Pediatria pre prax 1/2022
Primary mediastinal germ cell tumor
Purpose: Germ cell tumors are a heterogeneous group of tumors. The treatment and prognosis depend on the histological type, localisation, but also on the stage of the tumor and the age of the patient at the time of diagnosis. Case: 12-year-old boy was repeatedly examined by a pediatrician due to the persistent cough. He was treated with azithromycin due to suspicion of respiratory tract infection and positivity of IgM antibodies against Chlamydia pneumonia. Chest X-ray was negative. Treatment was without any clinical response. Dry cough lasted almost 6 months, patient was in good general condition, without dyspnoe, subfebrilities and weight loss 6 kg were later associated. Controlled chest X ray three months apart the previous one showed surprising finding of a giant heterogeneous tumor of the upper and middle anterior mediastinum and a massive pericardial effusion. CT scan confirmed pressure on surrounding vessels and structures. Elevated tumor markers (AFP) confirmed the diagnosis of primary mediastinal germ cell tumor, distant metastases were not detected. The tumor was inoperable, biopsy was risky. Chemotherapy was indicated. In its beginning there was a critical decompensation of the clinical condition with the need for intensive monitoring and supportive treatment, with puncture of the pericardial effusion. During chemotherapy AFP declines to normal levels, we observed favorable clinical and radiological response and tumor resection was possible (partial, via thoracotomy). Multicystic teratoma was confirmed by histological examination. Persistent metabolic activity on 18 FDG PET CT in residual tumor was present, adjuvant chemotherapy was administered postoperatively. The patient is without symptoms of disease activity for 1 year and 3 months after the end of the treatment, it is confirmed by imaging examinations and normal levels of oncomarkers. Conclusion: Primary mediastinal germ cell tumor is a rare malignancy in children and represents about 6 % of all mediastinal tumors. These tumors may be clinically asymptomatic for a long time, later non-specific symptoms such as cough, chest pain and weight loss may occur. Patients achieve long-term remission in 57–88 %. As with other oncological diagnoses, early diagnosis has a significant impact on the patient’s chances of a complete cure.
Keywords: mediastinal germ cell tumors, mediastinal tumors, cough, pericardial effusion, circulatory failure
