PEComa of the Uterus: A Rare Mesenchymal Tumor Displaying a ≪Snowstorm≫ Pattern at Magnetic Resonance Imaging

Verbeeck, N; Toukouki, Aman; Weis, Romain; Wymersch, Didier Van

doi:10.5334/jbr-btr.926

Cited by 5 publications

(3 citation statements)

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“…In our case, the mass’s vascularization appeared particularly accentuated (color Doppler score 3), in line with the existing literature [ 18 , 19 ]. Indeed, 8 cases out of 10 reported vascularization data showing significant vascular modifications characterized by a rich central vascular network ( Table 1 ).…”

Section: Discussionsupporting

confidence: 91%

“…The electronic medical database PubMed was used for research, combining the following terms: (“perivascular epithelioid cell tumor” or “PEComa”) and (“uterine” or “uterus”) and (“ultrasound” or “ultrasonography”). There were eleven articles in the English literature, referring specifically to the uterine PEComa ultrasound appearance for a total of 19 cases (including the present report) [ 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 ] ( Figure 4 ).…”

Section: Discussionmentioning

confidence: 99%

“…In the paper from Tirumani et al, one case of uterine PEComa was described as a well-circumscribed heterogeneous mass with no cystic areas or significant vascularity during the Doppler examination, being confused with uterine fibroid [ 21 ]. Four authors described malignancy characteristics (high central vascular network with low impedance and an imprecise border of the tumor but not hypo/anechoic or cystic area) that led to confusing it with a sarcoma [ 15 , 18 , 19 , 21 ]. The presence of a hypo-anechoic area inside the tumor led to a diagnosis of fibroid or degenerate leiomyoma in two cases [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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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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Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

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Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review

Wang,

Luo,

Cao

2024

Ultrasound in Obstet & Gyne

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ObjectiveTo describe the clinical and ultrasonographic features of uterine perivascular epithelioid cell tumor (PEComa) using standardized terminology.MethodsThis was a retrospective analysis of patients with uterine PEComa diagnosed and confirmed by pathology and immunohistochemistry at West China Second University Hospital, Sichuan University, Sichuan, China, between January 2010 and September 2023. The Morphological Uterus Sonographic Assessment (MUSA) consensus and the International Endometrial Tumor Analysis (IETA) consensus were utilized for the standardized description of the sonographic characteristics of uterine PEComa. We summarized the clinical and ultrasound features of uterine PEComa in cases from our center and those found in a review of the literature conducted using PubMed from 1 January 2013 to 30 September 2023 (inclusive).ResultsFive patients, aged 33–57 (median, 52) years with a total of six uterine PEComa lesions were included in our cohort. All cases had complete ultrasonographic and pathological images. None of the patients had a history of tuberous sclerosis complex. Two patients had malignant PEComa (one patient had two lesions) and three had benign PEComa, originating from the cervix, myometrium or uterine cavity. Patients presented with symptoms including increased vaginal discharge, vaginal bleeding and pelvic or abdominal pain. The three patients with benign PEComa underwent total hysterectomy and bilateral adnexectomy, tumor excision and conservative management, respectively, while both malignant cases underwent total hysterectomy and bilateral adnexectomy followed by chemotherapy. Regular follow‐up (from 6 to 24 months) revealed recurrence in one case. Two lesions were misdiagnosed as uterine fibroids, two as cervical cancer, one as metastatic cervical cancer (with myometrial invasion) and one was indeterminate. Ultrasound examination showed that most lesions displayed regular round or ovoid shapes (66.7%), uniform echoes (66.7%) and hypoechogenicity (66.7%), with one (16.7%) malignant PEComa showing cystic areas and one (16.7%) benign PEComa showing punctate calcifications. All lesions lacked shadowing and the majority showed moderate to abundant vascularity (color score of 3–4, 83.3%). The color score was 2–4 in the periphery in 100% of cases and internally in 83.3% of cases. The three benign PEComas showed similar characteristics in vascular distribution, with scattered internal vessels and peripheral vessels exhibiting a circular pattern. The literature search identified 11 articles describing the ultrasonographic appearance of 18 cases of uterine PEComa, with similar characteristics to those in our cohort.ConclusionsThe sonographic features of uterine PEComa include a uniform or non‐uniform hypoechogenic mass, typically round or ovoid with regular margins, occasionally containing cystic areas or calcifications, lacking shadowing and often showing moderate to abundant vascularity. Although the preoperative ultrasound diagnosis of uterine PEComa remains challenging, particularly given the non‐specific nature of the sonographic characteristics described here, dispersed intratumoral vessels and a peripheral circular vascular distribution may serve as diagnostic clues for uterine PEComa, but more cases are needed for confirmation. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

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Uterine PEComa with aggressive behavior: A review with an additional case of spontaneous vaginal expulsion

Gentile

Zinna

Zanella³

et al. 2020

Pathology - Research and Practice

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PEComa of the Uterus: A Rare Mesenchymal Tumor Displaying a ≪Snowstorm≫ Pattern at Magnetic Resonance Imaging

Cited by 5 publications

References 10 publications

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

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

Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review

Uterine PEComa with aggressive behavior: A review with an additional case of spontaneous vaginal expulsion

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