Spinal cord compression due to Kaposi's sarcoma

Kaposi's sarcoma (KS) is a mesenchymal tumor involving blood and lymphatic vessels and is associated with human herpes virus 8 (HHV8). It is the most common neoplasm in HIV patients [1Go]. Since the introduction of highly active anti-retroviral therapy (HAART) the incidence of KS has declined significantly, and the use of HAART has prolonged survival among patients diagnosed with KS [2Go]. We would like to present two patients with spinal cord compression, a very rare complication of KS.

Case 1

In 2002, a 33-year-old male presented with acute paraplegia. The patient was known to be HIV-1 seropositive since 1996, and he was diagnosed with KS of the gastro-intestinal tract and skin in 1999. Since 1999, several chemotherapeutic regimens had resulted in partial remission; during this time he had also been treated with HAART. His compliance over the years had often been poor, due to financial and insurance problems, but during the last 6 months prior to admission, his CD4 counts had been stable at around 100x106 cells/l. In that same period the HIV-RNA viral load had decreased from 230.599 to <50 copies/ml. The HHV8 load, determined using an in-house assay, showed a remarkable increase at the time of clinical deterioration (4.4x106 compared with 1.7x103 copies/ml 6 months previously). Physical examination was consistent with spinal cord compression at level L2, and imaging studies showed separate destructive lesions at the level of Th 10 and L2 (Figure 1A and BGo). The biopsy was compatible with KS. Emergency radiotherapy could not prevent progressive paralysis, and the patient deteriorated further, until he eventually died at the ICU of respiratory failure due to pulmonary hemorrhage. The autopsy showed massive dissemination of KS to almost all internal organs, including the lungs, spinal cord (Figure 2Go) and brain. Extensive literature search revealed just one similar case of spinal cord compression due to KS which we present here with follow-up [3].



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Figure 1 (A) Case 1: transversal T2 weighted image (MRI) showing an epidural mass with cord compression (arrow) of the myelum and with extension in the thoracic transverse process and thoracic paraspinal muscles. (B) Case 1: sagittal T2 weighted image reveals increased intensity in vertebral body at Th10 and L2 (arrows) with epidural extension and compression on the conus.

 


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Figure 2 Case 1: transversal sections through the vertebral bodies at levels from Th10 to L2 at autopsy show extensive infiltration with Kaposi's sarcoma. The yellow areas contain massive necrosis.

 
Case 2

A 55-year-old male was admitted to hospital in 1987 due to rapidly progressive lower extremity weakness and sphincter incontinence. After chemotherapy, he was in partial remission for KS of the skin and GI tract, which was diagnosed some 18 months earlier. Ioxol myelography followed by computed tomography demonstrated the presence of complete blockage of the contrast flow due to a mass at the Th5 level. Decompressive laminectomy with a biopsy of the mass confirmed presence of KS involving the epidural space and vertebral body. A low dilution Elisa HIV test was found to be positive, but higher dilution was negative. The patient was assumed to be immunocompromised since an IgG lamba monoclonal gammopathy had been diagnosed. After he refused further therapy, pneumonia eventually led to his death. A general autopsy was performed, but spinal cord and brain examination were not included, since the risk of contracting HIV through tissue was thought to be major at that time. Unfortunately, no blood or tissue of this patient is presently available for retrospective HHV8 or HIV testing.

We describe two patients with a remarkably similar course of disease in which spinal cord compression was caused by KS. In general, markers of immunodeficiency best predict survival in KS, yet the first patient progressed despite adequate HAART therapy. KS regression mostly occurs when HIV RNA levels are low, and this is usually associated with normalized CD4 counts and reduced HHV8 titer [4Go, 5Go]. In our patient, conversely, low HIV RNA did not lead to corresponding CD4 and HHV8 levels, and the patient's KS progressed. Therefore HHV8 levels may be more relevant to KS disease status than HIV levels.

In conclusion, these two cases prove that KS can lead to spinal cord compression and that this rare manifestation of KS can occur even in the presence of low HIV RNA levels after HAART.

G. van Twillert1,*, S. van Eeden2, F. J. B. Nellen1, M. Cornelissen1, Z. Wszolek3 and A. M. Westermann1

1 Department of Internal Medicine and Human Retrovirology; 2 Department of Pathology, Academic Medical Center, University of Amsterdam, The Netherlands; 3 Mayo Clinic, Jacksonville, FL, USA

*Correspondence to: Dr G. van Twillert, Departments of Internal Medicine and Human Retrovirology, Academic Medical Center, University of Amsterdam, The Netherlands. Email: govantwillert{at}amc.uva.nl

References

1. Beral V, Peterman TA, Berkelman RL et al. KS among persons with AIDS: a sexually transmitted infection? Lancet 1990; 335: 123–128.[ISI][Medline]

2. Tam HK, Zhang ZF, Jacobson LP et al. Effect of HAART on survival among HIV-infected men with Kaposi sarcoma or non-Hodgkin lymphoma. Int J Cancer 2002; 98: 916–922.[CrossRef][ISI][Medline]

3. Wszolek ZK, Bashir RM, Lorenzo AS. Kaposi's sarcoma as a cause of spinal cord compression in an immunocompromised patient. Southern Medical Journal 1990; 83: 723–724.

4. Gill J, Bourboulia D, Wilkinson J et al. Prospective study of the effects of antiretroviral therapy on Kaposi sarcoma associated with herpesvirus infection in patients with and without Kaposi sarcoma. J Acquir Immune Defic Syndr 2002; 31: 384–390.[Medline]

5. Cattelan AM, Calabro ML, Aversa SM et al. Regression of AIDS-related Kaposi sarcoma following antiretroviral therapy with protease inhibitors: biological correlates of clinical outcomes. Eur J Cancer 1999; 35: 1809–1815.[CrossRef][ISI][Medline]





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