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

BMC Cancer, Dec 2015

Background The 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 presentation Clear 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 (+). Conclusion Hepatic 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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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. BMC Cancer Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors Zu-Sen Wang 0 Lin Xu 0 Lin Ma 2 Meng-Qi Song 0 Li-Qun Wu 0 Xuan Zhou 1 0 Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University , Qingdao, Shandong 266003 , China 1 Department of Pathology, Affiliated Hospital of Qingdao University , Qingdao, Shandong 266003 , China 2 Department of General Surgery, Qingdao Eighth People's Hospital , Qingdao, Shandong 266003 , China Background: The 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 presentation: Clear 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 (+). Conclusion: Hepatic 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. CCMMT; Diagnosis; Immunohistochemistry; PEComa Background Perivascular epithelioid cell (PEComa) tumor has recently been cytopathologically defined. Histological and immunohistochemical analysis indicate that it has the obvious characteristics of perivascular epithelioid cells (PECs) [ 1, 2 ]. A type of tumor with hyaline cells that has the characteristics and similarity to a neoplasm with perivascular epithelioid cell differentiation is determined as follows according to the World Health Organization (2002) soft-tissue tumor classification: a neoplasm with perivascular epithelioid cell differentiation, including hepatic falciform ligament clear cell myomelanocytic tumors [3]. Because soft-tissue clear cell myomelanocytic tumor (CCMMT) is a newly identified tumor type, there have been very few previous studies. This tumor usually involves the uterus, followed by the sickle ligament and gut. There have been only two reported cases of CCMMT in the liver [ 4 ]; thus, the diagnosis and differential biological behavior of this neoplasm require further study. The present report involves the evaluation of a case of CCMMT and a review of the relevant literature. Case presentation A 29-year-old woman was admitted to our hospital in July 2014 with liver cancer rupture after intervention over a period of 1 month. She had a treatment history involving L-carnitine drugs proceeded by cesarean section (4 months ago), and no history of hepatitis and hepatocirrhosis. A physical examination revealed the following: the right side of the abdomen was slightly bloated; there was a 10-cm surgical scar on the hypogastrium, and a palpable 5 × 6 cm mass was present in the right upper abdomen; the mass was hard, smooth, and had good texture; and there was no pain when the mass was pressed. Laboratory examination revealed routine hepatic and renal function, and normal levels of serum electrolytes and alpha fetoprotein. Ultrasound examination of the digestive system demonstrated the following: a heterogeneous hyperechoic mass (15.5 × 9.6 × 14.2 cm) located in the right hepatic lobe; an irregular anechoic area in the mass; a clear boundary; and no obvious blood flow signal in the mass examined using color Doppler flow imaging (Fig. 1). A plain abdominal computed tomography (CT) scan showed a huge abdominal mass below the right segment of the anterior hepatic lobe, an uneven internal density, and high and low mixed density (Fig. 2). A dynamic enhanced CT scan of the upper abdomen demonstrated the following: a mixed highand low-density mass shadow in the right anterior hepatic lobe below segment S5; a clear boundary; a neat outline; significant uneven enhancement; internally, a large area of low-density non-enhanced shadow; multiple enlargement of the surrounding vessels; and a low-density coated edge. Hepatic arterial enhancement was uneven, but the venous and balance periods strengthened mo (...truncated)


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Zu-Sen Wang, Lin Xu, Lin Ma, Meng-Qi Song, Li-Qun Wu, Xuan Zhou. Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors, BMC Cancer, 2015, pp. 1004, 15, DOI: 10.1186/s12885-015-1992-4