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378
klinikinės–praktinės apžvalgos
INVASIVE UPPER URINARY TRACT UROTHELIAL CARCINOMA WITH
ATYPICAL CLINIC: CASE REPORT
VIRŠUTINIŲ ŠLAPIMO TAKŲ INVAZYVUS UROTELIO NAVIKAS SU ATIPINE KLINIKA:
KLINIKINIS ATVEJIS
Augustinas Matulevičius2, Edmundas Štarolis1
1Vilniaus miesto klinikinės ligoninės Chirurgijos klinikos Urologijos skyrius
2 Vilniaus universiteto ligoninės Santariškių klinikų Urologijos centras
1 Department of Urology, Vilnius City Clinical Hospital
2 Centre of Urology, Vilnius University Hospital Santariskiu Clinics
ABSTRACT
Key words: invasive upper tract urothelial carcinoma, radical nephroureterectomy, chemotherapy treatment.
Based on literature sources Urothelial carcinomas (UCs) are the fourth most common tumors. ey can be located in the
lower (bladder and urethra) or upper (pyelocaliceal cavities and ureter) urinary tract. Herein, we report a rare case of upper
tract high malignancy urothelial carcinoma with atypical clinic which looks like an apostematous pyelonephritis. Upper tract
urothelial carcinomas that invade the muscle wall usually have poor prognosis. Retrospectively assessing our patient has the
most common symptoms of urinary tract infection and malignancy. It was non-visible hematuria, flank pain, chronic urinary
tract infection, and also systemic symptoms (including anorexia, weight loss, malaise, fatigue, fever, and night sweats). e
right diagnose we have determined by biopsy and CTU. Open RNU with bladder cuff excision is the standard for high-risk
UTUC, regardless of tumor location and bladder cuff removal is imperative. AC adjuvant chemotherapy is the most widely
used treatment in patients with cancer after undergoing surgery. Unfortunately the overall survival rate of urothelial metas-
tatic tumor for this day is poor.
SANTRAUKA
Reikšminiai žodžiai: invazyvus viršutinių šlapimo takų urotelio navikas, radikali nefroureterektomija, chemoterapinis gydymas.
Remiantis literatūros šaltiniais, urotelio karcinoma yra ketvirtas pagal dažnumą navikas. Jis gali lokalizuotis apatiniuose (šla-
pimo pūslės ir šlaplės) arba viršutiniuose (inkstų geldelių ir šlapimtakių) šlapimo takuose. Šiame straipsnyje pristatome retą
klinikinį atvejį apie viršutinių šlapimo takų aukšto piktybiškumo urotelio naviką su netipine klinika, kuris prieš nustatant
tikslią diagnozę imitavo pielonefritą. Pacientai, kuriems nustatytas invazyvus urotelio navikas, peraugantis raumeninį sluoksnį
ir turintis atokių metastazių, turi nedaug galimybių išgyventi. Vertinant retrospektyviai, mūsų pacientą vargino viršutinių
šlapimo takų infekcijos simptomai. Tai buvo hematurija, šono skausmas, primenantis lėtinę šlapimo takų infekciją, taip pat
pacientę vargino sisteminiai simptomai (įskaitant anoreksiją, svorio netekimą, bendrą negalavimą, nuovargį, karščiavimą ir
prakaitavimą naktį). Tikslią diagnozę nustatėme tik atlikę inkstų biopsiją ir pilvo kompiuterinę tomografiją. Atvira radikali
nefroureterektomija, kai yra invazyvi viršutinio šlapimo trakto karcinoma, – auksinis gydymo standartas. Palaikomoji adju-
vantinė chemoterapija turėtų būti skiriama pacientams, sergantiems vėžiu po atliktos radikalios operacijos. Deja, bendras
išgyvenamumas, nustačius metastazavusį urotelio naviką, yra mažas.
Augustinas Matulevičius
Centre of Urology, Vilnius University
Hospital Santariskiu Clinics
Santariškiu str. 2, Vilnius
matuleviciusa@gmail.com
doi:10.15591/mtp.2016.061
teorija ir praktika 2016 - T. 22 (Nr. 4), 378–381 p.
INTRODUCTION
Based on literature sources Urothelial carcinomas
(UCs) are the fourth most common tumors [1]. ey can
be located in the lower (bladder and urethra) or upper
(pyelocaliceal cavities and ureter) urinary tract. UTUCs are
uncommon and account for only 5–10 % of UCs [1, 2],
with an estimated annual incidence in Western countries
379
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klinikinės–praktinės apžvalgos
of ~2 cases per 100,000 inhabitants. Pyelocaliceal tumors
are about twice as common as ureteral tumors. Epithelial
tumors of the kidney account for approximately 3 % of all
solid neoplasms, with adenocarcinoma or renal cell carcino-
ma (RCC) representing almost 85 % [3]. Herein, we report
a rare case of upper tract high malignancy urothelial carci-
noma with atypical clinic which looks like an apostematous
pyelonephritis. Upper tract urothelial carcinoma and RCC
are two different types of malignancy that are distinguished
on the basis of tissue type and location. Differentiation of
these malignancies is challenging but necessary because the
management strategies and standard of treatment differs. In
terms of diagnosis, the most common symptom is visible or
non-visible hematuria (70–80 %) [4]. Flank pain occurs in
20–40 % of cases, and a lumbar mass is present in 10–20 %
[5, 6]. Systemic symptoms (including anorexia, weight loss,
malaise, fatigue, fever, night sweats, or cough) associated
with UTUC should prompt more rigorous metastatic eva-
luation [5, 6]. Computed tomography urography (CTU)
has the highest diagnostic accuracy for high-risk patients
[4]. e sensitivity of CTU for UTUC is 0.67–1.0 and the
specificity is 0.93–0.99 [4]. Magnetic resonance urograp-
hy (MRU) is indicated in patients who cannot undergo
CTU, usually when radiation or iodinated contrast media
are contraindicated [7]. Urinary cytology should be perfor-
med as part of a standard diagnostic work-up. A cystosco-
py should be done to rule out concomitant bladder tumor.
Upper tract urothelial carcinomas that invade the muscle
wall usually have poor prognosis. e 5-year specific sur-
vival is < 50 % for pT2/pT3 and < 10 % for pT4 [8–10].
Radical nephroureterectomy must comply with oncological
principles, which consist of preventing tumor seeding by
avoiding entry into the urinary tract during resection [11].
In this article, we describe an unusual progressing case of
renal pelvic UC with atypical clinic.
CASE REPORT
A 72 age women who has started suffering by fever
37,5–37,8 Co about three months ago. Since then she has
lost 18 kg because of weak appetite. During all that time
she has got a treatment with antibiotic at 5 times, but effect
was extremely short and failed. She had the nonspecific
symptom of fatigue since 3 month previously. Of all me-
dical documents are known that patient was examined by
pulmonologist, urologist, gynecologist, but acute surgical
disease has not been established. At this time patient was
hospitalized for fever of unknown origin, anemia of un-
known origin and right side pain. No palpable mass was
detected on the physical examination. e urine and blood
analysis demonstrated bacterial urinary tract infection with
hematuria. e ultrasound has shown that right kidney
was diffuse abnormal and increased. e typical structure
was gone. e surrounding tissue was infiltrated and lo-
oks like a apostematous pyelonephritis. e ultrasound has
shown the urostasis and expanded ureter with inner mass.
e right adrenal gland was abnormal also and the lymph
nodes were enlarged in the inferior vena cava area and right
iliac area. e aspirate biopsy monitored by ultrasound has
shown invasive urothelial carcinoma of high malignant po-
tential (Immunophenotype: PANCK/CK7/CD10/PAX8/
INI1/GATA3/p63(+); CK20/RCC(-); Uroplakin III/Car-
boanhidrasis IX(+/-); CD20/CD3/CD30(-)). e Compu-
ted tomography urography has shown the right renal mass
with areas of necrosis and suspicious perirenal fat infiltra-
tion and invasion to the right adrenal gland. Some lymph
nodes were enlarged in the aortocaval area. A diagnosis of
invasive urothelial carcinoma (T3N1Mx) was made and a
radical nephrectomy with lymphadenectomy was planned.
Under general anesthesia, the patient was placed in the
supine position with the right side elevation. A midline ab-
dominal incision and right Kocher incision was done to
expose the right kidney. e right kidney artery was liga-
ted. e right kidney vena was ligated also. After that and
reconstruction was accomplished by use of no absorbable
sutures. En Bloc resection of the right renal mass and regio-
nal lymphadenectomy were performed successfully.
DISCUSSION
is case of upper tract invasive urothelial carcinoma
with unusual symptoms are unique and rare and for this re-
ason these patient with upper tract urothelial invasive carci-
noma was required to be investigated by professional team
with different specialist. Upper tract urothelial carcinoma
is an uncommon genitourinary malignancy that accounts
for about 5 % of urothelial cancers and less than 10 % of
renal tumors. Invasive urothelial carcinoma prognosis cor-
relates with histological grade and stage [12, 13]. Advanced
disease stages, such as aggressive invasion into renal paren-
chyma or perirenal fat carry a poor prognosis [14]. Less
than 30 cases reported in the literature we could find the
urothelial carcinoma with inferior vena cava thrombosis or
similar overgrowth [15]. Urothelial carcinomas of the renal
pelvis also more often appear to be of a higher stage than
their urinary bladder counterparts [16]. In our case, the
diagnose was determined by aspirating biopsy result and
preoperative CTU. e Computed tomography urography
has shown the right renal mass with areas of necrosis and
suspicious perirenal fat infiltration and invasion to the right
adrenal gland. Some lymph nodes were enlarged in the aor-
tocaval area. From this finding, a standard radical nephrec-
tomy and regional lymphadenectomy were performed. e
prognosis of patients with an invasive urothelial carcinoma
is poor compared with that of patients with RCC [17]. Up-
per tract urothelial carcinomas that invade the muscle wall
380 teorija ir praktika 2016 – T. 22 (Nr. 4)
klinikinės–praktinės apžvalgos
usually have poor prognosis. e 5-year specific survival is <
50 % for pT2/pT3 and < 10 % for pT4 [10, 18, 19]. Risk
stratification of upper tract urothelial carcinoma divided to
low and high risk upper tract urothelial carcinoma and it’s
listed below [20].
gnose was determined in advanced stage. In few article you
can find the good response of neoadjuvant chemotherapy
to downstaging, although survival data need to mature and
longer follow-up is awaited [25]. After a comprehensive se-
arch of studies examining the role of chemotherapy for up-
per tract urothelial cancer, the pooled evidence shows that
cisplatin-based adjuvant chemotherapy was beneficial for
prolonging survival [26]. Chemotherapy followed by retro-
peritoneal LND for isolated retroperitoneal recurrence after
nephroureterectomy for upper UTUC was feasible and safe
treatment that may be potentially therapeutic in selected
patients. e follow-up of upper tract urothelial carcinoma
patients after initial treatment on Invasive tumor cases is
Cystoscopy/urinary cytology at 3 months and then yearly
and CT urography every 6 months over 2 years and then
yearly.
RNU remains as the gold standard treatment for high-
risk UTUC. However, relapse and metastasis are highly
common in UTUC patients after RNU, affecting long-
term survival. Perioperative treatments have been used to
reduce relapse and prolong survival. However, the optimal
perioperative therapy is still uncertain. AC adjuvant che-
motherapy is the most widely used treatment in patients
with cancer after undergoing surgery. Leow, J. J. et al. su-
ggested the potential benefit in overall survival (OS) and
disease-free survival (DFS) of cisplatin-based AC in UTUC
[27]. A retrospective studies found that neo adjuvant che-
motherapy may prolong the survival of patients compared
with the matched cohort who underwent initial surgery,
but additional trials are necessary to confirm the treatment’s
utility [28]. On the basis of Literature we found that both
AC and NAC improve OS and DFS of UTUC patients
after RNU [29].
CONCLUSIONS
Urothelial carcinoma should be included in the diffe-
rential diagnosis of all renal tumors and the most of aposte-
matous pyelonephritis. Retrospectively assessing our patient
has the most common symptoms of urinary tract infection
and malignancy. It was non-visible hematuria, flank pain,
chronic urinary tract infection, and also systemic symptoms
(including anorexia, weight loss, malaise, fatigue, fever, and
night sweats). e right diagnose we have determined by
biopsy and CTU. In this case the team of urologist, vascu-
lar surgeon, anesthesiologist and diagnostic radiologist took
care the patient heath, however prognosis are poor. Open
RNU with bladder cuff excision is the standard for high-
risk UTUC, regardless of tumor location and bladder cuff
removal is imperative. On the basis in Literature we found
that both AC and NAC improve OS and DFS of UTUC
patients after RNU.
Figure 1. Risk stratification of upper tract urothelial carci-
noma [20]
* All of these factors need to be present
** Any of these factors need to be present
MDCT = multidetector-row computed tomography;
URS = ureterorenoscopy.
Talking about disease management UTUC with invasi-
ve to muscle layer must be treated radical. Open RNU with
bladder cuff excision is the standard for high-risk UTUC,
regardless of tumor location and bladder cuff removal is
imperative [11]. Radical nephroureterectomy must comply
with oncological principles, which consist of preventing tu-
mor seeding by avoiding entry into the urinary tract during
resection [11]. ere are no benefits of RNU in metastatic
disease, although it can be considered as palliative [11, 21].
Regardless aggressive radical nephrectomy, most patients
with metastasis died within six months of initial diagnosis.
Systemic chemotherapy can be used as an adjuvant tre-
atment for these patients, but the efficacy is unclear. Upper
tract urothelial carcinomas are urothelial tumors; therefore,
platinum-based chemotherapy is expected to have similar
efficacy as in bladder cancer. However, there are current-
ly insufficient data for recommendations. ere are several
platinum-based regimens [22], but the risk of impaired pos-
toperative function means that neoadjuvant chemotherapy
is only optional. Not all patients can receive chemotherapy
because of comorbidity and impaired renal function after
radical surgery. Chemotherapy-related toxicity, particular-
ly nephrotoxicity from platinum derivatives, may signifi-
cantly reduce survival in patients with postoperative renal
dysfunction [23, 24]. In our case we have bad prognosis.
e control all body CT showed many metastases in liver,
lung and bones. All symptoms of this patient before surgery
was unique and similar to urinary tract infection. e dia-
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teorija ir praktika 2016 – T. 22 (Nr. 4)
klinikinės–praktinės apžvalgos
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Gautas 2016 m. rugsėjo 26 d., aprobuotas 2016 m. spalio 17 d.
Submitted September 26, 2016, accepted October 17, 2016.
