Primary Chondrosarcoma nyob rau hauv L-shaped Hla Fused Raum Ectopia Coexisting Nrog Papillary Urothelial Carcinoma nyob rau hauv lub zais zis - ib tug enigmatic qhov chaw nrog rau cov neeg pluag prognosis

Hu rau:joanna.jia@wecistanche.com/ WhatsApp: 008618081934791

Mayank Kumar¹, Aasma Nalwa²ua al

Abstract

Thawj lub raum chondrosarcomas yog cov qog uas tsis tshua muaj qib siab hauv qhov xwm txheej thiab, hmoov tsis zoo, tau nkag siab tsis zoo rau cov kab mob thiab cov kab mob tsawg heev. Kev sib koom ua ke ntawm kev tsis sib haum xeeb hauv lub zais zis yog qhov tsis tshua muaj, nrog qhov tshwm sim ntawm papillary urothelial carcinoma nyob rau hauv lub zais zis hauv qhov no. Qhov kev nthuav qhia kho mob tsis yog tshwj xeeb, thiab cov kev tshawb nrhiav hluav taws xob tseem ceeb muaj qhov txwv tsis pub muaj kev kuaj mob tshwj xeeb ntawm lub koom haum no. Qhov kev kuaj mob zaum kawg tuaj yeem ua tiav ntawm kev kuaj mob histopathological ntawm cov qauv kuaj xyuas, uas yuav tsum tau kuaj xyuas ntau thiab kev qhia ntxaws. Txog tam sim no, tib txoj hauv kev kom ua tiav qhov kev kwv yees zoo dua yog los ntawm kev kuaj mob ntxov. Nws yog ib qho tsim nyog yuav tsum ua kom muaj peev xwm tshwm sim ntawm sarcomas ntawm qhov chaw tsis tshua muaj nyob rau hauv kev kuaj mob sib txawv. Cov cytogenetic thiab molecular abnormalities txuam nrog rau qhov chaw no yuav tsum tau elucidated kom ua tau ib tug ntau txaus siab rau cov kev tswj tag nrho ntawm tus neeg mob.

Ntsiab lus:chondrosarcoma; raum; lub raum ectopia; tso zis tso zis; urothelial carcinoma

Taw qhia

Thawj lub raum sarcomas yog cov qog tsis tshua muaj tshwm sim, suav txog tsuas yog 1 feem pua ​​- 5 feem pua ​​​​ntawm tag nrho cov raum tsis zoo (1). Ntawm cov no, thawj lub raum chondrosarcomas yog qhov tsawg kawg nkaus, tsuas yog qee qhov xwm txheej qhia hauv cov ntaub ntawv.

Qhov kev mob qog noj ntshav siab no muaj qhov tshwj xeeb biphasic histology nrog rau kev nkag siab tsis zoo ntawm pathogenesis, kab mob kab mob, thiab txawm tias qhov tsis zoo (2). Ntawm no peb piav qhia txog thawj rooj plaub uas sau tseg qhov tshwm sim ntawm thawj lub raum chondrosarcoma nyob rau hauv L-shaped crossed fused raum ectopia uas muaj ob qho tib si moieties, nrog rau cov papillary urothelial carcinoma nyob rau hauv lub zais zis.

cistanche salsa cov txiaj ntsigtuaj yeem kho tauCov tsos mob ntawm lub raum tsis ua haujlwm

Case Report

Ib tug txiv neej muaj hnub nyoog 50- xyoo muaj hematuria, nce zaus, thiab kub hnyiab thaum lub sij hawm micturition nrog rau sab laug flank mob rau 2 lub hlis. Muaj keeb kwm ntawm kev qaug zog, poob phaus, thiab tsis qab los noj mov thaum lub sijhawm no. Hauv kev kuaj xyuas, tus neeg mob tau noj tsis zoo nrog qhov muaj palpable sab laug sab plab plab.

Ultrasonography (USG) ntawm lub plab tau pom tias muaj txoj cai ectopicraumthiab sab laug-sab hydronephrosis. Ib heterogeneous loj tau sau tseg nyob rau hauv lub raum sab laug. Lwm polypoidal loj kuj tau pom nyob rau hauv lub lumen ntawm lub zais zis, txuas rau nws posterolateral phab ntsa.

Contrast-enhanced computerized tomography (CECT) ntawm lub plab mog pab nyob rau hauv lub raum anatomy thiab characterization ntawm lub loj, uas qhia tau hais tias L-shaped crossed fused raum ectopia. Txoj cairaumtsis muaj nyob rau hauv txoj cai lub raum fossa thiab nyob rau hauv nruab nrab, anterior mus rau lub aortic bifurcation ntawm L4-L5 theem. Nws yog malrotation thiab fused nrog qis ncej ntawm lub raum sab laug. Lub raum sab laug tau nthuav dav, nrog cov mos mos heterogeneous cov nqaij cuam tshuam nrog cov cheeb tsam cuam tshuam nrog cov cheeb tsam cuam tshuam nrog nruab nrab uas tsis txhim kho nruab nrab. Ob peb calcified foci tau pom nyob rau hauv pawg, nrog rau nruab nrab hydronephrosis. Hauv lub sijhawm ncua sijhawm (15 feeb), tsis muaj qhov sib txawv ntawm lub raum sab laug tau sau tseg. Lub cheeb tsam interpolar ntawm lub raum sab xis yog contiguously infiltrated los ntawm sab laug ncej ntawm lub raum loj. Cov qog me me thrombi tau tshwm sim nyob rau hauv segmental txoj cai raum cov hlab ntsha ntws mus rau thaj tsam interpolar. Aortocaval, para-aortic thiab sab laug raum hilar lymphadenopathy kuj tau sau tseg.

Nrog rau cov kev tshawb pom no, ib qho zoo-txhais polypoidal loj tau pom nyob rau sab laug posterolateral phab ntsa ntawm lub zais zis, infiltrating sab laug vesicoureteral hlws ris. Qhov nruab nrab thiab qhov nruab nrab ntawm sab laug ureter tau txuas nrog los ntawm qhov tso zis zais zis. Ib qho peripheral rim ntawm calcification tau muaj.

Vim muaj kev koom tes ntawm multifocal pab pawg ntawm cov moieties ntawm crossed fused raum ectopia, urinary zais zis, thiab sab laug ureter, lub radiological txawv diagnostics muaj yog multifocal transitional cell carcinoma, raum cell carcinoma (RCC) - mucinous adenocarcinoma variant thiab multifocal. lub raum sarcoma.

Tsis muaj qhov mob nyob deb tau pom ntawm kev ua haujlwm metastatic.

Thaum pib, transurethral resection ntawm lub zais zis lesion tau ua tiav. Kev kuaj xyuas microscopic pom cov yam ntxwv ntawm noninvasive papillary urothelial carcinoma, feem ntau qis-qib nrog cov qib siab focal thaj chaw, nrog rau cov dystrophic calcification thiab necrosis, thiab focal osseous metaplasia (Daim duab 1).

Tom qab ntawd tus neeg mob tau raug coj mus rau kev phais rau resection ntawm lub raum loj, thiab sab laug nephrectomy nrog ib nrab txoj cai nephrectomy kuj tau ua. Cov qauv tau xa mus rau kev kuaj histopathological. Cov txiaj ntsig tau pom tias cov tshuaj ntsiav tsis zoo. Txiav ntu ntawm sab laugraumpom cov qog ntsuas 14x11x10 cm, hloov tag nrho cov qauv qub. Lub raum pelvis tsis tau txheeb xyuas, thiab ib feem ntawm txoj cairaummuaj cov cystic thiab cov khoom, nrog cov qog ntsuas 3x2.5x2 cm (Daim duab 2).

Ntau qhov kev tshuaj xyuas los ntawm ob qho tib silub raumpom cov qog ua los ntawm cov cheeb tsam loj ntawm cartilaginous sib txawv nrog rau cov qog nqaij hlav hauv cov nplooj ntawv diffuse thiab fascicles. Cov cim pleomorphism thiab kev ua haujlwm mitotic tau sau tseg, 18-19/10hpf. Muaj kev hloov pauv sai sai rau qhov sib txawv nodules ntawm cov pob txha mos hyaline. Kev cuam tshuam stroma pom qhov chaw focal sib xws ntawm myxoid hloov nrog cov kab mob inflammatory cell infiltrate. Focal osteoid tsim tau tshwm sim, nrog ntau lub hlwb loj heev thiab cov khib nyiab apoptotic. Cov cheeb tsam ntawm qaib-xaim calcification thiab hemorrhage kuj tau txheeb xyuas, nrog rau cov ntu loj necrotic (Daim duab 3).

Cov qog hlwb tau immunopositive rau CD99 nrog muaj zog S100 protein qhia nyob rau hauv cov cheeb tsam ntawm cartilaginous sib txawv, immunonegative rau pan-cytokeratin, CK7, CK20, p63, desmin, thiab myogenin, thiab nrog tuav ntawm INI1 qhia (Figures 4 thiab 5). Cov yam ntxwv morphological thiab immunohistochemical no qhia tias muaj thawj chondrosarcoma nyob rau hauv lub raum ectopia hla ob qho tib si moieties.

Tus neeg mob tau ploj mus rau kev soj ntsuam tom qab tso tawm hauv tsev kho mob tom qab lub sijhawm tsis zoo tom qab kev phais ntawm 2 lub lis piam.

Cistanche tuaj yeem khocov cim qhia ntawm lub raum tsis ua haujlwm

Kev sib tham

Mesenchymal chondrosarcomas, thawj zaug tshaj tawm hauv xyoo 1959, yog cov neoplasms tsawg thiab muaj li ntawm 1 feem pua ​​​​ntawm tag nrho cov chondrosarcomas pom nyob rau hauv cov pob txha, nrog rau ib feem peb ntawm cov mob uas tshwm sim hauv cov nqaij mos thiab lwm yam kabmob. Extraskeletal mesenchymal chondrosarcoma qhov chaw muaj xws li lub taub hau thiab caj dab, qis extremities, pob tw, thiab retroperitoneum (3,4). Thawj lub raum chondrosarcoma yog qhov tsawg heev, tsuas yog muaj qee tus neeg mob tau tshaj tawm txij li lub koom haum tau piav qhia thawj zaug hauv xyoo 1984 (5).

Txawm hais tias tsis tshua muaj, qhov tshwm sim ntawm lub raum chondrosarcoma raws li qhov mob thawj zaug tsis yog qhov xav tau,lub raum thiab pob txha mosmuaj tib yam mesodermal keeb kwm (4). Txawm li cas los xij, qhov tseeb histogenesis tsis paub meej, thiab vim qhov tsis tshua muaj ntawm lub koom haum, muaj tsawg heev kev tshawb fawb txog pathogenesis.

Qhov kev lees paub zoo tshaj plaws yog qhov kev loj hlob ntawm qog hlav tshwm sim hauv ob theem, nrog rau kev hloov pauv hloov pauv hauv cov ntaub so ntswg tawg thaum lub sijhawm embryogenesis. Cov kev xav tsis ntev los no qhia tias qhov chaw no tshwm sim vim yog qhov sib txawv ntawm cov kab mob mesenchymal hauv cov qia hlwb (1). Qee qhov kev tshawb fawb tau soj ntsuam lub luag haujlwm ntawm kev hloov pauv mesenchymal-epithelial hauv pathogenesis (6).

Cov qog qhia tau hais tias tus yam ntxwv loj hlob, muaj cov cellular undifferentiated qog cell Cheebtsam thiab zes ntawm cov pob txha zoo-differentiated. Kev hloov pauv ntawm qhov tsis sib xws rau cov pob txha mos zes feem ntau yog sai. Cov kev kuaj mob sib txawv ntawm lub teeb microscopy tuaj yeem suav nrog Ewing sarcoma, me me cell osteosarcoma, dedifferentiated chondrosarcoma, thiab hemangiopericytoma (3). Lub koom haum yuav tsis meej pem nrog Wilm cov qog raws li cov qog nqaij hlav tsis sib xws thiab cov pob txha mos tuaj yeem ua yuam kev li blastemal thiab mesenchymal Cheebtsam (7). Txawm li cas los xij, tus cwj pwm zoo thiab qhov tsis muaj kev tsim osteoid thiab kev tshawb fawb immunohistochemical pab hauv kev kuaj mob.

Kev kuaj xyuas dav dav thiab kev soj ntsuam histological yog qhov tsim nyog los txiav txim siab txog qhov muaj nyob ntawm RCC qhia qhov txawv mesenchymal sib txawv nrog kev sib xyaw morphology. Kev hloov pauv sarcomatoid suav nrog kev sib txawv ntawm osteosarcomatous thiab chondrosarcomatous tsis tshua muaj tshwm sim hauv RCCs thiab tau tshaj tawm dav dav (8). Hauv peb qhov xwm txheej, txawm tias tom qab kuaj ntau ntu los ntawm ntau qhov chaw, tsis muaj pov thawj ntawm RCC.

Hla fused raum ectopia yog ib qho tsis tshua muaj congenital anomaly nyob rau hauvlub raumyog fused thiab nyob rau tib sab ntawm lub midline. Nws yog subclassified rau ntau hom raws li configuration ntawm fusion abnormality. Nyob rau hauv cov ntaub ntawv no, lub xub ntiag ntawm no anomaly thiab kev koom tes ntawm ob qho tib si moieties ua rau lub complexity ntawm phais. Txawm li cas los xij, kev ntsuas ua ntej ntawm lub raum thiab vascular anatomy siv cov duab ua lub luag haujlwm tseem ceeb hauv kev tswj hwm tus neeg mob tag nrho.

Rau qhov zoo tshaj plaws ntawm peb txoj kev paub, qhov tshwm sim ntawm kev sib koom ua ke ntawm lub raum chondrosarcoma nyob rau hauv crossed fused raum ectopia uas muaj ob qho tib si moieties, nrog papillary urothelial carcinoma nyob rau hauv lub zais zis tsis tau qhia ua ntej. Ib daim ntawv tshaj tawm dhau los tau piav qhia txog cov chaw no tshwm sim raws li qhov sib txawv ntawm qhov sib txawvraum(9). Lub koom haum no tej zaum yuav yog coincidental los yog tuaj yeem yog cov lus teb rau ib qho laj thawj uas tshwm sim.

Cov kev tshawb fawb duab ua lub luag haujlwm tseem ceeb hauv kev ntsuam xyuas ntawm lub plab plab. Txawm li cas los xij, txog niaj hnub no, tsis muaj qhov tshwj xeeb hluav taws xob pab hauv kev kuaj mob raum chondrosarcoma. Qhov feem ntau pom hluav taws xob yog qhov tshwm sim ntawm calcification hauv cov qog (1). Tsis tas li ntawd, kev kuaj mob ntawm thawj lub raum chondrosarcoma yuav tsum tau cais tawm ntawm metastasis mus rau lub raum.raumlos ntawm skeletal chondrosarcoma los ntawm kev kos duab.

Feem ntau ntawm cov teeb meem ntawm lub raum chondrosarcoma qhia hauv cov ntaub ntawv muaj metastasis ntawm qhov kev nthuav qhia thawj zaug.

Cov chaw rau metastasis muaj xws li daim siab, ntsws, ureter, thyroid caj pas, thiab femora (2,10). Simultaneous tshwm sim ntawm mesenchymal chondrosarcoma nyob rau hauv lubraumthiab spleen kuj tau tshaj tawm (11).

Muaj qee qhov kev tshawb fawb txog cytogenetic thiab molecular abnormalities hauv lub raum chondrosarcomas. FLI-1 qhia pom hauv Ewing sarcoma txawv nws ntawm mesenchymal chondrosarcoma thiab me me cell osteosarcoma (12). HEY1-NCOA2 fusion yog suav tias yog kev kuaj mob rau mesenchymal chondrosarcomas (13). Hauv cov pob txha, chondrosarcomas pom p53 kev hloov pauv tsuas yog hauv cov neeg tsawg. Thaum tam sim no, cov no tshwm sim hauv qib siab dua (14).

Kev nthuav qhia p53 nce ntxiv yog txuam nrog tag nrho cov kev pom tsis zoo hauv RCCs (15). Txawm li cas los xij, muaj qhov tsis txaus ntawm cov kev tshawb fawb txog tib yam txog thawj lub raum chondrosarcomas. Tsis tas li ntawd, kev koom tes nrog cov tsos mob tshwm sim tsis tau tsim, tej zaum vim yog qhov tsis tshua muaj ntawm tus mob.

Thaum tsis muaj cov txheej txheem kev kho mob tseeb, kev phais phais mob phais nrog cov npoo txaus thiab cov tshuaj kho mob ntxiv nrog ntau tus neeg ua haujlwm tau siv rau kev tswj hwm (16).

Ib qho tshuaj khomob uas ua tau rau kev kho mob ntxiv yog kev sib xyaw ntawm doxorubicin, vincristine, thiab cyclophosphamide, nrog rau kev tswj hwm ntawm etoposide thiab ifosfamide. Cov hlwb malignant mesenchymal qhia tau hais tias muaj kev nthuav qhia ntau ntxiv ntawm platelet-derived growth factor receptor (PDGFR- ). Yog li cov neeg ua haujlwm uas inhibit PDGFR- ua haujlwm, xws li dasatinib, sorafenib, thiab imatinib, tuaj yeem ua lub luag haujlwm hauv kev kho mob (17).

Xaus

Cov kab mob ntawm thawj lub raum chondrosarcoma yog to taub tsis zoo vim nws qhov tsis tshua muaj thiab tsis muaj sia nyob. Elucidation ntawm cytogenetic thiab molecular abnormalities yuav pab tsim ib tug meej kev kho mob raws tu qauv. Txog tam sim no, qhov kev cia siab zoo dua tuaj yeem ua tiav los ntawm kev kuaj mob ntxov thiab pib sai ntawm kev tswj kom tsim nyog. Nws yog ib qho tsim nyog yuav tsum nco ntsoov txog qhov tshwm sim ntawm sarcomas ntawm qhov chaw tsis tshua muaj vim tias nyob rau hauv cov xwm txheej txawv txawv, lub sij hawm muaj rau kev hloov cov txiaj ntsig ntawm tus kab mob yog txwv.

Tiv thaivmob raum cov tsos mobnrogKhawv koob Cistanche, nyem qhov no kom paub ntxiv.

Cov ntaub ntawv

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