Congenital rhabdomyosarcoma of the chest wall

Abstract

Introduction. Rhabdomyosarcoma (RMS) is the most common soft tissue malignancy of childhood, but is very rare in the neonatal period. It may arise anywhere in the body, but it has a predilection for the head and neck area, genito-urinary tract and the extremities. Chest wall is a rare site for RMS. We report the case of a neonate with embryonal RMS arising from the posterior chest wall muscles at birth. The case is reported because of the extreme rarity of RMS in neonates and its unusual location in the chest wall. CASE REPORT A 28-year-old woman (gravida 2, para 2) underwent a routine ultrasound examination at 33 weeks’ gestation and fetal ascites was observed. She was referred to a tertiary center and her serology (TORCH) was negative. At 34 GW, she gave birth to a female neonate (birth weight, 3,340 g; Apgar scores of 3, 4 and 7 at 1, 5 and 10 min, respectively) via planned cesarean section. The neonate was tracheally intubated soon after birth, and her heart rate gradually increased. Her abdomen was massively distended with palpable fluctuation, but no petechiae were detected. We performed drainage of ascites, about 530 ml over 4 days. The peritoneal fluid which showed normal transudate signs, no malignant cells. A blood test showed anaemia (86 g/l), thrombocytopenia (120 × 109/l). After 3 days the newborn was extubated, her breathing was normal. However, respiratory distress was progressive at one week old, she needed CPAP therapy and FiO2 – 1.0. At chest X-ray there was abnormal mediastinum and abnormal mass in the interior part of right lung, and after two days, the condition of the right lung worsened. We performed chest MRT, which showed probable intra-thoracic tumor with possible invasion of the abdominal wall (Fig. 1). Histological examination was performed, yielding a diagnosis of embryonal rhabdomyosarcoma. Metastasis was found in the diaphragm; no metastasis was found in the other organs. Surgical [...].