Liver perivascular epithelioid cell tumor in a patient with systemic lupus erythematosus

Tatsi, V.; Milionis, Haralampos; Goussia, Anna; Xydis, Vasileios; Glantzounis, George

doi:10.1016/j.ijscr.2018.10.063

Cited by 6 publications

(2 citation statements)

References 15 publications

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“…This category predominantly includes angiomyolipoma (AML), pulmonary clear cell “sugar” tumor, and lymphangioleiomyomatosis (LAM) [ 2 ]. Although the uterus is a frequent site of PEComa development, these tumors can also arise in diverse locations such as the kidney, pancreas, urinary bladder, uterus, and liver [ 3 , 4 ]. The majority of cases of PEComa present as benign lesions, with no universally accepted protocol for the diagnosis and treatment of treatment-naïve, recurrent, and malignant PEComa globally.…”

Section: Introductionmentioning

confidence: 99%

A retrospective clinical analysis of 11 cases of PEComa from different sites

Yan,

Zhou,

Wang

et al. 2024

World J Surg Onc

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Purpose The objective of this paper is to offer a thorough examination of the clinical presentations, etiology, and treatment strategies associated with perivascular epithelioid cell tumors (PEComas). Methods This retrospective study examined the comprehensive archival data of PEComa cases diagnosed at Beijing Hospital from 2015 to 2023. The pathology slides of all patients were thoroughly reassessed by two experienced pathologists. A thorough retrospective analysis was undertaken, incorporating clinicopathological data including gender, age at diagnosis, initial clinical manifestations, signs, disease onset site, tumor markers, imaging findings, therapeutic modalities, pathological features, immunohistochemical profiles, treatment responses, and prognostic indicators. Patients were evaluated for disease severity according to established pathological classification criteria and were followed up until the designated analysis cut-off date. In instances where patients were unable to be monitored on-site, they were contacted via telephone for postoperative follow-up inquiries. Results This study included 11 patients with ages ranging from 17 to 66 years old, presenting with the disease in multiple anatomical sites, including the retroperitoneum (2/11), liver (4/11), kidney (4/11), lung (1/11), and broad ligament of the uterus (1/11). Most patients presented with non-specific clinical symptoms and were subsequently diagnosed with space-occupying lesions upon physical examination. The tumor demonstrated progressive growth and enlargement, which could result in compression of neighboring organs. Preoperative imaging alone is insufficient for a definitive diagnosis of PEComa, but MRI can provide an initial evaluation of the tumor’s potential malignancy. Molecular marker testing specific to PEComa, such as HMB-45 (90.0%), SMA (81.8%), Melan-A (90.9%), vimentin (90.9%), and Desmin (36.3%), was conducted on all patients. No adjuvant therapies were administered postoperatively. Upon analysis, no instances of relapse at the primary site or the development of new tumors at other sites were observed. Regular imaging reviews of three patients with malignant PEComa post-surgery showed no evidence of recurrence. Conclusions The clinical presentation, tumor biomarkers, and imaging characteristics of PEComa lack specificity, necessitating dependence on pathology and immunohistochemistry for precise diagnosis. The mainstay of treatment consists of surgical resection, with patients typically experiencing a favorable prognosis.

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Section: Introductionmentioning

confidence: 99%

A retrospective clinical analysis of 11 cases of PEComa from different sites

Yan,

Zhou,

Wang

et al. 2024

World J Surg Onc

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“…The following are available online at , Table S1: Published cases of primary liver PEComas. References [ 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , ...…”

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Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review

Kvietkauskas,

Samuolyte,

Rackauskas

et al. 2024

Medicina

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A primary liver perivascular epithelioid cell tumor (PEComa) is an extremely rare entity. In this article, we present a case report with a review of the literature on the patients diagnosed with primary liver PEComa and an elaboration of diagnostic and treatment modalities. A systematic literature search was conducted using the terms “perivascular epithelioid cell tumor”, “PEComa”, “liver”, and “hepatic”. All articles describing patients diagnosed with primary liver PEComa were included. We identified a total of 224 patients of primary liver PEComa from 75 articles and a case from the present study with a significant preponderance of females (ratio 4:1) and with a mean age of 45.3 ± 12.1 years. Most of the patients (114 out of 224, 50.9%) were asymptomatic. A total of 183 (81.3%) patients underwent surgical hepatic resection at the time of diagnosis, while 19 (8.4%) underwent surveillance. Recurrence and metastases were detected in seven (3.1%) and six (2.7%) patients, respectively. In conclusion, surgical resection remains the cornerstone of therapy; however, the presence of nonspecific imaging features makes it difficult to reach a definite diagnosis preoperatively. Therefore, a multidisciplinary approach should be the gold standard in selecting the treatment modality.

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Cyclophosphamide/imipenem/vancomycin

2018

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Liver perivascular epithelioid cell tumor in a patient with systemic lupus erythematosus

Abstract: HighlightsPECOMA is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers showing a variable course.Association of SLE with hepatic PECOMA is unknown.Surgical resection is the preferred therapy.

Cited by 6 publications

References 15 publications

A retrospective clinical analysis of 11 cases of PEComa from different sites

A retrospective clinical analysis of 11 cases of PEComa from different sites

Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review

Cyclophosphamide/imipenem/vancomycin

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