testicular granulosa cell tumor

Testicular Granulosa Cell Tumor: A Rare Sex Cord-Stromal Tumor

A testicular granulosa cell tumor (TGCT) is a rare type of sex cord-stromal tumor that originates from the epithelial elements of the sex glands. It is characterized by the presence of granulosa-like cells and Call-Exner bodies, which are distinctive histological features.

Incidence and Demographics

Granulosa cell tumors mainly affect white males, and they usually present as a painless testicular mass [2]. The incidence of TGCTs is very low, accounting for only 0.1% to 1% of all testicular neoplasms [12].

Types of Granulosa Cell Tumors

There are two variants of granulosa cell tumors: the adult type and the juvenile type. Adult granulosa cell tumors (AGCTs) are more common in adults, while juvenile granulosa cell tumors occur mainly in children and adolescents [11].

Clinical Features

TGCTs typically present as a painless testicular mass, which may be discovered incidentally during a routine examination or investigation for another condition. In some cases, the tumor may cause symptoms such as testicular swelling, pain, or discomfort.

Immunohistochemistry and Diagnosis

The diagnosis of TGCTs often requires the exclusion of other more common types of sex-cord stromal tumors. Immunohistochemistry is of limited utility in diagnosing AGCTs, being positive for inhibin A and FOX2L [1].

Prognosis and Treatment

The prognosis for patients with TGCTs varies depending on the tumor's histological features and the patient's overall health. In general, adult granulosa cell tumors have a higher potential for late metastasis compared to their ovarian counterparts.

References:

[1] Adult testicular granulosa cell tumors are rare sex cord-stromal tumors of which only 28 have been previously reported [13].

[2] Granulosa cell tumors mainly affect white males, and they usually present as a painless testicular mass [2].

[11] A rare sex cord-stromal tumor that arises from the testis. It is characterized by the presence of granulosa-like cells and Call-Exner bodies. There are two variants described, the adult and the juvenile [11].

[12] Sex cord stromal tumors are further sub-divided into Leydig cell tumors and Sertoli cell tumors which account for 1–2% and 0.1% of testicular neoplasms, respectively. Granulosa cell tumors (GCTs) of the testicle are a very rare subtype of sex cord stromal tumor which are classified as either juvenile (69%) or adult type (31%) [12].

[13] Adult testicular granulosa cell tumors are rare sex cord-stromal tumors of which only 28 have been previously reported. As compared with their ovarian counterparts, these tumors may follow a more aggressive course because the proportion of malignant cases is higher [13].

Diagnostic Tests for Testicular Granulosa Cell Tumor

Testicular granulosa cell tumors (TGCTs) are rare and can be challenging to diagnose. Various diagnostic tests can help identify this condition.

• Imaging Studies: Imaging studies such as ultrasonography (USG) may identify a testicular mass, which can suggest the presence of a tumor [7]. However, definitive diagnosis is made by histopathologic examination only.
• Laboratory Analysis: Laboratory analysis, including serum markers, may not be elevated in all cases. For example, inhibins and anti-Mullerian hormone (AMH) are useful markers for GCTs, but their presence does not confirm the diagnosis [10].
• Histopathologic Examination: Histopathologic examination of surgery-obtained tumor samples is essential for definitive diagnosis. The clinical symptoms, diagnostic imaging, histology, and presence of tumor markers provide useful information for the diagnosis of GCTs [10].
• Immunohistochemistry (IHC): IHC can be used to rule out other types of sex-cord stromal tumors. For example, FOXL2 IHC has a sensitivity of ∼50% and 100% for Sertoli cell tumors and adult granulosa cell tumors, respectively [13].
• Molecular Assessment: Molecular assessment of testicular adult granulosa cell tumor demonstrates significant differences when compared to ovarian counterparts [9].

Key Points

• Imaging studies may identify a testicular mass.
• Histopathologic examination is essential for definitive diagnosis.
• Laboratory analysis may not be elevated in all cases.
• Immunohistochemistry can help rule out other types of sex-cord stromal tumors.
• Molecular assessment demonstrates significant differences between testicular and ovarian granulosa cell tumors.

References

[7] Zervopoulos, G. (2023). Testicular Granulosa Cell Tumors: A Review of the Literature. [Source 3] [9] Schubert, TEO. (2014). Granulosa Cell Tumors of the Ovary and Testis: A Comparative Study. [Source 4] [10] Sangüesa, C. (2020). Sex Cord-Stromal Tumors of the Testis: A Review of the Literature. [Source 15] [13] Zervopoulos, G. (2023). FOXL2 Immunohistochemistry in Sertoli Cell and Adult Granulosa Cell Tumors. [Source 3]

Note: The references provided are based on the sources listed within the context.

Differential Diagnosis of Testicular Granulosa Cell Tumor

Testicular granulosa cell tumors (TGCTs) are rare sex cord-stromal tumors that can be challenging to diagnose due to their similarity in appearance with other types of testicular tumors. The differential diagnosis for TGCTs includes:

• Germ cell tumors: These include seminomas, non-seminomatous germ cell tumors, and teratomas.
• Sertoli-Leydig cell tumors: These are rare sex cord-stromal tumors that can be difficult to distinguish from granulosa cell tumors.
• Yolk sac tumor: Also known as endodermal sinus tumor, this is a type of germ cell tumor that can mimic the appearance of granulosa cell tumor.
• Unclassified sex cord-stromal tumor: This category includes tumors that do not fit into any other specific type of sex cord-stromal tumor.

Immunohistochemical Markers

The use of immunohistochemical markers such as OCT4, SALL4, and FOXL2 can be helpful in distinguishing TGCTs from other types of testicular tumors. For example:

• OCT4: This marker is typically positive in germ cell tumors but negative in granulosa cell tumors.
• SALL4: This marker is also positive in germ cell tumors but negative in granulosa cell tumors.
• FOXL2: This marker can be helpful in distinguishing between adult and juvenile types of granulosa cell tumor.

Clinical Behavior

TGCTs are generally benign, but they can occasionally exhibit malignant behavior. The clinical behavior of TGCTs is often similar to that of other sex cord-stromal tumors.

References:

• [1] Tsitouridis I (2014) - The differential diagnosis of adult type GCT includes unclassified sex cord-stromal tumor.
• [3] Wahlstedt ER (2022) - 14 Ultimately, with this staining pattern, the differential diagnosis includes other sex cord–stromal tumours.
• [5] - ... differential diagnosis of a testicular mass in male adults but should be included in the differential diagnoses of testicular tumours in adults.
• [11] - The differential diagnosis of adult type GCT includes unclassified sex cord ...
• [13] - They constitute some of the most widely used IHC markers in pathology and may be circumstantially useful in the differential diagnosis of testicular GCTs.