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Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS)

Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, while in adults it is one of the rarer tumors. Its prognosis is better in children with current treatment modalities; however, it carries poorer prognosis in adults. Recent data on adult RMS is scarce from our pa...

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Autores principales: Rab, Saif ur, Bin Naeem, Sameen, Baloch, Naqib Ullah, Jhatial, Mussadique Ali, Waheed, Muhammad, Fasih, Samir, Kalsoom Awan, Umm-E
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9875868/
https://www.ncbi.nlm.nih.gov/pubmed/36712747
http://dx.doi.org/10.7759/cureus.32961
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author Rab, Saif ur
Bin Naeem, Sameen
Baloch, Naqib Ullah
Jhatial, Mussadique Ali
Waheed, Muhammad
Fasih, Samir
Kalsoom Awan, Umm-E
author_facet Rab, Saif ur
Bin Naeem, Sameen
Baloch, Naqib Ullah
Jhatial, Mussadique Ali
Waheed, Muhammad
Fasih, Samir
Kalsoom Awan, Umm-E
author_sort Rab, Saif ur
collection PubMed
description Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, while in adults it is one of the rarer tumors. Its prognosis is better in children with current treatment modalities; however, it carries poorer prognosis in adults. Recent data on adult RMS is scarce from our part of world. We report outcomes of adult patients with RMS, and with 40 patients; it is the first study to publish such a large data from Pakistan. Methods: This was a retrospective study that included 64 adult patients aged 18 years and older. After data extraction and scrutiny, a total of 40 patients were segregated with diagnosis of RMS of various varieties who were treated and followed up subsequently. International Business Machines (IBM) Statistical Package for Social Sciences (SPSS), version 26 (IBM Corp., Armonk, NY) was used to evaluate all of the gathered data. Results: Embryonal RMS (ERMS) was the most common subtype. Factors favoring better overall survival (OS) at 5 years were absence of nodal and distal metastases, treatment with surgery, margin negative resection, and absence of residual disease on postoperative imaging. Adjuvant radiation therapy (XRT) for positive resection margins as well as for residual disease on postoperative imaging also favored better OS at 5 years. Chemotherapy did impart a trend towards better OS; however, it was not significant. Histopathologic subtype and tumor size did not have any significant impact on outcomes. Median progression free survival (PFS) was 11 months and median OS was 15 months. Conclusions: Adult RMS is a rare disease entity with widely heterogeneous clinical picture and poorer outcomes as compared to the disease of childhood and adolescence. Further prospective studies with larger sample size are required to establish role of patient, disease, and treatment-related factors affecting outcomes in our population.
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spelling pubmed-98758682023-01-26 Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS) Rab, Saif ur Bin Naeem, Sameen Baloch, Naqib Ullah Jhatial, Mussadique Ali Waheed, Muhammad Fasih, Samir Kalsoom Awan, Umm-E Cureus Radiation Oncology Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, while in adults it is one of the rarer tumors. Its prognosis is better in children with current treatment modalities; however, it carries poorer prognosis in adults. Recent data on adult RMS is scarce from our part of world. We report outcomes of adult patients with RMS, and with 40 patients; it is the first study to publish such a large data from Pakistan. Methods: This was a retrospective study that included 64 adult patients aged 18 years and older. After data extraction and scrutiny, a total of 40 patients were segregated with diagnosis of RMS of various varieties who were treated and followed up subsequently. International Business Machines (IBM) Statistical Package for Social Sciences (SPSS), version 26 (IBM Corp., Armonk, NY) was used to evaluate all of the gathered data. Results: Embryonal RMS (ERMS) was the most common subtype. Factors favoring better overall survival (OS) at 5 years were absence of nodal and distal metastases, treatment with surgery, margin negative resection, and absence of residual disease on postoperative imaging. Adjuvant radiation therapy (XRT) for positive resection margins as well as for residual disease on postoperative imaging also favored better OS at 5 years. Chemotherapy did impart a trend towards better OS; however, it was not significant. Histopathologic subtype and tumor size did not have any significant impact on outcomes. Median progression free survival (PFS) was 11 months and median OS was 15 months. Conclusions: Adult RMS is a rare disease entity with widely heterogeneous clinical picture and poorer outcomes as compared to the disease of childhood and adolescence. Further prospective studies with larger sample size are required to establish role of patient, disease, and treatment-related factors affecting outcomes in our population. Cureus 2022-12-26 /pmc/articles/PMC9875868/ /pubmed/36712747 http://dx.doi.org/10.7759/cureus.32961 Text en Copyright © 2022, Rab et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Radiation Oncology
Rab, Saif ur
Bin Naeem, Sameen
Baloch, Naqib Ullah
Jhatial, Mussadique Ali
Waheed, Muhammad
Fasih, Samir
Kalsoom Awan, Umm-E
Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS)
title Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS)
title_full Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS)
title_fullStr Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS)
title_full_unstemmed Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS)
title_short Baseline Characteristics, Prognostic Factors, and Treatment Outcomes for Adult Patients With Rhabdomyosarcoma (RMS)
title_sort baseline characteristics, prognostic factors, and treatment outcomes for adult patients with rhabdomyosarcoma (rms)
topic Radiation Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9875868/
https://www.ncbi.nlm.nih.gov/pubmed/36712747
http://dx.doi.org/10.7759/cureus.32961
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