{"id":1706,"date":"2019-05-09T10:38:30","date_gmt":"2019-05-09T08:38:30","guid":{"rendered":"http:\/\/www.newslab.sk\/2019\/05\/09\/primarny-liposarkom-pankreasu-kazuistika-a-prehlad-literatury\/"},"modified":"2019-05-09T10:40:32","modified_gmt":"2019-05-09T08:40:32","slug":"primary-pancreatic-liposarcoma-the-case-report-and-literature-review","status":"publish","type":"post","link":"https:\/\/www.newslab.sk\/en\/primary-pancreatic-liposarcoma-the-case-report-and-literature-review\/","title":{"rendered":"Primary pancreatic liposarcoma \u2013 the case report and literature review"},"content":{"rendered":"

*All tables, charts, graphs and pictures that are featured in this article can be found in the .pdf attachment at the end of the paper.<\/strong><\/span><\/p>\n

 <\/p>\n

 <\/p>\n

\u00davod <\/strong><\/p>\n

Pankreas je \u017e\u013eazov\u00fd org\u00e1n s exokrinnou aj endokrinnou funkciou. N\u00e1dory pankreasu del\u00edme na z\u00e1klade histogen\u00e9zy na epitelov\u00e9 a mezench\u00fdmov\u00e9. N\u00e1dory pankreasu epitelov\u00e9ho p\u00f4vodu, kam patr\u00ed aj adenokarcin\u00f3m, predstavuj\u00fa 85 % zo v\u0161etk\u00fdch pankreatick\u00fdch n\u00e1dorov(1), zatia\u013e \u010do n\u00e1dory z ostatn\u00fdch \u0161trukt\u00far pankreasu predstavuj\u00fa 5 \u2013 15 % n\u00e1dorov, z toho mezench\u00fdmov\u00e9 n\u00e1dory tvoria len necel\u00e9 1 %(2). N\u00e1dory pankreasu vych\u00e1dzaj\u00face z tukov\u00e9ho tkaniva patria medzi ve\u013emi vz\u00e1cne tumory. Liposark\u00f3m patr\u00ed medzi naj\u010dastej\u0161ie m\u00e4kkotkanivov\u00e9 n\u00e1dory dospel\u00e9ho veku, tvor\u00ed 20 % z tejto skupiny n\u00e1dorov, typicky postihuje m\u00e4kk\u00e9 tkaniv\u00e1 kon\u010dat\u00edn a retroperitonea. Prim\u00e1rne retroperitone\u00e1lne n\u00e1dory predstavuj\u00fa 0,1 \u2013 0,2 % zo v\u0161etk\u00fdch malign\u00edt(3). Pod\u013ea dostupnej literat\u00fary sa liposark\u00f3m vyskytuje u oboch pohlav\u00ed rovnako bez vekovej predikcie. Retroperitone\u00e1lne liposark\u00f3my rast\u00fa pomaly a bez \u0161pecifick\u00fdch klinick\u00fdch pr\u00edznakov. N\u00e1lez viscer\u00e1lneho liposark\u00f3mu je ve\u013emi zriedkav\u00fd(4,5). Pacienti sa oby\u010dajne s\u0165a\u017euj\u00fa na bru\u0161n\u00fd diskomfort, nechutenstvo a chudnutie, bez elev\u00e1cie n\u00e1dorov\u00fdch markerov(6). Pod\u013ea dostupn\u00fdch liter\u00e1rnych zdrojov bolo op\u00edsan\u00fdch len desa\u0165 pr\u00edpadov liposark\u00f3mu pankreasu od roku 1979(1-3,7-12), z toho sedem v anglickom jazyku( 1-3,6-8,12) (tabu\u013eka 1)<\/em><\/strong>. Prv\u00fd pr\u00edpad spomenul Elliott et al. v roku 1980(7).<\/p>\n

 <\/p>\n

Kazuistika <\/strong><\/p>\n

Opisujeme pr\u00edpad 34-ro\u010dn\u00e9ho mu\u017ea, u ktor\u00e9ho sa z pln\u00e9ho zdravia n\u00e1hle objavili po jedle bolesti brucha a bru\u0161n\u00fd diskomfort. CT vy\u0161etrenie odhalilo v epigastriu ve\u013ek\u00fd tumor\u00f3zny proces sol\u00eddno-cystick\u00e9ho charakteru s akt\u00edvnym krv\u00e1can\u00edm do tumoru, bez mo\u017enosti ur\u010denia prim\u00e1rneho lo\u017eiska (obr\u00e1zok 1)<\/em><\/strong>. Pod\u013ea opera\u010dn\u00e9ho z\u00e1znamu i\u0161lo o n\u00e1dor tela pankreasu s prerastan\u00edm do malej predstery a k prie\u010dnemu hrub\u00e9mu \u010drevu. N\u00e1dor bol fragilnej, m\u00e4kkej konzistencie, s lo\u017eiskami nekr\u00f3z a krv\u00e1can\u00ed, ve\u013ekosti 30 x 25 x 25 cm a celkovej hmotnosti 1 800 g (obr\u00e1zok 2)<\/em><\/strong>. Z anamn\u00e9zy pacienta bol zauj\u00edmav\u00fd \u00fadaj o exstirp\u00e1cii myxoidn\u00e9ho liposark\u00f3mu z prav\u00e9ho stehna v minulosti. V tumore boli pr\u00edtomn\u00e9 lo\u017eisk\u00e1 krv\u00e1cania a diskr\u00e9tne nekr\u00f3zy. I\u0161lo o neostro ohrani\u010den\u00fd n\u00e1dor infiltruj\u00faci pankreatick\u00e9 tkanivo, so zavzat\u00edm duktov a Langerhansov\u00fdch ostrov\u010dekov, n\u00e1lez sved\u010dil o prim\u00e1rnom tumore tukov\u00e9ho tkaniva tela pankreasu. V mikroskopickom obraze bol n\u00e1dor tvoren\u00fd lipoblastmi vzh\u013eadu signet-ring buniek, v okrajoch l\u00e9zie s multivakuolizovan\u00fdmi lipoblastmi, zachyten\u00e9 boli aj \u013eahko atypick\u00e9 strom\u00e1lne bunky a okr\u00fahle primit\u00edvne bunky (asi 1 %).<\/p>\n

N\u00e1dorov\u00e1 popul\u00e1cia bola vnoren\u00e1 v nadbyto\u010dnej myxoidnej str\u00f3me, s tvorbou mukoidn\u00fdch jazierok a\u017e cystickej degener\u00e1cie. Pr\u00edtomn\u00e1 bola prominentn\u00e1 vaskulat\u00fara s tenk\u00fdmi sto\u010den\u00fdmi kapil\u00e1rami, vzh\u013eadu \u201echicken wire\u201c. N\u00e1dorov\u00e9 bunky vykazovali S100, vimentin, p16 pozitivitu, boli negat\u00edvne pre p53,CD34, SMA, HMB45 a s po\u010dtom mit\u00f3z (PHH3): 4 mit\u00f3zy na 10 HPF, s prolifera\u010dnou aktivitou (Ki-67): asi 25 % (obr\u00e1zky 3 \u2013 8)<\/em><\/strong>. Na z\u00e1klade histomorfologick\u00e9ho a imunohistochemick\u00e9ho vy\u0161etrenia bol n\u00e1lez uzavret\u00fd ako prim\u00e1rny myxoidn\u00fd liposark\u00f3m tukov\u00e9ho tkaniva tela pankreasu, pod\u013ea FNCLCC (The French Federation of Comprehensive Cancer Centers) sk\u00f3re 4 (diferenci\u00e1cia tumoru 2, nekr\u00f3zy 1, mit\u00f3zy 1), grade 2, staging pT2b, pNX, pMX.<\/p>\n

 <\/p>\n

Diskusia <\/strong><\/p>\n

Prim\u00e1rny liposark\u00f3m pankreasu je extr\u00e9mne zriedkav\u00fd tumor doteraz op\u00edsan\u00fd v desiatich pr\u00edpadoch zast\u00fapen\u00fd u oboch pohlav\u00ed a v \u0161irokom vekovom zast\u00fapen\u00ed (24 \u2013 78 rokov). Pacienti sa v\u00e4\u010d\u0161inou s\u0165a\u017eovali na bru\u0161n\u00fd diskomfort s boles\u0165ou \u017eal\u00fadka(3,7,8), na anorexiu a stratu hmotnosti(3), a v troch pr\u00edpadoch pacienti nemali \u017eiadne klinick\u00e9 \u0165a\u017ekosti( 1,12). V na\u0161om pr\u00edpade bol tumor objaven\u00fd pri vy\u0161etren\u00ed pre n\u00e1hle vzniknut\u00fa boles\u0165 brucha. Pri diagnostike m\u00f4\u017eu by\u0165 n\u00e1pomocn\u00e9 zobrazovacie vy\u0161etrovacie met\u00f3dy (MRI, CT), no liposark\u00f3m je \u010dasto na nerozoznanie od in\u00fdch typov sark\u00f3mov( 13). Pr\u00edtomnos\u0165 tukov\u00e9ho tkaniva vn\u00fatri n\u00e1doru m\u00f4\u017ee nasmerova\u0165 k diagn\u00f3ze liposark\u00f3mu.<\/p>\n

WHO klasifik\u00e1ciu m\u00e4kkotkanivov\u00fdch n\u00e1dorov z roku 2013(14) o rok nesk\u00f4r upravil Doyle et al.(15) a zmeny sa t\u00fdkali aj klasifik\u00e1cie n\u00e1dorov z tukov\u00e9ho tkaniva. Na z\u00e1klade spom\u00ednan\u00fdch klasifik\u00e1ci\u00ed s\u00fa n\u00e1dory z tukov\u00e9ho tkaniva rozdelen\u00e9 z h\u013eadiska biologick\u00e9ho spr\u00e1vania na tri skupiny: ben\u00edgne, intermedi\u00e1rne a mal\u00edgne. Na z\u00e1klade morfologick\u00fdch vlastnost\u00ed a cytogenetick\u00fdch aber\u00e1ci\u00ed WHO del\u00ed liposark\u00f3my do \u0161tyroch skup\u00edn: dobre diferencovan\u00e9 a dediferencovan\u00e9 liposark\u00f3my (WDLPS a DDLPS), myxoidn\u00fd liposark\u00f3m (MLS) a pleomorfn\u00fd liposark\u00f3m (PLS)(15). Histologick\u00e1 klasifik\u00e1cia je zalo\u017een\u00e1 najm\u00e4 na morfologick\u00fdch znakoch n\u00e1doru v z\u00e1kladnom farben\u00ed H & E. Amplifik\u00e1cia a overexpresia MDM 2 je charakteristick\u00e1 pre dobre diferencovan\u00e9 a dediferencovan\u00e9 liposark\u00f3my, na ktor\u00fdch diagnostiku n\u00e1m m\u00f4\u017ee pom\u00f4c\u0165 FISH met\u00f3da aj imunohistoch\u00e9mia(6). Myxoidn\u00fd liposark\u00f3m predstavuje 15 \u2013 20 % zo v\u0161etk\u00fdch liposark\u00f3mov a tvor\u00ed 5 % zo v\u0161etk\u00fdch m\u00e4kkotkanivov\u00fdch n\u00e1dorov( 14).<\/p>\n

Typicky postihuje hlbok\u00e9 m\u00e4kk\u00e9 tkaniv\u00e1 kon\u010dat\u00edn, zriedkakedy sa nach\u00e1dza retroperitone\u00e1lne alebo subkut\u00e1nne( 14). Na z\u00e1klade histomorfologick\u00fdch znakov myxoidn\u00fd liposark\u00f3m del\u00edme na low grade a high grade. Vo v\u00e4\u010d\u0161ine pr\u00edpadov je v\u0161ak myxoidn\u00fd typ liposark\u00f3mu v\u0161eobecne pova\u017eovan\u00fd za low grade n\u00e1dor s pomal\u00fdm rastom, s n\u00edzkym metastatick\u00fdm potenci\u00e1lom a s progres\u00edvnou de\u0161trukciou pankreatick\u00e9ho tkaniva(2,6). Tieto n\u00e1dory maj\u00fa celkovo lep\u0161iu progn\u00f3zu v porovnan\u00ed s dediferencovan\u00fdmi alebo pleomorfn\u00fdmi typmi liposark\u00f3mu, ozna\u010dovan\u00fdmi ako high grade n\u00e1dory, ktor\u00e9 sa vyzna\u010duj\u00fa vysok\u00fdm metastatick\u00fdm potenci\u00e1lom a agres\u00edvnym spr\u00e1van\u00edm. Dva hlavn\u00e9 faktory, ktor\u00e9 ovplyv\u0148uj\u00fa progn\u00f3zu pacienta, s\u00fa stupe\u0148 diferenci\u00e1cie n\u00e1doru a rozsah chirurgickej resekcie. Pod\u013ea dostupn\u00fdch \u00fadajov je p\u00e4\u0165ro\u010dn\u00e9 pre\u017e\u00edvanie po kompletnej chirurgickej resekcii medzi 41 \u2013 50 %(3,11). Len v jednom z \u00f4smich pr\u00edpadov bola op\u00edsan\u00e1 rekurencia n\u00e1doru po 44 mesiacoch po chirurgickej exc\u00edzii(8).<\/p>\n

 <\/p>\n

Z\u00e1ver <\/strong><\/p>\n

Pankreatick\u00fd myxoidn\u00fd liposark\u00f3m predstavuje raritn\u00fd n\u00e1dor pankreasu a faktorom ovplyv\u0148uj\u00facim progn\u00f3zu je hlavne primeranos\u0165 chirurgickej resekcie v \u010dase prv\u00e9ho zachytenia n\u00e1doru. Prezentovan\u00fd pr\u00edpad je zauj\u00edmav\u00fd pr\u00edtomnos\u0165ou dvoch myxoidn\u00fdch liposark\u00f3mov v dvoch r\u00f4znych lokalit\u00e1ch. Ned\u00e1 sa vyl\u00fa\u010di\u0165 mo\u017enos\u0165, \u017ee exstirpovan\u00fd n\u00e1dor zo stehna bol metast\u00e1zou prim\u00e1rneho liposark\u00f3mu pankreasu. \u00a0<\/em><\/strong><\/p>\n

 <\/p>\n

LITERAT\u00daRA<\/strong><\/p>\n

    \n
  1. Kim JY, et al. Primary mesenchymal tumors of the pancreas: single- center experience over 16 years. Pancreas 2014; 43(6): p. 959-68.<\/li>\n
  2. Jing-Yong Xu JD, Tan Guo, Jian Chen. Liposarcoma within uncinate process of pancreas revealing valuable multidisciplinary therapy: a case report and review of the literature. International Journal of Clinical and Experimental Medicine (IJCEM) 2018; 11(9): p. 8802-10243.<\/li>\n
  3. Dodo IM, et al. Successful outcome following resection of a pancreatic liposarcoma with solitary metastasis. World J Gastroenterol 2005; 11(48): p. 7684-5.<\/li>\n
  4. Kransdorf MJ, et al. Imaging of fatty tumors: distinction of lipoma and well-differentiated liposarcoma. Radiology 2002; 224(1): p. 99-104.<\/li>\n
  5. Lee SY, et al. Retroperitoneal liposarcomas: the experience of a tertiary Asian center. World J Surg Oncol 2011; 9: p. 12.<\/li>\n
  6. Machado MC, et al. Primary liposarcoma of the pancreas: A review illustrated by findings from a recent case. Pancreatology 2016; 16(5): p. 715-8.<\/li>\n
  7. Elliott TE, Albertazzi VJ, Danto LA. Pancreatic liposarcoma: case report with review of retroperitoneal liposarcomas. Cancer 1980; 45(7): p. 1720-3.<\/li>\n
  8. Kuramoto K, et al., Education and imaging. Hepatobiliary and pancreatic: large pancreatic liposarcoma. J Gastroenterol Hepatol 2013; 28(12): p. 1800.<\/li>\n
  9. Choux R, et al. Liposarcoma of the pancreas. Study of a case including ultrastructure]. Ann Anat Pathol (Paris) 1979; 24(3): p. 251-9.<\/li>\n
  10. Amano H, et al. A case of pancreatic liposarcoma (author\u2019s transl)]. Nihon Shokakibyo Gakkai Zasshi 1981; 78(7): p. 1475-9.<\/li>\n
  11. Milano C, et al. Liposarcoma of the pancreas. Report of a clinical case and review of the literature]. Acta Gastroenterol Latinoam 1988; 18(2): p. 133-8.<\/li>\n
  12. Matthews M, et al. Well differentiated liposarcoma, sclerosing type, of the pancreas a case report. Exp Mol Pathol 2016; 101(3): p. 320-322.<\/li>\n
  13. O\u2019Regan KN, et al. Imaging of liposarcoma: classification, patterns of tumor recurrence, and response to treatment. AJR Am J Roentgenol 2011; 197(1): p. W37-43.<\/li>\n
  14. Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F. WHO Classification of Tumours, IARC WHO Classification of Tumours. WHO 2013; 5(5): 468.<\/li>\n
  15. Doyle LA. Sarcoma classification: an update based on the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone. Cancer 2014; 120(12): p. 1763-74.<\/li>\n<\/ol>\n","protected":false},"excerpt":{"rendered":"

    *All tables, charts, graphs and pictures that are featured in this article can be found in the .pdf attachment at the end of the paper.     \u00davod Pankreas je \u017e\u013eazov\u00fd org\u00e1n s exokrinnou aj endokrinnou funkciou. N\u00e1dory pankreasu del\u00edme na z\u00e1klade histogen\u00e9zy na epitelov\u00e9 a mezench\u00fdmov\u00e9. N\u00e1dory pankreasu epitelov\u00e9ho p\u00f4vodu, kam patr\u00ed aj adenokarcin\u00f3m,<\/p>\n","protected":false},"author":7,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_mi_skip_tracking":false,"footnotes":""},"categories":[297],"tags":[1155,1157,785,1156],"class_list":["post-1706","post","type-post","status-publish","format-standard","hentry","category-pathology","tag-liposarcoma","tag-myxoid-liposarcoma","tag-pancreas","tag-soft-tissue-tumors","typ_clanku-casuistry"],"acf":{"abstrakt":"

    Adenocarcinoma of pancreas presents 85% of all pancreatic tumors, and only 1% of all pancreatic tumors are soft tissue tumors. Liposarcoma is the most common soft tissue sarcoma and accounts for 20% of all mesenchymal malignancies in adulthood. The tumor occurs most frequently in limbs and retroperitoneum, rarely in the visceral region. There was reported ten cases of primary liposarcoma of pancreas since 1979. We described a case of 34-year old male with sudden abdominal pain. CT confirmed an extensive epigastric tumorous process with bleeding into the tumor, with presumed tumor origin in the pancreas. Biopsy confirmed a primary liposarcoma of pancreas \u2013 myxoid type, with an achieved subsequent complete surgical excision of the tumor. <\/p>\n

    Keywords: liposarcoma, pancreas, soft tissue tumors, myxoid liposarcoma <\/p>\n","casopis":[{"ID":1633,"post_author":"7","post_date":"2019-05-09 08:56:48","post_date_gmt":"2019-05-09 06:56:48","post_content":"

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    • HCV infection \u2013 more than 20 years of drug development<\/li>\r\n<\/ul>","post_title":"newsLab","post_excerpt":"","post_status":"publish","comment_status":"closed","ping_status":"closed","post_password":"","post_name":"newslab-3","to_ping":"","pinged":"","post_modified":"2019-05-09 08:56:48","post_modified_gmt":"2019-05-09 06:56:48","post_content_filtered":"","post_parent":0,"guid":"http:\/\/www.newslab.sk\/?post_type=casopis&p=1633","menu_order":0,"post_type":"casopis","post_mime_type":"","comment_count":"0","filter":"raw"}],"strana":"56-59","upload_clanok":{"ID":1704,"id":1704,"title":"Prim\u00e1rny liposark\u00f3m pankreasu \u2013 kazuistika a preh\u013ead literat\u00fary","filename":"Prim\u00e1rny-liposark\u00f3m-pankreasu-\u2013-kazuistika-a-preh\u013ead-literat\u00fary.pdf","filesize":446266,"url":"https:\/\/www.newslab.sk\/wp-content\/uploads\/2019\/05\/Prim\u00e1rny-liposark\u00f3m-pankreasu-\u2013-kazuistika-a-preh\u013ead-literat\u00fary.pdf","link":"https:\/\/www.newslab.sk\/en\/primary-pancreatic-liposarcoma-the-case-report-and-literature-review\/primarny-liposarkom-pankreasu-kazuistika-a-prehlad-literatury-2\/","alt":"","author":"7","description":"","caption":"","name":"primarny-liposarkom-pankreasu-kazuistika-a-prehlad-literatury-2","status":"inherit","uploaded_to":1706,"date":"2019-05-09 08:36:42","modified":"2019-05-09 08:36:42","menu_order":0,"mime_type":"application\/pdf","type":"application","subtype":"pdf","icon":"https:\/\/www.newslab.sk\/wp-includes\/images\/media\/document.png"}},"_links":{"self":[{"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/posts\/1706","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/users\/7"}],"replies":[{"embeddable":true,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/comments?post=1706"}],"version-history":[{"count":0,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/posts\/1706\/revisions"}],"wp:attachment":[{"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/media?parent=1706"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/categories?post=1706"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/tags?post=1706"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}