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Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer

Retroperitoneal liposarcomas (RLPS) are rare tumors that have variable clinical behavior and complex treatment strategies based on presentation, histopathology, and genomics. Early identification is critical, and complete surgical resection remains the primary treatment, although chemotherapy and ra...

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Autor principal: Spicer, Jennifer L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Harborside Press LLC 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8631345/
https://www.ncbi.nlm.nih.gov/pubmed/35295543
http://dx.doi.org/10.6004/jadpro.2021.12.8.6
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author Spicer, Jennifer L.
author_facet Spicer, Jennifer L.
author_sort Spicer, Jennifer L.
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description Retroperitoneal liposarcomas (RLPS) are rare tumors that have variable clinical behavior and complex treatment strategies based on presentation, histopathology, and genomics. Early identification is critical, and complete surgical resection remains the primary treatment, although chemotherapy and radiation are used on individual bases. Presenting symptoms are often nonspecific; therefore, a high degree of suspicion is essential for early diagnosis. In this report, the management of a 37-year-old otherwise healthy male with a large RLPS causing right groin/testicular pain is presented. After three evaluations in the emergency department, the patient was diagnosed and received two cycles of doxorubicin/ifosfamide/mesna (AIM) neoadjuvant chemotherapy. His physical exam on presentation for second opinion demonstrated a large palpable abdominal mass and fullness around the right spermatic cord. There was no appreciable change in tumor size or distant metastases on repeat scanning. Given some obstructive symptoms, a multidisciplinary team advised neoadjuvant radiation followed by radical resection of RLPS. Final pathology demonstrated a 31-cm grade II well-differentiated (WD) liposarcoma with low-grade dedifferentiation. Scattered foci of microscopic positive WD margins were noted, and the remainder of margins were negative. Genomic evaluation showed amplification of CDK4, MDM2, and FRS2. A concise literature review of common presentations, histopathology, genomics, and treatment information is discussed herein. Thorough physical exams, attention to subtle findings, appropriate medical imaging studies, and a high index of suspicion when evaluating vague symptomatology can lead to earlier diagnosis and treatment of RLPS, and ultimately better patient outcomes.
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spelling pubmed-86313452022-03-15 Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer Spicer, Jennifer L. J Adv Pract Oncol Grand Rounds Retroperitoneal liposarcomas (RLPS) are rare tumors that have variable clinical behavior and complex treatment strategies based on presentation, histopathology, and genomics. Early identification is critical, and complete surgical resection remains the primary treatment, although chemotherapy and radiation are used on individual bases. Presenting symptoms are often nonspecific; therefore, a high degree of suspicion is essential for early diagnosis. In this report, the management of a 37-year-old otherwise healthy male with a large RLPS causing right groin/testicular pain is presented. After three evaluations in the emergency department, the patient was diagnosed and received two cycles of doxorubicin/ifosfamide/mesna (AIM) neoadjuvant chemotherapy. His physical exam on presentation for second opinion demonstrated a large palpable abdominal mass and fullness around the right spermatic cord. There was no appreciable change in tumor size or distant metastases on repeat scanning. Given some obstructive symptoms, a multidisciplinary team advised neoadjuvant radiation followed by radical resection of RLPS. Final pathology demonstrated a 31-cm grade II well-differentiated (WD) liposarcoma with low-grade dedifferentiation. Scattered foci of microscopic positive WD margins were noted, and the remainder of margins were negative. Genomic evaluation showed amplification of CDK4, MDM2, and FRS2. A concise literature review of common presentations, histopathology, genomics, and treatment information is discussed herein. Thorough physical exams, attention to subtle findings, appropriate medical imaging studies, and a high index of suspicion when evaluating vague symptomatology can lead to earlier diagnosis and treatment of RLPS, and ultimately better patient outcomes. Harborside Press LLC 2021-11 2021-11-01 /pmc/articles/PMC8631345/ /pubmed/35295543 http://dx.doi.org/10.6004/jadpro.2021.12.8.6 Text en © 2021 Harborside™ https://creativecommons.org/licenses/by-nc-nd/3.0/This article is distributed under the terms of the Creative Commons Attribution Non-Commercial Non-Derivative License, which permits unrestricted non-commercial and non-derivative use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Grand Rounds
Spicer, Jennifer L.
Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer
title Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer
title_full Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer
title_fullStr Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer
title_full_unstemmed Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer
title_short Retroperitoneal Liposarcoma: An Unusual Presentation of a Rare Cancer
title_sort retroperitoneal liposarcoma: an unusual presentation of a rare cancer
topic Grand Rounds
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8631345/
https://www.ncbi.nlm.nih.gov/pubmed/35295543
http://dx.doi.org/10.6004/jadpro.2021.12.8.6
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