Primary Multifocal Hepatic PEComa: A Rarely Encountered Diagnostic Dilemma

Nakra, Tripti; Das, Prasenjit; Gupta, Brijnandan; Yadav, Rajni; Singh, Anand; Dash, Nihar Ranjan; Madhusudhan, KS; Ramteke, Prashant

doi:10.1007/s12029-019-00352-4

Cited by 3 publications

(3 citation statements)

References 16 publications

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“…PEComas are rare mesenchymal neoplasms identified mostly in middle-aged patients with a female preponderance. [1][2][3] In 1992, Bonetti proposed the concept of perivascular epithelioid cells. 4 Zamboni coined the term PEComa for the family of related neoplasms comprising AML, CCMMT, CCST, LAM, and other NOS (angiomyolipoma, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, clear cell "sugar" lung tumor, lymphangioleiomyomatosislike tumors, not otherwise specified).…”

Section: Discussionmentioning

confidence: 99%

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Hepatic Perivascular Epithelioid Cell Tumor Mimicking Hepatocellular Carcinoma

et al. 2023

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Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms, and liver PEComas are extremely rare. They are usually discovered incidentally, and diagnostic pitfalls are frequent owing to similar imaging characteristics to other liver neoplasms. We present a patient whose evaluation was consistent with a right-sided hepatocellular carcinoma for which right hepatectomy was performed. Based on the final histopathological examination and immunohistochemistry, a diagnosis of PEComa was made. Immunohistochemistry plays a crucial role in arriving at the diagnosis, and resection represents the standard of care. A long-term follow-up is recommended because the natural history of PEComas is unpredictable.

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Section: Discussionmentioning

confidence: 99%

“…7 The majority of hepatic PEComas are single; however, cases with multifocal lesions have been reported. 3,8 They pose clinical and radiological diagnostic dilemmas, mimicking metastasis or HCC, especially when multifocal. As imaging usually ends up being equivocal, histology with immunohistochemistry is essential for a definite diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Hepatic Perivascular Epithelioid Cell Tumor Mimicking Hepatocellular Carcinoma

et al. 2023

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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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Hepatic and perihepatic PEComas: A study describing a series of five rare cases

Vijayanirmala,

Yadav,

Goyal

et al. 2024

Indian Journal of Pathology and Microbiology

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Background: Perivascular epithelioid cell tumors (PEComas) encompass a group of rare mesenchymal neoplasms, with dual melanocytic and muscular differentiation. Hepatic PEComas are rare and difficult to diagnose, and their behavior is still unclear. Materials and Methods: Herein, we report a total of five cases of hepatic and perihepatic PEComas over a period of the last 5 years from our and collaborating center’s archive. A detailed histological evaluation was done. A comprehensive panel of immunohistochemical stains was used and fluorescence in-situ hybridization analysis was performed for the TFE3 gene using break-apart probes. Result: All these patients were women, with an average age of presentation of 44 years. The lesions were in the right hepatic lobe: three cases, the left hepatic lobe: one case, and gastrohepatic ligament: one case. The preoperative clinicoradiological diagnoses were hepatocellular carcinoma (HCC), focal nodular hyperplasia, hemangioma, metastasis, and gastrointestinal stromal tumor, respectively. Surgical excision was performed in four cases with no further adjuvant therapy. Histopathological examination and subsequent immunophenotyping revealed a diagnosis of PEComa. Fluorescence in-situ hybridization analysis was performed for TFE3 gene rearrangement in four cases. Conclusions: This series highlights the fact that accurate histological diagnosis of hepatic or perihepatic PEComas is important to prevent unnecessary aggressive treatment, unlike primary hepatocellular carcinomas or hepatoid/epithelioid metastatic tumors.

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Primary Multifocal Hepatic PEComa: A Rarely Encountered Diagnostic Dilemma

Cited by 3 publications

References 16 publications

Hepatic Perivascular Epithelioid Cell Tumor Mimicking Hepatocellular Carcinoma

Hepatic Perivascular Epithelioid Cell Tumor Mimicking Hepatocellular Carcinoma

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

Hepatic and perihepatic PEComas: A study describing a series of five rare cases

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