| Abstract|| |
Adenosarcoma like tumor of the seminal vesicle is reported herein. A 35-year-old male presented with mass in the pelvis between bladder and rectum, involving the seminal vesicle and prostate. Mass recurred after enucleation in four years. Histologically, the tumor was multicystic with bland ciliated lining epithelium and sarcomatous stroma. A wide excision was performed followed with chemotherapy and radiotherapy. Adenosarcomas have a low grade recurrent malignant potential and should be recognized.
Keywords: Adenosarcoma, mullerian and retrovesicle, seminal vesicle
|How to cite this article:|
Chheda N, Bolegave M, Shet T, Tongaonkar H. Recurrent mullerian adenosarcoma like tumor of seminal vesicle. Indian J Pathol Microbiol 2010;53:342-4
|How to cite this URL:|
Chheda N, Bolegave M, Shet T, Tongaonkar H. Recurrent mullerian adenosarcoma like tumor of seminal vesicle. Indian J Pathol Microbiol [serial online] 2010 [cited 2014 Dec 22];53:342-4. Available from: http://www.ijpmonline.org/text.asp?2010/53/2/342/64331
| Introduction|| |
Mullerian tumors have been described at uterine and extra uterine sites in females.  Testicular and paratesticular tumors resembling ovarian tumors, though rare have been reported in males.  These tumors arise from mullerian metaplasia of the peritoneal lining of the tunica vaginalis, appendix testis or mullerian remnants between testis and spermatic cord.  The prostate gland is also notorious for harboring epithelial tumors similar to those observed in females. However, there are only anecdotal reports of such type of tumors in the seminal vesicle. 
We describe one such malignant tumor of mullerian origin in a man with a view to discuss diagnostic and management issues that plague these rare tumors.
| Case Report|| |
A 35-year-old male presented with increased frequency and pain during defecation with loss of appetite since one month. He had retention of urine of seven days duration. A colonoscopy showed an extra luminal mass compressing the lumen of the rectum and the sigmoid colon. A CT (computed axial tomography) scan revealed a midline globular low attenuated enhancing mass measuring 10x9.3x11 cm in the pelvic cavity between the rectum and the urinary bladder [Figure 1]. No calcification was seen in the tumor. The lesion was compressing the bladder and the prostate anteriorly and the rectum posteriorly in an expansile manner maintaining a circumscribed contour. This mass was enucleated, considering it to be benign. It was reported as localized cystic mesothelioma elsewhere, following which the patient was observed and remained asymptomatic for four years.
In 2007 he came to our institute with the recurrent complaints of increased frequency of micturition and dysuria. A CT scan performed now, revealed a similar cystic mass with septae in the rectovesical pouch indenting upon the seminal vesicle and posterior part of prostate. The mass was seen approximating the anterior wall of the rectum with lost interface and measured 11cm in largest dimension. No lymphadenopathy was noted. The previous histopathology slides were reviewed and a diagnosis of biphasic tumor of probably mullerian origin was offered. In view of recurrent disease that was not likely to be chemotherapy or radiotherapy sensitive, an exploratory laparotomy with wide excision of the mass along with a segment of rectum with colostomy was performed. Intra-operatively, the lesion was found adherent and possibly arising from the seminal vesicle. Subsequent to the histology report patient received postoperative chemotherapy and EBRT (external brachy radiotherapy). Chemotherapy regimen included six cycles of CAP chemotherapy (cisplatin, adriamycin and endoxan). After completion of therapy, a CT scan and colonoscopy did not reveal any abnormality and the patient is disease free for the last 10 months.
The excised recurrent tumor measured 6.5x4x1.5 cm and contained cystic areas. Internal surface of the cyst wall was rough and contents were serous.
Histological evaluation revealed a multicystic tumor, composed of an epithelium lining cystic spaces with an intervening stromal component [Figure 2]a. The epithelial component was composed of a single layer of benign columnar or cuboidal lining with fine vacoulation and terminal cilia [Figure 2]b and c. No mitoses were seen in the epithelial components. The stroma surrounding the glands was fibroblastic but very cellular with large pleomorphic stromal cells and multinucleate giant cells (inset of [Figure 2] a). The nuclei were hyperchromatic and showed marked variation in size and shape. Mitotic figures including atypical mitoses were noted. No teratomatous elements were seen. On immunohistochemistry the malignant stromal cells only expressed vimentin. The epithelial component stained with cocktail cytokeratin (CK), cytokeratin7 (CK 7) and epithelial membrane antigen (EMA). Calretinin, prostate specific antigen (PSA), HBME-1, S100 protein, CK 20, HMB-45 and desmin were negative in the stromal cells. The final diagnosis was mullerian adenosarcoma like tumor of seminal vesicle was offered.
| Discussion|| |
Primary seminal vesicle tumors are very rare and include both cysts and neoplasms. , Seminal vesicle tumors are mostly misdiagnosed as arising from other sites as they are very large by the time they present and it is difficult to pinpoint the exact site of origin. Within the pelvis a seminal vesicle is rarely thought of as a site of primary tumor. The most common malignant neoplasm of seminal vesicle is adenocarcinoma, followed by sarcomas. ,
The differential diagnoses considered in the case under discussion included biphasic tumors like adenocarcinoma with sarcomatoid change and mesothelioma. On immunohistochemistry both the epithelial lining and the sarcomatous component appeared distinct and unrelated with obvious different grade; hence an adenocarcinoma with sarcomatous change was ruled out. The absence of mesothelial markers on immunohistochemistry helped in ruling out a mesothelioma. Seminal vesicle is also a rare site for tumors of mullerian origin. The biphasic tumors of probable mullerian origin that are reported in the seminal vesicles include cystosarcoma phyllodes, cystic epithelial tumor of the seminal vesicle and adenosarcoma like tumor of the testis. ,,,, The relationship and distinctive features between these three entities cannot be adequately defined as each has been described in case reports only. A cystic epithelial stromal tumor is reported to have abnormal glands while the later two have benign glands. The cystic epithelial stromal tumor may be one end of spectrum of mullerian adenosarcoma in view of it's propensity for local recurrence. , In contrast to the adenosarcoma, the more common cystosarcoma phyllodes of seminal vesicle frequently show lung metastasis hinting at the high grade malignant potential.  Hence, a mullerian adenosarcoma of seminal vesicle with low grade malignant potential should be distinguished from the high grade phyllodes tumor of seminal vesicle. Though there is no distinct line of demarcation the absence of leaf like clefts (with a multicystic appearance) and fasciculated stroma made a phyllodes tumor less likely in the case reported herein. An interesting observation was that this tumor expressed cytokeratin 7 similar to that described in seminal vesicle adenocarcinomas indicating origin from mullerian like metaplasia from seminal vesicle epithelium rather than true mullerian nature. 
The clinical and histological findings in the case reported are identical in the case reported earlier.  Comparing the behavior of the previous case with our case it appears that such type of tumors that have been completely or radically excised do not recur. One case reported earlier had no recurrence after undergoing cystoprostatectomy.  The case in discussion too had a disease free interval of four years inspite of incomplete surgery indicating a slow growth potential.
The guidelines for the management of mullerian adenosarcoma of seminal vesicle are unclear and the role of adjuvant therapy still debatable. Though our case did receive adjuvant therapy it was in the setting of incomplete surgery and recurrence. In our opinion patients with radical complete surgery at presentation may be spared adjuvant chemotherapy/radiotherapy and these should be reserved for cases where complete excision is not possible.
To conclude, mullerian adenosarcoma, like tumor of seminal vesicles, is a rare tumor with indolent course and propensity for local recurrence. Accurate recognition and complete excision may help in managing these patients better.
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[Figure 1], [Figure 2]