Abstract
Superior mediastinal syndrome (SMS) is a relatively common emergency in the practice of Pediatric Oncology. It typically results from the compression of large airways and superior vena cava by a swiftly growing mass. T-lineage acute lymphoblastic leukemia or lymphoma, neuroblastoma, and germ cell tumor are the common etiologies of SMS in children. Occasionally, SMS can be an unexpected presentation of less common childhood cancers and a surprise for the diagnostic and treating teams. The present paper reports the diagnostic and therapeutic challenge of managing a 9-y-old boy with SMS resulting from mediastinal myeloid sarcoma. The presence of a sizeable intracardiac thrombus, in addition, contributed to the SMS. The initial pleural fluid cytology and image-guided fine-needle aspiration cytology of the mediastinal mass were nondiagnostic. A thoracotomy was subsequently performed to debulk the tumor for symptomatic relief and obtain tissue for diagnosis.
References
Zardi EM, Pipita ME, Afeltra A. Mediastinal syndrome: A report of three cases. Exp Ther Med. 2016;12:2237–40.
Tabacu E, Nemes R, Mitrea M, Crisan E, Galie N. Superior vena cava syndrome in a primary tuberculosis of mediastinal lymph nodes. Chest. 2012;142:251A.
Jain R, Bansal D, Marwaha RK, Singhi S. Superior mediastinal syndrome: emergency management. Indian J Pediatr. 2013;80:55–9.
Avni B, Koren-Michowitz M. Myeloid sarcoma: current approach and therapeutic options. Ther Adv Hematol. 2011;2:309–16.
Nair M, Kusumakumary P, Nair PS. Acute myeloid leukemia presenting as mediastinal mass. Indian J Pediatr. 2014;81:824–5.
Seth R, Bolia R, Jain R, Chopra A, Singh S, Kumar R. Superior mediastinal syndrome: a rare presenting feature of acute myeloid leukemia. Indian J Pediatr. 2013;80:165–7.
Author information
Authors and Affiliations
Contributions
PSR managed the patient, performed a literature review, and drafted the manuscript; VV was involved in bedside clinical management and diagnostic procedures; RJ contributed to the clinical management of the patient; KKS contributed to the clinical management and coordinated thoracotomy; SM was the cardiothoracic surgeon who performed thoracotomy; DC reported the histopathology; AKS reported the radiology; MR performed the cytological analysis of the pleural fluid, including flow cytometry; DB was the consultant in charge of the clinical management of the case and supervised, edited, and finalized the manuscript. DB will act as the guarantor for this paper.
Corresponding author
Ethics declarations
Informed Consent
The authors confirm that they have obtained all appropriate patient consent forms. The patients understand that their names and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Conflict of Interest
None.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Roy, P.S., Vohra, V., Jain, R. et al. Isolated Myeloid Sarcoma and Intracardiac Thrombus Resulting in Superior Mediastinal Syndrome. Indian J Pediatr 89, 591–593 (2022). https://doi.org/10.1007/s12098-021-04056-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12098-021-04056-1