Prostatic stromal sarcoma with rhabdoid features

Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, 138-736 Seoul, Korea.
Annals of diagnostic pathology (Impact Factor: 1.12). 12/2010; 14(6):453-6. DOI: 10.1016/j.anndiagpath.2009.10.008
Source: PubMed


Rhabdoid tumors have been reported in many different anatomic sites as an aggressive tumor and usually present with a rhabdoid tumor component (a composite tumor) rather than a pure rhabdoid tumor. Rhabdoid tumor in the prostate has been described only once in the prostatic region as a possible epithelial origin. Rhabdoid features in prostatic stromal sarcomas (PSSs) have never been described in the literature. Here, we report a case of a PSS with rhabdoid features. A 31-year-old man presented with a 4-month history of voiding difficulty and anal pain. Computed tomography of the abdomen revealed an ovoid mass in the prostate invading rectum and urinary bladder. A needle biopsy was diagnosed as an unclassified spindle cell sarcoma, and 2 cycles of adriamycin-based neoadjuvant chemotherapy were given, followed by radical prostatectomy. The prostatectomy specimen revealed a high-grade sarcoma with fascicles of highly cellular spindle cells and numerous mitoses with hemorrhage and necrosis. In areas, the tumor also contained sheets of loosely cohesive epithelioid cells with rhabdoid tumor component. Both spindle and rhabdoid tumor cells were positive for vimentin, CD34, and progesterone receptor and negative for desmin and cytokeratin immunostainings. The rhabdoid tumor cells retained INI1 expression. The tumor recurred in the bladder, and the patient died of sepsis. To the best of our knowledge, this is the first case of PSS with rhabdoid features. The tumor showed an aggressive clinical behavior with a short-term survival (7 months after diagnosis).

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    • "Subsequently, this type of tumor was recognized as a distinct and unique malignant renal tumor. MRTK has also been described at extrarenal sites in children and adults, including the urogenital system such as the bladder or prostate (7–9). "
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    ABSTRACT: Malignant rhabdoid tumors of the kidney (MRTKs) are extremely rare. Pure MRTKs in adult patients are particularly rare and have not been previously reported in China. Due to the non-specific clinical symptoms, it is difficult but also essential to be able to give a definite diagnosis. The present study reports a case of pure adult malignant rhabdoid tumor in a patient's left kidney with characteristic clinicopathological features. Considering the fact that the characteristic findings are often not observed in clinical symptom and imaging studies, the histological features, immunohistochemical staining and cytogenetic studies may aid in confirming the diagnosis of pure MRTKs. Although pure adult MRTKs remain extremely uncommon, it is necessary to consider this possibility when such types of renal tumors are encountered.
    Full-text · Article · May 2013 · Oncology letters
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    • "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 [13]. The tumor in our case was positive for CD56, CD99 (to our knowledge the second reported case) [14], synaptophysin and negative for EMA and cytokeratin. "
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    ABSTRACT: AbstractProstatic 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.Virtual slideThe virtual slide(s) for this article can be found here:
    Full-text · Article · Dec 2012 · Diagnostic Pathology
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    ABSTRACT: Prostatic stromal sarcoma (PSS) is quite rare. Herein, we describe magnetic resonance imaging (MRI) features of a PSS identified in a 26-year-old man with dysuria and hematuria. MRI clearly depicted the extent and multinodular appearance of the tumor, which was mainly located in the central zone of the prostate. The tumor appeared as a heterogeneously signal-hyperintense mass with a pseudocapsule on T2-weighted imaging. Contrast-enhanced T1-weighted MRI showed necrotic portions in the gradually enhanced solid mass, and diffusion-weighted imaging permitted the accurate assessment of the local extent of the tumor. Thus, the appearance on MRI was quite different from that of adenocarcinoma of the prostate.
    Full-text · Article · Jul 2011 · Korean journal of radiology: official journal of the Korean Radiological Society
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