Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Liver - A Case Report and Review of the Literature -

Ahn, Ji Hyun; Hur, Bang

doi:10.4132/koreanjpathol.2011.45.s1.s93

Cited by 10 publications

(7 citation statements)

References 16 publications

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“…However, they are negative for CgA (chromogranin A), Syn (synaptophysin), CK (creatine kinase), CD117, CD10, AFP (alpha feto protein) and EMA (epithelial membrane antigen) [2,11]. There have been some reports of progesterone receptor positivity suggesting that progesterone may have a role to play in the modulation of morphology that is seen in PEComas.…”

Section: Discussionmentioning

confidence: 99%

Malignant hepatic perivascular epithelioid cell tumor (PEComa) – Case report and a brief review

Banerjee

Kaushal

Sanket

et al. 2015

Journal of the Egyptian National Cancer Institute

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Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms which can arise from almost any location in the body. Diagnosing them pre-operatively is difficult as they mimic features of other hepatic neoplasms including hepatocellular carcinoma (HCC), fibrolamellar HCC, and focal nodular hyperplasia (FNH) among others. The unique feature of these tumors is the coexpression of muscle and melanocytic markers. These are identified immunohistochemically by the expression of Human Melanin Black-45 (HMB-45), Melan-A and Smooth Muscle Antigen (SMA) which are seen in the majority of tumors. The liver is uncommonly associated with a PEComa and the approach to a patient with hepatic PEComa is not well described. There is no consensus regarding the neo-adjuvant/adjuvant therapy in these patients. The natural history of this condition is not well documented making it an unpredictable disease. Here we have discussed a case and reviewed the literature concerning these rare tumors.

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Section: Discussionmentioning

confidence: 99%

Malignant hepatic perivascular epithelioid cell tumor (PEComa) – Case report and a brief review

Banerjee

Kaushal

Sanket

et al. 2015

Journal of the Egyptian National Cancer Institute

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“…But we think that several other cases can probably exhibit local recurrence or metastasis in long-term follow up. Ahn et al pointed out that this might be attributable to the fact that the follow-up durations of the reported cases were relatively short [7]. Parfitt et al reported a case of hepatic PEComa that lacked these worrisome features but later brought to unexpected clinical course of extensive metastasis during a long term follow-up [15].…”

Section: Discussionmentioning

confidence: 99%

“…Hepatic PEComas are very rare [6,7]. A gold standard for identification using diagnostic imaging studies is lacking and instead, the diagnosis of hepatic PEComa is obviously made on the basis of positive immunohistochemical staining for HMB45 and Melan A. Herein, we presented a case of partial hepatectomy specimen of primary hepatic PEComa occurring in 56-year-old women and accomplished a review of literature.…”

Section: Introductionmentioning

confidence: 99%

Hepatic perivascular epithelioid cell tumor (PEComa): a case report with a review of literatures

et al. 2017

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Hepatic perivascular epithelioid cell tumors (PEComas) are very rare. We report a primary hepatic PEComa with a review of the literature. A 56-year-old women presented with a nodular mass detected during the management of chronic renal failure and chronic hepatitis C. Diagnostic imaging studies suggested a nodular hepatocellular carcinoma in segment 5 of the liver. The patient underwent partial hepatectomy. A brown-colored expansile mass measuring 3.2×3.0 cm was relatively demarcated from the surrounding liver parenchyma. The tumor was mainly composed of epithelioid cells that were arranged in a trabecular growth pattern. Adipose tissue and thick-walled blood vessels were minimally identified. A small amount of extramedullary hematopoiesis was observed in the sinusoidal spaces between tumor cells. Tumor cells were diffusely immunoreactive for human melanoma black 45 (HMB45) and Melan A, focally immunoreactive for smooth muscle actin, but not for hepatocyte specific antigen (HSA).

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“…The cytoplasm of the tumor cells is clear and eosinophilic [ 22 ], the nucleus is usually small and round or oval in shape, has a small nucleolus, contains fine chromatin, is located in the meso-position, and nuclear division is rarely seen. However, in a few cases it is possible to observe obvious hyperchromatic irregular-shaped nuclei, an increase in the size of the nucleolus, an increase in the number of mitotic figures, the presence of melanophores, focal necrosis, and an intravascular tumor embolus; these findings indicate a poor prognosis [ 23 – 25 ]. PEComa-NOS and PEComa occurring in different regions of the body are similar.…”

Section: Discussionmentioning

confidence: 99%

Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors

Wang

et al. 2015

BMC Cancer

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BackgroundThe objective of the study was to explore the clinical expression, radiological and pathological features, differential diagnosis, and biological behavior of a clear cell myomelanocytic tumor. In a case involving a clear cell myomelanocytic tumor located in the hepatic falciform ligament, we evaluated clinical expression, radiological characteristics, histopathology, immunohistochemistry, and biological behavior; we also reviewed the relevant literature.Case presentationClear cell myomelanocytic tumor is a benign soft-tissue neoplasm that often occurs in women, and is expressed as a painless mass. The falciform ligament is its most frequent site of occurrence. The imaging characteristics of this lesion were uneven enhancement in the arterial phase, continuing to strengthen in the venous phase, and equal density in the balance phase. Histological and immunohistochemical analysis revealed the main transparent epithelioid cells and smooth muscle spindle cells to be HMB-45(+), smooth muscle actin(+), and melan-A (+).ConclusionHepatic vascular epithelioid cell tumors are very rare mesenchymal neoplasms. Few studies have investigated this tumor in the hepatic falciform ligament; consequently, its diagnosis and the selection of an appropriate treatment and follow-up protocol are challenging. Treatment outcome remains unpredictable. Therefore, clear cell myomelanocytic tumor should be viewed as a tumor with uncertain malignant potential requiring long-term follow-up.

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Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Liver - A Case Report and Review of the Literature -

Cited by 10 publications

References 16 publications

Malignant hepatic perivascular epithelioid cell tumor (PEComa) – Case report and a brief review

Malignant hepatic perivascular epithelioid cell tumor (PEComa) – Case report and a brief review

Hepatic perivascular epithelioid cell tumor (PEComa): a case report with a review of literatures

Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors

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