Solid Extratesticular Masses in Children: Radiographic and Pathologic Correlation

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1 Extratesticular Masses in hildren Pediatric Imaging Pictorial Essay Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved D E M N E U T R Y L I M I G O F I N G Tammy Sung 1 Wolfram F. J. Riedlinger 2 David. Diamond 3 Jeanne S. how 4 Sung T, Riedlinger WFJ, Diamond D, how JS Keywords: genitourinary tract imaging, pediatric imaging, scrotal disease, sonography, testicular mass DOI: /JR Received December 13, 2004; accepted after revision January 27, Department of Radiology, righam and Women's Hospital, 75 Francis St., oston, M ddress correspondence to T. Sung (tsung@partners.org). 2 Department of Pathology, hildren s Hospital oston, oston, M Department of Urology, hildren s Hospital oston, oston, M Department of Radiology, hildren s Hospital oston, oston, M JR 2006; 186: X/06/ merican Roentgen Ray Society Solid Extratesticular Masses in hildren: Radiographic and Pathologic orrelation OJETIVE. The purpose of this pictorial essay is to review the sonographic and pathologic appearances of the most common solid and complex extratesticular masses in children. ONLUSION. Solid or complex extratesticular masses, especially those that are rapidly growing and are painless, raise concerns regarding malignant rhabdomyosarcoma. Mimickers of rhabdomyosarcoma include inflammatory processes such as pseudotumor, chronic epididymitis, or meconium periorchitis. ecause sonography cannot distinguish benign from malignant, worrisome extratesticular masses should be biopsied or removed. nlike solid extratesticular masses U in adults, which are generally benign [1], 50% of painless extratesticular masses in children are malignant [2]. The most common neoplasm is paratesticular rhabdomyosarcoma. Evaluation of scrotal disease in children begins with history and physical examination. Sonography, the imaging technique of choice [3], characterizes scrotal lesions as intratesticular or extratesticular and solid, cystic, or complex [1]. ystic extratesticular masses such as spermatoceles or hydroceles are typically benign and have been well described [1]. Inguinal hernias, especially those containing bowel, are diagnosed easily by sonogram. However, a sonogram cannot determine if a solid and complex extratesticular mass is benign or malignant [4]. Using pathologic specimens obtained at hildren s Hospital oston between 1994 and 2004, this pictorial essay reviews the literature and describes common solid and complex extratesticular masses in children, including malignant rhabdomyosarcoma. ased on clinical presentation, masses are divided into two categories: painless and painful. Painless Masses Rhabdomyosarcoma Other than paratesticular lipomas found incidentally during surgery, rhabdomyosarcomas were the most commonly resected extratesticular masses in the past 10 Fig. 1 4-year-old boy who presented with painless enlargement of right scrotum due to paratesticular rhabdomyosarcoma., Sagittal sonogram of right testicle shows heterogeneous solid mass (arrows) encircling testis (T) separate from epididymis (E). fter surgical excision, pathology showed embryonal-type rhabdomyosarcoma. (Fig. 1 continues on next page) JR:186, February

2 Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved Fig. 1 (continued) 4-year-old boy who presented with painless enlargement of right scrotum due to paratesticular rhabdomyosarcoma., Photomicrograph of histopathologic specimen shows highly cellular neoplasm composed of abundant small, round blue cells arranged with lack of cohesive pattern. (H and E, 10), Photomicrograph of histopathologic specimen shows tumor, composed of undifferentiated small, round to spindle cells with dark nuclei and scant cytoplasm and interspersed differentiated rhabdomyoblasts with eosinophilic cytoplasm. Tumor cells are surrounded by connective tissue with variable myxoid appearance. (H and E, 60) Fig year-old boy who presented with enlarging painless right scrotal mass due to rhabdomyosarcoma., Sagittal sonogram shows hypoechoic mass (M) (arrow) that is separate from adjacent testis (T) and compresses epididymis (E). There is increased blood flow to mass by color Doppler (not shown). Surgical excision was performed., Photomicrograph of histopathologic specimen shows undifferentiated small, round to spindle cells with dark nuclei and scant cytoplasm embedded in connective tissue with focal myxoid appearance. Interspersed are well-differentiated rhabdomyoblasts with eosinophilic cytoplasm. (H and E, 60) years at hildren s Hospital oston. Four percent of these common childhood tumors occur in the paratesticular location [5] (Figs. 1 and 2). The embryonal subtype is the most common. Patients typically present with unilateral painless scrotal swelling not associated with fever. With multimodality therapy, the survival after rhabdomyosarcoma resection is reported to be 80% at 3 years [4]. Rhabdomyosarcomas vary in appearance from mostly solid to primarily cystic with solid nodules, reflecting the gross pathologic appearance of a solid mass with occasional cystic degeneration and hemorrhage. 484 JR:186, February 2006

3 Extratesticular Masses in hildren Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved Pseudotumor Fibrous pseudotumor (chronic periorchitis, nodular fibrous periorchitis, and nonspecific paratesticular fibrosis) (Fig. 3) and inflammatory pseudotumor (plasma cell granuloma) (Fig. 4) are benign, reactive, nonneoplastic lesions of the spermatic cord, Fig year-old boy with painless right scrotal mass secondary to fibrous pseudotumor., Sagittal sonogram of right scrotum shows hypoechoic mass (M) (arrow) in epididymis that is discrete and separate from testis (T)., Photomicrograph of histopathologic specimen shows granulation tissue composed of capillary-size vessels associated with chronic inflammatory lymphoplasmacytic cell infiltrate in background of focal hyalinized fibrous tissue. (H and E, 40) Fig year-old boy who presented with firm, mobile, nonpainful left scrotal mass secondary to inflammatory pseudotumor., Sagittal sonogram shows part of normal left testis (T) and large hypoechoic mass (M) (arrow) that is superior to testis. Mass is homogeneous and well defined and has mild peripheral vascular flow on color Doppler evaluation (not shown). (Fig. 4 continues on next page) epididymis, and tunica caused by fibroinflammatory reaction. Patients often have a history of trauma or infection [5]. On a sonogram, both variants of pseudotumor mimic rhabdomyosarcoma, and the diagnosis is rarely made before surgical resection [1]. However, on histologic sections, both lesions look distinctly different. Pseudotumors are commonly associated with hydroceles and hematoceles [5]. Meconium Periorchitis Meconium periorchitis (meconium granuloma, meconium vaginalitis) (Fig. 5) is a very JR:186, February

4 Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved Fig. 4 (continued) 17-year-old boy who presented with firm, mobile, nonpainful left scrotal mass secondary to inflammatory pseudotumor., Photomicrograph of histopathologic specimen shows predominantly collagen-filled stroma in a vaguely nodular pattern with rare interspersed chronic inflammatory cells. There is neither hemorrhage nor necrosis evident. (H and E, 20), Photomicrograph of histopathologic specimen shows associated mixed acute and chronic inflammatory infiltrate encompassing neutrophils, lymphocytes, plasma cells, histiocytes, eosinophils, and occasional mast cells. Within collagenous background are spindle cells without nuclear hyperchromasia or cytologic atypia. (H and E, 20) Fig. 5 7-day-old male neonate who presented with severe swelling of scrotum secondary to meconium periorchitis., Transverse sonogram of scrotum shows marked skin thickening and complex fluid collections surrounding testis (T). Multiple echogenic foci with shadowing (arrow) represent calcified meconium., Photomicrograph of histopathologic specimen reveals chronically inflamed myxoid stroma with massive accumulation of polymorphonuclear leukocytes and intermixed chronic inflammatory cells and with histiocytes harboring browning meconium pigment in their cytoplasm. (H and E, 40) rare masslike lesion that arises as a result of inflammatory reaction from meconium within the scrotal sac. Fewer than 30 cases are described in the literature [6]. In fetal and early postnatal periods, a patent processus vaginalis allows spilled meconium from bowel perforation to spread into the scrotal sac. onsistency and appearance of meconium in the scrotum evolve over time. Soft extratesticular mass at birth eventually hardens and becomes partially calcified [6]. Meconium causes foreign-body giant-cell reaction, chronic inflammation, and finally, scarring. Peritoneal and scrotal calcifications resulting from chronic inflammation are clues to a diagnosis of meconium periorchitis. 486 JR:186, February 2006

5 Extratesticular Masses in hildren Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved ellular Neurofibroma ellular neurofibroma (Fig. 6) of the spermatic cord is a rare solid extratesticular mass in adults and children [7]. Neurofibromas arise from nerve cells along the muscularis propria or ensheathing small nerves. This diagnosis should be considered in patients with neurofibromatosis 1 because this disease is associated with an increased incidence of neurofibromas. However, when there is no history of neurofibromatosis and the lesion is solitary, clinical and radiologic findings are nonspecific [7]. These smooth white-to-tan tumors rarely have secondary degenerative changes such as cyst formation or hemorrhage and appear homogeneous on a sonogram. Fig year-old boy who presented with hard, mobile, painless scrotal mass secondary to cellular neurofibroma., Transverse sonogram of bilateral testes (T) shows a hypoechoic mass (N) that is discrete from adjacent testicle., Sagittal sonogram of same patient shows hypoechoic paratesticular mass (N) (arrow) with increased flow on color Doppler that is discrete from adjacent testicle (T)., Photomicrograph of histopathologic specimen shows elongated, irregularly shaped cells without nuclear atypia. Lesional cells with wavy, dark-staining nuclei are arranged in interlacing bundles, small whorls, and short fascicles. They are embedded in moderate amounts of mucoid or collagenous matrix. Mast cells, lymphocytes, and occasional xanthoma cells are present elsewhere. (H and E, 40) Painful Scrotal Masses Epididymal Inflammation Epididymal inflammation leading to chronic epididymitis can be misdiagnosed as rhabdomyosarcoma. lthough clinical history and the presence of pain help to differentiate epididymal infection from tumor, the diagnosis is not always clear [8]. hronic epididymitis may feel and appear JR:186, February

6 Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved masslike (Fig. 7), mimicking extratesticular neoplasms. Fig year-old man who presented with palpable right scrotal mass and vague right testicular pain for 2 months secondary to chronic epididymitis., Sagittal sonogram of right scrotum shows a solid hypoechoic mass (E) that appears separate from testis (T) and epididymis with increased flow on color Doppler imaging. t inguinal exploration, biopsy of mass and pathology showed chronic epididymitis with fibrosis., Photomicrograph of histopathologic specimen shows variable numbers of chronic inflammatory cells (i.e., lymphocytes, plasma cells, and histiocytes within collagenized stroma surrounding unremarkable epididymal tubules and ducts). Lymphoid follicles with reactive germinal centers and noncaseating epithelioid granulomas, resulting from sperm-spillage, are seen elsewhere. (H and E, 20) Torsed ppendix The appendix epididymis and appendix testis are embryologic remnants of the mesonephric (wolffian) duct and paramesonephric (müllerian) duct, respectively [1]. Torsion of these appendages causes acute scrotal pain and a focal bluish discoloration beneath the skin (so-called blue-dot sign). tender nodule is commonly palpated on physical examination Fig year-old boy who presented with 1-week history of right groin and scrotal pain due to torsed appendix testis., Parasagittal sonogram shows right testis (T) with adjacent hyperechoic mass () without appreciable flow on color Doppler evaluation., Transverse sonogram of same patient shows heterogeneously echogenic paratesticular mass (). lthough physical examination and sonogram were consistent with torsed appendix testis, patient underwent surgical scrotal exploration. (Fig. 8 continues on next page) [3]. sonogram (Fig. 8) typically shows a hyperemic hyperechoic small oval mass adjacent to the testis or epididymis, which may be associated with epididymal inflammation and hydrocele. With time, the torsed appendages involute and may calcify [3]. 488 JR:186, February 2006

7 Extratesticular Masses in hildren Fig. 8 (continued) 15-year-old boy who presented with 1-week history of right groin and scrotal pain due to torsed appendix testis., Photomicrograph of histopathologic specimen shows marked hemorrhagic infarction and ectatic and congested vascular spaces with leakage and resulting fresh hemorrhage. Scattered hemosiderin pigment laden macrophages can be found in areas. (H and E, 10) Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved Fig. 9 4-day-old male neonate who presented with swollen penis and swollen, firm scrotum after circumcision, found to have scrotal abscesses., Sagittal sonogram shows skin thickening and hyperemia of scrotal skin consistent with cellulitis. omplex cystic collection is adjacent to testis (T)., Transverse sonogram shows marked increased flow surrounding testis (T) and adjacent complex fluid collection. t surgery, bilateral scrotal abscesses were incised and drained., Photomicrograph of histopathologic specimen shows sheets of abundant polymorphonuclear leukocytes and nuclear fragments consistent with abscess. (H and E, 20) JR:186, February

8 Downloaded from by on 11/19/17 from IP address opyright RRS. For personal use only; all rights reserved Scrotal bscess Scrotal abscess (Fig. 9) can be a complication of epididymoorchitis, trauma, or surgery. The clinical history and physical examination of a painful hyperemic scrotum help in making the diagnosis. On a sonogram, this extratesticular lesion can have a variety of appearances. reas of infection are generally hyperemic and heterogeneous in echotexture. If organized, an abscess may have a well-defined hyperemic wall. If there is gas within the collection, hyperechoic foci with dirty shadowing are seen. onclusion Solid or complex extratesticular masses, especially those that present as rapidly growing and painless, may be malignant rhabdomyosarcoma. history of trauma or acute pain and findings of erythema or tenderness help to distinguish acute inflammatory processes from possibly malignant lesions. Mimickers of rhabdomyosarcoma include chronic inflammatory processes such as pseudotumor, chronic epididymitis, or meconium periorchitis, and less commonly, other tumors. denomatoid tumors, common in adults, are rare in children and only one case appeared as an incidental finding in our series. If there is history of neurofibromatosis 1, the extratesticular mass may represent a neurofibroma. Lipomas are commonly found in pathologic specimens but are rarely the cause of the original surgery. Splenogonadal fusion [5] and supernumerary testes [1] are also occasionally diagnosed. ecause no pathologically proven cases were reported in our 10-year search, none was included in this article. lthough lymphomas have been reported in the literature, none was found in our surgical pathologic specimens over the past decade. ecause a sonogram cannot distinguish benign from malignant, worrisome extratesticular masses need to be biopsied or removed. References 1. Woodward PJ, Schwab M, Sesterhenn I. From the archives of the FIP: extratesticular scrotal masses radiologic-pathologic correlation. Radio- Graphics 2003; 23: ragona F, Talenti E, Santacatterina U, Perale R, Passerini Glazel G. Unusual, benign asymptomatic scrotal masses in children: case reports and review of the literature. Int Urol Nephrol 1994; 26: Frush DP, Sheldon. Diagnostic imaging for pediatric scrotal disorders. RadioGraphics 1998; 18: Skoog SJ. enign and malignant pediatric scrotal masses. Pediatr lin North m 1997; 44: kbar S, Sayyed T, Jafri SZ, Hasteh F, Neill JS. Multimodality imaging of paratesticular neoplasms and their rare mimics. RadioGraphics 2003; 23: Williams HJ, bernethy LJ, Losty PD, Kotiloglu E. Meconium periorchitis: a rare cause of a paratesticular mass. Pediatr Radiol 2004; 34: Jiang R, hen JH, hen M, Li QM. Male genital schwannoma: review of 5 cases. sian J ndrol 2003; 5: Mak W, hou K, Su, Huan SK, hang JM. Ultrasound diagnosis of paratesticular rhabdomyosarcoma. r J Radiol 2004; 77: JR:186, February 2006

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