9-year-old boy with several days of painful right chest wall swelling.
Following imaging studies, the patient underwent excisional biopsy and removal of tumor with partial resection of 9th rib.
Findings:
Fig. 1 Chest radiograph shows osteolytic changes with periosteal reaction in the anterior portion of 9th right rib; in addition, there is bulging of the overlying soft tissues of the chest wall.
Fig. 2 Ultrasound of the chest wall demonstrates an oval well-circumscribed heterogeneous mass.
Fig. 3 Color Doppler demonstrates prominent vascularity of the mass.
Fig. 4 Non-contrast CT shows a hypodense mass without distinct margins in the right chest wall around anterior portion of 9th rib;
there were no signs of pleural or lung pathology.
Primitive neuroectodermal tumor (PNET) / Ewing’s sarcoma (ES) of the chest wall is rare and has a poor prognosis (1).
Identification of a common cytological abnormality, t(11;22)(q24;q12), in Ewing’s sarcoma and PNET supports point of view that those two neoplasms are histogenetically related (2).
Their treatment and response to therapy are similar. Askin’s tumor is the name given to PNET tumors of the chest wall (3).
It is the most common malignant chest wall tumor in childhood.
The peak incidence occurs in second decade (but frequently occurs below 10 years of age). There is a 2:1 male:female predominance.
Patients typically present with painful chest wall mass; the most common systemic symptoms are fever and malaise (4).
The femur is the most common site of tumor, although flat bones (ribs, scapula, pelvis) are also common sites. With regard to the thoracic cage, PNET/ES predominates in the ribs with less common or rare involvement of the scapula, clavicle and sternum.
The general radiologic appearance of PNET/ES is a mass in the chest wall accompanied by destruction of the bone. Although bone involvement is an important characteristic, the soft tissue component may be much more extensive than the bony component. CT and MRI are used to define the extraosseus extent of the tumor.
In most cases, PNET/ES is treated using chemotherapy, radiation therapy and/or surgery (4).
References / Suggested Reading:
1. Pediatric case of the day. Coombs RJ, Bayar EA, Matlub YU, Velasco ME. Radiographics 1999; 19:241-4
2. Weiss SW, Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors.
Fourth edition 2001: 1289
3. Tateishi U, Gladish GW, Kusumoto M, et al. Chest wall tumors: radiologic findings and pathologic correlation. Part 2. Malignant tumors. Radiographics 2003; 23: 1491-1508
4. Gladish GW, Sabloff BM, Munden RF, et al. Primary thoracic sarcomas. Radiographics 2002; 22:621-37
Professor of Radiology, Urology & BME
Associate Chair for Education and Research.
Department of Imaging Sciences
University of Rochester School of Medicine
Submitted By
First Name
Norman
Last Name
Loberant
Affiliation
Western Galilee Hospital, Nahariya, Israel
Thank you for such an excellent submission.
Dr. Vikram Singh Dogra
Professor of Radiology, Urology & BME
Associate Chair for Education and Research.
Department of Imaging Sciences
University of Rochester School of Medicine