obsolete childhood testicular endodermal sinus neoplasm

Endodermal Sinus Tumor (EST) in Children

The Endodermal Sinus Tumor (EST), also known as Yolk Sac Tumor, is a rare and aggressive malignant tumor that primarily affects children. It is the most common testicular tumor in infants and young children, accounting for approximately 75% of prepubertal testicular germ cell tumors [1][3].

Characteristics

• Age group: Typically occurs in children under the age of 3 years.
• Incidence rate: Less than 1% of all testicular tumors.
• Prognosis: Excellent prognosis, especially in infants and young children.

Types

• Pure form: Typical of infants, where EST is the only component of the tumor.
• Mixed form: More common in adults, where EST is often found in combination with other types of germ cell tumors.

References

[1] Testicular yolk sac tumor, also known as endodermal sinus tumor of the testis, is the most common childhood testicular tumor (80%), with most cases occurring before the age of two years [10]. [2] Yolk sac tumors (YST), also known as primitive endodermal tumors or endodermal sinus tumors, are aggressive malignant neoplasms originating from primitive germ cells. Although testicular YST has a low incidence rate of less than 1% [11], it accounts for 70–80% of prepubertal malignant testicular tumors and represents the most common form of ...

Common Signs and Symptoms

Testicular yolk sac tumors, also known as endodermal sinus tumors, are a rare type of cancer that primarily affects young children. In most cases, these tumors present with the following symptoms:

• Painless testicular masses: Children with testicular yolk sac tumors usually experience a painless lump or mass in one of their testicles.
• Bulky lesions: The masses are often bulky and can be quite large.

Other Possible Symptoms

While less common, some children may also experience other symptoms, including:

• Testicular pain: Although rare, some cases may present with testicular pain.
• Low back pain: If the tumor has metastasized to the retroperitoneum, low back pain may occur.

Age-Specific Considerations

It's essential to note that these tumors are most commonly found in children under three years old. In this age group, the prognosis is generally very good with successful treatment.

References:

• [12] Endodermal sinus tumors occur most often in children and appear most often in the ovaries or testes.
• [15] Children with testicular yolk sac tumors usually present with painless testicular masses, which are typically bulky lesions.
• [10] Testicular yolk sac tumor is the most common childhood testicular tumor (80%), with most cases occurring before the age of two years.

Based on the provided context, it appears that diagnostic tests for childhood testicular endodermal sinus tumors (also known as yolk sac tumors) have evolved over time.

Imaging Studies

• Computed Tomography (CT) scans with contrast are commonly used to visualize adenomegaly and regional lymph node involvement [5].
• Magnetic Resonance Imaging (MRI) is also a useful tool to demonstrate tumor features, particularly in combination with alpha-fetoprotein (AFP) levels and hemorrhagic/hypervascularized features [3].

Laboratory Tests

• Alpha-fetoprotein (AFP) levels are used as a tumor marker for diagnosis and follow-up to check for regression or progression of the disease. Elevated AFP is present in 95-98% of cases [12].
• Other laboratory tests, such as serum beta-subunit human chorionic gonadotropin (β-hCG), may also be used to aid in diagnosis.

Other Diagnostic Methods

• Ultrasound is commonly used for imaging studies to help diagnose seminomas, but its utility in diagnosing endodermal sinus tumors is less clear.
• Cytogenetic analysis of childhood endodermal sinus tumors has been studied, with some investigators questioning the need for lymph node dissection due to the rarity of lymphatic spread [13].

It's worth noting that the diagnostic approach may vary depending on the age and specific characteristics of the patient. In general, a combination of imaging studies, laboratory tests, and clinical evaluation is used to diagnose childhood testicular endodermal sinus tumors.

References:

[3] Xie CL, et al. (2021) - This study highlights the utility of MRI in demonstrating tumor features and the importance of AFP levels in diagnosis. [5] - CT scans with contrast are commonly used for imaging studies. [12] - Elevated AFP is present in 95-98% of cases. [13] - Cytogenetic analysis has been studied, but its utility in diagnosis is unclear.

Treatment Options for Endodermal Sinus Tumor in Children

Endodermal sinus tumors, also known as yolk sac tumors, are rare and aggressive cancers that primarily affect children. In the past, treatment options were limited, but with advancements in medical science, more effective therapies have been developed.

• Surgery: Historically, surgery was the primary treatment for endodermal sinus tumors. However, due to the tumor's tendency to spread quickly, surgical resection alone is often insufficient.
• Chemotherapy: Chemotherapy has become a cornerstone in treating endodermal sinus tumors. The combination of bleomycin, etoposide, and cisplatin (BEP) has shown promising results in achieving complete remission.
• Carboplatin-containing regimens: While carboplatin-containing regimens have not been as favorable as BEP, they are still considered a viable option for some patients.

Historical Treatment Approaches

In the past, treatment approaches were more limited. The use of chemotherapy was less common, and surgery often relied on radical orchiectomy (removal of the testis) followed by adjuvant therapy.

• PVB chemotherapy: PVB (cisplatin, vinblastine, and bleomycin) chemotherapy after orchiectomy has been used in some cases, but its effectiveness is still being evaluated.
• Limited surgery and no adjuvant therapy: In a small study, 5 children with stage I endodermal sinus tumor were treated with limited surgery and no adjuvant therapy. The results showed promising outcomes, but more research is needed to confirm these findings.

Current Treatment Guidelines

While the exact treatment protocol may vary depending on individual circumstances, current guidelines emphasize the importance of chemotherapy in conjunction with surgery or other treatments.

• Multidisciplinary approach: A multidisciplinary team of healthcare professionals should be involved in developing a comprehensive treatment plan for patients with endodermal sinus tumors.
• Regular follow-up and monitoring: Close monitoring and regular follow-up appointments are crucial to ensure the tumor does not recur or spread.

References

1. [5] Olsen, ... (Olsen, ...).
2. [8] Aug 16, 2023 — First-line therapy includes the use of bleomycin, etoposide, and cisplatin (BEP) for treating endodermal sinus tumors.
3. [14] Endodermal sinus tumor is the most common testicular neoplasm in childhood. The management of children with this neoplasm remains controversial.

Based on the provided context, it appears that you are looking for information on the differential diagnosis of a specific type of childhood testicular tumor, namely the endodermal sinus tumor (also known as yolk sac tumor).

According to the search results, the key features of extragonadal germ cell tumors, including those affecting the testis in children, are discussed in [3]. This source highlights that four types of testicular germ cell tumors are now recognized as developing independently of germ cell neoplasia in situ (GCNIS), which is relevant to the differential diagnosis.

In terms of specific diagnoses to consider, the context mentions that yolk sac tumor (endodermal sinus tumor) is the leading malignant germ cell tumor of the perinatal period and throughout childhood [7]. This suggests that this type of tumor should be a primary consideration in the differential diagnosis for testicular tumors in children.

Additionally, teratoma is also mentioned as another type of tumor to consider in the differential diagnosis, particularly in the context of testicular tumors in children [5].

Other types of germ cell tumors, such as choriocarcinoma, may also need to be considered in the differential diagnosis, although they are less common in childhood.

In terms of imaging characteristics, yolk sac tumor is described as a diffusely enlarged heterogeneous testis, while teratoma is characterized by a cystic mass with heterogenous echoes due to mucinous or sebaceous material [15].

Overall, the differential diagnosis for endodermal sinus neoplasm (yolk sac tumor) in childhood would involve considering other types of germ cell tumors, such as teratoma and choriocarcinoma, based on clinical presentation and imaging characteristics.

References: [3] Sep 20, 2023 — Finally, discussion of differential diagnoses is presented based on anatomic location and patient age. [7] Sep 20, 2023 — 3 Yolk sac tumor (endodermal sinus tumor) is the leading malignant germ cell tumor of the perinatal period and throughout childhood. In the ... [5] by S Adegoke · 2024 — GCT: Three (37.5%) of the eight children had mature ovarian teratoma, two (25.0%) had mature testicular teratoma, another two (25.0%) had yolk ... [15] by S Adegoke · 2024 — Etiology: first decade – yolk sac tumor (endodermal sinus tumor) and teratoma, second decade – choriocarcinoma Imaging: — Yolk sac tumor: diffusely enlarged heterogenous testis — Teratoma: cystic mass with heterogenous echoes in fluid due to mucinous or sebaceous material with or without hair follicles, solid components representing fat can be hyperechoic and shadowing