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1. Terrier-Lacombe MJ, Guillou L, Chibon F, Gallagher G, Benhattar J, Terrier P, Ranchère D, Coindre JM: Superficial primitive Ewing's sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases. Mod Pathol; 2009 Jan;22(1):87-94
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Superficial primitive Ewing's sarcoma: a clinicopathologic and molecular cytogenetic analysis of 14 cases.
  • In the skin and subcutis, the diagnosis is often difficult, and performing molecular cytogenetic techniques may be helpful.
  • Clinical, histological, immunohistochemical, molecular cytogenetic, therapeutic, and follow-up data are reported.
  • They were all small round-cell proliferations with a strong membranous positivity for CD99.
  • Ewing's sarcoma translocations/fusion gene transcripts were detected in eight cases, both by FISH and reverse transcriptase (RT)-PCR.
  • Chemotherapy was given in ten patients and radiotherapy in six.
  • Given the difficulty of the diagnosis and the importance of an adapted treatment, a confirmation of the diagnosis by molecular or cytogenetic techniques is recommended when dealing with a superficial tumor.
  • [MeSH-major] Sarcoma, Ewing / genetics. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Antigens, CD / biosynthesis. Calmodulin-Binding Proteins / genetics. Cell Adhesion Molecules / biosynthesis. Child. Cytogenetic Analysis. Female. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Middle Aged. Oncogene Proteins, Fusion / genetics. RNA-Binding Proteins / genetics. Retrospective Studies. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 18820660.001).
  • [ISSN] 1530-0285
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Calmodulin-Binding Proteins; 0 / Cell Adhesion Molecules; 0 / EWSR1 protein, human; 0 / Oncogene Proteins, Fusion; 0 / RNA-Binding Proteins
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2. Belgaumi AF, Al-Kofide A, Sabbah R, Shalaby L: Precursor B-cell lymphoblastic lymphoma (PBLL) in children: pattern of presentation and outcome. J Egypt Natl Canc Inst; 2005 Mar;17(1):15-9
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  • [Title] Precursor B-cell lymphoblastic lymphoma (PBLL) in children: pattern of presentation and outcome.
  • PURPOSE AND BACKGROUND: Precursor B-cell lymphoblastic lymphoma (PBLL) is a rare subtype of NHL seen primarily in children or young adults.
  • There are approximately 100 immunophenotyped cases of PBLL; reported in the literature; most as single case reports or very small series.
  • There were three boys and the median age at diagnosis was 6 years (range 3-13).
  • One patient had a soft tissue mass in the upper thigh while one patient had a solitary bone lesion in the distal tibia.
  • Five patients were treated according to B-cell NHL type protocols.
  • Because of the specific diagnosis of PBLL, two of these patients were switched to an ALL-type protocol following post induction intensification; one died in remission due to encephalitis, while the other remained in CR almost 2 years after diagnosis.
  • A third patient suffered a loco-regional relapse 17 months after completing first line therapy, and was re-treated on an ALL-type protocol, and currently is in remission 25 months following relapse.
  • The fourth patient, who received 9 months of post induction therapy, remains free of disease 7 years following diagnosis.
  • The fifth patient had local and CNS progression on therapy, and died of his disease.
  • The last patient with a solitary bone lesion was misdiagnosed as Ewings' Sarcoma and received treatment for that disease.
  • He suffered an isolated CNS relapse, and is in CR 12 months following the relapse, on an ALL treatment protocol.
  • CONCLUSION: PBLL is a distinct B-cell NHL which involves extralymphatic sites, with particular predisposition for the upper aerodigestive tract.
  • Patients should not be treated on short intensive protocols used for other B-cell NHL but should receive treatment based on ALL protocols like those for treating T-cell LL.
  • [MeSH-major] B-Lymphocytes / pathology. Precursor Cell Lymphoblastic Leukemia-Lymphoma / drug therapy. Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Male. Neoplasm Staging. Retrospective Studies. Treatment Outcome

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  • (PMID = 16353078.001).
  • [ISSN] 1110-0362
  • [Journal-full-title] Journal of the Egyptian National Cancer Institute
  • [ISO-abbreviation] J Egypt Natl Canc Inst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
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3. Mysorekar VV, Harish K, Kilara N, Subramanian M, Giridhar AG: Embryonal rhabdomyosarcoma of the chest wall: a case report and review of the literature. Indian J Pathol Microbiol; 2008 Apr-Jun;51(2):274-6
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  • Embryonal rhabdomyosarcoma is a soft-tissue sarcoma which has a predilection for the head and neck area, genitourinary tract and the extremities.
  • We report a rare case of embryonal rhabdomyosarcoma of the chest wall in an 8-year-old girl, presenting as a destructive tumor in the rib and clinically and radiologically mimicking Ewing's sarcoma.
  • Histopathological examination showed a small round cell tumor.
  • Immunohistochemically, the positivity for muscle markers desmin and myogenin in the tumor cells proved to be useful for making a definitive diagnosis of embryonal rhabdomyosarcoma.
  • The patient is responding well to chemotherapy.
  • [MeSH-major] Rhabdomyosarcoma, Embryonal / diagnosis. Thoracic Neoplasms / diagnosis. Thoracic Wall / pathology
  • [MeSH-minor] Aneuploidy. Child. Diagnosis, Differential. Female. Humans. Sarcoma, Ewing / diagnosis

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  • (PMID = 18603708.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] India
  • [Number-of-references] 9
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4. Cho SH, Rhim SC, Hyun SJ, Bae CW, Khang SK: Intradural involvement of multicentric myxoid liposarcoma. J Korean Neurosurg Soc; 2010 Sep;48(3):276-80
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  • Liposarcomas are malignant tumors of the soft tissue, with myxoid liposarcoma being the second most common subtype, tending to occur in the limbs, particularly in the thighs.
  • She was histologically diagnosed with small round cell myxoid sarcoma and underwent adjuvant chemotherapy.
  • However, the patient died of multiple metastases 18 months after the first diagnosis.

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  • (PMID = 21082059.001).
  • [ISSN] 1598-7876
  • [Journal-full-title] Journal of Korean Neurosurgical Society
  • [ISO-abbreviation] J Korean Neurosurg Soc
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2966733
  • [Keywords] NOTNLM ; Cervical spine / Metastasis / Multicentric / Myxoid liposarcoma / lntradural
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5. Hasegawa T, Yamamoto S, Nojima T, Hirose T, Nikaido T, Yamashiro K, Matsuno Y: Validity and reproducibility of histologic diagnosis and grading for adult soft-tissue sarcomas. Hum Pathol; 2002 Jan;33(1):111-5
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  • [Title] Validity and reproducibility of histologic diagnosis and grading for adult soft-tissue sarcomas.
  • Soft-tissue sarcomas in adults show great variations in histologic type and grade.
  • A valid and reproducible prognostication system is needed to select patients with soft-tissue sarcomas who could benefit from adjuvant chemotherapy.
  • This study was conducted to assess the validity and reproducibility of diagnosis of the histologic type, MIB-1 grade, and mitosis grade, as well as of the 3 components of these grading systems.
  • MIB-1 grade is a recently proposed grading system for predicting the prognosis of patients with adult soft-tissue sarcomas on the basis of 3 criteria (tumor differentiation, necrosis, and MIB-1 score) and replaces the mitotic count in the French system with MIB-1 immunohistochemical staining.
  • Four surgical pathologists from 4 institutions who had experience in diagnostic soft-tissue tumor pathology reviewed 130 cases of soft-tissue sarcoma and independently determined histologic type and grade.
  • The validity of histologic diagnosis was measured by sensitivity and specificity, and that of grading was measured by kappa statistics and percentage agreement with the diagnosis of the expert panel at the National Cancer Center, which was defined as a gold standard.
  • The validity of the diagnosis of histologic type was high for synovial sarcoma, small round-cell sarcoma, and liposarcoma (sensitivity 89% to 100%; specificity, 98% to 100%) but low for malignant fibrous histiocytoma (MFH) and spindle-cell sarcoma (73% to 75%; 93% to 95%).
  • Interobserver reproducibility of histologic diagnosis was high for small round-cell sarcoma, synovial sarcoma, and liposarcoma (kappa = 0.92, 0.90, 0.87; percentage agreement = 99%, 97%, 96%, respectively); for grading, reproducibility was highest for tumor differentiation (0.78; 87%) and second highest for MIB-1 grade (0.68; 79%).
  • We conclude that diagnosis of the type of soft-tissue sarcoma for synovial sarcoma, small round-cell sarcoma, and liposarcoma and the MIB-1 grading system based on tumor differentiation are highly valid and reproducible among Japanese pathologists who are familiar with the grading system, whereas re-evaluation of histologic criteria is essential for other histologic types such as MFH and spindle-cell sarcoma.
  • [MeSH-major] Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • [Copyright] Copyright 2002 by W.B. Saunders Company
  • (PMID = 11823981.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Validation Studies
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Nuclear; 0 / Ki-67 Antigen; 0 / Nuclear Proteins
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6. Ohgaki K, Horiuchi K, Mizutani S, Sato M, Kondo Y: Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin. Int J Clin Oncol; 2010 Apr;15(2):210-4
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  • [Title] Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin.
  • A primitive neuroectodermal tumor (PNET) is a small round cell tumor that arises from the nerve crest.
  • This tumor usually occurs in the central nervous system or soft tissue, but it can occur in the kidney in rare cases.
  • Some of the cells formed a rosette structure and the tumor cells were positive for CD99, leading to diagnosis of PNET.
  • Severe multiple liver metastases occurred 6 months after surgery, and six courses of chemotherapy with ifosfamide, etoposide and doxorubicin were performed.
  • After this treatment, residual tumor was removed, but the tumor cells were absent histologically.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Kidney Neoplasms / surgery. Liver Neoplasms / drug therapy. Nephrectomy. Neuroectodermal Tumors, Primitive / drug therapy. Sarcoma, Ewing / drug therapy
  • [MeSH-minor] Biopsy. Doxorubicin / administration & dosage. Etoposide / administration & dosage. Humans. Ifosfamide / administration & dosage. Male. Positron-Emission Tomography. Tomography, X-Ray Computed. Treatment Outcome. Young Adult

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  • (PMID = 20186557.001).
  • [ISSN] 1437-7772
  • [Journal-full-title] International journal of clinical oncology
  • [ISO-abbreviation] Int. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Japan
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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7. Hamazaki M, Okita H, Hata J, Shimizu S, Kobayashi H, Aoki K, Nara T: Desmoplastic small cell tumor of soft tissue: molecular variant of EWS-WT1 chimeric fusion. Pathol Int; 2006 Sep;56(9):543-8
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  • [Title] Desmoplastic small cell tumor of soft tissue: molecular variant of EWS-WT1 chimeric fusion.
  • The soft-part tumor recurred in the parotid gland region 4 months later, and a second recurrence was noted on the left side of the neck 3 years and 3 months thereafter.
  • The patient had not received chemotherapy or local irradiation.
  • Histological and immunohistochemical examinations of the recurrent masses revealed morphological characteristics of small cell proliferation with desmoplastic stroma that were similar to those of the initial tumor.
  • These findings suggested a diagnosis of desmoplastic small cell tumor, despite its extra-abdominal location.
  • The histological diagnosis was confirmed by reverse transcriptase polymerase chain reaction, which demonstrated an EWS-WT1 chimeric fusion gene.
  • The chimeric fusion gene might be related to the tissue-specific phenotype of desmoplastic small cell tumors, although further investigation of this speculation is necessary.
  • [MeSH-major] Desmin / metabolism. Head and Neck Neoplasms / genetics. Head and Neck Neoplasms / pathology. Oncogene Proteins, Fusion / genetics. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Child. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Lymphoma, Non-Hodgkin / pathology. Microscopy, Electron, Transmission. Mucin-1 / metabolism. Neoplasm Recurrence, Local / pathology. Reverse Transcriptase Polymerase Chain Reaction. Rhabdomyosarcoma / pathology. Sarcoma, Ewing / pathology. Vimentin / metabolism

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  • (PMID = 16930335.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Desmin; 0 / EWS1-WT1 fusion protein, human; 0 / Mucin-1; 0 / Oncogene Proteins, Fusion; 0 / Vimentin
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8. Furuno Y, Nishimura S, Kamiyama H, Numagami Y, Saito A, Kaimori M, Nishijima M: Intracranial peripheral-type primitive neuroectodermal tumor. Neurol Med Chir (Tokyo); 2008 Feb;48(2):72-6
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  • [Title] Intracranial peripheral-type primitive neuroectodermal tumor.
  • His headache worsened and computed tomography revealed enlargement of the tumor and intracystic hemorrhage, so emergent operation was performed.
  • Histological examination showed solid growth of small round cells with uniform round nuclei and minimal cytoplasm.
  • The histological diagnosis was peripheral-type primitive neuroectodermal tumor (pPNET).
  • Following surgery, radiation therapy and chemotherapy were given.
  • Ewing's sarcoma and pPNET form a family of small round cell tumors arising in the bone or soft tissue.
  • MIC-2 is a useful marker in the differential diagnosis.
  • [MeSH-minor] Adolescent. Antigens, CD / metabolism. Biomarkers, Tumor / metabolism. Cell Adhesion Molecules / metabolism. Diagnosis, Differential. Frontal Lobe / pathology. Headache / etiology. Headache / pathology. Humans. Ki-67 Antigen / metabolism. Magnetic Resonance Imaging. Male. Temporal Lobe / pathology. Tomography, X-Ray Computed

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  • (PMID = 18296876.001).
  • [ISSN] 0470-8105
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Ki-67 Antigen
  • [Number-of-references] 20
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9. Andrés AM, Avila LF, Luis AL, Encinas JL, Sastre A, López-Gutiérrez JC, Martínez L, Queizán A, Martínez-Urrutia MJ, Jaureguizar E, Tovar JA: [Soft tissue sarcomas (1991-2004)]. Cir Pediatr; 2006 Oct;19(4):210-6
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  • [Title] [Soft tissue sarcomas (1991-2004)].
  • BACKGROUND: The aim of this study is to review the results of the treatment of soft tissue sarcomas (STS) in our Department during the last 13 years.
  • MATERIAL AND METHODS: Fifty-seven children (39 rhabdomyosarcomas (RMS) and 18 other types of sarcomas) have been treated.
  • The charts of 39 chidren were analysed evaluating several parameters (age, sex, location, histology, initial stage, clinical and surgical treatment and results) as prognostic factors using actuarial survival analyses and log-rank tests.
  • RESULTS: 1. RMS: Median age at diagnosis was 2.3 years (range 6 m-16y).
  • At diagnosis, 74% were in stages I or II.
  • Surgical biopsy was made before the definitive surgery in 12 cases.
  • In the remaining 8 children the diagnosis was made only after surgical resection.
  • 2. Other sarcomas: Median age at diagnosis was 10.9 years (range 4 days-15 years).
  • Among this group, there were 6 fibrosarcomas, 4 indifferentiated sarcomas, 3 synovial sarcomas, 2 abdominal desmoplastic small round cell tumours, 2 neurofibrosarcomas and 1 leiomyosarcoma.
  • Only 9 received chemotherapy and one radiotherapy.
  • CONCLUSIONS: Although the role of surgery is crucial, it is necessary to refine the initial histological diagnosis, because neither the PAAF or the biopsy have always been correct.
  • The negative prognostic factors in our series were metastases present at diagnosis, genitourinary location and alveolar (RMS), desmoplastic or indifferenciated histology.
  • [MeSH-major] Sarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology

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  • (PMID = 17352109.001).
  • [ISSN] 0214-1221
  • [Journal-full-title] Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica
  • [ISO-abbreviation] Cir Pediatr
  • [Language] spa
  • [Publication-type] Comparative Study; English Abstract; Journal Article
  • [Publication-country] Spain
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10. Ruymann FB, Grovas AC: Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas. Cancer Invest; 2000;18(3):223-41
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  • [Title] Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas.
  • Advances in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas continue in the Intergroup Rhabdomyosarcoma Study Group (IRSG) and European cooperative groups.
  • The use of molecular biology techniques in soft tissue sarcomas are redefining the classic pathology of these small blue cell tumors.
  • These advances confound the interpretation of consecutively run chemotherapy trials using historical comparisons.
  • The IRSG has reported improvement in the prognosis of both nonmetastatic and metastatic embryonal rhabdomyosarcoma as attributable to three, three-drug regimens that use cyclophosphamide at 2.2 g/m2 in either maintenance or induction and maintenance therapy.
  • The doublet of ifosfamide and etoposide in combination with vincristine, actinomycin D, and cyclophosphamide at 2.2 g/m2 achieved a remarkable 3-year survival of 58% in patients with metastatic rhabdomyosarcoma and undifferentiated soft tissue sarcoma.
  • Molecular studies in IRSG-V will be applied in the detection of occult bone marrow metastases and the evaluation of resection margins at initial and second-look surgery.
  • Long-term follow-up will be required in patients with gross residual sarcoma randomized to conventional and hyperfractionated radiotherapy in IRSG-IV to assess late effects.
  • As molecular discoveries advance the diagnosis and detection of rhabdomyosarcoma, it is hoped that the futuristic molecular based treatment strategies in development and early testing will further improve survival in high-risk patients with metastatic soft tissue sarcoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Sarcoma / diagnosis. Sarcoma / therapy. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / therapy

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  • (PMID = 10754991.001).
  • [ISSN] 0735-7907
  • [Journal-full-title] Cancer investigation
  • [ISO-abbreviation] Cancer Invest.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P30 CA16058; United States / NCI NIH HHS / CA / U10 CA03750-39; United States / NCI NIH HHS / CA / U10 CA24507-17
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 121
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11. Yang JY, Kim JM: Small cell extraskeletal osteosarcoma. Orthopedics; 2009 Mar;32(3):217
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  • [Title] Small cell extraskeletal osteosarcoma.
  • Extraskeletal osteosarcoma is a rare malignant mesenchymal neoplasm that accounts for <4% of all osteosarcomas and approximately 1.2% of all soft tissue sarcomas.
  • Among the extraskeletal osteosarcomas, the small cell type is extremely rare.
  • This article describes a 31-year-old man who had small cell extraskeletal osteosarcoma arising from the semimembranosus muscle.
  • An incisional biopsy was performed and the histopathological findings showed many small cells and osteoid formation.
  • The results were reported as a malignant small round cell tumor, consistent with an extraskeletal Ewing's sarcoma or primitive neuroectodermal tumor.
  • The patient refused neoadjuvant chemotherapy.
  • The final diagnosis was small cell extraskeletal osteosarcoma.
  • Adjuvant chemotherapy was performed using doxorubicin, ifosfamide, and cisplatin together with a total of 60 Gy of radiation therapy.
  • We performed chest computed tomography, magnetic resonance imaging, and positron emission tomography-computed tomography.
  • [MeSH-major] Muscle Neoplasms / pathology. Osteosarcoma / pathology. Sarcoma, Small Cell / pathology
  • [MeSH-minor] Adult. Antigens, CD / analysis. Biomarkers, Tumor / analysis. Cell Adhesion Molecules / analysis. Combined Modality Therapy. Disease-Free Survival. Humans. Male. Muscle, Skeletal / pathology

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  • (PMID = 19309043.001).
  • [ISSN] 1938-2367
  • [Journal-full-title] Orthopedics
  • [ISO-abbreviation] Orthopedics
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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12. Church DN, Bailey J, Hughes J, Williams CJ: Desmoplastic small round cell tumour: obstetric and gynecological presentations. Gynecol Oncol; 2006 Sep;102(3):583-6
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  • [Title] Desmoplastic small round cell tumour: obstetric and gynecological presentations.
  • BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a rare sarcoma primarily affecting young men.
  • Despite chemotherapy, the patient relapsed and died 27 months after diagnosis.
  • Although attaining a complete response to chemotherapy, she relapsed within 2 months and died 11 months after diagnosis.
  • CONCLUSION: DSRCT should be considered in the differential diagnosis of young women presenting with abdominal distension and multiple masses on imaging.
  • [MeSH-major] Abdominal Neoplasms / diagnosis. Pelvic Neoplasms / diagnosis. Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adult. Fatal Outcome. Female. Humans. Pregnancy. Pregnancy Complications, Neoplastic / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 16643996.001).
  • [ISSN] 0090-8258
  • [Journal-full-title] Gynecologic oncology
  • [ISO-abbreviation] Gynecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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13. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP: Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg; 2005 Jan;40(1):251-5
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  • [Title] Results of multimodal treatment for desmoplastic small round cell tumors.
  • PURPOSE: Desmoplastic small round cell tumors (DSRCTs) are rare aggressive neoplasms that frequently present with large symptomatic intraabdominal masses.
  • We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT.
  • Data were collected on patient demographics, presenting symptoms, tumor location and extent, treatment regimen, and overall survival.
  • RESULTS: A majority of patients were male (91%), Caucasian (85%), and with a median age of 19 (7-58) years old at diagnosis.
  • Thirty-three (50%) had positive lymph nodes and 27 (41%) had distant parenchymal metastases at diagnosis.
  • Twenty-nine of these patients (44%) underwent induction chemotherapy (P6), surgical debulking, and radiotherapy.
  • Three-year survival was 55% in those receiving chemotherapy, surgery, and radiotherapy vs 27% when all 3 modalities were not used (P < .02).
  • CONCLUSIONS: Multimodal therapy results in improved survival in patients with DSRCT.
  • [MeSH-major] Sarcoma, Small Cell / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Abdominal Neoplasms / pathology. Abdominal Neoplasms / surgery. Abdominal Neoplasms / therapy. Adolescent. Adult. Antineoplastic Agents / therapeutic use. Child. Combined Modality Therapy. Female. Humans. Lymphatic Metastasis. Male. Middle Aged. Neoplasm Metastasis. Pelvic Neoplasms / pathology. Pelvic Neoplasms / surgery. Pelvic Neoplasms / therapy. Radiotherapy. Surgical Procedures, Operative. Survival Analysis. Testicular Neoplasms / pathology. Testicular Neoplasms / surgery. Testicular Neoplasms / therapy. Thoracic Neoplasms / pathology. Thoracic Neoplasms / surgery. Thoracic Neoplasms / therapy. Treatment Outcome

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  • (PMID = 15868593.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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14. Bisogno G, Roganovich J, Sotti G, Ninfo V, di Montezemolo LC, Donfrancesco A, Mascarin M, Carli M: Desmoplastic small round cell tumour in children and adolescents. Med Pediatr Oncol; 2000 May;34(5):338-42
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  • [Title] Desmoplastic small round cell tumour in children and adolescents.
  • BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a rare highly aggressive neoplasm, and clinical studies are scarce.
  • PROCEDURE: We report six cases of children and adolescents (median age 14 years, range 6.9-17.5) with DSRCT (5 abdominal, 1 paratesticular) registered by the Italian Cooperative Group (ICG) for soft tissue sarcoma over a 9-year period.
  • Patients received a multidisciplinary treatment, including aggressive initial or delayed surgery and radiotherapy.
  • Chemotherapy regimen was based on the use of ifosfamide, vincristine, dactinomycin, and a few doses of antharacyclines (doxorubicin or epirubicin).
  • All patients received multidrug chemotherapy, and tumour reduction was obtained in the 4 evaluable patients.
  • Two patients remained progression-free 22 and 63 months after diagnosis, one is in third complete remission, whereas three died 10-25 months after diagnosis.
  • CONCLUSIONS: DSRCT is a chemosensitive tumour, but survival rates remain disappointing despite aggressive multimodality therapy.
  • [MeSH-major] Sarcoma, Small Cell / surgery
  • [MeSH-minor] Abdominal Neoplasms / drug therapy. Abdominal Neoplasms / radiotherapy. Abdominal Neoplasms / surgery. Adolescent. Antibiotics, Antineoplastic / administration & dosage. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Phytogenic / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Combined Modality Therapy. Dactinomycin / administration & dosage. Disease-Free Survival. Humans. Ifosfamide / administration & dosage. Male. Neoplasm Recurrence, Local. Prognosis. Remission Induction. Salvage Therapy. Survival Rate. Testicular Neoplasms / drug therapy. Testicular Neoplasms / radiotherapy. Testicular Neoplasms / surgery. Vincristine / administration & dosage

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  • [Copyright] Copyright 2000 Wiley-Liss, Inc.
  • (PMID = 10797355.001).
  • [ISSN] 0098-1532
  • [Journal-full-title] Medical and pediatric oncology
  • [ISO-abbreviation] Med. Pediatr. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Antibiotics, Antineoplastic; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; UM20QQM95Y / Ifosfamide
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15. Oya N, Aoki J, Shinozaki T, Watanabe H, Takagishi K, Endo K: Preliminary study of proton magnetic resonance spectroscopy in bone and soft tissue tumors: an unassigned signal at 2.0-2.1 ppm may be a possible indicator of malignant neuroectodermal tumor. Radiat Med; 2000 May-Jun;18(3):193-8
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  • [Title] Preliminary study of proton magnetic resonance spectroscopy in bone and soft tissue tumors: an unassigned signal at 2.0-2.1 ppm may be a possible indicator of malignant neuroectodermal tumor.
  • PURPOSE: To evaluate the utility of proton magnetic resonance spectroscopy in bone and soft tissue tumors, especially whether or not the N-acetyl aspartate signal (NAA) could be recognized in neurogenic tumors.
  • MATERIALS AND METHODS: Forty-nine proton magnetic resonance spectroscopy studies were performed in 60 bone and soft tissue tumors.
  • RESULTS: An unassigned signal at about 2.0-2.1 ppm was recognized in six of 47 lesions: clear cell sarcoma (2/2), Ewing sarcoma (1/1), malignant fibrous histiocytoma (1/3), malignant schwannoma (1/1), and mucoepidermoid carcinoma (1/1).
  • Neuroblastoma (1/1), primitive neuroectodermal tumor (1/1), and malignant melanoma (1/1) after chemotherapy or radiotherapy did not show this signal.
  • CONCLUSIONS: The assigned signal at about 2.0-2.1 ppm was detected in a small percentage of bone and soft tissue tumors and could be suggestive of an untreated malignant tumor of neuroectodermal origin.
  • [MeSH-major] Aspartic Acid / analogs & derivatives. Bone Neoplasms / diagnosis. Magnetic Resonance Spectroscopy. Neuroectodermal Tumors / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Child. Child, Preschool. Diagnosis, Differential. Female. Humans. Infant. Male. Middle Aged

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  • (PMID = 10972550.001).
  • [ISSN] 0288-2043
  • [Journal-full-title] Radiation medicine
  • [ISO-abbreviation] Radiat Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] JAPAN
  • [Chemical-registry-number] 30KYC7MIAI / Aspartic Acid; 997-55-7 / N-acetylaspartate
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16. Hornick JL, Jaffe ES, Fletcher CD: Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol; 2004 Sep;28(9):1133-44
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  • [Title] Extranodal histiocytic sarcoma: clinicopathologic analysis of 14 cases of a rare epithelioid malignancy.
  • Histiocytic sarcoma is a rare malignant neoplasm that occurs in lymph nodes, skin, and the gastrointestinal tract.
  • Only small numbers of bona fide examples exist in the world literature; cases arising primarily at extranodal sites are not well described and often seem to go unrecognized.
  • Seven tumors arose in soft tissue (6 lower limb; 1 upper limb), 5 in the gastrointestinal tract (1 involving both stomach and colon, 1 ileum, 2 rectum, 1 anus), 1 in the nasal cavity, and 1 in the lung.
  • Six patients were treated with postoperative radiation and 7 with chemotherapy (CHOP or ProMACE-MOPP).
  • Two tumors recurred locally, and 5 patients developed distant spread: 3 to lymph nodes, 1 to lung, and 1 to bone.
  • At the last follow-up, 2 patients have died of disseminated disease, 4 and 5 months following initial diagnosis.
  • Histiocytic sarcoma may arise primarily in soft tissue and shows reproducible histologic features, including abundant eosinophilic cytoplasm and a prominent inflammatory infiltrate.
  • Metastatic carcinoma, metastatic melanoma, and large cell non-Hodgkin lymphomas should be excluded by immunohistochemistry.
  • Histiocytic sarcoma has the potential for an aggressive clinical course, most often with lymph node involvement.
  • [MeSH-major] Sarcoma / pathology

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  • (PMID = 15316312.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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17. Khalid S, Adil SN, Vaziri IA: Granulocytic sarcoma in the absence of acute myeloid leukemia: a case report. Indian J Pathol Microbiol; 2007 Jan;50(1):88-90
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  • [Title] Granulocytic sarcoma in the absence of acute myeloid leukemia: a case report.
  • Granulocytic sarcoma is an extramedullary tumor composed of immature granulocytic precursor cells.
  • The most common sites of presentation are bone, periosteum, soft tissue, lymph node, skin, and infrequently small intestine.
  • It can occur without blood or bone marrow manifestations of leukemia and in this case, the diagnosis is difficult.
  • Our patient was initially diagnosed as a case of T-cell non Hodgkin's lymphoma and received one cycle of CHOP with only transient improvement in his symptoms.
  • Subsequently, his biopsy slides were reviewed at our centre and were reported as granulocytic sarcoma.
  • [MeSH-major] Sarcoma, Myeloid / diagnosis
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Biopsy. Bone Marrow / pathology. Cyclophosphamide / administration & dosage. Diagnosis, Differential. Doxorubicin / administration & dosage. Histocytochemistry. Humans. Leukemia, Myeloid, Acute / complications. Lymphoma, Non-Hodgkin / diagnosis. Lymphoma, Non-Hodgkin / drug therapy. Male. Neoplasms. Prednisolone / administration & dosage. Vincristine / administration & dosage

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  • (PMID = 17474271.001).
  • [ISSN] 0377-4929
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 9PHQ9Y1OLM / Prednisolone; VAP-cyclo protocol
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18. Ehrig T, Billings SD, Fanburg-Smith JC: Superficial primitive neuroectodermal tumor/Ewing sarcoma (PN/ES): same tumor as deep PN/ES or new entity? Ann Diagn Pathol; 2007 Jun;11(3):153-9
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  • [Title] Superficial primitive neuroectodermal tumor/Ewing sarcoma (PN/ES): same tumor as deep PN/ES or new entity?
  • Primitive neuroectodermal tumor/Ewing sarcoma (PN/ES) is a single clinical, morphologic, and molecular small round cell tumor entity.
  • These are generally found in deep soft tissue or bone of young male patients, with poor behavior.
  • Most cases were small (0.5-2.3 centimeters) and painful, and persisted for less than 1 year.
  • Nine cases with material for reverse transcription-polymerase chain reaction revealed 1 positive type 2 translocation (EWS exon 7 to Fli-1 exon 5), 4 negative, and 4 "unable to amplify."
  • Treatment was by wide excision; 9 received chemotherapy and 6 radiation.
  • Cutaneous PN/ES is a superficial round cell tumor in older women, with better prognosis than deep PN/ES.
  • [MeSH-major] Neuroectodermal Tumors / diagnosis. Neuroectodermal Tumors, Primitive / diagnosis. Sarcoma, Ewing / diagnosis. Skin Neoplasms / diagnosis
  • [MeSH-minor] Adolescent. Adult. Aged. Antigens, CD / genetics. Antigens, CD / metabolism. Cell Adhesion Molecules / genetics. Cell Adhesion Molecules / metabolism. Child. Child, Preschool. Diagnosis, Differential. Female. Gene Expression Regulation, Neoplastic. Humans. Male. Middle Aged. Prognosis. Retrospective Studies. Sex Characteristics. Vimentin / genetics. Vimentin / metabolism

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  • (PMID = 17498589.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules; 0 / Vimentin
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19. Thanakit V, Nelson SD, Udomsawaengsup S: Round cell liposarcoma of scrotum with indolent course in young adult. J Med Assoc Thai; 2005 Sep;88(9):1302-7
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  • [Title] Round cell liposarcoma of scrotum with indolent course in young adult.
  • Myxoid/Round cell liposarcoma accounts for one-half of all liposarcomas and occurs as the second most common subtype.
  • Both myxoid and round cell types share clinical, histological features and are accepted to represent a spectrum of lesions ranging from pure myxoid to near completely round cell liposarcoma.
  • Round cell liposarcoma is highly metastatic and is classified as high grade and poorly differentiated myxoid sarcoma.
  • Typical non-round cell myxoid liposarcoma is less metastatic and has more favorable prognosis.
  • Karyotypic study of myxoid and round cell liposarcomas reveal a characteristic reciprocal translocation t (12;16)(q13;p11) resulting in the fusion of CHOP gene with TLS gene in more than 90% of cases.
  • Most masses within the scrotal sac arise from the testis proper, and less likely from the extratesticular tissue.
  • Myxoid/round cell liposarcoma and round cell liposarcoma are rarely encounter in extratesticular soft tissue.
  • We reported a rare case of round cell liposarcoma (high grade myxoid liposarcoma) of extratesticular tissue.
  • To our knowledge, this is the first case of a large size (> 5cm) round cell liposarcoma arising from soft tissue within the scrotal sac of young adult with indolent course.
  • Simple excision or enucleation are inadequate therapies and wide excision of the hemiscrotum including inguinal soft tissue and nodes is recommended.
  • The role of retroperitoneal lymph node dissection and adjuvant chemotherapy remains controversial.
  • [MeSH-major] Carcinoma, Small Cell / pathology. Liposarcoma, Myxoid / pathology. Testicular Neoplasms / pathology
  • [MeSH-minor] Adult. Diagnosis, Differential. Humans. Male. Prognosis. Scrotum / pathology. Testicular Hydrocele / pathology

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  • (PMID = 16536120.001).
  • [ISSN] 0125-2208
  • [Journal-full-title] Journal of the Medical Association of Thailand = Chotmaihet thangphaet
  • [ISO-abbreviation] J Med Assoc Thai
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Thailand
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20. Mentzel T, Kuhnen C: Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases. Virchows Arch; 2006 Nov;449(5):554-60
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  • [Title] Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases.
  • Rhabdomyosarcoma (RMS) is currently classified into embryonal RMS, including its botryoid and spindle cell variants, alveolar RMS, including a solid variant, and pleomorphic RMS.
  • Most recently rare spindle cell and sclerosing, pseudovascular RMS have been reported in adults as well.
  • We analysed the clinicopathological and immunohistochemical features of seven new cases of spindle cell RMS arising in adult patients.
  • Five neoplasms were completely excised, in one incompletely excised neoplasm additional chemotherapy was given, and in one patient a biopsy was done only so far.
  • All neoplasms arose in subcutaneous and deep soft tissues with dermal involvement in one case, and the size of the neoplasms ranged from 4 to 19 cm in largest diameter.
  • In addition, focal areas reminiscent of sclerosing, pseudovascular RMS were noted in three cases, and in two cases each small solid areas with pleomorphic tumour cells as well as scattered round tumour cells were present.
  • In summary, spindle cell rhabdomyosarcoma represents a rare neoplasm in adulthood characterized clinically by a rather poor prognosis, and shows a broad morphological spectrum including most likely the sclerosing, pseudovascular variant.
  • Immunohistochemically, tumour cells in RMS stain positively for CD 99 and WT1 as well, which is of importance in the differential diagnosis to other mesenchymal neoplasms, whereas fast myosin does not represent a reliable marker for RMS in adults.
  • [MeSH-major] Rhabdomyosarcoma / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Antigens, CD / analysis. Antigens, CD99. Cell Adhesion Molecules / analysis. Female. Humans. Immunohistochemistry. Male. Middle Aged

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  • (PMID = 17013628.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD99; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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21. Poprach A, Michalová E, Pavlík T, Lakomy R, Vyskocil J, Nemeccek R, Zaloudík J, Vyzula R, Kocák I, Kocáková I: [Actual state of ex vivo chemoresistance testing of malignant tumors in Masaryk Memorial Cancer Institute Brno]. Klin Onkol; 2008;21(3):116-21
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  • Chemoresistance assay results may play a role in cancer management decision process.
  • Five groups of different types of cancer in particular clinical stages were defined for chemosensitivity testing with:.
  • (1) metastatic malignant melanoma, (2) soft tissue sarcoma (STS), either primary or recurrent/metastic, (3) primary or metastatic renal cancer, (4) recurrent ovarian cancer and (5) other diagnosis "on clinician's request".
  • Sensitivity to certain chemotherapy agent observed ex vivo does not necessarily mean that the cancer would also be sensitive to the same agent in vivo, however, ex vivo resistance with following in vivo sensitivity of the tumour has not been observed to date.
  • The cultivation of malignant cells is very uncertain in solid tumours, which consist of several malignant cell multiclones (benign/stromal cells may outgrow malignant cells).
  • The small number of successfully evaluated samples has not yet provided us to carry out proper statistical evaluation and clinical application.
  • [MeSH-major] Drug Screening Assays, Antitumor
  • [MeSH-minor] Drug Resistance, Neoplasm. Female. Humans. Kidney Neoplasms / drug therapy. Melanoma / drug therapy. Ovarian Neoplasms / drug therapy. Sarcoma / drug therapy

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  • (PMID = 19097421.001).
  • [ISSN] 0862-495X
  • [Journal-full-title] Klinická onkologie : casopis Ceské a Slovenské onkologické spolecnosti
  • [ISO-abbreviation] Klin Onkol
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Czech Republic
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22. Hadfield MG, Quezado MM, Williams RL, Luo VY: Ewing's family of tumors involving structures related to the central nervous system: a review. Pediatr Dev Pathol; 2000 May-Jun;3(3):203-10

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • This review consolidates information gleaned from several case reports and larger series on Ewing's sarcoma family of tumors (EFT) involving structures related to and found in the central nervous system (CNS).
  • These tumors involve the skull, the spinal column, adjacent soft tissues, the meninges, and the brain.
  • We have separated the cases by skull region and spinal column level, and we discuss the attendant differences in prognosis following treatment by neurosurgery, radiation, and chemotherapy.
  • Light and electron microscopic features can be used to differentiate EFT from other small round blue cell tumors that involve the CNS (central primitive neuroectodermal tumor, lymphoma, etc.).
  • [MeSH-major] Central Nervous System Neoplasms / diagnosis. Sarcoma, Ewing / diagnosis
  • [MeSH-minor] Adolescent. Child. Diagnosis, Differential. Epidural Neoplasms / diagnosis. Female. Genetic Techniques. Humans. Immunohistochemistry. Infant. Jaw Neoplasms / diagnosis. Male. Microscopy, Electron. Neuroectodermal Tumors, Primitive / diagnosis. Prognosis. Skull Neoplasms / diagnosis. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / secondary. Spinal Neoplasms / diagnosis

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  • (PMID = 10742406.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 122
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23. Antonescu CR, Rosenblum MK, Pereira P, Nascimento AG, Woodruff JM: Sclerosing epithelioid fibrosarcoma: a study of 16 cases and confirmation of a clinicopathologically distinct tumor. Am J Surg Pathol; 2001 Jun;25(6):699-709
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Sclerosing epithelioid fibrosarcoma (SEF) is an uncommon tumor of deep soft tissues, originally described in 1995 by Meis-Kindblom et al.
  • Histologically, the SEFs were composed predominantly of small to moderate-size round to ovoid, relatively uniform cells, often with clear cytoplasm, embedded in a hyalinized fibrous stroma.
  • Treatment consisted of intralesional excision (n = 2), attempted wide local excision (n = 11), and amputation (n = 3), with either adjuvant radiation therapy (n = 9) or chemotherapy (n = 3).
  • Eight patients (57%) died of disease 16 to 86 months after diagnosis.
  • SEF shares some pathologic features with two other fibrosing fibrosarcomas, low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes, but in the authors' experience behaves clinically as a fully malignant sarcoma.
  • [MeSH-major] Fibrosarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 11395547.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] United States
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24. Kebudi R, Görgün O, Ayan I: Oral etoposide for recurrent/progressive sarcomas of childhood. Pediatr Blood Cancer; 2004 Apr;42(4):320-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PROCEDURE: Twenty-one children (10 girls and 11 boys) with R/P sarcomas and a median age of 11 years (range 3-16 years) were enrolled in this study.
  • The diagnosis was Ewing sarcoma family tumor (ESFT) in seven, osteosarcoma in eight, rhabdomyosarcoma in four, clear cell sarcoma of soft tissue in one, fibrosarcoma in one patient.
  • They are alive with no evidence of disease (NED) 79 and 94 months from time of relapse/progressive disease (PD).
  • A patient developed acute myeloid leukemia and died.
  • CONCLUSIONS: Oral VP-16 therapy is simple, relatively nontoxic, and does not necessitate hospitalization.
  • The cure rate is small.
  • Given the risk of second malignancy, especially in children with previous exposure to topoisomerase II inhibitors and alkylating agents, this regimen may be used as a palliative treatment or in patients with poor prognosis.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / administration & dosage. Etoposide / administration & dosage. Sarcoma / drug therapy
  • [MeSH-minor] Administration, Oral. Adolescent. Child. Child, Preschool. Disease Progression. Female. Humans. Male. Neoplasms, Second Primary / chemically induced. Palliative Care. Recurrence. Remission Induction. Survival Rate. Treatment Outcome

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  • [Copyright] Copyright 2003 Wiley-Liss, Inc.
  • (PMID = 14966827.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide
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25. Attabib NA, West M, Rhodes RH: Peripheral primitive neuroectodermal tumor of the cavernous sinus: case report. Neurosurgery; 2006 May;58(5):E992; discussion E992
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: Ewing sarcoma/peripheral primitive neuroectodermal tumors (pPNET family) are small, round, blue cell tumors that have a decided predilection for young patients and commonly arise in bone and soft tissue.
  • INTERVENTION: The patient underwent debulking of the tumor, and the diagnosis of a pPNET was made based on histological, immunohistochemical, and molecular genetics (EWS-FLI1 fusion gene) findings.
  • The patient had adjuvant treatment with radiotherapy and chemotherapy.
  • After 14 months, the patient had no neurological deficits, and neuroimaging showed stable disease, although some chemotherapy complications occurred.
  • CONCLUSION: This is a case of cavernous sinus pPNET in a 48-year-old woman, in whom the diagnosis is supported by the presence of EWS-FLI1 fusion gene.
  • [MeSH-major] Cavernous Sinus / pathology. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / genetics

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  • (PMID = 16639307.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS
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26. Belaabidia B, Sellami S, Hamdaoui R, Essadki B: [Primary malignant non-Hodgkin skeletal muscle lymphoma: a case report]. Rev Chir Orthop Reparatrice Appar Mot; 2002 Sep;88(5):518-21
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Malignant non-Hodgkin lymphoma (NHL) is rarely encountered in soft tissue.
  • The differential diagnosis with other soft tissue tumors, particularly sarcoma, is difficult.
  • At computed tomography, the mass measured 17x14x7 cm and was situated in the biceps femoris.
  • Tumor biopsy demonstrated diffuse malignant small B-cell lymphomatous-plasma cell proliferation.
  • The diagnosis was primary NHL of the biceps femoris.
  • Wide resection was followed by chemotherapy.
  • Magnetic resonance imaging and computed tomography visualize characteristic features.
  • Diagnosis is formally established by pathology.
  • [MeSH-major] Leukemia, Lymphocytic, Chronic, B-Cell / diagnosis. Muscle Neoplasms / diagnosis
  • [MeSH-minor] Aged. Antineoplastic Agents / therapeutic use. Biopsy. Chemotherapy, Adjuvant. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Immunohistochemistry. Thigh. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 12399719.001).
  • [ISSN] 0035-1040
  • [Journal-full-title] Revue de chirurgie orthopédique et réparatrice de l'appareil moteur
  • [ISO-abbreviation] Rev Chir Orthop Reparatrice Appar Mot
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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27. Dunst J, Ahrens S, Paulussen M, Burdach S, Jürgens H: Prognostic impact of tumor perfusion in MR-imaging studies in Ewing tumors. Strahlenther Onkol; 2001 Mar;177(3):153-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • This has been demonstrated for head and neck cancers, gliomas and adult soft tissue sarcomas.
  • The median follow-up at the time of this analyses was 3 years.
  • All patients were treated according to the multicentric EICESS-92 protocol with multi-agent chemotherapy (VACA or VAIA or EVAIA) and local therapy (radiotherapy with 50-60 Gy or surgery or surgery with pre- or postoperative irradiation with 45-50 Gy).
  • Tumor site (central location vs proximal extremities vs distal extremities) had no impact on necrosis (p = 0.71).
  • 23 out of 79 patients had metastases (M1) at the time of diagnosis.
  • "Unfavorable" metastatic spread (bone or multiple metastases) was only noted in patients with high amount of necrosis (> 25% necrosis) whereas even large tumors did not show unfavorable metastases if they contained no or only small amounts of necrosis.
  • CONCLUSIONS: The presence of non-perfused (presumably necrotic) areas on pretreatment contrast-enhanced MR-images is associated with an increased risk of metastases, especially an unfavorable pattern of metastatic spread at diagnosis.
  • The small group of patients with non-necrotic tumors (13%) had an excellent prognosis suggesting that the absence of necrosis might be helpful in identifying a very favorable prognostic subgroup in Ewing tumors.
  • [MeSH-major] Bone Neoplasms / blood supply. Cell Hypoxia / physiology. Magnetic Resonance Imaging. Sarcoma, Ewing / blood supply
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Male. Necrosis. Prognosis. Regional Blood Flow / physiology. Survival Rate

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  • (PMID = 11285773.001).
  • [ISSN] 0179-7158
  • [Journal-full-title] Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]
  • [ISO-abbreviation] Strahlenther Onkol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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28. Budarin MA: [Prognostic factors in childhood sarcomas of neuroectodermal histogenesis]. Vopr Onkol; 2002;48(3):335-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [MeSH-major] Bone Neoplasms / therapy. Esthesioneuroblastoma, Olfactory / therapy. Nasal Cavity. Nose Neoplasms / therapy. Sarcoma, Ewing / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Infant. Male. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / mortality. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Neuroectodermal Tumors, Primitive, Peripheral / radiotherapy. Neuroectodermal Tumors, Primitive, Peripheral / therapy. Prognosis. Radiotherapy Dosage. Sarcoma, Small Cell / drug therapy. Sarcoma, Small Cell / mortality. Sarcoma, Small Cell / pathology. Sarcoma, Small Cell / radiotherapy. Sarcoma, Small Cell / surgery. Sarcoma, Small Cell / therapy

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  • (PMID = 12455358.001).
  • [ISSN] 0507-3758
  • [Journal-full-title] Voprosy onkologii
  • [ISO-abbreviation] Vopr Onkol
  • [Language] rus
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Russia
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