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Splenic-Gonadal Fusion: Case Report and Review of the Literature
0022-534 7/81/1264-0558$02.00$0
Vol. 126, October Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1981 by The Williams & Wilkins Co.
SPLENIC-GONADAL FUSION: CASE REPORT AND REVIEW OF THE LITERATURE LUIGI CECCACCI AND STEPHEN TOSI From the Departments of Pathology and Urology, Worcester Hahnemann Hospital, Worcester, Massachusetts
ABSTRACT
Splenic-gonadal fusion is a rare entity that presents as a testicular tumor. A case of this malformation is reported. We herein discuss its etiology, clinical presentation and associated malformations, and review the literature. A 29-year-old man was referred to our urology service because of a left testicular tumor. The patient had experienced discomfort in the left scrotum 6 weeks earlier. Examination revealed tenderness and induration of the upper pole of the testis and epididymis. The patient had received tetracycline and indomethacin without improvement. Re-examination revealed a 1 cm. tender mass at the upper pole of the left testis. Therefore, the patient was hospitalized for left inguinal exploration and possible left radical inguinal orchiectomy. History was unremarkable, there being no incidence of trauma or an undescended testis. The patient had had a recent viral illness and the pain had become worse after the illness. The family and social histories were not contributory. Physical examination was negative except for an indurated tender mass at the upper pole of the left testis. Laboratory diagnostic study, including human chorionic gonadotropin and a-fetoprotein, was within normal limits. A chest x-ray was negative. At exploration left orchiectomy was performed without biopsy. The abdomen was not explored. The patient did well and was discharged from the hospital 4 days postoperatively. The specimen consisted of a testis with 10 cm. of spermatic cord attached (see figure). At the upper pole of the testis and covered by albuginea there was a firm, gray-blue 1.3 cm. mass. On section the mass was well demarcated and composed ofredgray tissue with minute prominent follicle-like structures. A fibrous cord arose from its upper pole and ran proximally within the spermatic cord. Histologic sections revealed normal splenic tissue. The cord was composed of vascularized fibrofatty tissue.
graphically close together, that is before 8 weeks, when gonadal descent begins. Clinically, splenic-gonadal fusion usually is an incidental finding. However, scrotal tenderness has been reported after malaria attacks, systemic infections or long marches. There also is a much higher clinical incidence in patients with cryptorchidism. Approximately 25 per cent of the cases reported to date have been associated with cryptorchid testes. In addition, a number of cases of splenic-gonadal fusion have been reported to be associated with peromelus and micrognathia. Only 1 case has been diagnosed correctly before an operation. 3 In our review of the literature we noted that our case most resembles that described by Keizur (case 2), in that the ectopic tissue in both cases was found within the tunica albuginea in
COMMENT
Splenic-gonadal fusion is a rare malformation that consists of an abnormal connection between the spleen and the left gonad. The first detailed report of this anomaly was by Pommer in 1889.1 In the continuous variant there is a cord that connects the spleen and the scrotal ectopic splenic tissue .. The connecting -cord sometimes is composed of pure splenic tissue or of vascularized fibrous tissue, or is a beaded combination. In the discontinuous variant. the connection is lost and the ectopic splenic tissue, thus, appears as a scrotal accessory spleen. To date, there have been 18 cases reported of the continuous and 12 of the discontinuous type. 2 Our case constitutes the nineteenth one of the continuous variety. Of the total of 31 c;1;1ses reported of both variants only 3 have occurred in female patients and these have all involved the left ovary and have been of the continuous type. The cause of splenic-gonadal fusion is obscure. It is speculated that it must take place at a stage of the embryonal development when the splenic and gonadal anlages are topo-
Mass of splenic tissue at upper pole of testis. Note fibrous cord arising from its upper pole.
the upper pole of the left testis. 4 In many of the other cases reported the ectopic splenic tissue is found along the cord, in the epididymis or just outside the tunica albuginea. In these cases it may be possible to biopsy or excise the benign splenic tissue. However, it is considered good surgical practice to perform an inguinal orchiectomy without biopsy in all cases in which a mass occurs within the tunica albuginea and this was the approach taken in our case. Orchiectomy has been advocated because benign tumors of the testes are rare, accounting for <1 per cent of all testicular neoplasms. 5 It certainly is better to remove an occasional benign lesion than to compromise a good cancer operation by biopsying a mass that is contained within the tunica albuginea. For the most part benign lesions of the testis are cystseither epidermoid or simple. 6' 7 Splenic-gonadal fusion adds yet
Accepted for publication November 7, 1980. 558
SPLENIC-GONADAL FUSION
another diagnostic consideration in male patients with testicular masses. REFERENCES 1. Pommer, G.: Verwachsung des linken kryptorchischen Hodens und
Nebenhodens mit der Milz in einer Missgeburt mit zahlreichen Bildungsdefecten. Ber. d. naturw.-med. Ver. in Innsbruck, 18: 144, 1888-1889.
2. Putschar, W. G. J. and Manion, W. C.: Splenic-gonadal fusion. Amer. J. Path., 32: 15, 1956.
559
3. Kadlic, T.: Nebenmilz in einer angeborenen Skrotalhernie. Zentralbl. f. allg. Path. u. path. Anat., 81: 49, 1943.
4. Keizur, L. W.: Accessory spleen in scrotum: report of two cases. J. Urol., 68: 759, 1952. 5. Goli, V. R., Shepherd, R. R., Hayman, W. P. and Ramzy, I.: Epidermoid cyst of the testes. J. Urol., 123: 129, 1980. 6. Price, E. B., Jr.: Epidermoid cysts of the testis: a clinical and pathologic analysis of 69 cases from the Testicular Tumor Registry. J. Urol., 102: 708, 1969. 7. Tosi, S. E. and Richardson, J. R., Jr.: Simple cyst of the testis: case report and review of the literature. J. Urol., 114: 473, 1975.
Vol. 126, October Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1981 by The Williams & Wilkins Co.
SPLENIC-GONADAL FUSION: CASE REPORT AND REVIEW OF THE LITERATURE LUIGI CECCACCI AND STEPHEN TOSI From the Departments of Pathology and Urology, Worcester Hahnemann Hospital, Worcester, Massachusetts
ABSTRACT
Splenic-gonadal fusion is a rare entity that presents as a testicular tumor. A case of this malformation is reported. We herein discuss its etiology, clinical presentation and associated malformations, and review the literature. A 29-year-old man was referred to our urology service because of a left testicular tumor. The patient had experienced discomfort in the left scrotum 6 weeks earlier. Examination revealed tenderness and induration of the upper pole of the testis and epididymis. The patient had received tetracycline and indomethacin without improvement. Re-examination revealed a 1 cm. tender mass at the upper pole of the left testis. Therefore, the patient was hospitalized for left inguinal exploration and possible left radical inguinal orchiectomy. History was unremarkable, there being no incidence of trauma or an undescended testis. The patient had had a recent viral illness and the pain had become worse after the illness. The family and social histories were not contributory. Physical examination was negative except for an indurated tender mass at the upper pole of the left testis. Laboratory diagnostic study, including human chorionic gonadotropin and a-fetoprotein, was within normal limits. A chest x-ray was negative. At exploration left orchiectomy was performed without biopsy. The abdomen was not explored. The patient did well and was discharged from the hospital 4 days postoperatively. The specimen consisted of a testis with 10 cm. of spermatic cord attached (see figure). At the upper pole of the testis and covered by albuginea there was a firm, gray-blue 1.3 cm. mass. On section the mass was well demarcated and composed ofredgray tissue with minute prominent follicle-like structures. A fibrous cord arose from its upper pole and ran proximally within the spermatic cord. Histologic sections revealed normal splenic tissue. The cord was composed of vascularized fibrofatty tissue.
graphically close together, that is before 8 weeks, when gonadal descent begins. Clinically, splenic-gonadal fusion usually is an incidental finding. However, scrotal tenderness has been reported after malaria attacks, systemic infections or long marches. There also is a much higher clinical incidence in patients with cryptorchidism. Approximately 25 per cent of the cases reported to date have been associated with cryptorchid testes. In addition, a number of cases of splenic-gonadal fusion have been reported to be associated with peromelus and micrognathia. Only 1 case has been diagnosed correctly before an operation. 3 In our review of the literature we noted that our case most resembles that described by Keizur (case 2), in that the ectopic tissue in both cases was found within the tunica albuginea in
COMMENT
Splenic-gonadal fusion is a rare malformation that consists of an abnormal connection between the spleen and the left gonad. The first detailed report of this anomaly was by Pommer in 1889.1 In the continuous variant there is a cord that connects the spleen and the scrotal ectopic splenic tissue .. The connecting -cord sometimes is composed of pure splenic tissue or of vascularized fibrous tissue, or is a beaded combination. In the discontinuous variant. the connection is lost and the ectopic splenic tissue, thus, appears as a scrotal accessory spleen. To date, there have been 18 cases reported of the continuous and 12 of the discontinuous type. 2 Our case constitutes the nineteenth one of the continuous variety. Of the total of 31 c;1;1ses reported of both variants only 3 have occurred in female patients and these have all involved the left ovary and have been of the continuous type. The cause of splenic-gonadal fusion is obscure. It is speculated that it must take place at a stage of the embryonal development when the splenic and gonadal anlages are topo-
Mass of splenic tissue at upper pole of testis. Note fibrous cord arising from its upper pole.
the upper pole of the left testis. 4 In many of the other cases reported the ectopic splenic tissue is found along the cord, in the epididymis or just outside the tunica albuginea. In these cases it may be possible to biopsy or excise the benign splenic tissue. However, it is considered good surgical practice to perform an inguinal orchiectomy without biopsy in all cases in which a mass occurs within the tunica albuginea and this was the approach taken in our case. Orchiectomy has been advocated because benign tumors of the testes are rare, accounting for <1 per cent of all testicular neoplasms. 5 It certainly is better to remove an occasional benign lesion than to compromise a good cancer operation by biopsying a mass that is contained within the tunica albuginea. For the most part benign lesions of the testis are cystseither epidermoid or simple. 6' 7 Splenic-gonadal fusion adds yet
Accepted for publication November 7, 1980. 558
SPLENIC-GONADAL FUSION
another diagnostic consideration in male patients with testicular masses. REFERENCES 1. Pommer, G.: Verwachsung des linken kryptorchischen Hodens und
Nebenhodens mit der Milz in einer Missgeburt mit zahlreichen Bildungsdefecten. Ber. d. naturw.-med. Ver. in Innsbruck, 18: 144, 1888-1889.
2. Putschar, W. G. J. and Manion, W. C.: Splenic-gonadal fusion. Amer. J. Path., 32: 15, 1956.
559
3. Kadlic, T.: Nebenmilz in einer angeborenen Skrotalhernie. Zentralbl. f. allg. Path. u. path. Anat., 81: 49, 1943.
4. Keizur, L. W.: Accessory spleen in scrotum: report of two cases. J. Urol., 68: 759, 1952. 5. Goli, V. R., Shepherd, R. R., Hayman, W. P. and Ramzy, I.: Epidermoid cyst of the testes. J. Urol., 123: 129, 1980. 6. Price, E. B., Jr.: Epidermoid cysts of the testis: a clinical and pathologic analysis of 69 cases from the Testicular Tumor Registry. J. Urol., 102: 708, 1969. 7. Tosi, S. E. and Richardson, J. R., Jr.: Simple cyst of the testis: case report and review of the literature. J. Urol., 114: 473, 1975.