Hepatic PEComa: a potential pitfall in the evaluation of hepatic neoplasms

Khan, H.; Katz, Steven C.; Libbey, N. Peter; Somasundar, Ponnandai

doi:10.1136/bcr-2014-204122

Cited by 12 publications

(16 citation statements)

References 16 publications

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“…Patients admitted to both departments had no established diagnosis. In most cases, the lesions resembled HCC on CT and MRI [8,13]. All patients treated in our center developed hepatic lesions even though they had healthy livers, did not have a history of hepatitis, and did not have any factors that damage the parenchyma.…”

Section: Discussionmentioning

confidence: 90%

“…Usually, these are single lesions; however, multifocality was also reported more frequently in cases involving malignant lesions [12]. Differential diagnoses include other liver tumors, such as hepatocellular carcinoma (HCC), hemangiomas, focal nodular hyperplasia, and liver adenomas [8,13]. In liver ultrasound examination, computed tomography (CT), and magnetic resonance imaging (MRI), the tumors are often misdiagnosed as HCC.…”

Section: Introductionmentioning

confidence: 99%

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PEComa—A Rare Liver Tumor

Krawczyk

Ziarkiewicz‐Wróblewska

Wróblewski

et al. 2021

JCM

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PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our own experience. This retrospective observational study included all patients with PEComa who underwent surgical treatment in two departments between 2002 and 2020. A total of 20 patients were diagnosed with PEComa following histopathological examination. The age of the patients ranged from 21 to 73 years. The majority of patients were women (85%). In most patients, the tumors were incidental. In diagnostic studies, PEComas with high arterial vascularization have been described. Liver resection was the treatment of choice. There was only one postoperative complication. During histopathological evaluation, tumors were composed mostly of epithelioid cells, rarely with spindle cell components, thick-walled vessels, and adipocytes in different proportions. Melanocytic markers (HMB45, MelanA) and at least one smooth muscle marker were expressed in all tumors. Features suggestive of malignancy were found in three cases. In conclusion, PEComa is a rare liver tumor that is usually diagnosed incidentally. In radiological studies, tumors with high arterial vascularization are observed. Liver resection is the treatment of choice.

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Section: Discussionmentioning

confidence: 90%

Section: Introductionmentioning

confidence: 99%

PEComa—A Rare Liver Tumor

Krawczyk

Ziarkiewicz‐Wróblewska

Wróblewski

et al. 2021

JCM

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“…The diagnosis of hepatic PEComa mainly relies on the unique morphology of tumour cells and presence of specific immunological markers including HMB-45, SMA and Melan-A. Fine-needle biopsy is recommended for the diagnosis of hepatic PEComa, which effectively avoids the substantial trauma caused by surgery [22] . In addition, hepatic PEComas must be differentiated from other tumours.…”

Section: Discussionmentioning

confidence: 99%

Retrospective analysis of hepatic perivascular epithelioid cell tumour (PEComa) in a single centre for clinical diagnosis and treatment clinical diagnosis and treatment of hepatic PEComa

et al. 2022

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Our primary objective was to investigate the clinical features, diagnosis, treatment and prognosis of hepatic perivascular epithelioid cell tumour (PEComa). Thirty-five cases of pathologically proven hepatic PEComa that were treated in the Department of Hepatobiliary Centre of the First Affiliated Hospital of Nanjing Medical University from January 2008 to February 2019 were retrospectively analysed, and the literature was also reviewed. Twenty-nine females and 6 males were included in this study. The mean age of these patients was 48.0 years (range, 21–75 years). Thirteen patients complained of upper abdominal pain or discomfort, while others were accidentally discovered by imaging examination. Hepatic PEComas tended to occur in the right lobe of the liver (20 cases in the right lobe, 13 in the left lobe and 2 in the caudate lobe). Two cases were characterized by multiple tumours, and the remaining cases were single lesions (range, 1.2–12 cm). Only 8 cases were correctly diagnosed by the preoperative imaging examination, and the correct diagnosis rate was only 22.9%. The postoperative immunohistochemistry analysis showed that hepatic PEComas are positive for human melanoma black 45, Melan-A and smooth muscle actin, with the exception of 1 case that was negative for Melan-A. All patients undergoing an operation accepted regular follow-up, and the average time was 66.5 months (range, 3–132 months). Two patients who experienced tumour recurrence and 1 patient who died of cardiovascular disease, but the remaining patients showed no evidence of tumour recurrence or metastasis during the follow-up period. Hepatic PEComas are a rare type of tumours that mainly occur in young and middle-aged women. The lack of clinical manifestations and imaging findings increases the difficulty of determining a preoperative diagnosis, which mainly depends on the results of pathological examinations. Surgery is currently the only effective treatment, and long-term clinical follow-up is necessary due to the aggressive behaviour and relapse of hepatic PEComa in some patients.

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“…In the present study, hepatic PEComas presented as a heterogeneous hypointensity on T1WI and hyperintensity on T2WI. However, as the cystic components decrease and the substantial parts increase, the tumour will be hyperintense on T1WI [22]. Hepatic PEComas are viewed as high signal masses on DWI, whereas on ADC, they may present the results.…”

Section: Discussionmentioning

confidence: 99%

“…The diagnosis of hepatic PEComa mainly relies on the unique morphology of tumour cells and presence of speci c immunological markers including HMB-45, SMA and Melan-A. Fine-needle biopsy (FNB) is recommended for the diagnosis of hepatic PEComa, which effectively avoids the substantial trauma caused by surgery [22]. In additions, hepatic PEComas must be differentiated from other tumours.…”

Section: Discussionmentioning

confidence: 99%

New recognition of hepatic perivascular epithelioid cell tumours (PEComa): a retrospective analysis of 35 cases

Yang¹,

Qy²,

Zhou³

et al. 2020

Preprint

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Purpose Our primary objective was to investigate the clinical features, diagnosis, treatment and prognosis of hepatic perivascular epithelioid cell tumours (PEComa).Patients and Methods Thirty-five cases of pathologically proven hepatic PEComa that were treated in the Department of Hepatobiliary Center of the First Affiliated Hospital of Nanjing Medical University from January 2008 to February 2019 were retrospectively analysed, and the literature was reviewed.Results Twenty-nine females and 6 males were included in this study. The mean age of these patients was 48.0 years (range, 21-75 years). Thirteen patients complained of upper abdominal pain and discomfort, but the tumours in the other patients were identified by performing an imaging examination. Hepatic PEComas tended to occur in the right lobe of the liver (20 cases in the right lobe, 13 in the left lobe and 2 in the caudate lobe). Two cases were characterized by multiple tumours, and the remaining cases were single lesions (range, 1.2-12 cm). Only 8 cases were correctly diagnosed by the preoperative imaging examination, and the correct diagnosis rate was only 22.9%. The postoperative immunohistochemistry analysis showed that hepatic PEComas are positive for human melanoma black 45 (HMB-45), Melan-A and smooth muscle actin (SMA), with the exception of 1 case that was negative for Melan-A. All patients undergoing an operation accepted regular follow-up, and the average time was 66.5 months (range, 3-132 months). Two patients who experienced tumour recurrence and 1 patient who died due to cardiovascular disease, but the remaining patients showed no evidence of tumour recurrence or metastasis during the follow-up period.Conclusions Hepatic PEComa is a rare type of tumour that mainly occurs in young and middle-aged women. The lack of clinical manifestations and imaging findings increases the difficulty of determining a preoperative diagnosis, which mainly depends on the pathological examinations. Surgery is currently the only effective treatment, and long-term clinical follow-up is necessary due to the aggressive behaviour and relapse of hepatic PEComa in some cases.

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Hepatic PEComa: a potential pitfall in the evaluation of hepatic neoplasms

Cited by 12 publications

References 16 publications

PEComa—A Rare Liver Tumor

PEComa—A Rare Liver Tumor

Retrospective analysis of hepatic perivascular epithelioid cell tumour (PEComa) in a single centre for clinical diagnosis and treatment clinical diagnosis and treatment of hepatic PEComa

New recognition of hepatic perivascular epithelioid cell tumours (PEComa): a retrospective analysis of 35 cases

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