CASE 12032 Published on 30.08.2014

Askin tumour in a patient with a history of lymphoma in complete remission since 2012

Section

Chest imaging

Case Type

Clinical Cases

Authors

Marta Tovar Pérez, Elizabeth M. Núñez Peynado, Lucía Hernández Sánchez, Manuel L. Rodríguez Rodríguez.

Morales Meseguer University Hospital,
Radiology Department;
Avda. Marqués de los Velez, s/n,
30008, Murcia, Spain;
Email:martatovarperez@hotmail.com
Patient

55 years, male

Categories
Area of Interest Thoracic wall ; Imaging Technique Image manipulation / Reconstruction, CT-High Resolution
Clinical History
A 55-year-old man, diagnosed with stage IA Hodgkin lymphoma in 2000, treated with chemo and radiotherapy, in complete remission since October 2012, presented in the emergency department of our hospital with a clinical history of 1 month with persistent right coastal pain.
Imaging Findings
PA and lateral chest X-ray showed an extra-pulmonary well-defined, homogeneous lesion with fluid density located in the superior and posterior region of the right hemithorax, without evidence of bone involvement (Fig. 1).
Subsequently, a contrast-enhanced chest CT was performed and showed a 72x68x10 mm (APxTxCC) solid, well-defined and heterogeneous—probably with necrotic areas—soft tissue mass in the right upper chest wall, infiltrating the extra-pulmonary fat adjacent to the 4th-5th right intercostal spaces, abutting the major pulmonary fissure (Fig. 2, 3).
Two well-defined multilobulated nodules of 9 and 10 mm, in the RUL and LUL, respectively, were also found (Fig. 4).
The radiological diagnosis was right extra-pulmonary mass probably secondary to lymphomatous recurrence or metastasis.
CT-guided biopsy was performed and the histologic findings were consistent with an extra-skeletal Ewing sarcoma (Fig. 5).
Discussion
Background

Askin's tumour (AT) is a type of extra-skeletal Ewing sarcoma of the thoracic region first described by Askin et al in 1979. It grows in the soft tissues of the chest wall or rib cage [1-7] and has, as bone Ewing sarcoma, a neuroectodermal origin. Both of them share the t (11, 22) (q24, q12) chromosomal translocation. [1, 2, 3]
Occasionally, it was described in association with Hodgkin lymphoma radiotherapy, because it has been observed that radiotherapy increases the risk of developing solid tumours. The highest risk is in the irradiated region [6, 8].
It is more common in children and young adults, although it can occur in any age range. [1, 2, 4-6, 9]

Clinical perspective

Askin tumour usually presents with an insidious clinical history of chest wall pain, dyspnoea, cough, and can also be associated with weight loss, Horner syndrome, lymphadenopathy and haemoptysis. [1, 3-6, 9]

Imaging perspective

Chest radiography is usually the first imaging test to be performed and usually shows an extra-pulmonary mass with or without rib destruction (25-63% of cases), malignant pleural effusion and signs of mediastinal and local invasion. Calcifications are rare. Mediastinal lymphadenopathy, hiliar, and pneumothorax are other associated findings. [1, 3, 5]
CT usually reveals a large mass of heterogeneous density—due to the presence of areas of cystic degeneration, necrosis or haemorrhage—, which is often associated with rib destruction and pleural effusion. It can have both intra-and extra-thoracic component with extension to the diaphragm, pleura, pericardium, vertebrae and spinal cord [1, 2, 5, 6, 9, 10].
Imaging findings are nonspecific for Askin tumour. The most important role of the CT is to confirm the presence of a mass in the chest wall and estimate its extent [3, 5, 6]. It is also useful to assess treatment response, local recurrence and distant metastases. Likewise, imaging tests have an important role to guide biopsies and to determine whether tumour resection was complete. [6].

The final diagnosis requires biopsy of the lesion and cytological and immunohistochemical study. [4-6]

Outcome and take home message

Treatment consists of neoadjuvant chemotherapy, surgery and radiotherapy. [1, 3, 7, 9, 10]
Askin's tumour is a high-grade malignancy neoplasm with a poor prognosis that should be considered in the differential diagnosis of the diseases of the chest wall. [4-7, 9, 10] Differentiation from lymphoma is crucial because it requires surgical treatment. [6]
Differential Diagnosis List
Askin tumour
Lymphoma recurrence
Rhabdomyosarcoma
Neuroblastoma
Final Diagnosis
Askin tumour
Case information
URL: https://eurorad.org/case/12032
DOI: 10.1594/EURORAD/CASE.12032
ISSN: 1563-4086