British Journal of Radiology (2004) 77, 159-160
© 2004 British Institute of Radiology
doi: 10.1259/bjr/28175121
Primary rhabdomyosarcoma of the seminal vesicle
D A Sanghvi, MD, DNB
1
N C Purandare, DMRD, DNB
1
N A Jambhekar, MD
2
M H Thakur, MD
1 and
M S Joshi, MD
1
Departments of 1 Radiodiagnosis and 2 Pathology, Tata Memorial Hospital, Dr Ernest Borges Road, Parel, Mumbai 400 012, India
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Abstract
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Sarcomas of the seminal vesicle are very rare and poorly documented; as it is not always possible to pinpoint a truly vesicular origin of the pelvic mass due to local spread at the time of presentation. The purpose of the article is to document and characterize a rhabdomyosarcoma of the seminal vesicle of which to the knowledge of the authors there has been no previous report in the English literature.
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Introduction
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Primary malignancies of the seminal vesicles are rare. The validity of positive cases of this type is often questionable; as in many instances there is local spread of the malignant process and it is not always possible to confirm a truly vesicular origin of the pelvic tumour on imaging.
We present a case of primary sarcoma which on imaging studies was unequivocally topographically centred in the right seminal vesicle. The case was evaluated using transrectal ultrasound (TRUS) and CT.
Histopathology and immunohistochemistry (IHC) revealed that the tumour was a soft tissue sarcoma with skeletal muscle differentiation. Malignancies arising primarily in the seminal vesicles are uncommon. Most cases are carcinomas. A review of the literature revealed occasional documentation of sarcomas. The sarcomas showing myogenic differentiation could be either leiomyosarcomas or rhabdomyosarcomas. There were six previously reported cases of primary leiomyosarcomas of the seminal vesicle, not all of which were adequately substantiated with IHC or electron microscopy. There was no documentation of skeletal muscle differentiation in seminal vesicle sarcomas.
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Case report
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A 58-year-old man presented with backache and weight loss of 10 kg in 4 months. Routine blood investigations were normal. A digital rectal examination revealed a firm, asymmetrical mass in the region of the prostate and bladder base. Serum prostate specific antigen levels were 0.6 ng ml-1 (normal 0–4 ng ml-1). Urine culture was positive for Klebsiella pneumoniae. His chest radiograph showed multiple, nodular, parenchymal opacities.
TRUS revealed a hypoechoic mass in the right seminal vesicle, infiltrating the prostate and the right ureterovesical junction (Figure 1
), with secondary right sided hydronephrosis and hydroureter. A CT scan of the abdomen and pelvis revealed a lobulated, heterogeneously enhancing mass topographically centred in the right seminal vesicle (Figure 2). The mass measured 4.8 cm x 5.0 cm x 4.3 cm and had indistinct fat planes with the adjacent prostate gland and urinary bladder, suggestive of infiltration of these structures by the mass. The left seminal vesicle was uninvolved. Right sided hydronephrosis and hydroureter were seen on the CT scan, as a result of backpressure due to infiltration of the right ureterovesical junction by the mass. A CT scan of the thorax showed multiple, bilateral, nodular, parenchymal opacities, suggestive of pulmonary metastases. Also noted on the CT scan were osteolytic lesions in the ribs and dorsal vertebrae. A technetium-99m methyl diphosphonate bone scan showed increased tracer concentration in multiple ribs, dorsal vertebrae and the right femur, suggestive of skeletal metastases.
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Figure 1. Transrectal ultrasound shows a hypoechoic mass involving the right seminal vesicle. M, soft tissue mass; UB, urinary bladder.
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Figure 2. Axial post contrast CT image shows an enhancing soft tissue mass (asterix) topographically centred in the right seminal vesicle. The left seminal vesicle is uninvolved.
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A TRUS guided biopsy was performed. Routine histopathology with haematoxylin and eosin (H and E), and immunohistochemical studies confirmed a diagnosis of primary rhabdomyosarcoma of the seminal vesicle.
The patient is being treated with chemotherapy.
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Discussion
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Primary tumours of the seminal vesicles are rare. Most reported cases are carcinoma. A review of literature revealed 19 documented cases of primary sarcomas of the seminal vesicles, which had been reported between 1885 to 2000. Of these, there were five previously reported cases of primary leiomyosarcoma of the seminal vesicles [1–5] and one case of leiomyoma "with suggestion of sarcomatous potential" [6], but no previous report of a primary rhabdomyosarcoma of the seminal vesicle. We found two more references from non-English language literature alleging origin of the sarcoma in the seminal vesicle.
The authenticity of many of these reported cases have been questioned in subsequent reviews by authors such as Lazarus [7] and Schned et al [2]. Schned et al have mentioned in their scholarly review that many of the reported cases of primary sarcomas of the seminal vesicle are poorly documented. This is due in part to the tendency of this tumour to widely invade neighbouring structures, often obscuring the actual place of origin. Other reasons cited by them for questioning the validity of many of the previously reported cases is the lack of histological verification. Buck and Shaw [6] in their report on mesenchymal tumours of the seminal vesicles have also written of the difficulty to distinguish between tumours arising from the seminal vesicle and those arising from other tissue elements of the retrovesical space.
In comparison, in our case the tumour was topographically centred in the right seminal vesicle. In spite of distant dissemination as well as local spread of the malignant process, the CT images unequivocally showed the origin of the tumour to be from the seminal vesicle.
Histopathology and IHC studies were performed on the material obtained by TRUS guided biopsy. In view of the light microscopic findings and IHC studies demonstrating strong immunoreactivity with desmin, sarcomeric actin and vimentin and lack of reaction with smooth muscle actin, a diagnosis of rhabdomyosarcoma was made. Desmin is a sensitive and specific marker for rhabdomyosarcoma [8]. Although as many as 17% of non-myogenic sarcomas may display immunoreactivity for desmin, the reactivity is unequivocal and focal [8], unlike the diffuse positivity seen in the present case. To the knowledge of the authors, there has been no previous documentation in literature of skeletal muscle differentiation in sarcomas of the seminal vesicle.
Clinically, tumourous involvement of the seminal vesicles can yield a wide range of symptoms, depending in part on the tumour size and contiguous spread [9]. The most common symptoms in patients with primary tumours of the seminal vesicles include pelvic or perineal pain, voiding symptoms and urinary retention, or symptoms of metastases and the most common physical finding is a high, firm asymmetrical mass on rectal examination [10].
Since pelvic genitourinary sarcomas are rare tumours, the data regarding therapeutic approach and outcome are limited. Surgery is the treatment of choice in the absence of distant metastatic spread. In cases with widespread distant metastases, such as ours, chemotherapy is the preferred line of treatment. Rhabdomyosarcomas are very chemosensitive.
Although primary neoplasms of the seminal vesicles remain rare lesions, it is possible that the increased use of modern radiographic techniques, including TRUS, CT and MRI will allow for earlier recognition and the additional use of immunohistochemical staining techniques on material obtained with TRUS guided biopsy will allow for the differentiation of sarcomatous lesions from those of epithelial origins [11].
Received for publication September 24, 2002.
Revision received May 22, 2003.
Accepted for publication June 11, 2003.
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References
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- Tripathi VNP, Dick VS. Primary sarcoma of the urogenital system in adults. J Urol 1969;101:898–904.[Medline]
- Schned AR, Ledbetter JS, Selikowitz SM. Primary leiomyosarcoma of the seminal vesicle. Cancer 1986;57:2202–6.[Medline]
- Amirkhan RH, Molberg KH, Wiley EL, Nurenberg P, Sagalowsky AI. Primary leiomyosarcoma of the seminal vesicle. Urol 1994;44:132–5.
- Cordonnier C, Sevestre H, Petit J, Fillou X, Morfaux V, de Saint Maurp, et al. Rare tumours of the prostate or seminal vesicles. Report of a case of leiomyosarcoma. Ann Pathol 1996;16:37–40.[Medline]
- Muentener M, Hailemariam S, Dubs M, Hauri D, Sulser T. Primary leiomyosarcoma of the seminal vesicle. J Urol 2000;164:2027.[Medline]
- Buck AC, Shaw RE. Primary tumours of the retrovesicle region with special reference to mesenchymal tumours of the seminal vesicles. Br J Urol 1972;44:47–50.[Medline]
- Lazarus JA. Primary malignant tumours of the retrovesical region with special reference to malignant tumours of the seminal vesicles: report of a case of retrovesical sarcoma. J Urol 1946;55:190–205.
- Altmannsberger M, Weber K, Droste R, Osborn M. Desmin is a specific marker for rhabdomyosarcomas of human and rat origin. Am J Pathol 1985;118:85–95.[Abstract]
- Boreau J. Diagnosis and treatment of primary malignant tumours of the seminal vesicles. Recent Results Cancer Res 1977;60:157–62.
- Chiou RK, Limas C, Lange PH. Hemangiosarcoma of the seminal vesicle: case report and literature review. J Urol 1985;134:371–3.[Medline]
- Lamont JS, Hesketh PJ, de las Morenas A, Babayan RK. Primary angiosarcoma of the seminal vesicle. J Urol 1991;146:165–7.[Medline]
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Abstract
Introduction
