Urology Annals
About UA | Search | Ahead of print | Current Issue | Archives | Instructions | Online submissionLogin 
Urology Annals
  Editorial Board | Subscribe | Advertise | Contact
Users Online: 510   Home Print this page  Email this page Small font size Default font size Increase font size


 
Table of Contents
CASE REPORT
Year : 2019  |  Volume : 11  |  Issue : 2  |  Page : 211-213  

Splenorenal fusion mimicking renal cancer: One case report and literature review


1 Department of Urology, La Croix Saint Simon Hospital, Paris, France; Department of Surgery, Faculty of Medicine in Rabigh, King Abdulaziz University, Rabigh, Saudi Arabia
2 Department of Urology, La Croix Saint Simon Hospital, Paris, France
3 Department of Pathology, Tenon Hospital, Paris, France

Date of Submission16-Apr-2018
Date of Acceptance26-Dec-2018
Date of Web Publication4-Apr-2019

Correspondence Address:
Dr. Ahmed S Zugail
Department of Urology, La Croix Saint Simon Hospital, 125 Rue d'Avron, 75020 Paris, France

Login to access the Email id


DOI: 10.4103/UA.UA_47_18

Rights and Permissions
   Abstract 


Splenorenal fusion is an extremely rare benign entity. This abnormality is presented in a case of a 29-year-old-male patient. We discuss the distinction between this condition and renal splenosis and their embryology. The course of this condition and modalities of investigation including radiological imaging, management, and pitfalls are reviewed.

Keywords: Accessory spleen, ectopic spleen, splenic heterotopia, splenorenal fusion, splenosis


How to cite this article:
Zugail AS, Ahallal Y, Comperat EM, Guillonneau B. Splenorenal fusion mimicking renal cancer: One case report and literature review. Urol Ann 2019;11:211-3

How to cite this URL:
Zugail AS, Ahallal Y, Comperat EM, Guillonneau B. Splenorenal fusion mimicking renal cancer: One case report and literature review. Urol Ann [serial online] 2019 [cited 2019 Apr 19];11:211-3. Available from: http://www.urologyannals.com/text.asp?2019/11/2/211/254272




   Introduction Top


Renal neoplasms are common and are easily diagnosed with the widespread use of the various imaging techniques. However, literature is scarce regarding the lesions mimicking renal neoplasms as subsequent histopathology and/or surgery prove a wrong radiologic diagnosis. These masses may be composed of normal or benign renal tissue (renal pseudotumors), but atypical renal masses may be resected unnecessarily for the concern of renal malignancy. Of these tumors, an accessory spleen in the form of a splenorenal fusion. Gonzalez-Crussi et al. first reported splenorenal fusion in 1977. It presents in patients with no history of splenic trauma or splenectomy and can mimic primary renal neoplasms or metastatic disease.[1] It usually involves the left kidney, although the right side location has also been documented.[2]


   Case Report Top


We report a 29-year-old male patient with no medical, surgical, or traumatic history who was referred by his family physician to the urology outpatient clinic for an unexplained increased urinary frequency explored subsequently by an ultrasound showing a left upper pole kidney tumor. His blood work was normal. A computerized tomography (CT) scan revealed a 4-cm homogenous but hyperdense lesion located at the left kidney's upper pole [Figure 1] and a normo-anatomic spleen. Furthermore, a renal biopsy was noncontributive displaying richly vascularized granulation tissue and inflammatory cell infiltrates. After discussing the case in a multidisciplinary team meeting, the patient underwent a laparoscopic partial nephrectomy to manage a hypothetical malignancy suspected because of the large size of the tumor. The pathology report revealed a capsulated accessory spleen inside the kidney measuring 25 mm independent of the renal parenchyma [Figure 2]. The patient's renal function remained normal at a 1-year follow-up.
Figure 1: Coronal reconstructed computed tomography scan images. Left side: Showing the tumor (arrow head) with no contrast. Right side: Enhancement of the renal mass in the arterial phase (arrow head)

Click here to view
Figure 2: Histology of renal tissue (right side), which can be easily distinguished from splenic tissue (left side) separated by the capsule of the accessory spleen (H and E, ×40)

Click here to view



   Discussion Top


During the fifth embryonic week, the spleen arises as the result of a proliferation of peritoneal cells of the dorsal mesogastrium from mesenchymal cells that migrate between the leaves of the mesentery.[3] A failure of fusion of the mesenchymal cells results in accessory spleens.[4] In addition, as an outcome of the rotation of the embryonic gut, the splenic primordium lies close to the left mesonephric ridge.[5] There are two conflicting theories of spleno-visceral fusion.[6] The first one is the continuous theory that describes a band of tissue that adheres the spleen with the organ between the 7th and 8th week of gestation. The second theory is the discontinuous type that postulates migration of the splenic cells caudally to reach the developing mesonephric ridge and retroperitoneum, where fusion takes place. The only objection to this theory is that crossing of splenic tissue to the right side would be expected since there is no obstacle in the retroperitoneum.[2] The forms of ectopic splenic tissue (splenules) can be found as two types: accessory spleens and splenosis. Accessory spleens are congenital and arise from the left side of the dorsal mesogastrium during the embryological period of development.[5] Splenosis refers to splenic tissue that has been autotransplanted in a heterotopic location after splenic traumatism or splenectomy as defined by Beneke in 1910. We consider spleno-organ fusion a subtype of accessory spleens. Accessory spleens always conform to their embryological topographical limits even when reported within the pancreas, kidney, and scrotum and as an adnexal mass. Splenosis can be found in any location. Most usual locations (most frequent to less) are the peritoneum, omentum, and mesentery.[4] Splenosis has been reported in the pericardium, subcutaneous tissue, and even the brain. Epidemiologically, accessory spleens are found in up to 30% of the population.[2] The incidence of splenosis remains unknown, but its occurrence seems to be highest in males of young age that may reflect the increased capability of splenic cells to implant or it may simply represent the increased incidence splenic injury in young males. Most patients with splenic heterotopia are usually asymptomatic and found incidentally. Symptomatic patients may present with pain caused by infarction, intestinal obstruction due to the adhesive bands of the splenic implants, gastrointestinal hemorrhage, hydronephrosis caused by a mass compressing the ureter, as well as an enlarging abdominal mass. Patients can also present with symptoms of hypersplenism.[1] Radiological studies by ultrasound or CT scan and invasive laparotomies are all well-documented ways of incidental uncovering of ectopic spleens. Ultrasound examination will show round hypoechoic masses with homogenous echogenicity, and the nodules show a rim of a thin hyperechoic wall. CT scans show a uniform soft tissue density with homogenous, hypodense, contrast enhancement that differs clearly from the normal heterogeneity of the spleen.[7] None of these techniques is specific or sensitive. Recent reports have shown that ferumoxide-enhanced magnetic resonance imaging is a novel technique for diagnosing splenosis.[8] Ferumoxides are superparamagnetic iron oxides that are removed from the circulation by the reticuloendothelial system making them tissue specific. The gold standard diagnostic modality of choice is nuclear scintigraphy (splenic scan). The99mtechnetium (99m Tc) sulfur colloid test of the liver and spleen was first used to diagnose accessory spleens and/or splenosis due to its ability to localize the reticuloendothelial system.[3],[5] Moreover, scintigraphy using99m Tc heat-damaged red blood cells (RBCs) or indium111-labeled platelets is more sensitive and specific for splenic uptake, making these tests the current diagnostic tools of choice.[9] RBC scintigraphy was shown to be more sensitive than the sulfur colloid test in early splenosis, situations where minimal splenic tissue is present, functional hyposplenism, or poor splenic uptake or overlapping of the liver and spleen, causing poor visualization of splenic tissue.[4] Radiological-guided biopsy has been reported but was not useful for the diagnosis so far. Cytological smears alone may be insufficient since RBCs and white blood cells will appear on the slides and distinguishing the lesion from an organizing hematoma or a chronic inflammation can be cumbersome for pathologists, if not impossible.[5] The histology of an accessory spleen is identical to that of the spleen. Splenosis' tissue usually reveals bizarre architecture without a hilum and scantily formed capsules and tissue of any shape or size. Reports have described the tissue as lacking trabecular structures or having less elastic tissue than a normal one and presenting ill-formed or scarce white pulp with usual appearing red pulp. Two cases of splenosis described by Carr and Turk showed that the histology and immunohistochemistry were indistinguishable from that of a normal spleen.[10] Most reported cases of splenorenal fusion underwent total nephrectomy. To our knowledge, we report the first case treated by tumorectomy, thus preserving the kidney. In conclusion, splenorenal fusion is a congenital anomaly in which splenic tissue is present within the renal capsule. This condition mimics primary or secondary tumors on imaging. Although found incidentally by imaging techniques, it can present with symptoms. The diagnosis can be established with99m Tc-sulfur-colloid scan or99m Tc-labeled, denatured RBC scan. Recognition of this entity is important to avoid unnecessary surgery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.

Acknowledgments

We acknowledge Ms. Alexandra Lonchamp, Urology Secretary, La Croix Saint Simon Hospital, Paris, France, for her help in collecting some data.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Gonzalez-Crussi F, Raibley S, Ballantine TV, Grosfeld JL. Splenorenal fusion: Heterotopia simulating a primary renal neoplasm. Am J Dis Child 1977;131:994-6.  Back to cited text no. 1
    
2.
Rosenthal JT, Bedetti CD, Labayen RF, Christy WC, Yakulis R. Right splenorenal fusion with associated hypersplenism. J Urol 1981;126:812-4.  Back to cited text no. 2
    
3.
Dodds WJ, Taylor AJ, Erickson SJ, Stewart ET, Lawson TL. Radiologic imaging of splenic anomalies. AJR Am J Roentgenol 1990;155:805-10.  Back to cited text no. 3
    
4.
Sty JR, Conway JJ. The spleen: Development and functional evaluation. Semin Nucl Med 1985;15:276-98.  Back to cited text no. 4
    
5.
Forino M, Davis GL, Zins JH. Renal splenic heterotopia, a rare mimic of renal neoplasia: Case report of imaging and fine-needle aspiration biopsy. Diagn Cytopathol 1993;9:565-9.  Back to cited text no. 5
    
6.
Putschar WG, Manion WC. Splenicgonadal fusion. Am J Pathol 1956;32:15-33.  Back to cited text no. 6
    
7.
Maillard JC, Menu Y, Scherrer A, Witz MO, Nahum H. Intraperitoneal splenosis: Diagnosis by ultrasound and computed tomography. Gastrointest Radiol 1989;14:179-80.  Back to cited text no. 7
    
8.
Berman AJ, Zahalsky MP, Okon SA, Wagner JR. Distinguishing splenosis from renal masses using ferumoxide-enhanced magnetic resonance imaging. Urology 2003;62:748.  Back to cited text no. 8
    
9.
Kwok CM, Chen YT, Lin HT, Su CH, Liu YS, Chiu YC, et al. Portal vein entrance of splenic erythrocytic progenitor cells and local hypoxia of liver, two events cause intrahepatic splenosis. Med Hypotheses 2006;67:1330-2.  Back to cited text no. 9
    
10.
Carr NJ, Turk EP. The histological features of splenosis. Histopathology 1992;21:549-53.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
     Introduction
     Case Report
     Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed71    
    Printed9    
    Emailed0    
    PDF Downloaded21    
    Comments [Add]    

Recommend this journal