Co esponding au ho : Aboulaich Youness
Copy igh © 2025 Au ho (s) e ain he copy igh o his a icle. This a icle is published unde he e ms o he C ea i e Commons A ibu ion License 4.0.
A challenging diagnos ic and he apeu ic case epo : Ewing sa coma / p imi i e
neu oec ode mal umo o he bladde
Aboulaich Youness 1, , Kha bach Yousse 2 and Khallouk Abdelhak 2
1 MD, U ology esiden , U ology depa men , Tangie Uni e si y Hospi al, Facul y o Medicine and Pha macy, Abdelmalek
Essaâdi Uni e si y, 90000 Tangie , Mo occo.
2 MD, U ology p o esso , U ology depa men , Tangie Uni e si y Hospi al, Facul y o Medicine and Pha macy, Abdelmalek
Essaâdi Uni e si y, 90000 Tangie , Mo occo.
Wo ld Jou nal o Ad anced Resea ch and Re iews, 2025, 26(01), 4193-4198
Publica ion his o y: Recei ed on 21 Ma ch 2025; e ised on 27 Ap il 2025; accep ed on 30 Ap il 2025
A icle DOI: h ps://doi.o g/10.30574/wja .2025.26.1.1542
Abs ac
Backg ound: Bladde ES/PNET is a a e en i y wi h a ew cases desc ibed in he li e a u e. This a icle will ocus on an
a ypical esical p esen a ion o his umo .
Summa y: We epo a case o Ewing's sa coma a ising in he bladde in a 37-yea -old pa ien wi h ype 2 diabe es who
p esen ed wi h clo ing hema u ia and i i a i e symp oms o he lowe u ina y ac . On clinical examina ion, a ha d
and mobile hypogas ic mass was palpa ed. CT scan e ealed an abdominopel ic mass wi h p edominan ly ex a esical
de elopmen . Cys oscopy showed appa en displacemen o he esical dome wi hou clea lesions wi hin i . Gi en his
pe plexing p esen a ion, we pe o med mass excision wi h pa ial cys ec omy. Ana omopa hological,
immunohis ochemical, and molecula biology s udies suppo ed he diagnosis o Ewing's sa coma o esical o igin.
Un o una ely, he pa ien passed away one mon h la e due o complica ions ela ed o a diges i e obs uc ion.
Conclusion; The objec i e o ou p esen a ion was o desc ibe an addi ional case ha p esen ed bo h diagnos ic and
he apeu ic challenges, in o de o s imula e u he conside a ion and de i e clea ecommenda ions.
Keywo ds: Case epo ; Bladde ; ES/PNET; Exophy ic
1. In oduc ion
ES/PNET is a a e malignan p ima y umo cha ac e ized by small ound cells [1]. In he li e a u e, only 22 cases o
bladde ES/PNET ha e been documen ed [3-24]. I s p eope a i e diagnosis is s ill complica ed due o he absence o
speci ic imaging ea u es. De ini i e diagnosis elies on pos ope a i e pa hology, immunohis ochemis y, and gene ic
analysis. Due o he minuscule numbe o epo ed cases, es ablishing clea guidelines o i s managemen and
ea men is challenging. The e o e, ea ly diagnosis and ea men o ES/PNET pa ien s a e highly challenging. This
a icle desc ibes an unusual case o PNET in he bladde in an adul .
2. Case P esen a ion
We p esen he case o a 37-yea -old pa ien wi h a his o y o ype II diabe es ea ed wi h o al an idiabe ic agen s. The
pa ien p esen ed wi h in e mi en clo ing hema u ia o h ee mon hs, accompanied by i i a i e symp oms o he
lowe u ina y ac , including pollakiu ia and u gency. On clinical examina ion, a ha d abdominal mass was palpable in
he hypogas ic egion. Abdominal CT scan e ealed a nec o ic abdominopel ic mass in ading he esical dome wi h
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i egula con ou s, showing he e ogeneous enhancemen a e con as adminis a ion. The mass measu ed 15 cm x 10
cm in ans e se diame e and 14 cm in heigh , exe ing a mass e ec on he igh iliac u e e and causing sligh
in il a ion o he pe i esical a wi hou isible iliac lymphadenopa hy. Cys oscopy showed displacemen o he esical
dome by he desc ibed mass, wi h a heal hy mucosa.
The pa ien unde wen mass excision wi h pa ial cys ec omy. Mic oscopic examina ion e ealed a poo ly di e en ia ed
malignan umo p oli e a ion composed o small ound blue cells. F equen mi o ic igu es and he p esence o Home -
W igh ose es in some egions we e obse ed (Fig. 2a). Immunohis ochemical analysis suppo ed he diagnosis o
Ewing's sa coma, wi h ocal posi i i y o an i-NSE, an i-synap ophysin, and an i-CD99 an ibodies (Fig. 2b). Howe e ,
an i-CD20, an i-CD3, an i-PS100, an i-desmin, an i-myogenin, an i-pancy oke a in, an i-ERG, and R MaB an ibodies
showed nega i e s aining. The pa ien was e e ed o he oncology cen e o addi ional chemo he apy. Un o una ely,
one mon h la e , he pa ien passed away due o complica ions ela ed o a diges i e obs uc ion.
Figu e 1 Axial sec ion o an abdominopel ic CT scan showing an exophy ic de elopmen o PNET
Figu e 2 (a) Shee s o monomo phic small ound cells wi h hype ch oma ic nuclei and clea cy oplasm. (H&E, 400).
(b) His opa hology sec ion exhibi ing s ong memb anous CD99 immunos aining (H&E, 350)
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3. Discussion
A p imi i e neu oec ode mal umo (PNET) is a a e and malignan umo belonging o he Ewing's amily o umo s
[1]. I was i s desc ibed in 1918 [2]. These umo s pose bo h diagnos ic and he apeu ic challenges o clinicians and
pa hologis s.
The p ima y issue encoun e ed wi h a bladde ES/PNET is he diagnos ic p oblem due o i s occu ence a an unusual
age, he a i y o i s localiza ion in he bladde , and no ably i s a ypical p esen a ion as a p edominan ly exophy ic
umo .
Only 22 cases o bladde ES/PNET ha e been desc ibed in he li e a u e ( able 1). The younges pa ien was 10 yea s
old [11], he oldes was 81 yea s old [8], esul ing in a mean age o 41.9 yea s, which is younge han ha epo ed in
u o helial ca cinomas [25].
Table 1 Published cases o p imi i e neu oec ode mal umo (PNET) o he bladde o da e.
Au ho
Yea o
publica ion
Sex/age
Tumo size
(mm)
G ow h pa e n
T ea men
Bane jee e al. [3]
1997
M/21
80×60×40
Endophy ic
Cys ec omy+chemo he apy
Gousse e al. [4]
1997
F/15
30×20×20
Endophy ic
TURBT+ Pa ial cys ec omy
Men zel e al. [5]
1998
M /21
140×100×100
Endophy ic and exophy ic
TURBT + neph os omy
Desai. [6]
1998
F/38
120×70×35
Endophy ic and exophy ic
Cys ec omy
Colecchia e al. [7]
2002
F/61
-
Endophy ic and exophy ic
-
K üge e al. [8]
2003
M/81
-
Endophy ic and exophy ic
TURBT + neph os omy
Lopez-Bel an e
al. [9]
2006
F/21
90×80×60
Endophy ic
cys ec omy+chemo he apy
Ellinge e al. [10]
2006
M/72
-
Endophy ic and exophy ic
TURBT+ ileal condui
Osone e al. [11]
2007
M/10
10
Endophy ic
TURBT
Al Meshaan e al.
[12]
2009
F/67
30×25×10
Endophy ic
TURBT+ Pa ial cys ec omy
Busa o e al. [13]
2011
F/52
33×15×22
Endophy ic
TURBT+ chemo he apy
Okada e al. [14]
2011
M/65
50
Endophy ic
TURBT+ cys ec omy+
chemo he apy
Zheng e al. [15]
2011
M /74
-
Endophy ic and exophy ic
TURBT+ pallia i e su ge y+
chemo he apy
Rao e al. [16]
2011
F/14
150×120×75
Endophy ic and exophy ic
Pa ial cys ec omy
Sueyoshi e al. [17]
2014
M/10
135×131×129
Endophy ic and exophy ic
Double J ube + pa ial
cys ec omy+ chemo he apy
Lam e al. [18]
2016
F/30
64×94×77
Endophy ic
TURBT+ chemo he apy+
cys ec omy
Vallon haiel e al.
[19]
2016
F/27
103×98×47
Endophy ic
TURBT+ chemo he apy
Tonyalı e al. [20]
2016
F/38
40×26×25
Endophy ic
cys ec omy + chemo he apy
Zhang e al. [21]
2020
F/78
63×44
Endophy ic
TURBT
Gao e al. [22]
2020
F/45
30
Endophy ic
TURBT + cys ec omy +
chemo he apy
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Liu e al. [23]
2020
M/64
60×50
Exophy ic
Pa ial cys ec omy+
adio he apy
Jingyi e al. [24]
2023
F/19
55×36
Endophy ic
TURBT+ chemo he apy
P esen case
2025
M/37
150 × 100 ×
140
Exophy ic
Pa ial cys ec omy
These umo s ha e a p e e ence o he cen al ne ous sys em in child en and young adul s, al hough hey can
occasionally de elop in o he loca ions such as he ho acic wall, head and neck, kidneys, ad enal glands, emale geni al
ac , ex emi ies, and sc o al sac [23]. The p esence o a PNET in he bladde is ex emely a e.
The majo i y o he desc ibed cases (21/22) exhibi endophy ic g ow h, while 8 o hem a e accompanied by exophy ic
g ow h. Howe e , a umo o pu ely exophy ic de elopmen has only been epo ed in one case [23], and ou case is he
second. Cys oscopy con i med his p esen a ion by e ealing a comple ely heal hy esical mucosa, which aises
suspicions o an al e na i e diagnosis, such as a umo o igina ing om he diges i e ac .
Gi en he a i y o hese umo s, i is di icul o conside hem ini ially. A de ini i e diagnosis elies on
ana omopa hological s udy, immunohis ochemis y, and molecula biology [1].
Apa om he diagnos ic challenge, he second issue ha a ises is he ea men . Due o he limi ed numbe o pa ien s
wi h PNET, no ecommenda ions can be es ablished, and mos knowledge abou ea men has been de i ed om
s udies on he Ewing's sa coma amily and he epo ed cases in he li e a u e.
Se en pa ien s unde wen o al cys ec omy [3,6,9,14,18,20,22], i e unde wen pa ial cys ec omy [4,12,16,17,23], and
eigh unde wen only ansu e h al esec ion o he bladde [5,8,10,11,13,19,21,24]. Chemo he apy was associa ed in
mos cases, wi h a a o able esponse obse ed in some ins ances [19,24].
In ou case, we pe o med a pa ial cys ec omy wi h excision o he mass, and pos ope a i e chemo he apy was planned.
Howe e , he pa ien 's gene al condi ion apidly de e io a ed, p e en ing hem om ecei ing he chemo he apy.
Success ul ea men necessi a es a mul imodal app oach [3,9,20,22]. Mos pa ien s wi h appa en ly localized disease
ha e occul me as ases, he eby wa an ing addi ional he apy, such as chemo he apy [10]. Poo p ognosis has been
epo ed o hose wi h exis ing me as ases [12,13].
Abb e ia ions
• ES/PNET: Ewing Sa coma / P imi i e Neu oec ode mal Tumo o he Bladde
• CT: compu ed omog aphy.
4. Conclusion
In conclusion, clinicians and pa hologis s should be awa e o his a e en i y du ing diagnosis and managemen . Su ge y
suppo ed by chemo he apy should be conside ed as an op ion, especially in ad anced disease cases. Wi h he
desc ip ion o new cases and long- e m ollow-up esul s, ou knowledge and expe ience will con inue o imp o e.
Compliance wi h e hical s anda ds
Disclosu e o con lic o in e es
The au ho s epo no compe ing pe sonal o inancial in e es ela ed o his wo k.
S a emen o in o med consen
W i en in o med consen was ob ained om he pa ien o publica ion o his case epo and any accompanying
images.
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