Malignant chest wall neoplasms of bone and cartilage - BIR Publications

The British Journal of Radiology, 80 (2007), 678–684

PICTORIAL REVIEW

Malignant chest wall neoplasms of bone and cartilage: a pictorial review of CT and MR findings 1

P O’SULLIVAN, FFR, RCSI, 1H O’DWYER, N L MULLER, MD, PhD, FRCR(C)

FFR, RCSI,

2

J FLINT,

MD,

1

P L MUNK,

MD

and

1

Departments of 1Radiology and 2Anatomical Pathology, University of British Columbia, Vancouver General Hospital, Vancouver, BC, Canada

ABSTRACT. Malignant tumours of the chest wall are uncommon. The purpose of this pictorial essay is to describe the CT and MRI findings of malignant neoplasms affecting the bony skeleton of the chest wall and the costal cartilages. The most common primary malignant neoplasms involving the bony skeleton of the chest wall are chondrosarcoma, osteosarcoma and Ewing’s sarcoma/primitive neuroectodermal tumour. Metastases, multiple myeloma and invasive primary lung cancer are the most frequent secondary lesions. We performed a retrospective review of the radiology and pathology archive at our institution from 1 July 2000 to 31 December 2004 and identified 31 of these lesions. Several of these tumours have distinctive radiological features, allowing a confident radiological diagnosis to be suggested.

Primary tumours involving the bony skeleton of the chest wall are uncommon. The most common primary malignant tumour of the chest wall is chondrosarcoma [1]. Less common primary bony chest wall tumours include osteosarcoma and Ewing’s sarcoma/primitive neuroectodermal tumour (PNET). Multiple myeloma is a malignant tumour of plasmacytes most commonly seen in the bone marrow. It frequently involves the chest wall resulting in lytic lesions and bony expansion. The chest wall may also be involved by direct extension of pulmonary carcinoma and by metastases from extrathoracic malignancy. Imaging plays an important role in the diagnosis and management of neoplasms involving the bony skeleton of the chest wall. The radiological appearance of these tumours in the chest wall has not been extensively described. Our aim was to review the imaging features of neoplasms affecting the bony skeleton of the chest wall and the costal cartilages. During the review of our radiology and pathology archive we encountered 31 malignant tumours affecting the bone or cartilage of the chest wall. This included 10 (32%) metastatic chest wall lesions, 7 (22.5%) invasive lung primary tumours, 5 (16%) chondrosarcomas, 4 (13%) multiple myeloma chest wall tumours, 3 (9%) osteosarcomas and 2 (6.4%) Ewing’s sarcomas of the chest wall.

Received 4 November 2005 Revised 11 January 2006 Accepted 19 January 2006 DOI: 10.1259/bjr/82228585 ’ 2007 The British Institute of Radiology

occur more commonly in men and affect patients greater than 50 years of age. Chondrosarcomas are most commonly found in the anterior chest wall, in the superior five ribs and adjacent to the costochondral junction [4], or in the paravertebral regions. The majority of chondrosarcomas are seen to originate within bone; occasionally they may originate in the soft tissue [5]. The characteristic CT appearance of chondrosarcomas consists of a well-defined, lobulated mass of soft tissue attenuation with foci of dense chondroid matrix calcification (Figure 1). ‘‘Ring-like’’ or ‘‘arcs’’ of calcification can also be seen (Figure 2) [4]. The pattern of calcification has been described as nodular (Figure 3a) or peripheral in nature [6]. Bone destruction and invasion of the overlying soft tissue to varying degrees is also seen

Chondrosarcoma Chondrosarcomas may occur de novo or arise from bony exostosis or following radiotherapy [2, 3]. They Figure 1. 28-year-old female with a chondrosarcoma. CT Address correspondence to: P O’Sullivan, Department of Radiology, Vancouver General Hospital, 899 West 12th Avenue, Vancouver V5Z 1M9, Canada. E-mail: [email protected]

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image photographed on bone windows demonstrates dense amorphous matrix calcification within the tumour arising from the inferior angle of the scapula.

The British Journal of Radiology, August 2007

Pictorial review: Malignant chest wall neoplasms of bone and cartilage

Osteosarcoma

Figure 2. 56-year-old female with a chondrosarcoma. Axial CT image demonstrates large tumour arising from the right 6th rib, with ‘‘arcs’’ of central calcification.

(Figure 4). Chondrosarcomas may present without any visible matrix calcification. Instead, a predominantly myxoid matrix of uniform low soft tissue attenuation is seen. The MRI features include signal intensity similar to muscle on T1 weighted imaging and signal intensity greater than adipose tissue on T2 weighted images (Figure 3b). The chondroid matrix calcification produces a signal void on MRI (Figure 5). Contrast enhancement is often patchy, and a high signal periphery may be seen.

(a)

Osteosarcomas are high-grade malignant bone tumours, more commonly seen in children and adolescents [7]. The most frequent site of origin is the distal femur. Chest wall osteosarcomas are most commonly found in the ribs, scapula and clavicle [4]. Two age peaks are seen for chest wall osteosarcomas: the osseous form is seen in younger adult patients, while the less common extraosseous type is seen in patients over 50 years. Osteosarcomas contain neoplastic new bone or disorganized ossification. The typical CT presentation of osteosarcomas consists of matrix mineralization greatest at the centre of the lesion and decreasing towards the periphery. Some authors have suggested that it is possible to differentiate osteosarcoma from chondrosarcoma based these findings [8]. MRI usually reveals intensity higher than muscle on T1, and mixed to higher signal than muscle on T2, weighted images [4]. Signal void is also seen when dense matrix mineralization is present (Figure 6).

Ewing’s sarcoma and primitive neuroectodermal tumour Ewing’s sarcoma is a rare aggressive malignant tumour of bone. It occurs most commonly in the diaphysial and metadiaphysial regions of the lower extremities [9]. Metastases to lungs, bone or marrow are present in 25% of patients at the time of presentation [10]. PNET, previously classed as a separate tumour, is now thought to be an aggressive form of Ewing’s sarcoma [4]. Ewing’s and PNET are the most common malignant tumour of the chest wall in children and both

(b)

Figure 3. 62-year-old male with a chondrosarcoma. (a) CT shows the tumour with nodular calcification, arising from the posterior aspect of the left 5th costo-chondral junction. (b) Coronal T2 MRI shows high signal multilobulated anterior chest wall tumour (arrow), with areas of signal void due to calcification. The British Journal of Radiology, August 2007

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P O’Sullivan, H O’Dwyer, J Flint et al

Figure 4. 72-year-old male with chondrosarcoma. Axial CT shows a large mass arising from the anterior left 4th rib, with a marked periosteal reaction.

are highly aggressive in nature [8, 11]. They can also be seen in young adults. Primary chest wall Ewing’s sarcoma and PNET tumours usually develop as solitary lesions centred on the ribs (Figure 7), sternum, scapula, clavicle or paravertebral regions [4]. They tend to be large in size and have an inhomogeneous appearance due to rapid growth. CT findings include a large, inhomogeneous mass with poorly defined margins, displacing surrounding tissues (Figure 8a) and mixed internal attenuation, similar to muscle tissue with necrosis and/or calcification. On MRI, these tumours usually have a pattern of heterogeneous high signal on T1 and T2 weighted images, due to extensive haemorrhage and necrosis (Figure 8b) [11].

Multiple myeloma Multiple myeloma (MM) is a tumour of the bone marrow, composed of malignant plasmacytes. These cells grow in the bone marrow, and cause expansion and remodelling of bones within the chest wall. Extramedullary disease (outside the marrow) is uncommon. In one review of 432 patients with multiple myeloma, only 19 (4.4%) were identified as having extramedullary multiple myeloma [12]. MM often produces gross sternal expansion and distortion (Figure 9) and vertebral body destruction (Figures 10 and 11). The pathological tissue appears as soft tissue attenuation similar to muscle at CT, with a degree of enhancement post contrast. MRI typically reveals masses of uniform low signal on T1 weighted imaging, and uniform high signal on T2 weighted imaging. Later in the disease cortical breaches occur, and further spread from bone is seen, producing masses in the surrounding soft tissues. CT is superior to MRI in depicting early cortical breaches.

Lung carcinoma Aggressive primary lung carcinoma may extend directly into the bony chest wall. The accurate identification of locally advanced lung cancer spreading through to the chest 680

Figure 5. 70-year-old male with a chondrosaroma. (a) Axial T2 MRI shows paraspinal tumour with extensive central signal loss due to matrix calcification, and a high signal tumour margin.

wall is often difficult. Both CT and MRI have demonstrated a varying degree of success [13]. MRI appears the superior modality in relation to superior sulcus (Pancoast) tumours. However, the new 16 and 64 track multislice CT scanners with multiplanar reconstructions are demonstrating an increasing sensitivity in this area [14]. Chest wall invasion is clinically important (Figure 12), as when involved the lesion will be classed as a T3 lesion, which may not be considered for surgery. CT or MRI demonstration of bony destruction is the most reliable method to identify chest wall invasion by lung cancer. This finding, however, has a relatively low sensitivity. Other findings such as apparent soft tissue extension have a low specificity.

Metastatic disease Metastatic disease to the chest wall is not unusual. It is often seen at the terminal stages of malignant disease, or earlier in presentation with particularly aggressive disease. It is associated with a poorer outcome. Various patterns of metastatic disease to the bony chest wall can be seen. The imaging characteristics of the primary lesion may be observed within the bony metastatic deposit to the chest wall. When occurring in bone these characteristics are best evaluated on CT, such as the lytic pattern of myeloma, the sclerotic pattern of prostate or breast (Figure 13) or the expansile nature of renal cell carcinoma. Soft tissue tumours (e.g. lymphoma) when they spread to the bony chest wall (Figure14) also produce tissue of similar appearance and attenuation to the primary tumour. At MRI these lesions are usually low signal on T1 weighted imaging, and high signal on T2 weighted imaging. The British Journal of Radiology, August 2007


(a)

(b)

(c) Figure 6. 65-year-old female with an osteosarcoma. (a) Chest radiograph shows a large densely calcified mass overlying the left upper chest wall. (b) Axial CT shows a large densely calcified left chest wall mass, situated on the ribs and displacing overlying pectoralis muscles. (c) Coronal T1 MRI shows large chest wall mass with extensive central signal loss (arrow) due to tumour calcification.


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Figure 7. 30-year-old female with Ewing’s sarcoma. Axial CT demonstrates a large soft tissue attenuation mass arising from the left 4th rib, which is irregular and sclerotic.

(a)

Figure 9. 52-year-old female with multiple myeloma. Axial CT shows a large enhancing soft tissue tumour expanding the sternum, and extending into the anterior and posterior soft tissues. Note compression of the brachiocephalic vein and superior vena cava.

(b)

Figure 8. 32-year-old male with Ewing’s sarcoma. (a) Axial CT shows large soft tissue tumour (arrow) with well-defined margins. The ribs exhibit a marked periosteal reaction. (b) Axial T2 MRI shows large high signal chest wall mass, with intra-abdominal extension and liver compression.

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Figure 10. 88-year-old male with multiple myeloma. CT

Figure 12. 71-year-old male with right apical adenocarci-

shows diffuse infiltration of vertebrae and extensive bilateral extramedullary soft tissue extension.

noma (Pancoast’s tumour). CT shows superior sulcus tumour extending into the ribs and right axilla.

Recurrent disease

Conclusions

Local recurrence of malignant disease is not uncommon in the chest wall. Breast carcinoma has reported rates of ‘‘local recurrence’’ in the chest wall ranging from 5% to 20% [15, 16]. Sarcomas, such as chondrosarcoma and fibrosarcoma, are seen in the chest wall post radiation treatment for lung and breast malignancies.

Several chest wall tumours have characteristic features that suggest a particular diagnosis. Matrix mineralization is seen in both chondrosarcomas and osteosarcomas. Other lesions have characteristic locations. MM produces lytic, expansile lesions in the sternum and vertebrae. Patient’s age is an important factor. Ewing’s tumour is seen in the paediatric population. In elderly patients a calcified primary tumour is likely to be a chondrosarcoma. In adults the most common chest wall tumour without calcification is a plasmacytoma or metastatic deposit from a distant primary.

Figure 11. 56-year-old male with multiple myeloma. CT

Figure 13. 52-year-old female with previous left mastect-

image shows marked sternal expansion and deformity, and vertebral destruction with paravertebral soft tissue masses from myeloma.

omy for breast carcinoma (note prosthesis). A large right side chest wall recurrence is seen originating from the sclerotic expanded right 6th rib.


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Figure 14. 66-year-old male with prior history of lymphoma and bony chest wall recurrence. Extensive soft tissue attenuation tumour arises from the destroyed right 9th rib, and spreads into adjacent soft tissues.

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