass adhered heart, CVC, and diaphragm. The mass was fully resected although adhered part of CVC could not be completely resected. On histopathological findings, the mass was diagnosed as small-cell anaplastic carcinoma.

J. M. Kim, H. J. Han, B. Ku, G. Kim, K. M. Shim1, S. S. Kang2 and S. H. Choi*

2011-01-01

348

A huge intraventricular congenital anaplastic astrocytoma: case report with histopathological and genetic consideration.  

UK PubMed Central (United Kingdom)

Congenital malignant gliomas are rare brain tumors about which few reports have been published. We present the clinical course and genetic alterations in an infant with a congenital malignant glioma detected incidentally by ultrasonography at 36 weeks. The tumor occupied the right temporoparietal region, extended to the posterior fossa, and significantly compressed surrounding structures. The female infant was entirely normal without macrocrania, tense fontanel, or sucking difficulties. The tumor was subtotally resected by two-stage surgery; pathological diagnosis was anaplastic astrocytoma. Immunohistochemical staining was positive for p53 and negative for epidermal growth factor receptor. There was no O(6)-methylguanine-DNA methyltransferase (MGMT) gene promoter methylation, no 1p/19q loss of heterozygosity, and no isocitrate dehydrogenase 1 (IDH1) mutation. She underwent postoperative chemotherapy and is alive and well 12 months after surgery.

Yamashita S; Ryu S; Miyata S; Uchinokura S; Yokogami K; Uehara H; Moriguchi S; Iwakiri T; Marutsuka K; Ikenoue M; Sawa D; Yamada N; Kodama Y; Takeshima H

2012-04-01

349

Lymphomatoid Papulosis Followed by Anaplastic Large Cell Lymphoma in a Pediatric Patient  

Science.gov (United States)

Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP. However, this transformation rarely occurs in patients younger than 20 years of age. Here, we present the first known pediatric patient in Korea, a 12-year-old boy who developed a subcutaneous nodule on the scrotum 13 months after papulonecrotic lesions of LyP were identified on both lower extremities and face. Histological and immunohistochemical examination of the subcutaneous nodule revealed anaplastic large cell lymphoma (ALCL). A T-cell receptor gene rearrangement analysis demonstrated an identical rearranged pattern in the two specimens, indicating that a common T-cell clone had proliferated over time in both the LyP and ALCL lesions.

Min, Jung Ah; Oh, Shin Taek; Kim, Jung Eun; Cho, Baik Kee; Chung, Nak Gyun

2010-01-01

350

Brentuximab vedotin: treatment role for relapsed refractory systemic anaplastic large-cell lymphoma.  

UK PubMed Central (United Kingdom)

The identification of CD30 has been known since 1985. Trials exploring the targeted therapy focusing on this antigen have led to the successful development and approval of brentuximab vedotin (Adcetris(®)) for treatment of relapsed refractory systemic anaplastic large-cell lymphoma. Brentuximab vedotin has a high-level of response with generally durable remission. Common side effects of brentuximab vedotin include peripheral neuropathy, fatigue, nausea, arthralgia, and pyrexia. Grade 3-4 neutropenia, thrombocytopenia, and hyperglycemia have also been reported. Development of progressive multifocal leukoencephalopathy from John Cunningham virus is a very rare occurrence, but its seriousness has prompted the US FDA to mandate a black box warning. Brentuximab vedotin is currently being evaluated to be used in conjunction with other chemotherapy regimens, including in frontline therapies to induce potentially higher complete remission rate than chemotherapy alone and thus achieve potentially higher progression-free survival rates that might translate ultimately to improve overall survival.

Lai CM; Horowitz S

2013-08-01

351

MicroRNA 96 Is a Post-Transcriptional Suppressor of Anaplastic Lymphoma Kinase Expression  

Science.gov (United States)

Anaplastic lymphoma kinase (ALK) constitutes a part of the oncogenic fusion proteins nucleophosmin-ALK and echinoderm microtubule–associated protein like 4–ALK, which are aberrantly expressed in a subset of T-cell anaplastic large-cell lymphoma and non–small-cell lung cancer, respectively. The expression of mutated, constitutively active ALK also occurs in a subset of neuroblastoma tumors. ALK is believed to play an important role in promoting tumor survival. Nevertheless, the mechanisms underlying the expression of ALK in cancer cells are not completely known. MicroRNA (miR) has been implicated in the regulation of the expression of both oncogenes and tumor suppressor genes. We tested the hypothesis that the expression of ALK could be regulated by miR. Three Internet-based algorithms identified miR-96 to potentially bind with the ALK 3?-untranslated region. Notably, miR-96 levels were markedly decreased in ALK-expressing cancer cell lines and primary human tumors compared with their normal cellular and tissue counterparts. Transfection of the cell lines with miR-96 decreased levels of the different forms of ALK protein, without significant effects on ALK mRNA. Furthermore, miR-96 decreased the phosphorylation of ALK target proteins, including Akt, STAT3, JNK, and type I insulin-like growth factor receptor, and it down-regulated JunB. These effects were associated with reduced proliferation, colony formation, and migration of ALK-expressing cancer cells. These data provide novel evidence that decreases in miR-96 could represent a mechanism underlying the aberrant expression of ALK in cancer cells.

Vishwamitra, Deeksha; Li, Yong; Wilson, Desiree; Manshouri, Roxsan; Curry, Choladda V.; Shi, Bin; Tang, Xi Ming; Sheehan, Andrea M.; Wistuba, Ignacio I.; Shi, Ping; Amin, Hesham M.

2012-01-01

352

microRNA expression profiling identifies molecular signatures associated with anaplastic large cell lymphoma.  

Science.gov (United States)

Anaplastic large-cell lymphomas (ALCLs) encompass at least 2 systemic diseases distinguished by the presence or absence of anaplastic lymphoma kinase (ALK) expression. We performed genome-wide microRNA (miRNA) profiling on 33 ALK-positive (ALK[+]) ALCLs, 25 ALK-negative (ALK[-]) ALCLs, 9 angioimmunoblastic T-cell lymphomas, 11 peripheral T-cell lymphomas not otherwise specified (PTCLNOS), and normal T cells, and demonstrated that ALCLs express many of the miRNAs that are highly expressed in normal T cells with the prominent exception of miR-146a. Unsupervised hierarchical clustering demonstrated distinct clustering of ALCL, PTCL-NOS, and the AITL subtype of PTCL. Cases of ALK(+) ALCL and ALK(-) ALCL were interspersed in unsupervised analysis, suggesting a close relationship at the molecular level. We identified an miRNA signature of 7 miRNAs (5 upregulated: miR-512-3p, miR-886-5p, miR-886-3p, miR-708, miR-135b; 2 downregulated: miR-146a, miR-155) significantly associated with ALK(+) ALCL cases. In addition, we derived an 11-miRNA signature (4 upregulated: miR-210, miR-197, miR-191, miR-512-3p; 7 downregulated: miR-451, miR-146a, miR-22, miR-455-3p, miR-455-5p, miR-143, miR-494) that differentiates ALK(-) ALCL from other PTCLs. Our in vitro studies identified a set of 32 miRNAs associated with ALK expression. Of these, the miR-17?92 cluster and its paralogues were also highly expressed in ALK(+) ALCL and may represent important downstream effectors of the ALK oncogenic pathway. PMID:23801630

Liu, Cuiling; Iqbal, Javeed; Teruya-Feldstein, Julie; Shen, Yulei; Dabrowska, Magdalena Julia; Dybkaer, Karen; Lim, Megan S; Piva, Roberto; Barreca, Antonella; Pellegrino, Elisa; Spaccarotella, Elisa; Lachel, Cynthia M; Kucuk, Can; Jiang, Chun-Sun; Hu, Xiaozhou; Bhagavathi, Sharathkumar; Greiner, Timothy C; Weisenburger, Dennis D; Aoun, Patricia; Perkins, Sherrie L; McKeithan, Timothy W; Inghirami, Giorgio; Chan, Wing C

2013-06-25

353

microRNA expression profiling identifies molecular signatures associated with anaplastic large cell lymphoma.  

UK PubMed Central (United Kingdom)

Anaplastic large-cell lymphomas (ALCLs) encompass at least 2 systemic diseases distinguished by the presence or absence of anaplastic lymphoma kinase (ALK) expression. We performed genome-wide microRNA (miRNA) profiling on 33 ALK-positive (ALK[+]) ALCLs, 25 ALK-negative (ALK[-]) ALCLs, 9 angioimmunoblastic T-cell lymphomas, 11 peripheral T-cell lymphomas not otherwise specified (PTCLNOS), and normal T cells, and demonstrated that ALCLs express many of the miRNAs that are highly expressed in normal T cells with the prominent exception of miR-146a. Unsupervised hierarchical clustering demonstrated distinct clustering of ALCL, PTCL-NOS, and the AITL subtype of PTCL. Cases of ALK(+) ALCL and ALK(-) ALCL were interspersed in unsupervised analysis, suggesting a close relationship at the molecular level. We identified an miRNA signature of 7 miRNAs (5 upregulated: miR-512-3p, miR-886-5p, miR-886-3p, miR-708, miR-135b; 2 downregulated: miR-146a, miR-155) significantly associated with ALK(+) ALCL cases. In addition, we derived an 11-miRNA signature (4 upregulated: miR-210, miR-197, miR-191, miR-512-3p; 7 downregulated: miR-451, miR-146a, miR-22, miR-455-3p, miR-455-5p, miR-143, miR-494) that differentiates ALK(-) ALCL from other PTCLs. Our in vitro studies identified a set of 32 miRNAs associated with ALK expression. Of these, the miR-17?92 cluster and its paralogues were also highly expressed in ALK(+) ALCL and may represent important downstream effectors of the ALK oncogenic pathway.

Liu C; Iqbal J; Teruya-Feldstein J; Shen Y; Dabrowska MJ; Dybkaer K; Lim MS; Piva R; Barreca A; Pellegrino E; Spaccarotella E; Lachel CM; Kucuk C; Jiang CS; Hu X; Bhagavathi S; Greiner TC; Weisenburger DD; Aoun P; Perkins SL; McKeithan TW; Inghirami G; Chan WC

2013-09-01

354

Overexpression of c-erbB2 is a negative prognostic factor in anaplastic astrocytomas  

Directory of Open Access Journals (Sweden)

Full Text Available Abstract The epidermal growth factor receptor (EGFR) family, consisting of four tyrosine kinase receptors, c-erbB1-4, seems to be influential in gliomagenesis. The aim of this study was to investigate EGFR gene amplification and expression of c-erbB1-4 receptor proteins in human anaplastic astrocytomas. Formalin-fixed and paraffin-embedded sections from 31 cases were investigated by standard immunohistochemical procedures for expression of c-erbB1-4 receptor proteins using commercial antibodies. EGFR gene amplification was studied by fluorescence in situ hybridization using paraffin-embedded tissues. Two monoclonal antibodies, NCL-EGFR-384 and NCL-EGFR, were used for EGFR detection and they displayed positive immunoreactivity in 97% and 71%, respectively. For c-erbB2 detection three monoclonal antibodies, CB11, 3B5, and 5A2, were applied and they displayed positive immunoreactivity in 45%, 100%, and 52%, respectively. Positive immunostaining for c-erbB3 and c-erbB4 was encountered in 97% and 74%, respectively. The EGFR gene was amplified in 9 out of 31 tumors (29%). After adjusting for age, Karnofsky performance status, and extent of surgical resection, Cox multiple regression analysis with overall survival as the dependent variable revealed that c-erbB2 overexpression detected by the monoclonal antibody clone CB11 was a statistically significant poor prognostic factor (P = 0.004). This study shows the convenience and feasibility of immunohistochemistry when determining the expression of receptor proteins in tissue sections of human astrocytomas. The synchronous overexpression of c-erbB1-4 proteins in anaplastic astrocytomas supports their role in the pathogenesis of these tumors. Further, c-erbB2 overexpression seems to predict aggressive behaviour.

Gulati Sasha; Ytterhus Borgny; Granli Unn S; Gulati Michel; Lydersen Stian; Torp Sverre H

2010-01-01

355

MicroRNA expression profiling identifies molecular signatures associated with anaplastic large cell lymphoma  

DEFF Research Database (Denmark)

Anaplastic large-cell lymphomas (ALCLs) encompass at least 2 systemic diseases distinguished by the presence or absence of anaplastic lymphoma kinase (ALK) expression. We performed genome-wide microRNA (miRNA) profiling on 33 ALK-positive (ALK[+]) ALCLs, 25 ALK-negative (ALK[-]) ALCLs, 9 angioimmunoblastic T-cell lymphomas, 11 peripheral T-cell lymphomas not otherwise specified (PTCLNOS), and normal T cells, and demonstrated that ALCLs express many of the miRNAs that are highly expressed in normal T cells with the prominent exception of miR-146a. Unsupervised hierarchical clustering demonstrated distinct clustering of ALCL, PTCL-NOS, and the AITL subtype of PTCL. Cases of ALK(+) ALCL and ALK(-) ALCL were interspersed in unsupervised analysis, suggesting a close relationship at the molecular level. We identified an miRNA signature of 7 miRNAs (5 upregulated: miR-512-3p, miR-886-5p, miR-886-3p, miR-708, miR-135b; 2 downregulated: miR-146a, miR-155) significantly associated with ALK(+) ALCL cases. In addition, we derived an 11-miRNA signature (4 upregulated: miR-210, miR-197, miR-191, miR-512-3p; 7 downregulated: miR-451, miR-146a, miR-22, miR-455-3p, miR-455-5p, miR-143, miR-494) that differentiates ALK(-) ALCL from other PTCLs. Our in vitro studies identified a set of 32 miRNAs associated with ALK expression. Of these, the miR-17?92 cluster and its paralogues were also highly expressed in ALK(+) ALCL and may represent important downstream effectors of the ALK oncogenic pathway.

Liu, Cuiling; Iqbal, Javeed

2013-01-01

356

Frequent expression of follicular dendritic cell markers in Hodgkin lymphoma and anaplastic large cell lymphoma.  

UK PubMed Central (United Kingdom)

AIMS: Although the tumour cells of Hodgkin lymphoma (HL) are derived from mature B-cells, the lineage infidelity of Hodgkin/Reed-Sternberg cells (HRSs) often causes diagnostic problems. Recently introduced HRS markers are also positive for follicular dendritic cells (FDCs). We investigated the expression of several FDC markers in HL and anaplastic large cell lymphoma (ALCL) and evaluated their diagnostic efficacy. METHODS: Eighty-five cases of HL and 52 cases of ALCL were included in this study. Immunohistochemistry was performed for glioma-associated homologue (GLI) 3, class III ?-tubulin (TUBB3), fascin, clusterin, ?-synuclein, podoplanin, syntenin, CD21, CD35 and EGFR. RESULTS: HRSs were diffusely positive for GLI3, fascin and TUBB3; the mean positivity rates per case were 94% for GLI3, 82% for fascin, 69% for TUBB3, 17% for clusterin, 17% for ?-synuclein and 14% for syntenin. Podoplanin, CD21, CD35 and EGFR were almost negative. However, the frequency of marker expression was not associated with the histologic subtype or the presence of Epstein-Barr virus (EBV). ALCL showed a similar pattern to HL, but the overall frequency of positivity was lower than that observed in HL. The mean positivity rates were 56% for GLI3, 62% for fascin, 58% for TUBB3 and 21% for clusterin. The other markers were nearly negative. Anaplastic large cell lymphoma kinase positivity did not affect the expression rates. CONCLUSIONS: This study confirmed the frequent expression of FDC markers in HL and ALCL. Especially, GLI3, fascin and TUBB3 are the most sensitive markers. Further studies are required to evaluate the association between FDCs, HRSs and ALCL cells.

Kim SH; Choe JY; Jeon Y; Huh J; Jung HR; Choi YD; Kim HJ; Cha HJ; Park WS; Kim JE

2013-07-01

357

Primary cutaneous and systemic anaplastic large cell lymphoma: clinicopathologic aspects and therapeutic options.  

UK PubMed Central (United Kingdom)

Anaplastic large cell lymphoma (ALCL) is a biologic and clinically heterogenous subtype of T-cell lymphoma. Clinically, ALCL may present as localized (primary) cutaneous disease or widespread systemic disease. These two forms of ALCL are distinct entities with different clinical and biologic features. Both types share similar histology, however, with cohesive sheets of large lymphoid cells expressing the Ki-1 (CD30) molecule. Primary cutaneous ALCL (C-ALCL) is part of the spectrum of CD30+ lymphoproliferative diseases of the skin including lymphomatoid papulosis. Using conservative measures, 5-year disease-free survival rates are > 90%. The systemic ALCL type is an aggressive lymphoma that may secondarily involve the skin, in addition to other extranodal sites. Further, systemic ALCL may be divided based on the expression of anaplastic lymphoma kinase (ALK) protein, which is activated most frequently through the nonrandom t(2;5) chromosome translocation, causing the fusion of the nucleophosmin (NPM) gene located at 5q35 to 2p23 encoding the receptor tyrosine kinase ALK. Systemic ALK+ ALCLs have improved prognosis compared with ALK-negative ALCL, although both subtypes warrant treatment with polychemotherapy. Allogeneic and, to a lesser extent, autologous stem cell transplantation play a role in relapsed disease, while the benefit of upfront transplant is not clearly defined. Treatment options for relapsed patients include agents such as pralatrexate (Folotyn) and vinblastine. In addition, a multitude of novel therapeutics are being studied, including anti-CD30 antibodies, histone deacetylase inhibitors, immunomodulatory drugs, proteasome inhibitors, and inhibitors of ALK and its downstream signaling pathways. Continued clinical trial involvement by oncologists and patients is imperative to improve the outcomes for this malignancy.

Querfeld C; Khan I; Mahon B; Nelson BP; Rosen ST; Evens AM

2010-06-01

358

Primary cutaneous and systemic anaplastic large cell lymphoma: clinicopathologic aspects and therapeutic options.  

Science.gov (United States)

Anaplastic large cell lymphoma (ALCL) is a biologic and clinically heterogenous subtype of T-cell lymphoma. Clinically, ALCL may present as localized (primary) cutaneous disease or widespread systemic disease. These two forms of ALCL are distinct entities with different clinical and biologic features. Both types share similar histology, however, with cohesive sheets of large lymphoid cells expressing the Ki-1 (CD30) molecule. Primary cutaneous ALCL (C-ALCL) is part of the spectrum of CD30+ lymphoproliferative diseases of the skin including lymphomatoid papulosis. Using conservative measures, 5-year disease-free survival rates are > 90%. The systemic ALCL type is an aggressive lymphoma that may secondarily involve the skin, in addition to other extranodal sites. Further, systemic ALCL may be divided based on the expression of anaplastic lymphoma kinase (ALK) protein, which is activated most frequently through the nonrandom t(2;5) chromosome translocation, causing the fusion of the nucleophosmin (NPM) gene located at 5q35 to 2p23 encoding the receptor tyrosine kinase ALK. Systemic ALK+ ALCLs have improved prognosis compared with ALK-negative ALCL, although both subtypes warrant treatment with polychemotherapy. Allogeneic and, to a lesser extent, autologous stem cell transplantation play a role in relapsed disease, while the benefit of upfront transplant is not clearly defined. Treatment options for relapsed patients include agents such as pralatrexate (Folotyn) and vinblastine. In addition, a multitude of novel therapeutics are being studied, including anti-CD30 antibodies, histone deacetylase inhibitors, immunomodulatory drugs, proteasome inhibitors, and inhibitors of ALK and its downstream signaling pathways. Continued clinical trial involvement by oncologists and patients is imperative to improve the outcomes for this malignancy. PMID:20669794

Querfeld, Christiane; Khan, Irum; Mahon, Brett; Nelson, Beverly P; Rosen, Steven T; Evens, Andrew M

2010-06-01

359

Presence of an oligodendroglioma-like component in newly diagnosed glioblastoma identifies a pathogenetically heterogeneous subgroup and lacks prognostic value: central pathology review of the EORTC_26981/NCIC_CE.3 trial.  

UK PubMed Central (United Kingdom)

Glioblastoma (GBM) is a morphologically heterogeneous tumor type with a median survival of only 15 months in clinical trial populations. However, survival varies greatly among patients. As part of a central pathology review, we addressed the question if patients with GBM displaying distinct morphologic features respond differently to combined chemo-radiotherapy with temozolomide. Morphologic features were systematically recorded for 360 cases with particular focus on the presence of an oligodendroglioma-like component and respective correlations with outcome and relevant molecular markers. GBM with an oligodendroglioma-like component (GBM-O) represented 15% of all confirmed GBM (52/339) and was not associated with a more favorable outcome. GBM-O encompassed a pathogenetically heterogeneous group, significantly enriched for IDH1 mutations (19 vs. 3%, p = 0.003) and EGFR amplifications (71 vs. 48%, p = 0.04) compared with other GBM, while co-deletion of 1p/19q was found in only one case and the MGMT methylation frequency was alike (47 vs. 46%). Expression profiles classified most of the GBM-O into two subtypes, 36% (5/14 evaluable) as proneural and 43% as classical GBM. The detection of pseudo-palisading necrosis (PPN) was associated with benefit from chemotherapy (p = 0.0002), while no such effect was present in the absence of PPN (p = 0.86). In the adjusted interaction model including clinical prognostic factors and MGMT status, PPN was borderline nonsignificant (p = 0.063). Taken together, recognition of an oligodendroglioma-like component in an otherwise classic GBM identifies a pathogenetically mixed group without prognostic significance. However, the presence of PPN may indicate biological features of clinical relevance for further improvement of therapy.

Hegi ME; Janzer RC; Lambiv WL; Gorlia T; Kouwenhoven MC; Hartmann C; von Deimling A; Martinet D; Besuchet Schmutz N; Diserens AC; Hamou MF; Bady P; Weller M; van den Bent MJ; Mason WP; Mirimanoff RO; Stupp R; Mokhtari K; Wesseling P

2012-06-01

360

Presence of an oligodendroglioma-like component in newly diagnosed glioblastoma identifies a pathogenetically heterogeneous subgroup and lacks prognostic value: central pathology review of the EORTC_26981/NCIC_CE.3 trial.  

Science.gov (United States)

Glioblastoma (GBM) is a morphologically heterogeneous tumor type with a median survival of only 15 months in clinical trial populations. However, survival varies greatly among patients. As part of a central pathology review, we addressed the question if patients with GBM displaying distinct morphologic features respond differently to combined chemo-radiotherapy with temozolomide. Morphologic features were systematically recorded for 360 cases with particular focus on the presence of an oligodendroglioma-like component and respective correlations with outcome and relevant molecular markers. GBM with an oligodendroglioma-like component (GBM-O) represented 15% of all confirmed GBM (52/339) and was not associated with a more favorable outcome. GBM-O encompassed a pathogenetically heterogeneous group, significantly enriched for IDH1 mutations (19 vs. 3%, p = 0.003) and EGFR amplifications (71 vs. 48%, p = 0.04) compared with other GBM, while co-deletion of 1p/19q was found in only one case and the MGMT methylation frequency was alike (47 vs. 46%). Expression profiles classified most of the GBM-O into two subtypes, 36% (5/14 evaluable) as proneural and 43% as classical GBM. The detection of pseudo-palisading necrosis (PPN) was associated with benefit from chemotherapy (p = 0.0002), while no such effect was present in the absence of PPN (p = 0.86). In the adjusted interaction model including clinical prognostic factors and MGMT status, PPN was borderline nonsignificant (p = 0.063). Taken together, recognition of an oligodendroglioma-like component in an otherwise classic GBM identifies a pathogenetically mixed group without prognostic significance. However, the presence of PPN may indicate biological features of clinical relevance for further improvement of therapy. PMID:22249618

Hegi, Monika E; Janzer, Robert-Charles; Lambiv, Wanyu L; Gorlia, Thierry; Kouwenhoven, Mathilde C M; Hartmann, Christian; von Deimling, Andreas; Martinet, Danielle; Besuchet Schmutz, Nathalie; Diserens, Annie-Claire; Hamou, Marie-France; Bady, Pierre; Weller, Michael; van den Bent, Martin J; Mason, Warren P; Mirimanoff, René-Olivier; Stupp, Roger; Mokhtari, Karima; Wesseling, Pieter

2012-01-15

 
 
 
 
361

Atypical blood and bone marrow lymphocytes in ALK-positive anaplastic T-cell lymphoma: important lessons.  

UK PubMed Central (United Kingdom)

A 42-year-old man presented with a short history of fever, significant weight loss and bilateral neck swelling. A CT scan revealed widespread lymphadenopathy and hepatosplenomegaly. Lymph node morphology and immunohistochemistry confirmed a diagnosis of anaplastic lymphoma kinase (ALK)-positive anaplastic T-cell non-Hodgkin's lymphoma. Both the peripheral blood (PB) and bone marrow (BM) revealed atypical lymphocytes with cleaved nuclei, vacuolation and granulation, suggestive of disease involvement at both sites. However, BM and PB immunophenotyping and immunohistochemistry did not reveal typical markers (ALK and CD30) at either site. All features resolved with a short remission after conventional chemotherapy. Despite salvage chemotherapy and an autologous stem cell transplant, a relapse of the PB and BM features with stable radiological findings was noted. Rapid decline followed with pancytopenia resulting in death 12 months after the initial diagnosis.

Mahdi AJ; Elmusharaf N; Osman H; Thompson I

2013-01-01

362

Atypical blood and bone marrow lymphocytes in ALK-positive anaplastic T-cell lymphoma: important lessons.  

Science.gov (United States)

A 42-year-old man presented with a short history of fever, significant weight loss and bilateral neck swelling. A CT scan revealed widespread lymphadenopathy and hepatosplenomegaly. Lymph node morphology and immunohistochemistry confirmed a diagnosis of anaplastic lymphoma kinase (ALK)-positive anaplastic T-cell non-Hodgkin's lymphoma. Both the peripheral blood (PB) and bone marrow (BM) revealed atypical lymphocytes with cleaved nuclei, vacuolation and granulation, suggestive of disease involvement at both sites. However, BM and PB immunophenotyping and immunohistochemistry did not reveal typical markers (ALK and CD30) at either site. All features resolved with a short remission after conventional chemotherapy. Despite salvage chemotherapy and an autologous stem cell transplant, a relapse of the PB and BM features with stable radiological findings was noted. Rapid decline followed with pancytopenia resulting in death 12 months after the initial diagnosis. PMID:24038295

Mahdi, A J; Elmusharaf, N; Osman, H; Thompson, I

2013-09-13

363

Traumatic funicular phlebitis of the thoracic wall resembling Mondor's disease: a case report  

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Full Text Available Abstract Introduction Mondor's disease is a peculiar form of thrombophlebitis, involving a superficial vein in the subcutaneous fat of the breast or anterior chest wall. Case presentation The author presents a case of a 35-year-old male Japanese patient with cord-like induration in the right lateral thoracic wall. This lesion was diagnosed as traumatic funicular phlebitis, resembling Mondor's disease. Conclusion Traumatic funicular phlebitis, resembling Mondor's disease, is a clinical entity which may give suggestive insight to the etiology of Mondor's disease itself.

Kondo Takeshi

2011-01-01

364

Metachronous occurrence of a temporal ganglioglioma and a bifrontal anaplastic ependymoma: coincidence or an explainable pathological curiosity?  

UK PubMed Central (United Kingdom)

In this first-of-its-kind report, we describe an unusual case of a 32-year-old man who harboured two biologically distinct metachronous brain tumours. He presented with features of raised intracranial pressure 8 years after total excision of a right temporal ganglioglioma. Imaging showed a heterogenously contrast enhancing bifrontal lesion with ventricular extension. Histopathological examination revealed an anaplastic ependymoma. While a co-incidental occurrence is a possibility, there could be possible mechanisms to explain this pathological oddity.

Thakar S; Santosh V; Ghosal N; Furtado SV; Hegde AS

2011-12-01

365

Iodide uptake in human anaplastic thyroid carcinoma cells after transfer of the human thyroid peroxidase gene  

Energy Technology Data Exchange (ETDEWEB)

Human thyroperoxidase (hTPO) is critical for the accumulation of iodide in thyroid tissues. Poorly differentiated and anaplastic thyroid tumours which lack thyroid-specific gene expression fail to accumulate iodide and, therefore, do not respond to iodine-131 therapy. We consequently investigated whether transfer of the hTPO gene is sufficient to restore the iodide-trapping capacity in undifferentiated thyroid and non-thyroid tumour cells. The human anaplastic thyroid carcinoma cell lines C643 and SW1736, the rat Morris hepatoma cell line MH3924A and the rat papillary thyroid carcinoma cell line L2 were used as in vitro model systems. Employing a bicistronic retroviral vector based on the myeloproliferative sarcoma virus for the transfer of the hTPO and the neomycin resistance gene, the C643 cells and SW1736 cells were transfected while the L2 cells and MH3924A cells were infected with retroviral particles. Seven recombinant C643 and seven SW1736 cell lines as well as four recombinant L2 and four MH3924A cell lines were established by neomycin selection. They were studied for hTPO expression using an antibody-based luminescence kit, followed by determination of the enzyme activity in the guaiacol assay and of the iodide uptake capacity in the presence of Na{sup 125}I. Genetically modified cell lines expressed up to 1,800 times more hTPO as compared to wild type tumour cells. The level of hTPO expression varied significantly between individual neomycin-resistant cell lines, suggesting that the recombinant retroviral DNA was integrated at different sites of the cellular genome. The accumulation of iodide, however, was not significantly enhanced in individual recombinant cell lines, irrespective of low or high hTPO expression. Moreover, there was no correlation between hTPO expression and enzyme activity in individual cell lines. The transduction of the hTPO gene per se is not sufficient to restore iodide trapping in non-iodide-concentrating tumour cells. Future studies will have to concentrate on the possible expression of enzymatically active proteins or the transfer of multiple genes involved in iodide trapping. (orig.)

Haberkom, U. [Clinical Cooperation Unit Nuclear Medicine, German Cancer Research Center, Heidelberg (Germany); Dept. of Nuclear Medicine, Univ. of Heidelberg (Germany); Altmann, A.; Jiang, S.; Morr, I.; Mahmut, M. [Clinical Cooperation Unit Nuclear Medicine, German Cancer Research Center, Heidelberg (Germany); Eisenhut, M. [Dept. of Nuclear Medicine, Univ. of Heidelberg (Germany)

2001-05-01

366

Iodide uptake in human anaplastic thyroid carcinoma cells after transfer of the human thyroid peroxidase gene  

International Nuclear Information System (INIS)

Human thyroperoxidase (hTPO) is critical for the accumulation of iodide in thyroid tissues. Poorly differentiated and anaplastic thyroid tumours which lack thyroid-specific gene expression fail to accumulate iodide and, therefore, do not respond to iodine-131 therapy. We consequently investigated whether transfer of the hTPO gene is sufficient to restore the iodide-trapping capacity in undifferentiated thyroid and non-thyroid tumour cells. The human anaplastic thyroid carcinoma cell lines C643 and SW1736, the rat Morris hepatoma cell line MH3924A and the rat papillary thyroid carcinoma cell line L2 were used as in vitro model systems. Employing a bicistronic retroviral vector based on the myeloproliferative sarcoma virus for the transfer of the hTPO and the neomycin resistance gene, the C643 cells and SW1736 cells were transfected while the L2 cells and MH3924A cells were infected with retroviral particles. Seven recombinant C643 and seven SW1736 cell lines as well as four recombinant L2 and four MH3924A cell lines were established by neomycin selection. They were studied for hTPO expression using an antibody-based luminescence kit, followed by determination of the enzyme activity in the guaiacol assay and of the iodide uptake capacity in the presence of Na125I. Genetically modified cell lines expressed up to 1,800 times more hTPO as compared to wild type tumour cells. The level of hTPO expression varied significantly between individual neomycin-resistant cell lines, suggesting that the recombinant retroviral DNA was integrated at different sites of the cellular genome. The accumulation of iodide, however, was not significantly enhanced in individual recombinant cell lines, irrespective of low or high hTPO expression. Moreover, there was no correlation between hTPO expression and enzyme activity in individual cell lines. The transduction of the hTPO gene per se is not sufficient to restore iodide trapping in non-iodide-concentrating tumour cells. Future studies will have to concentrate on the possible expression of enzymatically active proteins or the transfer of multiple genes involved in iodide trapping. (orig.)

2001-01-01

367

Clinical and laboratory characteristics of systemic anaplastic large cell lymphoma in Chinese patients  

Directory of Open Access Journals (Sweden)

Full Text Available Abstract Background Systemic anaplastic large cell lymphoma (S-ALCL) is a rare disease with a highly variable prognosis and no standard chemotherapy regimen. Anaplastic lymphoma kinase (ALK) has been reported as an important prognostic factor correlated with S-ALCL in many but not all studies. In our study, we retrospectively analyzed 92 patients with S-ALCL from the Peking University Lymphoma Center for clinical and molecular prognostic factors to make clear the role of ALK and other prognostic factors in Han Chinese S-ALCL. Results The majority of Chinese S-ALCL patients were young male patients (median age 26, male/female ratio 1.7) and the median age was younger than previous reports regardless of ALK expression status. The only statistically significant different clinical characteristic in S-ALCL between ALK positive (ALK+) and ALK negative (ALK-) was age, with a younger median age of 22 for ALK+ compared with 30 for ALK-. However, when pediatric patients (?18) were excluded, there was no age difference between ALK+ and ALK-. The groups did not differ in the proportion of males, those with clinical stage III/IV (49 vs 51%) or those with extranodal disease (53 vs 59%). Of 73 evaluable patients, the 3-year and 5-year survival rates were 60% and 47%, respectively. Univariate analysis showed that three factors: advanced stage III/IV, lack of expression of ALK, and high Ki-67 expression, were associated with treatment failure in patients with S-ALCL. However, ALK expression correlated with improved survival only in patients younger than 14?years, while not in adult patients. In multivariate analysis, only clinical stage was an independent prognostic factor for survival. Expressions of Wilms tumor 1 (WT1) and B-cell lymphoma 2 protein (BCL-2) correlated with the expression of ALK, but they did not have prognostic significance. High Ki-67 expression was also a poor prognostic factor. Conclusions Our results show that ALK expression alone is not sufficient to determine the outcome of ALCL and other prognostic factors must be considered. Clinical stage is an independent prognostic factor. Ki-67 expression is a promising prognostic factor.

Wang Yan-Fang; Yang Yan-Li; Gao Zi-Fen; Zhou Chun-Ju; Gregg Xylina; Shi Yun-Fei; Wang Jing; Yang Xiao-Feng; Ke Xiao-Yan

2012-01-01

368

Survival and Compliance with the Use of Radiation Therapy for Anaplastic Thyroid Carcinoma  

International Nuclear Information System (INIS)

The purpose of this study was to evaluate the impact of the use of external radiation therapy (ERT) in terms of survival and compliance in patients with anaplastic thyroid carcinoma. Materials and Methods: The medical records of 17 patients with anaplastic thyroid carcinoma treated with ERT between 1993 and 2002 were retrospectively reviewed. ERT was administered after surgery in 14 patients and after a biopsy in three patients. Among the 14 patients who had undergone surgery, nine underwent a curative resection and five underwent a palliative resection. Six patients had associated well-differentiated thyroid carcinomas and 14 patients were diagnosed with a tumor size exceeding 5 cm. The radiation dose ranged from 6-70 Gy (median dose, 37.5 Gy). Eleven patients completed the planned course of ERT, whereas six patients did not. The follow-up period ranged from 1-104 months (median, 5 months; mean, 20 months). Results: Five patients started the ERT without the presence of a gross mass and all of the patients completed ERT without a re-growth of tumor. Twelve patients (four patients after a curative resection, five patients after a palliative resection and three patients after a biopsy) started ERT with a gross mass present and only six patients were able to complete the planned course of ERT. Among the six patients who completed ERT, two patients showed a marked regression of the tumor mass, whereas two patients showed slight regression and two patients showed no response. The median survival was five months (range, 1-104 months) and the mean survival was 21 months. The overall survival was 41% at 1-year, 24% at 2-years and 12% at 5-years. Significant prognostic factors included the number of primary tumors present, tumor size, whether surgery was performed and completion of ERT as planned. Long-term survivors showed a tendency of having smaller sized initial tumors and smaller sized pre-ERT tumors than the short-term survivors. Conclusion: This study suggests that patients with a small initial tumor (?5 cm), which was treated by surgery (curative resection or palliative resection) before ERT, and without rapid re-growth of the mass seen at the surgical site at the beginning of the ERT course, would be the best candidates for postoperative ERT. In contrast, patients with a large initial tumor (>5 cm) and did not undergo surgery before ERT or that rapid re-growth of the mass was observed at the surgical site are likely to have a short survival time, along with the interruption of ERT. In these cases, the role of ERT is very limited and the omission of ERT could be considered.

2008-01-01

369

Survival and Compliance with the Use of Radiation Therapy for Anaplastic Thyroid Carcinoma  

Energy Technology Data Exchange (ETDEWEB)

The purpose of this study was to evaluate the impact of the use of external radiation therapy (ERT) in terms of survival and compliance in patients with anaplastic thyroid carcinoma. Materials and Methods: The medical records of 17 patients with anaplastic thyroid carcinoma treated with ERT between 1993 and 2002 were retrospectively reviewed. ERT was administered after surgery in 14 patients and after a biopsy in three patients. Among the 14 patients who had undergone surgery, nine underwent a curative resection and five underwent a palliative resection. Six patients had associated well-differentiated thyroid carcinomas and 14 patients were diagnosed with a tumor size exceeding 5 cm. The radiation dose ranged from 6-70 Gy (median dose, 37.5 Gy). Eleven patients completed the planned course of ERT, whereas six patients did not. The follow-up period ranged from 1-104 months (median, 5 months; mean, 20 months). Results: Five patients started the ERT without the presence of a gross mass and all of the patients completed ERT without a re-growth of tumor. Twelve patients (four patients after a curative resection, five patients after a palliative resection and three patients after a biopsy) started ERT with a gross mass present and only six patients were able to complete the planned course of ERT. Among the six patients who completed ERT, two patients showed a marked regression of the tumor mass, whereas two patients showed slight regression and two patients showed no response. The median survival was five months (range, 1-104 months) and the mean survival was 21 months. The overall survival was 41% at 1-year, 24% at 2-years and 12% at 5-years. Significant prognostic factors included the number of primary tumors present, tumor size, whether surgery was performed and completion of ERT as planned. Long-term survivors showed a tendency of having smaller sized initial tumors and smaller sized pre-ERT tumors than the short-term survivors. Conclusion: This study suggests that patients with a small initial tumor ({<=}5 cm), which was treated by surgery (curative resection or palliative resection) before ERT, and without rapid re-growth of the mass seen at the surgical site at the beginning of the ERT course, would be the best candidates for postoperative ERT. In contrast, patients with a large initial tumor (>5 cm) and did not undergo surgery before ERT or that rapid re-growth of the mass was observed at the surgical site are likely to have a short survival time, along with the interruption of ERT. In these cases, the role of ERT is very limited and the omission of ERT could be considered.

Oh, Yoon Kyeong; Jang, Ji Young [Chosun University College of Medicine, Seoul (Korea, Republic of); Chung, Woong Ki [Chonnam National University College of Medicine, Gwangju (Korea, Republic of)

2008-12-15

370

Infiltrative corneal lesions resembling fibrous histiocytoma: clinical and pathologic findings in six dogs and one cat.  

UK PubMed Central (United Kingdom)

Infiltrating corneal lesions developed in 6 dogs and 1 cat. In each case, the site of origin appeared to be the corneal limbus. The lesions were characterized by continuous growth, a benign appearance, and a tendency to recur following excision keratoplasty. Each lesion was of a proliferative, inflammatory nature, histologically resembling fibrous histiocytoma. Of the 6 dogs in the series, 4 were Collies.

Smith JS; Bistner S; Riis R

1976-10-01

371

Human RECQ5? helicase promotes strand exchange on synthetic DNA structures resembling a stalled replication fork  

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The role of the human RECQ5? helicase in the maintenance of genomic stability remains elusive. Here we show that RECQ5? promotes strand exchange between arms of synthetic forked DNA structures resembling a stalled replication fork in a reaction dependent on ATP hydrolysis. BLM and WRN can also promo...

Kanagaraj, Radhakrishnan; Saydam, Nurten; Garcia, Patrick L.; Zheng, Lu; Janscak, Pavel

372

Canine sterile neutrophilic dermatitis (resembling Sweet’s syndrome) in a Dachshund  

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A 6-year-old Dachshund was presented with a 2-day history of lethargy, anorexia and cutaneous erythema, edema, and multifocal erythematous papules affecting the ventral abdomen, axillae, and groin. Microscopic examination revealed a sterile neutrophilic dermatitis resembling Sweet’s syndrome; howeve...

Gains, Malcolm J.; Morency, Andréanne; Sauvé, Frédéric; Blais, Marie-Claude; Bongrand, Yannick

373

AEROBIC AND ANAEROBIC BACTERIAL COUNTS OF NASAL WASHINGS: PRESENCE OF ORGANISMS RESEMBLING CORYNEBACTERIUM ACNES  

Digital Repository Infrastructure Vision for European Research (DRIVER)

Watson, Elinor D. (Washington University, St. Louis, Mo.), Nanci J. Hoffman, Richard W. Simmers, and Theodor Rosebury. Aerobic and anaerobic bacterial counts of nasal washings: Presence of organisms resembling Corynebacterium acnes. J. Bacteriol. 83:144–148. 1962.—Aerobic and anaerobic colony counts...

Watson, Elinor D.; Hoffman, Nanci J.; Simmers, Richard W.; Rosebury, Theodor

374

Langerhans cells in anaplastic Kaposi sarcoma with a paucivascular phenotype: a potential diagnostic pitfall.  

UK PubMed Central (United Kingdom)

Anaplastic Kaposi sarcoma (AKS), a rare variant of Kaposi sarcoma, has a poorly recognized histomorphologic spectrum, including a paucivascular phenotype, that mimics a range of undifferentiated malignancies. This study, that highlights the hitherto undocumented phenomenon of S100-protein-positive Langerhans cells (SLCs) as a potential diagnostic pitfall in paucivascular AKS, involved review of nine such AKS that required diagnostic immunohistochemical (IHC) work-up. All biopsies had a predominant or exclusive spindle or epithelioid cell infiltrate. The first three tumors were diagnosed as malignant peripheral nerve sheath tumor (2) and metastatic melanoma (1), based on S100-protein immunopositivity. Biopsy of a co-existent pigmented sole lesion (patient 3) demonstrated nodular KS. Subsequent IHC investigation of these three tumors demonstrated an endothelial phenotype and HHV8 immunopositivity, confirming AKS. CD1a and langerin staining of the S100-protein-positive cells confirmed Langerhans cells as the cause of the diagnostic pitfall. Subsequently, six further paucivascular AKS with intratumoral SLCs were recognized on histomorphological and IHC appraisal. In conclusion, heightened awareness of the histomorphologic spectrum, appropriate IHC investigation, and informed appraisal thereof, are critical to the diagnosis of AKS with an undifferentiated phenotype, and the avoidance of IHC pitfalls, such as those caused by under-recognition and misinterpretation of bystander SLCs in AKS.

Ramdial PK; Sing Y; Naicker S; Calonje E; Sewram V; Singh B

2011-04-01

375

Langerhans cells in anaplastic Kaposi sarcoma with a paucivascular phenotype: a potential diagnostic pitfall.  

Science.gov (United States)

Anaplastic Kaposi sarcoma (AKS), a rare variant of Kaposi sarcoma, has a poorly recognized histomorphologic spectrum, including a paucivascular phenotype, that mimics a range of undifferentiated malignancies. This study, that highlights the hitherto undocumented phenomenon of S100-protein-positive Langerhans cells (SLCs) as a potential diagnostic pitfall in paucivascular AKS, involved review of nine such AKS that required diagnostic immunohistochemical (IHC) work-up. All biopsies had a predominant or exclusive spindle or epithelioid cell infiltrate. The first three tumors were diagnosed as malignant peripheral nerve sheath tumor (2) and metastatic melanoma (1), based on S100-protein immunopositivity. Biopsy of a co-existent pigmented sole lesion (patient 3) demonstrated nodular KS. Subsequent IHC investigation of these three tumors demonstrated an endothelial phenotype and HHV8 immunopositivity, confirming AKS. CD1a and langerin staining of the S100-protein-positive cells confirmed Langerhans cells as the cause of the diagnostic pitfall. Subsequently, six further paucivascular AKS with intratumoral SLCs were recognized on histomorphological and IHC appraisal. In conclusion, heightened awareness of the histomorphologic spectrum, appropriate IHC investigation, and informed appraisal thereof, are critical to the diagnosis of AKS with an undifferentiated phenotype, and the avoidance of IHC pitfalls, such as those caused by under-recognition and misinterpretation of bystander SLCs in AKS. PMID:21418394

Ramdial, Pratistadevi K; Sing, Yetish; Naicker, Shaun; Calonje, Eduardo; Sewram, Vikash; Singh, Bhugwan

2011-03-03

376

Effectiveness of interferon-beta and temozolomide combination therapy against temozolomide-refractory recurrent anaplastic astrocytoma  

Directory of Open Access Journals (Sweden)

Full Text Available Abstract Background Malignant gliomas recur even after extensive surgery and chemo-radiotherapy. Although a relatively novel chemotherapeutic agent, temozolomide (TMZ), has demonstrated promising activity against recurrent glioma, the effects last only a few months and drug resistance develops thereafter in most cases. Induction of O6-methylguanine-DNA methyltransferase (MGMT) in tumors is considered to be responsible for resistance to TMZ. Interferon-beta has been reported to suppress MGMT in an experimental glioma model. Here we report a patient with TMZ-refractory anaplastic astrocytoma (AA) who was treated successfully with a combination of interferon-beta and TMZ. Case presentation A patient with recurrent AA after radiation-chemotherapy and stereotactic radiotherapy was treated with TMZ. After 6 cycles, the tumor became refractory to TMZ, and the patient was treated with interferon-beta at 3 × 106 international units/body, followed by 5 consecutive days of 200 mg/m2 TMZ in cycles of 28 days. After the second cycle the tumor decreased in size by 50% (PR). The tumor showed further shrinkage after 8 months and the patient's KPS improved from 70% to 100%. The immunohistochemical study of the initial tumor specimen confirmed positive MGMT protein expression. Conclusion It is considered that interferon-beta pre-administration increased the TMZ sensitivity of the glioma, which had been refractory to TMZ monotherapy.

Fujimaki Takamitsu; Ishii Hisato; Matsuno Akira; Arai Hajime; Nakagomi Tadayoshi

2007-01-01

377

Anaplastic lymphoma kinase: "Ligand Independent Activation" mediated by the PTN/RPTP?/? signaling pathway.  

UK PubMed Central (United Kingdom)

Anaplastic lymphoma kinase is essential in early development, differentiation, and maintenance of cell survival; nevertheless, the mechanism to activate ALK has remained elusive. ALK has remained an "Orphan Receptor." The studies cited below describe a unique mechanism termed "Ligand Independent Activation." It is shown that activation of ALK results when the cytokine pleiotrophin (PTN) interacts with its receptor, the receptor protein tyrosine phosphatase ?/? (RPTP?/?). Pleiotrophin inactivates the catalytic activity of RPTP?/?, which, when not inactivated, dephosphorylates phosphotyrosine sites in the activation domain of ALK; as a consequence of the inactivation of RPTP?/? by PTN, autophosphorylation and autoactivation of ALK rapidly follow. The PTN/RPTP?/? signaling pathway thus regulates the catalytic activity of ALK and tyrosine phosphorylation levels of ALK downstream target proteins. Furthermore, since ALK is only one of the key ALK phosphoproteins targeted by the PTN/RPTP?/? signaling pathway, the PTN/RPTP?/? signaling pathway has the potential to coordinately regulate tyrosine phosphorylation of other different key proteins in multiple cellular compartments. This article is part of a Special Issue entitled: Emerging recognition and activation mechanisms of receptor tyrosine kinases.

Deuel TF

2013-10-01

378

Anaplastic lymphoma kinase is required for neurogenesis in the developing central nervous system of zebrafish.  

UK PubMed Central (United Kingdom)

Anaplastic Lymphoma Kinase (ALK) was initially discovered as an oncogene in human lymphoma and other cancers, including neuroblastoma. However, little is known about the physiological function of ALK. We identified the alk ortholog in zebrafish (Danio rerio) and found that it is highly expressed in the developing central nervous system (CNS). Heat-shock inducible transgenic zebrafish lines were generated to over-express alk during early neurogenesis. Its ectopic expression resulted in activation of the MEK/ERK pathway, increased cell proliferation, and aberrant neurogenesis leading to mis-positioning of differentiated neurons. Thus, overexpressed alk is capable of promoting cell proliferation in the nervous system, similar to the situation in ALK-related cancers. Next, we used Morpholino mediated gene knock-down and a pharmacological inhibitor to interfere with expression and function of endogenous Alk. Alk inhibition did not affect neuron progenitor formation but severely compromised neuronal differentiation and neuron survival in the CNS. These data indicate that tightly controlled alk expression is critical for the balance between neural progenitor proliferation, differentiation and survival during embryonic neurogenesis.

Yao S; Cheng M; Zhang Q; Wasik M; Kelsh R; Winkler C

2013-01-01