Pediatric Case Report Ewing Sarcoma of the Scrotum Gwen M. Grimsby and Clanton B. Harrison Nonosseous Ewing sarcoma commonly occurs in the extremities or deep soft tissues. However, cutaneous and subcutaneous locations have been reported. A 3-year-old boy presented with a 2-year history of a painless, slowly growing midscrotal mass. Pathology after surgical excision revealed the lesion to be Ewing sarcoma. The patient is free of metastatic disease and is currently undergoing chemotherapy. Soft-tissue malignancies must be kept in the differential diagnosis of any solid paratesticular mass in a child. Although rhabdomyosarcoma is the most common, as this case demonstrates, other rare sarcomas are also possible. UROLOGY -: -e-, 2014. 2014 Elsevier Inc.
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n asymptomatic scrotal mass in a child can be a diagnostic dilemma secondary to the staggering list of possibilities in the differential diagnosis, including infectious etiologies, congenital anomalies, changes secondary to trauma, as well as benign and malignant lesions.1 In addition, because of the diverse embryologic origins of the scrotal contents, the environment is ripe for unusual pathologies, as this case highlights.2 A 3-year-old boy presented with a painless scrotal mass that on surgical excision was determined to be Ewing sarcoma.
CASE A 3-year-old boy presented with a scrotal mass noted by his family during bathing. The parents reported that the mass had been present for roughly 2 years and that it had slowly increased in size over time. The patient had been completely asymptomatic, with no pain or infection of the area and no difficulty in urination. He was born fullterm via normal spontaneous vaginal delivery, had no medical or surgical history, no allergies, and was not taking any medications. Physical examination revealed a well-nourished and well-developed child with a soft abdomen free of masses. His phallus was circumcised with a normal meatus, and his testicles were descended bilaterally with normal size and position. A mobile midline 2 2 cm scrotal lesion was noted. The lesion was nontender and not fixed to the overlying skin. A testicular ultrasound confirmed the presence of a 1.8-cm indeterminate heterogeneous vascular mass within the scrotal soft tissues interior to the testicles (Fig. 1). Because of the indeterminate nature of the lesion, it was felt the patient would be best served with a surgical excision. Financial Disclosure: The authors declare that they have no relevant financial interests. From the Division of Pediatric Urology, Department of Urology, UT Southwestern Medical Center, Dallas TX; and Center for Pediatric Urology, the Children’s Medical Center, Dallas, TX Reprint requests: Clanton B. Harrison, M.D., Center for Pediatric Urology, Children’s Medical Center, 1935 Medical District Drive, MC F4.04, Dallas, TX 75235. E-mail: [email protected] Submitted: January 31, 2014, accepted (with revisions): March 4, 2014
ª 2014 Elsevier Inc. All Rights Reserved
The patient was taken to the operating room and the mass excised via a scrotal incision. Surprisingly, the pathology report revealed an infiltrative neoplasm composed of sheets and nests of uniform round cells with round nuclei, salt and pepper chromatin, inconspicuous nucleoli, and scant to moderate pale cytoplasm, consistent with grade-3 Ewing sarcoma. Fluorescence in situ hybridization results confirmed the deletion of the 30 (telomeric) end of the EWSR1 (22q12) gene. The deletion of the 30 end of the EWSR1 gene has been previously reported only once in the literature and was associated with a chromosomal rearrangement.3 After consultation with oncology, the patient underwent a computed tomography scan of the chest, abdomen, and pelvis, a bone scan, and a bone marrow biopsy, all of which were negative for metastatic disease. Because of a positive surgical margin on the original resection, the patient returned to the operating room 2 weeks after the original surgery for a re-resection. Final pathology confirmed a small amount of residual disease with negative margins. The patient also had a central venous catheter placement with port at the time of the reresection and is currently receiving chemotherapy with vincristine, doxorubicin, and cyclophosphamide.
COMMENT We present a case of primary scrotal extraosseous Ewing sarcoma. To our knowledge, this is the first report in the literature of Ewing sarcoma in this unique location. Extraosseous Ewing sarcoma is histologically indistinguishable from Ewing sarcoma of the bone and is more common in the deep soft tissues.4 Primary cutaneous and subcutaneous sites are rarer and less aggressive than their bony or deep soft-tissue counterparts, with the most common locations being the trunk and extremities.4 However, extraosseous Ewing sarcoma has been previously reported in the groin region in a case of both an inguinal and a labia lesion.4,5 In this case, based on the location of the mass in the scrotum, as well as the lack of metastatic disease despite being present for 2 years, it is reasonable to assume that it would fall into the 0090-4295/14/$36.00 http://dx.doi.org/10.1016/j.urology.2014.03.002
1
the benign adenomatoid tumor, and malignancies of the epididymis are exceedingly rare.6 However, in children, rhabdomyosarcoma is a common cause of a solid paratesticular mass, and the aforementioned adenomatoid tumors are exceedingly rare.6 In addition, other malignant paratesticular lesions have been reported in children, including leiomyosarcoma, fibrosarcoma, liposarcoma, and now Ewing sarcoma.8 In fact, some authors have reported that of masses that occur in the scrotal wall or tunics, 59% are malignant soft-tissue tumors.9 Thus, many authors suggest surgical exploration for any solid paratesticular mass in a child.1,2,6 However, as in all cases, appropriate clinical judgment should be used based on factors such as the size and location of the lesion, presence of vascular flow on ultrasonography, and the rate of growth of the lesion, as many asymptotic scrotal masses have benign causes.
CONCLUSION
Figure 1. Testicular ultrasonography reveals a 1.8-cm indeterminate heterogeneous vascular mass within the scrotal soft tissues interior to the testicles. ML, midline; INF, inferior; TRANS, transverse.
A solid asymptotic scrotal mass in a 3-year-old boy was diagnosed as primary nonosseous Ewing sarcoma of the scrotum. Not only is this case the first report of Ewing sarcoma in this unique location, but it also emphasizes the broad differential of a paratesticular lesion. Soft-tissue malignancies must be considered in all paratesticular masses in children and thus treated accordingly. References
category of cutaneous and subcutaneous nonosseous Ewing sarcoma. This case also brings to light the difficulty of the diagnosis and thus the choice of treatment vs observation of a painless scrotal mass in a child. The differential diagnosis of a paratesticular mass is very broad and requires caution, because clinically it is very difficult to exclude malignancy.4,6 Unfortunately, ultrasonography, the diagnostic modality of choice, cannot differentiate between benign and malignant soft-tissue tumors of the scrotum.1,6,7 One approach is the diagnostic algorithm suggested by Skoog,1 which first distinguishes intratesticular or paratesticular location. As intratesticular masses are malignant until proven otherwise, these routinely require surgical exploration.1 A paratesticular mass should then be divided into either solid or cystic in nature, as cystic causes (hernias, hydroceles, hematoceles, varicoceles, and spermatoceles) can be treated or observed as indicated.1 It is the solid paratesticular mass that can be trickier. In an adult, the most common solid paratesticular mass is
2
1. Skoog SJ. Benign and malignant pediatric scrotal masses. Pediatr Clin North Am. 1997;44:1229-1250. 2. Rosevear HM, Mishail A, Sheynkin Y, Wald M. Unusual scrotal pathology: an overview. Nat Rev Urol. 2009;6:491-500. 3. Lee J, Hopcus-Niccum DJ, Mulvihill JJ, Li S. Cytogenetic and molecular cytogenetic studies of a variant of t(21;22), ins(22;21)(q12; q21q22), with a deletion of the 3’ EWSR1 gene in a patient with Ewing sarcoma. Cancer Genet Cytogenet. 2005;159:177-180. 4. Chow E, Merchant TE, Pappo A, et al. Cutaneous and subcutaneous Ewing’s sarcoma: an indolent disease. Int J Radiat Oncol Biol Phys. 2000;46:433-438. 5. Zampieri N, Carabaich A, Corroppolo M, et al. Two patients with tumours presenting as inguino-scrotal masses. Eur J Cancer Care (Engl). 2008;17:205-208. 6. Aragona F, Pescatori E, Talenti E, et al. Painless scrotal masses in the pediatric population: prevalence and age distribution of different pathological conditions—a 10 year retrospective multicenter study. J Urol. 1996;155:1424-1426. 7. Barth RA, Teele RL, Colodny A, et al. Asymptomatic scrotal masses in children. Radiology. 1984;152:65-68. 8. Montgomery JS, Bloom DA. The diagnosis and management of scrotal masses. Med Clin North Am. 2011;95:235-244. 9. Beccia DJ, Krane RJ, Olsson CA. Clinical management of nontesticular intrascrotal tumors. J Urol. 1976;116:476-479.
UROLOGY
-
(-), 2014
A
n asymptomatic scrotal mass in a child can be a diagnostic dilemma secondary to the staggering list of possibilities in the differential diagnosis, including infectious etiologies, congenital anomalies, changes secondary to trauma, as well as benign and malignant lesions.1 In addition, because of the diverse embryologic origins of the scrotal contents, the environment is ripe for unusual pathologies, as this case highlights.2 A 3-year-old boy presented with a painless scrotal mass that on surgical excision was determined to be Ewing sarcoma.
CASE A 3-year-old boy presented with a scrotal mass noted by his family during bathing. The parents reported that the mass had been present for roughly 2 years and that it had slowly increased in size over time. The patient had been completely asymptomatic, with no pain or infection of the area and no difficulty in urination. He was born fullterm via normal spontaneous vaginal delivery, had no medical or surgical history, no allergies, and was not taking any medications. Physical examination revealed a well-nourished and well-developed child with a soft abdomen free of masses. His phallus was circumcised with a normal meatus, and his testicles were descended bilaterally with normal size and position. A mobile midline 2 2 cm scrotal lesion was noted. The lesion was nontender and not fixed to the overlying skin. A testicular ultrasound confirmed the presence of a 1.8-cm indeterminate heterogeneous vascular mass within the scrotal soft tissues interior to the testicles (Fig. 1). Because of the indeterminate nature of the lesion, it was felt the patient would be best served with a surgical excision. Financial Disclosure: The authors declare that they have no relevant financial interests. From the Division of Pediatric Urology, Department of Urology, UT Southwestern Medical Center, Dallas TX; and Center for Pediatric Urology, the Children’s Medical Center, Dallas, TX Reprint requests: Clanton B. Harrison, M.D., Center for Pediatric Urology, Children’s Medical Center, 1935 Medical District Drive, MC F4.04, Dallas, TX 75235. E-mail: [email protected] Submitted: January 31, 2014, accepted (with revisions): March 4, 2014
ª 2014 Elsevier Inc. All Rights Reserved
The patient was taken to the operating room and the mass excised via a scrotal incision. Surprisingly, the pathology report revealed an infiltrative neoplasm composed of sheets and nests of uniform round cells with round nuclei, salt and pepper chromatin, inconspicuous nucleoli, and scant to moderate pale cytoplasm, consistent with grade-3 Ewing sarcoma. Fluorescence in situ hybridization results confirmed the deletion of the 30 (telomeric) end of the EWSR1 (22q12) gene. The deletion of the 30 end of the EWSR1 gene has been previously reported only once in the literature and was associated with a chromosomal rearrangement.3 After consultation with oncology, the patient underwent a computed tomography scan of the chest, abdomen, and pelvis, a bone scan, and a bone marrow biopsy, all of which were negative for metastatic disease. Because of a positive surgical margin on the original resection, the patient returned to the operating room 2 weeks after the original surgery for a re-resection. Final pathology confirmed a small amount of residual disease with negative margins. The patient also had a central venous catheter placement with port at the time of the reresection and is currently receiving chemotherapy with vincristine, doxorubicin, and cyclophosphamide.
COMMENT We present a case of primary scrotal extraosseous Ewing sarcoma. To our knowledge, this is the first report in the literature of Ewing sarcoma in this unique location. Extraosseous Ewing sarcoma is histologically indistinguishable from Ewing sarcoma of the bone and is more common in the deep soft tissues.4 Primary cutaneous and subcutaneous sites are rarer and less aggressive than their bony or deep soft-tissue counterparts, with the most common locations being the trunk and extremities.4 However, extraosseous Ewing sarcoma has been previously reported in the groin region in a case of both an inguinal and a labia lesion.4,5 In this case, based on the location of the mass in the scrotum, as well as the lack of metastatic disease despite being present for 2 years, it is reasonable to assume that it would fall into the 0090-4295/14/$36.00 http://dx.doi.org/10.1016/j.urology.2014.03.002
1
the benign adenomatoid tumor, and malignancies of the epididymis are exceedingly rare.6 However, in children, rhabdomyosarcoma is a common cause of a solid paratesticular mass, and the aforementioned adenomatoid tumors are exceedingly rare.6 In addition, other malignant paratesticular lesions have been reported in children, including leiomyosarcoma, fibrosarcoma, liposarcoma, and now Ewing sarcoma.8 In fact, some authors have reported that of masses that occur in the scrotal wall or tunics, 59% are malignant soft-tissue tumors.9 Thus, many authors suggest surgical exploration for any solid paratesticular mass in a child.1,2,6 However, as in all cases, appropriate clinical judgment should be used based on factors such as the size and location of the lesion, presence of vascular flow on ultrasonography, and the rate of growth of the lesion, as many asymptotic scrotal masses have benign causes.
CONCLUSION
Figure 1. Testicular ultrasonography reveals a 1.8-cm indeterminate heterogeneous vascular mass within the scrotal soft tissues interior to the testicles. ML, midline; INF, inferior; TRANS, transverse.
A solid asymptotic scrotal mass in a 3-year-old boy was diagnosed as primary nonosseous Ewing sarcoma of the scrotum. Not only is this case the first report of Ewing sarcoma in this unique location, but it also emphasizes the broad differential of a paratesticular lesion. Soft-tissue malignancies must be considered in all paratesticular masses in children and thus treated accordingly. References
category of cutaneous and subcutaneous nonosseous Ewing sarcoma. This case also brings to light the difficulty of the diagnosis and thus the choice of treatment vs observation of a painless scrotal mass in a child. The differential diagnosis of a paratesticular mass is very broad and requires caution, because clinically it is very difficult to exclude malignancy.4,6 Unfortunately, ultrasonography, the diagnostic modality of choice, cannot differentiate between benign and malignant soft-tissue tumors of the scrotum.1,6,7 One approach is the diagnostic algorithm suggested by Skoog,1 which first distinguishes intratesticular or paratesticular location. As intratesticular masses are malignant until proven otherwise, these routinely require surgical exploration.1 A paratesticular mass should then be divided into either solid or cystic in nature, as cystic causes (hernias, hydroceles, hematoceles, varicoceles, and spermatoceles) can be treated or observed as indicated.1 It is the solid paratesticular mass that can be trickier. In an adult, the most common solid paratesticular mass is
2
1. Skoog SJ. Benign and malignant pediatric scrotal masses. Pediatr Clin North Am. 1997;44:1229-1250. 2. Rosevear HM, Mishail A, Sheynkin Y, Wald M. Unusual scrotal pathology: an overview. Nat Rev Urol. 2009;6:491-500. 3. Lee J, Hopcus-Niccum DJ, Mulvihill JJ, Li S. Cytogenetic and molecular cytogenetic studies of a variant of t(21;22), ins(22;21)(q12; q21q22), with a deletion of the 3’ EWSR1 gene in a patient with Ewing sarcoma. Cancer Genet Cytogenet. 2005;159:177-180. 4. Chow E, Merchant TE, Pappo A, et al. Cutaneous and subcutaneous Ewing’s sarcoma: an indolent disease. Int J Radiat Oncol Biol Phys. 2000;46:433-438. 5. Zampieri N, Carabaich A, Corroppolo M, et al. Two patients with tumours presenting as inguino-scrotal masses. Eur J Cancer Care (Engl). 2008;17:205-208. 6. Aragona F, Pescatori E, Talenti E, et al. Painless scrotal masses in the pediatric population: prevalence and age distribution of different pathological conditions—a 10 year retrospective multicenter study. J Urol. 1996;155:1424-1426. 7. Barth RA, Teele RL, Colodny A, et al. Asymptomatic scrotal masses in children. Radiology. 1984;152:65-68. 8. Montgomery JS, Bloom DA. The diagnosis and management of scrotal masses. Med Clin North Am. 2011;95:235-244. 9. Beccia DJ, Krane RJ, Olsson CA. Clinical management of nontesticular intrascrotal tumors. J Urol. 1976;116:476-479.
UROLOGY
-
(-), 2014
