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Aggressive angiomyxoma presenting with back and perineal bulge; a complex surgical approach: A case report

INTRODUCTION: Aggressive angiomyxoma is a rare mesenchymal tumor occurring predominantly in the pelvi-perineal region. It is more common in females during their reproductive age. Our focus is on the surgical approach for a recurrent angiomyxoma. CASE PRESENTATION: We present a case of a 36-year-old...

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Detalles Bibliográficos
Autores principales: Faraj, Walid, Houjeij, Mohamad, Haydar, Ali, Nassar, Hussein, Nounou, Ghina, Khalife, Mohamad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4906141/
https://www.ncbi.nlm.nih.gov/pubmed/27281363
http://dx.doi.org/10.1016/j.ijscr.2016.05.047
Descripción
Sumario:INTRODUCTION: Aggressive angiomyxoma is a rare mesenchymal tumor occurring predominantly in the pelvi-perineal region. It is more common in females during their reproductive age. Our focus is on the surgical approach for a recurrent angiomyxoma. CASE PRESENTATION: We present a case of a 36-year-old female patient with a recurrent lower back and perineal bulges. One year ago, she had the same presentation and underwent resection of that mass in a peripheral hospital without available information. Five months later, she started to have the same bulge. Computed tomography scan and true cut biopsy were suggestive of angiomyxoma with single lung metastasis. The decision was to proceed with resection through a transabdominal and perineal incision, even in the presence of metastasis since it’s a very slow growing tumor with long life expectancy. The pathology was consistent with aggressive angiomyxoma. DISCUSSION: 90% of patients with angiomyxoma are women. Although it is benign, the tumor is still aggressive because of the high rate of local recurrence after resection. Angiomyxoma is a mesenchymal tumor, composed of fibroblasts within a myxoid background. Treatment is usually through wide local excision to achieve an R0 resection. Our approach was through a transabdominal and perineal incision, which allowed full control and mobilization of the tumor without disrupting the capsule. This surgical approach was not described previously in the literature. CONCLUSION: The principle to achieve cure in aggressive angiomyxoma is by optimizing exposure through two different incisions, wide excision while keeping the capsule intact and removing enbloc any invaded organ.