A systematic review of proton therapy in the treatment of chondrosarcoma of the skull base (original) (raw)

Abstract

Chondrosarcoma (CSA) of the skull base (SB) is an uncommon, slowly growing, neoplasm comprising approximately 0.1% of all intracranial tumors and 6% of SB lesions. Even though its growth is slow, CSA is a potentially lethal tumor. The therapeutic approach to CSA of the SB is still controversial and clinical experience is limited because of the relative rarity of this tumor. The use of proton therapy (PT) after maximal surgery is widely accepted, but there are no controlled studies demonstrating the need of PT and its superiority in comparison to radiotherapy with photons. We conducted a systematic review of the scientific literature published during the period between January 1980 and June 2008 on data regarding irradiation of CSA of the SB with PT and a series of inclusion criteria. During August 2008, two independent reviewers (M.A. and D.A.), by applying the key words "skull base", "chondrosarcoma", and "proton therapy" selected those studies from the PubMed database in which a minimum of ten patients received palliative, radical, or postoperative irradiation with protons and which furnished a minimum of 24 months of follow-up. Forty nine reports were retrieved. There were no prospective trials (randomized or nonrandomized) but just nine uncontrolled single-arm studies for PT mainly related to advanced and frequently incompletely resected tumors. According to the inclusion criteria, only four articles, reporting the most recent updated results of the publishing institution, were included in the analysis providing clinical outcomes for 254 patients in total. Therapeutic approach to CSA of the SB has traditionally relied on surgical control. Radiation therapy has demonstrated to be a valuable modality for local control in the postoperative setting or in advanced/inoperable cases treated with definitive intent. The use of PT following maximal surgical resection shows a very high probability of medium- and long-term cure with a relatively low risk of significant complications.

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Authors and Affiliations

  1. ATreP, Agenzia Provinciale per la Protonterapia, Via F.lli Perini, 181, 38100, Trento, Italy
    Maurizio Amichetti, Dante Amelio & Marco Cianchetti
  2. Department of Radiation Oncology, S. Andrea Hospital and University, La Sapienza, Rome
    Riccardo Maurizi Enrici
  3. Department of Radiation Oncology, S. Andrea Hospital, Rome, Italy
    Giuseppe Minniti

Authors

  1. Maurizio Amichetti
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  2. Dante Amelio
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  3. Marco Cianchetti
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  4. Riccardo Maurizi Enrici
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  5. Giuseppe Minniti
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Correspondence toMaurizio Amichetti.

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Markus Gross, Basel, Switzerland

Chondrosarcomas usually progress slowly resulting in relatively asymptomatic growth that leads to a late diagnosis. Therefore, extensive infiltration of the skull base and the surrounding critical structures are often seen when the diagnosis is confirmed. Due to the preferentially local spreading and the rarity of distant metastases, the goal of the primary therapy is to gain a long-lasting local control. Complete microsurgical removal is considered the gold standard in treatment of these tumors. Unfortunately, radical surgical resection without risk of severe postoperative morbidity is mostly impossible (Sekhar et al., 2001). In addition to that, the likelihood of microscopic residual disease is high.

In this paper, Amichetti and co-workers focus on this very rare entity of skull base chondrosarcomas. They reviewed the literature concerning different available radiation techniques used in the treatment of this disease, especially on proton irradiation and the comparison with photon or heavy ion particle therapy.

One may congratulate the authors on their effort in analyzing the literature about this very seldom tumor. In defiance of this enthusiasm, the question arises if there are substantial advantages of proton compared with photon irradiation in these tumors.

Percutaneous irradiation of skull base chondrosarcomas is administered either postoperatively as an additive procedure to prolong local control by slowing down the regrowth of the remaining microscopic tumor or it is part of a multidisciplinary approach of limited surgical radicality and ensuing irradiation to limit the overall treatment side effects. Radiotherapy also is used as a definitive treatment in situations where resection is not feasible, or where it could cause unacceptable morbidity and/or functional outcome. Conventional photon radiotherapy usually is limited to doses of 50-55 Gy due to the partial involvement of the surrounding tissue in the treated volume. This did not provide sufficient tumor control because chondrosarcomas are relatively radioresistant tumors requiring doses of at least 65 Gy. However, higher doses would bear the risk of substantial side effects. When, on the other hand, a sufficient radiation dose can be delivered to the tumor without exceeding the tolerance of the surrounding tissues, the photon radiotherapy is as effective as particle therapy. Steep dose gradients from the target volume to the periphery fulfil these prerequisites, and they are put into practice by modern techniques like stereotactic irradiation, intensity-modulated radiotherapy (IMRT) and image-guided radiotherapy. They allow for delivering higher doses in the tumor without a higher amount of side effects. The advantage of IMRT compared with 3D conformal radiotherapy is that dose delivery can be further sculpted to conform to the tumor volumes. Radiation doses of >65 Gy given by fractionated stereotactic radiotherapy are known to provide 5-year local control rates of approximately 70-100% with tolerable toxicity to surrounding neural tissue (Debus, 2000; Den, 2008). Therefore, by means of effectiveness photon therapy seems to be inferior in a part of the patients only.

However, these photon irradiation techniques result in a substantially greater volume of normal tissue receiving intermediate to low doses of radiation compared to proton therapy. Nevertheless, the overall conformality of radiation is similar for both modalities (Feuvret, 2007). The clinical benefit of this observed reduced integral dose to these structures is uncertain, but most notably children and young adults suffering from skull base chondrosarcomas should be treated by particle therapy.

References

1. Debus J, Schulz-Ertner D, Schad L, Essig M, Rhein B, Thilmann CO, Wannenmacher M (2000) Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47:591-596

2. Den RB, Goldberg Y, Werner-Wasik M, Corn BW, Daniel SR, Andrews DW, Evans J, Breneman R, Warnick R, Curran WJ (2008) Photon-based fractionated radiotherapy and radiosurgery for the treatment of chordomas and chondrosarcomas: A multi-institutional review. Int J Radiat Oncol Biol Phys 72:204

3. Feuvret L, Noel G, Weber DC, Pommier P, Ferrand R, De Marzi L, Dhermain F, Alapetite C, Mammar H, Boisserie G, Habrand JL, Mazeron JJ (2007) A treatment planning comparison of combined photon–proton beams versus proton beams–only for the treatment of skull base tumors. Int J Radiat Oncol Biol Phys 69:944-954

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Didier Frappaz, Lyon, France

This paper on the treatment of chondrosarcoma of the skull base by radiation therapy is a nice review of current literature. As usual, from 49 papers, only four are selected, but one includes nearly 90% of cases that represents a major but unavoidable bias. It deals with the usual pitfalls found when reviewing skull base tumors: papers that report heterogeneous series, mixing different pathologies such as chordomas; lack of accurate reporting of prospectively collected long-term toxicities; and lack of homogeneous indications and techniques.

Time has come for prospective trials that should be organized as phase II trials with local efficacy as primary endpoint and toxicity as one of the secondary endpoints.

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Amichetti, M., Amelio, D., Cianchetti, M. et al. A systematic review of proton therapy in the treatment of chondrosarcoma of the skull base.Neurosurg Rev 33, 155–165 (2010). https://doi.org/10.1007/s10143-009-0235-z

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