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LETTER TO EDITOR  
Year : 2013  |  Volume : 56  |  Issue : 4  |  Page : 474-476
Splenogonadal fusion: Pathological features of a rare scrotal mass


1 Department of Pathology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
2 Department of Urology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

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Date of Web Publication18-Jan-2014
 

How to cite this article:
Duhli N, Venkatramani V, Panda A, Manojkumar R. Splenogonadal fusion: Pathological features of a rare scrotal mass. Indian J Pathol Microbiol 2013;56:474-6

How to cite this URL:
Duhli N, Venkatramani V, Panda A, Manojkumar R. Splenogonadal fusion: Pathological features of a rare scrotal mass. Indian J Pathol Microbiol [serial online] 2013 [cited 2019 Dec 14];56:474-6. Available from: http://www.ijpmonline.org/text.asp?2013/56/4/474/125387


Sir,

Splenogonadal fusion is a rare congenital anomaly in which the spleen is abnormally connected to the gonad. A 28-year-old, unmarried gentleman presented with left testicular pain and progressive swelling of 3 months duration. On examination, the left testis was enlarged 6 cm × 5 cm and hard, with tenderness of the left epididymis and cord. The right hemiscrotum was empty with no palpable testis in the inguinal region. Evaluation revealed normal serum levels of tumor markers. An ultrasound scan of the scrotum revealed a hypoechoic area 3.7 cm × 2.2 cm × 2 cm replacing most of the left testicular parenchyma with increased vascularity, suggestive of a testicular neoplasm. The right testis was at the level of the deep inguinal ring measuring 2.3 cm × 1 cm × 1.4 cm with no intra-testicular lesions or microlithiasis. A contrast enhanced computed tomography (CT) of the abdomen revealed thickening and enhancement of the left spermatic cord, with no intra-abdominal lymphadenopathy or liver metastasis. Chest X-ray was normal. The gentleman was referred for cryopreservation of sperm, however, semen analysis revealed him to be azoospermic, with a subnormal serum testosterone of 102 ng/dl.

A left high inguinal orchiectomy and right orchidopexy was performed. Intra-operatively the left testis was enlarged 6 cm × 6 cm, with a normal external appearance of the tunica vaginalis. The right testis was found lateral to the right inferior epigastric artery and was pexed at the pubic tubercle.

A specimen of left inguinal orchiectomy measuring 6 cm × 5.5 cm × 2.5 cm with attached spermatic cord, 8 cm in length and 1 cm in diameter at the resection margin was studied. The tunica vaginalis was unremarkable. Sectioning revealed testicular parenchyma replaced by a circumscribed lesion measuring 3.5 cm × 4.5 cm × 1.5 cm, with a dark brown congested firm cut surface [Figure 1]. The lesion reached up to the external surface, but there was no breach. Adjacent unremarkable testicular parenchyma was present. Sectioning of the spermatic cord revealed no significant abnormality.

Microscopic examination showed a well-circumscribed, encapsulated lesion composed of mature splenic tissue containing red and white pulp. Adjacent testicular tissue showed atrophic changes [Figure 2]. There was no evidence of malignancy. Sections of the spermatic cord and vas deferens showed no significant lesion. The final diagnosis was given as ectopic splenic tissue consistent with discontinuous splenogonadal fusion.
Figure 1: Cut secti on of specimen revealing a dark brown, circumscribed lesion adjacent to testi cular parenchyma

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Figure 2: (a) Microscopic examinati on showing atrophic testi cular parenchyma adjacent to splenic ti ssue (H and E, ×100). (b) View of the splenic ti ssue (H and E, ×100)

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The gentleman was taught regular testicular self-examination for his right testis and placed on testosterone replacement at discharge.

Splenogonadal fusion is a rare congenital anomaly in which there is a fusion of the splenic and gonadal anlagen. [1] Boestrom first reported it in 1883 and about 150 cases are described in the literature. [2] It usually presents on the left side (98%) and can affect all age groups. [1] The male to female ratio is approximately 15-16:1. [1],[3] This may not reflect its true incidence in females because the ovary cannot be as readily examined as the testis. [3] Patients may present with a non-tender scrotal mass or detected at surgery for cryptorchidism or inguinal hernia. [1],[3] About 25-33% are associated with other congenital anomalies, including limb bud deformities, micrognathia and cardiac anomalies. [3]

Putschar and Manion classified splenogonadal fusion into two types. [1],[3] The continuous type is characterized by connection of the spleen and the gonad by a fibrous cord. Splenic tissue can sometimes present as beads throughout the fibrous cord or it can be present at either ends. [1],[3] Most of the cases reported in females are of the continuous type and this type accounts for about 55% of all cases. The discontinuous type has no connection between the spleen and gonad. It usually presents as an encapsulated mass within the tunica vaginalis. [1],[3]

The spleen develops as a trilobed condensation of mesoderm splenic anlage, which is visible in the 5 week embryo as proliferation of surface peritoneal cells on the left side of the dorsal mesogastrium. [3] As the spleen develops it rotates into proximity with the urogenital fold. Fusion of the two components probably occurs during the 5 th-8 th week of gestation. [1],[3] During 8-10 weeks, the gonad starts descending caudally and it has been suggested that the splenic tissue could be found anywhere along the pathway of descent. [1] This path of descent is sometimes visibly demonstrated by a band of tissue (fibrous or splenic). The limb buds and mandible develop at the same time, accounting for the close association of splenogonadal fusion (almost exclusively the continuous type) with peromelia and micrognathia. [3] The initiating events of the fusion are currently unknown, but it has been hypothesized that a mild inflammation of the peritoneal surfaces proximal to spleen and gonadal ridges initiates the fusion of the two organs. [1],[3]

Pre-operative diagnosis of the condition is extremely rare. Varma et al. reported on the ultrasound and spiral CT findings of this condition. [2] On ultrasound, a well-circumscribed cord-like lesion can be seen extending from the left hypochondrium into the scrotum. The testicle is seen separately, inferiorly to the splenic tissue and has a normal echotexture. [2],[4] Extensive vascularity is seen, usually more than in a neoplasm, however this finding is non-specific and subjective. [4] Contrast-enhanced CT reveals tissue with a similar enhancement to the spleen along this cord. Eccentric vessels that originate from the splenic hilum can be traced along the length of this cord and are separate from the gonadal vessels. [2] No imaging findings have been described for discontinuous variant. A Tc99 sulfur colloid scan can detect accessory splenic tissue, however, an extremely high index of suspicion would be required and it is often not readily available. [2],[4] Three cases of testicular neoplasia have been reported in association with splenogonadal fusion all had associated cryptorchidism. [5]

In our case, the patient presented with a testicular mass, did not have any related congenital anomalies and no fibrous band was identified. Based on these features, a diagnosis of discontinuous type of splenogonadal fusion anomaly was given.

Splenogonadal fusion anomaly is a rare condition. Pre-operative diagnosis is very unlikely, especially in the discontinuous type. Awareness, a high index of suspicion and the use of frozen section can prevent unnecessary orchiectomies.

 
   References Top

1.Karaman MI, Gonzales ET Jr. Splenogonadal fusion: Report of 2 cases and review of the literature. J Urol 1996;155:309-11.  Back to cited text no. 1
    
2.Varma DR, Sirineni GR, Rao MV, Pottala KM, Mallipudi BV. Sonographic and CT features of splenogonadal fusion. Pediatr Radiol 2007;37:916-9.  Back to cited text no. 2
    
3.Balaji KC, Caldamone AA, Rabinowitz R, Ortenberg J, Diamond DA. Splenogonadal fusion. J Urol 1996;156:854-6.  Back to cited text no. 3
    
4.Patafio FM, Dufton JA, Walker DR. Case of the month #173: Splenogonadal fusion. Can Assoc Radiol J 2011;62:302-4.  Back to cited text no. 4
    
5.Imperial SL, Sidhu JS. Nonseminomatous germ cell tumor arising in splenogonadal fusion. Arch Pathol Lab Med 2002;126:1222-5.  Back to cited text no. 5
    

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Correspondence Address:
Vivek Venkatramani
Department of Urology, Christian Medical College and Hospital, Vellore - 632 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.125387

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