Rare Primary Pulmonary Primitive Neuroectodermal Tumor: A Case Report and Literature Review

Wang, Ning; Dong, Shuangshuang; Wu, Chunlin; Wang, Ying; Meng, Lian; Ren, Yan; Cui, Xiaobin; Li, Manyu; Yan, Qi

doi:10.2147/ott.s283967

Cited by 3 publications

(4 citation statements)

References 28 publications

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“…Only 29 cases of pulmonary PNET have been documented to date, comprising 17 male and 12 female patients. Remarkably, approximately 82.8% of these 29 patients received a diagnosis of PNET before the age of 40 [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…The primary cause of death was attributed to tumor recurrence. Individuals with smaller tumors appeared to have the potential for achieving long-term survival [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Peripheral PNET commonly occurs in the chest wall, pelvis, paraspinal, retroperitoneum, limbs, abdomen and neck in children and adolescents [ 2 ]. Pulmonary PNET is rare, and fewer than 30 pulmonary PNET cases have been reported thus far [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

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

Zhang,

Shang,

et al. 2024

J Cardiothorac Surg

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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.

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Section: Discussionmentioning

confidence: 99%

“…The primary cause of death was attributed to tumor recurrence. Individuals with smaller tumors appeared to have the potential for achieving long-term survival [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

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

Zhang,

Shang,

et al. 2024

J Cardiothorac Surg

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“… 3 Tumor metastasis at diagnosis, large tumor size, and invasion of the heart are indicative of poor prognosis. 5 …”

mentioning

confidence: 99%

Primary Pulmonary Ewing's Sarcoma: A Surprise Diagnosis in a 52-Year-Old Active Smoker

Jamel,

Chiraz,

Marwa

2024

Open Respiratory Archives

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A rare radiotherapy-sensitive primitive neuroectodermal tumor with APC gene amplification in an adult: a case report and literature review

Song

Yang

et al. 2022

J Int Med Res

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Primitive neuroectodermal tumor (PNET) of the lung is rare in adults, and treatment options vary. We herein describe the disease course and follow-up of PNET in an adult. A 27-year-old man was admitted to our hospital because of cough and headache, and whole-exome sequencing revealed positive expression of the EWSR1-FLI1 fusion gene and amplification of the APC gene. Although the patient received multidisciplinary treatment including chemotherapy regimens of etoposide plus cisplatin; focal radiotherapy focusing on the cerebrum, lung, and kidneys; and a subsequent palliative gastrointestinal operation, he eventually died of multiple organ functional failure. His overall survival period was 18 months, and his progression-free survival period was 4 months. During the treatment, the patient showed remarkable sensitivity to radiotherapy. In conclusion, PNET of the lung in adult patients is extremely rare, and the prognosis is very poor. Involvement of a multidisciplinary team in the development of personalized therapeutic strategies is essential. This patient with APC gene amplification showed excellent sensitivity to radiotherapy for intrapulmonary and intracranial lesions, suggesting that APC gene amplification may be related to radiotherapy sensitivity. However, further clinical research is needed.

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Rare Primary Pulmonary Primitive Neuroectodermal Tumor: A Case Report and Literature Review

Cited by 3 publications

References 28 publications

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

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

Primary Pulmonary Ewing's Sarcoma: A Surprise Diagnosis in a 52-Year-Old Active Smoker

A rare radiotherapy-sensitive primitive neuroectodermal tumor with APC gene amplification in an adult: a case report and literature review

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