Sarcomatoid Transitional Cell Carcinoma of the Renal Pelvis

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					Sarcomatoid Transitional Cell Carcinoma of the Renal Pelvis                                  Case Report

Sarcomatoid Transitional Cell Carcinoma of the
Renal Pelvis: A Case Report
Yu-Li Lin, Qiang-Sheng Ke, Yuan-Ming Liaw, Hann-Chorng Kuo

Department of Urology, Buddhist Tzu Chi General Hospital, Hualien, Taiwan

     Sarcomatoid transitional cell carcinoma (TCC) of the renal pelvis is a rare tumor with sarcoma-like components
arising from the malignant transitional epithelium. The prognosis, according to previous reports, is very poor. Due to its
rarity, there have been few studies concerning the treatment efficacy. We herein report a case of sarcomatoid TCC aris-
ing from the renal pelvis. A 67-year-old female was admitted with the chief complaint of bilateral flank pain. A right-
renal heterogeneous mass measuring about 5 x 7 x 10 cm was incidentally found on a clinical image. A right radical
nephrectomy was performed. Histologically, a TCC in situ and another sarcoma-like area with prominent spindle cells
were identified. Immunohistochemistry was positive for cytokeratin. Sarcomatoid TCC of the renal pelvis was confirmed.
In spite of no ample evidence of an effective treatment, we recommend a radical, aggressive operation for early localized
disease. For the tumor with advanced stage or metastasis, systemic chemotherapy with a gemcitabine-based regimen
may be considered for this uncommon renal pelvic tumor due to its urothelial origin in nature. (JTUA 19:48-51, 2008)
Key words: renal cancer, sarcomatoid, spindle cell carcinoma, carcinosarcoma.

                   CASE REPORT                                  ing chest x-ray, an abdominal-to-pelvic computed to-
                                                                mographic (CT) scan, and whole-body bone scan were
     This 67-year-old female patient with a past history        performed. The CT scan showed a heterogeneous mass
of right ureteral stone 20 years ago presented to our clinic    measuring about 5 × 7 × 10 cm occupying the right re-
with bilateral flank pain for several years. Moreover,          nal pelvis and several enlarged para-aortic lymph nodes
intermittent gross hematuria was also noted. On physi-          were also noted (Fig. 1). A chest x-ray and bone scan
cal examination, bilateral mild knocking tenderness was         showed no evidence of lung or bone metastasis.
noted, but there was no palpable abdominal mass. Urine
analysis showed predominant occult blood and 6~8 red
blood cells (RBCs) per high-power field. Renal
sonography revealed bilateral stones and moderate bi-
lateral hydronephrosis. The kidney, ureter, and bladder
(KUB) X-ray study revealed multiple radiopaque stones
in the bilateral kidney. Under the impression of bilateral
renal stones, she was admitted to our ward.
     On the day of admission, bilateral percutaneous
nephrostomy drainage was performed to relieve the
hydronephrosis. Antegrade pyelography showed bilat-
eral caliectasis and multiple renal stones. A ureteroscopic
examination incidentally found a right renal pelvic tu-
mor with stone encrustation. A biopsy was taken, and
spindle cell sarcoma was our initial diagnosis.
     In the following days, a metastatic survey includ-

Address reprint requests and correspondence to:                 Fig. 1.   Computed tomographic scan demonstrating a right
Hann-Chorng Kuo, MD.                                                      renal heterogeneous mass measuring about 5 x 7 x
Department of Urology, Buddhist Tzu Chi General Hospital, 707
Chung Yang Road, Section 3, Hualien, Taiwan 970, R.O.C.                   10 cm (white arrows) with enlargement of several
Tel: 886-3-8561825 ext. 2117 Fax: 886-3-8560794                           para-aortic lymph nodes. Bilateral hydronephrosis
E-mail:                                                 and a right renal stone were also noted.

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                                                                                                                 Y. L. Lin, et al

     One week after the initial operation, a radical ne-
phrectomy was performed. A large whitish, intracapsular
solid right renal tumor with invasion to the renal sinus
and calyceal system was noted during the operation.
Several lymphadenopathies over the aortovenous area
were also identified. Gross examination revealed a huge
solid mass measuring 8.0 × 7.0 × 5.0 cm occupying the
renal pelvis which was whitish, gelatinous, and focally
necrotic on cutting (Fig. 2). Microscopic examination
revealed 2 main components of the tumor: 1 component
with TCC in situ and another sarcoma-like area with
prominent spindle cells. Some multinucleated giant cells
were also seen. Immunohistochemistry was positive for
cytokeratin, indicating an epithelial origin (Fig. 3A-D).
                                                                   Fig. 2.   Gross appearance of the tumor showing a solid mass
In addition, there was involvement of 2 lymph nodes in
                                                                             measuring 8.0 x 7.0 x 5.0 cm occupying the renal
4 para-aortic lymph nodes. Sarcomatoid TCC of the re-                        pelvis with whitish, gelatinous, and focal necrosis
nal pelvis with para-aortic lymph node involvement was                       on the cut surface.

Fig. 3.   Sarcomatoid transitional cell carcinoma (TCC) of the renal pelvis. (A) TCC in situ, (B, C) the sarcomatoid component
          with prominent spindle cells and some multinucleated tumor cells, and (D) a sarcomatoid area showing immunoreactivity
          for cytokeratin.

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Sarcomatoid Transitional Cell Carcinoma of the Renal Pelvis

Fig. 4.   Computed tomographic (CT) scan at 3 months after the operation showing (A) a recurrent bulky tumor with direct liver
          invasion and involvement of multiple lymph nodes; (B) tumor expansion from the previous percutaneous nephrostomy
          tract (white arrow).

the ultimate diagnosis (pT3N2M0). One course of adju-              is a term frequently confused with sarcomatoid
vant chemotherapy with gemcitabine was given.                      carcinoma. It has a malignant epithelial component as
Unfortunately, the patient's condition gradually                   does a sarcomatoid carcinoma. In addition, it has spe-
deteriorated, and a follow-up CT scan 3 months after               cific features of mesenchymal differentiation, such as
the radical operation showed recurrent bulky tumor with            chondrosarcomas, osteosarcomas, rhabdomyosarcomas,
liver invasion and multiple lymph node involvement.                and liposarcomas, which differ from a sarcomatoid
Direct tumor expansion from the previous percutaneous              carcinoma. However, these 2 tumors have frequently
nephrostomy tract was also noted (Fig. 4A,B).                      presented diagnostic difficulties because their morpholo-
                                                                   gies are similar in microscopic appearance with hema-
                     DISCUSSION                                    toxylin and eosin staining. As a result, an immunohis-
                                                                   tochemical study is usually necessary for a diagnosis.
     Sarcomatoid carcinoma is a high-grade epithelial              Immunoreactivity to cytokeratins and CD68 is specific
neoplasm characterized by a biphasic appearance with               for cells of epithelial origin and diagnostic for a sarco-
1 part of an epithelial component and another area with            matoid carcinoma. Carcinosarcomas do not stain for
a sarcoma-like appearance, which is largely composed               epithelial markers.4,5
of non-specific spindle and/or pleomorphic tumor gi-                    Most renal pelvis malignancies consist of TCC.
ant-cells.1 The actual histogenesis of sarcomatoid carci-          However, a sarcomatoid carcinoma of transitional cell
noma is still unclear. In 1 study, because they found that         origin in the renal pelvis is very rare. In a clinicopatho-
the sarcomatous component exhibited diffuse and in-                logic study of 108 cases of high-grade urothelial carci-
tense p53 oncoprotein expression, whereas the carcino-             nomas of the renal pelvis, only 8 cases were shown to
matous component showed a weaker and focal positivity,             be sarcomatoid carcinomas.6 Lopez-Beltran et al. re-
they assumed that this alteration of the p53 gene might            ported 13 cases of sarcomatoid TCC of the renal pelvis
be related to the sarcomatous transformation of the                since 1961.7 Metastatic disease or advanced tumor in-
tumor.2 In another study, it was thought that the sarco-           volvement of the parenchyma was noted with the diag-
matoid component of sarcomatoid carcinomas may re-                 nosis in all 13 patients. The prognosis was very poor
sult from either anaplastic changes or de-differentiation          with 8 patients dying within 2 years of diagnosis and a
related to the process of losing cell adhesion mole-               mean survival time of 11.2 months. Moreover, adjuvant
cules.3                                                            chemotherapy appeared to offer no response or survival
     In addition to the kidney and bladder, sarcomatoid            advantage.
carcinoma can also occur in the breast, larynx, pharynx,                The Mayo Clinic reviewed 26 cases of sarcomatoid
oral cavity, esophagus, and genital tract. Carcinosarcoma          carcinomas of transitional cell origin in the bladder dur-

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                                                                                                             Y. L. Lin, et al

ing a 59-year period. Sarcomatoid carcinoma of the blad-     chemotherapy with a gemcitabine-based regimen seems
der was more common in men than in women (with a 4:          to be reasonable for this uncommon renal pelvic tumor
1 ratio), and the mean survival was 9.8 months. The          due to its urothelial origin in nature.
pathologic stage was the main predictor of patient
survival. Chemotherapy offered little response and no                            REFERENCES
survival advantage.4
      Since sarcomatoid carcinomas of transitional cell       1. Hisataki T, Takahashi A, Taguchi K, et al. Sarcomatoid
origin are a very rare entity, they consistently pose a          transitional cell carcinoma originating from a duplicated
significant therapeutic challenge to clinicians. Although        renal pelvis. Int J Urol 2001;8:704-6.
                                                              2. Acikalin MF, Kabukcuoglu S. Sarcomatoid carcinoma
experience with the treatment of patients with sarcoma-          of the renal pelvis with giant cell tumor-like features:
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extensive operation to treat this disease. However, it re-       2005;12:199-203.
mains uncertain whether such an operation contributes         3. Ikegami H, Iwasaki H, Ohjimi Y, Takeuchi T, Ariyoshi A,
to improved survival.1 Thiel et al. presented the case of        Kikuchi M. Sarcomatoid carcinoma of the urinary
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                                                              4. Lopez-Beltran A, Pacelli A, Rothenberg HJ, et al. Car-
scopic nephroureterectomy was performed, and no fur-             cinosarcoma and sarcomatoid carcinoma of the bladder:
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out recurrence for more than 1 year.8                            1497-503.
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ate to treat these neoplasms in the same manner as con-          carcinoma of the renal pelvis: a case report. Pathol Int
ventional high-grade TCC with similar degrees of                 2000;50:562-7.
invasion.2,9 However, to our knowledge, no previous           6. Perez-Montiel D, Wakely PE, Hes O, Michal M, Suster
                                                                 S. High-grade urothelial carcinoma of the renal pelvis:
report is available for a sarcomatoid TCC of the renal           clinicopathologic study of 108 cases with emphasis on
pelvis treated with systemic chemotherapy. Froehner et           unusual morphologic variants. Mod Pathol 2006;19:494-
al.9 reported an 80-year-old patient with a sarcomatoid          503.
carcinoma of the bladder and lung metastasis. They dem-       7. Lopez-Beltran A, Escudero AL, Cavazzana AO, et al.
onstrated a remarkable response of the residual tumor            Sarcomatoid transitional cell carcinoma of the renal
in the lung and complete remission during 2 years of             pelvis: a report of five cases with clinical, pathological,
follow-up after treatment with 4 courses of gemcitabine          immunohistochemical, and DNA ploidy analysis. Pathol
                                                                 Res Pract 1996;192:1218-24.
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      In conclusion, sarcomatoid TCC of the renal pelvis         transitional cell origin confined to renal pelvis. Urology
is a very rare high-grade neoplasm with a very poor              2006;67:622.
prognosis. For early localized disease, a radical, aggres-    9. Michael F, Hans-Juergen G, et al: Durable complete
sive operation should be considered to improve the               remission of metastatic sarcomatoid carcinoma of the
outcomes; for a tumor with advanced stage or metastasis,         bladder with cisplatin and gemcitabine in an 80-year-
the prognosis is extremely poor. Despite no effective            old man. Urology 2001;58:799.
treatments having previously been reported, systemic

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