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1. Bisogno G, Carli M, Stevens M, Oberlin O, Treuner J, Scarzello G, Colombatti R, De Zen L, Pinkerton CR: Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue. Bone Marrow Transplant; 2002 Sep;30(5):297-302
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue.
  • The MMT4 study was designed to explore an intensive chemotherapy regimen (MMT4-89) and the role of high-dose melphalan (MMT4-91) in children with metastatic soft tissue sarcoma, including extraosseous peripheral neuroectodermal tumor (PNET).
  • Chemotherapy consisted of four CEVAIE cycles, each including three 3-week courses: CEV (carboplatin 500 mg/m(2), epirubicin 150 mg/m(2), vincristine 1.5 mg/m(2)), IVA ifosfamide 9 g/m(2), actinomycin 1.5 mg/m(2), vincristine 1.5 mg/m(2)), IVE (ifosfamide 9 g/m(2), etoposide 600 mg/m(2), vincristine 1.5 mg/m(2)).
  • In conclusion, despite the high CR rate, intensive chemotherapy with or without high-dose melphalan appeared to have little impact on the survival of patients with metastatic extraosseus PNET.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Melphalan / administration & dosage. Neuroectodermal Tumors / therapy. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Age Factors. Bone Marrow Transplantation. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Male. Peripheral Blood Stem Cell Transplantation. Remission Induction / methods. Survival Analysis. Treatment Outcome

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  • (PMID = 12209351.001).
  • [ISSN] 0268-3369
  • [Journal-full-title] Bone marrow transplantation
  • [ISO-abbreviation] Bone Marrow Transplant.
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] Q41OR9510P / Melphalan
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2. 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.
  • Magnetic resonance (MR) imaging revealed a large extraaxial tumor with cyst at the right frontotemporal region.
  • The solid part of the tumor was homogeneously enhanced on T(1)-weighted MR imaging after injection of gadolinium.
  • Digital subtraction angiography of the external carotid artery revealed sunburst appearance corresponding to the tumor, which was fed by the right middle meningeal artery.
  • His headache worsened and computed tomography revealed enlargement of the tumor and intracystic hemorrhage, so emergent operation was performed.
  • At surgery, the tumor strongly adhered to the dural membrane, and was obviously extraaxial.
  • The tumor and cyst were gross totally removed.
  • 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-major] Brain Neoplasms / pathology. Meningioma / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology
  • [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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3. Al Meshaan MK, Nayef M, Kwaider T, Otto W, Katchy KC: Peripheral primitive neuroectodermal tumor of the urinary bladder in an Arab woman with history of squamous cell carcinoma: a case report. J Med Case Rep; 2009;3:6840

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  • [Title] Peripheral primitive neuroectodermal tumor of the urinary bladder in an Arab woman with history of squamous cell carcinoma: a case report.
  • INTRODUCTION: Peripheral primitive neuroectodermal tumors of the urinary bladder are rare and tend to occur in an older age group than do their counterparts in bones and soft tissue.
  • CASE PRESENTATION: We report a case of peripheral primitive neuroectodermal tumor of the urinary bladder in a 67-year-old woman of Arab origin.
  • She had undergone transurethral resection followed by chemotherapy because of pulmonary metastasized muscle-invasive squamous cell carcinoma of the bladder in 2005.
  • Performing cystoscopy any tumor could be detected.
  • Control cystoscopy two months later showed a tumor mass of 3 cm in diameter at another location than described for the first tumor.
  • Tissue specimen after pathological analysis revealed a peripheral primitive neuroectodermal tumor with tumor cells reactive to cluster of differentiation 99, neuron-specific enolase and S100 protein and stained negative for other markers such as cytokeratins, epithelial membrane antigen, desmin, smooth muscle actin, chromogranin and leucocyte common antigen.
  • Staging computerized tomography was especially free from any hint on organ metastasis, but the patient died due to a cardiac problem only a few months later.
  • CONCLUSIONS: To the best of our knowledge, we report the eighth case of bladder peripheral primitive neuroectodermal tumors in literature and the first concerning an Arab patient.
  • It is also the first presentation of a peripheral primitive neuroectodermal tumor patient with a history of squamous cell carcinoma of the bladder.
  • As in other cases, expression of single-chain-type 1 glycoprotein and neural markers was positive and the disease was at an advanced stage at the time of diagnosis.

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  • (PMID = 19830128.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2726497
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4. Lee YY, Kim DH, Lee JH, Choi JS, In KH, Oh YW, Cho KH, Roh YK: Primary pulmonary Ewing's sarcoma/primitive neuroectodermal tumor in a 67-year-old man. J Korean Med Sci; 2007 Sep;22 Suppl:S159-63
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  • [Title] Primary pulmonary Ewing's sarcoma/primitive neuroectodermal tumor in a 67-year-old man.
  • Extraskeletal Ewing's sarcoma (EES) is a branch of neuroectodermal tumor (PNET), which is very rare soft tissue sarcoma.
  • Computed tomography, bone scintigraphy, and positron emission tomography confirmed the mass to have a primary pulmonary origin.
  • The diagnosis was confirmed both pathologically and genetically.
  • The mass lesion was resected, and the patient is currently undergoing chemotherapy.
  • [MeSH-major] Lung Neoplasms / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Sarcoma, Ewing / diagnosis
  • [MeSH-minor] Aged. Calmodulin-Binding Proteins / genetics. Chromosome Breakage. Chromosomes, Human, Pair 22 / genetics. Diagnosis, Differential. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. RNA-Binding Proteins / genetics

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  • (PMID = 17923745.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Calmodulin-Binding Proteins; 0 / EWSR1 protein, human; 0 / RNA-Binding Proteins
  • [Other-IDs] NLM/ PMC2694395
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5. Hormozi AK, Ghazisaidi MR, Hosseini SN: Unusual presentation of peripheral primitive neuroectodermal tumor of the maxilla. J Craniofac Surg; 2010 Nov;21(6):1761-3

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  • [Title] Unusual presentation of peripheral primitive neuroectodermal tumor of the maxilla.
  • The peripheral primitive neuroectodermal tumor (pPNET) is a rare and highly malignant soft tissue neoplasm in children and young adults.
  • Two years after diagnosis, she experienced diplopia, and then magnetic resonance imaging was done, which showed a mass in the optic chiasma and parasellar region.
  • The typical appearance resembled large noncalcified soft tissue masses in the magnetic resonance image and computed tomographic scan of the maxilla.
  • Diagnosis was established by immunohistochemical features.
  • She was treated with surgery, chemotherapy, radiation therapy, and gamma knife.
  • She was under close observation since then (approximately 8 mo), and there has been no recurrence of tumor up to now.
  • [MeSH-major] Maxillary Neoplasms / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Female. Follow-Up Studies. Humans. Neoadjuvant Therapy. Neoplasm Recurrence, Local / pathology. Optic Chiasm / pathology. Optic Nerve Neoplasms / secondary. Radiosurgery. Radiotherapy, Adjuvant. Sella Turcica / pathology. Skull Neoplasms / secondary

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  • (PMID = 21119416.001).
  • [ISSN] 1536-3732
  • [Journal-full-title] The Journal of craniofacial surgery
  • [ISO-abbreviation] J Craniofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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6. Bisogno G, Ferrari A, Bergeron C, Scagnellato A, Prete A, Alaggio R, Casanova M, D'Angelo P, Di Cataldo A, Carli M: The IVADo regimen--a pilot study with ifosfamide, vincristine, actinomycin D, and doxorubicin in children with metastatic soft tissue sarcoma: a pilot study of behalf of the European pediatric Soft tissue sarcoma Study Group. Cancer; 2005 Apr 15;103(8):1719-24
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  • [Title] The IVADo regimen--a pilot study with ifosfamide, vincristine, actinomycin D, and doxorubicin in children with metastatic soft tissue sarcoma: a pilot study of behalf of the European pediatric Soft tissue sarcoma Study Group.
  • BACKGROUND: The role of doxorubicin (Doxo) as part of multidrug regimens used to treat children with soft tissue sarcoma (STS) is controversial.
  • METHODS: Between July 2002 and February 2004, 29 evaluable patients were enrolled in this study; 19 patients had rhabdomyosarcoma, 5 patients had peripheral neuroectodermal tumor, and 5 patients had other types of STS.
  • Three courses of IVADo were to be administered in the initial part of treatment and analyzed for toxicity and tumor response.
  • Nonhematologic toxicity included Grade 3-4 mucositis (6.5% of cycles), constipation (9.7%), and peripheral neuropathy (6.5%).
  • All but 1 patient with a malignant schwannoma showed some degree of tumor volume reduction; however, considering only complete and partial remissions, the response rate was 76% (+/- 7.9%).
  • This combination will be investigated in high-risk patients with rhabdomyosarcoma in a randomized trial launched by the European pediatric Soft tissue sarcoma Study Group.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Sarcoma / drug therapy. Sarcoma / secondary

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  • [Copyright] (c) 2005 American Cancer Society.
  • (PMID = 15754335.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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7. Van Winkle P, Angiolillo A, Krailo M, Cheung YK, Anderson B, Davenport V, Reaman G, Cairo MS: Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children's Cancer Group (CCG) experience. Pediatr Blood Cancer; 2005 Apr;44(4):338-47
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  • [Title] Ifosfamide, carboplatin, and etoposide (ICE) reinduction chemotherapy in a large cohort of children and adolescents with recurrent/refractory sarcoma: the Children's Cancer Group (CCG) experience.
  • PROCEDURE: Children with recurrent/refractory sarcoma were treated with ifosfamide (1,800 mg/m2/day on day 0-4), carboplatin (400 mg/m2/day on day 0-1), etoposide (100 mg/m2/day on day 0-4) and either rhG-CSF (10 microg/kg/day vs. 5 microg/kg/day, CCG-0894, 71 patients), PIXY321 (500-1,000 microg/m2/day, CCG-0924, 14 patients), or rhG-CSF (5 microg/kg/day) and IL-6 (2.5-5 microg/kg/day, CCG-0931, 12 patients).
  • RESULTS: Ninety-seven patients were evaluable for tumor response, 56 male and 41 female, median age 14.1 years (range 2.8-22.5 years).
  • Tumor types were osteosarcoma (OTS) (n = 34), rhabdomyosarcoma (n = 27), Ewing sarcoma (EWS) (n = 21), soft tissue sarcoma-not otherwise specified (n = 5), undifferentiated sarcoma (n = 6), fibrosarcoma (n = 2), peripheral primitive neuroectodermal tumor (n = 1), and extraosseous Ewing (n = 1).
  • CONCLUSIONS: The ORR to ICE reinduction chemotherapy in children with recurrent/refractory sarcoma was 51%.
  • OS of 1 and 2 years appeared significantly improved in patients who had CR or PR following ICE reinduction therapy or who had rhabdomyosarcoma with embryonal histology.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Sarcoma / drug therapy
  • [MeSH-minor] Adolescent. Adult. Carboplatin / administration & dosage. Child. Child, Preschool. Colony-Stimulating Factors / administration & dosage. Etoposide / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Male. Multivariate Analysis. Proportional Hazards Models. Recurrence. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / mortality. Survival Rate

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  • (PMID = 15503297.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Clinical Trial, Phase I; Clinical Trial, Phase II; Journal Article; Randomized Controlled Trial; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Colony-Stimulating Factors; 6PLQ3CP4P3 / Etoposide; BG3F62OND5 / Carboplatin; UM20QQM95Y / Ifosfamide
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8. Adibi A, Barikbin R, Koleini N, Farghadani M, Mougouei K, Farshidfar F: Solitary epidural brain metastasis of a peripheral neuroepithelioma (a primitive neuroectodermal tumor): a case report. J Radiol Case Rep; 2008;2(1):16-9

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  • [Title] Solitary epidural brain metastasis of a peripheral neuroepithelioma (a primitive neuroectodermal tumor): a case report.
  • The patient was a known case of cervical soft tissue primitive neuroectodermal tumor (PNET) who has undergone surgery and radiotherapy 4 years ago.
  • Subsequently, surgery was performed and the resultant biopsy was neuroepithelioma.
  • After diagnosis the patient has undergone chemotherapy and radiotherapy.
  • This is the first reported case of epidural metastasis of a head and neck (peripheral) PNET.

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  • (PMID = 22470583.001).
  • [ISSN] 1943-0922
  • [Journal-full-title] Journal of radiology case reports
  • [ISO-abbreviation] J Radiol Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3303233
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9. Martin RC 2nd, Brennan MF: Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival? Arch Surg; 2003 Mar;138(3):281-5
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  • [Title] Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival?
  • The improvement in survival is primarily associated with the combination of surgery and chemotherapy.
  • HYPOTHESIS: Little is known about the outcome of adults with soft tissue ES or primitive neuroectodermal tumors (PNET).
  • Certain prognostic factors from soft tissue sarcomas (tumor size, tumor location, margin status, and initial presentation) in adults (>16 years) with ES/PNET will help to identify factors associated with outcome.
  • METHODS: Between July 1, 1982, and June 30, 2000, we identified 59 adult patients with primary soft tissue ES/PNET.
  • Clinicopathologic factors were correlated with the end points studied: patient factors, tumor factors, pathologic factors, status of surgical margins, adjuvant chemotherapy, and radiation therapy.
  • Median tumor size was 8 cm, with all lesions being high grade.
  • The median follow-up was 29 months (range, 6-222 months), with local recurrence identified in 13 patients (22%), with a median time to recurrence of 15 months (range, 5-200 months).
  • Initial presentation was the only predictor of long-term survival, with primary tumor-only presentation having a 5-year survival of 60% (median not reached) compared with primary tumor plus metastatic disease having a 5-year survival of 33% (median, 17 months) (P =.02).
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / mortality. Sarcoma, Ewing / mortality. Soft Tissue Neoplasms / mortality
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local

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  • (PMID = 12611575.001).
  • [ISSN] 0004-0010
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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10. 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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  • [Title] Peripheral primitive neuroectodermal tumor of the cavernous sinus: case report.
  • 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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11. Weissferdt A, Neuling K, English M, Arul S, McMullan D, Ely A, Bründler MA, Brown R: Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation. Pediatr Dev Pathol; 2006 May-Jun;9(3):229-33
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  • [Title] Peripheral primitive neuroectodermal tumor with postchemotherapy neuroblastoma-like differentiation.
  • We report the case of an 11-year-old girl with a retroperitoneal tumor in the left upper quadrant.
  • The girl was admitted to hospital with weight loss and a painless abdominal mass that on biopsy was diagnosed as a peripheral primitive neuroectodermal tumor/Ewing sarcoma (pPNET/EWS) of the soft tissue.
  • The patient underwent chemotherapy followed by surgical resection of the tumor 5 months after diagnosis.
  • The posttreatment residual viable tumor showed a morphologic appearance resembling a neuroblastoma.
  • This case is unusual in the sense of showing the typical gene fusion for pPNET/EWS both in the pretherapy sample with the typical morphological appearance of this tumor and in the posttherapy specimen showing neural differentiation suggestive of a neuroblastoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Cell Differentiation. Neuroblastoma / physiopathology. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / physiopathology
  • [MeSH-minor] Child. Chromosomes, Human, Pair 11. Chromosomes, Human, Pair 22. Cyclophosphamide / therapeutic use. Dactinomycin / therapeutic use. Disease-Free Survival. Female. Follow-Up Studies. Humans. In Situ Hybridization, Fluorescence. Karyotyping. Oncogene Fusion. Radiotherapy, Adjuvant. Time Factors. Tomography, X-Ray Computed. Translocation, Genetic. Vincristine / therapeutic use

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  • (PMID = 16944972.001).
  • [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] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; VAC protocol
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12. Koscielniak E, Morgan M, Treuner J: Soft tissue sarcoma in children: prognosis and management. Paediatr Drugs; 2002;4(1):21-8
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  • [Title] Soft tissue sarcoma in children: prognosis and management.
  • Soft tissue sarcomas (STS) account for approximately 7% of malignant neoplasms in children.
  • The heterogeneity of STS makes the diagnosis and therapy particularly difficult and should be reserved for specialized centers with expertise in treating cancer in children.
  • Major progress in the accuracy of diagnosis and classification has been made by the identification of specific, recurring genetic alterations t(2;13)(q35;q14) and t(1;13)(p36;q14) in alveolar rhabdomyosarcomas (RMS), t(X;18)(p11;q11) for synovial sarcoma (SS) and t(11;22)(q24;q12) or t(21;22)(q22;q12) for Ewing's tumor family.
  • As a result of large multicenter STS studies, such as the North-American Intergroup Rhabdomyosarcoma Study, the German Pediatric Soft Tissue Sarcoma Study Group (CWS), Italian Gruppo Cooperativo Italiano study and Sociètè Internationale d'Oncologie Pèdiatrique (SIOP) Malignant Mesenchymal Tumors study, the identification of more effective treatment strategies and improvement in prognosis have been made in the last 30 years.
  • Prognostic variables were identified and, by exploring novel therapeutic strategies, criteria were established for the use of preoperative chemotherapy and radiotherapy, and primary and delayed second-look surgery.
  • As a result of evaluation of different drugs active in STS, refinements in the utilization of chemotherapy have been made possible.
  • Clinical trials have also been instrumental in defining the late effects of treatment.
  • In STS the following drugs have proven to be useful: dactinomycin, vincristine, alkylating agents such as cyclophosphamide and ifosfamide, as well as anthracyclines such as doxorubicin (adriamycin) and epi-doxorubicin.
  • Recommendations for radiation are dependent on the primary site and size of the tumour, histology, patient age and the extent of disease before and after surgical resection.
  • In general, with conventional fractionation (1 x 1.8 to 2 Gy/day) radiotherapy doses between 40 and 50 Gy should be administered.
  • The German CWS group explored the effectiveness of 32 Gy when accelerated and hyperfractionated, and given simultaneously to chemotherapy, and found it adequate for local tumor control in patients with selected favorable prognostic factors.
  • When treated with a combination of chemotherapy and local therapy, STS showed an event-free survival between 50 and 80% [RMS 70%, extraskeletal Ewing sarcoma (EES) and peripheral neuroectodermal tumor (PNET) circa 50%, and SS 70 to 80%].
  • The value of high dose chemotherapy with hematopoietic stem cell rescue in patients with poor prognostic STS remains unclear and should be performed in controlled studies only.
  • [MeSH-major] Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols. Child. Combined Modality Therapy. Humans. Neoplasm Staging. Prognosis. Radiotherapy. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / surgery. Rhabdomyosarcoma / therapy

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  • (PMID = 11817983.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
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13. Llombart-Bosch A, Pellín A, Carda C, Noguera R, Navarro S, Peydró-Olaya A: Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript. Diagn Mol Pathol; 2000 Sep;9(3):137-44
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  • [Title] Soft tissue Ewing sarcoma--peripheral primitive neuroectodermal tumor with atypical clear cell pattern shows a new type of EWS-FEV fusion transcript.
  • This study describes a new case of Ewing sarcoma (ES)-peripheral primitive neuroectodermal tumor (pPNET) with unusual phenotype and fusion gene structure.
  • The tumor located in the inguinal area of a 15-year-old boy showed a highly aggressive behavior with hematogenous metastases after intensive chemotherapy and bone marrow transplant, causing death 28 months after diagnosis.
  • The tumor displayed a clear cell pattern, and several neuroectodermal markers proved positive both in the original tumor and in xenografts.
  • This neuroectodermal character was confirmed by electron microscopy.
  • Moreover, cytogenetically the tumor has an unusual chromosomal rearrangement, t(2;22)(q13;q22,t(3;18)(p21;q23); representing a new EWS-FEV fusion type in which exon 7 of EWS gene is fused with exon 2 of FEV gene.
  • This is the third published study of an ES-pPNET showing EWS-FEV fusion described, but it is the first study of a tumor with the aforementioned fusion points.
  • [MeSH-major] Chromosomes, Human, Pair 2 / genetics. Chromosomes, Human, Pair 22 / genetics. Neuroectodermal Tumors, Primitive / genetics. Oncogene Proteins, Fusion / genetics. Sarcoma, Ewing / genetics. Soft Tissue Neoplasms / genetics. Translocation, Genetic / genetics
  • [MeSH-minor] Adolescent. Animals. Combined Modality Therapy. Disease Progression. Exons / genetics. Fatal Outcome. Groin. Humans. Karyotyping. Male. Mice. Mice, Nude. Neoplasm Metastasis. Neoplasm Proteins / analysis. Prognosis. Reverse Transcriptase Polymerase Chain Reaction. Transplantation, Heterologous. Tumor Cells, Cultured

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  • (PMID = 10976720.001).
  • [ISSN] 1052-9551
  • [Journal-full-title] Diagnostic molecular pathology : the American journal of surgical pathology, part B
  • [ISO-abbreviation] Diagn. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / EWS-FEV fusion protein, human; 0 / Neoplasm Proteins; 0 / Oncogene Proteins, Fusion
  • [Number-of-references] 45
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14. Zimmermann T, Blütters-Sawatzki R, Flechsenhar K, Padberg WM: Peripheral primitive neuroectodermal tumor: challenge for multimodal treatment. World J Surg; 2001 Nov;25(11):1367-72

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Peripheral primitive neuroectodermal tumor: challenge for multimodal treatment.
  • The primitive neuroectodermal tumor (PNET) is an extremely aggressive soft tissue neoplasm that occurs in children and adolescents.
  • We retrospectively reviewed our therapeutic experience with a multidisciplinary approach, combining surgery, chemotherapy, and radiation therapy.
  • Treatment of PNET was carried out in compliance with the soft tissue protocol (CWS) from the German Society of Pediatric Oncology.
  • Biopsy-proven diagnosis was followed by chemotherapy, which in all cases led to partial remission, allowing excision of the remainder of the tumor without mutilation.
  • After excision, irradiation of the tumor site and two further sequences of chemotherapy were performed.
  • When PNET of the paravertebral region caused symptoms of paralysis and immediate surgery was required, postoperative chemotherapy, a second-look operation, and irradiation were undertaken.
  • Chemotherapy as the first stage is mandatory to avoid a mutilating surgical procedure and intraoperative tumor cell dissemination.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Child. Child, Preschool. Combined Modality Therapy. Female. Humans. Magnetic Resonance Imaging. Male. Radiotherapy, Adjuvant. Retrospective Studies. Second-Look Surgery. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 11760735.001).
  • [ISSN] 0364-2313
  • [Journal-full-title] World journal of surgery
  • [ISO-abbreviation] World J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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15. Blattner JM, Gable P, Quigley MM, McHale MT: Primitive neuroectodermal tumor of the uterus. Gynecol Oncol; 2007 Aug;106(2):419-22
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  • [Title] Primitive neuroectodermal tumor of the uterus.
  • BACKGROUND: : Primitive peripheral neuroectodermal tumors (PNETs) of the uterus are rare.
  • Recent data have demonstrated improved response rates with adjuvant chemotherapy.
  • Intraoperative findings were remarkable for a soft tissue mass in the lower uterine segment.
  • The patient underwent radical surgery, adjuvant chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VAC/IE), and whole pelvic radiation therapy.
  • CONCLUSION: : Only 14 case reports of primitive neuroectodermal tumors of the uterus have been published in the English literature to date.
  • No definitive conclusions concerning the therapeutic management and prognosis have been ascertained.
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / therapy. Uterine Neoplasms / diagnosis. Uterine Neoplasms / therapy
  • [MeSH-minor] Adult. Female. Humans. Pregnancy. Pregnancy Complications, Neoplastic / diagnosis

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  • (PMID = 17537492.001).
  • [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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16. Gostout BS, Lindor NM, DiMarco CS, Peethambaram PP, Clayton AC: Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature. Gynecol Oncol; 2003 Oct;91(1):247-53
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  • [Title] Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature.
  • BACKGROUND: Peripheral primitive neuroectodermal tumor (pPNET) is aggressive and rare, comprising 1% of soft tissue sarcomas.
  • Frozen-section examination at laparotomy revealed small round cell tumor confirmed as pPNET.
  • Chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide with mesna yielded complete response.
  • The patient's mother died of a similar tumor at age 52 years, and the patient's husband had adult Ewing sarcoma, constituting an unusual cluster of related tumors.
  • Surgical resection and multiagent chemotherapy may enhance survival.
  • [MeSH-major] Carcinoma, Small Cell / pathology. Neuroectodermal Tumors, Primitive / pathology. Peritoneal Neoplasms / pathology. Vaginal Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Humans

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  • (PMID = 14529689.001).
  • [ISSN] 0090-8258
  • [Journal-full-title] Gynecologic oncology
  • [ISO-abbreviation] Gynecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 25
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17. Somers GR, Shago M, Zielenska M, Chan HS, Ngan BY: Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report. Pediatr Dev Pathol; 2004 Sep-Oct;7(5):538-45
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report.
  • Primitive neuroectodermal tumor/Ewing sarcoma (PNET/ES) rarely occurs in the skin and subcutaneous tissues.
  • Despite wide local excision and chemotherapy, she rapidly developed cranial bone and brain metastases, followed by lung and skeletal metastases, and died shortly thereafter.
  • The recurrent tumor exhibited light microscopic features of a small, round, blue cell tumor with intracytoplasmic glycogen.
  • Cytogenetic analysis of the relapsed tumor showed a complex karyotype: 47,XX,i(1)(q10), der(4)t(4;19) (q33 approximately q35;q13.1), + 8,t(15;17)(q24;p11.2 approximately p12),der(19)t (19;20)(q13.1;p11.2),der(22)t(20;22)(q13;q13).
  • The clinical behavior and atypical and complex cytogenetic abnormalities exhibited by the tumor in this patient are unusual and represent the most aggressive end of the clinical spectrum of cutaneous and subcutaneous PNET/ES.
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / secondary. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / pathology. Subcutaneous Tissue / pathology
  • [MeSH-minor] Adolescent. Biomarkers, Tumor / analysis. Bone Neoplasms / secondary. Brain Neoplasms / metabolism. Brain Neoplasms / secondary. Chromosome Aberrations. Fatal Outcome. Female. Humans. Immunohistochemistry. Lung Neoplasms / secondary. Microscopy, Electron, Transmission. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 15547779.001).
  • [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] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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18. Heikaus S, Schaefer KL, Eucker J, Hogrebe E, Danebrock R, Wai DH, Krenn V, Gabbert HE, Poremba C: Primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis in a 46-year-old man-differential diagnosis and review of the literature. Hum Pathol; 2009 Jun;40(6):893-7
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  • [Title] Primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis in a 46-year-old man-differential diagnosis and review of the literature.
  • Peripheral primitive neuroectodermal tumor/Ewing's tumors are rare bone and soft tissue malignancies with a highly aggressive clinical course and early metastases occurring at multiple peripheral sites.
  • Here, we present for the first time a case of a 46-year-old man with a primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis.
  • The diagnosis of peripheral primitive neuroectodermal tumor/Ewing's tumor was established by histology, immunohistochemistry, and molecular pathology.
  • The tumor revealed a rapid progress in 2 months' time.
  • Therefore, the patient was included in the EURO-E.W.I.N.G.99 study and was placed on chemotherapy.
  • However, the tumor progressed during ongoing therapy, and the patient died in March 2008.
  • In conclusion, though being reported here for the first time, peripheral primitive neuroectodermal tumor/Ewing's tumors should be considered in the differential diagnosis of blue round cell tumors of the testis.
  • A rapid and correct diagnosis of this entity is crucial for fast and accurate therapy, which is stressed by the fatal case presented here.
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / pathology. Sarcoma, Ewing / pathology. Testicular Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Diagnosis, Differential. Fatal Outcome. Humans. Male. Middle Aged

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  • (PMID = 19269015.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 23
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19. Harder Y, Buechler U, Vögelin E: Primitive neuroectodermal tumor of the thumb metacarpal bone: a case report and literature review. J Hand Surg Am; 2003 Mar;28(2):346-52
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  • [Title] Primitive neuroectodermal tumor of the thumb metacarpal bone: a case report and literature review.
  • A 27-year-old otherwise healthy patient was diagnosed with a primitive neuroectodermal tumor of the thumb metacarpal bone of the left hand.
  • Based on a common chromosomal translocation this tumor shows a close relationship to Ewing's sarcoma.
  • The treatment consisted of neo- and adjuvant chemotherapy and marginal resection of the affected thumb metacarpal bone including periosseous soft tissue and reconstruction of the thumb by an intercalated segmental index pollicization.
  • [MeSH-major] Bone Neoplasms / surgery. Metacarpus / pathology. Neuroectodermal Tumors, Primitive, Peripheral / surgery. Thumb / pathology. Thumb / surgery

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  • (PMID = 12671870.001).
  • [ISSN] 0363-5023
  • [Journal-full-title] The Journal of hand surgery
  • [ISO-abbreviation] J Hand Surg Am
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 31
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20. Seddon BM, Whelan JS: Emerging chemotherapeutic strategies and the role of treatment stratification in Ewing sarcoma. Paediatr Drugs; 2008;10(2):93-105
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  • [Title] Emerging chemotherapeutic strategies and the role of treatment stratification in Ewing sarcoma.
  • It comprises Ewing sarcoma arising from bone and extraosseous Ewing sarcoma arising from soft tissues (which includes peripheral neuroectodermal tumors and Askin tumor arising from the chest wall).
  • Ewing sarcoma is treated successfully in many cases by a combination of chemotherapy, surgery, and radiotherapy.
  • A number of prognostic factors have been identified that can be used to stratify patients according to the risk of relapse, allowing optimization of treatment.
  • These can be categorized as tumor-related factors (presence of metastases, tumor site, volume, lactic dehydrogenase level, chromosomal translocation type, presence of fusion transcripts in blood and bone marrow), treatment-related factors (local therapy, histologic response to chemotherapy, radiologic response to chemotherapy, chemotherapy regimen), and patient-related factors (gender, age).
  • [MeSH-minor] Age Factors. Antineoplastic Protocols. Combined Modality Therapy. Humans. Pediatrics. Prognosis. Protein Kinases / physiology. Receptor, IGF Type 1 / physiology. Signal Transduction. TOR Serine-Threonine Kinases

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  • (PMID = 18345719.001).
  • [ISSN] 1174-5878
  • [Journal-full-title] Paediatric drugs
  • [ISO-abbreviation] Paediatr Drugs
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.10.1 / Receptor, IGF Type 1
  • [Number-of-references] 126
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21. Lowichik A, Zhou H, Pysher TJ, Smith L, Lemons R, Coffin CM: Therapy associated changes in childhood tumors. Adv Anat Pathol; 2000 Nov;7(6):341-59
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Therapy associated changes in childhood tumors.
  • Contemporary treatment regimens for the common solid tumors of childhood have led to increased numbers of post-treatment pathologic specimens from survivors.
  • Current therapeutic strategies for childhood cancers in North America require an accurate pathologic diagnosis and stratify patients based on combinations of clinical, biological, and pathologic features.
  • In several tumor systems, the pathologic response to therapy also modifies the treatment regimen.
  • Accurate pathologic interpretation of such specimens is critical in providing useful prognostic information for therapeutic decisions.
  • Standardized handling of post-therapy pathologic specimens, appropriate use of molecular and genetic studies, consideration of the differential diagnoses, and assessment of the potential biologic significance of therapy-induced pathologic changes are, therefore, critical for patient management and determination of treatment protocols.
  • [MeSH-minor] Adolescent. Bone Neoplasms / drug therapy. Bone Neoplasms / pathology. Bone Neoplasms / surgery. Child. Child, Preschool. Fibrosarcoma / pathology. Fibrosarcoma / therapy. Hepatoblastoma / pathology. Hepatoblastoma / therapy. Histocytochemistry. Humans. Kidney Neoplasms / drug therapy. Kidney Neoplasms / pathology. Kidney Neoplasms / surgery. Liver Neoplasms / pathology. Liver Neoplasms / therapy. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Osteosarcoma / pathology. Osteosarcoma / surgery. Prognosis. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / surgery. Sarcoma, Ewing / drug therapy. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / drug therapy. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / surgery. Wilms Tumor / drug therapy. Wilms Tumor / pathology. Wilms Tumor / surgery

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  • (PMID = 11078058.001).
  • [ISSN] 1072-4109
  • [Journal-full-title] Advances in anatomic pathology
  • [ISO-abbreviation] Adv Anat Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] UNITED STATES
  • [Number-of-references] 149
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22. Alaggio R, Ninfo V, Rosolen A, Coffin CM: Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases. Am J Surg Pathol; 2006 Mar;30(3):388-94

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  • [Title] Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases.
  • Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma.
  • In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis.
  • PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass.
  • Grossly, the tumors were nonencapsulated and had a multinodular appearance with focal infiltrative growth, a white fleshy cut surface, and a tumor diameter ranging from 2 to 15 cm.
  • Histologically, a diffuse growth of primitive spindle, polygonal, and round cells occurred in a myxoid background.
  • The tumor cells were arranged in a vaguely nodular pattern with peripheral collagenized stroma, higher cellularity at the periphery, and a delicate vascular network in the background.
  • One tumor had a complex karyotypic abnormality with rearrangements involving chromosomes Y, 9, and 3.
  • Three patients had recurrences or metastasis treated with a combination of surgery and chemotherapy.
  • One patient is alive with persistent locally aggressive disease, 2 are alive with no evidence of recurrence, 1 had a recurrence treated surgically without further follow-up information, 1 patient died with persistent tumor and sepsis 6 weeks after diagnosis, and 1 patient was lost to follow-up.
  • The morphologic appearance combined with the ultrastructural features and absence of the typical gene rearrangement of congenital-infantile fibrosarcoma are unique, and we propose that PMMTI represents a new category of pediatric fibroblastic-myofibroblastic tumor.
  • [MeSH-major] Fibrosarcoma / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Dermatofibrosarcoma / pathology. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Infant. Infant, Newborn. Male. Microscopy, Electron, Transmission. Neoplasm Recurrence, Local / pathology. Nerve Sheath Neoplasms / pathology. Oncogene Proteins, Fusion / genetics. Reverse Transcriptase Polymerase Chain Reaction

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  • (PMID = 16538060.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / ETV6-NTRK3 fusion protein, human; 0 / Oncogene Proteins, Fusion
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23. Bisogno G, Riccardi R, Ruggiero A, Arcamone G, Prete A, Surico G, Provenzi M, Bertolini P, Paolucci P, Carli M: Phase II study of a protracted irinotecan schedule in children with refractory or recurrent soft tissue sarcoma. Cancer; 2006 Feb 1;106(3):703-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Phase II study of a protracted irinotecan schedule in children with refractory or recurrent soft tissue sarcoma.
  • The Italian Soft Tissue Sarcoma (STS) Committee performed a multiinstitutional Phase II study to evaluate its effect on STS.
  • Thirty patients, 13 with peripheral primitive neuroectodermal tumor (PNET), 12 with rhabdomyosarcoma (RMS), 3 with desmoplastic small round cell tumor (DSRCT), and 2 with other STS were evaluable for response.
  • CONCLUSIONS: As a single agent in the treatment of recurrent and refractory STS, irinotecan administered on a daily x5 x2 schedule revealed a noteworthy response rate in a population of heavily pretreated patients, especially in the subset of patients with PNET.
  • [MeSH-major] Antineoplastic Agents, Phytogenic / administration & dosage. Camptothecin / analogs & derivatives. Sarcoma / drug therapy. Soft Tissue Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Disease Progression. Drug Administration Schedule. Female. Humans. Infant. Infusions, Intravenous. Male. Neutropenia / chemically induced. Treatment Outcome

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  • [Copyright] Copyright (c) 2005 American Cancer Society.
  • (PMID = 16369989.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 7673326042 / irinotecan; XT3Z54Z28A / Camptothecin
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24. Terenziani M, D'Angelo P, Bisogno G, Boldrini R, Cecchetto G, Collini P, Conte M, De Laurentis T, Ilari I, Indolfi P, Inserra A, Piva L, Siracusa F, Spreafico F, Tamaro P, Lo Curto M: Teratoma with a malignant somatic component in pediatric patients: the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience. Pediatr Blood Cancer; 2010 Apr;54(4):532-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PROCEDURE: The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC.
  • RESULTS: The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1).
  • Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosarcoma plus neuroectodermal sarcoma (1), malignant peripheral nerve sheath tumor (1).
  • The pediatric disease appears to be more heterogeneous in tumor site distribution and MSC histology than in adults.
  • Chemotherapy optimized for histology should include reagents directed to the somatic malignancy, if chemosensitive.
  • Malignant GCT warrants GCT-directed chemotherapy.
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Infant, Newborn. Italy. Male. Neoplasm Staging. Prognosis. Retrospective Studies. Treatment Outcome

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  • (PMID = 20049928.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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25. Sturla LM, Westwood G, Selby PJ, Lewis IJ, Burchill SA: Induction of cell death by basic fibroblast growth factor in Ewing's sarcoma. Cancer Res; 2000 Nov 1;60(21):6160-70
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Ewing's sarcoma is thought to arise after developmental arrest of primitive neural cells during embryogenesis.
  • All four of the Ewing's sarcoma cell lines examined expressed bFGF and FGF receptors, which were detected by immunofluorescence and Western blotting. bFGF-induced a significant dose-dependent decrease in Ewing's sarcoma cell proliferation on plastic and reduced anchorage-independent growth in soft agar.
  • Treatment of NuNu mice with bFGF decreased growth of the highly tumorigenic Ewing's sarcoma cell lines.
  • This novel observation may provide a new therapeutic strategy for Ewing's sarcomas.
  • [MeSH-major] Bone Neoplasms / pathology. Fibroblast Growth Factor 2 / pharmacology. Sarcoma, Ewing / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Animals. Apoptosis / drug effects. Cell Count. Cell Death / drug effects. Cell Differentiation / drug effects. Cell Division / drug effects. Cell Survival / drug effects. Female. Humans. Mice. Mice, Nude. Necrosis. Nerve Growth Factor / pharmacology. Neuroectodermal Tumors, Primitive, Peripheral / drug therapy. Neuroectodermal Tumors, Primitive, Peripheral / metabolism. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Receptors, Fibroblast Growth Factor / biosynthesis. Tumor Cells, Cultured. Xenograft Model Antitumor Assays

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  • (PMID = 11085540.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] UNITED STATES
  • [Chemical-registry-number] 0 / Receptors, Fibroblast Growth Factor; 103107-01-3 / Fibroblast Growth Factor 2; 9061-61-4 / Nerve Growth Factor
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