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1. Shiono S, Abiko M, Tamura G, Sato T: Malignant solitary fibrous tumor with superior vena cava syndrome. Gen Thorac Cardiovasc Surg; 2009 Jun;57(6):321-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Malignant solitary fibrous tumor with superior vena cava syndrome.
  • Mediastinal solitary fibrous tumors, particularly those that are malignant, are rare.
  • We report a case of malignant solitary fibrous tumor with superior vena cava syndrome and highly aggressive behavior.
  • Postoperative radiotherapy was performed due to incomplete resection, but the tumor relapsed within 4 months.
  • Gemcitabine-based chemotherapy was ineffective.
  • [MeSH-major] Mediastinal Neoplasms / complications. Solitary Fibrous Tumors / complications. Superior Vena Cava Syndrome / etiology
  • [MeSH-minor] Adult. Deoxycytidine / analogs & derivatives. Deoxycytidine / therapeutic use. Fatal Outcome. Female. Humans. Lung Neoplasms / drug therapy. Lung Neoplasms / secondary. Neoplasm Invasiveness. Pleural Neoplasms / drug therapy. Pleural Neoplasms / secondary. Radiotherapy, Adjuvant. Sternum / surgery. Tomography, X-Ray Computed. Treatment Outcome. Vascular Surgical Procedures

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  • [Cites] Chest. 2007 Mar;131(3):904-908 [17356113.001]
  • [Cites] Am J Surg Pathol. 1989 Aug;13(8):640-58 [2665534.001]
  • [Cites] Am J Surg Pathol. 1989 Jul;13(7):547-57 [2735490.001]
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  • (PMID = 19533281.001).
  • [ISSN] 1863-6705
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine
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2. Cecchetto G, Alaggio R, Dall'Igna P, Bisogno G, Ferrari A, Gigante C, Casanova M, Sotti G, Zanetti I, Carli M: Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies. Cancer; 2005 Nov 1;104(9):2006-12
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  • [Title] Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.
  • BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity.
  • The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period.
  • Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy.
  • Aggressive delayed surgery of residual disease, including compartmental resections, was recommended.
  • Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5.
  • Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1.
  • Nineteen of 21 patients with CTs-NRSTS were alive without disease: the 5-year overall survival (OS) and progression-free survival (PFS) were 94.4% and 79.3%, respectively; 23 of 31 patients with CTns-NRSTS were alive without disease: 5-year OS and PFS were 75.3% and 68.3%, respectively.
  • Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors.
  • Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor.
  • CONCLUSIONS: Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS.
  • Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread.
  • [MeSH-major] Extremities. Sarcoma / diagnosis
  • [MeSH-minor] Adolescent. Age Factors. Chemotherapy, Adjuvant. Child. Female. Humans. Italy. Male. Neoadjuvant Therapy. Survival Analysis

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  • [Copyright] (c) 2005 American Cancer Society.
  • (PMID = 16161038.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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3. Schuppert F, Berger D, Peters H, Schröder S, Schöfl C, Tischler J, Hiller WF, von zur Mühlen A: [A young woman with neurofibromatosis 1 (Recklinghausen disease), abdominal tumor and hypertension]. Dtsch Med Wochenschr; 2000 Nov 17;125(46):1390-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [A young woman with neurofibromatosis 1 (Recklinghausen disease), abdominal tumor and hypertension].
  • [Transliterated title] Eine junge Patientin mit Neurofibromatose Typ 1 (Morbus Recklinghausen), Unterbauchtumor und Hypertonus.
  • HISTORY AND ADMISSION FINDINGS: A 38-year-old woman, known to have type 1 neurofibromatosis (NF1; von Recklinghausen's disease) and recurrence of a malignant haemangiopericytoma in the lower abdomen developed hypertension.
  • Physical examination revealed tachycardia and paleness of the distal digits, in addition to multiple neurofibromas and café-au-lait spots.
  • INVESTIGATIONS: A tumour was found in the region of the right adrenal gland, in addition to the known haemangiopericytoma.
  • TREATMENT AND COURSE: Because of the extensive local changes the recurrent haemangiopericytoma was only partially resected.
  • At the same time a right adrenalectomy was performed without complication.
  • Instead she was given weekly palliative chemotherapy with adriamycin, with little improvement.
  • It is likely that the patient's emphysema was associated with the NF1, while the haemangiopericytoma was presumably unconnected with the NF1.
  • [MeSH-major] Abdominal Neoplasms / surgery. Hemangiopericytoma / surgery. Hypertension / complications. Neoplasm Recurrence, Local / surgery. Neoplasms, Second Primary / diagnosis. Neurofibromatosis 1 / diagnosis
  • [MeSH-minor] Adrenal Gland Neoplasms / diagnosis. Adult. Antineoplastic Agents / therapeutic use. Doxorubicin / therapeutic use. Exons. Fatal Outcome. Female. Humans. Nerve Tissue Proteins / genetics. Neurofibromin 1. Palliative Care. Pheochromocytoma / diagnosis. Skin Neoplasms / diagnosis. Tachycardia

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  • (PMID = 11129996.001).
  • [ISSN] 0012-0472
  • [Journal-full-title] Deutsche medizinische Wochenschrift (1946)
  • [ISO-abbreviation] Dtsch. Med. Wochenschr.
  • [Language] ger
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Nerve Tissue Proteins; 0 / Neurofibromin 1; 80168379AG / Doxorubicin
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4. Koch M, Nielsen GP, Yoon SS: Malignant tumors of blood vessels: angiosarcomas, hemangioendotheliomas, and hemangioperictyomas. J Surg Oncol; 2008 Mar 15;97(4):321-9
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  • [Title] Malignant tumors of blood vessels: angiosarcomas, hemangioendotheliomas, and hemangioperictyomas.
  • Sarcomas that arise from or resemble the components of blood vessels are uncommon and include angiosarcomas, hemangioendotheliomas, and hemangiopericytomas.
  • This article reviews the management of these three types of sarcomas.
  • Diagnosis can sometimes be difficult, with the diagnosis of hemangiopericytoma versus solitary fibrous tumor currently in debate.
  • The mainstay of treatment is surgical resection, sometimes combined with radiation therapy.
  • Adjuvant chemotherapy is unproven but can be considered.
  • For patients with advanced disease, various chemotherapeutic regimens may result in meaningful responses in a minority of patients.
  • [MeSH-major] Hemangioendothelioma / diagnosis. Hemangioendothelioma / therapy. Hemangiopericytoma / diagnosis. Hemangiopericytoma / therapy. Hemangiosarcoma / diagnosis. Hemangiosarcoma / therapy
  • [MeSH-minor] Chemotherapy, Adjuvant. Combined Modality Therapy. Humans. Neoplasms, Radiation-Induced. Prognosis. Radiotherapy / adverse effects. Skin Neoplasms / diagnosis. Skin Neoplasms / etiology. Skin Neoplasms / therapy

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18286475.001).
  • [ISSN] 0022-4790
  • [Journal-full-title] Journal of surgical oncology
  • [ISO-abbreviation] J Surg Oncol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 89
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5. Carvalho JR, Haddad L, Leonhardt FD, Marques Filho MF, Santos Rde O, Cervantes O, Abrahão M: Head and neck hemangiopericytoma in a child: case report. Sao Paulo Med J; 2004 Sep 2;122(5):223-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Head and neck hemangiopericytoma in a child: case report.
  • CONTEXT: Hemangiopericytoma is a relatively rare tumor, first described in 1942, with approximately 300 cases described in the literature to date.
  • We describe a case of malignant head and neck hemangiopericytoma in a child.
  • TYPE OF STUDY: Case report.
  • CASE REPORT: A twelve-year-old male patient noted the presence of a firm painless right-side retroauricular lymph node of 1 cm in diameter, which at first remained unchanged for six months, but subsequently enlarged progressively.
  • In November 2000, he presented nasal obstruction and voluminous epistaxis that required hospitalization and blood transfusion.
  • During dental treatment one month later, a cranial x-ray revealed bone alterations.
  • A subsequent computed tomography scan showed an extensive lesion of soft tissue density that had invaded the maxillary fossa, eroding the skull base and middle and nasal fossa.
  • The child was then referred to our service, where biopsy was performed, giving a diagnosis of hemangiopericytoma.
  • The patient was submitted to conservative surgery in April 2001, with only partial resection of the tumor because of its extent.
  • Histopathological examination of the specimen confirmed the presence of malignant hemangiopericytoma.
  • Following the surgery, the patient presented fast regrowth of the lesion, with partial response to chemotherapy and radiotherapy.
  • [MeSH-major] Head and Neck Neoplasms / diagnosis. Hemangiopericytoma / diagnosis
  • [MeSH-minor] Adult. Angiography. Child. Humans. Magnetic Resonance Imaging. Male. Tomography, X-Ray Computed

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  • (PMID = 15558145.001).
  • [ISSN] 1516-3180
  • [Journal-full-title] São Paulo medical journal = Revista paulista de medicina
  • [ISO-abbreviation] Sao Paulo Med J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Brazil
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6. Saynak M, Bayir-Angin G, Kocak Z, Oz-Puyan F, Hayar M, Cosar-Alas R, Karamustafaoglu A, Yurut-Caloglu V, Caloglu M, Yoruk Y: Recurrent solitary fibrous tumor of the pleura: significant response to radiotherapy. Med Oncol; 2010 Mar;27(1):45-8
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  • [Title] Recurrent solitary fibrous tumor of the pleura: significant response to radiotherapy.
  • Solitary fibrous tumor (SFT) of the pleura is an uncommon neoplasm with non-specific symptoms and non-pathognomonical radiological findings.
  • Surgery allows establishment of a definitive diagnosis as well as a cure of the disease.
  • The role of radiotherapy or chemotherapy in the management of the disease is unclear because of the rarity of the disease and the successful results of the surgical treatment.
  • Long-term clinical follow-up may be useful for the patients with SFT because of the potential adverse biological behavior of this tumor, which may lead to repeated recurrences and/or malignant transformation.
  • We reported a 66-year-old woman with recurrence of SFT in the right lung, which had significant response to external thoracic radiotherapy.
  • [MeSH-major] Neoplasm Recurrence, Local / radiotherapy. Solitary Fibrous Tumor, Pleural / radiotherapy
  • [MeSH-minor] Aged. Dyspnea / etiology. Female. Humans. Immunohistochemistry. Palliative Care. Tomography, X-Ray Computed. Treatment Outcome

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  • [Cites] Am J Surg Pathol. 1989 Aug;13(8):640-58 [2665534.001]
  • [Cites] J BUON. 2008 Jan-Mar;13(1):117-21 [18404798.001]
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  • (PMID = 19165637.001).
  • [ISSN] 1559-131X
  • [Journal-full-title] Medical oncology (Northwood, London, England)
  • [ISO-abbreviation] Med. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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7. Bien E, Kazanowska B, Dantonello T, Adamkiewicz-Drozynska E, Balcerska A, Madziara W, Rybczynska A, Nurzynska-Flak J, Solarz E, Kurylak A, Zalewska-Szewczyk B, Krawczyk M, Izycka-Swieszewska E, Rapala M, Koscielniak E: Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature. Ann Surg Oncol; 2010 Jul;17(7):1878-89
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature.
  • BACKGROUND: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival.
  • METHODS: A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols.
  • RESULTS: Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival.
  • Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis.
  • Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses.
  • Response to systemic treatment was poor (44%) and did not prevent local and distant relapses.
  • CONCLUSIONS: The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS.
  • RTX and delayed surgery should be performed more frequently and earlier in the disease course.
  • An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy.
  • The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.
  • [MeSH-major] Hemangioendothelioma / mortality. Hemangiopericytoma / mortality. Hemangiosarcoma / mortality. Sarcoma / mortality. Vascular Neoplasms / mortality

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  • (PMID = 20333551.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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8. Kozuka T, Kiura K, Katayama H, Fujii N, Ishimaru F, Ikeda K, Ueoka H, Hamasaki S, Yoshino T, Kashihara Y, Date H, Tanimoto M, Harada M: Tandem high-dose chemotherapy supported by autologous peripheral blood stem cell transplantation for recurrent soft tissue sarcoma. Anticancer Res; 2002 Sep-Oct;22(5):2939-44
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Tandem high-dose chemotherapy supported by autologous peripheral blood stem cell transplantation for recurrent soft tissue sarcoma.
  • BACKGROUND: Patients with recurrent soft tissue sarcoma (STS) are seldom curable, with 5-year survival rates of less than 10% in all large series.
  • The role of high-dose chemotherapy (HDC) with hematopoietic stem cell support in this disease has not been established.
  • CASE REPORT: We report on two patients with recurrent STS who were treated with tandem HDC supported by autologous peripheral blood stem cell transplantation (PBSCT).
  • One patient with malignant fibrous histiocytoma recurred with multiple lung metastases.
  • This patient achieved a partial response after two cycles of induction chemotherapy consisting of ifosfamide and epirubicin.
  • During four cycles of induction chemotherapy, peripheral blood stem cells (PBSCs) were harvested.
  • Tandem high-dose ICE regimen (ifosfamide 3 g/m2 on days-7 to -3, carboplatin 400 mg/m2 on days-7, -5 and 3, etoposide 500 mg/m2 on days-7, -5 and 3) supported by autologous PBSCT gave rise to further regression of the tumors.
  • Another patient with malignant hemangiopericytoma was treated by tandem high-dose ICE regimen supported by autologous PBSCT after the 3rd removal of abdominal tumors.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Hematopoietic Stem Cell Transplantation. Neoplasm Recurrence, Local / therapy. Sarcoma / therapy
  • [MeSH-minor] Adult. Carboplatin / administration & dosage. Dose-Response Relationship, Drug. Etoposide / administration & dosage. Histiocytoma, Benign Fibrous / drug therapy. Histiocytoma, Benign Fibrous / therapy. Humans. Ifosfamide / administration & dosage. Male. Retroperitoneal Neoplasms / drug therapy. Retroperitoneal Neoplasms / therapy

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  • (PMID = 12530021.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 6PLQ3CP4P3 / Etoposide; BG3F62OND5 / Carboplatin; UM20QQM95Y / Ifosfamide; ICE protocol 3
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9. Turowski B, Zanella FE: Interventional neuroradiology of the head and neck. Neuroimaging Clin N Am; 2003 Aug;13(3):619-45
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Vascular interventions are important and helpful for treatment of various pathologies of the head and neck.
  • Interventional neuroradiology of the head and neck includes image-guided biopsies, vessel occlusion, and local chemotherapy.
  • Knowledge of anatomy, functional relationships between intra- and extracranial vessels, and pathology are the basis for therapeutic success.
  • Neuroradiologic imaging, especially CT and MR imaging, and appropriate analysis of angiographic findings help ensure indication for treatment and plan an intervention.
  • Indications for image-guided biopsies are preverterbal fluid-collections, spinal and paraspinal inflammations and abscesses, deep cervical malignancies, vertebral body, and skull base tumors.
  • Indications for vessel occlusion are emergency situations to stop bleeding in vascular lesions (traumatic, malformation, or tumors) by reduction of pressure, preoperative reduction of blood flow to minimize the surgical risk, palliative occlusion of feeding vessels to produce tumor necrosis, or potential curative (or presurgical) occlusion of vascular malformations.
  • Pressure reduction to support normal coagulation, such as epistaxis, in hereditary hemorrhagic telangiectasia can be achieved by proximal vessel occlusion with large particles or platinum coils.
  • Examples of these interventions are: a hemangioma of the hard palate, a juvenile angiofibroma, a hemangiopericytoma, a malignant meningioma, a malignant fibrous histiocytoma, and a glomus tumor.
  • Effective treatment of vascular malformations, such as AV fistulas or angiomas, needs exact occlusion of the fistula or the angiomatous nidus, which is demonstrated in the case of an AV angioma of the base of the tongue.
  • Chemotherapy with local intra-arterial cisplatin combined with intravenous administration of sodium thiosulfate as antidote is indicated as an adjuvant modality in a multimodal regimen of oropharyngeal squamous cell carcinoma or as palliative treatment of recurrent and otherwise untreatable malignant tumors of the head and neck.
  • Palliative treatment of a bleeding oropharyngeal cancer is another example of interventional treatment.
  • Selective treatment, either occluding or pharmacologic, may be preoperative, palliative, or curative.
  • The objective is reduction of surgical risk, improvement of quality of life, or curative therapy of a lesion.
  • Thus, the interventional treatment should not be associated with morbidity or mortality.
  • Sometimes collateral lesions are unavoidable.
  • The benefits, risks, and expected damages of neuroradiologic interventions must be balanced during the informed consent procedure with the patient.
  • [MeSH-major] Head and Neck Neoplasms / radiography. Head and Neck Neoplasms / therapy. Neuroradiography. Radiology, Interventional

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  • (PMID = 14631695.001).
  • [ISSN] 1052-5149
  • [Journal-full-title] Neuroimaging clinics of North America
  • [ISO-abbreviation] Neuroimaging Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 40
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10. Schwab R, Schneider C, Junge K, Stumpf M, Becker HP, Schumpelick V: [Large solitary fibrous tumors of the pleura as incidental finding. 2 case reports of a rare tumor entity]. Chirurg; 2004 Feb;75(2):200-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Large solitary fibrous tumors of the pleura as incidental finding. 2 case reports of a rare tumor entity].
  • [Transliterated title] Grosse solitäre fibröse Tumoren der Pleura als Zufallsbefund. Zwei Kasuistiken zu einer Tumorrarität.
  • The solitary fibrous tumor (SFT) is a very rare and usually benign neoplasm.
  • This tumor is mostly located in the thoracic cavity, but it has also been reported in numerous sites including liver, skin, and meninges.
  • Due to its rarity diagnosis is often difficult.
  • Solitary fibrous tumors consistently express CD 34 and react negatively to epithelial markers.
  • The treatment of choice for solitary fibrous tumors is extensive surgical resection.
  • Up to now there is no evidence that radiation and chemotherapy are effective.
  • One-half of the patients with malignant tumors can be cured; the rest develop recurrences and metastases more often.
  • We report two cases of a 65- and a 75-year-old male patient with huge intrathoracic solitary fibrous subpleural tumors, discovered incidentally on routine chest X-ray.
  • [MeSH-major] Incidental Findings. Neoplasms, Fibrous Tissue / surgery. Pleural Neoplasms / surgery
  • [MeSH-minor] Aged. Biomarkers, Tumor / analysis. Diagnosis, Differential. Humans. Male. Pleura / pathology. Pleura / surgery. Tomography, X-Ray Computed

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  • (PMID = 14991184.001).
  • [ISSN] 0009-4722
  • [Journal-full-title] Der Chirurg; Zeitschrift fur alle Gebiete der operativen Medizen
  • [ISO-abbreviation] Chirurg
  • [Language] ger
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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11. López Almaraz R, Villafruela Alvarez C, Rodríguez Luis J, Doménech Martínez E: [Neonatal neoplasms: a single-centre experience]. An Pediatr (Barc); 2006 Dec;65(6):529-35
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  • [Transliterated title] Neoplasias neonatales: experiencia de un centro.
  • INTRODUCTION: Malignant tumors are uncommon in the neonatal period and benign tumors may have malignant potential.
  • OBJECTIVES: To describe the neoplasms diagnosed and treated in newborns (</= 28 days of life) in the Hospital Universitario de Canarias and their association with congenital abnormalities and to evaluate prenatal diagnosis of these tumors.
  • The variables analyzed were the percentage of neonatal neoplasms among the total number of cancer cases in children aged less than 14 years, their incidence among all the newborns in our hospital, sex, year of diagnosis, age at clinical diagnosis, the presence or absence of prenatal diagnosis, type of tumor (histologic diagnosis), association with syndromes or other congenital anomalies, treatment, and long-term outcome.
  • Males accounted for 43.8 % and females for 56.2 %, with a mean age at diagnosis of 5.5 days (range 1-28 days).
  • Five neonates (31.2 %) had a prenatal diagnosis, 60 % of which were made in the last 7 years of the study period.
  • Histologic diagnoses were neuroblastoma (n = 5; 31.2 %), teratoma/ germ cell tumor (n = 4; 25 %), soft tissue sarcoma (one fibrosarcoma of the thigh and two hemangiopericytoma of the back and heart; 18.8 %), and one case each of mesoblastic nephroma, cerebral tumor (ependymoblastoma), melanoma (associated with giant congenital melanocytic nevi), and acute leukemia (associated with Down syndrome).
  • Treatment consisted of surgery alone (n = 10; 62.5 %) and surgery plus chemotherapy (n = 5; 31.2 %); one patient received no treatment.
  • CONCLUSIONS: The neoplasms most frequently diagnosed in the neonatal period were solid tumors, mainly neuroblastoma and teratomas/germ cell tumors; 12.5 % were associated with syndromes or congenital anomalies.
  • In the last 7 years, the prenatal diagnosis of these entities has improved.
  • Most of the neoplasms responded to therapy, mainly surgery, and long-term outcome was favorable.

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  • [CommentIn] An Pediatr (Barc). 2007 Jul;67(1):85-6 [17663916.001]
  • (PMID = 17194321.001).
  • [ISSN] 1695-4033
  • [Journal-full-title] Anales de pediatría (Barcelona, Spain : 2003)
  • [ISO-abbreviation] An Pediatr (Barc)
  • [Language] spa
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Spain
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12. Vennarecci G, Ettorre GM, Giovannelli L, Del Nonno F, Perracchio L, Visca P, Corazza V, Vidiri A, Visco G, Santoro E: Solitary fibrous tumor of the liver. J Hepatobiliary Pancreat Surg; 2005;12(4):341-4
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  • [Title] Solitary fibrous tumor of the liver.
  • We report a new case of benign solitary fibrous tumor (SFT) of the liver.
  • On examination abdominal distension was present and palpation showed a large firm mass in the right hypochondrium and epigastrium.
  • The patient's past medical history was not significant and laboratory tests were normal.
  • Ultrasonography and computed tomography showed a large tumor, 20 cm in diameter, in the right lobe of the liver.
  • The tumor measured 30 x 28 x 14 cm and weighed 4725 g.
  • Microscopic evaluation showed a benign SFT of the liver with tumor cells typically positive for vimentin and CD34.
  • This is a rare neoplasm of mesenchymal origin that occasionally involves the liver in adult patients.
  • Most SFTs are benign, but some may have malignant histological features and recur locally or metastasize.
  • Because of their rarity, overall experience has not been significant and little has been published concerning this tumor, Including the present one, 28 cases have been reported in the English literature.
  • Surgery is the mainstay of treatment.
  • As SFT of the liver is often a benign neoplasm, chemotherapy or radiotherapy should not be necessary, and should be reserved for when resection is incomplete and/or histological examination reveals features of malignancy.
  • Surgeons must be aware of SFT of the liver, and this neoplasm should be included in the differential diagnosis of a single large hepatic mass.
  • [MeSH-major] Liver Neoplasms / pathology. Neoplasms, Fibrous Tissue / pathology

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  • (PMID = 16133706.001).
  • [ISSN] 0944-1166
  • [Journal-full-title] Journal of hepato-biliary-pancreatic surgery
  • [ISO-abbreviation] J Hepatobiliary Pancreat Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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13. Fatusi OA, Owotade FJ, Ojo OS: Maxillary haemangiopericytoma: a case report. West Afr J Med; 2004 Apr-Jun;23(2):180-4
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  • [Title] Maxillary haemangiopericytoma: a case report.
  • BACKGROUND: Haemangiopericytoma is a very rare slow-growing vascular tumour with a variable malignant potential, constituting less than 1% of all neoplasms.
  • It may arise from any blood vessel and in any organ of the body.
  • Primary haemangiopericytoma of bone is even rarer, constituting about 0.1% of bone tumours.
  • The tumour is extremely rare in Africans and particularly in the head and neck region.
  • STUDY DESIGN: We describe the case of a 66-year old Nigerian with haemangiopericytoma of the maxilla, who presented with a recurrent but painless jaw mass.
  • RESULTS: Surgical resection of this tumour is potentially bedevilled with the risk of torrential haemorrhage and high rate of recurrence.
  • This risk may be substantially reduced by wide surgical resection with a careful microscopical examination of the resection margins and the institution of adjuvant radiotherapy in incompletely resected tumours.
  • Chemotherapy has no known role in the management of haemangiopericytoma.
  • Postoperative radiation therapy appears to be effective against tumour recurrence.
  • [MeSH-major] Hemangiopericytoma / diagnosis. Maxillary Neoplasms / diagnosis. Neoplasm Recurrence, Local / diagnosis
  • [MeSH-minor] Aged. Biopsy. Fatal Outcome. Female. Hospitals, University. Humans. Nigeria / epidemiology. Precipitating Factors. Prognosis. Radiotherapy, Adjuvant. Rare Diseases / diagnosis. Rare Diseases / epidemiology. Rare Diseases / surgery

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  • (PMID = 15287302.001).
  • [ISSN] 0189-160X
  • [Journal-full-title] West African journal of medicine
  • [ISO-abbreviation] West Afr J Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Nigeria
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14. Ruhland B, Dittmer C, Thill M, Diedrich K, Fischer D: Metastasized hemangiopericytoma of the breast: a rare case. Arch Gynecol Obstet; 2009 Sep;280(3):491-4
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  • [Title] Metastasized hemangiopericytoma of the breast: a rare case.
  • Liposarcoma, leiomyosarcoma, rhabdomyosarcoma, as well as hemangiopericytoma, are part of the soft tissue sarcoma group.
  • Little is known about the progress, prognosis and dissemination of this infrequent tumor entity.
  • We present the case of a woman, who received primary diagnosis of a malignant hemangiopericytoma of the left breast.
  • She underwent a mastectomy with an axillary lymph node sampling (stage pT3 pN0 cM0), as adjuvant therapy was not mandatory.
  • Eight months after diagnosis, the patient presented with lumbar back pain, gluteal pain and right accentuated adynamia in both legs because of a disseminated osseous metastasis.
  • Diagnostic investigation presented a cerebral, pulmonary, cutaneous, hepatic and pleural metastatic disease.
  • Two months after initiation of chemotherapy the patient died.
  • Diagnostic criteria and treatment principles in the metastatic situation are presented in addition to the literature to give a review about this rare malignancy.
  • [MeSH-major] Bone Neoplasms / secondary. Breast Neoplasms / pathology. Hemangiopericytoma / secondary. Hemangiopericytoma / therapy
  • [MeSH-minor] Aged. Axilla. Fatal Outcome. Female. Humans. Lymph Node Excision. Lymphatic Metastasis. Mastectomy. Neoplasm Metastasis

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  • (PMID = 19169699.001).
  • [ISSN] 1432-0711
  • [Journal-full-title] Archives of gynecology and obstetrics
  • [ISO-abbreviation] Arch. Gynecol. Obstet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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15. Brega Massone PP, Lequaglie C, Conti B, Ferro F, Magnani B, Cataldo I: A particular case with long-term follow-up of rare malignant hemangiopericytoma of the lung with metachronous diaphragmatic metastasis. Thorac Cardiovasc Surg; 2002 Jun;50(3):178-80
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  • [Title] A particular case with long-term follow-up of rare malignant hemangiopericytoma of the lung with metachronous diaphragmatic metastasis.
  • The authors report the case of a woman with a primary hemangiopericytoma of the lung and successive metachronous diaphragmatic metastasis treated with surgery only according to the patient's decision.
  • The patient was first submitted to left lower lobectomy with resection of diaphragm and three ribs en-bloc for pulmonary sarcoma.
  • One year later, a chest computed tomography (CT) scan showed a small lesion above the diaphragm on the left side.
  • She refused the proposed operation and returned fifteen months later with a new thorax CT, and told us that she had in the meantime become pregnant and given birth.
  • A rethoracotomy to remove the metastatic diaphragmatic tumor was performed.
  • Chemotherapy with anthracycline and iphosphamide was proposed, but the patient again refused therapy for fear of side effects impairing another pregnancy.
  • Currently, the patient is alive and disease-free, 68 months after the first treatment.
  • [MeSH-major] Diaphragm / surgery. Hemangiopericytoma / secondary. Lung Neoplasms / pathology. Thoracic Neoplasms / secondary
  • [MeSH-minor] Adult. Female. Follow-Up Studies. Humans. Tomography, X-Ray Computed

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  • (PMID = 12077693.001).
  • [ISSN] 0171-6425
  • [Journal-full-title] The Thoracic and cardiovascular surgeon
  • [ISO-abbreviation] Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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16. Anand R, Gupta S: Hemangiopericytoma of the maxilla in a pediatric patient: a case report. J Dent Child (Chic); 2010 Sep-Dec;77(3):180-2
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  • [Title] Hemangiopericytoma of the maxilla in a pediatric patient: a case report.
  • The hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation.
  • Hemangiopericytoma is most commonly seen in adults, and only 5% to 10% of cases occur in children.
  • The tumor is extremely rare in the head and neck region (16%).
  • Cytogenic abnormalities have been present in some hemangiopericytoma cases.
  • Surgical resection remains the mainstay treatment.
  • Adjuvant chemotherapy and radiotherapy is appropriate for cases of incomplete resections and life-threatening tumors particularly in children.
  • A 4-year-old child patient with hemangiopericytoma of the maxilla presented with firm, recurrent, but painless jaw mass.
  • The lesion biopsy showed wellcircumscribed multiple lobules of tumor mass consisting of tightly packed, spindleshaped cells.
  • Chemotherapy and radiotherapy of the lesion was conducted.
  • The role of the pediatric dental team is extensive in children with hemangiopericytoma, who require a regular dental review.
  • [MeSH-major] Hemangiopericytoma / diagnosis. Maxillary Neoplasms / diagnosis
  • [MeSH-minor] Biopsy. Child, Preschool. Diagnosis, Differential. Humans. Male

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  • (PMID = 22164890.001).
  • [ISSN] 1935-5068
  • [Journal-full-title] Journal of dentistry for children (Chicago, Ill.)
  • [ISO-abbreviation] J Dent Child (Chic)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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17. Kusakabe H, Iwasaki H, Sano K, Kiyokane K: Expression of lung resistance protein in epithelioid sarcoma in vitro and in vivo. Arch Dermatol Res; 2000 Jun;292(6):292-300
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  • The incidence of epithelioid sarcoma among patients with malignant soft tissue tumors is small, but the rates of recurrence and metastasis of this type of sarcoma are high.
  • To date, effective chemotherapy for advanced epithelioid sarcoma has not been established and, furthermore, epithelioid sarcoma is known to exhibit multidrug resistance (MDR).
  • The results showed that the ES-OMC-MN and SFT-8606 cell lines were resistant to vincristine (IC50 1190 nM and 872 nM, respectively) and Adriamycin (IC50 921 nM and 650 nM, respectively), but sensitive to actinomycin D (IC50 < 10 nM).
  • The original tumor tissues from which the two cell lines were established were also found to be LRP-positive but not to express p-Gp or MRP.
  • Since cyclosporin A, known to be a modifier of p-Gp, also induced reversal of vincristine resistance in the ES-OMC-MN and SFT-8606 cell lines (IC50 6.2 nM and 17 nM, respectively), it is suggested that cyclosporin A acts as a modifier of MDR mediated by LRP.
  • [MeSH-minor] Antibiotics, Antineoplastic / pharmacology. Antineoplastic Agents / pharmacology. Antineoplastic Agents, Phytogenic / pharmacology. Dactinomycin / pharmacology. Doxorubicin / pharmacology. Drug Resistance, Multiple. Drug Resistance, Neoplasm. Epithelioid Cells / drug effects. Epithelioid Cells / metabolism. Humans. Immunohistochemistry. Neoplasm Proteins / genetics. Neoplasm Proteins / metabolism. RNA / genetics. RNA, Messenger / analysis. Reverse Transcriptase Polymerase Chain Reaction. Tumor Cells, Cultured. Vault Ribonucleoprotein Particles / genetics. Vault Ribonucleoprotein Particles / metabolism. Vincristine / pharmacology


18. Yamada N, Okuse C, Nomoto M, Orita M, Katakura Y, Ishii T, Shinmyo T, Osada H, Maeda I, Yotsuyanagi H, Suzuki M, Itoh F: Obstructive jaundice caused by secondary pancreatic tumor from malignant solitary fibrous tumor of pleura: a case report. World J Gastroenterol; 2006 Aug 14;12(30):4922-6
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  • [Title] Obstructive jaundice caused by secondary pancreatic tumor from malignant solitary fibrous tumor of pleura: a case report.
  • A 77-year-old man on systemic chemotherapy against postoperative bilateral multiple lung metastases of malignant solitary fibrous tumor of the pleura suffered from pruritus and jaundice.
  • Blood examination showed elevated levels of hepatobiliary enzymes.
  • Abdominal computed tomography showed a tumor with peripheral enhancement in the pancreatic head, accompanied with the dilatation of intra- and extra-hepatic bile ducts.
  • He was diagnosed as having obstructive jaundice caused by a pancreatic head tumor.
  • The pancreatic head tumor was presumably diagnosed as the metastasis of malignant solitary fibrous tumor of the pleura, because the findings on the pancreatic head tumor on abdominal CT were similar to those on the primary lung lesion of malignant solitary fibrous tumor of the pleura.
  • The pancreatic tumor grew rapidly after the implantation of metallic stent in the inferior part of the common bile duct.
  • Autopsy revealed a tumor that spread from the pancreatic head to the hepatic hilum.
  • Immunohistochemically the pancreatic head tumor cells were negative for staining of alpha-smooth muscle actin (alpha-SMA) or CD117, but positive for vimentin, CD34 and CD99.
  • These findings are consistent with those on malignant solitary fibrous tumor of the pleura.
  • We report the first case of obstructive jaundice caused by a secondary pancreatic tumor from malignant solitary fibrous tumor of the pleura.
  • [MeSH-major] Jaundice, Obstructive / etiology. Neoplasms, Fibrous Tissue / pathology. Pancreatic Neoplasms / complications. Pancreatic Neoplasms / secondary. Pleural Neoplasms / pathology
  • [MeSH-minor] Aged. Autopsy. Biomarkers, Tumor / blood. Fatal Outcome. Humans. Male

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  • (PMID = 16937484.001).
  • [ISSN] 1007-9327
  • [Journal-full-title] World journal of gastroenterology
  • [ISO-abbreviation] World J. Gastroenterol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Other-IDs] NLM/ PMC4087636
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19. Anand R, Gupta S: Hemangiopericytoma of maxilla in a pediatric patient: a case report. J Dent Child (Chic); 2010 Sep-Dec;77(3):180-2
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  • [Title] Hemangiopericytoma of maxilla in a pediatric patient: a case report.
  • The Hemangiopericytoma is a malignant vascular tumor arising from mesenchymal cells with pericytic differentiation.
  • Hemangiopericytoma is most commonly seen in adults, and only 5% to 10% of cases occur in children.
  • The tumor is extremely rare in the head and neck region (16%)1.
  • Cytogenic abnormalities have been present in some hemangiopericytoma cases.
  • Surgical resection remains the mainstay treatment.
  • Adjuvant chemotherapy and radiotherapy is appropriate for cases of incomplete resections and life-threatening tumors particularly in children.
  • A 4-year-old child patient with hemangiopericytoma of the maxilla presented with firm, recurrent, but painless jaw mass.
  • The lesion biopsy showed well-circumscribed multiple lobules of tumor mass consisting of tightly packed, spindle-shaped cells.
  • Chemotherapy and radiotherapy of the lesion was conducted.
  • The role of the pediatric dental team is extensive in children with hemangiopericytoma, who require a regular dental review.

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  • [Cites] J Pediatr Surg. 2001 Jun;36(6):908-12 [11381424.001]
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  • (PMID = 21785578.001).
  • [ISSN] 1935-5068
  • [Journal-full-title] Journal of dentistry for children (Chicago, Ill.)
  • [ISO-abbreviation] J Dent Child (Chic)
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA076023-02; United States / NCI NIH HHS / CA / R25 CA076023; United States / NCI NIH HHS / CA / R25 CA076023-02
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Vimentin
  • [Other-IDs] NLM/ NIHMS136956; NLM/ PMC3140132
  • [Keywords] NOTNLM ; Chemotherapy / Hemangiopericytoma / Prognosis / Radiotherapy / Vascular tumor
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20. Fujita A, Minase T, Takabatake H, Tagaki S, Sekine K: [A case of primary malignant hemangiopericytoma of the lung with marked response to combination chemotherapy with cisplatin, ifosfamide and gemcitabine]. Gan To Kagaku Ryoho; 2001 Mar;28(3):373-6
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  • [Title] [A case of primary malignant hemangiopericytoma of the lung with marked response to combination chemotherapy with cisplatin, ifosfamide and gemcitabine].
  • A chest CT scan revealed a giant mass lesion in the right middle and lower lobes of the lung and mediastinal lymphadenopathy.
  • Bronchoscopic findings showed a tumor which almost completely obstructed the intermediate bronchus.
  • Histopathological examination of a biopsy specimen demonstrated malignant hemangiopericytoma.
  • Two courses of chemotherapy that combined cisplatin, ifosfamide and gemcitabine were performed every 3 weeks.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Bronchial Neoplasms / drug therapy. Bronchial Neoplasms / secondary. Hemangiopericytoma / drug therapy. Hemangiopericytoma / secondary. Lung Neoplasms / pathology

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  • (PMID = 11265407.001).
  • [ISSN] 0385-0684
  • [Journal-full-title] Gan to kagaku ryoho. Cancer & chemotherapy
  • [ISO-abbreviation] Gan To Kagaku Ryoho
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antimetabolites, Antineoplastic; 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0W860991D6 / Deoxycytidine; B76N6SBZ8R / gemcitabine; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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21. Anatolian Medical Oncology Society Group, Ustaalioglu BB, Gumus M, Unal A, Cayir K, Sever O, Bilici A, Elkiran ET, Karaca H, Benekli M, Karaoglu A, Seker M: Malignancies diagnosed during pregnancy and treated with chemotherapy or other modalities (review of 27 cases): multicenter experiences. Int J Gynecol Cancer; 2010 Jul;20(5):698-703
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  • [Title] Malignancies diagnosed during pregnancy and treated with chemotherapy or other modalities (review of 27 cases): multicenter experiences.
  • The most common tumors diagnosed during pregnancy are breast and cervix cancer, Hodgkin lymphoma and non-Hodgkin lymphoma, leukemias, and malignant melanoma.
  • The aim of therapy in pregnancy is to give optimal treatment to the mother without harm to the fetus.
  • In the first trimester, organogenesis continues, so chemotherapy should not be given because of increasing risk of spontaneous abortion, fetal malformation, and mortality.
  • We evaluated mostly seen tumors during pregnancy and assessed treatment type and outcome of pregnancy after chemotherapy in our population.
  • RESULTS: The tumors associated with pregnancy were breast cancer, hematologic malignancies,gynecologic malignancies, sarcomas, and others.
  • The chemotherapy regimens were given in 17 of 27 patients in the second or third trimester of pregnancy.
  • Four of the patients were diagnosed with cervical cancer, hemangiopericytoma, chronic myeloid leukemia,and breast cancer during the first trimester, so their pregnancies were ended by therapeutic abortion.
  • Although 1 of the 3 fetuses who were exposed to chemotherapy in utero at the second or third trimester was born prematurely and low birth weight was diagnosed in the other 2 fetuses, fetal malformation was not seen in any of them.
  • CONCLUSIONS: We reported herein 27 patients with malignancies diagnosed during pregnancy; 17 patients received chemotherapy during the gestational period without any fetal or maternal abnormalities.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Pregnancy Complications, Neoplastic / drug therapy. Prenatal Exposure Delayed Effects / chemically induced

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  • (PMID = 20973261.001).
  • [ISSN] 1525-1438
  • [Journal-full-title] International journal of gynecological cancer : official journal of the International Gynecological Cancer Society
  • [ISO-abbreviation] Int. J. Gynecol. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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22. Suhr M, Gottschalk J, Kreusch T: Metastatic malignant solitary fibrous tumour to the infra-temporal fossa. Int J Oral Maxillofac Surg; 2003 Dec;32(6):659-61
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  • [Title] Metastatic malignant solitary fibrous tumour to the infra-temporal fossa.
  • Malignant solitary fibrous tumours (MSFTs) are rare tumours of fibrous origin, which can occur at all anatomical sites and represent 20% of solitary fibrous tumours.
  • Fine-needle aspiration cytology is not able to distinguish benign from malignant disease, and sufficient tissue has to be obtained for accurate histological diagnosis to be made.
  • Lesions > 10 cm in diameter and incomplete resection or non-resectability are predictive factors for poor long-term survival.
  • We present a 57-year-old patient with a presumably metastatic MSFT from the peritoneal cavity to the skull-base who is in a stable state 17 months after surgical debulking of the skull-base and removal of the peritoneal lesion, followed by post-operative chemotherapy.
  • We suggest the terminology metastatic malignant solitary fibrous tumour for a description of this disease.
  • [MeSH-major] Neoplasms, Fibrous Tissue / secondary. Peritoneal Neoplasms / pathology. Skull Base Neoplasms / secondary
  • [MeSH-minor] Antigens, CD34 / analysis. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Doxorubicin / administration & dosage. Female. Humans. Ifosfamide / administration & dosage. Immunohistochemistry. Middle Aged. Terminology as Topic

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  • (PMID = 14686422.001).
  • [ISSN] 0901-5027
  • [Journal-full-title] International journal of oral and maxillofacial surgery
  • [ISO-abbreviation] Int J Oral Maxillofac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Antigens, CD34; 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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23. Zawitkowska-Klaczyńska J, Katski K, Woźniak M, Kowalczyk JR: Characteristics and outcome of children with primary soft tissue sarcomas of extremities. Med Wieku Rozwoj; 2004 Apr-Jun;8(2 Pt 1):169-74
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  • [Title] Characteristics and outcome of children with primary soft tissue sarcomas of extremities.
  • OBJECTIVES: To determine the characteristics and outcome or patients with primary soft tissue sarcomas of extremities in children.
  • MATERIAL AND METHODS: Thirty-six patients treated for soft tissues sarcomas were enrolled into the study.
  • Features analysed: the incidence of soft tissues sarcoma of extremities, the time from first clinical symptoms to making the diagnosis, the primary site of tumour; histopathologic type of tumour, stage of disease, methods and results of the treatment.
  • RESULTS: The time From first symptoms to making the diagnosis was 5.4 months (mean).
  • The site of the tumour was the femur in 6 patients, arm in 3, knee in 1.
  • Histopathologic types: synovial sarcoma in 4 patients, malignant haemangiopericytoma in 2, rhabdomyosarcoma in 2, sarcoma myogenes in 1, primitive neuroectodermal tumour in l.
  • Stage of disease: III deg.
  • Patients underwent treatment according to the soft tissue sarcoma protocols.
  • Results of treatment: first complete remission was observed in 7 patients; second complete remission in 1, one patient is on postoperative treatment.
  • Combined treatment achieves full remission in the majority of patients with soft tissues sarcomas localized within the limbs.
  • 2. In patients with large tumours (>5 cm) the treatment should to be started with inductive chemotherapy, and the surgery should be postponed.
  • 3. Early excision of the tumour should be considered in cases of small tumours (< 5 cm), when resection with wide margin of healthy tissues is possible, without deteriorating the function of the limb or cosmetic damage.
  • [MeSH-major] Arm. Leg. Sarcoma / diagnosis. Sarcoma / therapy
  • [MeSH-minor] Adolescent. Chemotherapy, Adjuvant. Child. Disease-Free Survival. Female. Hemangiopericytoma / diagnosis. Hemangiopericytoma / therapy. Humans. Incidence. Male. Myosarcoma / diagnosis. Myosarcoma / therapy. Neoplasm Staging. Neuroectodermal Tumors / diagnosis. Neuroectodermal Tumors / therapy. Poland / epidemiology. Retrospective Studies. Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / therapy. Sarcoma, Synovial / diagnosis. Sarcoma, Synovial / therapy. Survival Analysis. Time Factors. Treatment Outcome

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  • (PMID = 15738590.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Poland
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24. Benesch M, Windelberg M, Sauseng W, Witt V, Fleischhack G, Lackner H, Gadner H, Bode U, Urban C: Compassionate use of bevacizumab (Avastin) in children and young adults with refractory or recurrent solid tumors. Ann Oncol; 2008 Apr;19(4):807-13
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  • [Title] Compassionate use of bevacizumab (Avastin) in children and young adults with refractory or recurrent solid tumors.
  • PATIENTS AND METHODS: Fifteen patients (male: n = 8; female: n = 7; median age, 14.6 years) received bevacizumab for recurrent or progressive solid tumors (carcinoma: n = 3; neuroblastoma: n = 2; astrocytoma grade III: n = 2; rhabdomyosarcoma: n = 2; nephroblastoma: n = 2; benign vascular tumors: n = 2; synovial sarcoma: n = 1; and malignant hemangiopericytoma: n = 1) on a compassionate basis.
  • Most patients received chemotherapy in addition to bevacizumab.
  • Duration of bevacizumab therapy ranged from 1.5 to 23 months.
  • CONCLUSIONS: Bevacizumab seems to have a good acute safety profile and some antitumor activity in heavily pretreated children and young adults with recurrent solid tumors.

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  • (PMID = 18056650.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 2S9ZZM9Q9V / Bevacizumab
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25. Kawamura S, Nakamura T, Oya T, Ishizawa S, Sakai Y, Tanaka T, Saito S, Fukuoka J: Advanced malignant solitary fibrous tumor in pelvis responding to radiation therapy. Pathol Int; 2007 Apr;57(4):213-8
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  • [Title] Advanced malignant solitary fibrous tumor in pelvis responding to radiation therapy.
  • Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that is benign in most cases.
  • Although SFT was first recognized to arise only in the pleura, recent reports indicate that SFT can involve a wide range of anatomical sites.
  • To date, 17 cases of pelvic SFT have been reported.
  • Herein is reported a case of a 74-year-old woman with a giant malignant SFT in the pelvis.
  • Along with massive invasion to adjacent organs and multiple lung metastases detected on radiography, biopsy from the tumor through the vaginal wall showed malignant looking spindle-cell neoplasm with increased cellularity, areas of necrosis, and high mitotic activity (5/10 high-power fields).
  • Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34, CD99, and bcl-2.
  • Based on pathological features and clinical presentation, diagnosis of malignant SFT was made.
  • The patient received systemic and the intra-arterial chemotherapy followed by whole pelvic radiation therapy (50 Gy).
  • Initial chemotherapies failed to control the tumor.
  • Afterwards, improvement was observed radiologically and pathologically in the 12 months' follow up after the radiation therapy.
  • This is the first report related to therapeutic remarks on advanced malignant SFT.
  • [MeSH-major] Carcinoma / radiotherapy. Neoplasms, Fibrous Tissue / radiotherapy. Pelvic Neoplasms / radiotherapy
  • [MeSH-minor] Aged. Disease Progression. Female. Humans. Magnetic Resonance Imaging. Neoplasm Metastasis / pathology

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  • (PMID = 17316417.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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26. Dürr HR, Nerlich A, Lienemann A, Müller PE, Refior HJ: Malignant hemangiopericytoma of the bone. Langenbecks Arch Surg; 2000 Apr;385(3):207-12
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  • [Title] Malignant hemangiopericytoma of the bone.
  • Hemangiopericytoma is a rare vascular tumor of pericyte origin with variable malignant potential.
  • Very rarely, this tumor occurs as a primary bone lesion.
  • We present a case of a highly malignant hemangiopericytoma of the proximal tibia.
  • Current therapy consists of radical resection of the tumor with postoperative radiation therapy being recommended.
  • Chemotherapy seems to be useful in disseminated disease.
  • The prognosis correlates to the histological grading of the tumor.

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  • (PMID = 10857492.001).
  • [ISSN] 1435-2443
  • [Journal-full-title] Langenbeck's archives of surgery
  • [ISO-abbreviation] Langenbecks Arch Surg
  • [Language] ENG
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
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27. Bień E, Godziński J, Balcerska A, Rapała M, Izycka-Swieszewska E, Stachowicz-Stencel T, Sulka W, Kazanowska B, Reich A, Chybicka A, Madziara W, Bohosiewicz J, Perek-Polnik M, Perek D, Mańkowski P, Jankowski A, Nurzyńska-Flak J, Kowalczyk J, Kurylak A, Wysocki M, Rybczyńska A, Wachowiak J, Zalewska-Szewczyk B, Bodalski J, Jaśkiewicz K: [Malignant vascular tumours in children -- report from the Polish Pediatric Rare Tumors Study]. Med Wieku Rozwoj; 2004 Apr-Jun;8(2 Pt 1):145-58
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  • [Title] [Malignant vascular tumours in children -- report from the Polish Pediatric Rare Tumors Study].
  • Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas.
  • THE AIM OF THE STUDY: To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms.
  • MATERIAL AND METHODS: The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002.
  • On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients).
  • RESULTS: Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease.
  • Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all.
  • 9 of the 10 patients of group I, died of disease progression.
  • 2 children relapsed and died of the disease.
  • Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free.
  • Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4.
  • Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression.
  • The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory.
  • The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease).
  • Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression.
  • [MeSH-major] Hemangioendothelioma. Hemangiopericytoma. Hemangiosarcoma
  • [MeSH-minor] Adolescent. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant / methods. Child. Child, Preschool. Female. Humans. Male. Poland. Prognosis. Radiotherapy Dosage. Radiotherapy, Adjuvant / methods. Retrospective Studies. Risk Factors. Survival Analysis. Time Factors. Treatment Outcome

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  • (PMID = 15738588.001).
  • [Journal-full-title] Medycyna wieku rozwojowego
  • [ISO-abbreviation] Med Wieku Rozwoj
  • [Language] pol
  • [Publication-type] English Abstract; Journal Article; Multicenter Study
  • [Publication-country] Poland
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28. Greenson JK: Gastrointestinal stromal tumors and other mesenchymal lesions of the gut. Mod Pathol; 2003 Apr;16(4):366-75
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  • [Title] Gastrointestinal stromal tumors and other mesenchymal lesions of the gut.
  • In the past 5 years, there has been a paradigm shift in our understanding of gastrointestinal stromal tumors (GISTs).
  • Once thought to be smooth muscle tumors, these uncommon neoplasms are now thought to differentiate along the lines of interstitial cells of Cajal, the pacemaker cells of the gut.
  • Along with this understanding comes an exciting new drug therapy (Gleevec) that for the first time offers real hope to patients with malignant stromal tumors.
  • Overall, approximately 60-70% of stromal tumors are from the stomach, 20-30% are from the small intestine, and <10% come from the esophagus, colon, rectum, omentum, and mesentery.
  • Between 10 and 30% of GISTs are malignant.
  • Stromal tumors should be studied in a site-specific fashion, as tumors from a given location in the gut have unique growth patterns and corresponding behaviors.
  • Although the most important tool needed to diagnose a GIST is still a hematoxylin and eosin-stained section, a confirmatory CD117 stain is recommended (and may be required for drug therapy).
  • True smooth muscle tumors, inflammatory fibroid polyps, fibromatoses, schwannomas, inflammatory myofibroblastic tumors, and solitary fibrous tumors all enter into the differential diagnosis of GISTs.
  • This article reviews the histologic features of these tumors in the context of recent molecular genetic and immunohistochemical advances.
  • [MeSH-major] Biomarkers, Tumor / analysis. Gastrointestinal Neoplasms / pathology. Mesoderm / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Benzamides. Diagnosis, Differential. Imatinib Mesylate. Immunohistochemistry. Molecular Biology. Piperazines / therapeutic use. Prognosis. Pyrimidines / therapeutic use

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  • (PMID = 12692202.001).
  • [ISSN] 0893-3952
  • [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; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Biomarkers, Tumor; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate
  • [Number-of-references] 55
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29. Knott PD, Gannon FH, Thompson LD: Mesenchymal chondrosarcoma of the sinonasal tract: a clinicopathological study of 13 cases with a review of the literature. Laryngoscope; 2003 May;113(5):783-90
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  • [Title] Mesenchymal chondrosarcoma of the sinonasal tract: a clinicopathological study of 13 cases with a review of the literature.
  • OBJECTIVES/HYPOTHESIS: Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin.
  • Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors.
  • RESULTS: Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these.
  • Tumors had an overall mean size of 5.1 cm.
  • Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix.
  • All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3).
  • The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y).
  • Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up.
  • CONCLUSIONS: Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women.
  • Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Disease-Free Survival. Female. Humans. Infant, Newborn. Male. Middle Aged. Neoplasm Staging. Retrospective Studies. Tomography, X-Ray Computed

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  • (PMID = 12792311.001).
  • [ISSN] 0023-852X
  • [Journal-full-title] The Laryngoscope
  • [ISO-abbreviation] Laryngoscope
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 30
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30. Güneş M, Günaldi O, Tuğcu B, Tanriverdi O, Güler AK, Cöllüoğlu B: Intracranial chondrosarcoma: a case report and review of the literature. Minim Invasive Neurosurg; 2009 Oct;52(5-6):238-41
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  • INTRODUCTION: Chondrosarcoma is a rare malignant tumor originating from cartilagenous tissue.
  • Cranial computed tomography and magnetic resonance imaging showed a mass lesion including calcification areas and homogenous contrast enhancement in the right parieto-occipital region.
  • The tumor was removed totally by a microsurgical technique.
  • A classical type chondrosarcoma was confirmed histopathologically.
  • Primary intracranial chondrosarcomas, constitute only less than 0.16% of all brain tumors.
  • Meningiomas, solitary fibrous tumor, chordoma, hemangiopericytoma, metastasis and vascular malformations should be considered as differential diagnoses.
  • Radical surgical removal of the tumor is the preferred management procedure.
  • Chemotherapy and radiotherapy may by added as adjuvant therapy.
  • We present the case of a patient with an intracranial chondrosarcoma, who has treated successfully with surgical removal.
  • [MeSH-major] Brain Neoplasms / diagnosis. Brain Neoplasms / surgery. Chondrosarcoma / diagnosis. Chondrosarcoma / surgery
  • [MeSH-minor] Adult. Humans. Magnetic Resonance Imaging. Male. Microsurgery. Neurosurgical Procedures. Prognosis. Tomography, X-Ray Computed. Treatment Outcome


31. Vedrine PO, Thariat J, Merrot O, Percodani J, Dufour X, Choussy O, Toussaint B, Dassonville O, Klossek JM, Santini J, Jankowski R: Primary cancer of the sphenoid sinus--a GETTEC study. Head Neck; 2009 Mar;31(3):388-97
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  • BACKGROUND: Primary involvement of the sphenoid sinus occurs in 2% of all paranasal sinus tumors and is associated with dismal prognosis.
  • Charts were reviewed for patient-, tumor-, and treatment-related parameters.
  • Pathologic findings included adenoid cystic carcinoma, adenocarcinoma, lymphoma, squamous cell carcinoma, sarcoma, neuroendocrine carcinoma, melanoma, and malignant hemangiopericytoma.
  • Radiotherapy was performed in 18 patients and chemotherapy in 12.
  • Surgery was rarely complete because of advanced stages at presentation, but it yielded better outcomes than other treatments without surgery in non lymphoma-cases.
  • Surgery, including debulking surgery, may be preferred to combined modality treatments without surgery.
  • Its apparently favorable impact on prognosis would need to be tested in homogenous histological groups of patients, which is impossible because of the rarity of the disease.
  • Highly conformal radiotherapy (adjuvant or definitive) should be encouraged and optimized with concurrent chemotherapy in advanced stages.
  • Aggressive multidisciplinary management including surgery, chemotherapy, and radiotherapy should be encouraged and adapted on histology and tumor extensions.
  • [MeSH-major] Paranasal Sinus Neoplasms / mortality. Paranasal Sinus Neoplasms / therapy. Sphenoid Sinus / pathology
  • [MeSH-minor] Adult. Aged. Carcinoma / mortality. Carcinoma / pathology. Carcinoma / therapy. Chemotherapy, Adjuvant. Cranial Nerve Diseases / complications. Diagnostic Imaging. Female. Humans. Lymphoma, B-Cell / mortality. Lymphoma, B-Cell / pathology. Lymphoma, B-Cell / therapy. Male. Melanoma / mortality. Melanoma / pathology. Melanoma / therapy. Meningioma / mortality. Meningioma / pathology. Meningioma / therapy. Middle Aged. Multivariate Analysis. Plasmacytoma / mortality. Plasmacytoma / pathology. Plasmacytoma / therapy. Preoperative Care. Radiotherapy, Adjuvant. Radiotherapy, Conformal

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  • (PMID = 18972425.001).
  • [ISSN] 1097-0347
  • [Journal-full-title] Head & neck
  • [ISO-abbreviation] Head Neck
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
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32. Parmar DN, Rose GE: Management of lacrimal sac tumours. Eye (Lond); 2003 Jul;17(5):599-606
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of lacrimal sac tumours.
  • PURPOSE: To report a series of 15 primary lacrimal sac tumours and present an algorithm in managing this rare condition.
  • METHODS: A retrospective review of the clinical, radiological, and pathological records of 15 patients with primary lacrimal sac tumours.
  • Main outcome measures Histologic evaluation and clinical follow-up, including tumour clearance and recurrence, were assessed.
  • The commonest tumour was non-Hodgkins B-cell lymphoma (five cases), followed by two cases each of squamous cell carcinoma and transitional cell carcinoma, one case of benign transitional papilloma, haemangiopericytoma, adenoid cystic carcinoma, mucoepidermoid carcinoma, mixed (squamous/transitional) carcinoma, and a highly malignant undifferentiated tumour.
  • Treatment modalities included surgery, radiotherapy and chemotherapy and, with a median follow-up of 30 months (range 2 months to 17 years), two patients had died from metastatic disease but nine patients remained without evidence of recurrent tumour.
  • CONCLUSIONS: Primary lacrimal sac tumours are extremely rare, require long-term follow-up for recurrence and metastasis, and can be fatal.
  • [MeSH-major] Eye Neoplasms / therapy. Lacrimal Apparatus Diseases / therapy
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Algorithms. Fatal Outcome. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Retrospective Studies. Treatment Outcome

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  • (PMID = 12855966.001).
  • [ISSN] 0950-222X
  • [Journal-full-title] Eye (London, England)
  • [ISO-abbreviation] Eye (Lond)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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33. Thanni LO: Extremity haemangiopericytoma, a case report from Nigeria. Afr Health Sci; 2005 Sep;5(3):261-4
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  • [Title] Extremity haemangiopericytoma, a case report from Nigeria.
  • Haemangiopericytoma is an uncommon soft tissue sarcoma of vascular origin.
  • Wide surgical excision is the mainstay of treatment.
  • However, adjuvant radiotherapy and chemotherapy are desirable because the malignant nature of this tumour is frequently unpredictable.
  • Adjuvant therapy is recommended for metastases, recurrence and incomplete resection.
  • Long term follow up is essential in all cases as recurrence can occur several years after treatment.
  • Where little or no experience with managing this tumor exists, it is important to be aware of its clinical behaviour and the treatment options, hence this case reports.
  • [MeSH-major] Hemangiopericytoma / diagnosis. Leg / physiopathology

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  • (PMID = 16245998.001).
  • [ISSN] 1729-0503
  • [Journal-full-title] African health sciences
  • [ISO-abbreviation] Afr Health Sci
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Uganda
  • [Other-IDs] NLM/ PMC1831924
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34. Essola B, Remmelink M, Kessler R, Scillia P, Rocmans P: [Primary pulmonary hemangiopericytoma: 2 new cases]. Rev Med Brux; 2003 Oct;24(5):408-15
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  • [Title] [Primary pulmonary hemangiopericytoma: 2 new cases].
  • We describe two new resected cases of primary pulmonary hemangiopericytoma and the review of cases published in the period 1954-2002.
  • The first patient has a large pulmonary mass of the right apex revealed by scapular pain.
  • The right upper lobectomy with free margins reveals hemangiopericytoma.
  • Pelvic and pulmonary metastases appear two years after surgery, treated by two series of chemotherapy without clinical response.
  • The second patient develops dry cough and thoracic pain with discovery of a cavitary mass in the right pulmonary field.
  • Three months after extended pneumonectomy, the intrathoracic tumour relapses and regresses partially under chemotherapy.
  • Histology and immunohistochemistry of both tumours closely related to solitary fibrous tumour confirm malignant hemangiopericytoma.
  • Primary pulmonary hemangiopericytoma is rare and may be benign or malignant.
  • Radical resection is the best treatment.
  • Chemotherapy and radiotherapy may improve the prognosis.
  • Compared with lung cancer, the tumour is a slow growing mass, often voluminous, with delayed symptoms, very few lymph node dissemination, rare brain metastasis, more frequent cutaneous or retroperitoneal dissemination, often after long-term and requiring indeed a 10 to 20 years follow-up.
  • [MeSH-major] Hemangiopericytoma. Lung Neoplasms

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  • (PMID = 14650317.001).
  • [ISSN] 0035-3639
  • [Journal-full-title] Revue médicale de Bruxelles
  • [ISO-abbreviation] Rev Med Brux
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Belgium
  • [Number-of-references] 48
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35. Ozden I, Yavuz E, Acarli K, Karabulut L, Yöney E, Cevikbaş U, Alper A: Primary lymphoma of the liver: report of a case. Surg Today; 2000;30(4):376-9
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  • [Title] Primary lymphoma of the liver: report of a case.
  • The patient was referred with the diagnosis of malignant hemangiopericytoma (with an open biopsy).
  • The physical examination, standard laboratory test results and tumor marker levels were all normal.
  • Histopathological and immunohistochemical examinations revealed non-Hodgkin's lymphoma of B-cell type.
  • The findings of a peripheral blood smear and bone marrow biopsy were normal.
  • These findings established the diagnosis of primary hepatic lymphoma.
  • The best treatment results have been obtained by a resection followed by chemotherapy when feasible.
  • [MeSH-major] Liver Neoplasms / diagnosis. Lymphoma, Non-Hodgkin / diagnosis
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Female. Humans

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  • (PMID = 10795873.001).
  • [ISSN] 0941-1291
  • [Journal-full-title] Surgery today
  • [ISO-abbreviation] Surg. Today
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] JAPAN
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36. Schneider LF, Gardner SL, Sharma S: Preoperative chemotherapy to salvage the hand in a case of giant infantile hemangiopericytoma. J Hand Surg Am; 2010 Jun;35(6):995-8
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  • [Title] Preoperative chemotherapy to salvage the hand in a case of giant infantile hemangiopericytoma.
  • Hemangiopericytoma (HPC) is a rare vascular tumor arising from contractile cells around blood vessels, with the potential for malignant degeneration.
  • Standard therapy for this tumor is surgical excision.
  • We report the case of a 6-month-old infant with giant HPC involving the hand.
  • Chemotherapy resulted in a decrease in tumor size, allowing for salvage of most of the hand and fingers.
  • Preoperative chemotherapy should be considered in the care of HPC tumors involving the upper extremity in children.
  • [MeSH-major] Hand. Hemangiopericytoma / drug therapy. Hemangiopericytoma / surgery. Limb Salvage / methods. Neoadjuvant Therapy. Vascular Neoplasms / drug therapy. Vascular Neoplasms / surgery
  • [MeSH-minor] Amputation. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Female. Humans. Infant. Magnetic Resonance Imaging. Preoperative Care

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  • [Copyright] Copyright 2010. Published by Elsevier Inc.
  • (PMID = 20513579.001).
  • [ISSN] 1531-6564
  • [Journal-full-title] The Journal of hand surgery
  • [ISO-abbreviation] J Hand Surg Am
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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37. Hatta C, Terada T, Okita J, Kakibuchi M, Kubota A, Sakagami M: Clinicopathological study of undifferentiated carcinoma of the parotid gland. Auris Nasus Larynx; 2003 Aug;30(3):273-7
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  • OBJECTIVE: Undifferentiated carcinoma of the salivary gland is a rare malignant tumor, and is difficult to distinguish from other poorly differentiated types of carcinoma or sarcoma.
  • Tumors in these two patients included a small portion of poorly differentiated epidermoid or mucoepidermoid carcinoma.
  • The remaining three patients did not show any differentiated portions, and histological findings demonstrated heterogeneous patterns of lymphoepithelial carcinoma, small cell carcinoma and unclassified (a pattern of malignant hemangiopericytoma), respectively.
  • All patients died of distant metastasis despite radical surgery, suggesting that chemotherapy is needed to improve patient outcomes.

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  • (PMID = 12927291.001).
  • [ISSN] 0385-8146
  • [Journal-full-title] Auris, nasus, larynx
  • [ISO-abbreviation] Auris Nasus Larynx
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
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38. Veronesi G, Spaggiari L, Mazzarol G, De Pas M, Leo F, Solli P, Pastorino U: Huge malignant localized fibrous tumor of the pleura. J Cardiovasc Surg (Torino); 2000 Oct;41(5):781-4
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  • [Title] Huge malignant localized fibrous tumor of the pleura.
  • Localized fibrous tumor is an unfrequent mesenchymal neoplasm.
  • The malignant variant of the pleura is exceptional and differential diagnosis with the more frequent benign type or with other neoplasms such as soft tissue sarcoma and mesothelioma is rarely possible in a preoperative setting.
  • The best treatment of this disease is radical surgical resection.
  • No definitive data exist about the role of chemotherapy.
  • We report a case of a giant right intrathoracic mass whose preoperative diagnosis, from an open biopsy, was consistent with sarcoma and, in a second review, with fibrous tumor of the pleura without any indication about malignancy.
  • In consideration of the apparent local radicality we did not perform any adjuvant treatment.
  • Six months after the operation a wide local recurrence was evident and a systemic treatment with Ifosfamide and Adriamicina is still in progress.
  • Preoperative diagnosis of malignancy has an important role as a therapeutic strategy in management of fibrous tumours of the pleura.
  • When there is suspicion of a malignant form neoadjuvant chemotherapy can represent a further tool to control poorly differentiated and large tumors, and a wide surgical resection of the lesion must be performed.
  • [MeSH-major] Fibroma / surgery. Pleural Neoplasms / surgery
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Combined Modality Therapy. Doxorubicin / therapeutic use. Humans. Ifosfamide / therapeutic use. Male. Middle Aged. Neoplasm Recurrence, Local / drug therapy. Pneumonectomy

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  • (PMID = 11149649.001).
  • [ISSN] 0021-9509
  • [Journal-full-title] The Journal of cardiovascular surgery
  • [ISO-abbreviation] J Cardiovasc Surg (Torino)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 80168379AG / Doxorubicin; UM20QQM95Y / Ifosfamide
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39. Radulescu D, Pripon S, Ciuleanu TE, Radulescu LI: Malignant primary pulmonary tumor with hemangiopericytoma-like features: conventional hemangiopericytoma versus solitary fibrous tumor. Clin Lung Cancer; 2007 Sep;8(8):504-8
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  • [Title] Malignant primary pulmonary tumor with hemangiopericytoma-like features: conventional hemangiopericytoma versus solitary fibrous tumor.
  • Although extremely rare, the primitive lung hemangiopericytoma in adults is similar to hemangiopericytomas with other soft tissue localizations.
  • Although generally benign and curable after radical surgery, it might also have a malignant clinical course with dissemination in both lungs, infiltration of vital organs (heart, pulmonary artery), extension to the adjacent tissues, and even pulmonary metastases.
  • The treatment of choice is the complete tumor resection with negative surgical margins after excision.
  • Certain histologic features might indicate a malignant potential.
  • The clinical outcome of patients is variable: some are cured after radical surgery and others might present relapse and recurrences that necessitate a second intervention, radiation therapy, and/or chemotherapy.
  • Over the years, the conventionally-defined hemangiopericytoma concept has evolved because of the nonspecific histologic growth pattern (characteristic monotonous appearance, moderate or high cellularity, and a well-developed branching vascular pattern) shared by numerous, unrelated benign or malignant lesions.
  • Hemangiopericytoma is no longer considered a specific entity but rather as a characteristic growth pattern; in the heterogeneous group of hemangiopericytoma-like neoplasms, many lesion categories have been individualized and defined.
  • We report an uncommon case of primitive lung tumor exhibiting hemangiopericytoma-like features, with an aggressive, fatal clinical course.
  • Because of the major histologic overlap between solitary fibrous tumor and hemangiopericytoma and lack of clear classification criteria, we encountered difficulty in including this case in a known clinical entity; primitive solitary fibrous tumor of the lung, which mimics lung hemangiopericytoma, seemed to be the most plausible diagnosis.
  • [MeSH-major] Hemangiopericytoma / pathology. Lung Neoplasms / pathology. Solitary Fibrous Tumors / pathology
  • [MeSH-minor] Aged. Diagnosis, Differential. Humans. Male. Radiography, Thoracic. Tomography, X-Ray Computed

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  • (PMID = 17922977.001).
  • [ISSN] 1525-7304
  • [Journal-full-title] Clinical lung cancer
  • [ISO-abbreviation] Clin Lung Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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40. Antoniello L, Cecchetto G, Carli M, Dall'Igna P, Bisogno G, Lo Piccolo R, Gigante C, Zanetti I, Guglielmi M: [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88]. Pediatr Med Chir; 2003 Jul-Aug;25(4):255-60
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  • [Title] [Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88].
  • Aim of the study was to evaluate the role of mutilating surgery in the patients with non chemosensitive soft tissue sarcomas (STS) registered in the Italian Studies.
  • Between 1979 and 1995, 114 patients (pts) with "non chemosensitive" localized STS were enrolled in the cooperative studies RMS-79 (33) and RMS-88 (81).
  • HISTOLOGY: fibrosarcoma 29, Malignant Perpheral Nerve Sheath Tumors (MPNST) 40, malignant fibrous histiocytoma 5, hemangiopericytoma 6, leiomyosarcoma 4, others 20, STS nos 10.
  • The cases were classified according to the IRS staging system as Gr. I 46, Gr. II 24, Gr. III 44.
  • Twelve out of 114 pts (7%), 5/33 (14%) in the first study and 7/81 (8%) in the second, underwent mutilating surgery: 8 pts (of whom 3 were < 2 y of age) had a fibrosarcoma and 4 a MPNST.
  • The mutilating procedure was carried out at diagnosis in 6 cases (4 in RMS-79 and 2 in RMS-and 88) and achieved radicality in 5/6 cases.
  • It was performed after ineffective chemotherapy (CT) in 5 pts (1 in RMS-79 and 4 in RMS-88).
  • OUTCOME: At present 6/12 pts, 5 with fibrosarcoma and 1 with MPNST, are alive with no evidence of disease (NED), 4 of the first and 2 of the second study.
  • Of the 5 Gr. I patients, 4 are alive (NED) and 1 died of 2nd tumor; 1 Gr.
  • III pts 1 is alive NED and 4 died (3 of metastatic spread and 1 of 2nd tumor); the pt amputated after repeated local relapses (Gr.
  • CONCLUSIONS: In the RMS-79 study the mutilations were frequent and were performed at diagnosis in several cases; this trend decreased in the 2nd study in which chemotherapy was attempted in most of the patients.
  • [MeSH-major] Sarcoma / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 15070267.001).
  • [ISSN] 0391-5387
  • [Journal-full-title] La Pediatria medica e chirurgica : Medical and surgical pediatrics
  • [ISO-abbreviation] Pediatr Med Chir
  • [Language] ita
  • [Publication-type] English Abstract; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Italy
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41. Chakrabarti B, Ghosh SK, Basu B, Gupta P, Ghorai S, Ray SG, Das C: Non-adenomatous non-epithelial carcinoma (hemangiopericytoma) of prostate treated with conservative surgery followed by adjuvant chemoradiation. Curr Oncol; 2009 Aug;16(4):71-3
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  • [Title] Non-adenomatous non-epithelial carcinoma (hemangiopericytoma) of prostate treated with conservative surgery followed by adjuvant chemoradiation.
  • Hemangiopericytoma is a malignant vascular tumour of soft tissue.
  • Microscopically, the tumour shows tightly packed cellular areas surrounding thin-walled branching blood vessels.
  • Traditionally these tumours are treated using wide surgical excision.
  • Only a very few cases of hemangiopericytoma of the prostate have been described worldwide.
  • The feasibility of managing such a case with a combination of conservative surgery and adjuvant anti-malignancy treatment is unexplored.
  • Here, we report a case of hemangiopericytoma of the prostate treated with local excision, with preservation of prostate, followed by adjuvant radiotherapy (40 Gy in 20 fractions to pelvis followed by 24 Gy in 12 fractions as boost to prostate) and chemotherapy (doxorubicin and iphosphamide).
  • Post-treatment computed tomography scan after 4 weeks showed regression of pelvic lymph nodes and a normal-appearing prostate.
  • Levels of serum prostate-specific and carcinogenic embryonic antigen were normal throughout the period of treatment.
  • To date, followup has been uneventful, except for occasional bouts of diarrhea.We conclude that conservative surgery followed by adjuvant radiation and chemotherapy, with subsequent close follow-up, may adequately control localized disease in selected cases of hemangiopericytoma of the prostate.
  • The role of conservative surgery in tumours located at other sites has yet to be defined.

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  • (PMID = 19672428.001).
  • [ISSN] 1198-0052
  • [Journal-full-title] Current oncology (Toronto, Ont.)
  • [ISO-abbreviation] Curr Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Canada
  • [Other-IDs] NLM/ PMC2722051
  • [Keywords] NOTNLM ; Carcinoma / hemangiopericytoma / prostate / treatment
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42. Hogle WP: Malignant hemangiopericytoma: a clinical overview and case study. Clin J Oncol Nurs; 2003 Jan-Feb;7(1):57-62
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  • [Title] Malignant hemangiopericytoma: a clinical overview and case study.
  • Although unusual, hemangiopericytoma (HPC) can be considered benign or malignant depending on histologic features.
  • In some cases, malignant HPC can behave as high-grade sarcomas.
  • When HPC is deemed malignant, radical surgical excision is the treatment of choice.
  • Adjuvant chemotherapy and radiation therapy offer limited success in patients with this malignancy.
  • This article details a case study of a man with metastatic malignant HPC that spanned 25 months and three major cancer centers.
  • Throughout the case study, chemotherapy, radiation therapy, and an experimental agent were employed to attempt to achieve control of the aggressive disease.
  • A better general understanding of this disease process is needed to meet the needs of patients with malignant HPC.
  • [MeSH-major] Hemangiopericytoma / diagnosis. Hemangiopericytoma / therapy. Lung Neoplasms / diagnosis. Lung Neoplasms / therapy. Neoplasms, Multiple Primary / diagnosis. Neoplasms, Multiple Primary / therapy
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Biopsy, Needle. Combined Modality Therapy. Disease Progression. Fatal Outcome. Humans. Male. Middle Aged. Neoplasm Staging. Radiotherapy / methods. Risk Assessment. Salvage Therapy. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 12629936.001).
  • [ISSN] 1092-1095
  • [Journal-full-title] Clinical journal of oncology nursing
  • [ISO-abbreviation] Clin J Oncol Nurs
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 37
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43. Poggio JL, Nagorney DM, Nascimento AG, Rowland C, Kay P, Young RM, Donohue JH: Surgical treatment of adult primary hepatic sarcoma. Br J Surg; 2000 Nov;87(11):1500-5
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  • [Title] Surgical treatment of adult primary hepatic sarcoma.
  • Optimal therapeutic approaches remain unclear.
  • METHODS: Twenty consecutive adult patients who had surgical treatment for primary hepatic sarcomas were reviewed.
  • Other than one patient with primary hepatic angiosarcoma who had a history of thorium dioxide colloid (Thorotrast) exposure 23 years before diagnosis, no predisposing causes were apparent.
  • No patient received neoadjuvant chemotherapy or radiotherapy but radiotherapy was delivered intraoperatively in one patient.
  • RESULTS: Leiomyosarcoma was the most common histological type of sarcoma diagnosed (five of 20 patients), followed by malignant solitary fibrous tumour (four) and epithelioid haemangioendothelioma (three).
  • Fourteen tumours were high-grade sarcomas and six were low grade malignancies.
  • Thirteen patients developed a recurrence.
  • Distant metastases (ten patients) and intrahepatic recurrence (six) were the predominant sites of initial treatment failure.
  • Six patients received salvage chemotherapy.
  • With complete resection, patients with high-grade tumours had a 5-year survival rate of 18 (95 per cent confidence interval 5-62) per cent compared with 80 (52-100) per cent for patients with low-grade tumours.
  • CONCLUSION: Surgical resection is the only effective therapy for primary hepatic sarcomas at present.
  • Better adjuvant therapy is necessary, especially for high-grade malignancies, owing to the high failure rate with operation alone.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antineoplastic Agents / therapeutic use. Disease-Free Survival. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Preoperative Care / methods. Retrospective Studies

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  • (PMID = 11091236.001).
  • [ISSN] 0007-1323
  • [Journal-full-title] The British journal of surgery
  • [ISO-abbreviation] Br J Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] ENGLAND
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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44. Cardillo G, Carbone L, Carleo F, Masala N, Graziano P, Bray A, Martelli M: Solitary fibrous tumors of the pleura: an analysis of 110 patients treated in a single institution. Ann Thorac Surg; 2009 Nov;88(5):1632-7
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  • [Title] Solitary fibrous tumors of the pleura: an analysis of 110 patients treated in a single institution.
  • BACKGROUND: Solitary (localized) fibrous tumors of the pleura (SFTP) are rare slow-growing neoplasms that generally have a favorable prognosis.
  • METHODS: The records of 110 patients (63 men; mean age 56.4 years; range, 17 to 79) surgically treated for SFTP from July 1990 to February 2008, were evaluated for demographics, operative procedure, histopathology, morbidity, mortality, postoperative chemotherapy or radiotherapy, and long-term follow-up.
  • The main surgical approach was video-assisted thoracoscopic surgery (69 procedures with a conversion rate of 14.5%); 40 patients underwent thoracotomy and 1 had sternotomy.
  • The visceral pleura was the site of origin in 95 tumors, the parietal pleura in 13, the mediastinal pleura in 2 cases.
  • Sixty-three tumors were pedunculated, 35 were sessile, and 12 were inverted fibroma.
  • Tumors were pathologically benign in 95 cases (86.4%), and malignant in 15 (13.6%).
  • Symptomatic patients presented with malignant tumors more often than asymptomatic (19.1% versus 9.5%).
  • The overall disease-free survival rate was 90.8% (95.7% in benign cases and 67.1% in malignant cases; p < 0.05).
  • Eight patients presented with recurrence of disease, 4 cases of which were malignant and 4 were benign.
  • CONCLUSIONS: Solitary fibrous tumor of the pleura is a rare disease that includes both benign and malignant variants.The outcome is mostly benign, with an overall 10-year survival rate of 97.5%.
  • Pathologically benign lesions show a better disease-free survival rate than malignant lesions (95.7% versus 67.1%; p < 0.05).
  • Surgery is the gold standard of treatment as neither radiotherapy nor chemotherapy proved to be effective.
  • [MeSH-major] Solitary Fibrous Tumor, Pleural / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Female. Humans. Male. Middle Aged. Treatment Outcome

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  • (PMID = 19853123.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Netherlands
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45. De Pas T, Toffalorio F, Colombo P, Trifirò G, Pelosi G, Vigna PD, Manzotti M, Agostini M, de Braud F: Brief report: activity of imatinib in a patient with platelet-derived-growth-factor receptor positive malignant solitary fibrous tumor of the pleura. J Thorac Oncol; 2008 Aug;3(8):938-41
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Brief report: activity of imatinib in a patient with platelet-derived-growth-factor receptor positive malignant solitary fibrous tumor of the pleura.
  • Malignant solitary fibrous tumor (MSFT) of the pleura is a rare neoplasm, with unpredictable biologic behavior and a low sensitivity to chemotherapy.
  • To the authors' knowledge, no other effective medical treatment is available for this disease.
  • We report the first evidence of the activity of imatinib in a symptomatic patient with a chemo- and radio-resistant advanced MSFT, who obtained a 21-months lasting major clinical benefit with a consistent reduction in tumor metabolism.
  • Immunostaining of tumor cells demonstrated the positivity for PDGFR-alpha and PDGFR-beta and the absence of c-KIT over-expression, in the absence of c-KIT and PDGRFR mutations; all the cells strongly and diffusely expressed the ligand PDGF A in the cytoplasm.
  • This profile suggests that the observed tumor response was mediated through the inhibition of the tyrosine kinase activity of PDGFR.
  • Treatment with imatinib should be considered for patients with recurrent or unresectable MSFTs with PDGFR expression.
  • [MeSH-major] Piperazines / therapeutic use. Pleural Neoplasms / drug therapy. Protein Kinase Inhibitors / therapeutic use. Pyrimidines / therapeutic use. Receptor, Platelet-Derived Growth Factor alpha / metabolism. Receptor, Platelet-Derived Growth Factor beta / metabolism. Solitary Fibrous Tumor, Pleural / drug therapy
  • [MeSH-minor] Adult. Benzamides. Female. Humans. Imatinib Mesylate. Immunoenzyme Techniques. Pneumonectomy. Protein-Tyrosine Kinases / antagonists & inhibitors. Proto-Oncogene Proteins c-kit / metabolism. Tomography, X-Ray Computed

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  • (PMID = 18670317.001).
  • [ISSN] 1556-1380
  • [Journal-full-title] Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer
  • [ISO-abbreviation] J Thorac Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Benzamides; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Proto-Oncogene Proteins c-kit; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor beta
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