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1. Staals EL, Bacchini P, Bertoni F: Dedifferentiated central chondrosarcoma. Cancer; 2006 Jun 15;106(12):2682-91
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  • METHODS: In this retrospective study, the clinical, radiographic, and histologic features and the treatments in 123 patients from the Rizzoli Institute were reviewed in an attempt to define which factors may be related to outcome in patients with dedifferentiated central chondrosarcoma.
  • The femur (62 patients), pelvis (28 patients), and humerus (20 patients) were the most common locations.
  • Radiographically, a soft tissue mass was present in 87% of patients, and a bimorphic pattern was appreciated in 53% of patients.
  • For 101 patients, surgery was a component of their definitive management.
  • In 25 patients, surgery was combined with chemotherapy.
  • CONCLUSIONS: Metastatic disease at diagnosis, malignant fibrous histiocytoma dedifferentiation, and a high percentage of dedifferentiated component were related to poorer outcomes.
  • There was no statistical evidence of any beneficial effect from adjuvant chemotherapy.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Cell Differentiation. Combined Modality Therapy. Drug Therapy. Female. Femur / pathology. Histiocytoma, Malignant Fibrous / pathology. Histiocytoma, Malignant Fibrous / radiography. Histiocytoma, Malignant Fibrous / therapy. Humans. Humerus / pathology. Male. Middle Aged. Neoplasm Metastasis. Pelvis / pathology. Prognosis. Retrospective Studies. Survival Analysis. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • [Copyright] Copyright 2006 American Cancer Society.
  • (PMID = 16691621.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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2. Dantonello TM, Int-Veen C, Leuschner I, Schuck A, Furtwaengler R, Claviez A, Schneider DT, Klingebiel T, Bielack SS, Koscielniak E, CWS study group, COSS study group: Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults: experiences of the CWS and COSS study groups. Cancer; 2008 Jun;112(11):2424-31
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  • [Title] Mesenchymal chondrosarcoma of soft tissues and bone in children, adolescents, and young adults: experiences of the CWS and COSS study groups.
  • BACKGROUND: Mesenchymal chondrosarcoma (MCS) is a rare tumor with a strong tendency toward late recurrences leading to reported 10-year survival rates below 50%.
  • The recommended treatment is resection with wide margins; the effectiveness of chemo- and radiotherapy remain poorly defined.
  • As reports about MCS in young patients are scarce, treatment and outcomes of children/adolescents/young adults in the CWS and COSS studies were investigated.
  • METHODS: Since 1977, 15 of >7000 CWS and COSS patients <or=25 years had a confirmed diagnosis of MCS.
  • Tumor sites were head/neck (n = 6), paravertebral (n = 3), pelvis (n = 3), limbs (n = 2), and kidney (n = 1).
  • Thirteen individuals received chemotherapy, 6 were irradiated.
  • Four recurrences occurred, all within 4 years from diagnosis (3 local, 1 combined; 2 of these in irradiated patients).
  • Seven of 8 patients whose tumors were completely resected during primary treatment, but only 4 of 7 patients with incomplete surgery survived disease-free.
  • CONCLUSIONS: This series of 15 MCS differs from others: the median age was young, most tumors were extraosseous, most patients received chemotherapy, and the outcome was better than published.
  • Treating MCS according to standard multimodal soft tissue/bone sarcoma regimens is proposed.
  • [MeSH-major] Bone Neoplasms / pathology. Chondrosarcoma, Mesenchymal / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Infant. Male. Neoplasm Staging. Prospective Studies. Radiotherapy Dosage. Survival Rate. Treatment Outcome

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  • [Copyright] (c) 2008 American Cancer Society.
  • (PMID = 18438777.001).
  • [ISSN] 1097-0142
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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3. Raney B, Stoner J, Anderson J, Andrassy R, Arndt C, Brown K, Crist W, Maurer H, Qualman S, Wharam M, Wiener E, Meyer W, Hayes-Jordan A, Soft-Tissue Sarcoma Committee of the Children's Oncology Group: Impact of tumor viability at second-look procedures performed before completing treatment on the Intergroup Rhabdomyosarcoma Study Group protocol IRS-IV, 1991-1997: a report from the children's oncology group. J Pediatr Surg; 2010 Nov;45(11):2160-8
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  • [Title] Impact of tumor viability at second-look procedures performed before completing treatment on the Intergroup Rhabdomyosarcoma Study Group protocol IRS-IV, 1991-1997: a report from the children's oncology group.
  • PURPOSES: The aims of the study were to compare results of clinical/radiographic studies before second-look procedures (SLP) with SLP specimens from patients with gross residual sarcoma at diagnosis and to relate tumor viability to outcome.
  • PATIENTS: Seventy-three patients underwent SLP before completing chemotherapy, with (n = 59) or without (n = 14) radiotherapy.
  • Tumor sites were bladder/prostate (n = 27), head/orbit/parameningeal (n = 22), extremity/trunk (n = 14), and retroperitoneum/pelvis (n = 10).
  • RESULTS: Of 14 patients, 1 (7%) with clinical/radiographic complete response (CR) had viable tumor.
  • Of 59 patients, 35 (59%) without CR had viable tumor.
  • Five-year failure-free survival (FFS) rates were 81% in 37 patients without viable tumor and 53% in 36 patients with viable tumor (Cox proportional hazards adjusted P = .05).
  • Five-year FFS rates were 67% in 15 patients with clear margins and 43% in 21 patients with tumor-involved margins (n = 18) or viable gross tumor (n = 3) (Cox proportional hazards adjusted P = .04).
  • Five-year survival was 78% to 79% among 73 patients with and 333 patients without SLP during treatment.
  • CONCLUSIONS: Second-look procedures can show whether viable tumor is present and may be beneficial in selected patients with rhabdomyosarcoma.
  • Disappearance of tumor (CR) usually correlated with no viable tumor at SLP.
  • However, 41% of patients without CR had no viable tumor.
  • Those without viable tumor had increased FFS but not survival compared to those with viable tumor.

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  • [Copyright] Copyright © 2010. Published by Elsevier Inc.
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  • (PMID = 21034938.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA-98543; United States / NCI NIH HHS / CA / U10 CA072989; United States / NCI NIH HHS / CA / U10 CA029511; United States / NCI NIH HHS / CA / CA-24507; United States / NCI NIH HHS / CA / CA-72989; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / CA-29511; United States / NCI NIH HHS / CA / U10 CA024507
  • [Publication-type] Clinical Trial, Phase IV; Comparative Study; Journal Article; Randomized Controlled Trial; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Other-IDs] NLM/ NIHMS265975; NLM/ PMC3128803
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4. Martin RC 2nd, Brennan MF: Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival? Arch Surg; 2003 Mar;138(3):281-5
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  • [Title] Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival?
  • The improvement in survival is primarily associated with the combination of surgery and chemotherapy.
  • HYPOTHESIS: Little is known about the outcome of adults with soft tissue ES or primitive neuroectodermal tumors (PNET).
  • Certain prognostic factors from soft tissue sarcomas (tumor size, tumor location, margin status, and initial presentation) in adults (>16 years) with ES/PNET will help to identify factors associated with outcome.
  • METHODS: Between July 1, 1982, and June 30, 2000, we identified 59 adult patients with primary soft tissue ES/PNET.
  • Clinicopathologic factors were correlated with the end points studied: patient factors, tumor factors, pathologic factors, status of surgical margins, adjuvant chemotherapy, and radiation therapy.
  • Median tumor size was 8 cm, with all lesions being high grade.
  • The most common site was the trunk (n = 22), with an even distribution of retroperitoneal, pelvis, buttock, and lower extremity (all n = 5).
  • The median follow-up was 29 months (range, 6-222 months), with local recurrence identified in 13 patients (22%), with a median time to recurrence of 15 months (range, 5-200 months).
  • Initial presentation was the only predictor of long-term survival, with primary tumor-only presentation having a 5-year survival of 60% (median not reached) compared with primary tumor plus metastatic disease having a 5-year survival of 33% (median, 17 months) (P =.02).
  • [MeSH-major] Neuroectodermal Tumors, Primitive, Peripheral / mortality. Sarcoma, Ewing / mortality. Soft Tissue Neoplasms / mortality
  • [MeSH-minor] Adolescent. Adult. Aged. Chemotherapy, Adjuvant. Female. Humans. Male. Middle Aged. Neoplasm Recurrence, Local

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  • (PMID = 12611575.001).
  • [ISSN] 0004-0010
  • [Journal-full-title] Archives of surgery (Chicago, Ill. : 1960)
  • [ISO-abbreviation] Arch Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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5. Gilbert NF, Cannon CP, Lin PP, Lewis VO: Soft-tissue sarcoma. J Am Acad Orthop Surg; 2009 Jan;17(1):40-7
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  • [Title] Soft-tissue sarcoma.
  • Soft-tissue sarcomas are rare malignancies of mesodermal origin.
  • Soft-tissue sarcomas of the extremities and pelvis are the most relevant to the orthopaedic surgeon.
  • The patient with extremity soft-tissue sarcoma typically presents with a painless, enlarging mass.
  • Advanced imaging techniques and biopsy, which are best done at tertiary referral centers, usually confirm the diagnosis.
  • Limb-sparing surgical resection is the mainstay of treatment.
  • The use of chemotherapy is controversial, and no standardized protocol has been established.
  • [MeSH-major] Sarcoma / diagnosis. Sarcoma / surgery. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / surgery
  • [MeSH-minor] Biopsy. Combined Modality Therapy. Diagnosis, Differential. Diagnostic Imaging. Humans. Neoplasm Staging. Prognosis. Radiotherapy, Adjuvant

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  • (PMID = 19136426.001).
  • [ISSN] 1067-151X
  • [Journal-full-title] The Journal of the American Academy of Orthopaedic Surgeons
  • [ISO-abbreviation] J Am Acad Orthop Surg
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 38
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6. Acosta JM, Tiao G, Stein JE, Mahour GH: Temporary relocation of testes to the anterior abdominal wall before radiation therapy of the pelvis or perineum. J Pediatr Surg; 2002 Aug;37(8):1232-3
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  • [Title] Temporary relocation of testes to the anterior abdominal wall before radiation therapy of the pelvis or perineum.
  • Recent advances in the treatment of pelvic malignancies in children has resulted in an increased life expectancy.
  • In the past, treatment of soft tissue sarcomas with simple surgical excision resulted in a recurrence rate of approximately 75%.
  • Combination of chemotherapy, radiotherapy, and surgery have significantly altered the outcome of advanced pelvic soft tissue sarcomas.
  • With the improved survival rate of patients treated with combination therapy, sterility secondary to radiation therapy has become an issue.
  • This alternative surgical approach should be considered in boys who are afflicted with a pelvic/perineal malignancy requiring radiation therapy.
  • [MeSH-major] Dimethylpolysiloxanes. Protective Clothing. Radiation Injuries / prevention & control. Radiation Protection / methods. Rhabdomyosarcoma, Embryonal / radiotherapy. Silicones. Soft Tissue Neoplasms / radiotherapy. Testis
  • [MeSH-minor] Adolescent. Buttocks. Child. Groin. Humans. Infant. Male. Neoplasm Recurrence, Local / prevention & control. Posture

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  • [Copyright] Copyright 2002, Elsevier Science (USA). All rights reserved.
  • (PMID = 12149714.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Dimethylpolysiloxanes; 0 / Silicones; 63148-62-9 / baysilon
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7. Wessalowski R, Schneider DT, Mils O, Hannen M, Calaminus G, Engelbrecht V, Pape H, Willers R, Engert J, Harms D, Göbel U: An approach for cure: PEI-chemotherapy and regional deep hyperthermia in children and adolescents with unresectable malignant tumors. Klin Padiatr; 2003 Nov-Dec;215(6):303-9
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  • [Title] An approach for cure: PEI-chemotherapy and regional deep hyperthermia in children and adolescents with unresectable malignant tumors.
  • BACKGROUND: Elevated temperatures of 40 - 44 degrees C increase the actions of various anticancer drugs including N-lost derivatives, cytotoxic antibiotics and platinum analoga.
  • In clinical usage thermochemotherapy (TCH) should facilitate surgical resection and ameliorate local tumor control.
  • Among these, 24 patients had extracranial non-testicular germ cell tumors and 15 patients soft tissue or chondrosarcomas.
  • INDICATION: locoregional relapse (n = 29) or unresectable tumor after neoadjuvant chemotherapy (n = 10).
  • Among these two groups, there were ten patients with poor response or progressive disease under primary or relapse chemotherapy.
  • Tumor site: pelvis (30), abdomen (4), head and neck (2), proximal leg (2) and lumbar spine (1).
  • TCH was followed by surgical tumor resection in 28/39 patients and/or radiotherapy in 13/39 patients.
  • CONCLUSION: TCH shows substantial therapeutic efficacy and facilitates complete tumor resection in 14 out of 28 operated patients.
  • Multimodal treatment including TCH, surgical resection and/or radiotherapy leads to sustained remission in the majority of patients with locoregional tumor recurrence.
  • The therapeutic effect is most pronounced, if TCH is administered at first relapse.
  • Therefore, a more valid assessment of treatment efficacy can only be made by a matched-pair comparison in cooperation with the clinical registers.
  • [MeSH-major] Abdominal Neoplasms / therapy. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Chondrosarcoma / therapy. Cisplatin / therapeutic use. Etoposide / therapeutic use. Germinoma / therapy. Head and Neck Neoplasms / therapy. Hyperthermia, Induced. Ifosfamide / therapeutic use. Lumbar Vertebrae. Pelvic Neoplasms / therapy. Sarcoma / therapy. Soft Tissue Neoplasms / therapy. Spinal Neoplasms / therapy
  • [MeSH-minor] Adolescent. Adult. Chemotherapy, Adjuvant. Chi-Square Distribution. Child. Child, Preschool. Combined Modality Therapy. Data Interpretation, Statistical. Female. Follow-Up Studies. Humans. Infant. Karnofsky Performance Status. Male. Neoadjuvant Therapy. Neoplasm Recurrence, Local. Time Factors. Treatment Outcome


8. Tran PT, Hara W, Su Z, Lin HJ, Bendapudi PK, Norton J, Teng N, King CR, Kapp DS: Intraoperative radiation therapy for locally advanced and recurrent soft-tissue sarcomas in adults. Int J Radiat Oncol Biol Phys; 2008 Nov 15;72(4):1146-53
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  • [Title] Intraoperative radiation therapy for locally advanced and recurrent soft-tissue sarcomas in adults.
  • PURPOSE: To analyze the outcomes of and identify prognostic factors for patients treated with surgery and intraoperative radiotherapy (IORT) for locally advanced and recurrent soft-tissue sarcoma in adults from a single institution.
  • Primary sites included retroperitoneum-pelvis (78%), extremity (8%), and other (14%).
  • Clinical characteristics were as follows: mean tumor size, 10 cm (range, 1-25 cm); high-grade histologic subtype (72%); and mean dose, 1,159 cGy (range, 600-1,600 cGy).
  • Postoperative radiation or chemotherapy was administered to 37% of IORT Sites and 32% of patients, respectively.
  • Outcomes measured were infield control (IFC), locoregional control (LRC), distant metastasis-free survival (DMFS), disease-specific survival (DSS), and treatment-related complications.
  • Prognostic factors found to be significant (p < 0.05) on multivariate analysis were prior DFI and tumor size for LRC, extremity location and leiomyosarcoma histologic subtype for DMFS, and prior DFI for DSS.
  • Our cohort had five Grade 3/4 complications associated with treatment or a 5-year Kaplan-Meier Grade 3/4 complication-free survival rate of 85%.
  • CONCLUSIONS: IORT after tumor reductive surgery is well tolerated and seems to confer IFC in carefully selected patients.
  • [MeSH-major] Neoplasm Recurrence, Local / radiotherapy. Neoplasm Recurrence, Local / surgery. Sarcoma / radiotherapy. Sarcoma / surgery. Soft Tissue Neoplasms / radiotherapy. Soft Tissue Neoplasms / surgery

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  • (PMID = 18394818.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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9. Petković M, Zamolo G, Muhvić D, Coklo M, Stifter S, Antulov R: The first report of extraosseous Ewing's sarcoma in the rectovaginal septum. Tumori; 2002 Jul-Aug;88(4):345-6
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  • Ewing's sarcoma is a highly malignant neoplasm of bone, which usually occurs during childhood.
  • The mass had a size of 9 x 6 cm, a soft tissue consistency, was partially movable and the patient felt the pain during palpation.
  • Computed tomography (CT) showed an inhomogeneous expansive mass in the rectovaginal septum measuring 8.7 x 6.1 cm, without any signs of rectum or bladder invasion.
  • The vascular structures of the pelvis were normal.
  • At laparotomy the process was judged inoperable and only biopsy of the tumor mass was carried out.
  • Histology showed a neoplasm with small, round to oval cells with scarce cytoplasm.
  • The patient was treated with chemotherapy followed by whole-pelvis external beam radiation and intracavitary brachytherapy.
  • RESULTS: A residual mass measuring 3.5 x 2.5 cm was visible on a control CT scan 18 months after treatment; however, the patient was feeling well and refused surgery to remove the residual mass.
  • [MeSH-major] Rectal Neoplasms / therapy. Sarcoma, Ewing / therapy. Vaginal Neoplasms / therapy

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  • (PMID = 12400989.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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10. Martin DR, Danrad R, Herrmann K, Semelka RC, Hussain SM: Magnetic resonance imaging of the gastrointestinal tract. Top Magn Reson Imaging; 2005 Feb;16(1):77-98
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  • Removal of fat signal for detection of enhancing normal and abnormal structures is critical.
  • Generalized protocol for comprehensive evaluation of the entire abdomen and pelvis can be used for the following bowel indications: type and severity of inflammatory bowel disease (IBD); identifying enteric abscesses and fistulae; preoperative staging of malignant neoplasms, including rectal carcinoma; differentiating postoperative and radiation therapy changes from recurrent carcinoma; follow-up evaluation of metastases response to localized ablative or systemic chemotherapy.
  • Drawbacks of this technique include: artifacts related to extreme sensitivity to field inhomogeneity, including air-soft tissue interfaces at the patient skin surface, and from retained bowel gas; retained bowel gas is dark against dark bowel wall, impairing bowel wall assessment; and True-FISP does not provide sensitivity for edema, which is superior on single-shot echo-train imaging.
  • Small/large bowel indications for MRI include: inflammatory bowel disease, infectious disease including abscess evaluation or for appendicitis, inflammatory conditions including ischemia, and partial obstruction, malnutrition, and neoplasm search.
  • [MeSH-major] Contrast Media. Gastrointestinal Diseases / diagnosis. Gastrointestinal Tract / pathology. Image Enhancement / methods. Image Interpretation, Computer-Assisted / methods. Magnetic Resonance Imaging / methods

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  • (PMID = 16314698.001).
  • [ISSN] 0899-3459
  • [Journal-full-title] Topics in magnetic resonance imaging : TMRI
  • [ISO-abbreviation] Top Magn Reson Imaging
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media
  • [Number-of-references] 71
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11. Nahas CS, Akhurst T, Yeung H, Leibold T, Riedel E, Markowitz AJ, Minsky BD, Paty PB, Weiser MR, Temple LK, Wong WD, Larson SM, Guillem JG: Positron emission tomography detection of distant metastatic or synchronous disease in patients with locally advanced rectal cancer receiving preoperative chemoradiation. Ann Surg Oncol; 2008 Mar;15(3):704-11
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  • [Title] Positron emission tomography detection of distant metastatic or synchronous disease in patients with locally advanced rectal cancer receiving preoperative chemoradiation.
  • Choice of optimal treatment--neoadjuvant chemoradiation versus systemic chemotherapy alone--depends on accurate assessment of distant disease.
  • We prospectively evaluated the ability of [18F]fluorodeoxyglucose ([18F]FDG) positron emission tomography (PET) to detect distant disease in patients with locally advanced rectal cancer who were otherwise eligible for combined modality therapy (CMT).
  • Sites other than the rectum, mesorectum, or the area along the inferior mesenteric artery were considered distant and were divided into nine groups: neck, lung, mediastinal lymph node (LN), abdomen, liver, colon, pelvis, peripheral LN, and soft tissue.
  • Confirmation was based on tissue diagnosis, surgical exploration, and subsequent imaging.
  • Greatest accuracy was demonstrated in detection of liver (accuracy = 99.9%, sensitivity = 100%, specificity = 98.8%) and lung (accuracy = 99.9%, sensitivity = 80%, specificity = 100%) disease; PET detected 11/12 confirmed malignant sites in liver and lung.
  • All 10 were correctly staged by pre-CMT PET; abdominopelvic computed tomography (CT) scans accurately detected nine of them.
  • PET may play a significant role in defining extent of distant disease in selected cases, thus impacting the choice of neoadjuvant therapy.
  • [MeSH-major] Neoplasms, Multiple Primary / radionuclide imaging. Positron-Emission Tomography. Rectal Neoplasms / radionuclide imaging. Rectal Neoplasms / therapy
  • [MeSH-minor] Combined Modality Therapy. Female. Humans. Male. Middle Aged. Neoadjuvant Therapy. Neoplasm Metastasis. Neoplasm Staging. Prospective Studies. Single-Blind Method

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  • [ErratumIn] Ann Surg Oncol. 2008 Apr;15(4):1265. Leibold, Tobias [added]
  • (PMID = 17882490.001).
  • [ISSN] 1534-4681
  • [Journal-full-title] Annals of surgical oncology
  • [ISO-abbreviation] Ann. Surg. Oncol.
  • [Language] eng
  • [Grant] United States / PHS HHS / / R01 82534-01
  • [Publication-type] Clinical Trial; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
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12. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP: Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg; 2005 Jan;40(1):251-5
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  • [Title] Results of multimodal treatment for desmoplastic small round cell tumors.
  • We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT.
  • Data were collected on patient demographics, presenting symptoms, tumor location and extent, treatment regimen, and overall survival.
  • RESULTS: A majority of patients were male (91%), Caucasian (85%), and with a median age of 19 (7-58) years old at diagnosis.
  • In 63 patients (96%), the primary tumor was located in the abdomen or pelvis.
  • Thirty-three (50%) had positive lymph nodes and 27 (41%) had distant parenchymal metastases at diagnosis.
  • Twenty-nine of these patients (44%) underwent induction chemotherapy (P6), surgical debulking, and radiotherapy.
  • Three-year survival was 55% in those receiving chemotherapy, surgery, and radiotherapy vs 27% when all 3 modalities were not used (P < .02).
  • Gross tumor resection was highly significant in prolonging overall survival; 3-year survival was 58% in patients treated with gross tumor resection compared to no survivors past 3 years in the nonresection cohort (P < .00001).
  • CONCLUSIONS: Multimodal therapy results in improved survival in patients with DSRCT.
  • [MeSH-major] Sarcoma, Small Cell / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Abdominal Neoplasms / pathology. Abdominal Neoplasms / surgery. Abdominal Neoplasms / therapy. Adolescent. Adult. Antineoplastic Agents / therapeutic use. Child. Combined Modality Therapy. Female. Humans. Lymphatic Metastasis. Male. Middle Aged. Neoplasm Metastasis. Pelvic Neoplasms / pathology. Pelvic Neoplasms / surgery. Pelvic Neoplasms / therapy. Radiotherapy. Surgical Procedures, Operative. Survival Analysis. Testicular Neoplasms / pathology. Testicular Neoplasms / surgery. Testicular Neoplasms / therapy. Thoracic Neoplasms / pathology. Thoracic Neoplasms / surgery. Thoracic Neoplasms / therapy. Treatment Outcome

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  • (PMID = 15868593.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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13. Eroğlu A, Kürkçüoğlu IC, Karaoğlanoğlu N, Alper F, Gündoğdu C: Extraskeletal Ewing sarcoma of the diaphragm presenting with hemothorax. Ann Thorac Surg; 2004 Aug;78(2):715-7
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  • Ewing sarcoma is a relatively uncommon malignant bone neoplasm that usually occurs in children and young adults and involves the major long bones, pelvis, and ribs.
  • After drainage, a roentgenogram film, computed tomography, ultrasonography, and magnetic resonance image showed a giant mass on the right diaphragm.
  • Primary diaphragmatic tumor was resected totally by right posterolateral thoracotomy, and histologically, an extraskeletal Ewing sarcoma was identified.
  • The patient received adjuvant radio-chemotherapy, and there was no evidence of disease 10 months after the operation.
  • Although extremely rare, extraskeletal Ewing sarcoma should be kept in mind in the differential diagnosis of diaphragmatic soft tissue tumors.
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Child. Combined Modality Therapy. Dacarbazine / administration & dosage. Doxorubicin / administration & dosage. Dyspnea / etiology. Female. Hemothorax / etiology. Humans. Ifosfamide / administration & dosage. Mesna / administration & dosage. Neoplasm Invasiveness. Organ Specificity. Radiotherapy, Adjuvant. Remission Induction. Ribs / pathology

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  • (PMID = 15276562.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; NR7O1405Q9 / Mesna; UM20QQM95Y / Ifosfamide; MAID protocol
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14. Jeon IS, Yi DY: Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus. Pediatr Hematol Oncol; 2009 Mar;26(2):85-8
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  • [Title] Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus.
  • Acute lymphoblastic leukemia (ALL), a primary hematologic malignancy that is especially common in childhood, occurs relatively rarely as a secondary malignant neoplasm.
  • Available data indicate that ALL often follows chemoradiotherapy for soft tissue sarcoma.
  • Perivascular epithelioid tumor (PEComa), a primitive mesenchymal tissue origin, can be classified as a soft tissue sarcoma.
  • An 11-year-old girl was diagnosed with ALL secondary to chemoradiotherapy (vincristine, ifosfamide, and anthracycline) and radiotherapy comprising 45 Gy to the whole pelvis for PEComa.
  • ALL, FAB L2, and immunophenotypically pro-B developed 16 months after the final chemotherapy treatment.

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  • (PMID = 19322738.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Anthracyclines; 0 / Topoisomerase II Inhibitors
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15. Altmann S, Lenz-Scharf O, Schneider W: [Therapeutic options for aggressive fibromatosis]. Handchir Mikrochir Plast Chir; 2008 Apr;40(2):88-93
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  • [Title] [Therapeutic options for aggressive fibromatosis].
  • INTRODUCTION: Aggressive fibromatosis, e.g., desmoid tumour, is a rare neoplasm of the connective tissue with local infiltrative growth.
  • Because of the high recurrence rates and destruction of the surrounding tissue, these tumours are classified as semi-malignant.
  • Radical surgical treatment with tumour excision accompanied by radiotherapy is the current standard therapy that can be supplemented by pharmacological treatment in a few cases.
  • PATIENTS AND METHOD: We report on 9 patients (5 males and 4 females) with surgical therapy for aggressive fibromatosis.
  • The neoplasm was located on the extremities in 7 cases (4 x upper extremity, 3 x lower extremity), one tumour was situated in the chin and one in the rectus abdominis muscle.
  • All patients were treated with radical tumour resection.
  • RESULTS: In 6 cases complete tumour resection (R0) was achieved.
  • In 3 cases a total tumour resection was impossible (R1 resection).
  • The aggressive fibromatosis had infiltrated the pelvis in 2 cases implying a hemipelvectomy for R0 resection which was not practicable.
  • In these cases, an adjuvant radiotherapy with 25 x 2 Gy was started postoperatively after the accomplished wound healing.
  • Treatment with chemotherapeutic agents was not necessary.
  • DISCUSSION: Aggressive fibromatosis is a semimalignant neoplasm of the connective tissue with an extremely high recurrence rate.
  • Therapy of choice is the radical surgical resection.
  • Pharmacological treatment should be considered for patients with unsuccessful local therapy.
  • [MeSH-major] Fibromatosis, Aggressive / therapy. Soft Tissue Neoplasms / therapy
  • [MeSH-minor] Adult. Arm. Chin. Female. Follow-Up Studies. Humans. Leg. Magnetic Resonance Imaging. Male. Middle Aged. Postoperative Care. Radiotherapy Dosage. Radiotherapy, Adjuvant. Rectus Abdominis. Surgical Flaps. Time Factors

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  • (PMID = 18437666.001).
  • [ISSN] 0722-1819
  • [Journal-full-title] Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft für Mikrochirurgie der Peripheren Nerven und Gefässe : Organ der Vereinigung der Deutschen Plastischen Chirurgen
  • [ISO-abbreviation] Handchir Mikrochir Plast Chir
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Germany
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16. Paulino AC, Mayr NA, Simon JH, Buatti JM: Locoregional control in infants with neuroblastoma: role of radiation therapy and late toxicity. Int J Radiat Oncol Biol Phys; 2002 Mar 15;52(4):1025-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Locoregional control in infants with neuroblastoma: role of radiation therapy and late toxicity.
  • Primary tumor was located in the adrenal gland in 26 (49%), abdomen/nonadrenal in 14 (26%), thorax in 9 (17%), neck in 2 (4%), and pelvis in 2 (4%).
  • All patients, except 11 with Stage 4S and 4 with Stage 4, had resection of the primary tumor.
  • Postoperative doses ranged from 15 to 25 Gy whereas preoperative doses ranged from 12 to 31 Gy using a median fraction size of 1.5 Gy.
  • Chemotherapy was employed in 22 of 53 patients (42%) with the most common agents being cyclophosphamide in 22 and doxorubicin in 11.
  • RESULTS: Tumor control: The 5-year overall and freedom from progression rates were 79 +/- 9% and 81 +/- 11%; the locoregional control rate was 88 +/- 9%.
  • Five of 6 infants <6 months of age and 1 of 7 >/=6 months developed musculoskeletal toxicity.
  • Musculoskeletal effects were seen in 6 RT patients and included bony hypoplasia in 6, scoliosis in 5, soft tissue hypoplasia in 3, slipped capital femoral epiphysis in 2, kyphosis in 1, and osteochondroma in 1.
  • Three required orthopedic intervention, all receiving >/=20 Gy.
  • One child developed bowel obstruction at 21 months and another developed a leiomyosarcoma in the treatment field 34 years after RT.
  • [MeSH-minor] Abdominal Neoplasms / mortality. Abdominal Neoplasms / pathology. Abdominal Neoplasms / radiotherapy. Combined Modality Therapy. Disease Progression. Female. Humans. Infant. Infant, Newborn. Lymphatic Metastasis. Male. Musculoskeletal Diseases / etiology. Neoplasm Staging. Pelvic Neoplasms / mortality. Pelvic Neoplasms / pathology. Pelvic Neoplasms / radiotherapy. Radiation Injuries / complications. Survival Rate. Thoracic Neoplasms / mortality. Thoracic Neoplasms / pathology. Thoracic Neoplasms / radiotherapy. Treatment Failure

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  • [CommentIn] Int J Radiat Oncol Biol Phys. 2002 Dec 1;54(5):1575; author reply 1575 [12459387.001]
  • (PMID = 11958898.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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17. Corgna E, Betti M, Gatta G, Roila F, De Mulder PH: Renal cancer. Crit Rev Oncol Hematol; 2007 Dec;64(3):247-62
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  • In Europe, renal cancer (that is neoplasia of the kidney, renal pelvis or ureter (ICD-9 189 and ICD-10 C64-C66)) ranks as the seventh most common malignancy in men amongst whom there are 29,600 new cases each year (3.5% of all cancers).
  • Metastases are typically found in the lung, soft tissue, bone, liver, cutaneous sites, and central nervous system.
  • The most important staging technique is a computed tomography (CT) scan of the whole abdomen.
  • Intrinsic resistance to chemotherapy has long been a hallmark of renal carcinoma.
  • Limited options are available for the systemic therapy, and no chemotherapeutic regimen is accepted as a standard of care.
  • Biologic agents represent the major effective therapies for widespread metastatic renal cancer.
  • [MeSH-major] Kidney Neoplasms / diagnosis. Kidney Neoplasms / pathology. Kidney Neoplasms / therapy
  • [MeSH-minor] Humans. Neoplasm Staging. Prevalence. Prognosis

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  • (PMID = 17662611.001).
  • [ISSN] 1040-8428
  • [Journal-full-title] Critical reviews in oncology/hematology
  • [ISO-abbreviation] Crit. Rev. Oncol. Hematol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Ireland
  • [Number-of-references] 134
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18. Weber K, Damron TA, Frassica FJ, Sim FH: Malignant bone tumors. Instr Course Lect; 2008;57:673-88
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  • Malignant bone tumors represent a small percentage of cancers nationwide and also are much less common than malignant soft-tissue tumors.
  • The rarity of the condition makes it imperative that orthopaedic surgeons in nononcologic practices are able to recognize the symptoms that suggest a possible bony malignancy to avoid inappropriate or delayed treatment.
  • The primary symptom of a patient with a malignant bone tumor is pain, which often occurs at rest or at night.
  • Patients with a likely malignancy require thorough staging to determine the extent of disease and a well-planned biopsy for accurate diagnosis.
  • Knowledge of specific tumor characteristics and treatment options for osteosarcoma, Ewing's sarcoma, chondrosarcoma, malignant fibrous histiocytoma, chordoma, and adamantinoma is important.
  • Patients with osteosarcoma and resectable Ewing's sarcoma are treated with chemotherapy followed by surgical resection.
  • Secondary sarcomas can occur in previously benign bone lesions and require aggressive treatment.
  • Specific techniques are available for the resection of malignant bone tumors from the upper extremities, lower extremities, pelvis, and spine.
  • The care of patients with primary malignant bone tumors requires a multidisciplinary approach to treatment.
  • [MeSH-minor] Global Health. Humans. Morbidity. Neoplasm Staging / methods. Prognosis

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  • (PMID = 18399615.001).
  • [ISSN] 0065-6895
  • [Journal-full-title] Instructional course lectures
  • [ISO-abbreviation] Instr Course Lect
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Number-of-references] 42
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19. Ricke J, Sehouli J, Hach C, Hänninen EL, Lichtenegger W, Felix R: Prospective evaluation of contrast-enhanced MRI in the depiction of peritoneal spread in primary or recurrent ovarian cancer. Eur Radiol; 2003 May;13(5):943-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The purpose of this study was to assess the accuracy of MRI in the staging of intra-abdominal tumor seeding of ovarian carcinoma.
  • The MRI protocol included fat-saturated T1-weighted spin-echo (SE) sequences pre- and post i.v. application of gadopentetate dimeglumine.
  • The criteria for tumor manifestation was contrast enhancement of intra-abdominal soft tissue lesions or peritoneum.
  • Laparotomy and histopathology confirmed 251 abdominal tumor locations.
  • Sensitivity was high in lower pelvis locations (73-83%) except for ureter or bladder infiltration (40%).
  • Magnetic resonance imaging based on contrast-enhanced fat-saturated T1 SE sequences improves planning of cytoreduction preceding chemotherapy in advanced primary or relapsed ovarian carcinoma.
  • [MeSH-minor] Abdominal Neoplasms / diagnosis. Abdominal Neoplasms / secondary. Adult. Aged. Aged, 80 and over. False Positive Reactions. Female. Gastrointestinal Neoplasms / diagnosis. Gastrointestinal Neoplasms / secondary. Genital Neoplasms, Female / diagnosis. Genital Neoplasms, Female / secondary. Humans. Liver Neoplasms / diagnosis. Liver Neoplasms / secondary. Lymphatic Metastasis. Middle Aged. Neoplasm Recurrence, Local. Neoplasms, Second Primary. Pelvic Neoplasms / diagnosis. Pelvic Neoplasms / secondary. Predictive Value of Tests. Prospective Studies. Sensitivity and Specificity. Single-Blind Method. Women's Health

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  • (PMID = 12695813.001).
  • [ISSN] 0938-7994
  • [Journal-full-title] European radiology
  • [ISO-abbreviation] Eur Radiol
  • [Language] eng
  • [Publication-type] Comparative Study; Evaluation Studies; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Contrast Media
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