|Year : 2021 | Volume
| Issue : 4 | Page : 190-193
A rare case of leiomyosarcoma of the prostate: Treatment and review of literature
Gopal Pemmaraju1, Sanjay Sharma2, Anand Parab1, Anuradha Singh1, Shubhangi Barsing1
1 Department of Advanced Centre for Radiation Oncology, Dr. Balabhai Nanavati Hospital, Mumbai, Maharashtra, India
2 Mumbai Cancer Clinic, Mumbai, Maharashtra, India
|Date of Submission||02-Mar-2021|
|Date of Acceptance||12-Mar-2021|
|Date of Web Publication||12-Jul-2021|
Dr. Gopal Pemmaraju
Department of Advanced Centre for Radiation Oncology, Dr. Balabhai Nanavati Hospital, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Leiomyosarcoma is most common in adults and constitutes 38%–52% of primary prostatic sarcomas. Primary prostatic leiomyosarcoma is extremely rare which accounts for 0.1% of all prostatic malignancies. Patients present with signs and symptoms generally encountered with prostatic enlargement. A 41-year-old gentleman presented with complaints of burning micturition and difficulty in passing urine and stools for 1 month. Magnetic resonance imaging of the pelvis showed a 10 cm × 5.6 cm × 5.9 cm heterogeneous mass lesion originating from the prostate gland extending into bilateral seminal vesicles. There is loss of fat planes with bladder, rectum, and rectosigmoid. Transrectal ultrasound-guided biopsy and immunohistochemistry confirmed the diagnosis as leiomyosarcoma of the prostate. Because of the proximity of the prostate with urinary bladder and difficulty in resection, the patient received neoadjuvant stereotactic body radiotherapy (SBRT) to a dose of 30 Gy in 5 fractions alternate day over a period of 2 weeks. The patient underwent cystoscopy, followed by radical prostatectomy 1 month post-SBRT. Histopathological examination of the prostatectomy specimen was suggestive of high-grade spindle cell neoplasm. The margins were focally involved by the tumor. Because of positive margins, the patient underwent adjuvant radiation treatment to postoperative primary tumor bed to a dose of 30 Gy in 15 fractions by conventional fractionation on Halcyon 2.0. The patient tolerated the treatment well with minimal side effects. The patient is symptom-free and disease-free 3 months posttreatment and needs regular and long follow-up.
Keywords: Leiomyosarcoma, prostatectomy, stereotactic body radiotherapy
|How to cite this article:|
Pemmaraju G, Sharma S, Parab A, Singh A, Barsing S. A rare case of leiomyosarcoma of the prostate: Treatment and review of literature. J Radiat Cancer Res 2021;12:190-3
|How to cite this URL:|
Pemmaraju G, Sharma S, Parab A, Singh A, Barsing S. A rare case of leiomyosarcoma of the prostate: Treatment and review of literature. J Radiat Cancer Res [serial online] 2021 [cited 2022 May 16];12:190-3. Available from: https://www.journalrcr.org/text.asp?2021/12/4/190/321221
| Introduction|| |
Sarcoma of the prostate arises from nonepithelial mesenchymal components of the prostatic stroma. Rhabdomyosarcoma is the histological variant commonly encountered in pediatric age group, while leiomyosarcoma is the most common in adults and constitutes 38%–52% of primary prostatic sarcomas., Primary prostatic leiomyosarcoma (PPLS) is extremely rare which accounts for 0.1% of all prostatic malignancies. Sambert first described leiomyosarcoma of the prostate in 1853.
PPLS patients present with signs and symptoms generally encountered with prostatic enlargement such as poor urine flow, increased urinary frequency, urgency, hesitancy, and voiding difficulties. PPLSs can be diagnosed with the help of histopathological examination and immunohistochemistry. Multimodality treatment with surgery, radiation therapy, and chemotherapy has been the treatment of choice. Radiation either as neoadjuvant, adjuvant, or palliative has been proven to give favorable outcomes.
| Case Report|| |
A 41-year-old gentleman with no prior history of radiation or family history presented with complaints of burning micturition and difficulty in passing urine and stools for 1 month. Magnetic resonance imaging (MRI) of the pelvis showed a 10 cm × 5.6 cm × 5.9 cm large heterogeneous signal intensity mass lesion originating from the prostate gland epicentered along its left half with loss of normal prostatic architecture and extending into bilateral seminal vesicles. There is obliteration of the fat planes between the lesion and urinary bladder and also with loss of fat planes with rectum and rectosigmoid [Figure 1]. The above-mentioned findings were confirmed on positron emission tomography-computed tomography (CT) and there was no spread elsewhere.
|Figure 1: Magnetic resonance imaging pelvis (axial) showing lesion in prostate|
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Transrectal ultrasound-guided biopsy from prostatic lesion showed sheets and bundles of moderately pleomorphic spindle cells with eosinophilic cytoplasm, granular chromatin, and invision nucleoli. The tumor cells expressed positivity for smooth muscle actin (SMA) and desmin and were immune-negative for S-100, CD-34, and CD-117, and the diagnosis was confirmed as leiomyosarcoma of the prostate.
Because of the proximity of the prostate with urinary bladder and difficulty in resection, the patient was planned for neoadjuvant stereotactic body radiotherapy (SBRT) after a detailed discussion between onco-uro surgeon and radiation oncologist. The patient received neoadjuvant SBRT on Halcyon 2.0 (M/s. Varian Medical Systems, USA) with daily Kilo Volt Cone beam Computed tomography (KVCBCT) imaging for patient position verification, to the gross tumor with a margin of 5 mm to a dose of 30 Gy in 5 fractions alternate day over a period of 2 weeks after immobilization on a stereotactic pelvic frame and was planned on Eclipse 15.6 Treatment planning system from M/s. Varian Medical Systems, USA [Figure 2],[Figure 3],[Figure 4],[Figure 5]. The patient experienced slight burning micturition 10–15 days post-SBRT but subsided with medications.
|Figure 2: Planning computed tomography scan (axial) showing the 95% dose distribution to gross tumor by stereotactic body radiotherapy|
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|Figure 3: Planning computed tomography scan (sagittal) showing the 95% dose distribution to gross tumor by stereotactic body radiotherapy|
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|Figure 4: Planning computed tomography scan (sagittal) showing the 95% dose distribution to gross tumor by stereotactic body radiotherapy|
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|Figure 5: Planning computed tomography scan (axial) showing 95% dose distribution to postoperative primary tumor bed in adjuvant treatment by volumetric modulated arc therapy|
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The patient underwent cystoscopy, followed by radical prostatectomy 1 month post-SBRT. Cystoscopy showed normal urethra, bilateral ureteric orifice, and free urinary bladder. Histopathological examination of the prostatectomy specimen was suggestive of high-grade spindle cell neoplasm with necrosis within the tumor. There was infiltration of the adipose tissue and seminal vesicles. The margins were focally involved by the tumor.
Because of the positive margins, the patient underwent adjuvant radiation treatment to postoperative primary tumor bed to a dose of 30 Gy in 15 fractions by conventional fractionation on Halcyon 2.0 [Figure 4] and [Table 1].
|Table 1: Coverage and doses to organs at risk with neoadjuvant stereotactic body radiotherapy and adjuvant volumetric modulated arc therapy treatment|
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The patient tolerated the treatment well with minimal side effects. The patient is symptom-free and disease-free 3 months posttreatment and needs regular and long follow-up.
| Discussion|| |
PPLSs are one of the highly aggressive and most common sarcomas of the prostate. It has high propensity of spread through lymphatics and blood vessels, with 25% of the patients presenting with metastasis at diagnosis. Lung is the most common site of metastasis followed by liver, bone, and brain.
Radiological investigations such as ultrasonography, CT scan, and MRI help in identification of PPLSs. CT scan and MRI are useful in assessing the features of the prostatic lesion, its extent, and its relation to the surrounding structure., Metastatic evaluation of the chest, abdomen, and brain should be routinely performed. Histopathologically, PPLSs tend to have spindle cells with enlarged hyperchromatic nuclei and have high mitotic activity. Immunohistochemistry is a useful tool in the diagnosis of PPLSs. They exhibit high positivity for vimentin, CD-44, and SMA; weakly positive for calponin, desmin, and keratin; but negatively stain for S-100, cytokeratin, CD-34, CD-117, and PSA. Molecular cytogenetic studies revealed clonal chromosomal rearrangement in chromosomes 2, 3, 9, 11, and 19.
There are no standard recommendations for the treatment of PPLSs, but multimodality treatment with surgery, radiation therapy, and/or chemotherapy seems to give best results. Radical cystoprostatectomy, radical retropubic prostatectomy, suprapubic prostatectomy, and pelvic exenteration are surgical procedures in practice.,
Radiation treatment plays a key role in a neoadjuvant or adjuvant setting. Patients with bulky disease mostly benefit from neoadjuvant radiation treatment. Adjuvant radiation treatment is highly recommended in cases with positive margins and nodal metastasis.
Kalbasi et al. have demonstrated that a 5-day regimen of neoadjuvant radiation is effective and safe as compared to conventional 5-week regimen in the treatment of soft tissue sarcomas.
Hypofractionation in prostate cancer as evident from many trials provides better tumor control and the results are comparable to normal fractionation owing to low (alpha/beta) ratio (1.4–3 Gy) with an increased therapeutic rate with reduced toxicities.,
Cihan showed the role and importance of SBRT as a neoadjuvant treatment for prostatic malignancies within a dose range of 30–36.5 Gy with minimal toxicities. The advent of noninvasive immobilization devices and target imaging before and during treatment made the treatment with SBRT more feasible.
Chemotherapy with anthracyclines, alkylating agents, vinca alkaloids, and platinum-based agents have been used in the treatment of PPLSs as neoadjuvant and adjuvant or as palliative with mixed results.,
The overall clinical outcome of PPLS is very poor with a median survival of 17 months. Metastasis at presentation and the presence of positive margins are the prognostic factors.
| Conclusion|| |
PPLS is a rare, aggressive tumor mostly seen in adult age group but with high malignant potential. Multidisciplinary approach with radiation therapy, surgery, and/or chemotherapy gives good results. SBRT is highly effective in the neoadjuvant setting with minimal side effects. Postoperative positive margins carry high prognostic significance and should be dealt with accordingly with radiation therapy or chemotherapy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]