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Review | DOI: https://doi.org/10.31579/2690-8794/228
Anthony kodzo-grey venyo. Retired urologist; medical examiner member or the royal college of pathologists of london. United Kingdom.
*Corresponding Author: Anthony kodzo-grey venyo. Retired urologist; medical examiner member or the royal college of pathologists of london. United Kingdom.
Citation: Anthony kodzo-grey venyo, (2024), Prostatic stromal sarcoma: review and update, Clinical Medical Reviews and Reports, 6(7); DOI:10.31579/2690-8794/228
Copyright: © 2024, Anthony kodzo-grey venyo. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Received: 29 August 2024 | Accepted: 05 September 2024 | Published: 12 September 2024
Keywords: prostatic stromal sarcoma; stromal sarcoma of the prostate gland; prostate biopsy; ultrasound-scan-guided prostate biopsy; serum prostate specific antigen; urinary retention
It has been iterated that the prostate stromal neoplasm originates from mesenchymal components of the prostate gland. Prostatic stromal sarcoma in human beings in 1998, was first classified into 2 types by Gaudin and associates including prostatic stromal sarcoma (PSS) and stromal tumours of uncertain malignancy potential. It has been suggested that PSS was especially rare which only accounts for less than 0.1% of primary prostate malignancies in adults. A number of authors had reported that the common manifestation of PSS was urinary retention and the serum prostatic-specific antigen (PSA) level had often remained within normal range. Nevertheless, with the low incidence of prostatic stromal sarcoma that had been reported, clinical information related to the rare neoplasm has remained not clear. Primary prostate sarcomas are rare, with one study estimating it to comprise of just 0.7% of prostatic malignancies. Prostatic stromal sarcoma typically emanates from specialized hormone-dependant mesenchymal cells of the prostatic stroma. A diverse histopathology pattern of prostatic stromal sarcoma in human beings had been reported in the literature as case reports or few case series, often closely related to stromal tumours of uncertain malignant potential (STUMP). In view of the rarity of the neoplasm, it would be envisaged that majority of clinicians all over the world would not have encountered a case of prostatic stromal sarcoma before. In order to provide updated information of primary prostatic sarcoma of the prostate gland afflicting human beings, it is important for all clinicians who encounter cases of primary prostatic sarcoma to report their cases with long-term follow-up outcome results so that new lessons would be learnt about the tumour.
It had been iterated that phyllodes tumour of the prostate gland, which was first described by Cox and Dawson in 1960, is an uncommon neoplasm. [1-2] It had been stated that phyllodes tumour of the prostate gland had been termed by various other names, including: cystic adenoma of the prostate, cyst-adeno-leio-myo-fibroma, cystic epithelial-stromal tumour, phyllodes type of atypical hyperplasia and cysto-sarcoma phyllodes. It had furthermore been iterated that the tumour is histologically similar to phyllodes tumour of the breast with a distinctive biphasic pattern of hyperplastic epithelium-lined cysts, leaf-like intraluminal epithelial-lined stromal projections, compressed and elongated silt-like epithelial-lined spaces, and variable cellular spindle cell stroma, at times with subepithelial condensation with or without atypia. The epithelial lining of the tumour is stated to be typically bland with a secretory cell layer and a basal cell layer. [1] [3]
It has in addition been iterated that the clinical significance and management of prostatic phyllodes tumour are not certain, as well as that the clinical course of the tumour had not been well defined due to the fact that the most reported cases had little or no follow up assessment reported. [1] It had also been stated that majority of reports of the tumour had described the phyllodes tumour as a variant of hyperplasia (phyllodes-type hyperplasia), whereas other cases had been described as malignant phyllodes tumours (cysto-sarcoma phyllodes) based upon the presence of many mitotic figures and prominent cytological atypia. [1] It had been pointed out that the precise criteria for separating benign and malignant stromal tumours of the prostate gland had not yet been clearly defined. In view of the rarity of prostatic stromal sarcoma in human beings that had been so far reported, it would be envisaged that majority of clinicians would not be familiar with the manifestation, diagnostic features, treatment and outcome of prostatic stromal sarcoma. The ensuing article on prostatic stromal sarcoma in human beings has been divided into two parts: (A): Overview, and (B) Miscellaneous narrations and discussions from some case reports, case series and studies related to prostatic stromal sarcoma.
To review and update the literature on prostatic stromal sarcoma.
Method
Internet data bases were searched, the search words that were used included: Prostatic stromal sarcoma; prostate stromal sarcoma; and stromal sarcoma of the prostate gland. Thirty-five (35) references were identified which were used to write the article on prostatic stromal sarcoma which has been divided into two parts: (A): Overview, and (B) Miscellaneous narrations and discussions from some case reports, case series and studies related to prostatic stromal sarcoma.
Results
[A] Overview
Definition / general
The ensuing summations had been made regarding general aspects of prostatic stromal sarcoma: [4]
Essential features
The essential features of prostatic stromal sarcoma had been summated as follows: [4]
Terminology
It has been iterated that the ensuing terminologies had tended to be utilised for prostatic stromal sarcoma of the prostate gland: [4]
Epidemiology
The epidemiology of prostatic stromal sarcoma had been summated as follows: [4]
Gaudin et al. [7] made the ensuing iteration:
Gaudin et al. [7] reviewed the clinicopathological features of 22 cases and had studied the immunohistochemical profile of 9 cases. They summarised the results as follows:
Gaudin et al. [7] made the ensuing conclusions:
Sites
Aetiology
Clinical features
The clinical manifestations of prostatic stromal sarcoma had been summated as follows: [4]
Diagnosis
Laboratory test
Radiology examination description
Prognostic factors
The factors for prognostication of prostatic stromal sarcoma had been summated as follows: [4]
Treatment
Microscopic (histologic) description
The ensuing summations had been made regarding the microscopy pathology examination features of prostatic stromal sarcoma. [4]
Positive stains
It has been iterated that immunohistochemistry staining studies of specimens of prostatic stromal sarcomas demonstrate tumour cells exhibit positive staining for tumour markers as follows: [4]
Negative stains
It has been iterated that immunohistochemistry staining studies of specimens of prostatic stromal sarcomas demonstrate tumour cells exhibit negative staining for tumour markers as follows: [4]
Molecular / cytogenetics description
The ensuing summations had been made regarding the molecular / cytogenetics features of prostatic stromal sarcomas. [4]
Differential diagnoses
The differential diagnoses of prostatic stromal sarcoma had been summated to include the ensuing: [4]
Bostwick and Egevad [6] stated the ensuing:
Gaudin et al. [7] made the ensuing iteration:
Gaudin et al. [7] reviewed the clinicopathological features of 22 cases and had studied the immunohistochemical profile of 9 cases. They summarised the results as follows:
Gaudin et al. [7] made the ensuing conclusions:
Shen et al. [8] stated the ensuing:
Shen et al. [8] included twenty-three patients who were diagnosed as being afflicted with STUMP from 2008 to 2019. They collected the clinicopathological and follow-up information of the patients. Shen et al. [8] divided in the subgroup analysis, the patients into a pure STUMP group (N = 18) and a mixed STUMP (STUMP coexisting with PSS) group (N = 5). Student's t test was used to compare the 2 groups. Shen et al. [8] summarised the results as follows:
Shen et al. [8] made the ensuing conclusions:
Herawi et al. [9] made the ensuing iterations:
Herawi et al. [9] studied 50 cases of STUMP and stromal sarcoma with regard to their clinical manifestation and follow-up assessments. They summarised the results as follows:
Herawi et al. [9] made the ensuing conclusions:
Fabio et al. [10] reported a 79-year-old man, who had manifested with dysuria and increased serum prostate-specific antigen level (21 ng/mL). Hed had MRI scan of the prostate gland which demonstrated bulky prostate enlargement but which was inconclusive in demonstrating neoplastic lesions. However, because of high clinical suspicion for neoplasm, trans-rectal biopsy of the prostate gland was undertaken and pathology examination of the prostate biopsy specimen showed features of stromal tumour of uncertain malignant potential mixed with foci of low-grade primitive prostate stromal sarcoma. 18F-FDG PET/CT scan was undertaken which demonstrated high FDG uptake that was consistent with neoplasm within the lower part of the hypertrophic prostate gland and focal areas of elevated FDG uptake, which was consistent with metastases within his spine, ribs, and femur.
Bostwick et al. [11] stated that phyllodes tumour of the prostate is an uncommon neoplasm of uncertain malignant potential. They studied a large series of phyllodes tumours in order to define the combination of histopathology examination features that are most useful for the prediction of patient outcome. Bostwick et al. [] obtained a total of 23 cases from their collective files from 1973 to 2002, and they evaluated many clinical and pathological features. They undertook a review of the reported cases of phyllodes tumour of the prostate. Bostwick et al. [3] summarised the results as follows:
Bostwick et al. [11] made the ensuing conclusions:
Wang et al. [12] made the ensuing iterations:
Wang et al. [12] undertook a study to investigate both amplification of the epidermal growth factor receptor (EGFR) gene by fluorescence in situ hybridization and the overexpression of EGFR, Her-2/neu, CD117 (c-kit), and androgen receptor by immunohistochemical staining in a series of 11 phyllodes tumours of the prostate. Wang et al. [12] reported the results as follows:
Wang et al. [12] made the ensuing conclusions:
Fabio et al. [13] reported a 79-year-old man, who had manifested with dysuria and increased serum prostate-specific antigen level (21 ng/mL). He had MRI scan of his prostate gland which demonstrated a bulky prostate enlargement but the MRI scan was inconclusive in demonstrating tumour lesions. However, due to his high clinical suspicion for prostatic tumour, trans-rectal ultrasound scan biopsy of the prostate was undertaken and pathology examination of the biopsy specimen revealed features that confirmed the diagnosis of stromal tumour of uncertain malignant potential mixed with foci of low-grade primitive prostate stromal sarcoma. He had 18F-FDG PET/CT scan which demonstrated high FDG uptake that was consistent with neoplasm within the lower part of the hypertrophic prostate gland and focal areas of elevated FDG uptake, that were consistent with metastases within his spine, ribs, and femur.
Mao et al. [14] reported an uncommon case of prostatic stromal sarcoma (PSS), which was treated with a robot-assisted laparoscopic radical prostatectomy (RLRP). Mao et al. [14] reported a 32-year-old man, who had manifested with obstructive voiding symptoms which had persisted over the preceding 2 years. He had a computed tomography scan of his pelvis which demonstrated an 8-cm prostatic mass protruding into the bladder. A trans-perineal ultrasound-guided prostate biopsy was undertaken and pathology examination of the biopsy specimen demonstrated features based upon which a diagnosis of PSS was made. An RLRP was undertaken, and neither chemotherapy nor radiotherapy were administered prior to or pursuant to the surgery. No recurrence of the tumour was demonstrated at 6 months post-surgery. Mao et al. [14] concluded that:
Pan and Epstein [16] stated the ensuing:
Pan and Epstein [16] undertook a study to detect the chromosomal imbalances in stromal sarcoma and STUMP by using array comparative genomic hybridization (aCGH). The study had consisted of two cases of stromal nodule, eight cases of STUMP (three degenerative atypia type, three myxoid type, one hypercellular type, and one phyllodes type), and four cases of stromal sarcoma, including a distant metastasis developed metachronously after a primary stromal sarcoma of the prostate. Pan and Epstein [16] extracted DNA from the representative paraffin-embedded formalin-fixed specimens and was submitted for aCGH. All stromal sarcomas and seven STUMPs revealed chromosomal aberrations. Pan and Epstein [16] summarised the results as follows:
Pan and Epstein [16] concluded that:
From a molecular genetic perspective, the recurrent chromosomal alterations support the concept of specialized stromal tumours of the prostate as a distinctive tumour entity.
Wickramasinghe et al. [17] stated the following:
Wickramasinghe et al. [17] reported the case of a 42 -year-old man, who had developed a subcutaneous metastatic deposit of a prostatic stromal cell sarcoma 5 years after he had undergone radical prostatectomy. Additional staging with CT- and PET-scan had demonstrated lymph node involvement in his neck and left axilla. A core biopsy of the skin lesion was undertaken, of which the histology had demonstrated a low-grade spindle cell tumour that was morphologically identical to a previously diagnosed prostatic stromal sarcoma. Wickramasinghe et al. [17] made the ensuing educative discussions:
Wickramasinghe et al. [17] made the ensuing conclusions:
Choi et al. [18] iterated the following:
Cavaliere et al. [19] stated the following:
Cavaliere et al. [19] reported the first case of PSS occurring in an adolescent. There was evidence of a good response to chemotherapy including ifosfamide, doxorubicin, vincristine and actinomycin-D, even though the final outcome was dismal. They iterated that their review of the English literature had revealed 14 additional patients with PSS, who were treated with chemotherapy: tumour shrinkage was reported in 4 of the 6 evaluable patients. Cavaliere et al. [19] concluded that:
Xu et al. [20] made the ensuing iterations:
Xu et al. [20] undertook a study, which had aimed to investigate the clinicopathological features which could be utilised to differentiate malignant solitary fibrous tumours (mSFTs) occurring within the prostate gland from prostatic stromal tumours. Xu et al. [20] identified a total of 15 patients who had mesenchymal tumours of the prostate in Nanjing Drum Tower Hospital from 2009 to 2019, including 3 mSFTs, 9 stromal tumours of uncertain malignant potential (STUMPs), and 3 prostatic stromal sarcomas (PSSs). Xu et al. [20] undertook immunohistochemistry staining studies for signal transducer and activator of transcription 6 (STAT6), aldehyde dehydrogenase 1 (ALDH1), CD34, desmin, smooth muscle actin (SMA), progesterone receptor (PR), CD117, and cytokeratin (CK) on representative sections from each tumour, and they analysed the clinical features, histology, and immunophenotype of these three groups were. Xu et al. [20] summarised the results as follows:
Xu et al. [20] concluded that a limited panel of STAT6, PR, and Ki-67 may be useful in differentiating between mSFTs, STUMPs, and PSSs.
Accosta et al. [21] made the ensuing iterations:
Accosta et al. [21] undertook a study, in which they utilised next-generation DNA and RNA sequencing to profile 25 primary prostatic mesenchymal neoplasms of possible specialized prostatic stromal origin, including cases originally diagnosed as PSS (11) and STUMP (14). Accosta et al. [21] compared morphologically, the series which comprised 20 cases with solid architecture (11 PSS and 9 STUMP) and 5 cases with phyllodes-like growth pattern (all STUMP). Accosta et al. [21] summarised the results as follows:
Accosta et al. [21] concluded that:
Kim et al. [22] made the ensuing iterations:
Kim et al. [22] reported a case of a PSS with rhabdoid features. Kim et al. [22] reported a31-year-old man, who had manifested with a 4-month history of voiding difficulty and anal pain. He had computed tomography (CT) scan of the abdomen which demonstrated an ovoid mass within his prostate gland invading his rectum and urinary bladder. He had a needle biopsy of the prostate gland and pathology examination of the biopsy specimen revealed features which were diagnosed as an unclassified spindle cell sarcoma, and 2 cycles of adriamycin-based neoadjuvant chemotherapy were given, followed by radical prostatectomy. The prostatectomy specimen upon pathology examination revealed a high-grade sarcoma with fascicles of highly cellular spindle cells and numerous mitoses with haemorrhage and necrosis. In areas, the tumour was also found to contain sheets of loosely cohesive epithelioid cells with rhabdoid tumour component. Both spindle and rhabdoid tumour cells upon immunohistochemistry staining studies were positive for vimentin, CD34, and progesterone receptor and negative for desmin and cytokeratin immunostainings. The rhabdoid tumour cells retained INI1 expression. The tumour recurred in the bladder, and the patient died of sepsis. Kim et al. [22] made the ensuing conclusions:
Ueda et al. [23] stated the ensuing:
Ueda et al. [23] reported a case of PSS. Ueda et al. [23] reported a 40-year-old male, who had manifested with urinary distention. He had Magnetic resonance imaging (MRI) scan, which demonstrated a large prostate mass, and the diagnosis was prostate sarcoma of uncertain differentiation, based upon pathology examination features of his ultrasound-guided needle biopsy specimens of his prostatic lesion. Total pelvic exenteration was undertaken and a pathological diagnosis of PSS was ultimately reached. Ten months pursuant to his surgery, there had been no signs of metastasis or recurrence.
Morikawa et al. [24] reported a unique case of prostatic stromal sarcoma (PSS), which had recurred within his pelvic cavity with massive high-grade prostatic intraepithelial neoplasia. Morikawa et al. [24] reported a 52-year-old man, who had manifested with urinary retention and who underwent a radical cystoprostatectomy. Pathology examination of the cystoprostatectomy specimen showed that the tumour tissues of the prostate gland had demonstrated an admixture of hyperplastic glands and markedly cellular stroma of spindle cells that were arranged in a fascicular pattern, and the tumour was diagnosed as PSS. 66 months pursuant to his operation, he had CT scans which demonstrated three recurrent tumours around the bilateral obturator and left iliopsoas region. The recurrent tumours were biphasic neoplasms, as before, but the epithelial component had grown prominent and manifested overt atypia in a manner resembling high-grade prostatic intraepithelial neoplasia. Morikawa et al. [24] concluded that their findings had indicated that not only the stromal component but also and the epithelial components of PSS may have malignant potential.
Razi et al. [1] reported a 35-year-old man, who was admitted to their hospital, about 12 years preceding publication of his case report, who had manifested with severe obstructive lower urinary tract symptoms and dysuria. He had been experiencing difficulties in voiding over a period of 4 months preceding his admission. He had digital rectal examination, which demonstrated a large and stony-hard tumour mass over the anterior wall of his rectum. He underwent trans-abdominal ultrasound scan which demonstrated a few heterogeneous echoic areas in a 110-gm prostate and some cystic areas with invasion to his urinary bladder neck. He also had magnetic resonance imaging (MRI) scan of his prostate gland which demonstrated a 25 mm well-circumscribed solid tumour with focal calcification within his prostate gland. (see figure 1).
Figure 1: Abdominal-pelvic magnetic resonance imaging revealed a round 2.5-cm diameter mass lesion in the prostate. A: coronal view B: sagittal view. Reproduced from: [1] under the Creative Commons Attribution License.
The result of his serum prostate-specific antigen (PSA) was within the normal range. He underwent trans-urethral prostatectomy (TURP) once, which was not successful; and again, within a month, the second TURP was undertaken. Nevertheless, after two months, the patient was catheterized again. Ultimately, 3 months ensuing his first admission, the prior surgical specimens were reevaluated again within the histopathology department of the authors precisely and based upon the diagnosis of the prostatic stromal tumour, radical retropubic prostatectomy was undertaken consequently. During the surgery, the prostate gland was felt to be an unusually grey-brown polypoidal cystic mass without capsule formation while the neck of his urinary bladder was found to be invaded by the tumour. With regard to pathology examination of the prostatectomy specimen, it was reported that upon transection of the specimen, an irregular polypoid shaped mass, which
measured 2 cm in greatest diameter was visualised within the right lateral posterior lobe of the prostate gland, showing cystic areas. In the microscopy histopathology examination of the specimen, a biphasic neoplasm with dominant stromal component exhibiting mild hypercellularity making frequent leaf-like projections were identified. Also, some cystic spaces were visualised in the tumour, all were lined by bland-looking epithelial cells. The stromal spindle cells were noted to be devoid of atypia and had demonstrated less than 1 mitosis/10 HPF. Necrosis was not seen meaning there was no evidence of necrosis within the tumour. The stromal cells had exhibited focally positive immunohistochemistry staining for Desmin. The luminal epithelial and basal cells had exhibited positive immunohistochemistry staining for PSA and 34βE12 in IHC study, respectively. Based upon all the aforementioned findings, the diagnosis of low grade (benign) phyllodes tumour was established (see figure 2).
Figure 2. A. Biphasic neoplasm with predominant stromal component making leaf-like projections. B. The clefts are lined by bland-looking epithelial cells (arrow). Reproduced from: [1] Under the Creative Commons Attribution License.
Pursuant to his discharge from the hospital, his periodical close follow-up assessment was planned since Phyllodes tumour of the prostate gland has malignant potential and a high recurrence rate. Nevertheless, in the reported patient, no evidence of recurrence was found during his 12-year follow-up assessments pursuant to his radical prostatectomy. Razi et al. [1] made the ensuing educative discussions:
Razi et al. [1] summated that their reported case had represented the morphological and immunohistochemical manifestations of Phyllodes tumour of the prostate gland and its long-term follow-up in a young male patient.
Hicks et al. [15] reported a 66-year-old man, who had initially manifested with low-pressure, acute urinary retention. He was catheterized and started on an alpha-blocker (tamsulosin). The initial assessment of his prostate gland by digital rectal examination demonstrated it to be enlarged and benign-feeling. After 2 weeks, he had an unsuccessful trial without catheter and was consented to undergo TURP. During his TURP, it was noted that he had abnormal appearing and chalk-white tissue within the left prostatic lobe on resection; the hardness of the resected tissue was such that it had damaged the resecting bipolar loop twice. Malignancy of the prostate gland was suspected but the histology report returned surprisingly as demonstrating high-grade prostatic stromal sarcoma, in which 80% of the 36 grams of TURP chippings had contained malignant, spindle cell proliferation without an organized fascicular pattern but with the presence of nuclear atypia, mitotic activity and tumour necrosis (see figures 3, 4, and 5).
Figure 4: Haematoxylin and Eosin staining with ×10 magnification demonstrating ovoid, short spindle cells without an organized growth pattern. Reproduced from [15] Under the Creative Commons Attribution Licence.
Figure 3: Macroscopic sections of the apical, mid-gland and basal areas of the prostate specimen demonstrating central TURP cavitation and abnormal residual tissue. Reproduced from [15] Under the Creative Commons Attribution Licence.
Figure 5: Haematoxylin and Eosin staining with ×40 magnification demonstrating hypercellularity, nuclear hyperchromasia and mitotic activity. Reproduced from [15] Under the Creative Commons Attribution Licence.
He had multiparametric magnetic resonance imaging (MRI) scan of his prostate and pelvis, which demonstrated a heterogeneous enhancement of the remaining prostate tissue. The prostatic capsule was intact; nevertheless, there were enlarged left iliac lymph nodes (see figures 6 and 7). He had staging computed tomography (CT) of thorax, abdomen and pelvis, which did not demonstrate any evidence of progression elsewhere within his body. He therefore underwent an open radical cysto prostatectomy with retroperitoneal lymph node dissection and urinary diversion.
Figure 6: T1-weighted axial MRI image with contrast demonstrating an enlarged prostate with inhomogenous signal. Reproduced from [15] Under the Creative Commons Attribution Licence.
Figure 7: T2-weighted axial MRI image with contrast demonstrating an enlarged prostate with inhomogenous signal. Reproduced from [15] Under the Creative Commons Attribution Licence.
Hicks et al. [15] made the ensuing educative discussions:
Hicks et al. [15] made the ensuing conclusions:
Zamparesee et al. [31] reported a case of a 71-year-old man, who had that developed progressive urinary obstruction symptoms and who was subjected to a transurethral prostatic resection (TURP). Histopathology examination of the prostate chips demonstrated that there was a diffuse proliferation of epithelioid and spindle cells that showed rare atypical mitotic figures. Immunohistochemistry staining studies of the specimen showed that the neoplastic cells had expressed diffusely CD34 and focally progesterone whereas no immunoreactivity was seen for cytocheratin, desmin, S-100, Bcl-2, chromogranin, CD117, and actin smooth muscle. A final diagnosis of low-grade prostatic stromal sarcoma (LG-PS) was made. Zamparesee et al. [31] stated that the lesion was a neoplasm and that in the literature, in fact, to their knowledge, only 6 cases had been reported by the time of the report of their case as well as all of these were alive and free of disease at follow-up. They also reported that their patient too was free of disease at 15 months from the diagnosis.
Batayneh D et al. [35] made a meeting presented with the ensuing abstract summation at the 2024 ASCO Genitourinary Cancers Symposium (Abstract 181):
Batayneh et al. [35] identified from 19,057 cases of prostate cancers, only 11 (<.01%) cases of primary sarcomas and they undertook a comprehensive genomic profiling (CGP) using FDA-approved hybrid capture-based system to assess all classes of genomic alterations (GA). They determined genomic-based ancestry, genomic signature, gLOH, MSI and TMB status by CGP. Batayneh et al. [35] predicted germline status was using a Somatic-Germline-Zygosity algorithm. PD-L1 expression was determined by IHC (Dako 22C3 TPC scoring). Batayneh et al. [35] summarised the results as follows:
Batyneh et al. [35] made the ensuing conclusions:
These tumours do not appear to be driven by “targetable” gene fusions and individual “targetable” mutations are uncommon
Conflict of Interest– nil
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