Dottersacktumor

Dottersacktumor, also known as yolk sac tumor or endodermal sinus tumor, is a malignant germ cell tumor most commonly found in children. It often secretes the tumor marker alpha-fetoprotein (AFP), which is typically elevated in serum and can be used to monitor response to treatment.

Epidemiology and sites of origin vary. Yolk sac tumors are the most common malignant germ cell tumor in children and can arise in the ovaries or testes. They may also occur in extragonadal midline locations such as the sacrococcygeal region, mediastinum, or retroperitoneum. In adults, the tumor is rarer and may present differently.

Pathology and diagnosis are characterized by specific histology and biomarker features. The tumor often shows Schiller-Duval bodies on microscopic examination, a distinctive pattern resembling primitive glomeruli. Immunohistochemistry typically demonstrates AFP positivity. Serum AFP levels are a key diagnostic and follow-up measure, often markedly elevated at diagnosis and decreasing with successful treatment. Imaging studies aid in locating the tumor and assessing spread, and biopsy confirms the diagnosis when feasible.

Clinical presentation depends on tumor location. Ovarian yolk sac tumors may present as an abdominal mass or pelvic symptoms; testicular tumors typically present as a testicular enlargement. Extragonadal tumors cause symptoms related to mass effect in the affected region.

Treatment generally involves surgical resection when possible, followed by chemotherapy. Platinum-based regimens, such as bleomycin, etoposide, and cisplatin (BEP), are commonly used and can achieve high cure rates in children. Prognosis is favorable in early-stage disease, with long-term survival improving substantially with modern treatment, though outcomes are more variable in adults and advanced cases. Ongoing research aims to reduce treatment toxicity and improve relapse-free survival.