Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2018 Dec 9;11(1):e224273.
doi: 10.1136/bcr-2018-224273.

Primitive neuroectodermal tumour with synchronous ipsilateral clear cell carcinoma of the kidney

Affiliations
Case Reports

Primitive neuroectodermal tumour with synchronous ipsilateral clear cell carcinoma of the kidney

Mohamed Taha et al. BMJ Case Rep. .

Abstract

We report the first case of a synchronous ipsilateral primitive neuroectodermal tumour (PNET) and clear cell renal cell carcinoma of the kidney. A 37-year-old man presented to the emergency department with a 24-hour history of colicky abdominal pain and visible haematuria. He had no relevant surgical or medical history. Physical examination was unremarkable apart from mild left flank tenderness. Triphasic CT of the abdomen and pelvis showed two solid lesions in the left kidney. Further staging CT of the chest showed no evidence of local or distal metastasis. He subsequently underwent laparoscopic radical nephrectomy. Pathological analysis of the kidney showed two synchronous renal tumours, a clear cell carcinoma and PNET of the kidney. The patient received adjuvant chemotherapy according to Ewing's sarcoma chemotherapy protocol. Surveillance CT scans at 3, 6 and 12 months showed no evidence of disease recurrence or metastasis.

Keywords: pathology; urological cancer; urological surgery.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
A coronal section of the CT scan showing the lower pole renal lesion which came as PNET of the kidney. PNET, primitive neuroectodermal tumour.
Figure 2
Figure 2
An axial section of the CT scan showing the upper pole renal lesion which came as RCC after histology. RCC, renal cell carcinoma.
Figure 3
Figure 3
Two distinct tumours are present in this nephrectomy specimen. A larger tumour is present in the lower pole and has a solid tan brown cut surface with extensive necrosis (PNET). A second smaller tumour is present in the upper pole with yellow solid and cystic cut surface (CCRCC). CCRCC, clear cell renal cell carcinoma; PNET, primitive neuroectodermal tumour.
Figure 4
Figure 4
High power view of the smaller tumour which shows classical morphology of clear cell renal cell carcinoma.
Figure 5
Figure 5
High power views of the larger tumour showing diffuse sheets of undifferentiated cell with moderate clear cytoplasm and vesicular nuclei.
Figure 6
Figure 6
The tumour is negative for pan cytokeratin AE1/AE3.
Figure 7
Figure 7
The tumour shows diffuse strong membranous staining with CD99.

Similar articles

References

    1. Thomas AZ, Adibi M, Slack RS, et al. . The role of metastasectomy in patients with renal cell carcinoma with sarcomatoid dedifferentiation: a matched controlled analysis. J Urol 2016;196:678–84. 10.1016/j.juro.2016.03.144 - DOI - PMC - PubMed
    1. Mor Y, Nass D, Raviv G, et al. . Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney. Med Pediatr Oncol 1994;23:437–40. 10.1002/mpo.2950230508 - DOI - PubMed
    1. Dotan ZA, Tal R, Golijanin D, et al. . Adult genitourinary sarcoma: the 25-year Memorial Sloan-Kettering experience. J Urol 2006;176:2033–9. 10.1016/j.juro.2006.07.021 - DOI - PubMed
    1. Carvajal R, Meyers P. Ewing’s sarcoma and primitive neuroectodermal family of tumors. Hematol Oncol Clin North Am 2005;19:501–25. 10.1016/j.hoc.2005.03.004 - DOI - PubMed
    1. Grier HE, Krailo MD, Tarbell NJ, et al. . Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 2003;348:694–701. 10.1056/NEJMoa020890 - DOI - PubMed

Publication types

MeSH terms