Trends of lymph node sampling and metastasis in pediatric and young adult patients with clear cell, epithelioid, and synovial sarcomas

2021 ◽  
Author(s):  
Jennine H. Weller ◽  
Carly Westermann ◽  
Palak Patel ◽  
Ross M. Beckman ◽  
Christine A. Pratilas ◽  
...  
Keyword(s):  
Lymph Node ◽  
Young Adult ◽  
Clear Cell ◽  
Adult Patients ◽  
Lymph Node Sampling ◽  
Synovial Sarcomas

scholarly journals Trends of lymph node sampling and metastasis in pediatric patients with clear cell, epithelioid, and synovial sarcomas

2021 ◽  
Author(s):  
Jennine Weller ◽  
Carly Westermann ◽  
Palak Patel ◽  
Ross Beckman ◽  
Christine Pratilas ◽  
...  
Keyword(s):  
Lymph Node ◽  
Lymph Node Metastasis ◽  
Synovial Sarcoma ◽  
Clear Cell ◽  
Epithelioid Sarcoma ◽  
Clear Cell Sarcoma ◽  
Nodal Metastasis ◽  
Node Metastasis ◽  
Lymph Node Sampling ◽  
Sampling Procedures

Background: Clear cell sarcoma (CCS), epithelioid sarcoma, and synovial sarcoma are rare tumors historically identified as high risk for lymph node metastasis. Nodal metastasis in adults with these subtypes has been described. This study investigates incident nodal metastasis and associated survival in children with these subtypes. Procedure: Using the National Cancer Database (2004-2015), we created a retrospective cohort of 1303 patients (aged ≤25 years) who underwent local control therapy for CCS, epithelioid sarcoma, and synovial sarcoma. Kaplan-Meier curves estimated overall survival (OS) by subtype. Stratifying on subtype, Cox regressions assessed OS by lymph node sampling status and nodal metastasis. Results: There were 103 (7.9%) patients with CCS, 221 (17.0%) with epithelioid sarcoma, and 979 (75.1%) with synovial sarcoma. Lymph node sampling was more frequent in patients with CCS (56.3%) and epithelioid sarcoma (52.5%) versus synovial sarcoma (20.5%, p<0.001). Synovial sarcoma metastasized to lymph nodes less frequently than CCS or epithelioid sarcoma (2.2% vs. 14.6% and 15.0%, p<0.001). Across all subtypes, lymph node metastasis was associated with inferior OS (HR 2.10, CI 1.44-3.07, p<0.001). Lymph node sampling was associated with improved OS in CCS (HR 0.35, CI 0.15-0.78, p=0.010), inferior OS in synovial sarcoma (HR 1.60, CI 1.13- 2.25, p=0.007), and no statistical association with OS in epithelioid sarcoma. Conclusions: Lymph node metastasis is rare in children with synovial sarcoma. Lymph node sampling procedures were not consistently performed for patients with CCS or epithelioid sarcoma, but improved OS supports routine lymph node sampling in children with CCS.

Sporadic neuroendocrine neoplasms in young-adult patients: Differences in natural history, prognosis and treatment compared to adult-elderly patients

2020 ◽  
Author(s):  
Barbara Altieri ◽  
Roberta Modica ◽  
Filomena Bottiglieri ◽  
Cicco Federica de ◽  
Antongiulio Faggiano ◽  
...  
Keyword(s):  
Young Adult ◽  
Natural History ◽  
Elderly Patients ◽  
Adult Patients ◽  
Neuroendocrine Neoplasms

scholarly journals GCT-08. PROTON BEAM RADIOTHERAPY FOR PEDIATRIC AND YOUNG-ADULT PATIENTS WITH INTRACRANIAL GERM CELL TUMOR

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii329-iii329
Author(s):  
Minako Sugiyama ◽  
Takayuki Hashimoto ◽  
Takashi Mori ◽  
Kazuya Hara ◽  
Yukayo Terashita ◽  
...  
Keyword(s):  
Young Adult ◽  
Adverse Events ◽  
Germ Cell ◽  
Cell Count ◽  
Proton Beam ◽  
Adult Patients ◽  
Short Term ◽  
Intracranial Germ Cell Tumor ◽  
X Ray ◽  
Proton Beam Radiotherapy

Abstract BACKGROUND To reduce treatment-related adverse events in pediatric and young-adult patients with brain tumors, proton beam radiotherapy (PBT) has recently been performed instead of conventional X-ray radiotherapy. However, whether PBT is as effective as X-ray radiotherapy has not been sufficiently investigated, especially in patients receiving whole-ventricular irradiation. METHODS We report a retrospective observation of 15 patients with intracranial germ cell tumors (GCT), who received PBT at our institution from April 2014 to September 2019. We evaluated their clinical course, short-term adverse events, and prognosis. RESULTS/ CONCLUSION Fifteen patients (9 males and 6 females; median age 13 years) who received PBT following induction chemotherapy were analyzed. Nine patients received 23.4–27.0 GyE of whole-ventricular irradiation due to GCT in the pituitary gland, pineal body, or hypothalamic area. Three patients received 23.4 GyE of whole-brain irradiation: one of them had boost irradiation for basal ganglia. Three patients received 30.6 GyE of craniospinal irradiation (CSI). Six of the 15 patients experienced nausea (grade 2, according to the CTCAE version 4.0). Four patients, including two who received CSI, showed myelosuppression: decrease in white blood cell count, lymphocyte cell count, and neutrophil count (grade 3). No other severe short-term adverse events of &gt;grade 2 was observed in any of the patients. At a median follow-up of 21 months (2-62 months) after irradiation. all patients are alive without recurrence. Our results may be encouraging and further investigations with a larger scale is warranted.

scholarly journals A long-term survivor of clear cell sarcoma-like tumor of the gastrointestinal tract with liver metastasis: a case report

2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takuhisa Okada ◽  
Yasumitsu Hirano ◽  
Shintaro Ishikawa ◽  
Hiroka Kondo ◽  
Toshimasa Ishii ◽  
...  
Keyword(s):  
Small Intestine ◽  
Lymph Node ◽  
Gastrointestinal Tract ◽  
Liver Metastasis ◽  
Surgical Resection ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma

Abstract Background Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is extremely rare. It is a mesenchymal neoplasm that usually forms in the small intestine of adolescents and young adults, is prone to local recurrence and metastasis, and has a high mortality rate. We report a patient with CCSLTGT with lymph node- and liver metastases, who continues to survive 6 years after initial surgical resection. Case presentation A 38-year-old woman presented with lightheadedness. Laboratory analysis revealed anemia (hemoglobin, 6.7 g/dL), and enhanced computed tomography (CT) demonstrated a mass in the small intestine, about 6 cm in diameter, with swelling of 2 regional lymph nodes. Double-balloon small intestine endoscopic examination revealed a tumor accompanied by an ulcer; the biopsy findings suggested a primary cancer of the small intestine. She was admitted, and we then performed a laparotomy for partial resection of the small intestine with lymph node dissection. Pathologic examination revealed CCSLTGT with regional lymph node metastases. About 3 years later, follow-up CT revealed a single liver metastasis. Consequently, she underwent a laparoscopic partial liver resection. Histopathologic examination confirmed that the liver metastasis was consistent with CCSLTGT. It has now been 3 years without a recurrence. Conclusion Repeated radical surgical resection with close follow-up may be the only way to achieve long-term survival in patients with CCLSTGT.

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