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A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma

Patient: Male, 73-year-old Final Diagnosis: Retroperitoneal dedifferentiated liposarcoma Symptoms: Abdominal pain Medication: — Clinical Procedure: Exploratory laparotomy Specialty: Oncology OBJECTIVE: Rare disease BACKGROUND: Retroperitoneal sarcomas are rare tumors, only affecting 2 to 5 people pe...

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Autores principales: Horowitz, Joel, Singhal, Mayank, Marrero, Dana, Bashjawish, Fuad, Leto, Dezarae, Winters, Maryselle, Jeberaeel, Julian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7070895/
https://www.ncbi.nlm.nih.gov/pubmed/32115569
http://dx.doi.org/10.12659/AJCR.919245
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author Horowitz, Joel
Singhal, Mayank
Marrero, Dana
Bashjawish, Fuad
Leto, Dezarae
Winters, Maryselle
Jeberaeel, Julian
author_facet Horowitz, Joel
Singhal, Mayank
Marrero, Dana
Bashjawish, Fuad
Leto, Dezarae
Winters, Maryselle
Jeberaeel, Julian
author_sort Horowitz, Joel
collection PubMed
description Patient: Male, 73-year-old Final Diagnosis: Retroperitoneal dedifferentiated liposarcoma Symptoms: Abdominal pain Medication: — Clinical Procedure: Exploratory laparotomy Specialty: Oncology OBJECTIVE: Rare disease BACKGROUND: Retroperitoneal sarcomas are rare tumors, only affecting 2 to 5 people per million population and accounting for 0.1% of all malignancies. Liposarcoma is the most common of all retroperitoneal sarcomas, responsible for approximately 20% of all sarcomas in adults. The most important prognostic factors are tumor grade, the presence of positive margins, tumor integrity, and degree of resection. CASE REPORT: Our patient was a 73-year-old man with abdominal pain whose CT scan of the abdomen and pelvis demonstrated a 15×15 cm heterogeneous, left-sided intra-abdominal mass. He underwent resection of the retroperitoneal tumor, left colectomy, and left nephrectomy. Final pathology demonstrated a high-grade, de-differentiated liposarcoma with a rhabdosarcomatous component. The postoperative course was complicated by a small intra-abdominal abscess and abdominal dehiscence. a CT scan after surgery showed a residual tumor of the retroperitoneal posterior margin. Re-exploration to resect the residual tumor and repair the fascial dehiscence were performed. The patient underwent an initial chemotherapy regimen with doxorubicin, then moved to targeted therapy with Palbociclib, and is now on chemotherapy using Eribulin. CONCLUSIONS: Achieving complete resection and the grade of the tumor at diagnosis are the 2 most important prognostic factors for patient survival in retroperitoneal liposarcoma, as survival rates are inversely proportional to the grade of the tumor. Even with the best resection attempts, there is always a risk of residual tumor cells within the tumor bed, which contribute to recurrence and need for additional surgical interventions. It is important to approach this disease process with a multidisciplinary team that includes surgical, medical, and radiation oncology to ensure the best survival outcomes. Retroperitoneal sarcoma recurrence and survival are directly related to the ability to achieve negative margins of resection, as well as the grade and size of the primary tumor. Adjuvant therapies that include radiation and immunotherapy may be effective in treating recurrent disease.
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spelling pubmed-70708952020-03-19 A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma Horowitz, Joel Singhal, Mayank Marrero, Dana Bashjawish, Fuad Leto, Dezarae Winters, Maryselle Jeberaeel, Julian Am J Case Rep Articles Patient: Male, 73-year-old Final Diagnosis: Retroperitoneal dedifferentiated liposarcoma Symptoms: Abdominal pain Medication: — Clinical Procedure: Exploratory laparotomy Specialty: Oncology OBJECTIVE: Rare disease BACKGROUND: Retroperitoneal sarcomas are rare tumors, only affecting 2 to 5 people per million population and accounting for 0.1% of all malignancies. Liposarcoma is the most common of all retroperitoneal sarcomas, responsible for approximately 20% of all sarcomas in adults. The most important prognostic factors are tumor grade, the presence of positive margins, tumor integrity, and degree of resection. CASE REPORT: Our patient was a 73-year-old man with abdominal pain whose CT scan of the abdomen and pelvis demonstrated a 15×15 cm heterogeneous, left-sided intra-abdominal mass. He underwent resection of the retroperitoneal tumor, left colectomy, and left nephrectomy. Final pathology demonstrated a high-grade, de-differentiated liposarcoma with a rhabdosarcomatous component. The postoperative course was complicated by a small intra-abdominal abscess and abdominal dehiscence. a CT scan after surgery showed a residual tumor of the retroperitoneal posterior margin. Re-exploration to resect the residual tumor and repair the fascial dehiscence were performed. The patient underwent an initial chemotherapy regimen with doxorubicin, then moved to targeted therapy with Palbociclib, and is now on chemotherapy using Eribulin. CONCLUSIONS: Achieving complete resection and the grade of the tumor at diagnosis are the 2 most important prognostic factors for patient survival in retroperitoneal liposarcoma, as survival rates are inversely proportional to the grade of the tumor. Even with the best resection attempts, there is always a risk of residual tumor cells within the tumor bed, which contribute to recurrence and need for additional surgical interventions. It is important to approach this disease process with a multidisciplinary team that includes surgical, medical, and radiation oncology to ensure the best survival outcomes. Retroperitoneal sarcoma recurrence and survival are directly related to the ability to achieve negative margins of resection, as well as the grade and size of the primary tumor. Adjuvant therapies that include radiation and immunotherapy may be effective in treating recurrent disease. International Scientific Literature, Inc. 2020-03-02 /pmc/articles/PMC7070895/ /pubmed/32115569 http://dx.doi.org/10.12659/AJCR.919245 Text en © Am J Case Rep, 2020 This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Articles
Horowitz, Joel
Singhal, Mayank
Marrero, Dana
Bashjawish, Fuad
Leto, Dezarae
Winters, Maryselle
Jeberaeel, Julian
A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma
title A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma
title_full A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma
title_fullStr A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma
title_full_unstemmed A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma
title_short A Multi-Modality Treatment of Retroperitoneal De-Differentiated Liposarcoma
title_sort multi-modality treatment of retroperitoneal de-differentiated liposarcoma
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7070895/
https://www.ncbi.nlm.nih.gov/pubmed/32115569
http://dx.doi.org/10.12659/AJCR.919245
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