Spinal cord ependymoma in children--results of postoperative radiotherapy.

PURPOSE: A retrospective study was performed to evaluate the results of postoperative radiation therapy of spinal cord ependymoma in children. METHODS AND MATERIALS: Between 1984 and 2005, 28 children with spinal cord ependymoma were treated with radiotherapy, after surgery and in three cases after chemotherapy as well. Median age at diagnosis was 13.3 years (range from 4.7 to 16.2 years). Ependymoma myxopapillare was identified in 13, ependymoma in 12 and anaplastic ependymoma in 3 cases. RESULTS: With a median follow-up of 8.7 years (range from 3 to 25 years) 22 patients were alive. The overall survival rate of 2, 5 and 10 years was 93%, 85% and 77% respectively, whereas progression free survival rate was 82%, 74% and 74% respectively. Patients with myxopapillary ependymoma had significantly better 5-year overall survival rate 100% than those with other histopathological types 60% (p=0.016). There were 2 relapse incidences observed among 13 patients with myxopapillary ependymoma, both underwent repeated surgery and reirradiation. In the group of 20 patients with gross total resection the overall 5-year survival rate was 100% in comparison with 62.5% with partial surgery, but it did not achieve statistical significance. CONCLUSIONS: The histological type of ependymoma myxopapillary was a statistical significant favourable prognostic factor. The gross total resection with adjuvant radiotherapy allows obtaining a high total survival rate.

Segmented photon beams technique for irradiation of postmastectomy patients.

AIM: To present the segmented photon beams technique (SPBT) for irradiation of postmastectomy patients. BACKGROUND: In majority of techniques for irradiation of posmastectomy patients, a few adjacent photon or electron beams were usually implemented in order to encompass different parts of the target. In the presented SPBT technique, the radiotherapy plan consists of 6 isocentric photon beams and the area CTV includes both the chest wall and the supraclavicular area. This makes it possible to provide a uniform dose to the CTV with no hot and cold points and enables the determination of doses for the entire volume of critical organs. METHODS AND MATERIAL: The treatment forward-IMRT plan comprises six isocentric 4 and 15 MV photon beams. Modulation of the dose distribution for each field was obtained by applying three segments on average. The total dose of 45 Gy was administered in 20 fractions. Dose distributions in target volume and organs at risk were evaluated for 70 randomly chosen patients. RESULTS: On average, 94.8% of the CTV volume received doses within 95-107% of the prescribed dose. The average volume of the heart receiving a dose of 30 Gy and lager was 2% for patients with left breast cancer. The average dose to the lung on the irradiation side was always lower than 15.5 Gy and the average V20 Gy was below 35.5%. CONCLUSIONS: The SPBT complies with requirements for high dose homogeneity within the target volume and satisfactory level of sparing of organs at risk.

Treatment of stage I seminoma: 25 years of experience.

AIM OF THE STUDY: To review management and outcomes in patients with stage I seminoma after orchidectomy. MATERIAL AND METHODS: Between 1979 and 2004 a total 292 patients with stage I seminoma were treated with adjuvant chemotherapy or radiotherapy or were placed on surveillance. Median age at diagnosis was 36 years (range 20-69), with median follow-up 76.5 months (range 11-294). Of the patients, 200 (68.5%) were treated with adjuvant chemotherapy, 72 (24.6%) were irradiated and 20 (6.8%) were placed on surveillance. RESULTS: The probability of 5-year overall survival and relapse-free survival for the entire group was 100% and 95.1% respectively. The 5-year relapse-free survival for adjuvant chemotherapy was 97.2%, for radiotherapy 94.6%, and 31.4% for the surveillance group. Of 24 (8.4%) patients who had relapse in lymph nodes and/or internal organs, 14/20 patients were in the surveillance group. All patients who had a relapse were salvaged successfully with chemotherapy. The toxicity of chemotherapy and radiotherapy was acceptable. No severe reactions were observed. CONCLUSION: Our results confirm the excellent prognosis for patients with stage I seminoma after orchidectomy treated with adjuvant chemotherapy or radiotherapy. The high rate of relapse in our surveillance group suggests the necessity of adjuvant treatment.

[Adenoid cystic carcinoma of the head and neck--a 10 years experience]. / Rak gruczolowato-torbielowaty glowy i szyi--doswiadczenie 10 lat.

INTRODUCTION: Adenoid cystic carcinoma (ACC) was first described in 1853 and 1854 by three french scientists, Robin, Lorain and Laboulbène. ACC is a rare cancer of the head and neck (about 1%) and it comprises from 6% to 10% of all malignant tumors of the salivary glands. 15%­30% of these tumors are located in submandibular gland, 30% occur in small salivary glands (in the palate, tongue, mouth, nose, sinuses, mucous membrane of the cheeks) and about 6% in parotid gland. Because of its specificity ACC is a challenge for the doctor ­ it is usually recognized at an advanced stage, spreads along the nerves, it has a high propensity for recurrence, often gives distant metastases, 10-year survival are very small. AIMS: Aim of this study is the epidemiological and clinical analysis of patients with adenoid cystic carcinoma. MATERIAL AND METHODS: Includes a retrospective analysis of 21 cases of patients with ACC of the head and neck were hospitalized at the ENT Clinic of the Warsaw Medical University between 2001 and 2011. Data from medical records contain information about the location of the tumor, symptoms and clinical signs, diagnostic methods, method of treatment and local recurrences and distant metastases. RESULTS: most cases ACC occurred in the large salivary glands (14 cases), in 5 cases in the nasal cavity and maxillofacial region, in 2 cases in the tongue. In order to accurately determine the extent of the tumor, its relationship to adjacent structures was performed computed tomography and magnetic resonance imaging. Most of patients before treatment have been made biopsy of the tumor or download tissues by forceps for histopathological examination. Reported symptoms depend on the location of the tumor. In 20 cases the patients were treated by surgical therapy and followed by radiotherapy, in one case because of the extent of the tumor was sent to radiotherapy. CONCLUSIONS: Surgical treatment of ACC requires a wide margin of healthy tissues; For the risk of local recurrences affected positive surgical margins, skull base locations of primary tumor and perineural invasion; Because of unpredictable nature of the tumor (local recurrences, distant metastasis), observation of patients after treatment should be carried out permanently, along with periodic imaging studies.

Reirradiation of relapsed brain tumors in children.

AIM: The aim of this study was to evaluate toxicity and response to fractionated reirradiation (FR) of relapsed primary brain tumors in children. BACKGROUND: The treatment options for recurrent brain tumors in children previously irradiated are limited. Reirradiation is performed with fear due to the cumulative late CNS toxicity and the lack of a significant chance of cure. MATERIALS AND METHODS: Between 2008 and 2009, eight children with a median age of 14.5 years with a diagnosis of a recurrent brain tumor underwent reirradiation. Initially, all patients were treated with surgery, chemotherapy and radiotherapy. The median time to the first recurrence after the initial treatment was 19.5 months. Intervals between radiotherapy courses were in the range of 5-51 mos. All retreatments were carried out with 3D image-based conformal methods. The total prescription dose was 40 Gy in a fraction of 5 × 2 Gy/week. The total cumulative dose ranged from 65 to 95 Gy (median: 75 Gy). The median cumulative biologically effective dose was 144 Gy (range: 126-181 Gy). RESULTS: The median overall survival and progression free survival measured from the beginning of reirradiation was 17.5 and 6.5 months, respectively. During the first evaluation, four patients showed a complete or partial response, two did not respond radiologically. Two children were progressive at the time of reirradiation. Among children with progression that occurred during the first year after reirradiation, only two progressed in the treatment area. The repeated irradiation was well tolerated by all patients. No late complications have been observed. CONCLUSION: In the absence of other treatment possibilities, the fractionated reirradiation with highly conformal three-dimensional planning could be a therapeutic choice in case of recurrent brain tumors in children. The control of craniospinal dissemination remains to be the main problem.

Craniospinal radiotherapy in children: Electron- or photon-based technique of spinal irradiation.

BACKGROUND: The prone position and electron-based technique for craniospinal irradiation (CSI) have been standard in our department for many years. But this immobilization is difficult for the anaesthesiologist to gain airway access. The increasing number of children treated under anaesthesia led us to reconsider our technique. AIM: The purpose of this study is to report our new photon-based technique for CSI which could be applied in both the supine and the prone position and to compare this technique with our electron-based technique. MATERIALS AND METHODS: Between November 2007 and May 2008, 11 children with brain tumours were treated in the prone position with CSI. For 9 patients two treatment plans were created: the first one using photons and the second one using electron beams for spinal irradiation. We prepared seven 3D-conformal photon plans and four forward planned segmented field plans. We compared 20 treatment plans in terms of target dose homogeneity and sparing of organs at risk. RESULTS: In segmented field plans better dose homogeneity in the thecal sac volume was achieved than in electron-based plans. Regarding doses in organs at risk, in photon-based plans we obtained a lower dose in the thyroid but a higher one in the heart and liver. CONCLUSIONS: Our technique can be applied in both the supine and prone position and it seems to be more feasible and precise than the electron technique. However, more homogeneous target coverage and higher precision of dose delivery for photons are obtained at the cost of slightly higher doses to the heart and liver.

A literature review of the recent radiotherapy clinical trials in pediatric brain tumors.

Primary central nervous system neoplasms are the second malignancy in children following leukemia. Despite developments in neurosurgery and new drugs in chemotherapy, irradiation is an essential part of the management in most of pediatric brain tumors. A good treatment strategy should consider not only survival but also the quality of life. The new approach of radiotherapy and importance of new drugs in combined treatment are recently considered. This article summarizes the recent clinical trials conducted in pediatric brain tumors management. Results of randomized study of pre-irradiation chemotherapy versus radiotherapy alone for medulloblastoma were presented by SIOP/UKCCSG PNET-3. The French M-SFOP 98 protocol considered hyper-fractionated radiotherapy with reduced boost volume, without chemotherapy and estimated impact on early relapses and intellectual function. The influence of radiotherapy quality on survival in high-risk medulloblastoma patients was evaluated in POG Trial 9031. In the treatment of low-grade glioma in children the effectiveness of novel combination chemotherapy was considered. Role of new drugs as temozolamid, topotecan and RMP-7 was investigated in pediatric high grade glioma and brain stem tumors. Impact of combined treatment on outcome of intracranial germ-cell tumors was investigated as well.

Patterns of failure in children with medulloblastoma treated with 3D conformal radiotherapy.

BACKGROUND AND PURPOSE: Craniospinal irradiation for medulloblastoma is one of the most complex techniques employed in radiotherapy. Many reports stress the impact of irradiation quality on survival in these patients. Our report presents the outcome and patterns of failure for 95 patients treated with 3D conformal radiotherapy (3D-CRT). MATERIALS AND METHODS: From 1998 to 2003, 95 children with medulloblastoma received 3D conformal radiotherapy. All of them were previously treated with surgery and chemotherapy. The brain and upper spinal cord were treated with two lateral 6MV photon fields. In four patients, the cribriform plate was irradiated by the additional field. For primary tumour bed we applied two or three photon beams. Spinal cord was irradiated either with 18-20MeV electron fields or with a mixed beam. RESULTS: With a median follow-up of 48 months, 32/95 patients suffered a multifocal (21) or isolated (11) recurrence. We evaluated every primary site of failure. In all patients, the recurrence appeared within the isodose level of 95-100%. CONCLUSIONS: Patterns of failure in medulloblastoma patients treated with 3D conformal radiotherapy indicated that the relapse was mainly associated with poor response to pre-irradiation chemotherapy. We believe that 3D conformal radiotherapy allows avoiding failures, related to radiotherapy uncertainties.

Evaluation of quality of life in long-term survivors of paediatric brain stem tumors, treated with radiotherapy.

The quality of life in long-term survivors of paediatric brain stem tumors, treated with radiotherapy is evaluated. They suffer predominantly from pre-treatment neurological impairments, which seriously influence their quality of life. The most often observed treatment sequelae are pituitary insufficiency and hearing loss.

Medulloblastoma in childhood: Impact of radiation technique upon the outcome of treatment.

BACKGROUND: Medulloblastoma is an infratentorial primitive neuroectodermal tumour, diagnosed in paediatric population. The radiotherapy is an essential method of treatment for these tumours. The impact of technical quality of radiation therapy on survival was recently considered. In this study treatment-related variables are analysed with a special focus on radiotherapy technical factors. PROCEDURE: The population of 158 children with a diagnosis of medulloblastoma had been undergoing postoperative radiotherapy at MCMCC in Warsaw in the period 1983-1997. The medical data of these patients were analysed. Simulation films of the whole-brain irradiation fields were retrospectively reviewed at 112 patients. The distance from the field margin to the cribriform plate and to the floor of the temporal fossa was assessed and correlated with supratentorial relapse. RESULTS: Five-year overall survival (OS) and 5-year disease free survival (DFS) were 46% and 40%, respectively. In multivariate analysis gender (P = 0.008), neurological status before radiotherapy (P = 0.029), M-stage (P < 0.001) and sequence of craniospinal irradiation (P = 0.019) were significant prognostic factors for OS. For DFS significant factors were M-stage (P < 0.001) and neurological status (P < 0.001). The cranial fields were not fully correctly covered at 43% patients. The field incorrectness was correlated with isolated supratentorial failure (P = 0.049). CONCLUSIONS: Our results are similar to those published in literature. M0-stage was the most powerful favourable prognostic factor. Male gender and neurological status before radiation treatment were associated with poor survival. Also protracted radiotherapy and quality of radiation technique may have an impact on the outcome.

Evaluation of radiotherapy for pediatric CNS tumors.

Despite the recent progress in neurosurgery and development of new chemotherapy drugs, radiotherapy is still an essential method of combined treatment for pediatric central nervous system tumors. The new approach of radiotherapy, such as conformal and stereotactic methods have recently been developed. These new methods and recommendations for treatment of pediatric brain tumors, such as astrocytoma, medulloblastoma, and ependymoma are presented. The side effects of treatment are also considered. In addition, the perspective of future development of radiotherapy in central nervous system tumors is presented.