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Mucinous Adenocarcinoma of the Renal Pelvis An Analysis of

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					                                                                                            Case Analyses et al
                                                                                                   C. C. Chen,




Mucinous Adenocarcinoma of the Renal Pelvis: An
Analysis of 5 Cases
Chao-Chih Chen1, Chien-Long Kuo2, Mao-Sheng Lin1, Hui-Lung Hsieh1,3, Tah-Chong Lin1,
Jungle C.H. Wu1

Departments of 1Urology and 2Pathology, Show-Chwan Memorial Hospital, Changhua, Taiwan
3
 Department of Urology, Chushan Show-Chwan Hospital, Nantou, Taiwan



OBJECTIVES: Mucinous adenocarcinomas are a rare lesion of the renal pelvis. We present our clinical experience
with this disease at Changhua Show-Chwan Memorial Hospital.
MATERIALS AND METHODS: We reviewed the medical records of patients presenting with renal pelvic tumors
from 1987 to 2007, and identified 5 cases of mucinous adenocarcinoma. Clinical data including age, gender, clinical
presentations, laboratory data, concomitant disease, treatment, and outcomes were retrospectively analyzed, and the
histopathological diagnoses were confirmed by a single pathologist.
RESULTS: The incidence of mucinous adenocarcinoma of epithelial tumors of the renal pelvis in our hospital was 1.4%
(5/349). The mean age of these 5 patients, all men, was 69.6 (range, 64~79) years. All patients had renal stones, and 4
patients had hydronephrosis with or without chronic pyelonephritis. Urinalysis confirmed mucin in the urine of 3 pa-
tients (60%). Two of the 5 patients (40%) died of a mucinous adenocarcinoma within 3 years of their diagnosis.
CONCLUSIONS: Early diagnosis of mucinous adenocarcinoma is difficult, and clinical presentations are usually
nonspecific. Mucin in the urine might be a sign of this disease, especially in patients who also have chronic irritation of
the renal pelvis. (JTUA 18:219-24, 2007)
Key words: mucinous adenocarcinoma, mucin, renal pelvis.



                    INTRODUCTION                                      The pathogenesis of this tumor is not clear. Several
                                                                 investigators have reported that mucinous adenocarci-
     The majority of malignant renal pelvic tumors are           noma of the renal pelvis is associated with a long dura-
epithelial in origin. Of these, approximately 90% are            tion of irritant conditions of the urothelium, such as
transitional cell carcinomas (TCCs). Adenocarcinomas             urolithiasis, inflammation, infection, and hydroneph-
are rare and account for < 1% of renal pelvic neoplasms.         rosis.1-15 Since epithelial metaplasia is commonly present
A mucinous adenocarcinoma is a subset of adenocarci-             in these conditions, the general consensus is that these
nomas in which cancer cells produce abundant extra-              tumors are derived from the metaplastic glandular mu-
cellular mucin. Reports of renal pelvic mucinous adeno-          cosa in response to chronic irritation of the uro-
carcinomas in the literature are limited to small series         thelium.1,2,7-9,13,14
and isolated case reports. To date, approximately 100                 In addition to reviewing the literature on this
cases have been reported in the English medical                  condition, we present our experience with 5 patients di-
literature.                                                      agnosed with mucinous adenocarcinoma of the renal
     Early diagnosis of this disease is difficult. Because       pelvis in the past 20 years at Show-Chwan Memorial
there are no specific clinical symptoms or laboratory tests      Hospital, Taiwan.
for this malignancy, in many cases the diagnosis of re-
nal pelvic mucinous adenocarcinoma is made by the                         MATERIALS AND METHODS
pathologist, and the tumors are incidentally detected from
nephrectomy specimens of other diseases.1-12                          Between 1987 and 2007, 5 patients with renal pel-
                                                                 vic mucinous adenocarcinoma were diagnosed at Show-
Address reprint requests and correspondence to:                  Chwan Memorial Hospital. The clinical history, includ-
Dr. Chao-Chih Chen
Department of Urology, Show-Chwan Memorial Hospital, 542 Chung   ing the age at diagnosis, gender, clinical presentations,
Shan Rd., Sec. 1, Changhua, Taiwan 500, R.O.C.                   laboratory data, concomitant diseases, treatment, and
Tel: 886-4-7256166 ext. 1293
E-mail: joe_chen69@yahoo.com                                     outcomes, were retrospectively reviewed from the medi-



JTUA 2007      18    No. 4                                                                                             ONV
Mucinous Adenocarcinoma of the Renal Pelvis



cal records. The histopathological diagnoses were re-             nying severe pyelonephritis.
viewed and confirmed by a single pathologist, and mu-                  Patient no. 4 was found to have a renal pelvic tumor
cinous adenocarcinoma was recognized by confirmation              during percutaneous nephrolithotomy (PCN-L) surgery
of tall columnar epithelial lining with basally located           for a left renal staghorn stone, and a subsequent nephre-
nuclei. Focal pseudostratification and papillary tufting          ctomy was performed a few days later.
of epithelial cells were also noted. Evidence of mitosis               The tumor of patient no. 3 was incidentally dis-
was rare. In addition to these findings, renal parenchyma         covered during PCN-L surgery for a right renal stone.
invasion was noted (Fig. 1), and mucin stains were posi-          After histological confirmation and to avoid further
tive (Fig. 2).                                                    dialysis, the patient received electrocautery through the
                                                                  PCN route because of renal insufficiency. Unfortunately,
                        RESULTS                                   he died of disease 3 years after the operation, at which
                                                                  time he was discovered to have retroperitoneal invasion.
     From 1987 to 2007, 349 cases of urothelial carci-                 Among the patients who received a nephrectomy, a
noma of the renal pelvis were diagnosed in our hospital.          subsequent ureterectomy was not performed. However,
Five of the cases (1.4%) were histologically confirmed            none of these patients had tumor recurrence in the re-
as being mucinous adenocarcinoma. The clinical fea-               maining ureter or urinary bladder.
tures and characteristics of the patients are shown in                 Patient no. 1 died of pulmonary metastasis 4 months
Table 1.                                                          after the diagnosis. Patient no. 2 died of natural causes 8
     All of the patients were male, and the age at diag-          years after the diagnosis without tumor recurrence. The
nosis ranged from 64 to 79 (mean, 69.6) years. The main           remainder of our patients who underwent a nephrectomy
clinical presentations of patients were gross or micro-           were still alive with regular follow-up in our hospital at
scopic hematuria (n = 5) and flank pain (n = 3). It is            the time of this writing.
notable that 3 patients (60%) experienced mucin in the
urine, which was detected by urinalysis. However, in all                               DISCUSSION
of the patients, mucoid material was noted in the dilated
collecting system when the renal pelvis was opened. All                Mucinous adenocarcinomas of the renal pelvis are
of the patients had renal stones, and 4 patients (80%)            a rare condition, and they are often reported as isolated
had hydronephrosis with or without pyelonephritis.                cases. To date, only about 100 cases have been reported
     None of the patients was diagnosed preoperatively            in the English medical literature. Lesions from muci-
by imaging studies. Three patients were diagnosed from            nous adenocarcinoma are usually incidentally detected
the resected surgical specimen. Notably, the operations           from surgically resected specimens. One of the predomi-
were conducted to address renal stones with accompa-              nant presenting features of this condition is copious,




Fig. 1.   High-power microscopic examination of the renal         Fig. 2.   Tumor cells positive for mucin special stain. (H&E,
          pelvis revealing the tall, simple to pseudostratified             400×)
          columnar epithelium including goblet cells. Renal pa-
          renchymal invasion can be observed. (H&E, 400×)



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                                                                                                              C. C. Chen, et al



thick, viscid mucous material in the renal pelvis and                   Because this type of tumor is uncommon, a preop-
calyces.1-6,8-11,13,15                                             erative diagnosis is rarely made, and most cases are di-
     In India, a much higher prevalence (16.7%) of ad-             agnosed from resected specimens by a pathologist. The
enocarcinomas of the renal pelvis has been reported.13             symptoms of these patients are often nonspecific, and
The reason for this increased incidence is not clear. In           hematuria (gross or microscopic) is the most common
addition, the majority of these renal pelvic adenocarci-           sign in the majority of cases. In addition, flank pain and/
nomas were not reported in sufficient detail to provide a          or a palpable abdominal mass may also be clinical symp-
basis for further histological subclassification. Terris and       toms in late-stage patients.
colleagues suggested that this may result from dietary                  In most patients, standard imaging studies, includ-
or other environmental factors prevalent in India.15               ing intravenous pyelography (IVP), ultrasonography,
     The first major review of this condition was pre-             renal scans, and even computed tomographic (CT) scans
sented by Aufderheide and Streitz in 1974, with 28 cases           or magnetic resonance imaging (MRI), might not be able
summarized. They found that the majority of the cases              to identify these tumors preoperatively. Similarly, no
occurred in patients older than middle age and were usu-           specific presentation differentiates mucous adenocarci-
ally associated with a long history of infection, stones,          noma from TCCs by clinical symptoms or imaging
or hydronephrosis.4 Similar findings were also demon-              modalities.
strated in subsequent reports in the medical literature.                Some special features of this infrequently diagnosed
In our series, although small, the mean age at diagnosis           tumor may help clinicians. First, a long history of pre-
and the preexisting factors are similar to those of previ-         senting symptoms is a common finding: an average 4-
ous reports.                                                       year duration of symptoms was found by Murphy and
     Although it was reported in the earlier literature that       Stevenson.6 This prolonged duration of symptoms was
no difference exists in the prevalence of mucinous ad-             also noted by Aufderheide and Streitz.4 Second, this
enocarcinoma between genders,4,5,14 a significantly male           condition is often found to be associated with chronic
predominance was found in our series, with all of the              irritation from nephrolithiasis, infection, or obstruction.
patients being male. Interestingly, the phenomenon of              Urolithiasis was present in the majority of cases reported
male predominance was also noted other literature from             earlier. However, in a review by Spires and colleagues
Asian countries (India, Japan, Hong Kong, Malaysia,                in 1993, who found that only 31% of cases presented
Singapore, and Taiwan) with a 3:1 male-to-female ratio             with stones, most of the cases (92%) showed evidence
(excluding our 5 cases).7-13,15,16 However, the relation-          of hydronephrosis.13 In our series, all patients had renal
ship between this ratio and different races is difficult to        stones, and 80% had hydronephrosis.
accurately interpret given the small number of cases.                   Since this tumor is located in the renal pelvis, the



Table 1. Characteristics, clinical presentations, management and outcome

                      Age (yr)/       Clinical
Case no.     Year      Gender       presentations     Associated conditions      Management                 Outcome

  1          1988       79/M       Microscopic          Renal stone,              Nephrectomy       Died of lung metastasis 4
                                   hematuria            hydronephrosis                              months after surgery
                                   Flank pain           Chronic pyelonephritis
                                   Mucin in urine
  2          1989       72/M       Gross hematuria      Renal stone,              Nephrectomy       Died of natural causes 8
                                                        hydronephrosis                              years after surgery
                                   Flank pain           Chronic pyelonephritis
  3          1990       64/M       Microscopic          Renal stone               PCN               Died of disease 3 years
                                   hematuria                                      electrocautery    after surgery with
                                                                                                    retroperitoneal invasion
                                   Mucin in urine       Hydronephrosis
  4          1996       67/M       Microscopic          Renal stone               Nephrectomy       Alive with regular
                                   hematuria                                                        follow-up
                                   Flank pain           Hydronephrosis
  5          2002       69/M       Microscopic          Renal stone               Nephrectomy       Alive with regular
                                   hematuria                                                        follow-up
                                   Mucin in urine



JTUA 2007     18    No. 4                                                                                                  OON
Mucinous Adenocarcinoma of the Renal Pelvis



tumor cells may exfoliate into the lower urinary tract,              Urothelial glandular metaplasia often develops in
and a urine cytologic examination may be used as a di-          response to chronic irritation and inflammation, leading
agnostic tool. However, only a small number of cases            many investigators to speculate that the metaplasia is a
have been diagnosed by urine cytology.1,12 The reason           result of a sequence of changes in the transitional epi-
for this may be that these cases are frequently associ-         thelium through successive stages of pyelitis grandulosa,
ated with an obstructive uropathy or even a non-func-           pyelitis cystica, and pyelitis grandularis.14 Some authors
tioning kidney, so the urine obtained from the urinary          believe that it arises from foci of intestinal metaplasia
bladder might not contain tumor cells. Thus, Yonekawa           from chronic irritation.2 Glandular metaplasia of the
and colleagues presented a case of mucinous adenocar-           urothelium, which develops as a response to chronic
cinoma arising in the renal pelvis diagnosed by cath-           irritation, may progress to dysplasia and an adenocar-
eterized urine cytology, and they indicated that a preop-       cinoma. To avoid confusion between mucinous adeno-
erative diagnosis is possible by cytologic findings of          carcinomas and metaplasia, Aufderheide and Streitz pro-
catheterized urine together with clinical data.16               posed the following criteria for malignancy: (1) histo-
     It is notable that mucous material in the urine was        logical evidence of architecture or cellular atypism; (2)
discovered in most of our patients (3/5). This finding          microscopic evidence of invasion of the renal pelvic wall
has not been reported in other published literature. In         and renal parenchyma, or of nodal or distant metastasis;
general, clear mucous in the urine might not be a               and (3) evidence of overt invasion or recurrence, or of
problem, and it is usually found in patients who have           nodal or distant metastasis.4 All of our cases fulfilled
urinary tract infections, chronic cystitis, pyelitis, pro-      these criteria.
statitis, and bladder reconstruction or contamination.               On the other hand, some authors considered that this
Although it is not specific for a mucinous adenocar-            tumor may directly develop from transitional cells.
cinoma, it might be an important sign to remind clini-          Takehara and colleagues presented a case of an adeno-
cians to keep this disease in mind for the differential         carcinoma of the renal pelvis which was not associated
diagnosis, especially in patients with any preexisting          with chronic inflammation, and glandular metaplasia was
pathologic conditions as described above.                       not found in any sections of the resected specimens.
     Since the normal mucosa of the renal pelvis does           Results of the immunohistochemical examinations in-
not contain glandular epithelium, the mechanism for a           dicated that the adenocarcinoma of the renal pelvis in
mucin-secreting tumor to develop in the urothelium is           that case may have had a similar biological nature to a
not well understood. As seen with a mucinous adeno-             conventional TCC, and the authors hypothesized that it
carcinoma that developed into chronic fistula-in-ano,           may have originated by stepwise development from a
most of the published cases were noted to be associated         preexisting TCC of the renal pelvis.12
with a prolonged duration of a chronic irritative status             Liwnicz postulated that neoplasia of the adenocar-
such as urolithiasis, severe pyelonephritis, obstructive        cinoma is merely an advancement via chromosomal
uropathy, and congenital malformations. The patho-              transformation from a diploid to polyploid archi-
physiology of a mucinous adenocarcinoma is in part              tecture.2 The recent application of molecular biological
believed to be secondary to chronic inflammatory                techniques may provide a possible pathogenesis of these
changes.                                                        tumors. The p53 gene is known to play an important
     Many authors have postulated that these tumors             role in regulating the cell cycle, and loss of p53 regula-
originate from metaplastic urothelium, and this hypoth-         tion can lead to uncontrolled tumor growth and
esis is supported by both clinical and pathological             aggression. In mucinous adenocarcinomas of the colon,
observations.1,2,7-9,13,14 It is known that the urinary tract   a lower p53 protein expression and a frequent DNA rep-
epithelium has the potential for metaplasia, usually con-       lication error have been reported.18 However, because
verting to squamous epithelium and occasionally to glan-        of the limited number of patients studied, these phenom-
dular epithelium. Although with longstanding responses          ena have not been reported in mucinous adenocarcino-
to noxious stimuli, metaplasia of the urothelium is usu-        mas of the renal pelvis.
ally squamous, but the occurrence of glandular meta-                 Because this is an uncommon tumor and only lim-
plasia is also recognized. 1 Experimental studies by            ited clinical experience exists, standard treatments have
Mostoti in 1954 emphasized the unique ability of the            not been subjected to randomized double-blind prospec-
transitional epithelium to respond with glandular meta-         tive analyses. However, most authors believe that a radi-
plastic changes to stimuli such as infection or irrita-         cal nephrectomy remains the preferred surgical treatment
tion.17 However, this is commonly observed in older pa-         and should be performed in any suspected cases because
tients with bladder exstrophy.                                  reports indicate that 1/2 of the recurrences occurred



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                                                                                                         C. C. Chen, et al



locally at the edge of the retained ureteral tissue. Down-   2 years after surgery.2,3,5-7 In addition, some authors de-
ward seeding and spillage of tumor cells from surgical       duced that mucinous adenocarcinomas have a greater
manipulation have also been reported. 2-6 Thus, a            propensity for metastasis and behave more aggressively.
nephroureterectomy with ipsilateral bladder cuff exci-       We also observed this poor prognosis in our patients: 2
sion has been recommended as the treatment of choice         (40%) died of the disease within 3 years of diagnosis.
in these patients by many clinicians, similar to what is         Some current studies have reported better outcomes
recommended for a TCC of the upper urinary tract.            in patients with mucinous adenocarcinomas of the renal
     In fact, nearly all of these cases were undetected      pelvis than those previously reported.9-11,13 These recent
preoperatively, and these patients were thought to have      analyses reveal that a poor prognosis is related to the
some benign infections or inflammatory conditions, such      stage of the disease being treated and to the extent of
as chronic pyelonephritis, abscess, or an infected stone     spread, but is not related to the mucinous histology itself.
disease, so subsequently an additional procedure of a        Therefore, the key to long-term survival seems to be a
ureterectomy was performed in many cases. It is impor-       high index of suspicion.
tant to recognize this tumor intraoperatively to assure
adequacy of resection. Although seldom seen, the possi-                         CONCLUSIONS
bility of this tumor should be kept in mind when treat-
ing any patient with recurrent infection and hydrone-             Mucinous adenocarcinomas of the renal pelvis are
phrosis. Frozen sectioning may be helpful if mucoid ma-      often associated with urolithiasis and long-standing in-
terial is encountered during a simple nephrectomy.           fection and/or obstruction, and possibly originate from
Hence, special care must be taken in handling cases in-      metaplastic urothelium. The presentation of this rare
volving chronic stone and/or infections, especially if       tumor is usually late, and the symptoms are nonspecific.
mucoid material is found in the collecting system. Care-     Mucinous material in the urine might be a sign to alert
ful manipulation of the kidney is essential to avoid rup-    clinicians, especially in patients who also have renal
ture and spillage of the tumor cells. In our patients, no    stones, chronic pyelonephritis, and hydronephrosis.
local recurrence was noted despite the lack of a subse-
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JTUA 2007     18   No. 4                                                                                              OOP
Mucinous Adenocarcinoma of the Renal Pelvis



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OOQ                                                                                                   JTUA 2007      18    No. 4

				
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