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CASE REPORT  
Year : 2014  |  Volume : 57  |  Issue : 3  |  Page : 453-455
Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres


Department of Radiology, First Clinical Medical College, Shanxi Medical University, Taiyuan 030001, Shanxi Province, China

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Date of Web Publication14-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

How to cite this URL:
Tan Y, Zhang H, Wang Xc. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Indian J Pathol Microbiol [serial online] 2014 [cited 2021 Aug 4];57:453-5. Available from: https://www.ijpmonline.org/text.asp?2014/57/3/453/138766



   Introduction Top


Clear cell myomelanocytic tumor (CCMT) of the falciform ligament/ligamentum teres was first described in 2000 [1],[2] as a variant of perivascular epithelioid cell tumor (PEComa). [3],[4],[5] Hepatic CCMTs are extremely rare, with only 10 cases reported in the English literature. [1],[2],[6],[7] 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 Top


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.
Table 1: Primary hepati c CCMTs

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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 Top


Primary hepatic PEComa are rare and include angiomyolipoma (ALM) and CCMTs. [4] CCMTs have been reported only within the abdomen of young subjects, mostly women. The tumor is usually found in the falciform ligament/ligamentum teres. [1],[6] 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. [1],[2],[6] CCMTs have occasionally been associated with tuberous sclerosis. [8],[9] 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. [1],[10] The pathological features of PEComa (including CCMTs) are: [4],[5],[10] (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. [11] 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. [12],[13],[14],[15],[16] Contrast-enhanced ultrasonography shows early uptake of contrast into the tumor with rapid drainage. There is little fat seen in these tumors. [15],[16],[17] 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. [18],[19],[20],[21],[22] There are several reports of distant metastases after surgical resection of the primary tumor. [20],[23],[24] 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. [25],[26]


   Conclusion Top


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.

 
   References Top

1.
Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, et al. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: A novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults. Am J Surg Pathol 2000;24:1239-46.  Back to cited text no. 1
    
2.
Tanaka Y, Ijiri R, Kato K, Kato Y, Misugi K, Nakatani Y, et al. HMB-45/melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: Additional example of clear cell 'sugar' tumors. Am J Surg Pathol 2000;24:1295-9.  Back to cited text no. 2
    
3.
Cuevas Herreros O, Escobar Lezcano L, Rodriguez Blaco M, Artigas Raventós V. PEComa, a rare epitheliod cell tumor. Cir Esp 2014; Epub ahead of print.  Back to cited text no. 3
    
4.
Tan Y, Xiao EH. Hepatic perivascular epithelioid cell tumor (PEComa): Dynamic CT, MRI, ultrasonography, and pathologic features - analysis of 7 cases and review of the literature. Abdom Imaging 2012;37:781-7.  Back to cited text no. 4
    
5.
Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F. PEComas: The past, the present and the future. Virchows Arch 2008;452:119-32.  Back to cited text no. 5
    
6.
Priola AM, Priola SM, Cataldi A, Marci V, Fava C. Acute abdomen as an unusual presentation of hepatic PEComa. A case report. Tumori 2009;95:123-8.  Back to cited text no. 6
    
7.
Larbcharoensub N, Karnsombut P, Jatchavala J, Wasutit Y, Nitiyanant P. Primary hepatic clear cell myomelanocytic tumor. Case report and review of the literature. APMIS 2007;115:1454-9.  Back to cited text no. 7
    
8.
Doyle LA, Hornick JL, Fletcher CD. PEComa of the gastrointestinal tract: Clinicopathologic study of 35 cases with evaluation of prognostic parameters. Am J Surg Pathol 2013;37:1769-82.  Back to cited text no. 8
    
9.
Ishibashi T, Nakayama K, Nakayama N, Katagiri H, Ishikawa N, Miyazaki K. Perivascular epithelial cell tumor arising from polypoid adenomyoma: A case report. Eur J Gynaecol Oncol 2013;34:104-6.  Back to cited text no. 9
    
10.
Schoolmeester JK, Howitt BE, Hirsch MS, Dal Cin P, Quade BJ, Nucci MR. Perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: Clinicopathologic and immunohistochemical characterization of 16 cases. Am J Surg Pathol 2014;38:176-88.  Back to cited text no. 10
    
11.
Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: A clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol 2005;29:1558-75.  Back to cited text no. 11
    
12.
Fang SH, Zhou LN, Jin M, Hu JB. Perivascular epithelioid cell tumor of the liver: A report of two cases and review of the literature. World J Gastroenterol 2007;13:5537-9.  Back to cited text no. 12
    
13.
Högemann D, Flemming P, Kreipe H, Galanski M. Correlation of MRI and CT findings with histopathology in hepatic angiomyolipoma. Eur Radiol 2001;11:1389-95.  Back to cited text no. 13
    
14.
Yamamoto H, Oda Y, Yao T, Oiwa T, Kobayashi C, Tamiya S, et al. Malignant perivascular epithelioid cell tumor of the colon: Report of a case with molecular analysis. Pathol Int 2006;56:46-50.  Back to cited text no. 14
    
15.
Shen HQ, Chen DF, Sun XH, Li X, Xu J, Hu XB, et al. MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. Rom J Morphol Embryol 2013;54:643-7.  Back to cited text no. 15
    
16.
Tan Y, Zhang H, Xiao EH. Perivascular epithelioid cell tumour: Dynamic CT, MRI and clinicopathological characteristics - analysis of 32 cases and review of the literature. Clin Radiol 2013;68:555-61.  Back to cited text no. 16
    
17.
Akitake R, Kimura H, Sekoguchi S, Nakamura H, Seno H, Chiba T, et al. Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. Intern Med 2009;48:2083-6.  Back to cited text no. 17
    
18.
Untrauer JB, Giannini PJ, Talmon GA, Johansson SL. Malignant PEComa involving the mandible: Report of a unique case. Oral Surg Oral Med Oral Pathol Oral Radiol 2014;117:e3-8.  Back to cited text no. 18
    
19.
Williamson SR, Bunde PJ, Montironi R, Lopez-Beltran A, Zhang S, Wang M, et al. Malignant perivascular epithelioid cell neoplasm (PEComa) of the urinary bladder with TFE3 gene rearrangement: Clinicopathologic, immunohistochemical, and molecular features[J]. Am J Surg Pathol 2013;37:1619-26.  Back to cited text no. 19
    
20.
Parfitt JR, Bella AJ, Izawa JI, Wehrli BM. Malignant neoplasm of perivascular epithelioid cells of the liver. Arch Pathol Lab Med 2006;130:1219-22.  Back to cited text no. 20
    
21.
Bergamo F, Maruzzo M, Basso U, Montesco MC, Zagonel V, Gringeri E, et al. Neoadjuvant sirolimus for a large hepatic perivascular epithelioid cell tumor (PEComa). World J Surg Oncol 2014;12:46.  Back to cited text no. 21
    
22.
Natella V, Merolla F, Giampaolino P, Bifulco G, Mainenti PP, Insabato L. A huge malignant perivascular epithelioid cell tumor (PEComa) of the uterine cervix and vagina. Pathol Res Pract 2014;210:186-8.  Back to cited text no. 22
    
23.
Lai HY, Chen CK, Lee YH, Tsai PP, Chen JH, Shen WC. Multicentric aggressive angiomyolipomas: A rare form of PEComas. AJR Am J Roentgenol 2006;186:837-40.  Back to cited text no. 23
    
24.
Guglielmetti G, De Angelis P, Mondino P, Terrone C, Volpe A. PEComa of soft tissues can mimic lymph node relapse in patients with history of testicular seminoma. Can Urol Assoc J 2013;7:E651-3.  Back to cited text no. 24
    
25.
Navarro-Pelayo Lainez MM, Ramos-Font C, Rebollo Aguirre AC, Rodríguez-Fernández A, Llamas-Elvira JM. Perivascular epithelioid tumors: Utility of the positron emission tomography with 18F-fluorodesoxyglucose (PET-TAC FDG) in their staging and follow-up. Rev Esp Med Nucl 2010;29:258-62.  Back to cited text no. 25
    
26.
Arnold RT, Myers DT. Visualization of renal angiomyolipoma on F-18 FDG PET/CT. Clin Nucl Med 2009;34:539-40.  Back to cited text no. 26
    

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Correspondence Address:
Hui Zhang
Department of Radiology, First Clinical Medical College, Shanxi Medical University, Taiyuan 030001, Shanxi Province
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.138766

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