Radiotherapy for aneurysmal bone cysts : A rare indication.

BACKGROUND: Aneurysmal bone cysts (ABC) are rapidly growing benign osseous lesions composed of blood-filled channels separated by fibrous septa. Since the value of external beam radiotherapy (EBRT) for ABC has not been well defined, the German Cooperative Group on Radiotherapy for Benign Diseases performed the national register study described herein. PATIENTS AND METHODS: Five German institutions collected data regarding clinical features, treatment concepts, and outcome for patients with ABC who had been referred for local EBRT over the past 30 years. RESULTS: Between 1990 and 2015, 10 patients with ABC were irradiated (5 female/5 male). Median age was 23 years (range 14-40 years). Involved sites were: spine (n = 3), sacrum/pelvis (n = 2), shoulder/scapula (n = 2), humerus (n = 1), femur (n = 1), and radius (n = 1). The median EBRT total and fractional doses were 28 Gy (range 5-40 Gy) and 2 Gy (range 1-2 Gy), respectively. Median follow-up was 65 months (range 12-358 months). Persistent pain relief was achieved for all patients. However, long-term follow-up response data were only available for 7/10 patients. All 7 patients exhibited a radiological response and experienced no recurrent disease activity or pain during follow-up. Acute and late radiogenic toxicities ≥ grade 3 and secondary malignancies were also not observed. CONCLUSION: Primary or adjuvant EBRT seems to be an effective and safe treatment option for persistent or recurrent ABC. Fractionated doses below 30 Gy may be recommended.

DEGRO guidelines for the radiotherapy of non-malignant disorders : part III: hyperproliferative disorders.

BACKGROUND AND PURPOSE: Radiation therapy (RT) is an established and effective treatment modality in the management of a large variety of hyperproliferative disorders and benign neoplasms. Objective of this article is to summarize the updated DEGRO consensus S2e guideline recommendations. MATERIALS AND METHODS: This report comprises an overview of the relevant aspects of the updated guidelines with regard to treatment decision, dose prescription, and RT technique for a selected group of disorders including Morbus Dupuytren (MD)/Morbus Ledderhose (ML), keloids, Peyronie's disease (induratio penis plastica, IPP), desmoid tumors, pigmented villonodular synovitis (PVNS), symptomatic vertebral hemangiomas (sVH), and Gorham-Stout syndrome (GSS). On the basis of results in the literature, we attempted to classify the level of evidence (LoE) and the grade of recommendation (GR) according to the Oxford criteria. RESULTS: There is comprehensive evidence in the literature that RT is a reasonable and effective treatment modality for the treatment of all the above-mentioned disorders. The LoE varies from 2c to 4, and GR varies from A to C. CONCLUSIONS: The use of RT can be recommended for the interdisciplinary management of most of the reported disorders. It can be used in the primary treatment approach and as an effective adjunct to other treatment modalities or in some indications as a valuable alternative treatment option. We hope that the updated DEGRO S2e consensus guideline recommendations are a helpful tool for radiation oncologists in the clinical decision-making process.

DEGRO guidelines for the radiotherapy of non-malignant disorders. Part II: Painful degenerative skeletal disorders.

BACKGROUND AND PURPOSE: The purpose of this article is to summarize the updated DEGRO consensus S2e guideline recommendations for the treatment of benign painful degenerative skeletal disorders with low-dose radiotherapy. MATERIALS AND METHODS: This overview reports on the role of low-dose radiotherapy in the treatment of enthesiopathies (shoulder syndrome, trochanteric bursitis, plantar fasciitis, and elbow syndrome) and painful arthrosis (knee, hip, hand, and finger joints). The most relevant aspects of the DEGRO S2e Consensus Guideline Radiation Therapy of Benign Diseases 2014 regarding diagnostics, treatment decision, dose prescription as well as performance of radiotherapy and results are summarized. RESULTS: For all indications mentioned above, retrospective and some prospective analyses have shown remarkable effects in terms of pain relief. Nevertheless, the Level of Evidence (LoE) and the Grade of Recommendation (GR) vary: LoE 1b-4 and GR A-C. CONCLUSION: Low-dose radiotherapy for painful degenerative skeletal disorders is effective in the majority of the patients and therefore it may be a reasonable therapeutic alternative when simple and non-invasive methods have been used without persistent success. For all discussed entities, single fraction doses of 0.5-1.0 Gy and total doses of 3.0-6.0 Gy/series applied with 2-3 fractions per week are recommended.

Radiotherapy in langerhans cell histiocytosis - a rare indication in a rare disease.

INTRODUCTION: Langerhans Cell Histiocytosis (LCH) represents a rare benign disorder, previously designated as "Histiocytosis X", "Type II Histiocytosis" or "Langerhans Cell Granulomatosis". Clinical presentation includes osteolysis, ulcerations of skin and soft tissues but also involvement of the CNS is described.Because treatment concepts are not well defined the German Cooperative Group on Radiotherapy for Benign Diseases performed a retrospective analysis. METHODS AND MATERIAL: Eight closely cooperating centres collected patients' data of the past 45 years. As study endpoints disease free survival, recurrent disease, death and therapy related side effects were defined. RESULTS: A total of 80 patients with histologically proven LCH were irradiated within the past 45 years. According to the LCH classification of Greenberger et al. 37 patients had stage Ia, 21 patients stage Ib, 13 patients stage II and 9 patients stage IIIb and the median age was 29 years. The median Follow up was 54 months (range 9-134 months). A total of 39 patients had a surgical intervention and 23 patients a chemotherapy regimen.Radiation treatment was carried out with a median total dose of 15 Gy (range 3-50.4 Gy). The median single fraction was 2 Gy (range 1.8-3 Gy).Overall, 77% patients achieved a complete remission and 12.5% achieved a partial remission. The long-term control rate reached 80%. Within an actuarial overall 5-year survival of 90% no radiogenic side and late effects ≥EORTC/RTOG II° were observed. CONCLUSION: In the present study a large collective of irradiated patients was analysed. Radiotherapy (RT) is a very effective and safe treatment option and even low RT doses show sufficient local control.

Gorham-Stout syndrome of the pelvic girdle treated by radiation therapy: a case report.

BACKGROUND: The Gorham-Stout syndrome (GSS) is a rare, benign idiopathic and progressive disorder causing massive osteolysis due to a vascular hyperproliferation replacing the bony structure. Clinical experience concerning the efficacy of radiation therapy (RT) is limited to about 50 of an overall 200 cases reported worldwide. CASE REPORT: A 24-year-old bedridden woman had histologically proven GSS with destruction of the anterior pelvic girdle and received RT for a total dose of 45.0 Gy applied in 5 weekly fractions of 1.8 Gy. In addition, the patient received intravenously 4 mg zoledronic acid once a month. One year after the combined treatment, complete pain relief occurred, and the patient was able to walk without the use of appliances. Imaging studies revealed no progression of the osteolysis but only minimal signs of remineralization. CONCLUSION: Combined treatment with RT and bisphosphonate administration can prevent the progression of osteolysis in GSS. Total doses of 40-45 Gy are recommended.

Radiation therapy for Gorham-Stout syndrome: results of a national patterns-of-care study and literature review.

PURPOSE: The German Cooperative Group on Radiotherapy for Benign Diseases conducted a national patterns-of-care study to investigate the value of radiation therapy (RT) in the management of Gorham-Stout syndrome. METHODS AND MATERIALS: In 2009 a structured questionnaire was circulated to 230 German RT institutions to assess information about the number of patients, the RT indication and technique, and the target volume definition, as well as accompanying treatments, outcome data, and early or late radiation toxicity. RESULTS: In November 2009 responses were available from 197 departments (85.6%): 29 university hospitals (14.7%), 89 community hospitals (45.2%), and 79 private RT offices (40.1%). Of these institutions, 8 (4.0%) had experience using RT, for a total of 10 cases in various anatomic sites. Four patients underwent irradiation postoperatively, and six patients received primary RT. The total doses applied after computed tomography-based treatment planning ranged from 30 to 45 Gy. After a median follow-up period of 42 months, local disease progression was avoided in 8 cases (80.0%). In 2 of these cases a progression occurred beyond the target volume. Acute and late toxicity was mild; in 4 patients RT was associated with Grade I side effects according to Radiation Therapy Oncology Group/European Organisation for Research and Treatment of Cancer criteria. The literature analysis of 38 previously published articles providing results after the use of RT in 44 patients showed stable or regressive disease in 77.3%. CONCLUSIONS: RT may prevent disease progression effectively in Gorham-Stout syndrome in 77% to 80% of cases. Total doses ranging from 30 to 45 Gy applied after computed tomography-based treatment planning are recommended.

[The significance of radiation therapy for symptomatic vertebral hemangiomas (SVH)]. / Der Stellenwert der Strahlentherapie bei symptomatischen Wirbelkörperhämangiomen (sWKH).

PURPOSE: To evaluate the efficacy of radiation therapy (RT) for symptomatic vertebral hemangioma (SVH). MATERIAL AND METHODS: Based on the Registry for Rare Benign Disorders (RRBD) of the German Cooperative Group on Radiation Therapy for Benign Diseases (GCG-BD), the clinical information, treatment plans and outcome data from seven cooperating German RT institutions were analyzed retrospectively. RESULTS: Over a period of 39 years (1969-2008), a total of 84 patients with 96 symptomatic lesions underwent RT. The predominant indication was pain in 97.6%, and, in addition, 28.6% of patients had neurological deficits. The median total dose was 34 Gy (4.5-45 Gy), and the median single dose 2.0 Gy (0.5-3.0 Gy). After a median follow-up of 68 months (6-422 months), complete symptom relief (CR) occurred in 61.9% of patients, 28.6% had partial relief, and 9.5% had no relief (NR). The overall response rate (CR + PR) was 90.5%. In 26.2% of patients, radiologic signs of remineralization were noted. After a median follow-up of 70 months (8-124 months), symptom progression occurred in eight patients (9.5%). Therefore, the long-term rate of local control was 80.9%. Multivariate statistical analysis revealed a significantly higher rate of symptom relief and local control for total doses > or = 34 Gy. Side effects > RTOG/EORTC grade 2 were not observed. CONCLUSION: RT is a safe and effective for treatment of SVH. Total doses of at least 34 Gy are recommended to achieve optimal treatment response.

Radiation therapy for treatment of pigmented villonodular synovitis: results of a national patterns of care study.

PURPOSE: The German Cooperative Group on Radiotherapy in Benign Diseases (GCG-BD) conducted a pattern-of-care study (PCS) to analyze the radiation therapy (RT) practice for pigmented villonodular synovitis (PVNS). METHODS AND MATERIALS: In 2007, a structured questionnaire to assess the number of patients, the pretreatments, the RT indication, technique, target volume concepts, outcome data, and possible early or late toxicity was circulated to 227 institutions. RESULTS: Until August 2008, a response was available from 189 institutions (83.2 %), of whom 19 (10.0 %) experienced RT for PVNS. Complete clinical information was available for 41 patients from 14 RT departments. Thirty patients (73.2 %) received postsurgical RT because of primary incomplete resection, 11 patients (26.8 %) as an adjunct after complete resections of recurrences or unclear resection status. The total doses ranged from 30 to 50 Gy (median, 36 Gy), the median single dose was 2.0 Gy. Local control was achieved 95.1%, and 82.9% had no or only slight functional impairment. The early and late toxicity was mild (

[Radiotherapy in painful gonarthrosis. Results of a national patterns-of-care study]. / Strahlentherapie bei schmerzhafter Kniegelenkarthrose (Gonarthrose): Ergebnisse einer deutschen Patterns-of-Care-Studie

BACKGROUND AND PURPOSE: After a patterns-of-care study (PCS) in 2003/2004 addressing benign disorders in general, the German Cooperative Group on Radiotherapy for Benign Diseases (GCG-BD) conducted several multicenter cohort studies including the use of radiotherapy (RT) in painful gonarthrosis (GNA). MATERIAL AND METHODS: From 2006 to 2008, a PCS for GNA was conducted in all German RT institutions using a standardized structured questionnaire. Patient accrual, patient number, pretreatment, pain record, treatment indications, RT technique, and target volume concepts for painful GNA were assessed. In addition, the long-term functional and subjective outcomes were evaluated. RESULTS: 238/248 institutions (95.9%) returned the questionnaire: 50 (21%) reported no clinical experience with RT in GNA, while 188 (79%) institutions treated 4,544 patients annually (median 15; range one to 846 cases per institution). Indications for treatment were acute pain symptoms in 18.9%, chronic pain in 95.3%, and treatment-refractory pain in 81.1%. The median total dose was 6 Gy (range 3-12 Gy), with a median single dose of 1 Gy (0.25-3 Gy). 40.4% of the institutions applied two fractions and 51.4% three fractions weekly. RT was delivered with orthovoltage units (25%), linear accelerators (79.6%), and cobalt-60 units (8.3%). 42 institutions evaluated the long-term clinical outcome in a total of 5,069 cases. Median pain reduction for at least 3 months was reported in 60% (5-100%), median pain reduction for at least 12 months in 40% (10-100%), and median persistent pain reduction in 27.8% (10-85%) of the treated patients. In 30% of patients (7-100%), a second RT series was applied for inadequate pain response or early pain recurrence. No radiogenic acute or chronic side effects were observed. CONCLUSION: This PCS comprises the largest number of cases reported for RT in painful and refractory GNA. Despite variations in daily RT practice, high response and low toxicity for this treatment in a very large number of painful and refractory GNA cases renders low-dose RT an effective conservative therapy which can be applied prior to surgical procedures.

Radiation therapy for early stages of morbus Ledderhose.

PURPOSE: To evaluate the efficacy of radiation therapy (RT) in the treatment of early stages of benign plantar fibromatosis (Morbus Ledderhose [ML]). PATIENTS AND METHODS: From 2003 to 2008, 24 patients (33 sites) with a mean age of 52 years received RT for symptomatic ML. Prior to RT, 19 patients complained of pain and 15 had walking difficulties. 21 patients (28 sites) were irradiated with orthovoltage X-rays and three (five sites) received electron-beam irradiation. The RT protocol consisted of five weekly fractions of 3.0 Gy (15 Gy), repeated after 6 weeks to a total dose of 30 Gy in 20 patients (28 sites). In four patients (five sites), two single fractions of 4.0 Gy were applied, repeated at intervals of 4 weeks to total doses of 24-32 Gy. Primary study endpoints were the prevention of disease progression and the avoidance of a surgical intervention. Secondary endpoints were pain relief, improvement of gait, and patients' subjective satisfaction measured with a linear analog scale (LAS). RESULTS: After a median follow-up of 22.5 months, none of the patients experienced a progression of number and size of the lesions or the clinical symptoms. In eleven sites (33.3%) complete remission of cords or nodules occurred, in 18 (54.5%) a reduced number or size was noted, and four sites (12.1%) were unchanged. Pain relief was achieved in 13/19 patients (68.4%), and an improvement of gait abnormalities was noted in 11/15 patients (73.3%). The patients' subjective satisfaction measured by means of the LAS revealed a median improvement of 3.5 points in 22/24 patients (91.6%). Skin or soft tissues toxicities RTOG grade > 2 were not noted. CONCLUSION: RT is effective for treatment of the early stages of ML and may obviate the need for a surgical intervention. Long-term follow-up studies including a larger number of patients are required to define the role of RT in the management of this disorder.

Radiotherapy for symptomatic vertebral hemangiomas: results of a multicenter study and literature review.

PURPOSE: The current study analyzes the potential role of radiotherapy (RT) in symptomatic vertebral hemangioma (SVH). METHODS AND MATERIALS: Seven cooperating German institutions collected clinical information, treatment plans, and outcome data for all patients with SVH referred for local RT. RESULTS: From 1969 to 2008, a total of 84 patients with 96 symptomatic lesions were irradiated for SVH. The primary indication for radiotherapy was pain (97.6%), and 28.6% of patients had additional neurological symptoms. RT was performed at a median total dose of 34 Gy, with a median single dose of 2.0 Gy. After receiving a median follow-up of 68 months, the overall patient response rate was 90.5%. Complete symptom remission occurred in 61.9% of patients, 28.6% of patients had partial pain relief, and 9.5% of patients had no pain relief. In 26.2% of patients, radiological signs of reossification were observed in long-term follow-up but not significantly correlated with pain relief. Most importantly, total doses of >/=34 Gy resulted in significantly greater symptomatic relief and control rate than total doses of <34 Gy. CONCLUSIONS: This study consists of the largest database of cases reported so far using RT for SVH. RT is easy, safe, and effective for pain relief treatment for SVH. Total doses of at least 34 Gy give the best symptomatic response.

Radiation therapy for prevention of heterotopic ossification about the elbow.

PURPOSE: To evaluate the radiologic and functional outcome after prophylactic radiation therapy (RT) for prevention of heterotopic ossification (HO) about the elbow joint. PATIENTS AND METHODS: 20 patients with symptomatic HO were treated using perioperative single-dose RT of 7.0 Gy. 15 patients had excision of preexisting functionally relevant HO, and received RT for prevention of a recurrence, and five patients were treated prophylactically because of risk factors. In 13 patients RT was applied within 5 h preoperatively, seven patients received postoperative RT. The local control was evaluated by plain radiographs and the functional outcome was assessed by use of the Mayo Elbow Performance Score (MEPS). RESULTS: After a mean follow-up of 43.3 months, two patients had recurrence of the HO and were treated with further surgical resection. In the remaining 18 patients no progression of the HO was observed. At follow-up, the MEPS varied from 45 to 100 with 13 patients having no functional impairment (MEPS 100). Five patients experienced a slight limitation of the elbow with an MEPS ranging from 75 to 95, and only two had persistence of marked impairment with an MEPS of 45 and 50, respectively. Adverse effects related to use of RT were observed in none of the treated cases. CONCLUSION: Pre- or postoperative RT is effective for prevention of HO about the elbow joint and can be recommended as an integral component of interdisciplinary treatment in complicated elbow fractures. At this, the use of MEPS has proven to be a feasible method for evaluation of the functional outcome.

Interstitial high-dose-rate-brachytherapy in advanced esthesioneuroblastoma.

Esthesioneuroblastoma is a rare neuroectodermal tumor of the nasal vault with an aggressive biological behavior that is characterized by local recurrence, atypical distant metastasis, and poor long-term prognosis. The treatment regimen consists of surgical resection, radiation therapy, and chemotherapy in various, mainly stage-dependent, combinations. We report two cases of primary metastatic and locally recurrent disease, which were treated with computed tomography-guided interstitial high-dose-rate brachytherapy in palliative and curative intent, respectively. Computed tomography-guided interstitial high-dose-rate brachytherapy should be considered as a feasible treatment option for advanced esthesioneuroblastoma.

CT-guided interstitial HDR brachytherapy for recurrent glioblastoma multiforme. Long-term results.

BACKGROUND AND PURPOSE: Recurrences of glioblastoma multiforme (GBM) within previously irradiated volumes pose a serious therapeutic challenge. This retrospective study evaluates the long-term tumor control of recurrent GBM treated with interstitial high-dose-rate brachytherapy (HDR-BRT). PATIENTS AND METHODS: Between 1995 and 2003, 84 patients were treated for recurrent cerebral GBM located within previously irradiated volumes. All patients had received adjuvant external radiotherapy following primary surgery, with a focal dose up to 60 Gy. The median recurrent tumor volume was 51 cm(3) (3-207 cm(3)), and the HDR-BRT consisted of an afterloading (192)Ir implant which delivered a median dose of 40 Gy (30-50 Gy). Catheter implantation was implemented using interactive computed tomography (CT) guidance under local anesthesia and sedoanalgesia. RESULTS: After a median follow-up of 61 months, 5/84 patients (6%) were alive. The median post-BRT survival was 37 weeks, and the median overall survival 78 weeks. Moderate to severe complications occurred in 5/84 cases (6%). CONCLUSION: For patients with recurrences of GBM within previously irradiated volumes, CT-guided interstitial HDR-BRT is a feasible treatment option that can play an important role in providing palliation.

Radiation therapy for painful heel spurs: results of a prospective randomized study.

PURPOSE: To evaluate the efficacy of two different dose-fractionation schedules for radiation therapy (RT) in patients with painful heel spurs. PATIENTS AND METHODS: 130 patients were randomized into two groups: the low-dose (LD) group (n = 65 heels) received a total dose of 3.0 Gy given in two weekly fractions of 0.5 Gy; in the high-dose (HD) group (n = 65 heels), two weekly fractions of 1.0 Gy were applied over 3 weeks (total dose 6.0 Gy). In 24 sites of the HD group and 17 sites of the LD group, a second RT course was given. The results were assessed using a five-level function score which was documented before RT, at the end of each RT course, and at 6 weeks and 6 months thereafter. RESULTS: At 6-month follow-up, RT led to a highly significant reduction of symptoms in both groups. In the HD group, 31 sites were classified as excellent (score: 90-100), 13 as good (score: 70-85), twelve as moderate (score: 45-65), and nine as poor (score: 0-40). In the LD group, 35 sites were classified as excellent, eight as good, ten as moderate, and twelve as poor. The comparison of the difference of the sum score and the single criteria before RT and at 6 months after RT using the Wilcoxon-Mann-Whitney U-test revealed no statistically significant difference of response to RT between both groups. CONCLUSION: RT is an effective treatment option for the management of inflammatory heel spurs. The dose for an RT course should not exceed 3.0 Gy.

Pilomatrix carcinoma with lymph node and pulmonary metastases.

CASE REPORT: A 72-year-old male patient developed a locally recurrent pilomatrix carcinoma (PC) of the right upper shank. Within a 2-year period he also developed bilateral pulmonary as well as inguinal, cervical and paraaortic lymph node metastases. After external-beam radiation therapy (EBRT) alone, sole interstitial high-dose-rate (HDR) brachytherapy (BRT) or postoperative EBRT with interstitial HDR-BRT boost, the patient demonstrated regressive or stable disease at the lymphatic sites. Systemic chemotherapy with intravenous paclitaxel failed to assure a substantial pulmonary response. In the course of the disease after 28 months pulmonary progression has led to continuing clinical deterioration. CONCLUSION: In accordance with literature data the hitherto course of this case corroborates that radiation therapy (RT) modalities can play an important role in the treatment of PC.

[Functional outcome after megavoltage irradiation for heel spurs]. / Funktionelle Ergebnisse nach Megavoltbestrahlung beim Fersensporn.

PURPOSE: To evaluate results on the functional outcome and to determine prognostic factors and long-term response to low-dose megavoltage irradiation. PATIENTS AND METHODS: A total dose of 6.0 Gy given in two weekly fractions of 1.0 Gy was applied to 305 sites (252 patients). After 6 weeks, 97 sites (31.8%) received a second radiotherapy (RT) course. Assessment system was a function score which was documented before RT, at the end of each RT course, and at 6 weeks and 6 months after treatment. After an observation period of >/= 24 months, a follow-up examination was attempted to evaluate the late response. RESULTS: At 6-month follow-up, 85.6% responded with a score improvement. The outcome was excellent (score: 90-100) in 135/305 sites (44.3%), good (score: 70-85) in 60/305 sites (19.7%), moderate (score: 45-65) in 63/305 (20.7%) sites, and poor (score: 0-40) in 47/305 sites (15.4%). 231/305 sites (75.7%) had no or mild pain. 296/305 (97,0%) had no or only slight limitations in work and 253/305 (82,9%) in daily activities. 255/305 (83,6%) had no or slight discomfort in gait. The long-term follow-up after a mean observation period of 48.4 months revealed 15 recurrences (7.3%). The patients' age, sex, and the duration of symptoms before initiation of RT ( 6 months) did not prove to be prognostic factors. No early or late toxicity related to the use of RT was detected. CONCLUSION: Megavoltage 6-MV photon-beam irradiation is a safe, effective and long-acting treatment modality in the management of heel spur patients. The function score has been proven to be a feasible method in clinical practice for evaluation of treatment outcome.

Pigmented villonodular synovitis of the distal radioulnar joint.

CASE REPORT: A 32-year-old female patient underwent surgical synovectomy in the right distal radioulnar joint because of local recurrence of pigmented villonodular synovitis (PVNS) after previous surgery. 7 weeks after her second surgery she received fractionated radiation therapy (RT) to a total dose of 36.0 Gy given in five weekly fractions of 2.0 Gy. Apart from a mild skin erythema the patient had no early morbidity related to the use of RT. After a follow-up of 36 months no local recurrence was detected. CONCLUSION: In accordance with literature data the hitherto course of the presented case corroborates that RT is a safe and effective treatment option for the prevention of PVNS recurrence.