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

Gentile, Marco; Zinna, Mariaconcetta; Zanella, Caterina; Costanza, Antonio; Dalfior, Daniela; Sina, Sokol; Greco, Filippo; Fiorica, Francesco; Colombari, Romano; Catapano, P; Bonetti, Antonella; Remo, Andrea

doi:10.1016/j.prp.2020.152991

Cited by 5 publications

(3 citation statements)

References 14 publications

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“…smooth muscle actin, desmin and caldesmon) [5,6]. PEComas occur most frequently in the abdominopelvic cavity, the retroperitoneum and the uterus [7,8]. To date, approximately 150 cases of PEComas of different localizations were described, but only 90 cases originated from the gynecological tract.…”

Section: Discussionmentioning

confidence: 99%

Perivascular Epithelioid Cell Neoplasm (Pecoma) of the Uterus: A Case Report and Review of the Literature

Anic¹,

Karch²,

Straub³

et al. 2021

COS

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2.1. Background: Perivascular epithelioid cell tumors (PEComas) of the uterus encompass a rare family of mesenchymal neoplasms characterized by co-expression of smooth muscle and melanocytic markers in epithelioid or spindle cells. Due to their unclear malignant potential, a close follow-up after surgical resection is mandatory. Case Presentation:We present the case report of a 55-yearold woman who underwent hysterectomy due to the diagnosis of a uterus myomatosus with growing tendency. Preoperative imaging by ultrasonography and magnetic resonance imaging scan (MRI) did not show specific findings. A uterine PEComa was diagnosed by morphology and immunohistology. Conclusion:Uterine PEComas are extremely rare tumors, which, in most cases, are diagnosed incidentally based on specific morphological and immunohistochemical features.

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Section: Discussionmentioning

confidence: 99%

Perivascular Epithelioid Cell Neoplasm (Pecoma) of the Uterus: A Case Report and Review of the Literature

Anic¹,

Karch²,

Straub³

et al. 2021

COS

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“…In general, the prognosis is favorable [9]. However, there are cases with aggressive behavior that present distant metastases in 32% of cases, or disease recurrence after treatment in 58% of women [4]. Finally, although rarely, uterine PEComas can be found in pregnancy and may cause severe obstetric complications, such as uterine rupture, hemoperitoneum, or retained placenta [10].…”

Section: Discussionmentioning

confidence: 99%

“…Patients with a uterine PEComa have a wide variety of clinical outcomes [3]. The risk of these tumors' aggressive behavior has been linked to several factors that are evaluable using histology following surgical resection [4].…”

Section: Introductionmentioning

confidence: 99%

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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Current Treatment Options: Uterine Sarcoma

Lewis,

Liang,

Mason

et al. 2024

Curr. Treat. Options in Oncol.

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

Cited by 5 publications

References 14 publications

Perivascular Epithelioid Cell Neoplasm (Pecoma) of the Uterus: A Case Report and Review of the Literature

Perivascular Epithelioid Cell Neoplasm (Pecoma) of the Uterus: A Case Report and Review of the Literature

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

Current Treatment Options: Uterine Sarcoma

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