Splenogonadal fusion associated with delayed skeletal maturation: A case report and review of the literature

In this report of a 13-year old boy, we describe the first case of splenogonadal fusion (SGF) associated with growth hormone deficiency, delayed skeletal maturation and stunting, and provide a review of the literature on SGF based on a search in major medical indexes using the descriptors “splenogonadal”, “splenogonadal fusion” and “congenital anomalies of the spleen”. Predominant in males (15:1), SGF is a rare congenital anomaly with only around 200 cases described involving the presence of splenic tissue in the gonads and, in some cases, a fibrous cord connecting the two structures. SGF may be associated with severe limb anomalies, micrognathism and testicle cancer. Despite frequently increased testicle volume, the condition is usually asymptomatic. Knowledge of SGF is important in the differential diagnosis of malignancies, avoiding unnecessary orchiectomy. Nevertheless, SGF has been observed in association with malignant tumors, especially in patients with cryptorchidism

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References

[1]Putschar WG, Manion WC. SplenicGonadal Fusion. Am J Pathol. 1956;32(1):15–33.

[2]Kumar S, Jayant K, Agrawal S, Parmar KM, Singh SK.. A rare case of continuous type splenogonadal fusion in a young male with primary infertility. Case Rep Urol. 2014; 2014:796761.

[3]Arancio M, Marchetti C, Landi G, Mina A, Delsignore A, Marcato M, Martinengo C. [A rare case of female splenogonadal fusion]. Urologia. 2009; 76(2):137-9.

[4]Varga I, Galfiova P, Adamkov M, Danisovic L, Polak S, Kubikova E, Galbavy S. Congenital anomalies of the spleen from an embryological point of view. Med SciMonit. 2009;15(12):RA269-76.

[5]Pauli RM, Greenlaw A. Limb deficiency and splenogonadal fusion. Am J Med Genet. 1982; 13(1):81-90.

[6]Sneath WA. An Apparent Third Testicle consisting of a Scrotal Spleen. J Anat Physiol. 1913;47(Pt 3):340-2.

[7]von Hochstetter, A. Milzgewebeimlinken Ovarium des linken Individual teiles Einesmenschlichen Thoracopagus. Virchows Arch. f. path. Anat. 1953-54, 324, 36-54.

[8]Cortes D, Thorup JM, Visfeldt J. The pathogenesis of cryptorchidism and splenogonadal fusion: a new hypothesis. Br J Urol. 1996;77(2):285-90.

[9]Le Roux PJ, Heddle RM. Splenogonadal fusion: is the accepted classification system accurate? BJU Int. 2000; 85(1):114-5.

[10]Khairat AB, Ismail AM. Splenogonadal fusion: case presentation and literature review. J Pediatr Surg. 2005;40(8):1357-60.

[11]Lopes RI, de Medeiros MT, Arap MA, Cocuzza M, Srougi M, Hallak J. Splenogonadal fusion and testicular cancer: case report and review of the literature. Einstein (Sao Paulo). 2012;10(1):92-5.

[12]Imperial SL, Sidhu JS. Nonseminomatous germ cell tumor arising in splenogonadal fusion. Arch Pathol Lab Med. 2002;126(10):1222-5.

[13]Thomsen BM, Wierod FS, Rasmussen KC. Combined malignant testicular tumor and splenogonadal fusion. A case story. Scand J Urol Nephrol. 1997;31(4):393-5.

[14]Falkowski WS, Carter MF. Splenogonadal fusion associated with an anaplastic seminoma. J Urol. 1980; 124(4):562–4.

[15]Karaman MI, Gonzales ET Jr. Splenogonadal fusion: report of 2 cases and review of the literature. J Urol. 1996;155(1):309-11.

[16]Patel RV. Splenogonadal fusion J Pediatr Surg. 1995; 30(6):873-4.

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