• Ewing sarcoma
• chemotherapy
• epididymis neoplasms
• extraosseous Ewing sarcoma
• male genital system

• Humans
• Male
• Sarcoma, Ewing/diagnostic imaging
• Sarcoma, Ewing/diagnosis
• Sarcoma, Ewing/therapy
• Sarcoma, Ewing/pathology
• Epididymis/diagnostic imaging
• Epididymis/pathology
• Young Adult
• Genital Neoplasms, Male/diagnosis
• Genital Neoplasms, Male/diagnostic imaging
• Genital Neoplasms, Male/therapy
• Genital Neoplasms, Male/pathology
• Ultrasonography, Doppler, Color
• Magnetic Resonance Imaging
• Tomography, X-Ray Computed

• gyfybsky-2023-04 the Doctor Start-up Fundamental Hospital for Guizhou Medical University of China
• 2023-402 the Guizhou Provincial Basic Research Program (Natural Science) of China

Primitive neuroectodermal tumor (PNET), especially located in the prostate, is a rare tumor that mainly occurs in young men. Bladder or rectum invasion and distant metastasis are strongly associated with a poor prognosis. Combination therapy, including radical surgery, adjuvant chemotherapy, and radiotherapy, is available. We present a case of prostatic PNET and a review of 17 cases identified in the literature.

A 58-year-old man was admitted complaining of dysuria for 2 years. Computed tomography and magnetic resonance imaging showed a large cystic-solid mass in the pelvic cavity compressing the surrounding bladder and rectum. The mass was iso- to hyperintense on T1-weighted imaging (WI) and heterogeneously hyperintense on T2WI. Cystic degeneration and necrosis were seen in the tumor, and solid tissues within the mass enhanced on contrast-enhanced scan. The patient underwent robot-assisted laparoscopic pelvic tumor resection. Histologically, the presence of many small round cells that were positive for expression of CD99, vimentin, and synaptophysin established the diagnosis of PNET in the prostate after surgery. The patient underwent adjuvant chemotherapy. During 34 mo of follow-up, the patient had no signs or symptoms of recurrence or residual disease.

We present the case of the oldest prostatic PNET patient, who has a good prognosis. This illustrates how older men with prostatic PNET may also benefit from the combination therapy, like younger adults, and achieve a long-term survival. As always, PNET should be considered in the differential diagnosis of aggressive prostatic tumors in young men.

• Primitive neuroectodermal tumor
• Prostate
• Combination therapy
• Magnetic resonance imaging
• CD99
• Case report

Primitive neuroectodermal tumor (PNET)/Ewing's sarcoma tumors are a group of highly malignant tumors composed of small, round cells showing neuroectodermal differentiation that commonly affects soft tissue and bone. PNET involving the genitourinary system is rare and PNET of the penile urethra is rarer still. It exhibits a highly aggressive biological behavior with poor prognosis. We report a case of a 27-year-old male presenting with penile swelling and difficulty in passing urine. Examination revealed a firm penile urethral mass. Pathological and immunohistochemical results of the specimen obtained from urethroscopic biopsy followed by total penectomy confirmed the diagnosis of PNET of the urethra. He received combination chemotherapy: ifosfamide and etoposide (IE) + vincristine, Adriamycin, and cyclophosphamide (VAC). In conclusion, PNET has to be considered in the differential diagnosis of a penile urethral mass in young patients.

• Double barrel sigmoidostomy
• Primitive neuroectodermal tumor
• Prostate
• Total pelvic exenteration

• Ewing sarcoma family of tumours
• extra-skeletal
• fluorescence in situ hybridisation
• reverse-transcriptase polymerase chain reaction
• skeletal
• visceral

• metastasis
• paraplegia
• peripheral
• primitive neuroectodermal tumor
• prostate cancer

• Anatomía patológica
• Biología molecular
• Ewing sarcoma
• Fluorescence in situ hybridization
• Hibridación fluorescente in situ
• Molecular biology
• Pathology
• Prostate neoplasms
• Sarcoma de Ewing
• Tumores prostáticos

• Peripheral primitive neuroectodermal tumor
• renal tumor
• round cell tumor

• 12E7 Antigen/metabolism
• Biomarkers, Tumor/analysis
• Humans
• Immunohistochemistry
• Kidney/pathology
• Kidney Neoplasms/diagnosis
• Kidney Neoplasms/pathology
• Kidney Neoplasms/surgery
• Male
• Middle Aged
• Nephrectomy
• Neuroectodermal Tumors, Primitive/diagnosis
• Neuroectodermal Tumors, Primitive/pathology
• Neuroectodermal Tumors, Primitive/surgery

• Adult
• Chemotherapy
• Ewing sarcoma
• Pelvic neoplasms
• Prostate
• Surgery

• Adult
• Diagnosis, Differential
• Female
• Humans
• Kidney Neoplasms/diagnosis
• Kidney Neoplasms/diagnostic imaging
• Kidney Neoplasms/therapy
• Neuroectodermal Tumors, Primitive/diagnosis
• Neuroectodermal Tumors, Primitive/diagnostic imaging
• Neuroectodermal Tumors, Primitive/therapy
• Tomography, X-Ray Computed

The present study reports a rare case of primitive neuroectodermal tumor (PNET) of the prostate. A 23-year-old male presented to Oita Medical Center (Oita, Japan) with the complaint of dysuria and anal pain. A large mass in the prostate and a number of swollen lymph nodes in the pelvic region were identified by a computed tomography scan and magnetic resonance imaging. The patient was, thus, admitted to Oita University Hospital (Yufu, Japan), where a biopsy of the prostate gland was performed. Histological analysis revealed small round cells that were positive for MIC-2 expression and fluorescent in situ hybridization analysis detected a translocation involving Ewing sarcoma breakpoint region 1 at chromosome 22q12. Thus, a diagnosis of PNET of the prostate was established. Systemic chemotherapy was the selected treatment, however, a poor response was obtained. The patient succumbed approximately four months after the initial onset of symptoms. PNET of the prostate has been reported in eight cases worldwide; in comparison, the present case exhibited the most unsatisfactory outcome.

• primitive neuroectodermal tumor
• prostate
• rapid progression

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

• Diagnosis, Differential
• Humans
• Magnetic Resonance Imaging
• Male
• Neuroectodermal Tumors, Primitive/pathology
• Neuroectodermal Tumors, Primitive/surgery
• Prostate/pathology
• Prostatic Neoplasms/pathology
• Sarcoma, Ewing/pathology
• Sarcoma, Ewing/surgery
• Young Adult