Broad ligament perivascular epithelioid cell tumor (pecoma) of uncertain malignant potential

Mathew, Mary; Rao, Lakshmi; Nagel, Bhawna

doi:10.5146/tjpath.2014.01252

Cited by 4 publications

(3 citation statements)

References 7 publications

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“…In addition, PEComas of the broad ligament have also been reported by other authors. [9][10][11][12][13] Few cases of AML involving the female genital tract have been reported, but AML of broad ligaments is very rare. 14,15 A detailed search in Google Scholar and PubMed using the keywords 'Angiomyolipoma' and 'Broad ligament' yielded only two cases.…”

Section: Discussionmentioning

confidence: 99%

Angiomyolipoma of the broad ligament

Dutta¹,

Marbaniang²,

Dey³

et al. 2020

Autops. Case Rep.

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Angiomyolipoma is a benign mesenchymal neoplasm of the renal parenchyma, accounting for 1% of all renal parenchymal tumors. However, this entity may rarely occur in extrarenal sites. Extrarenal angiomyolipoma has been documented in various sites of the body, but angiomyolipoma of the broad ligament was reported in only two cases. We report the reputed third case of angiomyolipoma of the broad ligament in a 33-year-old female, who presented clinically with abdominal distension. With a working diagnosis of low-grade neoplasm, an en-bloc excision of the left broad ligament mass was performed. Based on histopathology and immunohistochemistry, a diagnosis of the classical variant of angiomyolipoma of the left broad ligament was made. The post-operative period was uneventful with no recurrence after 6 months of follow-up.

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Section: Discussionmentioning

confidence: 99%

Angiomyolipoma of the broad ligament

Dutta¹,

Marbaniang²,

Dey³

et al. 2020

Autops. Case Rep.

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“…The uterine corpus is the most common site for gynecologic PEComas; however, they have also been reported in the cervix, 4‐18 vagina, 5,19‐26 broad ligament, 5,27‐33 ovary, 23,34‐38 and rarely, the vulva 39 . Overall, most gynecologic PEComas present in patients in their fifth to sixth decades (range 6‐79 years), 5,17,23,24,40‐42 but those in the vagina, broad ligament, and vulva may occur at an earlier age (mean 28, 25, and 20 years, respectively) 43 .…”

Section: Clinicopathologic Featuresmentioning

confidence: 99%

Perivascular epithelioid cell tumors (PEComa) of the gynecologic tract

Bennett

Oliva

2020

Genes Chromosomes & Cancer

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PEComas of the female genital tract are rare mesenchymal neoplasms that are most common in the uterus, but also may occur in other gynecologic locations. As they morphologically and immunohistochemically resemble smooth muscle tumors, distinction between the two entities is often challenging, and may be aided by molecular analysis. Thus far, two distinct molecular groups‐classic PEComas with TSC mutations and TFE3‐translocation associated PEComas with TFE3 fusions have been described. Recognition of the first group is imperative as these patients may benefit from targeted therapy with mTOR inhibitors, if malignant. This review will focus on recognition of the morphologic and immunophenotypic features of PEComas, as well as the role of molecular testing in their diagnosis and treatment, analysis of the different algorithms to predict behavior, and differential diagnosis.

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“…PEComas could be localized in a variety of anatomical sites, e.g., the lung, kidney, uterus, and breast [ 3 ]. Due to PEComas’ clinical behavior, they may be classified into benign, uncertain malignant potential, and malignant tumors [ 3 , 4 , 5 , 6 , 7 , 8 ]. A special algorithm has been proposed for assessing the accurate diagnosis.…”

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confidence: 99%

Perivascular Epithelioid Cell Tumor with Uncertain Malignant Potential Arising in the Round Ligament

Badlaeva,

Tregubova,

Kruglyak

et al. 2024

Diagnostics

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A 12-year-old adolescent was diagnosed with a right-sided solid mass in the round ligament of the uterus. The chief complaints were abdominal pain and pelvic discomfort. She underwent laparoscopic tumor resection. Histological examination demonstrated a trabecular growth pattern of epithelioid cells with mitotic activity (3 per 50 HPF), which expressed melanocytic and myoid markers. Due to aforementioned findings, a final diagnosis of perivascular epithelioid cell tumor (PEComa) with uncertain malignant potential was made. To the best of our knowledge, this localization of PEComa is considered to be infrequent with only occasionally reported cases.

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Broad ligament perivascular epithelioid cell tumor (pecoma) of uncertain malignant potential

Cited by 4 publications

References 7 publications

Angiomyolipoma of the broad ligament

Angiomyolipoma of the broad ligament

Perivascular epithelioid cell tumors (PEComa) of the gynecologic tract

Perivascular Epithelioid Cell Tumor with Uncertain Malignant Potential Arising in the Round Ligament

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