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  Patient de 32 ans, admis pour prise en charge dune tumeur rénale droite. Depuis quelques mois, constipation rebelle, suivie dune douleur lombaire droite type de lourdeur. Un CT Scan fait en externe : Grosse masse rénale droite : 9x7 cm, médio-rnale, prenant le pôle inférieur et comprimant la veine rénale avec adénopathies paracaves   Patient 32 yo male complaining of right lumbar pain. CTscan reveals a renal mass compressing the renal vein, with enlarged lymphnodes.  
  Macroscopie   Gross  
  La piéce de nephrectomie radicale soumise contient une formation tumorale ovoïde de 12 cm de g.d. au niveau de la face interne supra-pyelique, médullaire, de consistance ferme. Elle semble trés bien délimitée empiétant sur la corticale, avec une zone d'insertion sur la capsule ou elle est calcifiée. Sa tranche de section est homogne, grisatre, par place friable, necrotique. Par ailleurs, les cavités pyéliques contiennent des calculs.   The radical nephrectomy specimen shows a large mass mesuring12 cm, implanted on the internal aspect of the kidney, in the medulla, relatively well circumscribed encroaching on the cortex. Focally the capsule is tightly adherent to the mass, with large calcified area. It is firm in consistancy, homogenous grayish on its cut surface, focally friable, necrotic. Stones are noted in the renal pelvis.  
  Au faible grossissement la tumeur est compacte, bien démarque du tissu rnal avec des foyers d'essaimage périphériques.   Low power discloses a cellular tumor, well delineated from the renal tissue, with foci of peripheral implants.  
  Des zones de microcalcifications priphriques, dystrophiques sont observes. Parfois larges notamment dans la zone capsulaire.   Areas of dystrophic microcalcifications, peripheral in the tumor are observed, sometimes large in the capsular area.  
  La tumeur est forme de larges traves et de cordons, d'allure endocrinoïde   The tumor has an endocrinod pattern, formed by anastomosing cords of variable thickness.  
  Les cordons prennent souvent une disposition rubanée.      
  L'immunohistochimie objective une positivit la chromogranine A   Immunohistochemistry, positivity to Chromogranin A  
  Dans les zones mdullaires, il existe des embols vasculaires lymphatiques. Les ganglions para-caves soumis sont galement envahis par la tumeur, présentant les mêmes caractéres cytologiques.   The medullary areas demonstrate vascular, lymphatic tumor enboli. The lymphnodes submitted are invaded by the tumor, showing a similar cytological pattern.  
  L'urine soumise en per-opratoire, comporte de rares amas et cellules tumorales.   Urine from OR, reveals rare clusters of tumor cells.  

Diagnostic proposé







Carcinome neuro-endocrine bien différencié (T. carcinoïde) rénal avec métastases ganglionnaires para-caves


Proposed diagnosis:







Kidney neuro-endocrine carcinoma well differenciated (Carcinoid tumor) with lymphnode metastasis


Le bilan ralis n'objective pas d'extension tumorale ou de primitif par ailleurs.

Workup is negative for tumor extension, or primary elsewhere.  



1: Hinyokika Kiyo  2000 Oct;46(10):715-7  

A case of primary renal carcinoid tumor 

Maeda S, Itou H, Hasegawa T, Abe K, Katou N, Ikemoto I, Onodera S, Ohishi Y.

Department of Urology, Jikei University School of Medicine.

The patient was a 44-year-old woman in whom a mass of the right kidney was found when she underwent abdominal ultrasonography in a physical checkup. The computed tomography of abdomen and the magnetic resonance imaging of abdomen revealed a 3 x 3 cm solid mass at the right kidney. Excretory urograms showed a deformity of the right collecting system due to a mass lesion. The specimen of the resected tumor showed a cellular arrangement specific to carcinoid tumor and positive reaction of Grimelius stain. Electron microscopy confirmed the presence of dense-core granules in the tumor cells. Gastrointestinal investigation after the operation revealed no particular findings suggesting a carcinoid syndrome. No evidence of metastasis or recurrence was noted for 5 months after the operation. This was a very rare case of primary renal carcinoid tumor, representing the 12th case in the literature in Japan. 


2: Cent Afr J Med  1999 Dec;45(12):327-9  

Primary renal carcinoid: report of a case. 

Muthuphei MN. 

A 28 year old female presented with a right renal mass. She was treated with a radical nephrectomy. Histologically the tumour revealed the classical appearance of a carcinoid. The opposite kidney contained a multilocular cystic renal cell carcinoma. The histogenesis of a renal carcinoid is discussed.

3: Int J Urol  2000 May;7(5):184-8

Primary carcinoid tumor in a horseshoe kidney. 

Isobe H, Takashima H, Higashi N, Murakami Y, Fujita K, Hanazawa K, Fujime M, Matsumoto T. 

Department of Urology, Juntendo University School of Medicine, Tokyo, Japan.

A case of primary carcinoid tumor arising within a horseshoe kidney in a 51-year-old woman is reported. The tumor was found incidentally by computed tomography (CT) during a check-up for a suspected gall bladder polyp. Histologic, immunohistochemical and electron microscopic analyses of this tumor revealed features typical of carcinoid tumor. Primary carcinoid tumor of the kidney is extremely rare and only 32 cases were previously reported, including five cases in horseshoe kidneys. None of these five cases in horseshoe kidneys demonstrated any evidence of local or distant metastases and all were alive at the time of reporting without evidence of disease after up to 3 years of follow up. The present case, even with accompanying lymph nodal metastasis, also has had no evidence of local recurrence or distant metastasis for 3 years post operation. Primary carcinoid tumor arising within horseshoe kidneys appear to be more benign than those within non-horseshoe kidneys.


4: Eur J Gastroenterol Hepatol  1999 Sep;11(9):1059-60 

Renal cell carcinoma presenting as a pseudo-carcinoid tumour.

Tormey WP, Kay EW, Hickey DP.

Department of Pathology, Beaumont Hospital, Dublin, Ireland. 

A 38-year-old woman presented with hypertension and a renal cell carcinoma. Raised urinary 5-hydroxyindoleacetic acid values >300 micromol/l, predictive of carcinoid tumour, were found during the perioperative period. The values returned towards normal 2 weeks post-surgery. Immunohistochemical examination of the renal cell carcinoma with neuroendocrine markers was negative. Despite a diligent search, no carcinoid tumour could be identified and the patient was well 4 years later. There was no dietary or drug explanation for this pseudocarcinoid. 

5:Pathol Int  1998 May;48(5):412-5 

Renal small cell carcinoma (neuroendocrine carcinoma) without features of transitional cell carcinoma.

Masuda T, Oikawa H, Yashima A, Sugimura J, Okamoto T, Fujioka T.

Department of Pathology, School of Medicine, Iwate Medical University, Morioka, Japan. tmasuda@iwate-med.ac.jp

Seventeen cases of renal small cell carcinoma have been reported in the literature. Approximately half of the reported cases show combined features of transitional cell carcinoma. Presented herein is a case of renal small cell carcinoma in a 37-year-old Japanese male who had been treated for 10 years with famotidine for duodenal ulcer. He suffered from sudden-onset chest pain at presentation and myxoma of the right atrium was suspected. He was treated by atriotomy and a tumor was removed from the right atrium and pulmonary artery. Histological examination, however, revealed it to be small cell carcinoma. Accordingly, a radical operation was performed for the removal of a tumor found in the right kidney. Histological examination of the tumor confirmed the presence of renal small cell carcinoma without any features of transitional cell carcinoma. It is reported that long-term administration of an histamine 2 (H2) receptor antagonist may produce carcinoid tumors in rodents and enterochromaffin-like cell hyperplasia in humans. The possible relationship between neuroendocrine carcinoma and H2 receptor antagonist therapy is discussed.

6: J Surg Oncol  1998 Jun;68(2):113-9 

Renal carcinoid and horseshoe kidney: a frequent association of two rare entities--a case report and review of the literature. 

Begin LR, Guy L, Jacobson SA, Aprikian AG. 

Department of Pathology, McGill University, Sir Mortimer B. Davis Jewish General Hospital, Montreal, Canada.

We report the case of a primary renal carcinoid tumor associated with a horseshoe malformation in a 43-year-old man who presented with testicular pain. The tumor was centrally located and purely solid and had features ascribed to hindgut neuroendocrine neoplasia. The relative risk of developing a carcinoid tumor in a horseshoe kidney is estimated to be x82. 

7: Scand J Urol Nephrol  1997 Dec;31(6):575-8

Primary carcinoid tumour with ossification masquerading as calyx stone in a horseshoe kidney.

Lodding P, Hugosson J, Hansson G. 

Urology Division, University of Goteborg, Ostra Hospital, Sweden. 

A case of renal carcinoid tumour arising within a teratoma in a horseshoe kidney in a 23-year-old male is reported. The presenting symptom was abdominal pain, and bone tissue within the tumour was initially misinterpreted as a calyx stone, leading to unsuccessful attempts at extracorporeal shock-wave lithotripsia and percutaneous lithotripsia. This unusual tumour is over-represented in patients with horseshoe kidney and other congenital developmental renal defects.


8: Ultrastruct Pathol  1997 May-Jun;21(3):307-10 

Endocrine carcinoma of the kidney. 

Gurley SD, Luna MA, Ordonez NG, Mackay B. 

Department of Pathology, University of Texas M. D. Anderson Cancer Center,Houston 77030, USA.

A 4-cm mass in the right kidney of a 43-year-old female had an endocrine appearance by light microscopy and electron microscopy confirmed this impression, demonstrating the presence of numerous cytoplasmic granules of endocrine caliber. Unusual features were patchy immunoreactivity for chromogranin, and polarity of the granules within the neoplastic cells. Extrarenal extension and liver metastasis were documented.