History of the rare cancer network and past research.

Approximately, twenty years ago, the Rare Cancer Network (RCN) was formed in Lausanne, Switzerland, to support the study of rare malignancies. The RCN has grown over the years and now includes 130 investigators from twenty-four nations on six continents. The network held its first international symposium in Nice, France, on March 21-22, 2014. The proceedings of that meeting are presented in two companion papers. This manuscript reviews the history of the growth of the RCN and contains the abstracts of fourteen oral presentations made at the meeting of prior RCN studies. From 1993 to 2014, 74 RCN studies have been initiated, of which 54 were completed, 10 are in progress or under analysis, and 9 were stopped due to poor accrual. Forty-four peer reviewed publications have been written on behalf of the RCN

COVID-19 Outbreak and Cancer Radiotherapy Disruption in Lombardy, Northern Italy

none37nononeJereczek-Fossa B.A.; Palazzi M.F.; Soatti C.P.; Cazzaniga L.F.; Ivaldi G.B.; Pepa M.; Amadori M.; Antognoni P.; Arcangeli S.; Buffoli A.; Beltramo G.; Berlinghieri S.; Bignardi M.; Bracelli S.; Bruschieri L.; Castiglioni S.; Catalano G.; Di Muzio N.; Fallai C.; Fariselli L.; Filippi A.R.; Gramaglia A.; Italia C.; Lombardi F.; Magrini S.M.; Nava S.; Orlandi E.; Pasinetti N.; Sbicego E.L.; Scandolaro L.; Scorsetti M.; Stiglich F.; Tonoli S.; Tortini R.; Valdagni R.; Vavassori V.; Marvaso G.Jereczek-Fossa, B. A.; Palazzi, M. F.; Soatti, C. P.; Cazzaniga, L. F.; Ivaldi, G. B.; Pepa, M.; Amadori, M.; Antognoni, P.; Arcangeli, S.; Buffoli, A.; Beltramo, G.; Berlinghieri, S.; Bignardi, M.; Bracelli, S.; Bruschieri, L.; Castiglioni, S.; Catalano, G.; Di Muzio, N.; Fallai, C.; Fariselli, L.; Filippi, A. R.; Gramaglia, A.; Italia, C.; Lombardi, F.; Magrini, S. M.; Nava, S.; Orlandi, E.; Pasinetti, N.; Sbicego, E. L.; Scandolaro, L.; Scorsetti, M.; Stiglich, F.; Tonoli, S.; Tortini, R.; Valdagni, R.; Vavassori, V.; Marvaso, G

Curative external beam radiotherapy in patients over 80 years of age with localized prostate cancer: a retrospective rare cancer network study

PURPOSE: To analyse tolerance and outcome of patients over 80 years of age who choose external beam radiation therapy to the prostate as a curative treatment. METHODS AND MATERIAL: We evaluated acute and late side effects, biological DFS (bDFS) and actuarial survival as well as causes of death in relation to the clinical status including co-morbidity, PSA value, Gleason score and modalities of external radiotherapy in patients with localised prostate cancer >80 years of age. RESULTS: From January 1990 to December 2000, 65 eligible cases (median age: 81) were treated by 12 different participating institutions in the Rare Cancer Network. Tumour stage was T1N0M0, T2N0M0 and T3N0M0 for 10, 40, and 15 patients, respectively. Median follow-up was 65 months (range 22-177). Five-year overall survival rate was 77% with a 5-year bDFS rate of 73%. The incidence of grade 3 early toxicity was 12% and 9% for urinary and digestive tract, respectively. CONCLUSIONS: Radiation therapy given with curative intent is well tolerated in this selected group of patients aged over 80 years with localised prostate cancer. Results in terms of survival do not suggest a deleterious impact of this treatment. Therefore the authors recommend that radiation therapy with curative intent should not be withheld in selected elderly patients with localised prostate cancer

Outcome and patterns of failure in testicular lymphoma: a multicenter Rare Cancer Network study.

PURPOSE: To assess the outcome and patterns of failure in patients with testicular lymphoma treated by chemotherapy (CT) and/or radiation therapy (RT). METHODS AND MATERIALS: Data from a series of 36 adult patients with Ann Arbor Stage I (n = 21), II (n = 9), III (n = 3), or IV (n = 3) primary testicular lymphoma, consecutively treated between 1980 and 1999, were collected in a retrospective multicenter study by the Rare Cancer Network. Median age was 64 years (range: 21-91 years). Full staging workup (chest X-ray, testicular ultrasound, abdominal ultrasound, and/or thoracoabdominal computer tomography, bone marrow assessment, full blood count, lactate dehydrogenase, and cerebrospinal fluid evaluation) was completed in 18 (50%) patients. All but one patient underwent orchidectomy, and spermatic cord infiltration was found in 9 patients. Most patients (n = 29) had CT, consisting in most cases of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) with (n = 17) or without intrathecal CT. External RT was delivered to scrotum alone (n = 12) or testicular, iliac, and para-aortic regions (n = 8). The median RT dose was 31 Gy (range: 20-44 Gy) in a median of 17 fractions (10-24), using a median of 1.8 Gy (range: 1.5-2.5 Gy) per fraction. The median follow-up period was 42 months (range: 6-138 months). RESULTS: After a median period of 11 months (range: 1-76 months), 14 patients presented lymphoma progression, mostly in the central nervous system (CNS) (n = 8). Among the 17 patients who received intrathecal CT, 4 had a CNS relapse (p = NS). No testicular, iliac, or para-aortic relapse was observed in patients receiving RT to these regions. The 5-year overall, lymphoma-specific, and disease-free survival was 47%, 66%, and 43%, respectively. In univariate analyses, statistically significant factors favorably influencing the outcome were early-stage and combined modality treatment. Neither RT technique nor total dose influenced the outcome. Multivariate analysis revealed that the most favorable independent factors predicting the outcome were younger age, early-stage disease, and combined modality treatment. CONCLUSIONS: In this multicenter retrospective study, CNS was found to be the principal site of relapse, and no extra-CNS lymphoma progression was observed in the irradiated volumes. More effective CNS prophylaxis, including combined modalities, should be prospectively explored in this uncommon site of extranodal lymphoma

Primary spinal epidural lymphoma: patients' profile, outcome, and prognostic factors: a multicenter Rare Cancer Network study

PURPOSE: To assess the clinical profile, treatment outcome, and prognostic factors in primary spinal epidural lymphoma (PSEL). METHODS AND MATERIALS: Between 1982 and 2002, 52 consecutive patients with PSEL were treated in nine institutions of the Rare Cancer Network. Forty-eight patients had an Ann Arbor stage IE and four had a stage IIE. Forty-eight patients underwent decompressive laminectomy, all received radiotherapy (RT) with (n = 32) or without chemotherapy (n = 20). Median RT dose was 36 Gy (range, 6-50 Gy). RESULTS: Six (11%) patients progressed locally and 22 (42%) had a systemic relapse. At last follow-up, 28 patients were alive and 24 had died. The 5-year overall survival, disease-free survival, and local control were 69%, 57%, and 88%, respectively. In univariate analyses, favorable prognostic factors were younger age and complete neurologic response. Multivariate analysis showed that combined modality treatment, RT volume, total dose more than 36 Gy, tumor resection, and complete neurologic response were favorable prognostic factors. CONCLUSIONS: Primary spinal epidural lymphoma has distinct clinical features and outcome, with a relatively good prognosis. After therapy, local control is excellent and systemic relapse occurs in less than half the cases. Combined modality treatment appears to be superior to RT alone

Outcome and prognostic factors in orbital lymphoma: a Rare Cancer Network study on 90 consecutive patients treated with radiotherapy.

To assess the outcome and prognostic factors in patients with orbital lymphoma treated by radiotherapy (RT). Between 1980 and 1999, 90 consecutive patients with primary orbital lymphoma were treated in 13 member institutions of the Rare Cancer Network. A full staging workup was completed in 56 patients. Seventy-eight patients had low-, 6 intermediate-, and 6 high-grade lymphoma, and 75 had a single orbital localization. All patients underwent RT with a median dose of 34.2 Gy (range 4.0-50.4). Eleven patients received chemotherapy in addition to RT. After RT, local control was achieved in 97% of the patients. Local progression occurred in 2% and local relapse 1%. The rate of systemic relapse was 20%, and 9% of the patients developed metachronous contralateral eye involvement. The 5-year disease-free survival, overall survival, and cause-specific survival rate was 65%, 78%, and 87%, respectively. In univariate analyses, the statistically significant favorable prognostic factors were younger age, low grade, normal erythrocyte sedimentation rate, absence of muscular infiltration, complete response to treatment, conjunctival localization, and normal lactate dehydrogenase value for overall survival, disease-free survival, and freedom from treatment failure. In multivariate analysis, the favorable factors were younger age and low grade for overall and disease-free survival; a favorable response, conjunctival localization, and complete staging were highly significant for disease-free survival and freedom from treatment failure. Neither the RT technique nor the total dose influenced the outcome. Cataract and xerophthalmia were the most prominent late toxicities. Moderate- to low-dose RT alone is able to control primary orbital lymphoma with low morbidity. A full staging workup is warranted in these patients. Prognostic factors were identified that could be useful in the overall management of this uncommon site of primary lymphoma

Treatment results of 165 pediatric patients with non-metastatic nasopharyngeal carcinoma: a Rare Cancer Network study

PURPOSE: This Rare Cancer Network (RCN) study was performed in pediatric nasopharyngeal carcinoma (PNPC) patients to evaluate the optimal dose of radiotherapy and to determine prognostic factors. PATIENTS AND METHODS: The study included 165 patients with the diagnosis of PNPC treated between 1978 and 2003. The median age was 14 years. There were 3 (1.8%) patients with stage I, 1 (0.6%) with IIA, 10 (6.1%) with IIB, 60 (36.4%) with III, 44 (26.7%) with IVA, and 47 (29%) with IVB disease. While 21 (12.7%) patients were treated with radiotherapy (RT) alone, 144 (87.3%) received chemotherapy and RT. The median follow-up time was 48 months. RESULTS: The actuarial 5-year overall survival (OS) was 77.4% (95% CI: 70.06-84.72), whereas the actuarial 5-year disease-free survival (DFS) rate was 68.8% (95% CI: 61.33-76.31). In multivariate analysis, unfavorable factors were age >14 years for LRC (p=0.04); male gender for DMFS (p=0.03); T3/T4 disease for LRFS (p=0.01); and N3 disease for DFS (p=0.002) and OS (p=0.002); EBRT dose of less than 66 Gy for LRFS (p=0.02) and LRRFS (p=0.0028); and patients treated with RT alone for LRFS (p=0.0001), LRRFS (p=0.007) and DFS (p=0.02). CONCLUSION: Our results support the current practice of using combined radiation and chemotherapy for optimal treatment of NPC. However, research should be encouraged in an attempt to reduce the potential for long-term sequelae in pediatric patients given their relatively favorable prognosis and potential for longevity