Base of skull chordomas in children and adolescents - A clinicopathologic study of 73 cases

Harvard University, Cambridge, Massachusetts, United States
American Journal of Surgical Pathology (Impact Factor: 4.59). 08/2006; 30(7):811-8. DOI: 10.1097/01.pas.0000209828.39477.ab
Source: PubMed

ABSTRACT Chordomas in children and adolescents comprise <5% of all chordomas and most frequently develop in the skull base. These tumors are believed to behave more aggressively than chordomas in adults and may have unusual morphology. This study examines a large series of pediatric skull base chordomas treated with a standardized protocol to characterize the behavior and morphology of these tumors. There were 31 males and 42 females ranging from 1 to 18 (mean 9.7) years. Forty-two cases (58%) were conventional chordomas, some of which had unusual histopathologic features. Chondroid chordomas comprised 23% of cases. Fourteen tumors (19%) were highly cellular and had a solid growth pattern with no myxoid matrix or lobular architecture. Eight of these had cytologic features of conventional chordoma cells including physaliferous cells (cellular chordoma). The remaining cellular tumors were composed of poorly differentiated epithelioid cells set in a fibrous stroma and lacked physaliferous cells (poorly differentiated chordoma). All variants studied by immunohistochemistry showed positive staining for cytokeratin, epithelial membrane antigen, S100 protein, and vimentin. Mitoses and necrosis were seen in all variants. Follow-up data were available for all patients and ranged from 1 to 21 (mean 7.25) years. The survival rate was 81%. All but 1 patient with poorly differentiated chordoma died of disease. Overall, base of skull chordomas in children and adolescents treated with proton beam radiation have better survival than chordomas in adults. However, poorly differentiated chordomas are highly aggressive tumors.

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    ABSTRACT: Въведение: Ръководството се състои от две основни глави- „ Лъчелечение на нетуморните заболявания“ и „Лъчелечение на доброкачествени тумори“. След всяка от главите с Библиография. Ръководството е предназначено за широк кръг от лекари- лични лекари, редица специалисти (кожни, очни, ушни, невролози, педиатри и т.н.), разбира се и за лъчетерапевти с цел разширяване на техните познания. След обширен литературен обзор от световната практика и международно приети консенсуси, както и от нашия скромен опит, се постарахме да представим ефективното лъчелечение при доброкачествените заболявания. Нашата основна цел е чрез разширяване на познанията на медицинските специалисти да подобрим качеството на живот на много пациенти, които действително се нуждаят от качествено лъчелечение. Въпреки риска от късни лъчеви реакции (предимно от страна на кожата, карциногенеза и генетични промени) след облъчване с йонизиращи лъчения, ЛЛ продължава да е актуално, приемливо и ефикасно за редица доброкачествени заболявания. След натрупан световен опит чрез редица рандомизирани проспективни проучвания, бе отчетен траен противовъзпалителен и противоболков ефект при много възпалителни и ставнодегенеративни заболявания, както и благоприятен ефект върху ставната подвижност. Изключително важно е трайното лъчелечебно подобряване на качеството на живот при много пациенти. Високата лъчерезистентност на доброкачествените тумори, често налага ЛЛ на малки туморни обеми (КМО) с високи канцерицидни дози (достатъчни за унищожаване на диференцираната туморна клетка). Лъчелечението се базира на подобни на онкологичните лъчетерапевтични изисвания и стандарти. Голяма част от доброкачествените тумори са дълбоко разположени, факт изискващ високоенергийно фотонно ЛЛ, генерирано от линеен ускорител. За максимална защита на околните нормални тъкани и органи, се налага конформално ЛЛ с модерна високоенергийна лъчетерапевична апаратура –ускорител или гаманайф, прилагане на високотехнологични лъчетерапевтични режими- стереотактично лъчелечение, три-D конформално ЛЛ и интензивно модулирано ЛЛ. Основната цел при ЛЛ на доброкачествените тумори е подтискане на клетъчната пролиферация, което определя ДОД – от 1,8-3 Gy и общата огнищна доза (ООД) 45-60Gy. Целта е постигане на туморна ерадикация или спиране на туморния растеж при значимо минимизиране на късната детерминирана лъчева токсичност на нормалните тъкани и органи.
    Галакта- Колор принт edited by Доц. д-р Лена Маринова,дмн, 05/2010; , ISBN: 978-957-92254-5-7
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    ABSTRACT: BACKGROUND The current study was conducted to evaluate the long-term results of irradiation with carbon ions in a raster scanning technique in patients with skull base chordomas. METHODS Between 1998 and 2008, a total of 155 patients (76 men and 79 women) with a median age of 48 years (range, 15 years-85 years) were irradiated with carbon ions using a raster scan technique. The irradiation was performed at the Society for Heavy Ion Research in Darmstadt, Germany. The median total dose was 60 gray (relative biological effectiveness) at 3 gray (relative biological effectiveness) per fraction. The median boost planning target volume was 70 mL (range, 2 mL-294 mL). Local control (LC) and overall survival (OS) were evaluated using the Kaplan-Meier method, whereas long-term toxicity was evaluated via questionnaires. RESULTSThe median follow-up was 72 months (range, 12 months-165 months). All patients had residual macroscopic tumors at the initiation of radiotherapy. The authors observed 55 local recurrences during follow-up, as well as systemic disease progression in 4 patients. The resulting 3-year, 5-year, and 10-year LC rates were 82%, 72%, and 54%, respectively, whereas the 3-year, 5-year, and 10-year OS rates were 95%, 85%, and 75%, respectively. Age <48 years and a boost volume >75 mL were associated with a significantly improved LC and OS. Primary treatment resulted in a significantly better OS probability. No higher late toxicity could be detected after carbon ion treatment. CONCLUSIONS Carbon ion therapy appears to be a safe and effective treatment for patients with skull base chordoma, resulting in high LC and OS rates. Cancer 2014;120:3410-3417. (c) 2014 American Cancer Society.
    Cancer 11/2014; 120(21). DOI:10.1002/cncr.28877 · 4.90 Impact Factor
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    ABSTRACT: Background Compared with photon therapy, proton-beam therapy (PBT) offers compelling advantages in physical dose distribution. Worldwide, gantry-based proton facilities are increasing in number, but no such facilities exist in Canada. To access PBT, Canadian patients must travel abroad for treatment at high cost. In the face of limited access, this report seeks to provide recommendations for the selection of patients most likely to benefit from PBT and suggests an out-of-country referral process. Methods The MEDLINE, EMBASE, PubMed, and Cochrane databases were systematically searched for studies published between January 1990 and May 2014 that evaluated clinical outcomes after PBT. A draft report developed through a review of evidence was externally reviewed and then approved by the Alberta Health Services Cancer Care Proton Therapy Guidelines steering committee. Results Proton therapy is often used to treat tumours close to radiosensitive tissues and to treat children at risk of developing significant late effects of radiation therapy (RT). In uncontrolled and retrospective studies, local control rates with PBT appear similar to, or in some cases higher than, photon RT. Randomized trials comparing equivalent doses of PBT and photon RT are not available. Summary Referral for PBT is recommended for patients who are being treated with curative intent and with an expectation for long-term survival, and who are able and willing to travel abroad to a proton facility. Commonly accepted indications for referral include chordoma and chondrosarcoma, intraocular melanoma, and solid tumours in children and adolescents who have the greatest risk for long-term sequelae. Current data do not provide sufficient evidence to recommend routine referral of patients with most head-and-neck, breast, lung, gastrointestinal tract, and pelvic cancers, including prostate cancer. It is recommended that all referrals be considered by a multidisciplinary team to select appropriate cases.
    Current Oncology 10/2014; 21(5):251-62. DOI:10.3747/co.21.2207 · 1.64 Impact Factor