scholarly journals High-dose Chemotherapy Response in Adults with Relapsed/Refractory Small Round Cell Tumours

2022 ◽  
Vol 32 (1) ◽  
pp. 51-56
Keyword(s):  
High Dose Chemotherapy ◽  
High Dose ◽  
Chemotherapy Response ◽  
Round Cell ◽  
Small Round Cell

High-Dose Chemotherapy in Poor-Prognosis Adult Small Round-Cell Tumors: Clinical and Molecular Results From a Prospective Study

2002 ◽  
Vol 20 (8) ◽  
pp. 2181-2188 ◽  
Author(s):  
Alexia Bertuzzi ◽  
Luca Castagna ◽  
Andrea Nozza ◽  
Vittorio Quagliuolo ◽  
Licia Siracusano ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Local Treatment ◽  
Prognostic Significance ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Round Cell ◽  
Fusion Transcripts ◽  
Small Round Cell ◽  
Round Cell Tumors

PURPOSE: The prognosis of metastatic/high-risk localized small round-cell tumors (SRCTs) treated conventionally is dismal. In this phase II study, we explored a high-dose chemotherapy (HD-CT) approach and analyzed the clinical significance of fusion transcripts detection. PATIENTS AND METHODS: From June 1997 to November 1999, 28 SRCT patients (median age, 26 years; 14 peripheral primitive neuroectodermal tumors [pPNETs], seven rhabdomyosarcomas [RMSs], and seven desmoplastic small round-cell tumors [DSRCTs]) received induction chemotherapy with ifosfamide, epirubicin, and vincristine followed by HD-CT. Local treatment (radiotherapy and/or surgery) was performed when possible. Molecular analysis was performed on peripheral-blood and leukapheresis products by reverse-transcriptase polymerase chain reaction. RESULTS: Overall response (OR) was 65% (18 of 28), with 40% complete response and 25% partial response. According to histology, the OR rate was 86% in pPNET and 43% in both RMS DSRCT. With a median follow-up of 35 months, median overall survival was 16 months and median progression-free survival (PFS) was 10 months. PFS was statistically better in pPNET than other histologic types (P = .0045). No correlation was found between the fusion transcript and clinical outcome during follow-up. Furthermore, transcript detection in leukapheretic products was not of prognostic significance. CONCLUSION: Intensive HD-CT seems to enhance the response rate and survival when compared with conventional treatment in poor-prognosis pPNET. The poor results of this treatment in RMS and DSRCT do not support the inclusion of such an approach in these patient subsets. No definitive conclusions can currently be drawn concerning the clinical implications of the detection of fusion transcripts during treatment or follow-up.

scholarly journals High-Dose Chemotherapy with Stem Cell Rescue in Desmoplastic Small Round Cell Tumor: A Single-Institution Experience and Review of the Literature

Sarcoma ◽  
2018 ◽  
Vol 2018 ◽  
pp. 1-10
Author(s):  
Kayleen Bailey ◽  
Michael Roth ◽  
Daniel Weiser ◽  
Jonathan Gill
Keyword(s):  
Cell Tumor ◽  
High Dose ◽  
Cochrane Central Register ◽  
Round Cell ◽  
Review Of The Literature ◽  
Histologic Diagnosis ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Significant Difference

Purpose. Desmoplastic small round cell tumor (DSRCT) is a rare cancer that predominantly affects males averaging 21 years of age at the time of diagnosis. We describe four cases from our institution and place them within the context of a comprehensive review of the literature. Patients and Methods. Study population included any patient who received treatment at Children’s Hospital at Montefiore (CHAM) with histologic diagnosis of DSRCT. A search of the electronic databases PubMed, Cochrane Central Register of Controlled Trials, MEDLINE, and EMBASE for the terms “desmoplastic” AND “small” AND “round” AND “cell” AND “tumor” was performed. Results. One CHAM patient died of disease at 39 months, one patient has relapsed disease at 40 months, and two patients have no evidence of disease at 60 and 91 months. In the literature review, the 3-year OS was 36% and 5-year OS was 13%. There was a statistically significant difference in OS between no transplant and SCT in remission (p=0.004); however, there was no difference between no transplant and SCT not in remission (p=0.23). Conclusion. Given the poor prognosis in DSRCT, this study supports further prospective research into the possible benefit of consolidation of autologous SCT in patients with DSRCT who are in remission, with the alternative inference that these patients in remission may fare well without SCT. Our retrospective review of the literature does not support SCT for patients who are not in remission.

scholarly journals PEDT-04 SIX CASES OF RETINOBLASTOMA WITH CNS INVOLVEMENT

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii16-ii16
Author(s):  
Chikako Kiyotani ◽  
Shinichi Tsujimoto ◽  
Kyohei Isshiki ◽  
Masahiro Sugawa ◽  
Noriyuki Azuma ◽  
...  
Keyword(s):  
Optic Nerve ◽  
Spinal Metastasis ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Chemotherapy Response ◽  
Pineal Tumor ◽  
Csf Cytology ◽  
Cns Involvement ◽  
Suprasellar Tumor ◽  
Nerve Invasion

Abstract Although the survival rate of intraocular retinoblastoma (RB) is nearly 100%, the outcome of central nervous system (CNS) involvement or Trilateral retinoblastoma (TRb: very rare RB which associated with brain tumor) is dismal. We retrospectively reviewed our six cases of these rare tumors. Their ages at diagnosis are 0y3m-1y10m (median 1y3m) (Male 4, Female 2). Only one had RB family history. Their affected eyes were bilateral 2, unilateral 3 and no 1. Their CNS diseases were suprasellar tumor 3, pineal tumor 1 and cerebrospinal fluid (CSF) cytology positive 2. Two of the suprasellar tumor patients had spinal metastasis. Three of the six patients were TRb. One TRb patient was treated with chemotherapy and high-dose chemotherapy without radiotherapy. Although he suffered with secondary osteosarcoma seven years later, he got complete remission and alive 5 years more without any tumor recurrence. The second TRb patient was treated with chemotherapy and local radiotherapy but relapsed 20 months later. The third TRb patient was chemotherapy resistant. Two CSF positive patients had optic nerve invasion. One patient with chiasm invasion died 11 months later because of treatment resistance. The other patient with optic nerve invasion before optic canal had no CNS tumor nor CSF involvement at diagnosis. Chemotherapy before enucleation was given to avoid dissemination. However, CSF cytology became positive after enucleation and remained even with intensified chemotherapy. Finally, he got remission with radiotherapy and high-dose chemotherapy, and alive without disease for 3.8 years. The last patient had suprasellar genetically classified retinoblastoma tumor and cerebrospinal metastasis. This patient showed good chemotherapy response and is still under treatment. Even with &quotso called° fatal RB cases, some case could survive with intensified therapy. Data accumulation is necessary for better survival of these tumors.

scholarly journals Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

2021 ◽  
Vol 11 ◽  
Author(s):  
Guixia Wei ◽  
Xinyao Shu ◽  
Yuwen Zhou ◽  
Xia Liu ◽  
Xiaorong Chen ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Cell Tumor ◽  
High Dose ◽  
Rapid Progression ◽  
Round Cell ◽  
Differentiation Markers ◽  
Soft Tissue Neoplasm ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Intra-abdominal desmoplastic small round cell tumor (IDSRCT) is a rare and highly malignant soft tissue neoplasm, which is characterized by rapid progression and poor prognosis. The mechanism underlying the development of this neoplasm remains elusive, but all cases are characterized by the chromosomal translocation t (11;22) (p13; q12), which results in a formation of EWSR1-WT1 gene fusion. The diagnosis of IDSRCT is often made with core-needle tissue biopsy specimens or laparoscopy or laparotomy. Immunohistochemical analyses have shown the co-expression of epithelial, neuronal, myogenic, and mesenchymal differentiation markers. FISH or reverse transcription polymerase chain reaction detecting EWS-WT1 fusion can be performed to assist in molecular confirmation. There is no standard of care for patients with IDSRCT currently, and majority of newly diagnosed patients received the aggressive therapy, which includes >90% resection of surgical debulking, high-dose alkylator-based chemotherapy, and radiotherapy. More recently, targeted therapy has been increasingly administered to recurrent IDSRCT patients and has been associated with improved survival in clinical conditions. Immunotherapy as a possible therapeutic strategy is being explored in patients with IDSRCT. In this review, we summarize currently available knowledge regarding the epidemiology, potential mechanisms, clinical manifestations, diagnosis, treatment, and prognosis of IDSRCT to assist oncologists in comprehensively recognizing and accurately treating this malignancy.

scholarly journals Intraabdominal and ganglionic desmoplastic small round cell tumor: a case series

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
S. Slim ◽  
I. Zemni ◽  
A. Bouida ◽  
M. Bouhani ◽  
N. Boujelbene ◽  
...  
Keyword(s):  
Abdominal Mass ◽  
Case Series ◽  
Cell Tumor ◽  
High Dose ◽  
Round Cell ◽  
North African ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Presenting Symptoms ◽  
Small Round Cell

Abstract Introduction Desmoplastic small round cell tumor is a rare malignancy with poor prognosis, affecting young male patients. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. In late stages, metastases may be present. Aim We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of four patients with desmoplastic small round cell tumor at our institution. Cases presentation The first three cases reported are 32-, 17-, and 30-year-old North African males with intraabdominal desmoplastic small round cell tumor treated by surgery, chemotherapy, and radiation therapy with different follow-ups. The final case is a 16-year-old North African male with ganglionic desmoplastic small round cell tumor but no evidence of a tissue mass. He underwent two lines of chemotherapy with no response. The patient was lost after 2 years of follow-up. In all cases, desmoplastic small round cell tumor was confirmed by presence of t(11,22) (p13,q12) translocation. Conclusion Treatment of desmoplastic small round cell tumor is based on multidisciplinary therapy. Despite high-dose chemotherapy, extensive surgical resection, and radiotherapy, desmoplastic small round cell tumor remains lethal.

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