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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 386-388
Primitive neuroectodermal tumor of prostate

1 Department of Radiotherapy, Maulana Azad Medical College and Lok Nayak Hospital, Bahadur Shah Zafar Marg, New Delhi - 110 002, India
2 Department of Pathology, Maulana Azad Medical College and Lok Nayak Hospital, Bahadur Shah Zafar Marg, New Delhi - 110 002, India

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Primitive neuroectodermal tumors (PNETs) are malignant proliferations of small, undifferentiated neuroectodermal cells occurring mainly in children and share the same reciprocal translocation between chromosomes 11 and 22 and the same patterns of biochemical and oncogene expression as osseus and extraosseus Ewing's sarcoma. Some PNETs occur in the brain, while others (the peripheral PNETs) occur in sites outside the brain, such as in the extremities, pelvis and the chest wall. They mostly originate in the chest, pelvis and retroperitoneum; in rare cases, occurrence in the head and neck area has also been seen. We present a rare case of primitive neuroectodermal tumor arising in the prostate gland in a 25-year-old male. To the best of our knowledge, this is the first documentation of a primary peripheral primitive neuroectodermal tumor in the prostate gland of any patient from India and rarely reported in English literature.

Keywords: Neuron-specific enolase, primitive neuroectodermal tumor, prostate

How to cite this article:
Kumar V, Khurana N, Rathi A K, Malhotra A, Sharma K, Abhishek A, Bahadur A K. Primitive neuroectodermal tumor of prostate. Indian J Pathol Microbiol 2008;51:386-8

How to cite this URL:
Kumar V, Khurana N, Rathi A K, Malhotra A, Sharma K, Abhishek A, Bahadur A K. Primitive neuroectodermal tumor of prostate. Indian J Pathol Microbiol [serial online] 2008 [cited 2023 Feb 5];51:386-8. Available from:

   Introduction Top

Primitive neuroectodermal tumor (PNET) is classically a tumor of the soft tissue or bone in children and young adults, although cases are seen to occur in all ages. Within the last decade, the clinicopathologic spectrum of primitive neuroectodermal tumor has been markedly expanded by recognition that the tumor may also have a visceral origin. These tumors show a predilection for bones and soft tissues in the paraspinal region and lower extremities. [1] PNET is closely related to osseous and extraosseous Ewing sarcoma (EWS), sharing the same chromosomal translocation that results in the fusion of EWS gene with a member of erythroblast transformation-specific domain family genes. The vast majorities of PNETs express a surface antigen encoded by the Mic-2 gene and carry a nonrandom cytogenetic marker, which allows pathologists to identify immunohistochemically equivocal cases. [2] Primitive peripheral neuroectodermal tumor has been described in a variety of primary visceral sites, [3],[4],[5],[6],[7],[8],[9] but to the best of our knowledge, it has never been reported in the prostate gland. We report a case of PNET occurring in a 25-year-old man who presented with a mass within the prostate gland. The diagnosis was confirmed by trucut biopsy from the prostate mass and immunohistochemistry.

   Case History Top

A 25-year-old man with a history of intermittent burning micturition for the last 3 years and symptom of bladder outflow obstruction for the last 4 months presented in our outpatient department. He had no history of any major illness or documented malignancy in the past. The patient's systemic examinations were within normal limits. On digital rectal examination, the prostate was enlarged with nodular surface and upper border was reaching 6 cm above the anal verge. Clinically, rectal mucosa was free and there was no obvious bleeding per rectum. On CT scan, a mass measuring 6.7 × 5.5 cm was seen in the region of the prostate gland, not separately defined from the prostatic tissue, with extraprostatic extension of the lesion seen on the left, extending upto the lateral pelvic wall with involvement of the left ureter which showed proximal dilatation. There was involvement of the wall of the urinary bladder; more so in the left side, with the involvement of bilateral seminal vesicle. Bilateral kidney showed mild-to-moderate hydroureteronephrosis. The patient's renal profiles were grossly deranged for which he underwent bilateral percutaneous nephrostomy following which his renal functions were normalized.

The patient's serum prostate-specific antigen level was 0.88 ng/mL and β-human chorionic gonadotropin and prostate-specific acid phosphatase values were within normal ranges.

Pathological findings

A transrectal prostate biopsy was performed and a diagnosis of small round cell tumor consistent with PNET was rendered. Microscopically, the tumor was made up of solid nests and sheets of round cells containing intracytoplasmic glycogen identified by PAS with and without diastase predigestion, accompanied by a scanty fibrovascular stroma. The round cells had dark round to oval nuclei with high nuclear cytoplasmic ratio, hyperchromatic nuclei, brisk mitosis and pseudorosetting around blood vessels. Immunohistochemical staining revealed viable neoplastic cells showing diffuse, intense cell-surface immunoreactivity for CD99. The tumor cells were positive for vimentin, S100, neuron-specific enolase and synaptophysin (the latter focally) and negative for cytokeratin, desmin and CD45 [Figure 1] and [Figure 2].

A final diagnosis of PNET prostate with extension to left ureter, urinary bladder and bilateral seminal vesicle was made.

The patient was started on chemotherapy based on Adriamycin, Vincristine, Ifosfamide and Actinomycin-D in standard dosages. The patient was re-evaluated radiologically after four cycles of chemotherapy which showed almost 75% regression of the disease.

   Discussion Top

Two features that render the diagnosis of PNET difficult are its immunophenotypic profile, which often shows multidirectional differentiation along epithelial, neuroendocrine and/or mesenchymal lines and its occurrence in unusual sites, that is, sites other than bone or soft tissues.

The common acinar prostatic adenocarcinoma constitutes 95% of all malignant prostatic neoplasms. Numerous rare, morphological variants of prostatic carcinoma have been identified in the last two decades. These atypical prostate cancers account for less than 5% of all prostatic malignancies. These prostatic malignancies are listed in [Table 1]. [3]

Extraskeletal PNET mainly occurs in the paravertebral region, the chest wall and the lower extremities and less commonly in the pelvis, retroperitoneum, or the upper extremities. Exceptional sites of occurrence include several organs of the genitourinary system, such as kidney, ureter, bladder, testis and seminal vesicles, stomach, omentum as well as many other visceral sites (ovary, pancreas, uterus, parotid gland and lungs). [3],[4],[5],[6],[7],[8],[9],[10] Reported intracranial sites for PNET include cavernous sinuses, jugular foramen and duramater at the frontal base.

The diagnosis of PNET is mainly based on conventional histologic examination, but sometimes a confident categorization of the tumor may be difficult. The main differential diagnoses considered in our case, taking into account the age of the patient and the tumor site, were embryonal rhabdomyosarcoma, malignant lymphoma and desmoplastic small round cell tumor. Rhabdomyosarcoma is the most common sarcoma of the bladder and prostate gland, but only a few cases have been reported in patients older than 20 years. The tumor cells are usually small and stellate, with hyperchromatic nuclei often showing atypical mitoses and exhibiting a greater variability in size and shape than those of PNET. In our case, the morphologic findings and the negative immunostaining for desmin and myogenin ruled out this possibility. Malignant lymphoma was easily ruled out by negativity for CD45. Finally, a possible secondary involvement of the prostate gland by desmoplastic small round cell tumor was excluded by the lack of expression of cytokeratins and desmin.

CD99, a glycoprotein product of the MIC2 gene, is present on the cell surface of the vast majority of PNET cases (90%) and therefore represents a useful marker for the diagnosis of PNET, but it is not entirely specific. Indeed, CD99 positivity has been observed in lymphoblastic lymphoma (90%), rhabdomyosarcoma (20-25%), poorly differentiated sarcoma (75%) and approximately 50% of mesenchymal chondrosarcomas.

   References Top

1.Dehner LP. Peripheral and central primitive neuroectodermal tumors: A nosologic concept seeking a consensus. Arch Pathol Lab Med 1986;110:997-10051.  Back to cited text no. 1  [PUBMED]  
2.Quezado M, Benjamin DR, Tsokos M. EWS-FLI-1 fusion transcripts in three peripheral primitive neuroectodermal tumors of the kidney. Hum Pathol 1997;28:767-71.  Back to cited text no. 2  [PUBMED]  
3.Varghese SL, Grossfeld GD. The prostate gland: Malignancies other than adenocarcinomas: A clinically relevant approach to imaging. Radiol Clin North Am 2000;38:179.  Back to cited text no. 3  [PUBMED]  
4.Charny CK, Glick RD, Genega EM, Meyers PA, Reuter VE, La Quaglia MP. Ewing's sarcoma/primitive neuroectodermal tumor of the ureter: A case report and review of the literature. J Pediatr Surg 2000;35:1356-8.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Banerjee SS, Eyden BP, McVey RJ, Bryden AA, Clarke NW. Primary peripheral primitive neuroectodermal tumor of urinary bladder. Histopathology 1997;30:486-90.  Back to cited text no. 5    
6.Aguirre P, Scully RE. Primitive neuroectodermal tumor of the testis: Report of a case. Arch Pathol Lab Med 1983;107:643-5.  Back to cited text no. 6  [PUBMED]  
7.Bjerklund Johansen TE, Huseby A, Stenwig JT. Extraskeletal Ewing's sarcoma contiguous with the seminal vesicle. Scand J Urol Nephrol 1988;22:237-9.  Back to cited text no. 7    
8.O'Sullivan MJ, Perlman EJ, Furman J, Humphrey PA, Dehner LP, Pfeifer JD. Visceral primitive peripheral neuroectodermal tumors: A clinicopathologic and molecular study. Hum Pathol 2001;32:1109-15.  Back to cited text no. 8    
9.Soulard R, Claude V, Camparo P, Dufau JP, Saint-Blancard P, Gros P. Primitive neuroectodermal tumor of the stomach. Arch Pathol Lab Med 2005;129:107-10.  Back to cited text no. 9    
10.Tanida S, Tanioka F, Inukai M, Yoshioka N, Saida Y, Imai K, et al . Ewing's sarcoma-peripheral primitive neuroectodermal tumor (pPNET) arising in the omentum as a multilocular cyst with intracystic hemorrhage. J Gastroenterol 2000;35:933-40.  Back to cited text no. 10    

Correspondence Address:
Vikash Kumar
O.P.D. Room No. 124, Department of Radiotherapy, Lok Nayak Hospital, Delhi Gate, Bahadur Shah Zafar Marg, New Delhi - 110 002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.42518

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  [Figure 1], [Figure 2]

  [Table 1]

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