Z Gastroenterol 2015; 53(05): 399-408
DOI: 10.1055/s-0034-1399391
Kasuistik
© Georg Thieme Verlag KG Stuttgart · New York

Das PECom der Leber – eine neue Entität der Lebertumoren?

Primary perivascular epitheloid cell tumour (PEComa) of the liver – is a new entity of the liver tumors?
S. Panahova
1   Medizinische Klinik I, Universitätsklinikum Tübingen, Tübingen, Germany
,
H. Rempp
2   Abteilung für diagnostische und interventionelle Radiologie, Radiologische Klinik, Eberhard-Karls-Universität Tübingen, Tübingen, Germany
,
B. Sipos
3   Institut für Pathologie und Neuropathologie, Universitätsklinikum Tübingen, Germany
,
N. P. Malek
1   Medizinische Klinik I, Universitätsklinikum Tübingen, Tübingen, Germany
,
B. Boozari
1   Medizinische Klinik I, Universitätsklinikum Tübingen, Tübingen, Germany
› Author Affiliations
Further Information

Publication History

14 February 2015

15 March 2015

Publication Date:
12 May 2015 (online)

Zusammenfassung

Die perivaskulär epitheloiden Zelltumoren (PECom) der Leber sind seltene Tumoren, die immunhistochemisch durch eine duale Expression von melanozytären und glattmuskulären Markern gekennzeichnet sind. Durch die moderne Diagnostik werden sie zunehmend erfasst. In dieser Arbeit berichten wir über den Fall eines PEComs der Leber als Zufallsbefund. Die klinischen und bildmorphologischen Charakteristika des Tumors werden beleuchtet und mit der Literatur verglichen. Das Management von Patienten mit PECom der Leber ist bisher nicht standardisiert, sodass eine Histologie inkl. Immunhistochemie zur Differenzierung unumgänglich ist. Wichtig ist bei nicht sicher klassifizierbaren Tumoren der Leber auch an diese seltene Entität zu denken.

Abstract

Perivascular epitheloid cell tumor (PEComa) is a rare tumor, characterized by dual Expression of smooth muscle and melanocytic markers. Due to the development of diagnostic procedures, we now diagnose PEComa more often. We report about a case of PEComa of the liver as an accidental finding. We analyze the clinical and morphological characteristics of this tumor and compare it with the data of the literature. Management of patients with PEComa is not yet standardized; therefore biopsy with immunhistochemical staining is necessary for the diagnosis. In case of liver tumors which cannot be classified by their morphology on imaging modalities, it is important to think about this rare entity.

 
  • Literatur

  • 1 Liu D, Shi D, Xu Y et al. Management of perivascular epithelioid cell tumor of the liver: a case report and review of the literature. Oncology letters 2014; 7: 148-152
  • 2 Moench C, Burck I, Bug R et al. Das Angiomyolipom der Leber – eine seltene Differenzialdiagnose der Leberraumforderung. Z Gastroenterol 2008; 46: 54-57
  • 3 Zimmermann A, von der Brelie C, Berger B et al. Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: hepatic PEComa as an emerging entity. Histol Histopathol 2008; 23: 1185-1193
  • 4 Hettinger Y, Reißmann N, Müller J et al. Ein Fall von PECom bei einem 24-jährigen Patienten. Z Gastroenterologie 2011; 49 -K17
  • 5 Reder C, Körber J, Meyer D. PECom des Omentum majus. Z Gastroenterol 2013; K51-K472
  • 6 Akitake R, Kimura H, Sekoguchi S et al. Perivascular Epithelioid Cell Tumor (PEComa) of the Liver diagnosed by Contrast-enhaced Ultrasonography. Internal Medicine 2009; 48: 2083-2086
  • 7 Sadhu S, Kumar Pattari S, Sarkar S et al. PEComa of falciform ligament – an extremely rare tumor. Indian J Surg 2008; 70: 200-202
  • 8 Zhang X, Wang L, Jiang Y et al. Hepatic perivascular epithelioid cell tumors – not otherwise specified: a case report. J South Med Univ 2014; 34: 1-4
  • 9 Bleeker JS, Fernando Quevedo J, Folpe AL. Malignant Perivascular epithelioid Cell Neoplasm: Risk stratification and Treatment Strategies. Sarcoma Volume 2012 (2012), Article ID 541626, 12 pages
  • 10 Martignoni G, Pea M, Reghellin D et al. PEComas: the past, the present and the future. Virchows Arch 2008; 452: 119-132
  • 11 Jafari A, Fischer HP, von Websky M et al. Primary PEComa of the Liver: Case Report and Review of the literature. Z Gastroenterrol 2013; 51: 1096-1100
  • 12 Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol 2010; 41: 1-15
  • 13 Fletcher CD, Unni KK, Mertens F (eds) World Health Organization Classification of Tumors of Pathology and Genetics: Tumors of Soft Tissue and Bone. Volume 4. Lyon, France: IARC Press; 2002
  • 14 Khaja F, Carilli A, Baidas S et al. PEComa: a perivascular epithelioid cell tumor in the liver – a case report and review of the literature. Hindawi publishing corporation. Case reports in medicine Volume 2013 (2013), Article ID 904126
  • 15 Priola AM, Priola SM, Cataldi A et al. Acute abdomen as an unusual presentation of hepatic PEComa. A case report. Tumori 2009; 95: 123-128
  • 16 Tan Y, Zhang H, Xiao EH. Perivascular epithelioid cell tumor: dynamic CT, MRI and clinicopathological charakteristics-Analysis of 32 cases and review of the literature. Clinical Radiology 2013; 68: 555-561
  • 17 Petersen J. Die neue WHO-Klassifikation und aktuelle Ergebnisse in der Weichteiltumorpathologie. Pathologe 2013; 34: 436-448
  • 18 Ameurtesse H, Chbani L, Bennani A et al. Primary perivascular epithelioid cell tumor of the liver: new case report and literature review. Diagnostic Pathology 2014; 9: 149
  • 19 Fang SH, Zhou LN, Jin M et al. Perivascular epithelioid cell tumor of the liver: A report of two cases and review of the literature. World Journal of Gastroenterology : WJG 2007; 13: 5537-5539
  • 20 Yamasaki S, Tanaka S, Fujii H et al. Monotypic epithelioid angiomyolipoma of the liver. Histopathology 2000; 36: 451-456
  • 21 Tryggvason G, Blöndal S, Goldin RD et al. Epithelioid angiomyolipoma of the liver: case report and review of the literature. APMIS 2004; 112: 612-616
  • 22 Parfitt JR, Bella AJ, Izawa JI et al. Malignant neoplasm of perivascular epithelioid cells of the liver. Arch Pathol Lab Med 2006; 130: 1219-1222
  • 23 Larbcharoensub N, Karnsombut P, Jatchavala J et al. Primary hepatic clear cell myomelanocytic tumor. Case report and review of the literature. APMIS 2007; 115: 1454-1459
  • 24 Svajdler M, Bohus P, Goc V et al. Perivascular epithelioid cell tumor (PEComa) of the liver: a case report and review of the literature. Cesk Patol 2007; 43: 18-22
  • 25 Paiva CE, Neto FAM, Agaimy A et al. Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor. World Journal of Gastroenterology  2008; 14: 800-802
  • 26 Strzelczyk JM, Durczynski A, Szymanski D et al. Primary perivascular epithelioid cell tumor (PEComa) of the liver: report of a case. Surg Today 2009; 39: 916-921
  • 27 Priola AM, Priola SM, Cataldi A et al. Acute abdomen as an unusual presentation of hepatic PEComa. A case report. Tumori 2009; 95: 123-128
  • 28 Perez SB. Tumor de cellulas epithelioides perivasculares (PEComa) hepatico. CIR ESP 2009; 85: 179-189
  • 29 Cheung TT, Trendell-Smith N, Poon RT. Primary perivascular epithelioid cell tumour (PEComa) of the liver. BMJ Case Rep 2013; pii: bcr2013008706
  • 30 Zhao LJ, Yang YJ, Wu H et al. Perivascular epithelioid cell tumor of the liver: a case report and literature review. Eur Rev Med Pharmacol Sci 2013; 17: 1665-1668
  • 31 Patra S, Vij M, Kota V et al. Pigmented perivascular epithelioid cell tumor of the liver: report of a rare case with brief review of literature. J Cancer Res Ther 2013; 9: 305-307
  • 32 Yu D, Tang S. Hepatic perivascular epithelioid cell tumor: a case report and review of the literature. Intern Med 2013; 52: 1333-1336
  • 33 Shen HQ, Chen DF, Sun XH et al. MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. Rom J Morphol Embryol 2013; 54: 643-647
  • 34 Wen MC, Jan YJ, Li MC et al. Monotypic epithelioid angiomyolipoma of the liver with TFE3 expression. Pathology 2010; 42: 300-302
  • 35 Ahn JH, Bang H. Primary perivascular epithelioid cell tumor (PEComa) of the liver: a case report and review of the literature. Korean J Pathol 2011; 45: 93-97
  • 36 Mentzel T, Reisshauer S, Rütten A et al. Cutaneous clear cell myomelanocytic tumour: a new member of the growing family of perivascular epithelioid cell tumours (PEComas). Clinicopathological and immunohistochemical analysis of seven cases. Histopathology 2005; 46: 498-504
  • 37 Calder KB, Schlauder S, Morgan MB. Malignant perivascular epithelioid cell tumor (“PEComa”): a case report and literature review of cutaneous/subcutaneous presentations. J Cutan Pathol 2008; 35: 499-503
  • 38 Walsh SN, Sangüeza OP. PEComas: a review with emphasis on cutaneous lesions. Semin Diagn Pathol 2009; 26: 123-130