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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2016  |  Volume : 27  |  Issue : 4  |  Page : 787-790
Metastatic neuroendocrine tumor of small cell type in a kidney transplant recipient

1 Department of Nephrology, Hospital Tengku Ampuan Afzan, Pahang, Malaysia
2 Department of Internal Medicine, Kulliyyah of Medicine, International Islamic University Malaysia, Pahang, Malaysia
3 Department of Pathology, Hospital Tengku Ampuan Afzan, Pahang, Malaysia
4 Department of Radiology, Hospital Tengku Ampuan Afzan, Pahang, Malaysia

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Date of Web Publication5-Jul-2016


Malignancy is a significant long-term complication of successful renal transplantation. Not only the rate is higher but also cases are highly aggressive. We report a case of metastatic, small cell, neuroendocrine tumor in a post-renal transplant patient with progressive left inguinal nodes and right lumbar swellings. He had a remarkably elevated serum lactate dehydrogenase levels with multiple metastatic masses in the left inguinal, left iliac fossa, and right lower abdominal wall on abdominal computed tomography scan. Excisional biopsy of a left inguinal node revealed extensive infiltration with malignant cells in it. Immunohistochemistry staining was positive for synaptophysin, prostatic specific antigen, and CD56. While the primary small cell carcinoma of genitourinary organs is rare, the case illustrates the highly aggressive nature of the disease in a kidney transplant recipient.

How to cite this article:
Mohd. Kamil A, Draman CR, Seman M, Kalavathy R, Mubarak M Y. Metastatic neuroendocrine tumor of small cell type in a kidney transplant recipient. Saudi J Kidney Dis Transpl 2016;27:787-90

How to cite this URL:
Mohd. Kamil A, Draman CR, Seman M, Kalavathy R, Mubarak M Y. Metastatic neuroendocrine tumor of small cell type in a kidney transplant recipient. Saudi J Kidney Dis Transpl [serial online] 2016 [cited 2022 Nov 26];27:787-90. Available from: https://www.sjkdt.org/text.asp?2016/27/4/787/185259

   Introduction Top

High-incidence rate of malignancy in organ transplant recipients is well described and generally associated with poor clinical prognosis. [1] Chronic use of immunosuppressant is a significant factor for rapid progression and poor outcome in the kidney transplant recipients (KTR). [2] Although the incidence of various cancers in KTR was significantly higher than the wait-listed patients, the relative risk for prostate and ovarian cancer was lower. [3]

We report a case of aggressive, metastatic small cell, neuroendocrine tumor of genitourinary organs in a KTR and review literature regarding the disease progress and outcome.

   Case Report Top

A 49-year-old Chinese male with end-stage renal disease underwent deceased kidney transplantation with immediate graft function in 1999. He was followed up in our clinic with a stable graft function, and creatinine level was 80-120 mmol/L. His maintenance immunosuppressive therapy consisted of prednisolone 7.5 mg daily, mycophenolic mofetil 1 g daily and oral cyclosporine A 75 mg daily in divided doses. His other medical problems included chronic atrial fibrillation secondary to ischemic heart disease, hypertension, and hyperlipidemia. His blood pressure was well controlled with atenolol 100 mg daily, perindopril 8 mg daily, and amlodipine 10 mg daily. He was also prescribed daily warfarin 2.5 mg and atorvastatin 40 mg. His latest HBs Antigen, anti-HCV, and CMV serology were negative.

After 13-year of follow-up, he presented in the clinic with progressive, painless left inguinal and left leg swelling for one-month duration. The symptoms were not associated with fever, constitutional symptoms, pruritus, or night sweat. He also denied chronic, productive cough, urinary symptoms, or any alteration in his bowel habit. Clinically, there were palpable, nontender, and matted left inguinal nodes with left leg pitting edema. The cervical and axillary nodes and liver and spleen were not enlarged, but a superficial and painless right lumbar mass was palpable. The rest of the systemic examination was unremarkable.

The patient had markedly increased serum lactate dehydrogenase of 1408 μ/L. The inguinal and lower limb ultrasound Doppler scan showed multiple, heterogeneous masses in left inguinal region and no evidence of venous thrombosis. His peripheral blood and white cell differential count and renal, liver, and coagulation profiles and prostate specific antigen (PSA) were normal. Epstein-Barr viral (EBV) DNA polymerase chain reaction was not detectable. A chest radiograph was unremarkable, but thoracic and abdominal computed tomography (CT) scan showed multiple soft tissue masses in the left iliac fossa [Figure 1]a and right lower abdominal wall [Figure 1]b. There was also a left inguinal soft tissue mass with an area of necrosis compressing the left femoral vessels [Figure 2]a. The urinary bladder and prostate were unremarkable [Figure 2]b. The transplanted kidney had normal parenchymal enhancement and excretion.
Figure 1: CT images showed (a) left iliac fossa mass (long arrow) and (b) subcutaneous soft tissue masses on the right side (short arrow) at the level of the lower pole of transplanted kidney (K).

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Figure 2: CT images revealed soft tissue mass with necrosis at the left inguinal region with edema of the subcutaneous tissue on the left side (a). The urinary bladder (UB) and prostate gland (P) were normal (b).

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The patient had left inguinal lymph node excisional biopsy, which showed a completely distorted structure with extensive malignant cells infiltration. The cells were arranged in small sheets, nests, and clusters without rosette or gland formation. The tumor cells showed hyperchromatic nuclei, coarse chromatin with small nucleoli, and scanty cytoplasm. The mitotic activity was 6-20/HPF. Immunohistochemistry staining for synaptophysin, PSA, and CD56 were significantly positive [Figure 3] but not for chromogranin A, EBV-LMP, LCA, or CD 20.
Figure 3: Histological examination of the lympnode biopsy showed the tumor cells were arranged in small sheets, nests and clusters without gland or rosette formation. The cells had small nucleolus with scanty cytoplasm and high mitotic index (3a). The tumor cells were also stained positive for synaptophysin (3b), PSA (3c) and CD 56 (3d).

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While awaiting further workup and definitive management of his malignancy, our patient expired unexpectedly following an acute cardiac event

   Discussion Top

Our patient had a de novo, highly aggressive type of neuroendocrine tumor with metastasis during the presentation to the clinic. The malignant cells demonstrated a highly active mitotic figure with dense chromatin. A positive neuro-endocrine stain is suggestive of aggressive, small-cell carcinoma (SCC). Rannikko et al in their recent publication reported a similar case, but their case was of a highly immunosuppressed patient following repeated kidney transplant. [4] Although phenotypically different, our patient was also at high risk for malignancies following a 13-year of successful renal transplantation. In fact, data from the US Renal Data Systems showed that the cumulative incidence of cancer other than non melanoma skin cancer correlated well with duration of post-transplant period. SCC of urogenital organs is rare; sixty cases had been reported until 1998, and none was in a KTR. [5] Although the incidence rate for prostatic carcinoma in kidney transplant patients is currently reported as 1.4%, most of the cases were adenocarcinoma. [6] Furthermore, the most common type of genitourinary tumors in renal recipients is still the transitional cell carcinoma of the urinary bladder followed by renal cell carcinoma. [7]

Our patient had an aggressive SCC of probably prostatic origin as reported earlier that 25% of prostatic SCC cases may present with advanced disease without much of symptoms locally. [5]

Rapid progression of the tumor is related to the degree of immunosuppression in KTRs. Although azathioprine was associated with more locally advanced and metastatic disease of prostate carcinoma, the overall state of immunosuppression determined the risk of malignancy in the KTRs. [6] However, a newer agent such as mycophenolic mofetil was found to have a lower risk for malignancy compared to the other anti-metabolites; our patient had mycophenolic mofetil for his maintenance treatment.

   Conclusion Top

We reported an aggressive neuroendocrine, SCC of probably prostatic origin in a KTR. The aggressiveness of the tumor is supported by the involvement of regional nodes and skin deposits at the time of presentation. Prolonged exposure to immunosuppressive agents is the most significant risk factors in this patient.

Conflict of interest: None declared.

   References Top

Barrett WL, First MR, Aron BS, Penn I. Clinical course of malignancies in renal transplant recipients. Cancer 1993;72:2186-9.  Back to cited text no. 1
Trivedi MH, Agrawal S, Muscato MS, Metzler MH, Marshall JB. High grade, synchronous colon cancers after renal transplantation: Were immunosuppressive drugs to blame? Am J Gastroenterol 1999;94:3359-61.  Back to cited text no. 2
Kasiske BL, Snyder JJ, Gilbertson DT, Wang C. Cancer after kidney transplantation in the United States. Am J Transplant 2004;4:905-13.  Back to cited text no. 3
Rannikko A, Krogerus L, Nyberg M, Salmela K, Ruutu M. Rapid progression of small cell carcinoma in a renal transplant recipient. Int J Urol 2006;13:817-9.  Back to cited text no. 4
Mackey JR, Au HJ, Hugh J, Venner P. Genitourinary small cell carcinoma: Determination of clinical and therapeutic factors associated with survival. J Urol 1998;159:1624-9.  Back to cited text no. 5
Elkentaoui H, Robert G, Pasticier G, et al. Therapeutic management of de novo urological malignancy in renal transplant recipients: The experience of the French department of urology and kidney transplantation from Bordeaux. Urology 2010;75:126-32.  Back to cited text no. 6
Einollahi B, Simforoosh N, Lessan-Pezeshki M, et al. Genitourinary tumor following kidney transplantation: A multicenter study. Transplant Proc 2009;41:2848-9.  Back to cited text no. 7

Correspondence Address:
Che Rosle Draman
Department of Internal Medicine, Kulliyyah of Medicine, International Islamic University Malaysia, Pahang
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.185259

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