Pleomorphic Spindle Cell Sarcoma Ntawm Adrenal Gland: Ib qho tsis tshua muaj tshwm sim ntawm lub plab loj loj thiab kev kuaj mob nyuaj
Dec 22, 2023
Abstract
Primary adrenal pleomorphic spindle cell sarcoma (PSCS) yog ib qho mob mesenchymal uas tsis tshua muaj heev uas yav tas los hu ua malignant fibrous histiocytoma. Nws feem ntau tshwm sim nyob rau hauv extremities, retroperitoneum, peritoneal kab noj hniav, thiab tsis tshua muaj nyob rau hauv visceral kab mob. Peb tshaj tawm thawj kis ntawm PSCS nyob rau hauv cov qog adrenal sab laug nyob rau hauv ib tug 65- poj niam muaj hnub nyoog uas muaj lub plab loj loj nrog cov tsos mob tsis meej. Ntau qhov kev kuaj mob sib txawv ua rau muaj kev sib tw hauv kev tsim qhov kev kuaj mob. Txawm li cas los xij, qhov no tau kov yeej los ntawm qhov tsim nyog hluav taws xob, kev ua haujlwm hauv kev ua haujlwm, histological thiab qhov tseem ceeb tshaj plaws, kev tshawb pom ntawm immunohistochemical. Tus neeg mob tau txais kev phais tag nrho ntawm cov qog thiab muaj qhov rov zoo tsis zoo. Nws tseem nyob tsis muaj metastasis lossis rov tshwm sim los txog rau hnub dhau los ntawm nws 18 lub hlis tom qab kev ua haujlwm tom qab txawm tias muaj qhov tsis zoo ntawm cov qog no.
Taw qhia
Pleomorphic spindle cell sarcoma (PSCS), uas yav tas los hu ua malignant fibrous histiocytoma (MFH), tshwm sim los ntawm fibroblasts thiab histiocytes [1]. PSCSs tau muab faib ua histologically rau tsib pawg, qhov twg hom storiform-pleomorphic yog ntau tshaj. Muaj ob peb tsab ntawv ceeb toom txog cov pob txha metaplastic thiab pob txha mos hauv PSCS cov qog [2]. Lub Koom Haum Saib Xyuas Kev Noj Qab Haus Huv Ntiaj Teb tau faib PSCS raws li qhov tsis muaj qhov sib txawv lossis tsis cais cov sarcomas vim lawv cov caj ces tsis paub. Lawv raug tshaj tawm nyob rau hauv extremities (67-75%), retroperitoneum (6-16%), peritoneal kab noj hniav (5-10%), tsis tshua muaj nyob rau hauv lub plab viscera (siab, gall zais zis thiab spleen), thiab pom tsis tshua muaj heev nyob rau hauv retroperitoneal kab mob. (lub raum) [3, 4]. Rau peb txoj kev paub, tsis muaj qhov xwm txheej ntawm thawj tus neeg mob qog nqaij hlav qog adrenal.
PSCS sawv cev rau pawg neeg tsawg tsawg ntawm cov qog adrenal cortical hu ua mesenchymal thiab stromal qog. Primary adrenal PSCS yog ib qho kev kuaj mob ntawm kev cais tawm vim muaj ntau yam kev kuaj mob sib txawv rau cov qog nqaij hlav hauv thaj tsam ze ntawm lub qog adrenal thiab cov kab mob ntawm cov qog nqaij hlav thiab cov qog adrenal. Yog li, kev kho mob, hluav taws xob, histological thiab, qhov tseem ceeb tshaj, cov cim immunohistochemical ua lub luag haujlwm tseem ceeb hauv kev kuaj mob. Peb tshaj tawm ntawm no thawj rooj plaub ntawm tus thawj adrenal PSCS nrog focal osseous metaplasia, uas nthuav tawm raws li lub plab loj loj.
CASE REPORT Ib tus poj niam muaj hnub nyoog 65- xyoo uas muaj qhov tsis meej pem sab laug sab plab plab, mob nraub qaum, plab plab, lethargy thiab ib pob uas tsis tuaj yeem xaiv ntawm sab laug lumbar thiab iliac cheeb tsam rau 3 lub hlis dhau los. Nws tsis kam lees tej yam genitourinary, gastrointestinal lossis gynecological tsos mob. Nws cov kev tshawb fawb tau qhia txog microcytic-hypochromic anemia, erythrocyte sedimentation tus nqi ntawm 92 mm nyob rau hauv thawj teev thiab C-reactive protein ntawm 24 mg / l. Tsis muaj monoclonal bands nyob rau hauv cov ntshav cov protein electrophoresis. Nws lub raum thiab daim siab cov ntaub ntawv, 9 AM cortisol, testosterone, 24- teev cov zis metanephrine thiab vanillyl mandelic acid tag nrho nyob rau hauv ib txwm txwv. Tsis muaj kev tshawb pom tseem ceeb los ntawm pan endoscopy.
Kev kuaj mob plab plab (US) tau qhia txog 12 × 12 cm khoom loj los ntawm sab laug suprarenal cheeb tsam (Daim duab 1). Contrasted computed tomography (CT) qhia txog 17.5 × 11.5 cm txhim kho qhov mob hauv thaj tsam sab laug suprarenal, hloov tag nrho cov qog adrenal sab laug. Lub raum sab laug tau hloov pauv tsis zoo, thiab ntxhib calcification tau pom ntawm qhov chaw ntawm qhov chaw uas tsis muaj qog nqaij hlav loj. Lub contralateral adrenal caj pas, retroperitoneal thiab peritoneal qauv yog ib txwm (Figs 2 thiab 3). Kev tshuaj xyuas thawj cov kab mob qog noj ntshav uas nthuav tawm raws li cov nyiaj tso rau hauv adrenal siv kev kuaj mob, pan endoscopy thiab sib piv CT yog qhov tsis zoo. Lub sijhawm no, peb qhov kev kuaj mob ua haujlwm tsis yog qhov zais cia sab laug pheochromocytoma, thiab xaiv laparoscopic sab laug adrenalectomy tau npaj.
Hmoov tsis zoo, nws nthuav tawm nrog qhov loj ntawm lub pob thiab ua rau cov tsos mob tshwm sim dhau los ua ntej qhov kev npaj phais. Kev kuaj mob sai hauv Asmeskas tau pom tias qhov loj ntawm cov qog mus rau 20 × 18 cm thiab yog li qhib sab laug adrenalectomy tau ua. Thaum lub sij hawm ntawm kev phais, peb pom ib tug loj zoo-hais tias 25 × 17cm capsulated mass nyob rau hauv sab laug suprarenal cheeb tsam (Fig. 4) uas yog inferiorly displacing sab laug raum thiab txoj hnyuv. Peb tsis tuaj yeem txheeb xyuas cov qog adrenal sab laug. Ua tiav resection ntawm cov qog tau ua tiav, thiab nws raug tso tawm nyob rau hnub thib peb tom qab ua haujlwm tom qab qis qis rov qab tsis zoo.
Kev ntsuam xyuas histopathological tau qhia txog 24 × 21 × 15 cm qog uas hnyav 2.645 kg (Fig. 4b) nrog fibrotic, khoom, thiab cystic degenerative hloov pauv yam tsis muaj capsular infiltration lossis vascular invasion. Muaj cov ntawv diffuse ntawm storiform-spindle hlwb nrog cov cellularity sib txawv, txawv txawv multilobulated thiab liab qab nuclei, eosinophilic inclusions thiab siab mitotic kev ua si (Fig. 5). Foci ntawm pob txha metaplasia tau sau tseg hauv thaj tsam nyuaj ntawm cov tshuaj ntsiav. Tsis muaj cov ntaub so ntswg adrenal tau pom nyob rau hauv tag nrho cov qauv. Cov qog stained immunohistochemically zoo rau CD68, CD99, CD34 (40%), desmin (20%) thiab cov leeg nqaij du (SMA) (10%) tab sis tsis zoo rau Pan-CK, S100, Melan A, epithelial membrane antigen (EMA) ), human melanoma black 45 (HMB45), CD117, B cell lymphoma 2, chromogranin thiab synaptophysin (Fig. 6). Tsis muaj metastases raug txheeb xyuas thaum kuaj. Peb xa tus neeg mob mus rau oncology rau kev ntsuam xyuas, tab sis lawv tsis pom zoo kom siv tshuaj kho mob, thiab tsis muaj kev rov tshwm sim dua los ntawm 18- hli tom qab kev ua haujlwm.
Kev sib tham
Cov qog adrenal feem ntau yog asymptomatic lossis tshwm sim nrog lub plab plab nrog cov tsos mob tsis tshwj xeeb, zoo ib yam li peb tus neeg mob [5]. Ntawm cov spectrum ntawm cov qog adrenal, thawj cov qog mesenchymal yog qhov tsawg heev. Thawj leiomyosarcomas yog hom malignant tshaj plaws [6]. Thaum lub sij hawm ntawm kev nthuav qhia, peb cov kev kuaj mob sib txawv muaj xws li thawj retroperitoneal sarcoma, qog nqaij hlav metastatic, liposarcoma, leiomyosarcoma, spindle cell carcinoma, malignant peripheral paj sheath hlav, plab hnyuv stromal hlav, malignant melanoma, synovial pheochytoma 6.
Txawm hais tias PSCS tuaj yeem tshwm sim los ntawm ib feem ntawm lub cev, lawv feem ntau yog los ntawm cov hlwb mesenchymal primitive [3]. PSCS tsuas yog tau tshaj tawm nyob rau hauv sab, plab thiab retroperitoneal viscera nyob rau hauv cov ntaub ntawv tab sis tsis nyob rau hauv cov ntaub so ntswg adrenal. Qhov tsis tshua muaj ntawm PSCS thiab qhov tsis muaj qhov pheej hmoo ntawm PSCS, xws li hluav taws xob, kev raug mob, kev kho mob ntev, kev phais mob thiab kub hnyiab, ntxiv rau tsis muaj cov ntaub ntawv qhia ua ntej, qhia tias thawj qhov kev kuaj mob qog nqaij hlav adrenal yog qhov tsis tshua muaj qhov sib txawv ntawm qhov no. cas [7]. Bhagavan et al. tshaj tawm tsib kis ntawm
Daim duab 2. CT duab: (a) ntu coronal thiab (b) sagittal seem; lub hnub qub liab nyob rau hauv (a, b) qhia cov qog thiab lub taub hau liab nyob rau hauv (a, b) qhia qhov tsis zoo ntawm lub raum sab laug
Daim duab 4. (a) Lub plab ntawm tus neeg mob tom qab induction ntawm chav ua hauj lwm thiab (b) macroscopic tsos ntawm cov qog nrog lub tuab capsule; lub hnub qub dub nyob rau hauv (a) qhia cov qog nyob rau sab laug plab thiab hauv (b) qhia txog thaj tsam ntawm thickening ntawm cov tshuaj ntsiav sib raug rau osseous metaplasia.
MFH nyob rau hauv extremities thiab pelvis nrog metaplastic pob txha thiab pob txha tsim, zoo ib yam li kev tshawb nrhiav osseous metaplasia nyob rau hauv cov ntaub ntawv no [2]. Qhov tshwm sim tsis tshua muaj tshwm sim ntawm cov pob txha metaplastic thiab pob txha mos hauv PSCS nthuav tawm qhov teeb meem tshwj xeeb hauv kev sib txawv ntawm cov nqaij mos osteogenic thiab chondrosarcomas, malignant mesenchymal thiab loj cell hlav [2].
Cov ncauj lus kom ntxaws immunohistochemistry yog xav tau los tsim kev kuaj mob ntawm PSCS ntawm lub qog adrenal. PSCS muaj qhov sib txawv zoo rau CD68, CD34 thiab CD99. Gastrointestinalnal stromal qog, malignant peripheral paj sheath qog, malignant melanoma, synovial sarcoma, spindle cell carcinoma, thiab pheochromocytoma yog qhov zoo rau CD117, S100, HMB45, cytokeratin, EMA, synaptophysin thiab chromogranin, Leiomyosarcoma yog qhov zoo rau desmin, vimentin thiab SMA [8].
Kev phais phais nrog cov npoo dawb yog qhov tseem ceeb ntawm kev kho mob rau PSCS, thaum siv xov tooj cua, kws khomob, thiab tshuaj tiv thaiv kab mob kuj tau piav qhia. Cov qog loj, qhov chaw, qhov chaw thiab hom kab mob histological yog qhov kev txiav txim loj ntawm kev kwv yees hauv PSCS [9]. PSCS yog ib qho mob qog nqaij hlav uas muaj qhov rov muaj dua thiab metastasis txawm tias ua tiav qhov kev txiav. Kev soj ntsuam mus sij hawm ntev nrog kev yees duab yog xav tau los nrhiav kev rov tshwm sim thiab metastasis.
Hauv kev xaus, peb tshaj tawm thawj rooj plaub ntawm thawj tus kabmob PSCS, uas tau pib tshawb xyuas raws li pheochromocytoma. Peb hais txog qhov tseem ceeb ntawm kev txiav txim siab PSCS hauv kev kuaj mob sib txawv rau cov qog adrenal loj nrog rau lwm hom sarcomas. Tsim kom muaj kev kuaj mob thiab ntsuas qhov kev ntsuas ntawm PSCS yuav tsum ib txwm suav nrog hluav taws xob, histological thiab tag nrho cov roj teeb ntawm kev tshawb fawb immunohistochemical. PSCS muaj kev txhawj xeeb txawm tias muaj kev phais tas, uas yuav xav tau kev saib xyuas nyob ze.
TXOJ CAI
Cov kws sau ntawv xav ua tsaug rau Dr Benjamin Flink, MD, MPH, Bariatric thiab General Surgeon ntawm Northside Tsev Kho Mob hauv Atlanta, GA, USA, thiab Dr OMO. Siddiqa, Tus Kws Sau Npe hauv Tshuaj Pathology ntawm National Tsev Kho Mob ntawm Sri Lanka, Colombo, rau lawv cov kev pabcuam hauv kev kho lus, kev sau ntawv, formatting thiab kho duab ntawm phau ntawv no.
TXOJ CAI NTAWM INTEREST STATEMENT Tsis muaj leej twg tshaj tawm.
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