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Prostate leiomyosarcoma: A rare tumor

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Abstract

Leiomyosarcoma is a rare neoplasm with a poor survival rate. Between June 2018 to January 2019 we treated a rare case of Prostate Leiomyosarcoma initially metastatic to the lung and the liver with multiple pelviclymph nodes. Five cycles of chemotherapy and palliative radiotherapy have been delivered with a mean follow up of 1 year .Chestabdominal-pelvic CT scan with MRI and bone scan are necessarily to assess clinical staging. The objective of this study is to review our experience in the management of Prostate Leiomyosarcoma at the Casablanca Cancer Center of Mohammed VI University of Health Sciences with a literature review of this rare neoplasm.
Corresponding author: I Benali
Mohammed VI University of Health Sciences (UM6SS), Casablanca, Morocco.
Copyright © 2021 Author(s) retain the copyright of this article. This article is published under the terms of the Creative Commons Attribution Liscense 4.0.
Prostate leiomyosarcoma: A rare tumor
I Benali 1, *, M Allaoui 2 and A Mejdoubi 3
1 Mohammed VI University of Health Sciences (UM6SS), Casablanca, Morocco.
1 Department of Radiotherapy, Casablanca Cancer Center, Cheikh Khalifa International Hospital, Casablanca, Morocco.
2 Department of Pathological Anatomy, Cheikh Khalifa International Hospital, Casablanca, Morocco.
3 Department of medical Oncology, Casablanca Cancer Center, Cheikh Khalifa International Hospital, Casablanca, Morocco.
GSC Advanced Research and Reviews, 2021, 09(02), 103107
Publication history: Received on 02 October 2021; revised on 15 November 2021; accepted on 17 November 2021
Article DOI: https://doi.org/10.30574/gscarr.2021.9.2.0257
Abstract
Leiomyosarcoma is a rare neoplasm with a poor survival rate. Between June 2018 to January 2019 we treated a rare
case of Prostate Leiomyosarcoma initially metastatic to the lung and the liver with multiple pelviclymph nodes. Five
cycles of chemotherapy and palliative radiotherapy have been delivered with a mean follow up of 1 year
.Chestabdominal-pelvic CT scan with MRI and bone scan are necessarily to assess clinical staging. The objective of this
study is to review our experience in the management of Prostate Leiomyosarcoma at the Casablanca Cancer Center of
Mohammed VI University of Health Sciences with a literature review of this rare neoplasm.
Keywords: Prostate leiomyosarcoma; Multimodal treatment; Genitourinary sarcoma; Onco-Urology; Case Report
1. Introduction
Prostatic leiomyosarcoma is a rare primary tumor in adults representing 38% to 52% of primary prostate sarcomas
that accounts for less than 0, 1% of prostate malignancies. Its diagnosis is generally made at an advanced stage due to
clinical and radiological none specificity, the prognosis remains poor. Chestabdominal-pelvic CT scan with MRI and bone
scan are necessarily to assess clinical staging .The aim of this work is to present a rare case of Prostatic Leiomyosarcoma
treated with chemotherapy and decompressive analgesic radiotherapy with a literature review regarding the clinical
features, diagnostic modalities and therapeutic aspects of this rare entity.
2. Patient and Case report
A 40-year-old male patient, without medical or surgical history of interest. He has presented since March 2017 rectal
pain tension and burning with urination. Macro hematuria wasn’t detected; he hadn't a family history of genitourinary
cancer. On examination; the bladder was palpable and on digital rectal examination the prostate was asymmetrically
enlarged with nodular variegated consistency. Urinary analysis and blood biochemistry reports were normal including
prostate specific antigen (PSA = 0.46ng). On trans-rectal ultrasonogram [TRUS], prostate volume was 430 ccs with an
ill-defined, heterogeneous, with an aspect of varicocele on the left and testicular atrophy on the right. The prostate
biopsy and Immunohistochemical analysis (figure 4- figure 5) both revealed that the tumor was “Leiomyosarcoma of
the Prostate” . On further evaluation with Chestabdominal-pelvic CT scan ( figure 1- figure 2- figure 3 ) it was seen that
this large heterogeneous mass of the prostate presented irregular contours with area of infiltration of the bladder base
and anterior rectal wall. Multiple presacral lymph nodes and pathological lesions have been observed in the liver and
lungs. Bone scan was normal. After the multidisciplinary meeting the patient received palliative radiotherapy with a
total dose of 30Gy on 10 fractions five days per a week, on oncology hospitalization he had undergone 5 cycles of
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combination chemotherapy (Ifosfamide 1500 mg/m2 and doxorubicin at 50 mg/m2 for three days at three-weekly
intervals) but he died 2 months after the last cycle. The patient agreed to the conditions of this study. No data were used
to support this study.
2.1. What is already known on this topic
Leiomyosarcoma of the prostate is an uncommon and highly aggressive tumor that accounts for less than 0.1% of
primary prostate malignancies with median survival estimated in 17 months.
2.2. What this study adds
The goal of this study was to support the multidisciplinary approach (urology, radiation therapy and chemotherapy)
for appropriate management of this devastating malignancy.
Figure 1 Chestabdominal-pelvic CT Scan before treatment (axial plan)
Figure 2 Chestabdominal-pelvic CT Scan before treatment (sagittal plan)
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Figure 3 Chestabdominal-pelvic CT Scan after treatment (axial plan)
Figure 4 Strong expression of Desmine in immunohistochemical analysis
Figure 5 Partially necrotic fusocellular tumor proliferation
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3. Discussion
Leiomyosarcoma of the Prostate is a rare primary malignant tumor 1, histological types can be divided into prostate
leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma and spindle cell sarcoma 2 with an average age of 58 years. The
most frequent reason for consultation is an urinary obstructive syndrome including: hematuria, burning with urination,
perineal pain and weight loss as observed in our case(1-2). Usually, most of the patients with a prostate sarcoma were
diagnosed at an advanced stage, in fact our patient has demonstrable metastases to the lung and to the liver as well
these results in the lack of early specific clinical symptoms which impacts overall survival rate (2-3).The dosage of
prostatic markers (PSA, PAD) is typically normal because of histological type (not epidermoid) (4-5).The pathological
diagnosis, supplemented by an immunohistochemical study is essential for the classification of these tumors
(1).Recommendations regarding the management of prostate sarcomas weren’t sufficient and contradictory. Sen and
associate evaluated the results of 13 patients treated at the Mayo Clinic between 1970 and 1985 (6-7) , 10 patients were
treated for the cure: 3 with leiomyosarcoma, 2 with rhabdo-myosarcoma and 5 with carcinosarcoma. No distinction
was made regarding the histological subtypes. All 3 patients who were treated with partial cystectomy had recurrence.
Neither report systematically included chemotherapy in the management. In an attempt to improve survival for patients
with leiomyosarcoma of the bladder or prostate, adjunctive chemotherapy and radiotherapy were combined with
surgical excision. However, the use of adjuvant chemotherapy increased cystic necrosis without actual tumor response.
Post-operative radiotherapy was reserved for residual disease. In our experience leiomyosarcoma tends to be locally
advanced before metastasizing. The mean follow up was approximatively 1 year than the patient died. No clear-cut
conclusions can be drawn with a tumor this rare. Palliative radiotherapy was indicated to reduce pain symptoms not
for curative intention because the tumor was very extensive locally. It is interesting that the clinical response was
apparent after chemotherapy making us believing that probably the combination of chemotherapy and an operation
offer the best results for these aggressive tumors. The CT scan and MRI make it possible to assess the local and general
extension of the tumor. The radiological appearance of leiomyosarcoma is not specific. It is generally a large
heterogeneous tumor which can raise the bladder floor or invade it. Endorectal ultrasound is superior in the assessment
of local extension. The treatment of this tumor has not yet been codified, it includes surgery, pre or postoperative
radiotherapy and neoadjuvant or adjuvant chemotherapy with anthracyclines, alkylating agents and/or alkaloids as
appropriate. It depends on age, general condition, tumor volume, grade of malignancy and extension workup (8). The
prognosis of prostatic leiomyosarcomas is often unfavorable. Survival is very variable depending on the series. It is on
average less than 10% in 5 years.
4. Conclusion
Adult prostatic leiomyosarcoma is a rare tumor, often metastatic at diagnosis, with normal PSA levels. CT and especially
MRI play an important role in the assessment of extension and post-treatment follow-up, but only the Prostate biopsy
supplemented by immunohistochemical analysis can confirm the diagnosis. Their therapeutic management is not
currently codified, and their prognosis remains very poor and can only be improved with a multi-disciplinary approach
and early diagnosis, making it possible to perform complete radical surgery, the only effective therapy.
Compliance with ethical standards
Acknowledgments
All the authors contributed in this study.
Funding statement
The authors received no financial support for the research authorship and or publication of this article.
Disclosure of conflict of interest
The authors declare no conflict of interest.
Statement of informed consent
Written informed consent was obtained from the patient for publication of this case report and accompanying images.
A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.
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[8] M Cruz Ruiz, J Calleja Escudero, JI Peral Martínez, JR Cortiñas González J Rivera Ferro.
... Primary prostate leiomyosarcoma is the most common sarcoma affecting the prostate, although it is generally a rare tumor. 3 Leiomyosarcoma of prostate was described by Gaudin et al 4 in 1998. World Health Organization classifies this tumor as a distinct spindle cell neoplasm. ...
... 5,6 The etiology of primary prostate leiomyosarcoma is unknown or poorly understood. 3 Histological types can be divided into prostate leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma and spindle cell sarcoma. Benali et al 3 ...
... 3 Histological types can be divided into prostate leiomyosarcoma, rhabdomyosarcoma, fibrosarcoma and spindle cell sarcoma. Benali et al 3 ...
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Sarcoma of the prostate: a single institutional re-view
  • N L Janet
  • A W May
  • R S Akins
Janet NL, May AW, Akins RS. Sarcoma of the prostate: a single institutional re-view. Am J ClinOncol.
Genitourinary sarcomas in adults
  • M J Morse
  • W F Whitmore
Morse MJ, Whitmore WF. Genitourinary sarcomas in adults. In: Surgical Management of Soft Tissue Sarcomas. Edited by M. H. Shiu and M. F. Brennan. Philadelphia: Lea and Febiger. 1989; 189-207.