23 April 2014 - Case of the Week #309
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Thanks to Drs. Joseph Fullmer, SUNY Upstate Medical University (USA) and Wei Huang, University of Wisconsin Hospital and Clinics (USA), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.
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• Patil: McGraw-Hill Specialty Board Review Anatomic Pathology
• Dabbs: Diagnostic Immunohistochemistry
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• Amin: Urological Pathology
• Lakhani: WHO Classification of Tumours of the Breast
• Montgomery: Biopsy Interpretation of theGastrointestinal Tract Mucosa: Volume 1: Non-Neoplastic
• Montgomery: Biopsy Interpretation of theGastrointestinal Tract Mucosa: Volume 2: Neoplastic
• Fletcher: WHO Classification of Tumours of Soft Tissue and Bone
• Fang: Cytopathology Review
• Bostwick:Urologic Surgical Pathology
Case of the Week #309
A 16 year old boy presented with a chronic left testicular mass, with no recent change in size. Serum tumor markers were normal. Imaging revealed a homogenous, 3.6 cm mass adjacent to the left testis. It did not have the classic appearance of testicular cancer and may have represented polyorchidism, but a malignant rhabdomyosarcoma or an infectious cause could not be excluded without biopsy or excision. A left radical orchiectomy was performed.
What is your diagnosis?
Splenogonadal fusion syndrome
The microscopic sections show histologically normal splenic tissue adherent to testicular parenchyma. Splenogonal fusion syndrome, see also Spleen chapter, is a rare congenital condition, usually presenting before age 20, with 50%+ present before age 10. In males, in occurs only in the left testis. It can also occur in females (see Ovary nontumor chapter).
The fusion is termed either continuous (attaching to spleen) or discontinuous (intrascrotal splenic nodules attached to testis, spermatic cord, epididymis, appendix of testis or appendix of epididymis). The continuous form is associated with limb-bud anomalies such as peromelia (severe congenital anomalies of extremities identical to thalidomide embryopathy) and micrognatia (small jaw) (Urology 1988;32:521, OMIM #183300). The discontinuous type is rarely associated with cardiac defects.
Gross examination shows splenic tissue well demarcated from the gonad / testis. Microscopically, as in this case, normal splenic parenchyma is identified, but there may also be fibrosis, thrombi, calcification, fatty degeneration and hemosiderin. The testicular tissue may be normal, or have atrophy or fibrosis of seminiferous tubules, increased Leydig cells, or thrombosis of spermatic vessels.
The differential diagnosis includes lymphoma or a germ cell tumor.
Optimal treatment is excision of the ectopic splenic tissue only, to prevent testicular atrophy, torsion or infarction, and preserve fertility (Ann R Coll Surg Engl 2006;88:163, Exp Ther Med 2013;6:816).
Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
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