Grand Rounds Solid Testicular Mass in a 63-Year-old Man Chandy Ellimoottil, Sarah Perz, Vikas Mehta, Thomas M. Turk, and John Wheeler
THE CASE
A
63-year-old man presented to our clinic with complaints of a right testicular mass. This mass was noted 3 weeks earlier while the patient was performing self-examination in the shower. He reported some aching but denied frank pain. He also denied voiding symptoms, gross hematuria, penile discharge, or weight loss; however, he reported rare night sweats. His urologic history was significant only for a right inguinal hydrocele repair, at age 13, and a vasectomy. His medical and surgical history was significant for coronary artery bypass, hypothyroidism, hypertension, and type 2 diabetes. He had a remote 38 pack-year smoking history. On physical examination, the patient had a large fixed right testicular mass. Complete blood cell count and blood biochemistry results were within normal limits. Serum alpha-fetoprotein, b-human chorionic gonadotropin, and lactate dehydrogenase levels were not elevated. Scrotal ultrasound revealed small bilateral epididymal cysts, small bilateral hydroceles, and a normal-appearing left testicle. A heterogeneous. 6-cm well-circumcised solid mass was present on the inferior pole of the right testicle (Fig. 1). The mass appeared to have a capsule, although this finding could not be definitively determined from the ultrasound alone. Chest x-ray imaging did not show any pulmonary nodules. Computed tomography of the abdomen and pelvis with contrast revealed small lymph nodes in the periaortic, internal and external iliac chain, and in the mesentery. The largest node was in the periaortic region, measuring 8 mm, while the other nodes were in a 5- to 7-mm range.
DIFFERENTIAL DIAGNOSIS Most solid testicular tumors are malignant. Considerations for a malignant testicular mass are germ cell tumors, including seminoma and nonseminomatous germ cell tumor, as well as non-germ cell tumors, including Leydig cell, Sertoli cell, and granulosa cell tumors. In an older man, lymphoma, spermatocytic seminoma, and a metastatic lesion are on the top of the differential. Financial Disclosure: The authors declare that they have no relevant financial interests. From the Departments of Urology and Pathology, Loyola University Medical Center, Maywood, Illinois, and the Edward Hines Veterans Hospital, Hines, Illinois Reprint requests: Chandy Ellimoottil, M.D., Department of Urology, Loyola University Medical Center, 2160 S First Ave, Fahey Center, Rm 261, Maywood, IL 60153. E-mail:
[email protected] Submitted: August 6, 2012, accepted (with revisions): August 25, 2012
Published by Elsevier Inc. All Rights Reserved
Leiomyosarcoma is a rare malignant primary testicular tumor.1 Patients with these tumors range in age from 19 to 76 years and present with painless testicular enlargement and normal tumor markers. Only 13 such cases have been reported.1 Rare benign solid tumors of the testis include epidermoid cyst, Leydig cell hyperplasia, fibroma of gonadal stromal origin, testicular hemangioma, and leiomyoma.2 Testicular hemangioma typically occurs in younger patients, although it has been reported in patients as old as 73 years.3 Fibroma of gonadal stromal origin can occur in patients who are aged 30 to 40 years as a painless testicular mass.3 Sarcoidosis and tuberculosis can also cause masses with a heterogeneous appearance, although tuberculosis more commonly affects the epididymis. Testicular hemorrhage, sometimes occurring spontaneously, may simulate a focal mass as well.2 Of the benign solid masses, epidermoid cysts are the most common, but tend to arise in younger men and adolescents. Although these lesions are cysts from a pathologic standpoint, they contain solid material.2 Paratesticular tumors include adenomatoid tumors, testicular appendage with torsion, leiomyoma, lipoma, and fibrous pseudotumor. Of paratesticular tumors, adenomatoid tumors are the most common and represent 30% of all paratesticular neoplasms.2 The appearance on ultrasound imaging of our patient’s mass, with varying levels of echogenicity, was also concerning for inflammation or abscess. However, given the likelihood that a solid testicular mass would be malignant, the patient underwent a right inguinal orchiectomy. The orchiectomy was without complications, and the right testicle was sent for pathologic diagnosis.
PATHOLOGY (PRESENTED BY VIKAS MEHTA, M.D.) Gross examination of the testis revealed a firm, wellcircumscribed, tan-white lesion measuring 6 cm in greatest dimension, with a whorled appearance on cut section. The tumor occupied most of the testicle. Histologic examination revealed a spindle cell tumor arising adjacent to the tunica in the region of the right testis (Fig. 2A). The cells had indistinct cell borders. The nuclei were elongated (and enlarged in some cells) with small nucleoli (Fig. 2B). Low mitotic activity was seen. Immunohistochemistry studies revealed that the spindle cells were positive for smooth muscleespecific actin (Fig. 2C) and desmin and negative for human melanoma 0090-4295/12/$36.00 http://dx.doi.org/10.1016/j.urology.2012.08.050
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Figure 1. (A) Scrotal ultrasound image shows the right testicle (7.0 4.0 4.5 cm) with small 0.6-cm epididymal and intratesticular simple cysts. In the mid to lower pole, there is a large heterogeneous solid mass measuring 6.2 4.8 5.3 cm. Echotexture and echogenicity is heterogeneous. There is dense shadowing posterior to the mass. No calcifications are noted. There is a small hydrocele. (B). Doppler imaging shows vascular flow within the mass. (Color version available online.)
Figure 2. (A) Photomicrograph of the histopathologic specimen shows normal testicular tissue adjacent to the leiomyoma, which is composed of spindle cells arranged in fascicles (arrow; hematoxylin and eosin stain, original magnification 10). (B) The tumor cells are spindle-shaped with minimal atypia and mitotic figures. (C) The tumor cells are diffusely positive for smooth muscle actin by immunohistochemistry (original magnification 10). (D). Ki67 showed a low proliferation index by immunohistochemistry (original magnification 10). (Color version available online.)
black 45. The proliferation index by Ki-67 was less than 10% (Fig. 2D). These findings were consistent with the diagnosis of a benign smooth muscle neoplasm, a leiomyoma.
DISCUSSION Leiomyoma is a benign soft-tissue tumor that originates from smooth muscle. Testicular leiomyomas are uncommon, with only a few dozen cases reported.3,4 Leiomyomas of the male urogenital tract are most common in the renal capsule, but they have also been reported in the tunica vaginalis, tunica albuginea, spermatic cord, and epididymis.4,5 Leiomyoma is the second 10
most common epididymal neoplasm across all ages, representing 6% of epididymal tumors.4,6 There have only been 2 case reports of intratesticular leiomyomas.5 Testicular leiomyomas present in men aged between 30 and 60 years, most commonly in the fifth decade of life.3,4 Patients report minimal pain but notice fullness and asymmetry between testicles. Similar to a patient reported by Thomas et al,5 this tumor was quite large, measuring 6 cm in its longest dimension, with the tumor taking up most of the testicle. Other cases have reported smaller tumors. The etiology of testicular leiomyomas is not well described. These tumors can arise from any structure containing smooth muscle. Specifically in the testis, it has UROLOGY 81 (1), 2013
been postulated that the tumor may arise from the smooth muscle contractile cells in the tunica propria of the seminiferous tubules.4 Although this tumor was sent for outside review, it is unclear whether the specimen arose from the testicle or tunica albuginea. On gross pathologic examination, these tumors are well circumscribed, solid, may or may not contain calcifications, and have a whorled cut surface. Stains are positive for desmin and smooth muscle actin.3 The diagnosis is challenging to make without surgical resection. The appearance of a leiomyoma is somewhat variable on ultrasound imaging and difficult to distinguish from malignant tumors. The appearance on ultrasound imaging been described as a whirling pattern with multiple narrow areas of shadowing and no obvious calcifications.2 Variable echogenicity has been reported with leiomyomas. In most cases, there is an associated hydrocele.5 Although benign neoplasms may not require resection, leiomyomas can rarely be distinguished from malignancy on imaging.7 Given the rare occurrence of such tumors, especially in the testis, it is unlikely that a definitive diagnosis would be made before orchiectomy, and radical orchiectomy remains the treatment of choice.5 This is particularly true if the tumor takes up most of the normal parenchyma.8 Testis-sparing surgery has been suggested for small lesions that can be enucleated.6 Some features that might suggest leiomyoma include an older aged patient with a well-defined round or ovoid mass and no clinical symptoms or tumor markers.7 However, given that testicular leiomyomas are a challenge to diagnose preoperatively, treatment in most case reports has been radical orchiectomy.
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As part of his staging, this patient was found to have subcentimenter nodes in his paraaortic and iliac chains; however, the significance of these nodes in a patient with a benign-appearing testicular mass is unclear. We elected to perform a follow-up computed tomography scan in several months to better characterize these lymph nodes. Solid testicular masses are commonly malignant; however, several benign entities are in the differential diagnosis. Testicular leiomyoma is a rare benign neoplasm that has a distinct histologic appearance but is challenging to diagnose preoperatively using imaging. Owing to this dilemma, radical orchiectomy remains the treatment of choice. References 1. Moona MS, Fatima D, Turezbek A. Primary testicular leiomyosarcoma. J Pak Med Assoc. 2011;61:1014-1016. 2. Park SB, Lee WC, Kim JK, et al. Imaging features of benign solid testicular and paratesticular lesions. Eur Radiol. 2011;21:22262234. 3. Frias-Kletecka MC, MacLennan GT. Benign soft tissue tumors of the testis. J Urol. 2009;182:312-313. 4. Kullolli VS, Kullolli S, Pawar S, et al. Leiomyoma of testis e a case report. Indian J Surg. 2011;73:233-235. 5. Thomas J, Rifkin M, Nazeer T. Intratesticular Leiomyoma of the body of the testis. J Ultrasound Med. 1998;17:785-787. 6. Ahmed HU, Arya M, Muneer A. Testicular and paratesticular tumours in the prepubertal population. Lancet Oncol. 2010;5: 476-483. 7. O’Brien J, Loftus B, Barrett C. Leiomyoma of the testis: a rare testicular mass. J Clin Ultrasound. 2008;36:240-242. 8. Destito A, Servello C, Pisanti F. Leiomyoma of the testis. Scand J Urol Nephrol. 1999;5:338-339.
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