Ganglioneuroma presenting as subpulmonic effusion—a differential to consider?

Abstract

Ganglioneuroma is a benign, slow-growing neurogenic tumor arising from neural crest cells. It is extremely rare (1/1,000,000) and is located most commonly in the posterior mediastinum (41.5%), retroperitoneum (37.5%), and adrenal glands (21%). We present a case of a 62-year-old lady who had complaints of shortness of breath on exertion and dyspnea for the past 3 months. She had no other significant history. Computerised tomography (CT) scan of the thorax suggested left-sided loculated subpulmonic pleural effusion, 14 × 12 cm in dimension. She underwent assisted video-assisted thoracoscopic surgery (VATS) exploration of the thorax with debridement and drainage of subpulmonic collection that was abutting the diaphragm, along with release of trapped lung. Histopathological examination showed multiple ruptured cystic masses with nodules; microscopical evidences of Schwann cells, ganglion cells, and spindle cells—all these along with immunohistochemistry—revealed features consistent with ganglioneuroma. Postoperative recovery was uneventful, and the patient did not have any complaints or other limitations to daily life activities at 6 months’ follow-up. Ganglioneuroma is essentially benign in nature, asymptomatic, and rare. A systematic review of the literature has shown that giant-sized ganglioneuromas (size more than 10 cm) have rarely been reported. Surgical excision and clearance is the treatment modality of choice. In our case, due to large size and difficulty in access and mobilisation of the mass adherent to the diaphragm, assisted VATS had to be performed. We increased the size of the utility port from 5 to 10 cm and used a rib retractor for better surgical negotiation. This could have been more challenging, as there have been incidences where ganglioneuromas have extended both into the thoracic and abdominal cavities and even involved vital organs and vessels. Regular follow-up is essential, as late recurrence and slow progression potential is a known complication.