{"id":1174,"date":"2017-01-31T20:59:29","date_gmt":"2017-01-31T19:59:29","guid":{"rendered":"http:\/\/www.newslab.sk\/2017\/01\/31\/malobunkovy-hyperkalcemicky-karcinommaligny-rhabdoidny-tumor-ovaria-kazuistika\/"},"modified":"2017-10-04T14:26:09","modified_gmt":"2017-10-04T12:26:09","slug":"small-cell-hypercalcaemic-carcinomarhabdoid-tumour-of-the-ovary-a-case-study","status":"publish","type":"post","link":"https:\/\/www.newslab.sk\/en\/small-cell-hypercalcaemic-carcinomarhabdoid-tumour-of-the-ovary-a-case-study\/","title":{"rendered":"Small-cell Hypercalcaemic Carcinoma\/Rhabdoid Tumour of the Ovary: A Case Study"},"content":{"rendered":"<pre><span style=\"color: #ff0000;\"><strong>*All tables, charts, graphs and pictures that are featured in this article can be found in the .pdf\u00a0\r\nattachment at the end of the paper.\r\n\r\n<\/strong><\/span><\/pre>\n<p><strong>\u00da<\/strong><strong>vod<\/strong><\/p>\n<p>Malobunkov\u00fd karcin\u00f3m ov\u00e1ria hyperkalcemick\u00e9ho typu (MKOTH) je ve\u013emi zriedkav\u00fd tumor vyskytuj\u00faci sa zv\u00e4\u010d\u0161a u mlad\u00fdch \u00a0\u017eien. V typick\u00fdch pr\u00edpadoch sa u t\u00fdchto pacientok zis\u0165uje hyperkalc\u00e9mia. Tumor je vysoko agres\u00edvny a m\u00e1 zl\u00fa progn\u00f3zou (1, 2, 3, 4). Celkom ned\u00e1vno boli v tomto n\u00e1dore zisten\u00e9 genetick\u00e9 zmeny typick\u00e9 pre mal\u00edgny rhabdoidn\u00fd tumor, a preto niektor\u00ed autori za\u010dali pou\u017e\u00edva\u0165 pre MKOTH aj ozna\u010denie \u00a0mal\u00edgny rhabdoidn\u00fd tumor ov\u00e1ria (5,\u00a06). Z na\u0161ej praxe prezentujeme pr\u00edpad tohto raritn\u00e9ho tumoru.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>O<\/strong><strong>pi<\/strong><strong>s pr\u00edpadu<\/strong><\/p>\n<p>22-ro\u010dn\u00e1 pacientka v prvom trimestri tehotenstva bola prijat\u00e1 pre bolesti podbru\u0161ka. \u00a0Sonograficky bol zisten\u00fd tumor prav\u00e9ho ov\u00e1ria a n\u00e1sledne bola preveden\u00e1 hysterek t\u00f3mia s bilater\u00e1lnou adnexot\u00f3miou. Po stanoven\u00ed diagn\u00f3z y nasledovala lie\u010dba chemo &#8211; terapiou. Pacientka exitovala 12 mesiacov po oper\u00e1cii v d\u00f4sledku rekurencie tumoru v panve a bru\u0161nej dutine. \u00a0Hyperkalc\u00e9mia nebola zisten\u00e1.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>V\u00fdsledky<\/strong><\/p>\n<p>Makroskopicky i\u0161lo o ovoidn\u00fd tumor priemeru do 10 cm, na reze sol\u00eddny, bledosiv\u00fd a z \u010dasti cystick\u00fd, s kompletnou \u00a0tenkou pseudokapsulou. Histologicky bol tumor tvoren\u00fd preva\u017ene sol\u00eddnou popul\u00e1ciou mal\u00fdch okr\u00fahlych buniek, s nekr\u00f3zami a folikulom podobn\u00fdmi priestormi (obr\u00e1zok 1 a 2). Fok\u00e1lne boli bunky raden\u00e9 do trabek\u00fal a pruhov (obr\u00e1zok 3). Cytoplazma buniek bola m\u00e1lo objemn\u00e1, \u010dasto temer nezrete\u013en\u00e1. Jadr\u00e1 mali monot\u00f3n- ny high-grade vzh\u013ead s po\u010detn\u00fdmi mit\u00f3zami a nen\u00e1padn\u00fdmi jadierkami. V ojedinel\u00fdch lo\u017eisk\u00e1ch bol tumor sarkomatoidn\u00fd, s vretenovit\u00fdmi bunkami bohat\u0161\u00edmi na cytoplazmu \u00a0(obr\u00e1zok 4). \u010cas\u0165 buniek \u00a0mala mal\u00e9 \u00a0mno\u017estvo excentricky ulo\u017eenej cytoplazmy, ale prominentn\u00e9 \u00a0nukleoly typick\u00e9 pre rhabdoidn\u00fa cytomorfol\u00f3giu \u00a0sme nena\u0161li. Medzibunkov\u00e1 str\u00f3ma mala vzh\u013ead fibr\u00f3zny kolagenizovan\u00fd alebo myxoidn\u00fd. Imunohistochemicky bol tumor pozit\u00edvny dif\u00fazne na viment\u00edn (obr\u00e1zok 5), WT1 (obr\u00e1zok 6) a p53. Fok\u00e1lne boli pozit\u00edvne pancytokerat\u00edn AE1\/AE3 (obr\u00e1zok 7), epitelov\u00fd membr\u00e1nov\u00fd antig\u00e9n \u00a0(EMA) \u00a0(obr\u00e1zok \u00a08), INI1, CD99 a CD10. Negat\u00edvne boli kalretin\u00edn, inhib\u00edn, estrog\u00e9nov\u00e9 a progester\u00f3nov\u00e9 receptory, LCA, OCT3\/4, c-kit, PLAP, S100-prote\u00edn, HMB45, melan A, CD56, synaptofyz\u00edn, chromogran\u00edn, calpon\u00edn, alfa-hladkosvalov\u00fd akt\u00edn, desm\u00edn, kaldesmon, neurofilament \u00a0prote\u00edn a Fli-1.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>Dis<\/strong><strong>k<\/strong><strong>usia<\/strong><\/p>\n<p>Klinick\u00e9 aj patologick\u00e9 n\u00e1lezy v na\u0161om pr\u00edpade sved\u010dia o MKOTH. Tento zriedkav\u00fd tumor bol op\u00edsan\u00fd Scullym et al. a Dickersinom et al. (1, 4). Po t\u00fdchto pr\u00e1cach nasledovali publik\u00e1cie \u010fal\u0161\u00edch kazuist\u00edk a s\u00e9ri\u00ed a v s\u00fa\u010dasnosti je v p\u00edsomn\u00edctve viac ako 300 pr\u00edpadov (1, 7, 8). Okrem tumorov s \u201etradi\u010dn\u00fdm\u201c malobunkov\u00fdm obrazom boli pozorovan\u00e9 aj l\u00e9zie s obsahom v\u00e4\u010d\u0161\u00edch buniek, ktor\u00e9 vykazovali niekedy rhabdoidn\u00fa cytomorfol\u00f3giu, \u010d\u00edm sa podobali na tzv. mal\u00edgny \u00a0rhabdoidn\u00fd \u00a0tumor \u00a0(9). Predpoklad\u00e1me, \u017ee pr\u00e1ve tak\u00e9to n\u00e1lezy viedli Kupryjanczyka \u00a0et al. k molekul\u00e1rno-genetick\u00e9mu p\u00e1traniu po\u00a0mut\u00e1ci\u00e1ch, ktor\u00e9 sa typicky vyskytuj\u00fa v rhabdoidn\u00fdch tumoroch (10). Ide o alter\u00e1cie g\u00e9nov <em>S<\/em><em>M<\/em><em>A<\/em><em>R<\/em><em>C<\/em><em>B<\/em><em>1 \u00a0<\/em>a <em>S<\/em><em>M<\/em><em>A<\/em><em>R<\/em><em>C<\/em><em>A<\/em><em>4 <\/em>(11, 12, 13). Kupryjanczyk \u00a0et al. (10) zistili, \u017ee aj v MKOTH je charakteristick\u00e1 mut\u00e1cia <em>S<\/em><em>M<\/em><em>A<\/em><em>R<\/em><em>C<\/em><em>4<\/em><em>\/<\/em><em>BR<\/em><em>G<\/em><em>1 \u00a0<\/em>(podobne ako v rhabdoidn\u00fdch tumoroch), \u010do bolo potvrden\u00e9 aj \u010fal\u0161\u00edmi \u0161t\u00fadiami (5,\u00a014). Z tohto\u00a0 d\u00f4vodu \u00a0navrhuj\u00fa niektor\u00ed autori (5, 6) ozna\u010dova\u0165 MKOTH ako mal\u00edgny rhabdoidn\u00fd tumor ov\u00e1ria, \u010d\u00edm by sa MKOTH vlastne preradil medzi sark\u00f3my. Tak\u00e1to klasifik\u00e1cia je podporen\u00e1 \u010fal\u0161\u00edmi znakmi, ktor\u00e9 s\u00fa \u201espolo\u010dn\u00e9\u201c pre obe spom\u00ednan\u00e9 jednotky: \u010dast\u00e1 hyperkalc\u00e9mia, \u010dast\u00e1 imunohistochemick\u00e1 polyfenotypia, mo\u017en\u00fd \u00a0famil\u00e1rny v\u00fdskyt (3, 5, 15). Nevysvetlen\u00e9 ale zost\u00e1vaj\u00fa pri MKOTH n\u00e1lezy netypick\u00e9 pre rhabdoidn\u00fd tumor, ako s\u00fa mucin\u00f3zne \u017eliazky a \u010dast\u00e1 absencia \u201eklasickej\u201c rhabdoidnej morfol\u00f3gie. Taktie\u017e molekul\u00e1rno-genetick\u00e9 \u00a0n\u00e1lezy alter\u00e1ci\u00ed <em>S<\/em><em>M<\/em><em>A<\/em><em>R<\/em><em>C<\/em><em>4 \u00a0<\/em>v in\u00fdch n\u00e1doroch (endometrioidn\u00fd karcin\u00f3m, pulmon\u00e1lne a pankreatick\u00e9 karcin\u00f3my) (16, 17) znamenaj\u00fa ich ni\u017e\u0161iu \u0161pecificitu na ur\u010dovanie histogen\u00e9zy.<\/p>\n<p><strong>Klinick\u00fd obraz MKOTH zah\u0155\u0148a:<\/strong> mlad\u00fd vek s medi\u00e1nom \u00a024 \u2013 28 rokov (1, 8), hyperkalc\u00e9mia v dvoch tretin\u00e1ch pr\u00edpadov (1), vo v\u00e4\u010d\u0161ine pr\u00edpadov pri prvej diagn\u00f3ze \u0161t\u00e1dium vy\u0161\u0161ie ako \u0161t\u00e1dium I (a\u017e 75 % pacientok) \u00a0(3). Hodnoty be\u017ene pou\u017e\u00edvan\u00fdch s\u00e9rov\u00fdch markerov b\u00fdvaj\u00fa ne\u0161pecifick\u00e9, respekt\u00edve nepr\u00ednosn\u00e9 (AFP, beta-HCG, CA125, LDH). Diagnostick\u00fa hodnotu m\u00e1 len n\u00e1lez hyperkalc\u00e9mie zistenej u mladej \u017eeny s n\u00e1dorovou l\u00e9ziou adnex. V na\u0161om pr\u00edpade ale nebola hyperkalc\u00e9mia \u00a0zisten\u00e1, ako tomu b\u00fdva v tretine pr\u00edpadov (1).<\/p>\n<p>Histologicky pri MKOTH nach\u00e1dzame popul\u00e1ciu mal\u00fdch buniek s v\u00fdraznou atypiou, vysokou mitotickou aktivitou a \u010dastou nekr\u00f3zou. Bunky s\u00fa usporiadan\u00e9 zv\u00e4\u010d\u0161a sol\u00eddne, len fok\u00e1lne s v\u00e4\u010d\u0161\u00edm mno\u017estvom intercelul\u00e1rnej v\u00e4zivovej str\u00f3my. Menej \u010dast\u00e9 s\u00fa trabekuly a pruhy buniek. Tvar buniek je okr\u00fahly a ovoidn\u00fd. V sol\u00eddnych \u010dastiach vznikaj\u00fa dehiscenciou \u00a0bunkov\u00fdch spojov \u00a0charakteristick\u00e9 \u00a0dutiny \u00a0r\u00f4znej ve\u013ekosti, podobn\u00e9 folikulom. Zriedka sa na\u0161li v tumore mucin\u00f3zne \u00a0\u017eliazky alebo \u201esignet ring\u201c bunky,\u00a0vretenobunkov\u00e1 sarkomatoidn\u00e1 zmena (ako v na\u0161om pr\u00edpade), popul\u00e1cia\u00a0v\u00e4\u010d\u0161\u00edch buniek alebo \u0161trukt\u00fara neodl\u00ed\u0161ite\u013en\u00e1 od rhabdoidn\u00e9ho tumoru (3). Imunohistochemicky \u00a0exprimuje tumor viment\u00edn a (aspo\u0148 fok\u00e1lne) epitelov\u00e9 markery CK a\/alebo EMA. Temer kon\u0161tantn\u00e1 je absencia expresie prote\u00ednu BRG1 (ktor\u00fd je produktom g\u00e9nu <em>S<\/em><em>M<\/em><em>A<\/em><em>R<\/em><em>C<\/em><em>A<\/em><em>4<\/em>) (6). \u010ealej boli op\u00edsan\u00e9 expresie p53, CD56, WT1, kalretin\u00ednu, CD10, \u201eparathyroid-related\u201c polypeptidu, alfa-hladkosvalov\u00e9ho \u00a0akt\u00ednu (3, 15). Negat\u00edvne s\u00fa estrog\u00e9nov\u00e9 \u00a0i progester\u00f3nov\u00e9 receptory (argument proti \u201esex-cord\u201c p\u00f4vodu tumoru, ako aj proti origu z mulleri\u00e1nskeho, respekt\u00edve povrchov\u00e9ho \u00a0epitelu ov\u00e1ria) (15).<\/p>\n<p>Histologick\u00e1 \u00a0diferenci\u00e1lna \u00a0diagn\u00f3za \u00a0je \u0161irok\u00e1. Zah\u0155\u0148a \u00a0cel\u00e9 \u00a0spektrum malobunkov\u00fdch l\u00e9zi\u00ed ov\u00e1ria, ako s\u00fa n\u00e1dor z buniek granul\u00f3zy, dysgermin\u00f3m, malobunkov\u00fd karcin\u00f3m typu pulmon\u00e1lneho, endometri\u00e1lny strom\u00e1lny sark\u00f3m, tumory skupiny PNET\/Ewing, alveol\u00e1rny rhabdomyosar- k\u00f3m, desmoplastick\u00fd \u201esmall round cell\u201c tumor, mal\u00edgny lymf\u00f3m, mal\u00edgny melan\u00f3m. Naj\u010dastej\u0161\u00edm probl\u00e9mom v diferenci\u00e1lnej diagn\u00f3ze s\u00fa granul\u00f3zocelul\u00e1rny n\u00e1dor a dysgermin\u00f3m. Granul\u00f3zocelul\u00e1rne tumory nemaj\u00fa jadr\u00e1 tak v\u00fdrazne atypick\u00e9 ako v MKOTH a exprimuj\u00fa \u201esex-cord\u201c markery,<\/p>\n<p>\u201esex-steroid\u201c receptory a BRG1. Dysgermin\u00f3m m\u00f4\u017ee obsahova\u0165 folikulom podobn\u00e9 priestory, ale m\u00e1 svetlej\u0161ie bunky, str\u00f3mu s lymfocytmi a odli\u0161n\u00fd imunofenotyp (pozit\u00edvne PLAP, OCT3\/4, c-kit, D2-40). Pulmon\u00e1lny typ karcin\u00f3mu je negat\u00edvny na viment\u00edn a pozit\u00edvny na neuroendokrinn\u00e9 markery. \u010casto obsahuje endometrioidn\u00fa alebo \u201eurotel-like\u201c zlo\u017eku. Endometri\u00e1lny strom\u00e1lny sark\u00f3m m\u00e1 charakteristick\u00fa bohat\u00fa vaskulariz\u00e1ciu. \u010eal\u0161ie vy\u0161\u0161ie spomenut\u00e9 tumory maj\u00fa imunofenotypy odli\u0161n\u00e9 od MKOTH, \u010do umo\u017e\u0148uje ich odl\u00ed\u0161enie. Potrebn\u00fd je ale v\u017edy pri konkr\u00e9tnej diferenci\u00e1lnej diagn\u00f3ze panel protil\u00e1tok, pri\u010dom treba ma\u0165 na pam\u00e4ti polyimunofenotypiu MKOTH.<\/p>\n<p>Terapia MKOTH\u00a0 je v menej pokro\u010dil\u00fdch \u0161t\u00e1di\u00e1ch v prvom rade chi- rurgick\u00e1, zv\u00e4\u010d\u0161a \u00a0podobn\u00e9ho \u00a0rozsahu ako pri high-grade \u00a0ovari\u00e1lnych karcin\u00f3moch. N\u00e1sledne sa aplikuje chemoterapia (etopozid + cisplatina), pr\u00edpadne aj r\u00e1dioterapia (18). N\u00e1zory na optim\u00e1lnu lie\u010dbu sa ale pri prvom \u0161t\u00e1diu zna\u010dne l\u00ed\u0161ia. Powel et al. odpor\u00fa\u010daj\u00fa \u00a0dokonca \u00a0v indikovan\u00fdch pr\u00edpadoch \u00a0fertilitu zachov\u00e1vaj\u00faci \u00a0z\u00e1krok, s n\u00e1slednou onkologickou terapiou a stagingovou \u00a0oper\u00e1ciou \u00a0(19). Naproti tomu \u00a0Pautier et al. operuj\u00fa radik\u00e1lne, s pelvickou a paraaort\u00e1lnou lymfadenekt\u00f3miou \u00a0a n\u00e1slednou chemoterapiou (20). V pokro\u010dil\u00fdch \u0161t\u00e1di\u00e1ch je odpor\u00fa\u010dan\u00e1 neoadjuvantn\u00e1 chemoterapia a n\u00e1sledn\u00e1 stagingov\u00e1 oper\u00e1cia (18).<\/p>\n<p>Progn\u00f3za tumoru je celkovo ve\u013emi nepriazniv\u00e1, diktovan\u00e1 vysokou agresivitou n\u00e1doru \u00a0(1, 3). L\u00ed\u0161i sa viac menej \u00a0pod\u013ea \u0161t\u00e1di\u00ed. Len u tretiny pacientok so \u0161t\u00e1diom 1a b\u00fdva po oper\u00e1cii obdobie bez ochorenia. Ide hlavne o pacientky s vekom nad 30 rokov, norm\u00e1lnou \u00a0kalc\u00e9miou, tumorom priemeru pod 10 cm, absenciou ve\u013ek\u00fdch buniek a po absolvovan\u00ed radik\u00e1lnej chirurgickej lie\u010dby (1). Pri vy\u0161\u0161\u00edch \u0161t\u00e1di\u00e1ch je progn\u00f3za infaustn\u00e1, s \u00famrt\u00edm do 2 rokov v drvivej v\u00e4\u010d\u0161ine pr\u00edpadov.<\/p>\n<p>&nbsp;<\/p>\n<p><strong>Z<\/strong><strong>\u00e1<\/strong><strong>ver<\/strong><\/p>\n<p>Prezentovan\u00fd pr\u00edpad zriedkav\u00e9ho MKOTH bol z h\u013eadiska kliniky aj patol\u00f3gie podobn\u00fd doposia\u013e publikovan\u00fdm pr\u00edpadom. Histologicky \u0161lo o konven\u010dn\u00fd typ tohto tumoru, s preva\u017euj\u00facou high-grade popul\u00e1ciou mal\u00fdch buniek a typick\u00fdmi folikulom podobn\u00fdmi priestormi. V tumore \u00a0bola fok\u00e1lne i menej obvykl\u00e1 sarkomatoidn\u00e1 \u0161trukt\u00fara, bez rhabdoidnej a mucin\u00f3znej morfol\u00f3gie. Imunohistochemicky vykazoval tumor len fok\u00e1lnu expresiu epitelov\u00fdch markerov. Hyperkalc\u00e9mia \u00a0nebola zisten\u00e1. Priebeh bol u pacientky infaustn\u00fd, s \u00famrt\u00edm\u00a012 mesiacov po diagn\u00f3ze, \u010do potvrdzuje vysoko mal\u00edgny charakter tohto n\u00e1doru.<\/p>\n<p><em>P<\/em><em>o\u010fa<\/em><em>k<\/em><em>ovanie: <\/em><em>T<\/em><em>ento \u010dl\u00e1nok vznikol v\u010faka podpore <\/em><em>v r\u00e1mci OP V\u00fdskum a v\u00fdvoj pre projekt: Dobudovanie technickej infra\u0161trukt\u00fary v oblasti v\u00fdskumu diagnostick\u00fdch postupov a met\u00f3d v r\u00e1mci v\u010dasnej diagnostiky naj\u010dastej\u0161\u00edch onkologick\u00fdch ochoren\u00ed u \u017eien, ITMS 26210120026, spolufinancovan\u00fd zo zdrojov Eur\u00f3pskeho fondu region\u00e1lneho rozvoja.<\/em><\/p>\n<p>&nbsp;<\/p>\n<p><strong>L<\/strong><strong>it<\/strong><strong>e<\/strong><strong>ra<\/strong><strong>t<\/strong><strong>\u00fa<\/strong><strong>ra<\/strong><\/p>\n<p><strong>1<\/strong><strong>. <\/strong>Young RH, Oliva E, Scully RE. Small cell carcinoma of the ovary, hypercalcemic typ A clinicopathological analysis of 150 cases. <em>A<\/em><em>m J Surg Pathol. <\/em>1994;18(11):1102\u20131116.<\/p>\n<p><strong>2<\/strong><strong>. <\/strong>Dickersin GR, Kline IW, Scully RE. Small cell carcinoma of the ovary with hypercalcemia: a report of 11 cases. <em>C<\/em><em>an<\/em><em>c<\/em><em>e<\/em><em>r<\/em><em>. <\/em>1982;49(1):188\u2013197.<\/p>\n<p><strong>3<\/strong><strong>. <\/strong>Clement PB. Selected miscellaneous ovarian lesions: small cell carcinomas, mesothelial lesions, mesenchymal and mixed neoplasms, and non-neoplastic lesions. <em>M<\/em><em>o<\/em><em>d Pathol.\u00a0<\/em>2005;18(Suppl. 2):S113\u2013129.<\/p>\n<p><strong>4<\/strong><strong>. <\/strong>Scully RE. <em>T<\/em><em>umor<\/em><em>s <\/em><em>o<\/em><em>f the ovary and maldeveloped gonads. Atlas of Tumor Pathology. <\/em>2nd series, Fascicle 16. Washington, DC: Armed Forces Institute of Pathology; 1979.<\/p>\n<p><strong>5<\/strong><strong>. <\/strong>Foulkes WD, Clarke BA, Hasselblatt M, et al. No small surprise \u2013 small cell carcinoma of the ovary, hypercalcaemic type, is a malignant rhabdoid tumour. <em>J Pathol. <\/em>2014;233(3):209\u2013214.<\/p>\n<p><strong>6<\/strong><strong>. <\/strong>Karanian-Philippe M, Velasco V, Longy M, <em>e<\/em><em>t al. <\/em>SMARCA4 (BRG1) loss of expression is a use- ful marker for the diagnosis of ovarian small cell carcinoma of the hypercalcemic type (ova- rian rhabdoid tumor): a comprehensive \u00a0analysis of 116 rare gynecologic \u00a0tumors, 9 soft tissue tumors, and 9 melanomas. <em>A<\/em><em>m J Surg Pathol. <\/em>2015;39(9):1197\u20131205.<\/p>\n<p><strong>7<\/strong><strong>. <\/strong>Kapoun M, Bouda J, Presl J, et al. 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Genomic characterization of non-small-cell lung cancer in African Americans by targeted massively parallel sequencing. <em>J Clin Oncol. <\/em>2015;33(17):1966\u20131973.<\/p>\n<p><strong>1<\/strong><strong>7<\/strong><strong>. <\/strong>Strehl JD, Wachter DL, Fiedler J, et al<em>. <\/em>Pattern of SMARCB1 (INI1) and SMARCA4 (BRG1) in poorly differentiated endometrioid \u00a0adenocarcinoma \u00a0of the uterus: \u00a0analysis of a series with emphasis\u00a0 on a novel SMARCA4-deficient\u00a0 dedifferentiated rhabdoid variant. <em>An<\/em><em>n Diagn Pathol. <\/em>2015;19(4):198\u2013202.<\/p>\n<p><strong>1<\/strong><strong>8<\/strong><strong>. <\/strong>Harrison ML, Hoskins P, du Bois A, et al. Small cell of the ovary, hypercalcemic type \u2013 analysis of combined \u00a0experience and recommendation \u00a0for management. A GCIG study. <em>G<\/em><em>yn<\/em><em>e<\/em><em>col Oncol. <\/em>2006;100(2):233\u2013238.<\/p>\n<p><strong>1<\/strong><strong>9<\/strong><strong>. <\/strong>Powell JL, McAfee,RD, McCoy RC, et al. Uterine and ovary conservation in advanced small cell carcinoma of the ovary. <em>O<\/em><em>bstet Gynecol. <\/em>1998;91(5 Pt 2):846\u2013848.<\/p>\n<p><strong>2<\/strong><strong>0<\/strong><strong>. <\/strong>Pautier P, Ribrag V, Duvillard \u00a0P, et al. Results of a prospective dose \u2013 intense regimen in 27 patients with small cell carcinoma \u00a0of the ovary of the hypercalcemic \u00a0type. <em>An<\/em><em>n Oncol.\u00a0<\/em>2007;18(12):1985\u20131989.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>*All tables, charts, graphs and pictures that are featured in this article can be found in the .pdf\u00a0 attachment at the end of the paper. \u00davod Malobunkov\u00fd karcin\u00f3m ov\u00e1ria hyperkalcemick\u00e9ho typu (MKOTH) je ve\u013emi zriedkav\u00fd tumor vyskytuj\u00faci sa zv\u00e4\u010d\u0161a u mlad\u00fdch \u00a0\u017eien. V typick\u00fdch pr\u00edpadoch sa u t\u00fdchto pacientok zis\u0165uje hyperkalc\u00e9mia. Tumor je vysoko agres\u00edvny<\/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":[763,764,745,762],"class_list":["post-1174","post","type-post","status-publish","format-standard","hentry","category-pathology","tag-immunohistochemistry","tag-malign-rhabdoid-tumour","tag-ovary","tag-small-cell-carcinoma-of-hypercalcaemic-type","typ_clanku-casuistry"],"acf":{"abstrakt":"<p><strong>Introduction: <\/strong>Small-cell ovarian carcinoma of hypercalcaemic type is a very rare tumour in young women, which has recently been classified in the group of malign rhabdoid tumour.<\/p>\n<p><strong>Case description and results: <\/strong>The presented case is a tumour of a 22-year old patient. This is a tumour of the right ovary with diameter of 10cm. Hypercalcemia has not been detected. Therapy comprised hysterectomy and bilateral adnexectomy with subsequent chemotherapy. Histological findings corresponded with small-cell hypercalcaemic carcinoma, conventional histological picture with very rare deposits of sarcomatoid spindle-cell change. Other, less typical structures, such as mucinous mucous membranes or a population of large or rhabdoid cells has not been found in the tumour. From immunohistochemistry perspective, the tumour expressed cytokeratin AE1\/AE3, EMA, WT1, CD99, INI1 and CD10. Estrogen and progesterone receptors were negative, as well as inhibin, calretinin, OCT3\/4, c-kit, PLAP, LCA, S100-protein, HMB45, melan A, chromogranin, CD56, synaptophysin, alpha-smooth muscle actin, desmin, kaldesmon, Fli-1 and neurofilament protein. However, the patient progressed negatively. Despite the treatment, she expired 12 months after the surgery.<\/p>\n<p><strong>Discussion: <\/strong>The paper discusses histological and immunohistochemical diagnosis of the tumour and its latest classification among malign rhabdoid tumours.<\/p>\n<p><strong>Conclusion: <\/strong>The described case demonstrates a typical clinical and pathological picture of a small-cell hypercalcaemic carcinoma of the ovary.<\/p>\n<p><strong>Key words: <\/strong>ovary, small-cell carcinoma of hypercalcaemic type, immunohistochemistry, malign rhabdoid tumour<\/p>\n","casopis":[{"ID":991,"post_author":"7","post_date":"2017-02-01 09:43:42","post_date_gmt":"2017-02-01 08:43:42","post_content":"<ul>\r\n \t<li>Pulmonary aspergillosis<\/li>\r\n \t<li>Infections caused by cytomegalovirus \u2013 diagnosis and therapy<\/li>\r\n \t<li>Long-term molecular remission as a precondition for successful pregnancy in patients with chronic myelocyte leukemia<\/li>\r\n \t<li>Chromosome 11 aberrations in a patient with acute myeloid leukemia \u2013 a case study<\/li>\r\n \t<li>New biomarkers in diagnosing IgA nephropathy<\/li>\r\n<\/ul>","post_title":"newslab","post_excerpt":"","post_status":"publish","comment_status":"closed","ping_status":"closed","post_password":"","post_name":"newslab-2016-02","to_ping":"","pinged":"","post_modified":"2017-08-16 21:36:48","post_modified_gmt":"2017-08-16 19:36:48","post_content_filtered":"","post_parent":0,"guid":"http:\/\/www.newslab.sk\/casopis\/newslab-2016-02\/","menu_order":0,"post_type":"casopis","post_mime_type":"","comment_count":"0","filter":"raw"}],"strana":"129","upload_clanok":{"ID":1175,"id":1175,"title":"Newslab_2_2016_Malobunkov\u00fd hyperkalcemick\u00fd karcin\u00f3m","filename":"Newslab_2_2016_Malobunkov\u00fd-hyperkalcemick\u00fd-karcin\u00f3m.pdf","filesize":196915,"url":"https:\/\/www.newslab.sk\/wp-content\/uploads\/2017\/01\/Newslab_2_2016_Malobunkov\u00fd-hyperkalcemick\u00fd-karcin\u00f3m.pdf","link":"https:\/\/www.newslab.sk\/en\/small-cell-hypercalcaemic-carcinomarhabdoid-tumour-of-the-ovary-a-case-study\/newslab_2_2016_malobunkovy-hyperkalcemicky-karcinom\/","alt":"","author":"7","description":"","caption":"","name":"newslab_2_2016_malobunkovy-hyperkalcemicky-karcinom","status":"inherit","uploaded_to":1174,"date":"2017-02-01 19:25:48","modified":"2017-02-01 19:25:48","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\/1174","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=1174"}],"version-history":[{"count":0,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/posts\/1174\/revisions"}],"wp:attachment":[{"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/media?parent=1174"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/categories?post=1174"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.newslab.sk\/en\/wp-json\/wp\/v2\/tags?post=1174"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}