Prostatic stromal sarcoma with neuroectodermal differentiation
© Yamazaki et al.; licensee BioMed Central Ltd. 2012
Received: 13 September 2012
Accepted: 14 October 2012
Published: 7 December 2012
Prostatic stromal sarcoma is a fairly rare tumor that constitutes approximately 0.1–0.2% of all prostatic cancers. Detailed characteristics of the tumor are still unclear due to its rarity.
We describe a case of prostatic stromal sarcoma in a 63 year-old man who suffered from urinary obstructive symptoms. Palliative transuterine resection was performed and the preliminary histopathological diagnosis was neuroendocrine carcinoma. After chemotherapy, total pelvic exenteration was performed. Histopathologically, the tumor was composed of monotonously proliferating small to medium-sized round cells, which existed in compact islands with loose or dense fibrovascular networks. Immunohistochemically, the tumor cells were widely positive for vimentin, CD56, CD99 and focally positive for synaptophysin, CD10, progesterone receptor, desmin and CD34, but negative for EMA, cytokeratin, estrogen receptor, S-100 and myoglobin. Most of the previously reported tumors exhibited positive stainability for CD10 and progesterone receptor. In addition to these markers, expressions of CD56, CD99 and synaptophysin were characteristically detected in our case. To the best of our knowledge, we present the first case of prostatic stromal sarcoma with characteristic immunohistochemical staining properties. Although the biological characteristics of this rare tumor have not yet been elucidated, these findings suggest prostatic stromal sarcoma can potentially show neuroectodermal differentiation.
The virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/7291874028051262
Prostatic stromal sarcoma (PSS) is a fairly rare tumor, constituting approximately 0.1% of all prostatic cancers[1, 2]. Prostatic sarcoma and related proliferative lesions, including prostatic phyllodes tumors, have been classified as prostatic stromal tumors of uncertain malignant potential (STUMP) and prostatic stromal sarcoma (PSS) based on cellularity, mitotic index, cellular atypia and necrosis (WHO 2004). Some STUMP cases were reported as malignant transformation into PSS. There has not yet been a clear differentiation between PSS and STUMP due to the rarity of these tumors. In this article, prostatic stromal sarcoma has potentially neuroectodermal characteristics.
Prostatic stromal sarcoma is a rare tumor that constitutes approximately 0.1–0.2% of all prostatic cancers[1, 2]. Prostatic mesenchymal tumors sometimes cannot be clearly classified as histological entities due to their rarity. Except for prostatic mesenchymal tumors with specialized differentiation, a histologically characteristic classification involves prostatic stromal sarcoma and prostatic stromal proliferation of uncertain malignant potential (STUMP)[6–8]. These tumors have been described on the basis of the histological similarity to phyllodes tumors and have to be differentially diagnosed as other specialized types of tumor, such as GIST, leiomyosarcoma, rhabdomyosarcoma(RMS), and fibrosarcoma. RMS is the most common sarcoma of the prostate, but is rarely reported in adult patients. Prostatic stromal sarcoma and STUMP express female hormone receptors, especially progesterone receptor. These tumors seem to be derived from female hormone-dependent stromal cells. Depending on the cellularity, mitosis and necrosis, these two tumors are histologically evaluated. An acceptable histological grading of stromal sarcoma has not yet been proposed due to the rarity of the tumor. Hasegawa reported that the ki-67 index was related to the prognosis of the tumor.
Recent immunohistochemical analysis revealed that both prostatic stromal sarcoma and STUMP usually express CD10, CD34 and the progesterone receptor[3, 7, 8, 10–12]. This tumor sometimes positively reacts with smooth muscle actin. However, the detailed histopathological characteristics of the tumor cells have not yet been clearly elucidated. Kim reported a case of prostatic stromal sarcoma with rhabdoid features. The tumor in our case was positive for CD56, CD99 (to our knowledge the second reported case), synaptophysin and negative for EMA and cytokeratin. This is the first description of prostatic stromal sarcoma with immunohistochemically positive stainability for synaptophysin. There are other prostatic non-epithelial malignancies with potential neuroectodermal differentiation such as carcinosarcoma, ectomesenchymoma and primitive neuroectodermal tumor (PNET), which have to be distinguished from our case of prostatic stromal sarcoma. Patient age is a useful marker for differential diagnosis, although PNET and malignant ectomesenchymoma are rarely reported in young adult prostate cancer patients[15–18]. Most PNET cases reveal positive immunoreactivity for CD99, but are negative for progesterone receptor. Carcinosarcoma contains elements of epithelial malignancy which exhibit some types of cytokeratins.
PSS is suspiciously derived from mesenchymal pluripotent stem cells in the prostatic stroma. As few cases of STUMP change to PSS in the history, some genetic transformations are considered to be related to PSS[4, 19]. Arva showed that some cases of prostatic squamous cell carcinoma were derived from hormonal or radiation-treated prostatic adenocarcinoma. Unexpected irradiation may be one of the risk factors of PSS[21, 22]. Babarović reported a case of high grade angiosarcoma arising in fibroadenoma and suggested the possibility that severe inflammatory reaction, for example silicon granuloma, may cause malignant transformation of stromal cells in the affected area[23, 24]. These factors may give rise to an exuberant stromal response and cause some genetic events in the mesenchymal pluripotent stem cells. We could classify PSS as two categories, which are de novo tumorigenesis and malignant transformation step by step. However, there are no authentic molecules which directly cause prostatic stromal tumorigenesis. Fibroblast growth factor 8 (FGF8), the eighth member of the fibroblast growth factor family contains alternatively spliced mRNA isoforms. Fibroblast growth factor 8b(FGF8b) is an androgen-induced growth factor with potent oncogenic activity[25–27]. Elo reported the results of prostate-targeted fibroblast growth factor 8b transgenic mice. These mice showed progressive changes in prostatic stroma, as well as the prostatic epithelium. Furthermore, FGF8b is the predominant FGF8 spliceform necessary for proper posterior neural formation in Xenopus. Amsterdam reported the relationship between fgf8 misregulation and neuronal tumors in Zebrafish. This knowledge suggests FBF8b is related to potential neuroectodermal differentiation in prostatic stromal tumorigenesis. Some hormonal imbalances, including androgen imbalance, may cause prostatic stromal progression, which could lead to prostatic stromal neoplasia. We hereby point out that PSS may also possess neuroectodermal characteristics.
We describe a rare case of prostatic stromal sarcoma (PSS) presenting with characteristic immunohistochemical staining properties. To our knowledge, this case is the first case of PSS with positive stainability for synaptophysin, as well as CD99 and CD56.
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 Diagnostic Pathology.
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