
ectomenchymoma

60 vayass vare praayamulla rogikalil kesukal report cheyyappettittunde pradhaanamaayum kuttikalil sambhavikkunna naadeevyavasthayudeyo mriduvaaya tissuevinteo apoorvavum athivegam valarunnathumaya tumouranu ectomenchymoma. thalayilum kazhuthilum adivayattilum periniyatthilum vrishanathilum kaikaalukalilum ectomenchymomakal roopappedaam. marakamaaya ectomenchymoma ennum ithine vilikkunnu.
neuroectodermal gadakangal [ganglian koshangal prathinidheekarikkunnu koodaathe/allengil ganglionoroma, ganglionoroblasto, neuroblastoma, peripheral premitteevu neuroectodermal tumours-pinettu], onno athiladhikamo messenkymal neoplastic gadakangal, saadhaaranayaayi rabdomosarcoma enniva polulla nannayi verthirikkapetta allengil moshamaayi verthirikkapetta neuroblastic koshangal chernnathaanu sophtt tissukaludeyo cnsinteyo apoorva tumour. ettavum amgeekarikkappetta sidhaantham soochippikkunnathu eee tumour migratory neural krestt cellukalude avashishtangalil ninnum angane ectomenchymil ninnum undaakunnuvennaan.
klinikkal chithram.
tumour pradhaanamaayum 15 vayassinu thaazheyulla kuttikale badhikkunnu, ekadesham 20 sathamaanam muthirnnavaril maathrame kaanappedunnulloo, ekadesham 60 sathamaanam purushanmaarum 40 sathamaanam sthreekalum (1). thalayum kazhuthum (bhramanapathavum nasopharynx), kendra nadiviewham, adivayarum retroperitoniyavum, pelvis, perinium, vrishanavum prostettum (1) ennivayaanu ettavum saadhaaranamaaya sthaanangal. klinikkal lakshanagal nirddishtamalla, saadhaaranayaayi praadeshika tumour compression nuzhanjukayattavum moolamaanu undaakunnath.
histopathi.
eee tumourinte pradhana savisheshathakal onnukil ectodermal derivateevukal (neuroblastukal, ganglian cellukal) allengil messenkymal gadakangal kooduthalum prathinidheekarikkunnath interlacing bandilukalile pokkamulla koshangal palappozhum rabdomioblastic vyathyasam kaanikkunnu, strap polullathum raakkat aakruthiyilullathumaaya koshangal (2-6) ulppede. myofibril polulla ghadanayum cross straishanukalum thirichariyaan kazhiyum. liposarcoma polullathum condroid phosiyukalum oru adhika kandethalayirikkum. fibrosarcoma polullathum fibrus histiosaittoma polullathumaaya pradeshangalum rosettu roopeekaranathodukoodiya neurofibromatus, neuroblastic gadakangalum nireekshikkaam. ganglian koshangal pakwathayillaathathum asaadhaaranavumaayathaayi kaanapedaam, avaa dvi-allengil multinucleated aakam, koodaathe nissel padaarthatthinte thelivukal kaanikkunnu (2-6).
rabdomioblastukalum moshamaayi verthiricha cheria koshangalum desmin myosinum posittivitti kaanikkumbol neural eriyakal ezz-100-nodu vyathyasthamayi sensitiivu aanu. ganglian koshangal enn. ezz. iii. kku shakthamayi positteev aanu. ectodermal ghadakam chilappol kuravaayirikkaamennum avaganikkamennum choondikkaanikkendathu pradhaanamaan, athesamayam chemotherapic sheshamulla saambilukalil ganglioneuroma ghadakam vardhikkukayum amithamaavukayum cheiyunnu. 
rabdomiosarcoma, traittan tumour, terratoma, wilms tumour, benin, matuver ectomenchymoma (ectomenchymal hamartoma) enniva deferential diagnosis pariganikkanam.
gaveshanam.
goldsby thudangiyavar. vrukkayude oru ectomenchymoma hyperdiploid ennavum 12,15 chromosomukalkkidayil oru translocation kaanikkunnathayi report cheythu (8). floris thudangiyavar. koshangalude oru upavibhaagathile hyperploydisathilum athupole thanne chromosomukalaaya 2,311,20 ennivayude nettangalilum avayude report cheyyappetta cases hyperploydisathil ithu kaanapedunnu, ithu alviolar rabdomiosarcomayumayi pothuvaayulla kandethalaan. poost chemotherapy maathrkayil aprathyakshamaaya praadhamika tumourile 2 vyathiriktha chromosome <aii. dii. 2>, <aii. dii. 1> amplification pradeshangalum avar kandethi. koodaathe, pretreatment biopsy immunohystochemistrystry vazhi hmga 1, hmga 2 proteinukalude sakthamaaya aavishkaaravum therapic sheshamulla aavishkaaram nashtappedunnathum athuvazhi oongoojenik vipuleekaranathinu (9) hmga kudumbatthile proteinukale credit cheiyunnu.
charitraparamaaya pashchaathalam.
neural kresttil ninnu uthbhavichathayi vishwasikkappedunna neoplaasangalude oru vaividhyamaarnna grouppaanu mem. itharathilulla tumourinulla aadya soochanakal orupakshe shuvangshotty aandu nestti (1971), holimon aandu rosenblum (1971) (2-3) ennivayil ninnayirikkaam. athinusesham nakayum mattullavarum adhika sambhavanakal nalki. (1975), carcioglu thudangiyavar. (1977), cosuto thudangiyavar. (1982), kawamoto thudangiyavar. (1987).
kosem thudangiyavar. 2004 leeii oru avalokanathil 44 mem kesukal shekharikkukayum shasthrakriyaykk munbo sheshamo chemotherapy upayogichu vichedikkal mikacha phalangal nalki ennu kandethukayum cheythu. mouttanum mattullavarum report cheytha anchu kesukalil 13 ll oru maranam maathramaan. aakramanathmakamaaya chemotherapy mathiyaya sasthrakriyayum 7 muthal 50 maasam vare rogarahitha edavela anuvadichu. radical sarjikkalinte nettam
ableshan ettavum pradhanappetta roganirnaya gadakamaayi thonnunnu (10).
paraamarshangal.
1. cosen emm, ibiloglu aii, bakkan vii, coseloglu bii (2004) ectomessenchymoma cases report saahithyathinte avalokanavum. turke jeey peediyaatt 46:82-87.
2. shuvangshotty ezz, nestti milligram (1971) mixed messenkymal, neuropithalial uthbhavathinte neoplasm. jeey neuropathol exp neurol 30:290-309.
3. holimon jl, rosenblum vii (1971) "gangliorhabdomiyosar ectomenchyminte oru tumour". jeey neuro sarj 34:417-422.
4. marakamaaya messenkaimomayumaayi bandhapetta naaka emm, matsumoto ezz, shiray tii, ittoo tii (1975) ganglioneuroblastamot. cancer 36:1050-1056.
5. carcioglu isad, semeren eiii, mathes esje (1977) ectomenchymoma. ganglionic, shwanian, melanositic, rabdomioblastic deferentiation enniva kaanikkunna migratory neural kresttinte (ectomenchime) avashishtangalude marakamaaya tumour. cancer 39:2486-2496.
6. cosuto sii, komalli eiii, bandelloni aarr (1982) ectomenchymoma. rund kesukalude report. wirchos oru paathol anaat histopathol 398:185-195 kamaanam cheiyunnu.
7. cavamoto eh, weidner enn, augostini arm juuniyar, jaff aarr (1987) sophtt tissuevinte marakamaaya ectomenchymoma. rund kesukalude report saahithyathinte avalokanavum. cancer 59:1791-1802.
8. goldsby reey, bruggers cs, brothman aarr, shornsen piechu, bekwit jebi, paishar tije (1998) vrukkayile spindle sell sarcoma, ganglionic gadakangal (marakamaaya ectomenchymoma) chromosomal asaadhaaranathakalumaayi bandhappettathum saahithyathinte avalokanavum. jeey peediyaatt hemattol oongool 20 (2): 160-164.
9. floris jii, debic-rictor emm, wosniac qaa, magrini ezz, maaphiyoletti jii, dii vever aii, tellini jii, skayot aarr (2007) marakamaaya ectomenchymoma janithaka profile rabdomosarcomatus deferentiatione prathifalippikkunnu. moll paathol 16 (4): 243-248.
10. mouttan ezz. sii., rosenberg echu. ezz., kohen emm. sii., drutt aarr, iii. emm. emm. ezz. emm., kaskula roow (1996) kuttikkaalatthe marakamaaya ectomenchymoma. peediyaatt paathol laab medu 16 (4): 607-624.

