Patient Case Discussion
Case Summary
Presented was a case of a young girl (nearly 4 years old) with recurrent pneumonia and a growing retrocardiac mass, which was later identified as a large paraspinal neuroblastoma. The surgical team discussed the diagnostic process, surgical approach, and histological findings of the tumor, which was determined to be a ganglioneuroblastoma with favorable histology. The oncology follow-up plan and potential complications of the surgical procedure were also addressed.
Retrocardiac Mass in a Child
The girl was born via C-section and had a possible bicuspid aortic valve with mild stenosis. She also had a history of recurrent pneumonia and eczema. The family had no history of lung diseases or malignancies. The girl had two episodes of pneumonia, with the second one showing a retrocardiac consolidation on a chest X-ray. The radiology team confirmed the findings and noted that the mass was becoming more pronounced over time, growing slowly over a year.
Mediastinal Mass and Pneumonia Diagnosis
The mass was initially thought to be a retrocardiac pneumonia, but subsequent X-rays showed it was persistent. It was suggested that a follow-up X-ray after a potential pneumonia diagnosis could help identify if the mass was related to the pneumonia. The team agreed that the mass could have contributed to the pneumonia because of external compression. But a decision to use a CT scan instead of an MRI was not clear. However, the CT scan showed a large, well-defined posterior mediastinal mass with calcifications and extension to the neural foramina.
Large Paraspinal Neuroblastoma in a Child
There was a large paraspinal neuroblastoma in the child, which was heterogeneous and had extensions to the neural foramina. The tumor was MIBG positive, indicating it was an active tumor. The differential diagnosis included neural crest tumors. The surgical team considered the possibility of observing the tumor if it was asymptomatic and not invading nearby structures. However, the size of the tumor and its MIBG positivity suggested that intervention was necessary. The markers for catecholamines were negative, which was unusual for an MIBG positive tumor. The team agreed that the tumor was likely a ganglioneuroblastoma or neuroblastoma, and that it was too large to be observed under a COG protocol.
Laparoscopic Tumor Excision Challenges Discussed
The surgical team discussed the challenges of performing a laparoscopic excision of a tumor, noting the difficulty in retracting the dense mass and the need to convert to a small thoracotomy. The team successfully identified and clipped three arteries feeding the tumor, and removed as much of the mass as possible, with the goal of achieving a gross negative margin without excessive morbidity. The pathology report confirmed the tumor's large size and dense nature, with the team not attempting to remove all extensions into the neural foramina to avoid complications.
Ganglioneuroblastoma: Favorable Histology Discussion
The histology of the tumor was discussed, noting its homogeneous appearance with focal calcification and fleshy areas. The team identified mature and immature tumor types, with the latter appearing more myxoid and containing smaller cells and neurofilaments. This led to the conclusion that the tumor was a ganglioneuroblastoma, a favorable histology at any age.
Ganglio Neuroblastoma Surgical Approach Discussion
This was a case of a ganglio neuroblastoma with favorable histology, where the surgical margins were focally positive but the neuroblastic components were minimal. The oncology follow-up plan for the patient involved regular follow-up without adjuvant chemotherapy or radiation. The surgical approach involved a semi-prone position for the patient, which allowed for better access to the paraspinal space. The team also discussed potential complications.