Aggressive multimodal therapy may prolong disease-free survival in recurrent primary retroperitoneal embryonal carcinoma

• 5% of extragonadal germ cell tumours are currently considered to be of primary retroperitoneal origin.
• Retroperitoneal EGGCTs are assumed to a poor prognosis group even if they fulfill the IGCCCG criteria for good, or intermediate prognosis.
• The combination of radical surgery and chemotherapy may prolong disease-free survival even in a second- or third-line treatment.

IntroductionPrimary retroperitoneal extragonadal tumours relapsing after initial chemotherapy have a poor prognosis.Presentation of the caseWe report a case of primary retroperitoneal embryonal carcinoma in a patient with negative open testes biopsy. After the first line of chemotherapy (4 cycles BEP) secondary surgery with extirpation of a retroperitoneal residual mass was performed. The residuum proved histologically to be a mature teratoma, and no adjuvant treatment was given according to current recommendations. The patient had regular follow-up. 3.5 years later, patient developed recurrence in the ipsilateral adrenal gland, which was treated with surgery and 4 cycles of salvage VeIP chemotherapy. Seven months after the second surgical intervention the patient underwent multivisceral “desperation surgery” for early metastatic disease progression followed by 2 cycles of salvage TIP chemotherapy. The patient is currently disease-free at 34 months.ConclusionInitial postchemotherapy retroperitoneal lymph node dissection is crucial for local retroperitoneal disease control. Aggressively treated metastatic recurrent disease does not preclude prolonged survival. Despite a generally poor prognosis, repeated complex oncosurgical therapy for retroperitoneal extragonadal tumours may be worthwhile.