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Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review

Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. One of the most frequent localizations of PEComas is the female genitourinary tract, and the uterus is the most involved site after the kidney. Correct preoperative diagnosis is rarely achieved due to the presenc...

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Autores principales: Giannella, Luca, Delli Carpini, Giovanni, Montik, Nina, Verdecchia, Valeria, Puccio, Francesca, Di Giuseppe, Jacopo, Tsiroglou, Dimitrios, Goteri, Gaia, Ciavattini, Andrea
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7459969/
https://www.ncbi.nlm.nih.gov/pubmed/32756336
http://dx.doi.org/10.3390/diagnostics10080553
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author Giannella, Luca
Delli Carpini, Giovanni
Montik, Nina
Verdecchia, Valeria
Puccio, Francesca
Di Giuseppe, Jacopo
Tsiroglou, Dimitrios
Goteri, Gaia
Ciavattini, Andrea
author_facet Giannella, Luca
Delli Carpini, Giovanni
Montik, Nina
Verdecchia, Valeria
Puccio, Francesca
Di Giuseppe, Jacopo
Tsiroglou, Dimitrios
Goteri, Gaia
Ciavattini, Andrea
author_sort Giannella, Luca
collection PubMed
description Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. One of the most frequent localizations of PEComas is the female genitourinary tract, and the uterus is the most involved site after the kidney. Correct preoperative diagnosis is rarely achieved due to the presence of nonspecific imaging features. We report a case of a uterine PEComa with particular reference to ultrasound’s role in characterizing this rare occurrence. Case presentation: a 45-year-old White woman came to our observation for cyclic abdominopelvic pain and chronic constipation. The pre-surgical ultrasound examination showed a heterogeneous tumor that was 4 cm in size, localized on the right anterolateral uterine wall. The mass had well-delimited borders and a central hypoechoic portion. The use of color Doppler showed a rich, irregular vasculature in the center with low impedance. The preoperative diagnostic hypothesis was of a smooth muscle tumor of uncertain malignant potential. After careful counseling, a surgical approach was decided upon, including a total laparoscopic hysterectomy with bilateral salpingectomy. The histological and phenotypical features were consistent with a uterine PEComa. At the last follow-up, two years after surgery, the patient is alive and well. Conclusions: Uterine PEComa is a rare occurrence that should be included in the differential diagnosis of uterine wall tumors. It can appear as a small uterine mass with heterogeneous echogenicity and a rich vascular pattern during an ultrasound evaluation. This diagnostic suspicion may assist in better surgical planning.
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spelling pubmed-74599692020-09-02 Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review Giannella, Luca Delli Carpini, Giovanni Montik, Nina Verdecchia, Valeria Puccio, Francesca Di Giuseppe, Jacopo Tsiroglou, Dimitrios Goteri, Gaia Ciavattini, Andrea Diagnostics (Basel) Case Report Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. One of the most frequent localizations of PEComas is the female genitourinary tract, and the uterus is the most involved site after the kidney. Correct preoperative diagnosis is rarely achieved due to the presence of nonspecific imaging features. We report a case of a uterine PEComa with particular reference to ultrasound’s role in characterizing this rare occurrence. Case presentation: a 45-year-old White woman came to our observation for cyclic abdominopelvic pain and chronic constipation. The pre-surgical ultrasound examination showed a heterogeneous tumor that was 4 cm in size, localized on the right anterolateral uterine wall. The mass had well-delimited borders and a central hypoechoic portion. The use of color Doppler showed a rich, irregular vasculature in the center with low impedance. The preoperative diagnostic hypothesis was of a smooth muscle tumor of uncertain malignant potential. After careful counseling, a surgical approach was decided upon, including a total laparoscopic hysterectomy with bilateral salpingectomy. The histological and phenotypical features were consistent with a uterine PEComa. At the last follow-up, two years after surgery, the patient is alive and well. Conclusions: Uterine PEComa is a rare occurrence that should be included in the differential diagnosis of uterine wall tumors. It can appear as a small uterine mass with heterogeneous echogenicity and a rich vascular pattern during an ultrasound evaluation. This diagnostic suspicion may assist in better surgical planning. MDPI 2020-08-03 /pmc/articles/PMC7459969/ /pubmed/32756336 http://dx.doi.org/10.3390/diagnostics10080553 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Giannella, Luca
Delli Carpini, Giovanni
Montik, Nina
Verdecchia, Valeria
Puccio, Francesca
Di Giuseppe, Jacopo
Tsiroglou, Dimitrios
Goteri, Gaia
Ciavattini, Andrea
Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review
title Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review
title_full Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review
title_fullStr Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review
title_full_unstemmed Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review
title_short Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review
title_sort ultrasound features of a uterine perivascular epithelioid cell tumor (pecoma): case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7459969/
https://www.ncbi.nlm.nih.gov/pubmed/32756336
http://dx.doi.org/10.3390/diagnostics10080553
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