Chest wall chondrosarcoma with extradural extension

Clinical Cases 24.08.2022
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 32 years, female
Authors: Prof. Dr. S. Babupeter, Dr. Sree Vandana G., Prof. Dr. R. Ravi
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Details
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AI Report

Clinical History

A 32-year-old woman presented to our hospital with three months history of bilateral lower extremity weakness and inability to walk. She was referred for Magnetic Resonance Imaging of the thoracic spine to our department.

Imaging Findings

MRI of the thoracic spine was performed in a 3 Tesla MRI (Siemens Healthineers, Skyra, Erlangen, Germany) as per our Institute protocol. MRI revealed a large, lobulated and predominantly T2 hyperintense lesion along the posterior aspect of the left 5th rib. There was extradural extension into the posterior aspect of the adjacent spinal canal, causing compression of the spinal cord (Figures 1 and 2).T1 and STIR images revealed alterations of the bone marrow signal in the 5th thoracic vertebra (Figures 3 and 4).To further characterize the lesion, contrast-enhanced computed tomography imaging of the chest was performed, which showed an aggressive type of periosteal reaction at the left 5th rib and a large, heterogeneous and enhancing soft tissue component (Figures 5 and 6).CT also revealed multiple foci of stippled calcifications within the soft tissue component and adjacent spinal canal (Figure 7).

PET CT revealed high metabolic activity within the lesion and adjacent 5th thoracic vertebra (Figure 8).

Discussion

Background

Chondrosarcoma is the most common primary bone malignancy of the chest wall, accounting for 30% of all primary bone malignancies and 33% of all primary rib tumours. About 10% of chondrosarcoma occurs in the chest wall, most of which are identified adjacent to the costochondral junction[1]. They are usually found between the ages of 40 and 70 and are more common in men.

Clinical Perspective

Pain is the most common symptom and is reported by 95% of patients. Pain is often insidious and progressive, with an average time to manifest being 1 to 2 years. A palpable soft tissue mass is present in 30% to 80% of patients at the time of presentation. Pathological fractures are the main symptom in 3% to 17% of patients[3]. Our case is unique in its clinical presentation of bilateral lower limb weakness being caused by chondrosarcoma of the chest wall due to concomitant vertebral infiltration and associated posterior epidural soft tissue component causing spinal cord compression. An evaluation of the entire extent of the mass by multimodality imaging and histopathological correlation is crucial for proper patient management.

Imaging  Perspective

The typical appearance of a chondrosarcoma on multidetector CT images is a well-defined mass with a combination of soft tissue and chondroid matrix, which includes rings, arcs and stippled patterns of calcification. Invasion and destruction of adjacent bony structures are common. At MR imaging, the cartilage background of chondrosarcoma accounts for the iso to hypointense signal on T1-weighted images and hyperintense signal on T2-weighted images[1]. Following the intravenous administration of contrast agent, the lesion showed heterogenous enhancement, predominantly at the periphery of the tumour [2]. In our case, CT demonstrated aggressive periosteal reaction involving the left 5th rib associated with a lobulated soft tissue component which showed enhancing septations and specks of calcifications within. The chondroid matrix of the lesion accounts for the high signal intensity on T2 weighted images. There was also extradural extension of the lesion with infiltration of the 5th thoracic vertebra. PET CT revealed high metabolic activity reflecting the malignant nature of the lesion.

Histopathology

CT-guided biopsy of the lesion was done, and histopathological examination revealed features of Grade 2 Chondrosarcoma with extensive myxoid areas.

Differential Diagnosis List

Chest wall chondrosarcoma with vertebral infiltration and extradural extension
Malignant peripheral nerve sheath tumour (MPNST)
Ewing sarcoma
Chest wall metastases

Final Diagnosis

Chest wall chondrosarcoma with vertebral infiltration and extradural extension

Figures

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Axial T2 weighted image through the chest shows a lobulated high signal intensity lesion at the left side of the posterior me

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Sagittal T2 weighted image through chest shows the extradural extension of the lesion which causes compression of the spinal

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(A and B) Sagittal T1 weighted images through chest shows altered marrow signal changes in the 5th and 6th thoracic vertebra
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(A and B) Sagittal T1 weighted images through chest shows altered marrow signal changes in the 5th and 6th thoracic vertebra

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Coronal STIR image of chest shows high signal intensity lesion in the left upper hemithorax (blue arrow) with STIR hyperinten

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(A and B) Axial non contrast CT image of the chest in mediastinal window shows aggressive periosteal reaction (blue oval) inv
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(A and B) Axial non contrast CT image of the chest in mediastinal window shows aggressive periosteal reaction (blue oval) inv

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(A and B) Axial contrast enhanced CT image of chest in soft tissue window shows enhancing septations (orange arrows) within t
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(A and B) Axial contrast enhanced CT image of chest in soft tissue window shows enhancing septations (orange arrows) within t

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(A and B) Axial non-contrast CT image of the chest in bone window shows multiple foci of calcifications within the lesion (bl
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(A and B) Axial non-contrast CT image of the chest in bone window shows multiple foci of calcifications within the lesion (bl

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(A and B) Axial PET image with CT overlay.The large lesion shows heterogenous metabolic activity, predominantly in the upper
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(A and B) Axial PET image with CT overlay.The large lesion shows heterogenous metabolic activity, predominantly in the upper