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A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report

INTRODUCTION AND IMPORTANCE: Dermatofibrosarcoma Protuberans (DFSP) is a rare and fatal variant of Spindle Cell Sarcoma. It has an annual incidence rate of 0.8 to 4.5 cases per one million individuals. It's locally aggressive and has vague and masquerading clinical presentations. Misdiagnosis i...

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Autores principales: Al Laham, Omar, Atia, Fareed, Shaheen, Jack, Alkhoury, Lama, Ibrahim, Dana, Alchikh Omar, Mohamad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9284050/
https://www.ncbi.nlm.nih.gov/pubmed/35777334
http://dx.doi.org/10.1016/j.ijscr.2022.107334
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author Al Laham, Omar
Atia, Fareed
Shaheen, Jack
Alkhoury, Lama
Ibrahim, Dana
Alchikh Omar, Mohamad
author_facet Al Laham, Omar
Atia, Fareed
Shaheen, Jack
Alkhoury, Lama
Ibrahim, Dana
Alchikh Omar, Mohamad
author_sort Al Laham, Omar
collection PubMed
description INTRODUCTION AND IMPORTANCE: Dermatofibrosarcoma Protuberans (DFSP) is a rare and fatal variant of Spindle Cell Sarcoma. It has an annual incidence rate of 0.8 to 4.5 cases per one million individuals. It's locally aggressive and has vague and masquerading clinical presentations. Misdiagnosis is devastating as it can lead to time wasting, expenditure of unnecessary resources, and possibly raise morbidity and mortality for patients. It is warranted to raise preoperative clinical awareness to achieve prompt surgical therapeutic interventions to reach an up-to-par prognosis. CASE PRESENTATION: We demonstrate the case of a 50-year-old previously healthy Middle Eastern male patient, who was referred to our General Surgery clinic with the chief complaint of an expansive bulge in his left iliac fossa. Preoperative imaging could not exclude a neoplastic cause behind the presentation. Based on the clinical picture, a surgical intervention was decided. CLINICAL DISCUSSION: Our patient's treatment was consummated by means of classical surgical resection of the lesion with adequate negative margins and referring him to an oncologist specialized in DFSP to undergo the necessary adjuvant treatment. Definitive diagnosis was firmly entrenched postoperatively after finalization of the histopathological and immunohistochemical analyses of the resected protuberance. CONCLUSION: DFSP is an eminently rare entity, especially DFSPs which originate from a surgical scar -as was our patient's- and fluctuates in its clinical presentation, thus, it is our responsibility to depict, study this malignant tumor, and document its incidence, so that we can make ironclad clinical decrees to plummet the morbidity and mortality of this relentless neoplasia.
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spelling pubmed-92840502022-07-16 A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report Al Laham, Omar Atia, Fareed Shaheen, Jack Alkhoury, Lama Ibrahim, Dana Alchikh Omar, Mohamad Int J Surg Case Rep Case Report INTRODUCTION AND IMPORTANCE: Dermatofibrosarcoma Protuberans (DFSP) is a rare and fatal variant of Spindle Cell Sarcoma. It has an annual incidence rate of 0.8 to 4.5 cases per one million individuals. It's locally aggressive and has vague and masquerading clinical presentations. Misdiagnosis is devastating as it can lead to time wasting, expenditure of unnecessary resources, and possibly raise morbidity and mortality for patients. It is warranted to raise preoperative clinical awareness to achieve prompt surgical therapeutic interventions to reach an up-to-par prognosis. CASE PRESENTATION: We demonstrate the case of a 50-year-old previously healthy Middle Eastern male patient, who was referred to our General Surgery clinic with the chief complaint of an expansive bulge in his left iliac fossa. Preoperative imaging could not exclude a neoplastic cause behind the presentation. Based on the clinical picture, a surgical intervention was decided. CLINICAL DISCUSSION: Our patient's treatment was consummated by means of classical surgical resection of the lesion with adequate negative margins and referring him to an oncologist specialized in DFSP to undergo the necessary adjuvant treatment. Definitive diagnosis was firmly entrenched postoperatively after finalization of the histopathological and immunohistochemical analyses of the resected protuberance. CONCLUSION: DFSP is an eminently rare entity, especially DFSPs which originate from a surgical scar -as was our patient's- and fluctuates in its clinical presentation, thus, it is our responsibility to depict, study this malignant tumor, and document its incidence, so that we can make ironclad clinical decrees to plummet the morbidity and mortality of this relentless neoplasia. Elsevier 2022-06-22 /pmc/articles/PMC9284050/ /pubmed/35777334 http://dx.doi.org/10.1016/j.ijscr.2022.107334 Text en © 2022 The Authors https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Al Laham, Omar
Atia, Fareed
Shaheen, Jack
Alkhoury, Lama
Ibrahim, Dana
Alchikh Omar, Mohamad
A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report
title A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report
title_full A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report
title_fullStr A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report
title_full_unstemmed A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report
title_short A unique case of Dermatofibrosarcoma Protuberans arising from an inguinal hernial repair scar in a Middle Eastern male - A Case Report
title_sort unique case of dermatofibrosarcoma protuberans arising from an inguinal hernial repair scar in a middle eastern male - a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9284050/
https://www.ncbi.nlm.nih.gov/pubmed/35777334
http://dx.doi.org/10.1016/j.ijscr.2022.107334
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