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Society for Pediatric Radiology – Poster Archive
Final ID: Poster #: CR-074
Atypical Appearance of a Rare Pediatric Tumor: Juvenile Granulosa Cell Tumor of the Testis
Purpose or Case Report:
Juvenile Granulosa Cell Tumor of the Testis is a rare testicular tumor that falls within the category of stromal cord neoplasms. There have been less than 50 cases described in the literature with all cases being described as having a cystic component (1, 2). We present a case of a 6 month old boy presenting with unilateral scrotal swelling. Ultrasound evaluation revealed an entirely solid, hypervascular, intratesticular mass. Alpha-fetoprotein levels were negative. At surgery, radical orchiectomy was performed and pathology revealed a Juvenille Granulosa Cell Tumor.
Testicular tumors arising in the neonatal and prepubescent period are a unique set of tumors distinct from their adult counterparts. In this younger age group, germ cell tumors predominate. Of the germ cell tumors, Yolk Sac tumors are the main tumor of clinical significant, and the reason why alpha-fetoprotein is such a relevant marker (3). Stromal cell tumors account for only a small percentage of testicular tumors within this age group. When they do occur, Juvenile Granulosa Cell Tumors (JGC) predominate, particularly if the child is under 1 year of age, with this tumor type being the most common testicular tumor present at birth (3,4). JGC tumors are associated with genetic and structural anomalies of the Y chromosome, ambiguous genitalia, and contralateral undescended testis. One of their defining pathologic characteristics is positive staining with Inhibin (3).To date there are no cases of metastatic JGC tumors. In all reported cases, Sonographic evaluation demonstrates a grossly multicystic tumor (2). The adult variant of Granulosa Cell tumors can appear as a solid mass with little or no cystic component and carries the risk of metastasis of approximately 20% (2). Surgical management of testicular JGC tumors has largely been radical orchiectomy but, some studies have shown tumor sparing excision to be curative in cases with salvageable testicular parenchyma (2). This finding highlights the importance of including stromal cell tumors, particularly JGC, in the differential for solid appearing testicular masses in the neonate. With more reported cases of JGC tumors of the testes, surgical management could include a more conservative approach. Although JGC tumor of the testis is not known to be malignant, given the atypical features of this tumor and similarities with its more malignant adult counterpart, close surveillance is warranted to ensure benign course.
Methods & Materials:
Results:
Conclusions:
Juvenile Granulosa Cell Tumor of the Testis is a rare testicular tumor that falls within the category of stromal cord neoplasms. There have been less than 50 cases described in the literature with all cases being described as having a cystic component (1, 2). We present a case of a 6 month old boy presenting with unilateral scrotal swelling. Ultrasound evaluation revealed an entirely solid, hypervascular, intratesticular mass. Alpha-fetoprotein levels were negative. At surgery, radical orchiectomy was performed and pathology revealed a Juvenille Granulosa Cell Tumor.
Testicular tumors arising in the neonatal and prepubescent period are a unique set of tumors distinct from their adult counterparts. In this younger age group, germ cell tumors predominate. Of the germ cell tumors, Yolk Sac tumors are the main tumor of clinical significant, and the reason why alpha-fetoprotein is such a relevant marker (3). Stromal cell tumors account for only a small percentage of testicular tumors within this age group. When they do occur, Juvenile Granulosa Cell Tumors (JGC) predominate, particularly if the child is under 1 year of age, with this tumor type being the most common testicular tumor present at birth (3,4). JGC tumors are associated with genetic and structural anomalies of the Y chromosome, ambiguous genitalia, and contralateral undescended testis. One of their defining pathologic characteristics is positive staining with Inhibin (3).To date there are no cases of metastatic JGC tumors. In all reported cases, Sonographic evaluation demonstrates a grossly multicystic tumor (2). The adult variant of Granulosa Cell tumors can appear as a solid mass with little or no cystic component and carries the risk of metastasis of approximately 20% (2). Surgical management of testicular JGC tumors has largely been radical orchiectomy but, some studies have shown tumor sparing excision to be curative in cases with salvageable testicular parenchyma (2). This finding highlights the importance of including stromal cell tumors, particularly JGC, in the differential for solid appearing testicular masses in the neonate. With more reported cases of JGC tumors of the testes, surgical management could include a more conservative approach. Although JGC tumor of the testis is not known to be malignant, given the atypical features of this tumor and similarities with its more malignant adult counterpart, close surveillance is warranted to ensure benign course.
Methods & Materials:
Results:
Conclusions:
More abstracts on this topic:
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Poster____CR-074.pdf
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