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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 649

Renal primitive neuroectodermal tumor as a second malignancy after chemotherapy and radiation for Non-Hodgkin's Lymphoma - treatment-related or just poor old bad luck?: A case report

Department of Surgical Oncology, Prince Aly Khan Hospital, Mumbai, Maharashtra, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Jean-Louis de Menezes
Department of Surgical Oncology, Prince Aly Khan Hospital, Nesbit Road, Mazagon, Mumbai - 400 010, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.138124

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 > Abstract 

Peripheral primitive neuroectodermal tumor (PNET) is a rare histology to be found in primary tumors of the kidney. There are less than a hundred cases reported in the English literature. Most of these have been diagnosed after surgery for a renal neoplasm diagnosed on imaging. PNET has rarely been reported as a second malignancy, and has never been reported as a second malignancy after non-Hodgkin's lymphoma (NHL). Herein, we present our case of a 38-year-old female who developed a second malignancy in the kidney after the treatment for NHL.

Keywords: Non-Hodgkin′s lymphoma complication, rare tumours, renal primitive neuroectodermal tumor, renal tumours, second malignancy

How to cite this article:
de Menezes JL, Patil HM, Kannan R, Pradhan SA. Renal primitive neuroectodermal tumor as a second malignancy after chemotherapy and radiation for Non-Hodgkin's Lymphoma - treatment-related or just poor old bad luck?: A case report. J Can Res Ther 2015;11:649

How to cite this URL:
de Menezes JL, Patil HM, Kannan R, Pradhan SA. Renal primitive neuroectodermal tumor as a second malignancy after chemotherapy and radiation for Non-Hodgkin's Lymphoma - treatment-related or just poor old bad luck?: A case report. J Can Res Ther [serial online] 2015 [cited 2020 Jul 10];11:649. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/649/138124

 > Introduction Top

Peripheral primitive neuroectodermal tumors (PNETs) are a member of the Ewing's sarcoma family of tumors, and originate in bones and soft tissues. Intra-abdominal PNETs comprise less than 15% of the total share of PNETs in the body, being the majority located in the axial skeleton and truncal soft tissues. [1] Renal site for a primary PNET is rare. [2] PNET is also rarely found as a second malignancy. We present here the case of a PNET arising as a second primary in the kidney of a patient previously treated for non-Hodgkin's lymphoma (NHL).

 > Case report Top

A female patient diagnosed with NHL of the nasopharynx at the age of 27 was treated with chemotherapy (CHOP regimen) followed by radiotherapy to the nasopharynx, in 2001. She was subsequently asymptomatic and clinico-radiologically disease-free until she presented a decade later with abdominal pain and an episode of hematuria. A CT scan of the abdomen revealed an enhancing mass in the left kidney with suspected involvement of the left renal vein [Figure 1]. A whole body PET-computed tomography (CT) scan revealed no evidence of regional or distant metastatic disease, nor did it show any uptake or lesion in the nasopharynx. Nasopharyngoscopy did not reveal any disease in the nasopharynx. She underwent a trans-peritoneal radical nephrectomy with resection of the vein thrombus.
Figure 1: CT scan Image showing tumor extending into renal pelvis

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Histology revealed a malignant blue round cell tumor of the kidney, measuring 7.5 cm in size with extension into the renal vein, but without extra-renal spread or metastatic lymphadenopathy. Immunohistochemistry revealed positivity for the product of the MIC-2 gene (CD-99) [Figure 2] without positivity for leukocyte common antigen (LCA), S100, vimentin or cytokeratin. She did not receive any adjuvant chemotherapy or radiation. She was disease-free for 10 months, when she presented with a neck nodal metastasis. Metastatic workup showed presence of bilateral pulmonary metastases along with vertebral metastases. She subsequently received chemotherapy, but only had a partial response.
Figure 2: Photomicrograph showing the tumor immunostaining positive (golden-brown) for CD99 extending into the normal renal parenchyma in an infiltrative pattern

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There was no attempt made to identify a germline mutation which might explain this second malignancy in the current patient due to social and economic constraints.

 > Discussion Top

PNETs are most commonly seen outside the CNS in the paravertebral soft tissues and chest wall, and less frequently in the lower extremities. The kidney is an uncommon site for development of a PNET, with less than a hundred reported in English medical literature. Although PNET was first identified as an entity by Arthur Stout in 1918, the first renal PNET was reported by Seemayer only in 1975. [3] Renal PNETs have been reported between 3 and 78 years of age, although the mean age at diagnosis is 27 years. Pain is followed by hematuria as the most frequent symptom. Around a quarter of the patients have been found to have renal vein involvement. [1] The overall prognosis of these tumors remains poor even after multimodality treatment, with the largest series reporting an estimated survival of 60% at three years. Most patients have received adjuvant chemotherapy, and some have received adjuvant radiation to the renal bed. [4]

There are only a handful of reports of PNET developing as a second malignancy after treatment for acute lymphoblastic leukemia (ALL) and Hodgkin's disease. There is a report of a PNET developing within the field of radiation. There are no known reports in a Medline database search of a PNET developing in a patient treated for NHL. There is an increased risk of developing soft tissue sarcomas after treatment for NHL, and the risk is highest more than 10 years of completing treatment. There is also an increased risk of renal tumors after use of cyclophosphamide for the treatment of NHL. There is a higher incidence of development of a second malignancy in patients treated for NHL before the age of 45 years. The most common second malignancies are hematological. Among the solid tumors, lung and bladder cancers are the more common second malignancies. [5]

 > Conclusion Top

We report a case of a renal PNET developing as a second malignancy after treatment of nasopharyngeal NHL in a young lady. This association may need further investigation, given the poor prognosis of this condition, as evidenced by the outcome of the patient in this report. Due consideration should be given to the use adjuvant chemotherapy even in apparently localized disease.

 > References Top

Jürgens H, Bier V, Harms D, Beck J, Brandeis W, Etspüler G, et al. Malignant peripheral neuroectodermal tumors: A retrospective analysis of 42 patients. Cancer 1988;61:349-57.  Back to cited text no. 1
Aghili M, Rafiei E, Mojahed M, Zare M. Renal primitive neuroectodermal tumor: Does age at diagnosis impact outcomes? Rare Tumors 2012;4:e15.  Back to cited text no. 2
Seemayer TA, Thelma WL, Bolande RP. Peripheral neuroectodermal tumours. Perspect Pediatr Pathol 1975;12:151-2.4:e15   Back to cited text no. 3
Thyavihally YB, Tongaonkar HB, Gupta S, Kurkure PA, Amare P, Muckaden MA, et al. Primitive neuroectodermal tumor of the kidney: A single institute series of 16 patients. Urology 2008;71:292-6.  Back to cited text no. 4
Brennan P, Scélo G, Hemminki K, Mellemkjaer L, Tracey E, Andersen A, et al. Second primary cancers among 109 000 cases of non-Hodgkin′s lymphoma. Br J Cancer 2005;93:159-66.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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