|Year : 2014 | Volume
| Issue : 3 | Page : 453-455
|Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres
Yan Tan, Hui Zhang, Xiao-chun Wang
Department of Radiology, First Clinical Medical College, Shanxi Medical University, Taiyuan 030001, Shanxi Province, China
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|Date of Web Publication||14-Aug-2014|
| Abstract|| |
Clear cell myomelanocytic tumors (CCMTs) of the falciform ligament/ligamentum teres are extremely rare. CCMTs are a variant of perivascular epithelioid cell tumors. We present a case of hepatic CCMT in a 54-year-old woman with abdominal pain. The patient had an 8.8 cm well-demarcated tumor in the right lobe of the liver. Contrast-enhanced computed tomography showed a heterogeneous mass that enhanced significantly in the arterial and portal venous phases, and was less enhanced in the delayed phase. The patient underwent a right hemihepatectomy and cholecystectomy. The tumor cells had clear to slightly eosinophilic cytoplasm, vesicular nuclei, and were positive for HMB-45 and smooth muscle actin. The patient had no recurrence after 36 months follow-up. A review of the literature identified 10 hepatic CCMTs. Hepatic CCMTs are usually benign tumors of young women that present as large masses located in the right lobe of the liver.
Keywords: Computed tomography, pathology, liver, perivascular epithelioid cell tumor, clear cell myomelanocytic tumors
|How to cite this article:|
Tan Y, Zhang H, Wang Xc. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Indian J Pathol Microbiol 2014;57:453-5
| Introduction|| |
Clear cell myomelanocytic tumor (CCMT) of the falciform ligament/ligamentum teres was first described in 2000 , as a variant of perivascular epithelioid cell tumor (PEComa). ,, Hepatic CCMTs are extremely rare, with only 10 cases reported in the English literature. ,,, Due to this rarity, little is known about its behavior. We report our first case of hepatic CCMT and a review of the literature.
| Case report|| |
A 54-year-old woman presented with right upper abdominal intermittent colic of about 4 years duration and weight loss over the 2 preceding months. Physical examination revealed tenderness to palpation in the right upper abdominal quadrant and liver enlargement. Serum hepatitis B surface antigen was identified and serum α-fetoprotein levels were in the normal range. There was no family history of tuberous sclerosis. Ultrasonography revealed an 8.8 cm well-demarcated hypoechoic tumor in the right lobe of the liver, near the falciform ligament. Power Doppler ultrasonography demonstrated displaced vessels around the tumor. Computed tomography (CT) revealed [Figure 1] an 8.5 cm × 7.5 cm × 8.5 cm mass that did not involve the portal vein or biliary duct system. Focal nodular hyperplasia (FNH) or a malignancy was suspected. The patient underwent a right hemihepatectomy and cholecystectomy. A well-circumscribed and encapsulated 8.5 cm tumor was seen. The tumor had a solid growth pattern with abundant vascularity and dilated vascular channels. The tumor was composed of oval and polygonal clear cells [Figure 2]a, which expressed HMB-45 [Figure 2]b and smooth muscle actin (SMA) [Figure 2]c, and did not express S-100 protein. There were no mature adipocytes or thick-wall blood vessels and no dilated lymph vessels. Pathological diagnosis was CCMT. The patient was followed for 36 months without any recurrence. A literature review was performed to better characterize the clinical behavior of this uncommon tumor [Table 1]. This study was approved by the institutional review board of our institution. A written informed consent from the patient was obtained.
|Figure 1: Nonenhanced computed tomography (CT) imaging revealed a heterogeneous low density solid tumor in the right lobe of the liver (a); contrast-enhanced CT imaging showed the mass had uneven, significant enhancement during the arterial (b) and portal venous phases (c), and was less enhanced during the delayed phase (d)|
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|Figure 2: H and E staining showed focal nuclear pleomorphism with small eosinophilic nucleoli (a). Cells were positive for HMB-45 (b) and smooth muscle actin (c) (black arrow)|
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| Discussion|| |
Primary hepatic PEComa are rare and include angiomyolipoma (ALM) and CCMTs.  CCMTs have been reported only within the abdomen of young subjects, mostly women. The tumor is usually found in the falciform ligament/ligamentum teres. , We reviewed literature and also found that CCMTs presented most often in young women (mean age: 20.8 years) as large masses (mean diameter: 8.3 cm) in the right lobe of the liver. Patients most often present with abdominal pain. Asymptomatic CCMTs have also been incidentally diagnosed during ultrasound or CT imaging performed for other reasons. Routine laboratory tests are not diagnostic. ,, CCMTs have occasionally been associated with tuberous sclerosis. , Our female patient presented with right upper abdominal quadrant intermittent colicky pain of about 4 years duration, without tuberous sclerosis.
Clear cell myomelanocytic tumors are characterized by a fascicular and nested proliferation of uniform spindle cells with clear to lightly eosinophilic cytoplasm, vesicular nuclei, and small, prominent nucleoli. , The pathological features of PEComa (including CCMTs) are: ,, (1) Cells with epithelioid appearance closely related to dilated vascular channels; (2) abundant cytoplasmic glycogen with premelanosomes, hemidesmosomes, and poor intracellular junctions; (3) expression of HMB-45, microphthalmia transcription factor and melan-A (MART 1). These markers are compatible with melanocytic differentiation of the tumor. Smooth muscle markers, such as muscle actin HHF35, h-caldesmon and SMA, are also expressed. There are too few cases reported to estimate the biological behavior of these tumors. Folpe et al.  suggested that a high mitotic activity (>1/50 high-power fields), atypical mitotic figures, coagulative tumor necrosis, marked pleomorphism and hypercellularity, nuclear atypia, infiltrative type of growth pattern, and large tumor size (>70 mm) were features potentially related to malignancy. Our patients had the tumor characteristics of a benign tumor. Only one of the 11 CCMTs reported in the literature was malignant.
Imaging characteristics of hepatic PEComa (including CCMTs) are variable. Previous reports have suggested that the right lobe of the liver is the most common site. Tumors are hypervascular and enhance during contrasted CT and MRI imaging. ,,,, Contrast-enhanced ultrasonography shows early uptake of contrast into the tumor with rapid drainage. There is little fat seen in these tumors. ,, CCMTs usually present as a solitary liver tumor of unknown etiology and can reach a large size. The differential diagnosis includes liver adenoma, hepatocellular carcinoma, hepatoblastoma, FNH, and atypical hepatocellular carcinoma.
Surgical resection with a margin of normal liver has been most frequently used to treat these uncommon tumors. Most CCMTs are benign, although aggressive PEComas (including CCMTs) and metastases have been reported. ,,,, There are several reports of distant metastases after surgical resection of the primary tumor. ,, Long-term periodic follow-up is required due to the uncertain clinical behavior of these tumors. Positron emission tomography-CT imaging has been used as part of routine follow-up. ,
| Conclusion|| |
We presented a case of hepatic CCMT and a review of the literature. Hepatic CCMTs are usually benign tumors occurring in young women that present as a large mass in the right lobe of the liver. Our patient had no recurrence after 36 months of follow-up.
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Department of Radiology, First Clinical Medical College, Shanxi Medical University, Taiyuan 030001, Shanxi Province
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]
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