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Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report

INTRODUCTION: Hemophilic pseudotumor is a rare but well documented complication seen in approximately 1–2% of patients with hemophilia. The incidence continues to decrease, likely because of increasingly sophisticated techniques in managing factor deficiency. We present a case of hemophilic pseudotu...

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Autores principales: Allen, Sorcha, Reeder, Craig B., Kransdorf, Mark J., Beauchamp, Christopher P., Zarka, Matthew A., Mookadam, Farouk
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5021780/
https://www.ncbi.nlm.nih.gov/pubmed/27615056
http://dx.doi.org/10.1016/j.ijscr.2016.08.032
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author Allen, Sorcha
Reeder, Craig B.
Kransdorf, Mark J.
Beauchamp, Christopher P.
Zarka, Matthew A.
Mookadam, Farouk
author_facet Allen, Sorcha
Reeder, Craig B.
Kransdorf, Mark J.
Beauchamp, Christopher P.
Zarka, Matthew A.
Mookadam, Farouk
author_sort Allen, Sorcha
collection PubMed
description INTRODUCTION: Hemophilic pseudotumor is a rare but well documented complication seen in approximately 1–2% of patients with hemophilia. The incidence continues to decrease, likely because of increasingly sophisticated techniques in managing factor deficiency. We present a case of hemophilic pseudotumor in a patient without hemophilia, an exceptionally rare entity, and outline a hybrid approach to treatment. PRESENTATION OF CASE: The patient presented with a left sided iliopsoas mass and associated radiculopathy, with a history of a poorly characterized bleeding diathesis and Noonan’s syndrome. He had no history of trauma and was not being treated with anti-coagulation. Of note, factors VIII, IX and XI were normal. An open biopsy was consistent with hemophilic pseudotumor. The patient underwent a hybrid procedure of preoperative embolization of the left internal iliac and left deep circumflex arteries followed by surgical debridement and resection, with an excellent outcome. DISCUSSION: Hemophilic pseudotumor is rarely seen in patients with hemophilia, and even less frequently in patients without. Trauma is often the inciting event. A high index of clinical suspicion is required in order to secure the diagnosis, as the radiographic appearance is non-specific. Our patient had no history of trauma, although we question whether his underlying bleeding diathesis may have predisposed him to developing the pseudotumor. Surgery remains the cornerstone of management in these cases. CONCLUSION: Within the literature, there are only two other cases of hemophilic pseudotumor occurring in a non-hemophiliac patient, highlighting the rarity of this case and the associated diagnostic dilemma.
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spelling pubmed-50217802016-09-21 Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report Allen, Sorcha Reeder, Craig B. Kransdorf, Mark J. Beauchamp, Christopher P. Zarka, Matthew A. Mookadam, Farouk Int J Surg Case Rep Case Report INTRODUCTION: Hemophilic pseudotumor is a rare but well documented complication seen in approximately 1–2% of patients with hemophilia. The incidence continues to decrease, likely because of increasingly sophisticated techniques in managing factor deficiency. We present a case of hemophilic pseudotumor in a patient without hemophilia, an exceptionally rare entity, and outline a hybrid approach to treatment. PRESENTATION OF CASE: The patient presented with a left sided iliopsoas mass and associated radiculopathy, with a history of a poorly characterized bleeding diathesis and Noonan’s syndrome. He had no history of trauma and was not being treated with anti-coagulation. Of note, factors VIII, IX and XI were normal. An open biopsy was consistent with hemophilic pseudotumor. The patient underwent a hybrid procedure of preoperative embolization of the left internal iliac and left deep circumflex arteries followed by surgical debridement and resection, with an excellent outcome. DISCUSSION: Hemophilic pseudotumor is rarely seen in patients with hemophilia, and even less frequently in patients without. Trauma is often the inciting event. A high index of clinical suspicion is required in order to secure the diagnosis, as the radiographic appearance is non-specific. Our patient had no history of trauma, although we question whether his underlying bleeding diathesis may have predisposed him to developing the pseudotumor. Surgery remains the cornerstone of management in these cases. CONCLUSION: Within the literature, there are only two other cases of hemophilic pseudotumor occurring in a non-hemophiliac patient, highlighting the rarity of this case and the associated diagnostic dilemma. Elsevier 2016-08-25 /pmc/articles/PMC5021780/ /pubmed/27615056 http://dx.doi.org/10.1016/j.ijscr.2016.08.032 Text en © 2016 The Authors http://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
Allen, Sorcha
Reeder, Craig B.
Kransdorf, Mark J.
Beauchamp, Christopher P.
Zarka, Matthew A.
Mookadam, Farouk
Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report
title Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report
title_full Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report
title_fullStr Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report
title_full_unstemmed Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report
title_short Hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—A case report
title_sort hemophilic pseudotumor in a non-hemophilic patient treated with a hybrid procedure of preoperative embolization of the feeding arteries followed by surgical resection—a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5021780/
https://www.ncbi.nlm.nih.gov/pubmed/27615056
http://dx.doi.org/10.1016/j.ijscr.2016.08.032
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