Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report

Introduction: We describe a rare tumor arising from the prostate gland: Perivascular Epithelioid Cells tumor (PEC-ome). A 54-years old was treated for acute urinary retention with alpha-blockers at presentation due to benign prostate enlargement (65 cc) with asymmetric middle lobe and regular PSA (0...

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Main Authors: Giulia Sbrollini, Alessandro Conti, Andrea Benedetto Galosi, Vito Lacetera, Rodolfo Montironi, Lorenzo Montesi, Giovanni Muzzonigro
Format: Article
Language:English
Published: PAGEPress Publications 2014-12-01
Series:Archivio Italiano di Urologia e Andrologia
Subjects:
Online Access:http://www.pagepressjournals.org/index.php/aiua/article/view/5061
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author Giulia Sbrollini
Alessandro Conti
Andrea Benedetto Galosi
Vito Lacetera
Rodolfo Montironi
Lorenzo Montesi
Giovanni Muzzonigro
author_facet Giulia Sbrollini
Alessandro Conti
Andrea Benedetto Galosi
Vito Lacetera
Rodolfo Montironi
Lorenzo Montesi
Giovanni Muzzonigro
author_sort Giulia Sbrollini
collection DOAJ
description Introduction: We describe a rare tumor arising from the prostate gland: Perivascular Epithelioid Cells tumor (PEC-ome). A 54-years old was treated for acute urinary retention with alpha-blockers at presentation due to benign prostate enlargement (65 cc) with asymmetric middle lobe and regular PSA (0.92 ng/ml). After 5 months, patient developed a second acute urinary retention episode and nodules in the left lung; he was treated with transurethral resection of the prostate and left lobectomy. Results: Histological examination of prostate and lung tissue gave the same diagnosis: leiomyosarcoma with atypical morphological features and patient was observed for 4 months. Considering the uncommon diagnosis, pathological review by the uro-pathologist at our Hospital was done. Additional immunohistochemistry was done and both tumors showed similar and typical features of metastatic PEC-ome (T1b N0 M1). Therefore a new staging showed local and distant progression with prostatic mass and small lung metastasis. Three cycles of Gemcitabine and Pazopanib were administered, but 2 months later a new urinary retention occurred, despite chemotherapy. Patient referred to our Hospital for salvage pelvic surgery with lymph node dissection. Final pathological diagnosis was PECome of the prostate stage pT4 pN0 R0 M1. Conclusions: PEC-ome is a rare but rapidly invasive mesothelial tumor with early metastatic potential. When this tumors originates from the fibromuscular stroma of the prostate it mimics benign prostatic enlargement and causes LUTS. Expert pathology aided by immunoisthochemistry is the cornerstone of diagnosis. There are no pathognomonic imaging on ultrasound or symptoms suggesting the presence of PECome in early stage. A multidisciplinary approach is necessary and radical surgery should be done to treat this aggressive cancer.
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spelling doaj.art-4027d7905a93451896f422afc91ddeb32022-12-21T22:12:43ZengPAGEPress PublicationsArchivio Italiano di Urologia e Andrologia1124-35622282-41972014-12-0186439339410.4081/aiua.2014.4.3934191Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case reportGiulia Sbrollini0Alessandro Conti1Andrea Benedetto Galosi2Vito Lacetera3Rodolfo Montironi4Lorenzo Montesi5Giovanni Muzzonigro6Department of Urology, “Augusto Murri” General Hospital, ASUR Marche, Fermo; Institute of Urology, Ospedali Riuniti, Politechnic University of the Marche, AnconaDepartment of Urology, “Augusto Murri” General Hospital, ASUR Marche, Fermo; Institute of Urology, Ospedali Riuniti, Politechnic University of the Marche, AnconaDepartment of Urology, “Augusto Murri” General Hospital, ASUR Marche, FermoInstitute of Urology, Ospedali Riuniti, Politechnic University of the Marche, AnconaInstitute of Pathology, Ospedali Riuniti, Politechnic University of the Marche, AnconaInstitute of Urology, Ospedali Riuniti, Politechnic University of the Marche, AnconaInstitute of Urology, Ospedali Riuniti, Politechnic University of the Marche, AnconaIntroduction: We describe a rare tumor arising from the prostate gland: Perivascular Epithelioid Cells tumor (PEC-ome). A 54-years old was treated for acute urinary retention with alpha-blockers at presentation due to benign prostate enlargement (65 cc) with asymmetric middle lobe and regular PSA (0.92 ng/ml). After 5 months, patient developed a second acute urinary retention episode and nodules in the left lung; he was treated with transurethral resection of the prostate and left lobectomy. Results: Histological examination of prostate and lung tissue gave the same diagnosis: leiomyosarcoma with atypical morphological features and patient was observed for 4 months. Considering the uncommon diagnosis, pathological review by the uro-pathologist at our Hospital was done. Additional immunohistochemistry was done and both tumors showed similar and typical features of metastatic PEC-ome (T1b N0 M1). Therefore a new staging showed local and distant progression with prostatic mass and small lung metastasis. Three cycles of Gemcitabine and Pazopanib were administered, but 2 months later a new urinary retention occurred, despite chemotherapy. Patient referred to our Hospital for salvage pelvic surgery with lymph node dissection. Final pathological diagnosis was PECome of the prostate stage pT4 pN0 R0 M1. Conclusions: PEC-ome is a rare but rapidly invasive mesothelial tumor with early metastatic potential. When this tumors originates from the fibromuscular stroma of the prostate it mimics benign prostatic enlargement and causes LUTS. Expert pathology aided by immunoisthochemistry is the cornerstone of diagnosis. There are no pathognomonic imaging on ultrasound or symptoms suggesting the presence of PECome in early stage. A multidisciplinary approach is necessary and radical surgery should be done to treat this aggressive cancer.http://www.pagepressjournals.org/index.php/aiua/article/view/5061Perivascular epithelioid cell neoplasmsUltrasoundProstate neoplasmsDiagnosisSurgery
spellingShingle Giulia Sbrollini
Alessandro Conti
Andrea Benedetto Galosi
Vito Lacetera
Rodolfo Montironi
Lorenzo Montesi
Giovanni Muzzonigro
Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report
Archivio Italiano di Urologia e Andrologia
Perivascular epithelioid cell neoplasms
Ultrasound
Prostate neoplasms
Diagnosis
Surgery
title Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report
title_full Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report
title_fullStr Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report
title_full_unstemmed Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report
title_short Perivascular epithelioid cell tumor (PEC-ome) of the prostate: Ultrasound feature in case report
title_sort perivascular epithelioid cell tumor pec ome of the prostate ultrasound feature in case report
topic Perivascular epithelioid cell neoplasms
Ultrasound
Prostate neoplasms
Diagnosis
Surgery
url http://www.pagepressjournals.org/index.php/aiua/article/view/5061
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