Indian Journal of Pathology and Microbiology
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CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 147-149
A case of a sporadic malignant peripheral nerve sheath tumor of the urinary bladder with concomitant in situ urothelial carcinoma treated by transuretheral resection


1 Department of Pathology, Izmir Ataturk Training and Research Hospital, Izmir, Turkey
2 Department of Urology, Izmir Ataturk Training and Research Hospital, Izmir, Turkey

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Date of Web Publication7-Mar-2011
 

   Abstract 

Malignant peripheral nerve sheath tumor (MPNST) of the urinary bladder is a very rare clinical entity. The association of such a tumor with urothelial carcinoma is even more unusual. Differential diagnosis between coexisting two distinct primary tumors and carcinosarcoma of the urinary bladder is very important as both the treatment and prognosis vary widely. Herein, we report a case of an MPNST with a concomitant in situ urothelial carcinoma in a 53-year-old man. To our knowledge, this is the first documented case of MPNST of the bladder that is treated by transuretheral resection which is in contrast with the previous reports that used cystectomy.

Keywords: Bladder tumor, carcinoma in situ, malignant peripheral nerve sheath tumor, sarcoma, transuretheral resection, treatment

How to cite this article:
Sari A, Bal K, Tunakan M, Ozturk C. A case of a sporadic malignant peripheral nerve sheath tumor of the urinary bladder with concomitant in situ urothelial carcinoma treated by transuretheral resection. Indian J Pathol Microbiol 2011;54:147-9

How to cite this URL:
Sari A, Bal K, Tunakan M, Ozturk C. A case of a sporadic malignant peripheral nerve sheath tumor of the urinary bladder with concomitant in situ urothelial carcinoma treated by transuretheral resection. Indian J Pathol Microbiol [serial online] 2011 [cited 2014 Sep 1];54:147-9. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/147/77376



   Introduction Top


Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms accounting for approximately 5-10% of all soft tissue sarcomas. Approximately, 50-60% of MPNSTs are associated with Von Recklinghausen's disease (VRD). [1] Bladder is an extremely rare location for this malignancy. [2] In this article, we report the first case of a sporadic MPNST of the bladder with a concomitant urothelial carcinoma in situ, which was successfully treated with transurethral resection (TUR).


   Case Report Top


A 53-year-old male patient got admitted to the urology outpatient clinic with the complaint of gross painless hematuria which was present nearly for the last 20 days. His physical examination was normal. He had history of cigarette smoking 40 packs per year for 40 years. Ultrasound examination showed a solid mass lesion of 30 × 25 mm originating from the anterior wall of the bladder. The patient was scheduled for TUR. The cystoscopy findings were typical for a bladder tumor. The lesion was solid in gross appearance, approximately 4 cm in greatest diameter, located on the anterior bladder wall and had a pedicle. Complete resection of the tumor was performed and the tumor base was fulgarized. A single dose of mitomycin was administered postoperatively and the patient was discharged the next day without complications. Microscopic examination revealed a highly cellular tumor with intervening myxoid areas. Spindle and wavy shaped tumoral cells were mitotically active and had hyperchromatic and pleomorphic nuclei [Figure 1] and [Figure 2]a. Perivascular crowding of tumoral cells and alternating hyper and hypocellular areas were striking [Figure 2]. There was no necrosis, and no entrapped epithelial elements were observed. Urothelium was focally present [Figure 1] and elsewhere was ulcerated. Tumor was located just beneath the urothelium. Immunohistochemically, tumoral cells were only focally positive for S100 [Figure 3]a. Smooth muscle actin, desmin, neurofilaman, CD 57, cytokeratin 7 and pancytokeratin were all negative. With the aid of immunohistochemistry, diagnosis of MPNST was established. Following consultation with neurology department, the patient underwent diagnostic work-up for detection of neurofibromatosis. Physical examination and magnetic resonance imaging of the cervical spinal cord and cranium revealed no evidence of neurofibromatosis. Three weeks after the initial operation, the patient underwent second TUR in order to eradicate residual tumor and to prevent local relapse. There was a granulation tissue related to the first resection without macroscopically detectable tumor. A deeper and wider resection of the tumor bed was performed, reaching the inner layers of detrusor muscle. Microscopic examination of the second TUR revealed intact lamina propria, muscularis propria and showed in situ urothelial carcinoma [Figure 3]b. The patient received six cycles of intracavitary immunotherapy with BCG. Control cystoscopy and the urine cytology were negative for tumor relapse. The patient is free of disease and under close surveillance for the last 24 months.
Figure 1: Serpentine-shaped tumoral cells with hyperchromatic nuclei are embedded in a myxoid stroma just beneath the urothelium. (H and E, ×200)

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Figure 2: (a) Perivascular crowding of tumoral cells; (b) alternating hyper and hypocellular areas composed of intersecting fascicles of spindle-shaped tumoral cells. (H and E, ×100)

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Figure 3: (a) Focal S100 immunostaining in tumoral cells (IHC, ×400); (b) urothelial carcinoma in situ, in second TUR. (H and E, ×100)

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   Discussion Top


MPNSTs are rare neoplasms originating from the neural sheath of peripheral nerves. The histologic spectrum of MPNST is broad and the diagnosis rests on combination of some microscopic features, none of which is diagnostic by itself. S100 protein, the most widely used antibody for nerve sheath tumors, is positive only in 50% of MPNSTs. [1] Another diagnostic marker, Leu 7 immunoreactivity, is reported to be positive in 30-40% of the cases. [1] Our case showed focal positivity with S100 protein and was negative for Leu 7 staining.

Bladder is an extremely rare location for MPNST, and to the best of our knowledge, only six previous cases have been reported in the literature [Table 1]. [2],[3],[4],[5],[6],[7] Four of these cases arose from malignant degeneration of pre-existing neurofibromas in the setting of VRD. [3],[4] The other case had a history of transurethral resected bladder ganglioma with NF, [2] and only one case was reported to be sporadic. [5]
Table 1: Reported cases of MPNST of the urinary bladder

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The occurrence of multiple tumors in one organ is very unusual. Seven cases of coexisting sarcoma and carcinoma of the bladder have been reported till date. [8] In all cases, the carcinomatous component was urothelial carcinoma. The major differential diagnosis of these cases, as in our case, was sarcomatoid carcinoma. The most striking point in all previously reported cases was the absence of the admixture of the carcinomatous and sarcomatous components. In our case, i) the absence of CK positivity ii) two distinct tumors not admixing with each other, iii) microscopic and immunohistochemical features that are consistent with MPNST, and iii) muscularis propria being free of the tumor in contrast to the sarcomatoid carcinomas, which almost always present at higher stages, [9] made us evaluate these tumors as two seperate tumors rather than sarcomatoid carcinoma. In the case of multiple tumors having different histogenesis, treatment should be directed to each as distinct and separate entities. [10]

MPNSTs are high grade sarcomas with a high tendency for local recurrence and distant metastasis. The management of MPNST consists of early and radical tumor resection. [5] However, there is no established treatment for bladder MPNST as these tumors are very rare and cystectomy was conducted in the majority of the previously reported cases. [2],[3],[5],[6] In our case, we successfully treated our patient with TUR without relapse after 11 months of follow-up.

In conclusion, differential diagnosis between coexisting two distinct primary tumors and carcinosarcoma of the urinary bladder is important as both the treatment and prognosis vary widely. The management of MPNST of the bladder is not still well defined, but, as the present case indicates, complete resection of the tumor by TUR seems to be an effective treatment option. However, regardless of whether complete or incomplete resection is done, continued long-term follow-up should be carried out for all patients with MPNST of the bladder due to the risk of recurrences and for detecting early metastasis.

 
   References Top

1.Rosai J. Soft tissues. In: Ackerman R, editor. Surgical Pathology. Philadelphia: Mosby; 2004. p. 2237-371.  Back to cited text no. 1
    
2.Kalafatis P, Kavantzas N, Pavlopoulos PM, Agapitos E, Politou M, Kranides A. Malignant peripheral nerve sheath tumor of the urinary bladder in von Recklinghausen disease. Urol Int 2002;69:156-9.  Back to cited text no. 2
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3.Dahm P, Manseck A, Flossel C, Saeger HD, Wirth M. Malignant neurofibroma of the urinary bladder. Eur Urol 1995;27:261-3.  Back to cited text no. 3
    
4.Hulse CA. Neurofibromatosis: Bladder involvement with malignant degeneration. J Urol 1990;144:742-3.  Back to cited text no. 4
[PUBMED]    
5.Mimata H, Kasagi Y, Ohno H, Nomura Y, Iechika S. Malignant neurofibroma of the urinary bladder. Urol Int 2000;65:167-8.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Rober PE, Smith JB, Sakr W, Pierce JM Jr. Malignant peripheral nerve sheath tumor (malignant schwannoma) of urinary bladder in von Recklinghausen neurofibromatosis. Urology 1991;38:473-6.  Back to cited text no. 6
[PUBMED]    
7.Ross JA. A case of sarcoma of the urinary bladder in von Recklinghausen's disease. Br J Urol 1957;29:121-6.  Back to cited text no. 7
[PUBMED]    
8.Bakaris S, Resim S, Tasci AI, Demirpolat G. A rare case of synchronous leiomyosarcoma and urothelial cancer of the bladder. Can J Urol 2008;15:3920-3.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.Histological variants of urothelial carcinoma. In: Epstein JI, Amin MB, Reuter VE, editors. Bladder biopsy interpretation. Philadelphia: Lippincott Williams and Wilkins; 2004. p. 120-5.  Back to cited text no. 9
    
10.Chen KT. Coexisting leiomyosarcoma and transitional cell carcinoma of the urinary bladder. J Surg Oncol 1986;33:36-7.  Back to cited text no. 10
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Correspondence Address:
Aysegul Sari
Ataturk Caddesi, Firat Apt. No:184, kat:3, daire:6, PK:35210, Alsancak, Izmir
Turkey
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DOI: 10.4103/0377-4929.77376

PMID: 21393901

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    Figures

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