International Urology and Nephrology 34: 373–375, 2002. © 2003 Kluwer Academic Publishers. Printed in the Netherlands.
373
Solitary neurofibroma of the spermatic cord Charalampos Deliveliotis, Stephen Albanis, Andreas Skolarikos, John Varkarakis, Vasilios Protogerou, Nikolaos Tamvakis & Eugene Alargof Department of Urology, Sismanoglio Hospital, Medical School of Kapodistrian, University of Athens, Greece
Abstract. We report a case of solitary neurofibroma of the spermatic cord. Such benign tumors arise from perineural and Schwann cells and may be located in various parts of the body, but are rarely observed in the spermatic cord. No clinical or laboratorial manifestations of von Recklinghausen disease (Neurofibromatosis) were identified. Distinct criteria have been established for a diagnosis of von Recklinghausen disease, so that a solitary neurofibroma may not represent this complex. Only a little number of solitary neurofibromas of the spermatic cord are reported in the literature. Key words: Neurofibroma, Spermatic cord
Case report A 74-year-old white Caucasian male, 90 kgs of body weight and of good general condition, presented in January 2002, complaining of an incidental observatin-palpation of a mass at his right inguinal area. Our physical examination indicated a palpable, rubbery hard, painless, ovular mass, seized approximately 6 × 4 cm. The patient was under medication for hyperurichemia (alopourinol 100 mg daily) and for hypertension (indapamide 2,5 mg and fosinapril 10 mg daily). His personal and family medical history were free of congenital or hereditary diseases. During his hospitalization he was submitted to the routine check up as well as to more detailed blood tests. All were normal. Ultra sound of the genitalia and inguinal areas showed a hyperechogenic lesion at his right inguinal area. Computed tomography of upper and lower abdomen and retroperitoneal space diagnosed a propable inguinal hernia. The differential diagnosis included hernia and tumors originated from the muscular, neural, fatty tissue of the cord and from regional lymph nodes. We proceeded to open surgical exploration, with spinal anesthesia. A right inguinal incision was performed. Our dissection presented an oval, whitish, rubbery hard tumor which occupied a large proportion
of the spermatic cord; the lesion was firmly attached to the cord a frozen section microscopic examination indicated sarcoma(!). A radical orchidectomy was performed. The sample was photographed (Figure 1) and sent for histopathological evaluation. The patient had an uneventful postoperative course. The histopathological report wrote of a normal testis and adnexas of it. The tumor seized 4×4×1 cm and occupied most of the excised spermatic cord. Macroscopically the tumor consisted of a moderate number of cells, was non-encapsulated and had a double morphology. There were areas of moderate cellular presence on collagenious background, also with a small amount of mucous. The cells had ellipsoid, atractoid and spindle-like shapes. They did not show atypia or mitotic activity. Other areas had also a small number of cells on myxoid background. Cells in those areas were ovular or atractoid in shape and showed some nucleous atypia due to ischemic degeneration. There was also no aitotic activity noticed. The whole cellular and background structure was that of a neurofibroma (Figure 2). Immunoistochemical staining with S-100 protein presented antigen expression in only a small number of cells; a finding which also suggests the diagnosis above. Pathology of the testis showed no abnormality whatsoever.
374
Figure 1.
Figure 2.
375 Discussion Neurofibroma is a benign tumor of the nerve sheath originating from the Schwann cells. It can be solitary or multiple. It is also characterised by hyperplasia of the intraneural collagene fibres. Both solitary and multiple neurofibromas have similar histopathological appearance. Solitary tumor can occur in neurofibromatosis and may be its only clinical manifestation, exemplifying a type of neurofibromatosis known as the “forme frustes”. The exact origin of the tumor was not clear. Potentially it might arise from the genitofemoral or the ilioinguinal nerve or even from smaller nerve axons and degenerised its occupying area. Fifty percent of the patients produce symptoms, in relation with tumor seize and location. Differential diagnosis between neurofibroma, neurilemmoma and other tumors is accomplished by using S-100 protein staining (negative for neurofibromas), electron microscopy, computed tomography, chromosomal, immunoistochemical and flowcytometrical tests. The similarity to sarcoma in frozen section biopsy must also be underlined and it has to do with the rare finding of such tumor as well as with the fact that some neurofibromas show hyperchromatism and irregularity in seize and shape of nuclei. The indicated treatment is local surgical excision. Frozen section microsopic examination must be performed at the time of operation o ascertain whether the tumor is benign or malignant. Bibliography suggests that solitary neurofibromas do not recur or become malignant.
Revisit of the patient four months after his operation showed no recurrence and his present status is excellent.
References 1. Sanchez Chapado M, Aranda Lassa JM, Gaballero Gomaz M et al. Tumores del cordon: a portacion de un neurofibroma. Arch Esp Urol (Jan–Feb) 1988; 41: 23–26. 2. Schulte TL, McDonald JR, Priestley JT. Tumors of the spermatic cord, report of a case of neurofibroma. JAMA 1939; 112: 2405. 3. Sheela S, Riccardi VM, Ratner N. Angiogenic and invasive properties of neurofibroma Schwann cells. J Cell Biol 1990; III: 645. 4. Potter GK, Feldman JS. Neoplasms of the peripheral nervous system. Clin Pediatr Med Surg 1990; 7: 141. 5. Dwosh J, Mininberg D, Schlossberg S, Peterson P. Neurofibroma involving the penis in a child. J Urol 1984; 132: 988. 6. Papademitriou KS. Systemic Pathology: Peripheral Nerves’ Disorders. Athens: Litsas Publications, 1990: 803. 7. Yoshimura K, Maeda O, Saiki S et al. Solitary neurofibroma of scrotum. J Urol 1990; 143: 823. 8. Young RH, Scully RE. Miscellaneous neoplasms and non neoplastic lesions. In: Talerna A, Roth LM, eds. Pathology of the Testis and Its Adnexa. Contemporary Issues in Surgical Pathology, Vol. 7. New York: Churchill Livingstone, 1986: chap. 5. 9. Livolsi V, Schiff M. Myxoid neurofibroma of the testis. J Urol 1977; 118: 341. 10. Issa MM, Vagol R, Tsang D. Intrascotal neurofibromas. Urology 1993; 41: 350. Address for correspodnence: Associate Professor Charalampos Deliveliotis 4, Monis Petraki Street, Zip Code: 115 21 Kolonaki, Athens, Greece Phone: 0932 274744, +10 7229880; Fax: +10 7215448, +10 6137325