The patient presented with bilateral leg swellings with pain and paresthesia. Venogram demonstrated an occlusion of the right common iliac vein at the IVC. The paresthesia progressed and CT of the pelvis, MR of the lumbrosacral spine and MR angiography and venography were performed.
The patient, 2 days post lower segment caesarean section, presented with bilateral leg swellings, worse on the right, with pain and paresthesia affecting mainly the left leg. Venogram demonstrated an occlusion of the right common iliac vein at the IVC with extensive venous collaterals (Fig. 1). Coumarin therapy was commenced.
Three weeks later her paresthesia had progressed to the gluteal and genital regions with development of urinary and faecal incontinence. CT of the pelvis revealed an enhancing lobulated sacral tumour with fluid levels compressing the sacral spinal canal and extending into the right greater sciatic notch, with an enlarged IVC containing peripherally enhancing thrombus (Fig. 2). MRI confirmed the sacral mass containing fluid levels and loss of the sacral canal (Fig. 3). MR angiography and venography confirmed the slightly enhancing tumour thrombus within the IVC (Fig. 4).
Review of the original venogram showed right sacral bone destruction (Fig. 1). Subsequent CT of the thorax demonstrated multiple pulmonary metastases (Fig. 5).
Sacral surgical biopsy showed multinucleate giant cells with no aggressive features, confirming the diagnosis of giant cell tumour (osteoclastoma). In the presence of IVC "thrombus" with the "benign" metastases, a Gunter Tulip IVC filter was placed in the infra renal IVC with incidental demonstration of upper moiety hydronephrosis within the left duplex pelvicalyceal system (Fig. 6).
Subsequently, as the sacral giant cell tumour was deemed to be unresectable, the patient received external beam radiotherapy to the sacrum. Unfortunately her disease progressed quite rapidly despite radiotherapy with an increase in both the sacral and pulmonary disease, with her demise 8 months from presentation.
Giant cell tumour of the sacrum is rare and can increase in size during pregnancy with pelvic pain and limb numbness. A subgroup of primary sacral giant cell tumours can behave very aggressively with pulmonary deposits at the time of presentation. In other cases pulmonary metastases develop at a much later time despite initially successful curettage, sacrectomy, or radiotherapy.
Occasionally sacral giant cell tumour undergoes spontaneous dedifferentiation into osteosarcoma without prior radiotherapy; this may be the course this case followed, notwithstanding the benign surgical biopsy. A recent paper suggests that the multinucleate giant cells are recruited and have their osteoclastic activity promoted by the associated transformed fibroblastic stromal cells.
Although a previous paper describes an incidental deep vein thrombosis with sacral giant cell tumour, we believe that this is the first described case of direct giant cell tumoral thrombus within the IVC, which succinctly explains the presence of pulmonary deposits. A similar picture is well known with renal cell carcinoma.
Aggressive sacral giant cell tumour
Based on the provided X-ray, CT, MRI, and MR angiography/venography images, the following findings are observed:
Considering the patient's history and the above imaging findings, the following diagnoses are to be considered:
Based on the imaging and the patient’s characteristics, the first diagnosis (giant cell tumor of the sacrum) is most indicative, especially with intravascular extension into the IVC forming a tumor thrombus and lung metastases.
Considering the patient’s age (29-year-old female), osteolytic expansile destruction of the sacrum, tumor extension into blood vessels, and lung metastases, the most likely diagnosis is:
Aggressive Giant Cell Tumor of the Sacrum, accompanied by tumor thrombus formation and lung metastases.
Further confirmation should be obtained through comprehensive surgical pathological biopsy results and molecular pathology testing if necessary.
Treatment should be based on the size, location, and invasiveness of the tumor, generally including:
Rehabilitation Training and Exercise Prescription: Given involvement of the sacrum and surrounding soft tissues, as well as possible pulmonary metastases, the rehabilitation plan should adhere to the following principles:
Throughout rehabilitation, be vigilant for potential risks such as covert bone thinning or insufficient local bone strength leading to fractures, as well as lower extremity edema or deep vein thrombosis. Under specialist and rehabilitation therapist guidance, regular follow-up assessments should be conducted, adjusting the training plan as necessary.
Disclaimer: The above report is based on available data for reference only and does not replace in-person consultation or professional medical advice. Specific treatment and rehabilitation plans should be developed based on clinical examinations, pathological confirmations, and individualized patient factors.
Aggressive sacral giant cell tumour
