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Inicio Gastroenterología y Hepatología (English Edition) Our experience in the surgical treatment of liver PEComa
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Vol. 40. Issue 1.
Pages 24-28 (January 2017)
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Vol. 40. Issue 1.
Pages 24-28 (January 2017)
Scientific letter
DOI: 10.1016/j.gastre.2015.10.003
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Our experience in the surgical treatment of liver PEComa
Nuestra experiencia en el tratamiento del PEComa hepático
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Ibai Otegi Altolagirre
Corresponding author
ibaiotegi@gmail.com

Corresponding author.
, Mario de Miguel Valencia, Pablo Sánchez Acedo, Cruz Zazpe Ripa, Antonio Tarifa Castilla, Javier Herrera Cabezón, Marisa Gómez Dorronsoro, Javier Jiménez Mendioroz
Servicio Navarro de Salud, Complejo Hospitalario de Navarra, Pamplona, Navarra, Spain
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Tables (2)
Table 1. Liver PEComas described in the literature review.
Table 2. Images and anatomopathological characteristics and immunohistochemistry.
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Liver PEComas are extremely rare and differential diagnosis is complex. The treatment of choice is surgical removal. We present our experience (4 cases in 6 years) and a literature review.

Case 1

A 46-year-old female with a history of right hepatectomy in 1995 due to a hepatocellular carcinoma (HCC).

After 10 years, damage to segment II was found. The biopsy showed non-tumour hepatocytes. Four years later, the magnetic resonance imaging (MRI) scan showed a growth in size, hypervascular behaviour and a biopsy consistent with HCC.

There was 2.4cm of damage in segment II on the intraoperative ultrasound, and a limited resection was performed.

The final outcome was PEComa.

The histological preparations from the prior resection were requested and they were confirmed as a match with a liver PEComa (company unknown in 1995).

Case 2

A 45-year-old female with a malignant melanoma previously resected in her lower left limb, Clark IV. After 8 years, the follow-up CT scan found a solid 25mm lesion on the MRI described as: hypervascular, hyperintense in T1 and hypointense in T2.

Biopsy consistent with PEComa.

On a morphologically normal liver, the intraoperative ultrasound identified a 2.5cm hyperechoic lesion, which was well defined in segment VIII. A limited resection was performed.

Case 3

A 48-year-old female diagnosed with ocular choroidal melanoma. The extension study identified a 7cm mass in the liver hilum with a liver PEComa biopsy.

During surgery, a lump in the caudate lobe was observed which compressed the vena cava and the left and middle suprahepatic veins (SHV). The caudate lobe was resected.

The eye lesion was subsequently treated using brachytherapy.

Case 4

A 53-year-old male with a history of frequent alcohol consumption examined due to general symptoms.

In the CT scan a 3cm nodule was identified, adjacent to the left SHV, hypervascular with uniform uptake in the arterial phase.

Biopsy: angiomyolipoma.

In the intraoperative ultrasound, a 3cm hyperechoic lesion was seen, which reached the left and middle SHV. A left hepatectomy was performed.

Images, pathological descriptions, immunohistochemical markers and the follow-up time are shown in Table 1.

Table 1.

Liver PEComas described in the literature review.

Author, year  Age and sexLocation  Size (cm)  Metastatic disease  Follow-up 
Tsui, 1999  56  RLL  6.5  No  2 years 
Tsui, 1999  37  RLL  –  – 
Tsui, 1999  41  RLL  No  4 years 
Tsui, 1999  46  RLL  12.5  No  12 years 
Tsui, 1999  41  –  –  – 
Yamasaki, 2000  30  RLL  No  1 year 
Dalle, 2000  70  RLL  26  Hepatic  5 years 
Yukichi, 2000  13  Lig. Teres  No  22 months 
Xu, 2001  35  RLL  2×–   
Lin, 2002  30  RLL  3.6×–   
Parfitt, 2006  60  RLL  14  Multiple  9 years 
Svajdler, 2007  55  LLL  3.5  –  – 
Rouquie, 2007  67  LLL  No  2 years 
Song Hua, 2007  67  Caudate  No  1 year 
Song Hua, 2007  56  LLL  No  1 year 
Labcharoensub, 2007  31  RLL  1.8  No  6 months 
Li, 2007  56  LLL  5×–  – 
Wang, 2007  46  RLL  4×–  – 
Fang, 2007  56  LLL  5.1  No  24 months 
Fang, 2007  56  LLL  5.1  No  24 months 
Paiva, 2008  51  LLL  0.8  No  2 years 
Sánchez, 2008  32  RLL  No  6 months 
Qiu, 2008  67  RLL  15×12  –  – 
Liu, 2008  31  RLL  8×–  – 
Han, 2008  44  RLL  2×1.6  –  – 
Zimmermann, 2008  53  –  No  17 months 
Chen, 2009  36  RLL  7×–  – 
Chen, 2009  45  RLL  5.5×–  – 
Chen, Li, 2009  37  RLL  5×–  – 
Strzelczyk, 2009  57  RLL  20  No  – 
Akitake, 2009  36  LLL  3.5  No  18 months 
Priola, 2009  36  LLL  11  No  34 months 
Liu, 2010  32  RLL  5.5×5.5  –  – 
Zhu, 2010  26  RLL  5×–  – 
Shi, 2010  41  LLL  5.5×–  – 
Shi, 2010  48  RLL  8×–  – 
Deng, 2011  66  LLL  3×3.5  –  – 
He, 2011  35  RLL  3.5×–  – 
Zou, 2011  54  RLL  6×–  – 
Selvaggi, 2011  42  RLL  Yes  1 month 
Ahn, Hur, 2011  36  LLL  6.5  No  3 months 
Zhang, Wang, 2012  55  RLL  3×–  – 
Gao, 2012  59  RLL  6×–  – 
Yan, Tan, 2012  38  RLL  No  48 
Yan, Tan, 2012  34  RLL  4.3  Yes  14 
Yan, Tan, 2012  49  LLL  2.5  No  32 
Yan, Tan, 2012  75  RLL  No  36 
Yan, Tan, 2012  33  RLL  2.5  No  27 
Yan, Tan, 2012  71  RLL  –  No  29 
Yan, Tan, 2012  41  LLL  Multiple  Yes  12 
Liu, 2014  25  LLL  2.5×–  – 
Otegi, 2015  46  LLL  2.4  No  6 years 
Otegi, 2015  45  RLL  2.9  No  4 years 
Otegi, 2015  48  Caudate  No  18 months 
Otegi, 2015  53  LLL  2.5  No  11 months 

RLL, right lobe of liver; LLL, left lobe of liver; F, female; M, male; –, not found.

There were no deaths and no post-operative complications during the post-operative follow-up.

In 1991, Pea et al.1 described the presence of a cell type with specific cytoplasmic characteristics and strong positivity for HMB-45 in the angiomyolipomas (AML). One year later, these cells were given the name of perivascular epithelioid cell (PEC).2

In 2001, the World Health Organisation (WHO) defined PEComas as a group of rare mesenchymal tumours comprised of perivascular epithelioid cells with well-defined histologic and immunohistochemical characteristics.3

Currently the following are included in the PEComas group: angiomyolipoma (AML), pulmonary lymphangioleiomyomatosis, clear cell “sugar” tumours and clear cell myomelanocytic tumours of the falciform ligament.4

In a systematic review in 2012, Bleeker et al.5 compiled 234 cases. The uterus is the most common location, and it is rare in the liver. In our literature search, we found 51 described cases of liver PEComas (Table 2).

Table 2.

Images and anatomopathological characteristics and immunohistochemistry.

  Case 1  Case 2  Case 3  Case 4 
Pieces 
 
 
 
 
Macroscopic description  White nodule that has a maximum diameter of 1.6×1.7cm  Well-defined nodule, 2.9cm in diameter with yellowish and dark brown coloured areas  Subserosal nodule 9cm in diameter  Nodular lesion 2.5cm in diameter with a liver-like colour and granular consistency 
Microscopic description  Proliferation of epithelioid and spindle cells with eosinophil cytoplasm. Thick vascular structures were noticed, which break up both spindle and epithelioid cells. In one of the slices, adipose tissue was found interspersed with the cellular proliferation  Lumps that show different sized mature adipocytes, interspersed with epithelioid-like cells with large, clear, eosinophilic cytoplasm in groups and around veins with uneven walls  Proliferation of eosinophilic polygonal cells associated with veins which are dilated in appearance and showing a proliferation of elongated, myoid-like cells, epithelioid and adipocyte cells  Proliferation of polygonal cells with a cytoplasm that oscillates between clear and eosinophilic and a diffuse or trabecular growth with abundant medium-sized veins with surrounding epithelial cells. Approximately 30% of fat globules were observed interspersed with this proliferation 
IHC positive  Melan-A
HMB-45
Actin
MITF, c-Kit 
Melan-A
HMB-45
Actin
Calponin 
Melan-A
HMB-45
Actin 
Melan-A
HMB-45
Actin 
IHC negative  Hepatocyte
S-100 
S-100  S-100  S-100 
Ki-67 index  1%  <1%  1%  1% 
Follow-up  6 years  4 years  18 months  11 months 

The clinical presentation of the liver PEComa is variable and nonspecific, usually diagnosed by chance.6–8

The differential diagnosis of liver PEComa includes HCC, haemangioma, focal nodular hyperplasia and liver adenoma.8

In 2012, Tan and Xiao6 published 7 cases of liver PEComas. Radiologically they were well-defined masses with uniform density. In both the CT scan and the intravenous contrast-enhanced MRI, there was varied and premature enhancement in the arterial phase with uniform clearance in venous and late phases. The radiological characteristics are similar to the HCCs. We must consider the PEComas in light of radiological patterns described in patients without elevated alpha-fetoprotein (AFP) and no hepatitis or cirrhosis.

The PEComas are made up of epithelioid cells, linked with vascular structures, eosinophil cytoplasm, abundant glycogen and the characteristic presence in the ultrastructure of premelanosomes. They show immunoreactivity against melanocytic markers HMB-45, microphthalmia transcription factor (MITF), Melan-A and PNL2 melanoma-associated antigen, which indicates melanocytic differentiation between the tumours. They are generally negative for the S-100 which makes the differential diagnosis with melanoma less difficult.4,8

Two of the cases have a history of a melanoma, which makes the differential diagnosis with metastases of this tumour more difficult. But in both cases, in addition to the negativity for the S-100, vascular and adipose proliferation were recognised, which is typical of AML.

The co-expression of actin and HMB45/Melan-A is indispensable for diagnosing PEComa and, together with the negativity for the S-100, it allows for a differential diagnosis with a metastatic melanoma.

They can show variable expression for other melanocytic markers, including the S-100 protein, tyrosinase and the microphthalmia transcription factor (MTF) and for other muscle markers such as smooth muscle myosin (SMMS) and calponin.

c-Kit expression in PEComa cases is also described.

The majority of published PEComa cases seem to have a benign behaviour, although cases of tumour recurrence or distant metastasis have been described.5 The tumour size (greater than 7cm) and the pathological characteristics (high mitotic index and cellular necrosis) are considered a threat for recurrence.6

The most effective currently accepted treatment is surgery.5,9 Adjuvant therapy is advised along with chemotherapy in PEComas with risk indicators.5,10

Despite PEComas being extremely rare, they are part of an emerging group of tumours.

References
[1]
M. Pea, F. Bonetti, G. Zamboni, G. Martignoni, L. Fiore-Donati, C. Doglioni.
Clear cell tumor and angiomyolipoma.
Am J Surg Pathol, 15 (1991), pp. 199-200
[2]
F. Bonetti, M. Pea, G. Martignoni, G. Zamboni.
PEC and sugar.
Am J Surg Pathol, 16 (1992), pp. 307-308
[3]
J.R. Parfitt, A.J. Bella, J.I. Izawa, A.L. Folpe.
Neoplasms with perivascular epithelioid cell differentiation (PEComa). World Health Organization Classification of tumours: pathology and genetics of tumors of soft tissue and bone.
IARC, (2002), pp. 221-222
[4]
G. Martignoni, M. Pea, D. Reghellin, G. Zamboni, F. Bonetti.
PEComas: the past, the present and the future.
Virchows Arch, 452 (2008), pp. 119-132
[5]
J.S. Bleeker, J.F. Quevedo, A.L. Folpe.
Malignant perivascular epithelioid cell neoplasm: risk stratification and treatment strategies.
Sarcoma, 2012 (2012), pp. 541626
[6]
Y. Tan, E.H. Xiao.
Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features – analysis of 7 cases and review of the literature.
Abdom Imaging, 37 (2012), pp. 781-787
[7]
F. Khaja, A. Carilli, S. Baidas, A. Sriharan, S. Norford.
PEComa a perivascular epithelioid cell tumor in the liver – a case report and review of the literature.
Case Rep Med, 2013 (2013), pp. 904126
[8]
S.H. Fang, L.N. Zhou, M. Jin, J.B. Hu.
Perivascular epithelioid cell tumor of the liver: a report of two cases and review of the literature.
World J Gastroenterol, 13 (2007), pp. 5537-5539
[9]
J.R. Parfitt, A.J. Bella, J.I. Izawa, B.M. Wehrli.
Malignant neoplasm of perivascular epithelioid cells of the liver late widespread metastases with long-term follow-up.
Arch Pathol Lab Med, 130 (2006), pp. 1219-1222
[10]
C.L. Lai, K.F. Hsu, J.C. Yu, C.J. Chen, C.B. Hsieh, D.C. Chan, et al.
Malignant perivascular epithelioid cell tumor of the mesentery: a case report and literature review.
Onkologie, 35 (2012), pp. 114-117

Please cite this article as: Otegi Altolagirre I, de Miguel Valencia M, Sánchez Acedo P, Zazpe Ripa C, Tarifa Castilla A, Herrera Cabezón J, et al. Nuestra experiencia en el tratamiento del PEComa hepático. Gastroenterol Hepatol. 2017;40:24–28.

Copyright © 2015. Elsevier España, S.L.U., AEEH and AEG
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