Correspondence
Leiomyosarcoma of prostate: A rare, aggressive tumor ABSTRACT Leiomyosarcoma of prostate is a rare, aggressive tumor with bizarre presentation at advanced stage. Though definite treatment modalities are not recommended till now, innovative combined multimodality treatment strategies may help to improve the prognosis of patients with this tumor of poor survival rate. Here we report a case of leiomyosarcoma of the prostate which presented with chest metastasis. KEYWORDS: Cancer, leiomyosarcoma, prostate, sarcoma,
INTRODUCTION Sarcoma of the prostate is an extremely rare neoplasm that accounts for less than 0.1% of primary prostate malignancies.[1] Leiomyosarcoma is the most common primary sarcoma of the prostate in adults and constitutes 38-52% of primary prostatic sarcomas.[2] It has an aggressive clinical course.[3] Here we present such a case of leiomyosarcoma of the prostate and a review of the literature regarding the clinical features, diagnostic modalities and therapeutic aspects of this rare entity. CASE HISTORY A 35-year-old male patient presented with complaints of narrow, intermittent urinary stream for three months and progressive perineal pain for 15 days. On examination, the bladder was palpable, genitalia were normal and on digital rectal examination, the
prostate was non tender, lobulated, asymmetrically enlarged with variegated consistency. Urinalysis and blood biochemistry reports were normal except serum prostate specific antigen (P.S.A.)which was slightly higher (5.31 ng/ml). On trans rectal ultrasonogram [T.R.U.S.], prostate volume was 363 cc with an ill-defined, heterogeneous 66×61.1×80.3 mm mass lesion on the left posterolateral aspect of the prostate gland [Figure 1]. Twelve-core TRUSguided prostate biopsy revealed that the tumor was composed of spindleshaped cells with elongated, plump nuclei, nuclear pleomorphism and brisk mitotic activity [Figure 2]. Its final impression was leiomyosarcoma of the prostate. On further evaluation with computed tomography (CT) scan, it was seen that this large heterogeneous mass was arising from the left posterolateral aspect of the prostate gland with area of infiltration in bladder base, anterior rectal wall and left posterolateral pelvic wall [Figure 3]. The presacral group of lymph nodes were multiple, enlarged [Figure 4] and there were small nodules on the left lung base [Figure 5]. Bone scan was normal. On radiotherapy
Jitendra Pratap Singh1, Debashis Chakraborty2, Malay Kumar Bera1, Dilipkumar Pal1 Departments of Urology1, Pathology2, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India For correspondence: Dr. JitendraPratap Singh, Hno.166-b, Shakti Nagar, Faizabad Road, Lucknow, Uttar Pradesh, India. E-mail:jpims99@ gmail.com
Access this article online Website: www.cancerjournal.net DOI: 10.4103/0973-1482.126482 PMID: 24518734 Quick Response Code:
Figure 1: Transrectal ultrasonogram showing hypo-echoic lesion in prostate
Figure 2: Histopathology (H2EX40) showing spindled and pleomorphic nuclei, and bizarre multipolar mitotic figure
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
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Singh, et al.: A rare, aggressive tumor
Figure 3: CT scan pelvis showing involvement of adjacent organ and lymph node
Figure 4: CT scan pelvis showing enlarged presacral lymph nodes
normal limits, which is not surprising given the nonepithelial origin of this entity. Diagnosis is usually accomplished with ultrasoundguided transrectal needle biopsy or transurethral resection in most patients, and rarely by open surgical procedures.[3,6]
DISCUSSION
The majority of leiomyosarcomas are high-grade hypercellular lesions composed of intersecting bundles of eosinophilic spindle-shaped cells with increased mitotic activity and moderate to severe nuclear atypia.[5] Vimentin, smoothmuscle actin and desmin are commonly expressed by this tumor. Cyto keratin and progesterone receptor expression has also been reported in leiomyosarcomas.[5] High-grade leiomyosarcoma typically exhibits prominent necrosis and cystic degeneration. Low-grade leiomyosarcomas, with moderate atypia, scattered mitoses, and a focally infiltrative growth pattern around benign prostate glands, are very rare.[5] Cytogenetic analysis of primary prostatic leiomyosarcomas reveals clonal chromosomal rearrangements involving chromosomes 2, 3, 9, 11, and 19.[7]
Leiomyosarcoma of the prostate is a rare primary malignant neoplasm of prostatic smooth muscle that accounts for less than 0.1% of all prostate malignancies.[3]Leiomyosarcoma is the most common histological type in adults[2] whereas rhabdomyosarcoma is more common in pediatric patients.[4]
Clinical staging is further enhanced by crosssectional pelvic imaging by CT or magnetic resonance imaging (MRI) scan. Since lung is the most common site of metastasis followed by liver and bone, chest CT scan is an important component of the metastatic evaluation of this rare entity.[2,3]
Patients’ presentation is common in the age range from 41 to 78 years with a mean age of 61 years.[5] The lack of early specific symptoms results in more advanced disease at presentation. Individuals most commonly present with signs and symptoms of urinary obstruction as in our case. Additional associated symptoms include perineal pain, hematuria, burning on ejaculation, constipation and weight loss.[2,3] Up to a third of patients have demonstrable metastases at presentation, usually to the lung, and sometimes to the liver.[2,3]
Due to the rarity of prostatic leiomyosarcoma, definite treatment recommendations are yet to be established. Multimodality treatment combinations including surgery, pre- or postoperative radiation therapy and neoadjuvant or adjuvant chemotherapy have been used. [2,3]Radical retropubic prostatectomy is a good curative option for prostateconfined sarcoma. [2] Among the various chemotherapeutic regimens, anthracyclin (doxorubicin or epirubicin)based combination with alkylating agents (cyclophosphamide, ifosamide) and vinca alkaloids (vinblastin or vincristine) or platinumbased combination have been used with mixed results.[2,8,9]
Figure 5: CT scan showing a metastatic nodule in the left lung base
consultation, patient received two cycles of combination chemotherapy (Ifosamide 1600 mg/m2 and Epirubicin at 40 mg/m2 for three days at three-weekly intervals) but he died after one month of the second cycle.
Rectal examination reveals nonspecific enlargement of the prostate. Serum prostate specific antigen is typically within 744
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
Singh, et al.: A rare, aggressive tumor
Overall five-year survival of prostatic leiomyosarcoma is very poor and ranges between 0-60%.[2,3,6,7] It may be relatively better in patients with complete curative resection and low mitotic activity on biopsy.[6]
5.
REFERENCES
8.
1. 2.
3.
4.
Miedler JD, MacLennan GT. Leiomyosarcoma of the prostate. J Urol 2007;178:668. Sexton WJ, Lance RE, Reyes AO, Pisters PW, Tu SM, Pisters LL. Adult prostate sarcoma: The M. D. Anderson Cancer Centre Experience. J Urol2001;166:521-5. Cheville JC, Dundore PA, Nascimento AG, Meneses M, Kleer E,Farrow GM,et al. Leiomyosarcoma of the prostate: Report of 23 cases. Cancer 1995;76:1422-7. Morse, MJ,Whitmore,W.F. Genitourinary sarcomas in adults. In: ShiuMH,editior.Surgical Management of Soft Tissue Sarcomas. Philadelphia: Lea and Febiger; 1989. p. 189-207.
6. 7.
9.
Hansel DE, Herawi M, Montgomery E,Epstein JI. Spindle cell lesions of the adult prostate. Mod Pathol 2007;20:148-58. Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW. Adult urological sarcoma. J Urol 1992;147:1032-6. Ahlering TE, Weintraub P, Skinner DG. Management of adult sarcomas of the bladder and prostate. J Urol 1988;140:1397-9. Suppiah R, Wood L, Elson P, Budd GT.Phase I/II study of docetaxel, ifosfamide, and doxorubicin in advanced, recurrent of metastatic soft tissue sarcoma. Invest New Drugs2006;24:509-14. Serrone L, Zeuli M, Gamucci T, Nardi M,Cognetti F.A phase II study of dose-intense ifosfamide plus epirubicin with hematopoietic growth factors for the treatment of patients with advanced soft tissue sarcomas; a novel sequential schedule.Cancer Chemother Pharmacol 2001;47:206-10.
Cite this article as: Singh JP, Chakraborty D, Bera MK, Pal D. Leiomyosarcoma of prostate: A rare, aggressive tumor. J Can Res Ther 2013;9:743-5. Source of Support: Nil, Conflict of Interest: None declared.
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
745
Copyright of Journal of Cancer Research & Therapeutics is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Leiomyosarcoma of prostate: A rare, aggressive tumor ABSTRACT Leiomyosarcoma of prostate is a rare, aggressive tumor with bizarre presentation at advanced stage. Though definite treatment modalities are not recommended till now, innovative combined multimodality treatment strategies may help to improve the prognosis of patients with this tumor of poor survival rate. Here we report a case of leiomyosarcoma of the prostate which presented with chest metastasis. KEYWORDS: Cancer, leiomyosarcoma, prostate, sarcoma,
INTRODUCTION Sarcoma of the prostate is an extremely rare neoplasm that accounts for less than 0.1% of primary prostate malignancies.[1] Leiomyosarcoma is the most common primary sarcoma of the prostate in adults and constitutes 38-52% of primary prostatic sarcomas.[2] It has an aggressive clinical course.[3] Here we present such a case of leiomyosarcoma of the prostate and a review of the literature regarding the clinical features, diagnostic modalities and therapeutic aspects of this rare entity. CASE HISTORY A 35-year-old male patient presented with complaints of narrow, intermittent urinary stream for three months and progressive perineal pain for 15 days. On examination, the bladder was palpable, genitalia were normal and on digital rectal examination, the
prostate was non tender, lobulated, asymmetrically enlarged with variegated consistency. Urinalysis and blood biochemistry reports were normal except serum prostate specific antigen (P.S.A.)which was slightly higher (5.31 ng/ml). On trans rectal ultrasonogram [T.R.U.S.], prostate volume was 363 cc with an ill-defined, heterogeneous 66×61.1×80.3 mm mass lesion on the left posterolateral aspect of the prostate gland [Figure 1]. Twelve-core TRUSguided prostate biopsy revealed that the tumor was composed of spindleshaped cells with elongated, plump nuclei, nuclear pleomorphism and brisk mitotic activity [Figure 2]. Its final impression was leiomyosarcoma of the prostate. On further evaluation with computed tomography (CT) scan, it was seen that this large heterogeneous mass was arising from the left posterolateral aspect of the prostate gland with area of infiltration in bladder base, anterior rectal wall and left posterolateral pelvic wall [Figure 3]. The presacral group of lymph nodes were multiple, enlarged [Figure 4] and there were small nodules on the left lung base [Figure 5]. Bone scan was normal. On radiotherapy
Jitendra Pratap Singh1, Debashis Chakraborty2, Malay Kumar Bera1, Dilipkumar Pal1 Departments of Urology1, Pathology2, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India For correspondence: Dr. JitendraPratap Singh, Hno.166-b, Shakti Nagar, Faizabad Road, Lucknow, Uttar Pradesh, India. E-mail:jpims99@ gmail.com
Access this article online Website: www.cancerjournal.net DOI: 10.4103/0973-1482.126482 PMID: 24518734 Quick Response Code:
Figure 1: Transrectal ultrasonogram showing hypo-echoic lesion in prostate
Figure 2: Histopathology (H2EX40) showing spindled and pleomorphic nuclei, and bizarre multipolar mitotic figure
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
743
Singh, et al.: A rare, aggressive tumor
Figure 3: CT scan pelvis showing involvement of adjacent organ and lymph node
Figure 4: CT scan pelvis showing enlarged presacral lymph nodes
normal limits, which is not surprising given the nonepithelial origin of this entity. Diagnosis is usually accomplished with ultrasoundguided transrectal needle biopsy or transurethral resection in most patients, and rarely by open surgical procedures.[3,6]
DISCUSSION
The majority of leiomyosarcomas are high-grade hypercellular lesions composed of intersecting bundles of eosinophilic spindle-shaped cells with increased mitotic activity and moderate to severe nuclear atypia.[5] Vimentin, smoothmuscle actin and desmin are commonly expressed by this tumor. Cyto keratin and progesterone receptor expression has also been reported in leiomyosarcomas.[5] High-grade leiomyosarcoma typically exhibits prominent necrosis and cystic degeneration. Low-grade leiomyosarcomas, with moderate atypia, scattered mitoses, and a focally infiltrative growth pattern around benign prostate glands, are very rare.[5] Cytogenetic analysis of primary prostatic leiomyosarcomas reveals clonal chromosomal rearrangements involving chromosomes 2, 3, 9, 11, and 19.[7]
Leiomyosarcoma of the prostate is a rare primary malignant neoplasm of prostatic smooth muscle that accounts for less than 0.1% of all prostate malignancies.[3]Leiomyosarcoma is the most common histological type in adults[2] whereas rhabdomyosarcoma is more common in pediatric patients.[4]
Clinical staging is further enhanced by crosssectional pelvic imaging by CT or magnetic resonance imaging (MRI) scan. Since lung is the most common site of metastasis followed by liver and bone, chest CT scan is an important component of the metastatic evaluation of this rare entity.[2,3]
Patients’ presentation is common in the age range from 41 to 78 years with a mean age of 61 years.[5] The lack of early specific symptoms results in more advanced disease at presentation. Individuals most commonly present with signs and symptoms of urinary obstruction as in our case. Additional associated symptoms include perineal pain, hematuria, burning on ejaculation, constipation and weight loss.[2,3] Up to a third of patients have demonstrable metastases at presentation, usually to the lung, and sometimes to the liver.[2,3]
Due to the rarity of prostatic leiomyosarcoma, definite treatment recommendations are yet to be established. Multimodality treatment combinations including surgery, pre- or postoperative radiation therapy and neoadjuvant or adjuvant chemotherapy have been used. [2,3]Radical retropubic prostatectomy is a good curative option for prostateconfined sarcoma. [2] Among the various chemotherapeutic regimens, anthracyclin (doxorubicin or epirubicin)based combination with alkylating agents (cyclophosphamide, ifosamide) and vinca alkaloids (vinblastin or vincristine) or platinumbased combination have been used with mixed results.[2,8,9]
Figure 5: CT scan showing a metastatic nodule in the left lung base
consultation, patient received two cycles of combination chemotherapy (Ifosamide 1600 mg/m2 and Epirubicin at 40 mg/m2 for three days at three-weekly intervals) but he died after one month of the second cycle.
Rectal examination reveals nonspecific enlargement of the prostate. Serum prostate specific antigen is typically within 744
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
Singh, et al.: A rare, aggressive tumor
Overall five-year survival of prostatic leiomyosarcoma is very poor and ranges between 0-60%.[2,3,6,7] It may be relatively better in patients with complete curative resection and low mitotic activity on biopsy.[6]
5.
REFERENCES
8.
1. 2.
3.
4.
Miedler JD, MacLennan GT. Leiomyosarcoma of the prostate. J Urol 2007;178:668. Sexton WJ, Lance RE, Reyes AO, Pisters PW, Tu SM, Pisters LL. Adult prostate sarcoma: The M. D. Anderson Cancer Centre Experience. J Urol2001;166:521-5. Cheville JC, Dundore PA, Nascimento AG, Meneses M, Kleer E,Farrow GM,et al. Leiomyosarcoma of the prostate: Report of 23 cases. Cancer 1995;76:1422-7. Morse, MJ,Whitmore,W.F. Genitourinary sarcomas in adults. In: ShiuMH,editior.Surgical Management of Soft Tissue Sarcomas. Philadelphia: Lea and Febiger; 1989. p. 189-207.
6. 7.
9.
Hansel DE, Herawi M, Montgomery E,Epstein JI. Spindle cell lesions of the adult prostate. Mod Pathol 2007;20:148-58. Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW. Adult urological sarcoma. J Urol 1992;147:1032-6. Ahlering TE, Weintraub P, Skinner DG. Management of adult sarcomas of the bladder and prostate. J Urol 1988;140:1397-9. Suppiah R, Wood L, Elson P, Budd GT.Phase I/II study of docetaxel, ifosfamide, and doxorubicin in advanced, recurrent of metastatic soft tissue sarcoma. Invest New Drugs2006;24:509-14. Serrone L, Zeuli M, Gamucci T, Nardi M,Cognetti F.A phase II study of dose-intense ifosfamide plus epirubicin with hematopoietic growth factors for the treatment of patients with advanced soft tissue sarcomas; a novel sequential schedule.Cancer Chemother Pharmacol 2001;47:206-10.
Cite this article as: Singh JP, Chakraborty D, Bera MK, Pal D. Leiomyosarcoma of prostate: A rare, aggressive tumor. J Can Res Ther 2013;9:743-5. Source of Support: Nil, Conflict of Interest: None declared.
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
745
Copyright of Journal of Cancer Research & Therapeutics is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
