Juvenile granulosa cell tumor of the testis: prenatal diagnosis and prescrotal approach

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Abstract

Neonatal testicular tumors are rare and should be considered in the differential diagnosis of newborn scrotal masses. Juvenile granulosa cell tumor (JGCT) accounts for about 5% of all prepubertal testis tumors. As a benign neoplasm, radical orchiectomy is sufficient for treatment. We report a case of a newborn with a prenatal diagnosis of scrotal mass. After surgery, the histological diagnosis was juvenile granulosa cell tumor. To date the patient is healthy.

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Serum alpha fetoprotein (AFP) levels in normal infants.

K Sudar, L Book, Qing J. Wu (1980)

Serum alpha-fetoprotein (AFP) levels were monitored in 32 normal babies consecutively from 2 to 3 days, 2 wk, and 2 and 4 months after birth. In addition, serum AFP concentration was also measured in 116 random specimens from infants with normal liver enzymes and 10 infants born immaturely. Results were combined to establish normal AFP levels for infants at various ages. Serum AFP disappeared rapidly after birth. We found that it was not until 8 months of age that the normal AFP level in infants approached adult level. The half-lives of AFP degradation were estimated to be 5.5 days between birth and 2 wk, 11 days between 2 wk to 2 months, and 33 days between 2 and 4 months of age. In contrast to earlier belief, we felt that some AFP synthesis still exists after birth; however, the rate of synthesis may also decrease with age.

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Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis.

Aseem Shukla, Dale S Huff, Douglas A. Canning … (2004)

Juvenile granulosa cell tumor (JGCT) of the testis is a rarely diagnosed subset of testicular stromal tumors. Although this variant of testicular stromal tumor is predominantly a benign entity in prepubertal patients, limited experience precludes a complete understanding of its clinical presentation and pathological diagnosis. We reviewed all cases of testicular tumors at Children's Hospital of Philadelphia between 1976 and 2002 in males younger than 18 years. We specifically reviewed our experience with JGCT in terms of presentation, surgical treatment and long-term outcome. We also reviewed the microscopic findings and histochemical techniques used to confirm the diagnosis. We identified 77 tumors during the defined interval, of which 3 (3.9%) were JGCTs. All 3 patients with JGCT were first noted to have a testis mass soon after birth. All presented with a firm, unilateral testicular mass. Ultrasonographic findings were consistent with a complex, multiseptated, hypoechoic mass. Two of the 3 patients underwent radical orchiectomy. Testis sparing mass excision was performed in 1 patient. Grossly the tumors were partially cystic masses. Histologically positive immunostaining with inhibin-alpha and negative staining for alpha-fetoprotein (AFP) reliably differentiated JGCTs from yolk sac tumors. At a mean followup of 8.5 years (range 5 to 14) no metastases or local tumor recurrences have been diagnosed. To our knowledge we report the first case of testis sparing enucleation of a JGCT with a 5-year recurrence-free followup. Testis sparing enucleation is now our procedure of choice for tumors in neonates and prepubertal children with serum AFP in the normal range for age. JGCT should be suspected in neonates presenting at birth with a complex, cystic mass of the testis. Positive immunostaining for inhibin-alpha and a lack of AFP staining have consistently corroborated the pathological diagnosis in our experience and they should be applied for pediatric testis tumors that may mimic yolk sac tumor pathology.

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Testis-sparing surgery for benign testicular tumors in children.

(2001)

In this retrospective survey we identify preoperative and intraoperative criteria of nonmalignancy and analyze the result of conservative treatment of a testicular mass. A total of 22 surgeons responded to a questionnaire concerning lesions treated during the last 15 years in children between 0 and 15 years old. By definition alpha fetoprotein and beta human chorionic gonadotropin are within the normal limits at this age. The results were evaluated regarding evolution, growth of the preserved testis and local or distant recurrences. Benign tumor of the testis (83 cases) represented 48% of all cases. Orchiectomy was performed in 27 cases and conservative treatment in 56. The final histopathological diagnosis was benign germinal tumor in 48 cases, cysts in 18, gonadal stromal tumor in 13 and rare lesions (lipoma, hemangioma) in 4. No definitive clinical criteria of nonmalignancy were identified but some symptoms were suggestive of nonmalignancy. Ultrasound results were more conclusive and provided the diagnosis of teratoma, epidermoid cyst and particularly simple cyst. The conservative treatment performed 56 times was a simple biopsy in 2 children with bilateral lesions and enucleation in 52. Enucleation was performed in 43% of cases using a pedicle clamp and in 50% with frozen section. The frozen section was changed in 12 cases due to therapeutic decision for preservation (10) and orchiectomy (2). There were no contradictions between the definitive histopathological examination and frozen section. Secondary orchiectomy was performed for neonatal granular tumor. Average followup in 56 cases of conservative treatment was 4.8 years (range 6 months to 15 years). Neither secondary testicular atrophy nor any local or distant recurrence was recorded. A testicular tumor in children has a 50% chance of being benign. Treatment selection according to some clinical, biological, radiological and frozen section findings should allow us to decide on testis-sparing surgery without additional oncological risk, and with an aesthetic, psychological and functional benefit.

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Author and article information

Journal

Journal ID (nlm-ta): Ital J Pediatr

Journal ID (iso-abbrev): Ital J Pediatr

Title: Italian Journal of Pediatrics

Publisher: BioMed Central

ISSN (Electronic): 1824-7288

Publication date Collection: 2012

Publication date (Electronic): 6 December 2012

Volume: 38

Page: 67

Affiliations

[1 ]Division of Pediatric Surgery, Department of Pediatrics, Obstetrics and Reproductive Medicine, University of Siena, Siena, Italy

Article

Publisher ID: 1824-7288-38-67

DOI: 10.1186/1824-7288-38-67

PMC ID: 3585781

PubMed ID: 23217189

SO-VID: 4a4b3784-d83e-4bb6-ba33-234f4ab9ab08

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This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Juvenile granulosa cell tumor of the testis: prenatal diagnosis and prescrotal approach

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Pediatric Pulse: Advancing Neonatal and Child Health

Most cited references 7

Serum alpha fetoprotein (AFP) levels in normal infants.

Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis.

Testis-sparing surgery for benign testicular tumors in children.

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