juvenile type testicular granulosa cell tumor

Juvenile Type Testicular Granulosa Cell Tumor: A Rare but Benign Neoplasm

Testicular juvenile granulosa cell tumors (JGCTs) are a rare and benign type of sex cord-stromal tumor that primarily affects infants within the first 6 months of life [1]. These tumors account for less than 5% of all neoplasms of the prepubertal testis [2].

Characteristics

Juvenile granulosa cell tumors are typically composed of primitive appearing granulosa cells growing in solid and follicular patterns [3]. The adult granulosa cell-like pattern is rare, but when present, it can be distinguished by cells with oval to angulated nuclei and scant cytoplasm [4].

Clinical Presentation

JGCTs commonly present as a painless testicular, inguinal, or intra-abdominal mass [5]. A minority of cases are found during a workup for genital ambiguity, undescended testes, or testicular torsion [6-7].

Prognosis and Treatment

Juvenile granulosa cell tumors have an excellent prognosis, with no reported metastases or recurrences in the literature [8]. Surgical treatment is recommended, and gonadectomy is often performed to remove the tumor [9].

Key Statistics

• JGCTs account for less than 5% of all neoplasms of the prepubertal testis [2].
• These tumors primarily affect infants within the first 6 months of life [1].
• The adult granulosa cell-like pattern is rare, but when present, it can be distinguished by cells with oval to angulated nuclei and scant cytoplasm [4].

References

[1] Vatta F. (2019). Juvenile-type granulosa cell tumor of the testis: A case report. [Cited by 7]

[2] Collins K. (2023). Testicular juvenile granulosa cell tumors: A review of the literature. [Cited by 5]

[3] Nov 7, 2023. Juvenile granulosa cell tumor: A neoplasm composed of primitive appearing granulosa cells.

[4] Kao CS. (2015). Adult granulosa cell-like pattern in juvenile granulosa cell tumors. [Cited by 68]

[5] Bulotta AL. (2023). Granulosa cell tumor: A benign neoplasm with a rare adult granulosa cell-like pattern.

[6] Testicular juvenile granulosa cell tumors: A review of the literature. [Cited by 4]

[7] Prepubertal testicular tumors: A review of the literature. [Cited by 3]

[8] Juvenile granulosa cell tumor of the testis: A case report. [Cited by 2]

[9] Gonadectomy for juvenile granulosa cell tumor: A review of the literature.

Common Signs and Symptoms

Juvenile-type testicular granulosa cell tumors are rare sex cord-stromal tumors that can occur in children and adolescents. The most common signs and symptoms of this condition include:

• Painless lump in the scrotum: A painless lump or mass in the testicle is the most common sign of juvenile-type testicular granulosa cell tumor [1, 5].
• Feeling of weight in the scrotum: Some children may experience a feeling of weight or heaviness in the scrotum due to the presence of the tumor [1].
• Testicular mass: A painless testicular mass is another common presentation of this condition, which can be felt by a healthcare provider during a physical examination [6, 7].

Other Possible Symptoms

In some cases, children with juvenile-type testicular granulosa cell tumors may also experience:

• Bloating: Some children may experience bloating or swelling in the scrotum due to the tumor [5].
• Pain: Although rare, some children may experience pain in the scrotum or abdomen due to the tumor [7].

Important Notes

It's essential to note that these symptoms can also be caused by other conditions, and a definitive diagnosis of juvenile-type testicular granulosa cell tumor requires a biopsy and histopathological examination. If you suspect any of these symptoms in your child, it's crucial to consult with a healthcare provider for proper evaluation and treatment.

References:

[1] Context result 1 [2] Context result 5 [3] Context result 6 [4] Context result 7

Diagnostic Tests for Juvenile Type Testicular Granulosa Cell Tumor

The diagnosis of juvenile type testicular granulosa cell tumor (JGCT) can be challenging due to its rarity and similarity in appearance to other testicular tumors. However, several diagnostic tests can help confirm the presence of JGCT.

• Blood Tests: Blood tests are often done to find tumor markers, such as alpha-fetoprotein (AFP), which is elevated in some cases of JGCT [3].
• Ultrasonography: Ultrasonography is a valuable imaging modality for diagnosing testicular tumors, including JGCT. It can help identify the presence and characteristics of the tumor, such as its size, location, and texture [5].
• Computed Tomography (CT) Scanning or Magnetic Resonance Imaging (MRI): CT scanning or MRI may be used to evaluate the extent of disease and rule out metastasis or involvement of other organ systems prior to surgery [8].
• Cytogenetic Studies: Cytogenetic studies, such as fluorescence in situ hybridization (FISH) and/or array comparative genomic hybridization (aCGH), can provide valuable information on the genetic characteristics of the tumor [9].

Key Points

• Blood tests may be used to find tumor markers.
• Ultrasonography is a useful imaging modality for diagnosing testicular tumors, including JGCT.
• CT scanning or MRI may be used to evaluate the extent of disease and rule out metastasis or involvement of other organ systems prior to surgery.
• Cytogenetic studies can provide valuable information on the genetic characteristics of the tumor.

References

[3] Blood tests to find tumor markers are often done when a testicular tumor is suspected. Elevated levels of alpha-fetoprotein (AFP) may be present in some cases of JGCT. [5] Ultrasonography is the best imaging modality to diagnose testicular tumours, including JGCT. [8] Abdominopelvic CT scanning or MRI may help in diagnosing intraperitoneal spread or involvement of other organ systems prior to surgery. [9] Cytogenetic studies, such as FISH and/or aCGH, can provide valuable information on the genetic characteristics of the tumor.

Treatment Options for Juvenile Type Testicular Granulosa Cell Tumor

Juvenile type testicular granulosa cell tumors (JGCTs) are rare sex cord-stromal tumors that primarily affect children and adolescents. While surgical removal of the affected testicle is often considered the primary treatment, there are other drug-based treatments that have shown promise in managing this condition.

Surgical Treatment

Radical orchiectomy, which involves the removal of the affected testicle, is often performed on an outpatient basis [2]. This procedure is usually recommended for children with a testicular tumor and can help find and likely cure the cancer [1].

Chemotherapy Regimens

Chemotherapy regimens such as cisplatin (PEb) or carboplatin (JEb) have been used successfully in children to treat JGCTs [6, 13, 14, 20]. BEP is a common regimen that has shown effectiveness in managing this condition [6].

Targeted Therapy

The use of pazopanib, an investigational multitargeted receptor tyrosine kinase inhibitor, has been reported to have a major response in treating testicular granulosa cell tumors [11]. This treatment option shows promise and warrants further investigation.

**Other Considerations

The differential diagnosis of juvenile-type testicular granulosa cell tumor (JGCT) includes several conditions that can present with similar clinical and histological features.

Key Conditions to Consider:

• Yolk Sac Tumor: This is a malignant germ cell tumor that can present with elevated alpha-fetoprotein (AFP) levels. It is often distinguished