|Year : 2017 | Volume
| Issue : 2 | Page : 92-94
Prostatic leiomyosarcoma: A rare and aggressive tumor
Noora Saeed, Sayeedul Hasan Arif, Saima Khan, Suhail Ur Rehman
Department of Pathology, JNMC, AMU, Aligarh, Uttar Pradesh, India
|Date of Web Publication||26-Jul-2017|
Department of Pathology, JNMC, AMU, Aligarh, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Primary prostatic sarcomas of adults are very rare with the most common type being prostatic leiomyosarcoma. They occur in men in their late age with the clinical features similar to that of prostatic carcinoma, so diagnosis can only be made after the resection of prostate and histopathological examination. Histopathologically, they are mitotically active tumors composed of interlacing fascicles and bundles of smooth muscle with areas of necrosis. These tumors have an aggressive clinical course and bad prognosis. Here, we present a case of 58 years male who presented with urinary complaints and lower abdominal pain. After microscopic and immunohistochemical examination of the specimen following transurethral resection of prostate, a final diagnosis of prostatic leiomyosarcoma was made.
Keywords: Histopathology, leiomyosarcoma, prostate, sarcoma
|How to cite this article:|
Saeed N, Arif SH, Khan S, Rehman SU. Prostatic leiomyosarcoma: A rare and aggressive tumor. Saudi Surg J 2017;5:92-4
| Introduction|| |
Leiomyosarcoma of prostate is an extremely rare neoplasm which accounts for <0.1% of primary prostate malignancies. It is the most common primary sarcoma of the prostate in adults and comprises 38%–52% of primary prostatic sarcomas. It has a more aggressive clinical course as compared to carcinoma prostate. We hereby present a case of prostatic leiomyosarcoma and a review of the literature regarding the clinical features, diagnostic modalities, and therapeutic aspects of this rare entity.
| Case Report|| |
A 58-year-old male presented in the surgery outpatient department with chief complaints of difficulty in micturition, intermittent urinary flow, and diffuse lower abdominal pain for 6 months. The patient also gave history of weight loss and loss of appetite for 1 year. On abdominal examination, there was tenderness in lower abdomen while on digital rectal examination, the prostate was nontender, asymmetrically enlarged, lobulated, and firm in consistency. Rest of the physical examination was unremarkable. His routine blood investigations were within normal limits. Serum prostate-specific antigen (PSA) at presentation was 0.675 ng/ml. On transrectal ultrasonogram, prostate was enlarged (4.9 cm × 4.9 cm × 4 cm = 49 g) with normal parenchymal echotexture along with an ill-defined, hypoechoic lesion (4 cm × 3.6 cm × 3 cm = 24 ml) in the left lobe of prostate (central and transitional zone) suggestive of neoplastic nodule [Figure 1]a. The patient then underwent transurethral resection of prostate. Grossly, multiple firm tissue pieces aggregate measuring 3 cm × 2.5 cm were received in the histopathology laboratory, Department of Pathology. Microscopic examination revealed interlacing fascicles and bundles of atypical spindle-shaped cells with elongated mild to moderately pleomorphic nuclei and focal areas of increased mitosis. Few foci of necrosis were also seen. Occasional glands with corpora amylacea were seen lined by uniform, bland-looking epithelium. The final impression of poorly differentiated malignancy favoring sarcoma was made with the differentials being: leiomyosarcoma, fibrosarcoma, and carcinosarcoma [Figure 1]b. An immunohistochemical panel involving smooth muscle actin (SMA), desmin, and cytokeratin (CK) was advised. Immunohistochemistry showed cytoplasmic positivity of tumor cells for SMA and desmin while negative, they were negative for CK [Figure 1]c; however, the glandular component in the tumor was positive for pan CK [Figure 1]d confirming the diagnosis of low-grade leiomyosarcoma. The patient was referred to radiotherapy department where he underwent chemotherapy with ifosfamide, dacarbazine, and farmurobicin. After three courses of chemotherapy, the patient then received radiotherapy (60 Gy in 30 fraction, 5/week). The patient was on regular follow-up for 6 months and was clinically asymptomatic.
|Figure 1: (a) Ultrasound of prostate showing enlarged size with normal parenchymal echotexture along with an ill-defined, hypoechoic lesion in the left lobe of prostate suggesting of neoplastic nodule. (b) Histopathological section showing interlacing fascicles and bundles of atypical spindle-shaped cells with elongated mild to moderately pleomorphic nuclei. Occasional glands with corpora amylacea lined by uniform bland looking epithelium are also seen. (c) Immunohistochemical staining showing cytoplasmic positivity of tumor cells for smooth muscle actin. (d) Immunohistochemistry showed cytoplasmic positivity of the glandular epithelial cells for cytokeratin|
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| Discussion|| |
Leiomyosarcoma of prostate accounts for <0.1% of prostate malignancies. It is the most common type of sarcomas in prostate gland among the adults, while the sarcomas common in pediatric age group are rhabdomyosarcoma.,
Patients with leiomyosarcoma of the prostate usually present in the age ranging from 41 to 78 years, with a mean age of 60 years. They have signs and symptoms similar to that of patients with carcinoma of prostate, i.e., urinary obstruction, lower abdominal pain, hematuria, burning during micturition, constipation and weight loss., Similarly, our patient presented with difficulty in urination with intermittent flow and lower abdominal pain. Up to 25% of patients present with metastatic diseases at the time of diagnosis, with common sites of metastases being lungs, bones and rarely liver.
On digital rectal examination, there is nonspecific enlargement of the prostate. PSA is a serum marker which is seen to be elevated tumors of epithelial origin such as benign prostatic hyperplasia and carcinoma prostate, however since leiomyosarcoma is a mesenchymal tumor, PSA is typically within normal limits. Similarly, our patient also had normal PSA levels. Diagnosis is usually made by ultrasound guided transrectal needle biopsy or transurethral resection and less commonly by transperineal biopsy or computed tomography (CT)-guided biopsy. Very rarely there is need of open surgical procedures such as suprapubic prostatectomy.
On microscopic examination, leiomyosarcomas are composed of intersecting bundles of eosinophilic spindle-shaped cells with moderate to severe nuclear atypia and increased mitotic activity. High-grade leiomyosarcomas show prominent areas of necrosis and cystic degeneration. Leiomyosarcomas can very rarely be of low-grade type, which exhibits moderate atypia, scattered mitoses, and a focally infiltrative growth pattern around benign prostate glands. Our case was fitting in the second rarer category of a low-grade leiomyosarcoma, which showed infiltration of the malignant sarcomatous element within the benign prostatic glands.
The spindle cells of the tumor usually express vimentin, SMA, and desmin and are negative for S-100 and CD-117. Some leiomyosarcomas express CK and progesterone.,
On cytogenetic analysis prostatic leiomyosarcomas usually show clonal chromosomal rearrangements involving chromosomes 2, 3, 9, 11, and 19.
For clinical staging as well as to know the extent of disease, radiological examination of the pelvis by CT or magnetic resonance imaging scan is required. Among the metastatic sites, since lung is the most predisposed site of metastasis, chest CT scan is an important component of the metastatic evaluation of this rare entity., Being a rare entity, the definite treatment recommendations for prostatic leiomyosarcoma are yet to be established. However, the multimodality treatment combinations include surgery, pre- or post-operative radiation therapy and neoadjuvant or adjuvant chemotherapy.,
Radical retropubic prostatectomy is a good curative option for sarcoma confined to prostate.
Among the various chemotherapeutic regimens, anthracycline (doxorubicin or epirubicin) based combination with alkylating agents (cyclophosphamide, ifosfamide) and vinca alkaloids (vinblastine or vincristine) or platinum-based combination has been used with mixed results.,, Overall 5-year survival of prostatic leiomyosarcoma is very poor, ranging from 0% to 60%., It is relatively better in patients with complete curative resection and low mitotic activity on biopsy.
| Conclusion|| |
Leiomyosarcoma of the prostate is a rare and aggressive neoplasm that usually presents with symptoms similar to carcinoma prostate, so diagnosis merely on the clinical ground is difficult. For proper and complete management, a multidisciplinary approach is required which includes urological, radiological, and medical oncology consultations.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
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 Center Experience. J Urol 2001;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 WF. Genitourinary sarcomas in adults. In: Shiu MH, editor. Surgical Management of Soft Tissue Sarcomas. Philadelphia: Lea and Febiger; 1989. p. 189-207.
Hansel DE, Herawi M, Montgomery E, Epstein JI. Spindle cell lesions of the adult prostate. Mod Pathol 2007;20:148-58.
Singh JP, Chakraborty D, Bera MK, Pal D. Leiomyosarcoma of prostate: A rare, aggressive tumor. J Cancer Res Ther 2013;9:743-5.
Raj DH, Dash PK, Mohanty J, Sarangi PK. Leiomyosarcoma of the prostate-an unexpected histopathological outcome. BMJ Case Rep 2016;2016. pii: Bcr2016215594.
Kelley TW, Borden EC, Goldblum JR. Estrogen and progesterone receptor expression in uterine and extrauterine leiomyosarcomas: An immunohistochemical study. Appl Immunohistochem Mol Morphol 2004;12:338-41.
Limon J, Dal Cin P, Sandberg AA. Cytogenetic findings in a primary leiomyosarcoma of the prostate. Cancer Genet Cytogenet 1986;22:159-67.
Suppiah R, Wood L, Elson P, Budd GT. Phase I/II study of docetaxel, ifosfamide, and doxorubicin in advanced, recurrent, or metastatic soft tissue sarcoma (STS). Invest New Drugs 2006;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.
Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW, et al.
Adult urological sarcoma. J Urol 1992;147:1032-6.