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Primitive neuroectodermal tumor/Ewing's sarcoma with long-term follow-up.

Mitsuhiro Fukushima¹, Kiyoshi Koizumi, Yuki Nakajima

¹Department of Surgery II, Nippon Medical School, Tokyo, Japan.

The Japanese Journal of Thoracic and Cardiovascular Surgery : Official Publication of the Japanese Association for Thoracic Surgery = Nihon Kyobu Geka Gakkai Zasshi

|March 24, 2005

Summary

A rare primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) was found in a 32-year-old woman's chest. This PNET/EWS case highlights successful long-term survival after surgical resection and radiotherapy.

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Area of Science:

Oncology
Pathology
Radiology

Background:

Primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) is a rare and aggressive cancer.
Diagnosis often occurs in children and young adults, but adult cases are documented.

Observation:

A 32-year-old female presented with an asymptomatic, well-defined, rounded mass in the left lower lung.
Imaging revealed a distinct mass adjacent to the anterolateral thoracic wall, with a stalk emanating from it.
Histological examination confirmed PNET/EWS, characterized by atypical small round cells positive for MIC2 gene product.

Findings:

The patient underwent surgical resection and adjuvant radiotherapy (50 Gy).
No adjuvant chemotherapy was administered.
A small recurrent nodule in the left lung was resected four years later.

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Implications:

This case demonstrates a 7-year survival with no recurrence or metastasis for over 3 years post-second surgery.
It underscores the importance of early detection and multimodal treatment for PNET/EWS in adults.
The findings contribute to understanding the clinical behavior and management of thoracic PNET/EWS.