Operative treatment of pulmonary primitive neuroectodermal tumor: a case report and literature review

Yiyuan Zhang; Ke Shang; Jialin Li; Mengyao Sun; Xiaoying Gu

doi:10.1186/s13019-024-02563-8

Operative treatment of pulmonary primitive neuroectodermal tumor: a case report and literature review

J Cardiothorac Surg. 2024 Mar 5;19(1):109. doi: 10.1186/s13019-024-02563-8.

Authors

Yiyuan Zhang¹, Ke Shang², Jialin Li¹, Mengyao Sun³, Xiaoying Gu⁴

Affiliations

¹ Department of Thoracic surgery, The First Hospital of Jilin University, Changchun, 130021, China.
² Department of Anesthesiology, The First Hospital of Jilin University, Changchun, 130021, China.
³ Department of Cardial Surgery, The First Hospital of Jilin University, Changchun, 130021, China.
⁴ Department of Anesthesiology, The First Hospital of Jilin University, Changchun, 130021, China. xiaoyingg@jlu.edu.cn.

Abstract

Background: Pulmonary primitive neuroectodermal tumor (PNET), a member of the Ewing sarcoma family of tumors, is a rare malignancy that is associated with a grim prognosis. To date, fewer than 30 cases of pulmonary PNET have been reported. In this case report, we present the clinical details of a 12-year-old girl with pulmonary PNET who underwent surgical treatment. We also conducted an analysis and summary of other relevant studies and the surgical outcomes.

Case presentation: In May 2018, a 12-year-old girl was admitted with symptoms of cough and blood-tinged phlegm. A computed tomography scan revealed a large mass, measuring 12.9 cm × 8.1 cm, in the right middle and lower lungs. A percutaneous lung biopsy confirmed poorly differentiated tumor cells with a nested growth pattern. Immunohistochemical staining demonstrated positive expression of CD99, CD56, Vimentin, and Synaptophysin. The patient was diagnosed with pulmonary PNET. Following three cycles of neoadjuvant chemotherapy, a substantial reduction in tumor volume was observed. Subsequently, the patient underwent a surgical procedure involving pneumonectomy and partial resection of the left atrium with the assistance of cardiopulmonary bypass. The patient was discharged 37 days after surgery. During a three-year follow-up period, she exhibited no signs of tumor recurrence and has successfully returned to school.

Conclusions: This case highlights the successful management of an advanced PNET with neoadjuvant chemotherapy, pneumonectomy, and partial resection of the left atrium employing cardiopulmonary bypass. The patient remained disease-free after three years. Our analysis of surgically treated cases indicates that neoadjuvant chemotherapy can contribute to improved prognoses for PNET patients. It is crucial to emphasize that complete surgical excision remains the cornerstone of treatment, underscoring the importance of surgeons considering radical surgical approaches whenever feasible for patients with pulmonary PNETs.

Keywords: Clinical outcomes; Prognosis; Pulmonary primitive neuroectodermal tumors; Surgery with cardiopulmonary bypass.

Publication types

Review
Case Reports

MeSH terms

Child
Female
Humans
Lung
Neoadjuvant Therapy
Neoplasm Recurrence, Local*
Neuroectodermal Tumors, Primitive* / diagnosis
Neuroectodermal Tumors, Primitive* / surgery
Pneumonectomy