Clinical Nephrology, Vol. 71 No.

2/2008 (214-216)

Kaposis sarcoma in renal transplantation:

Report of three cases
Case
Report
2009 Dustri-Verlag Dr. K. Feistle
ISSN 0301-0430

E. Razeghi1, A. Hadadi2, P. Khashayar3 and G. Pourmand4

1Nephrology, 2Infectious

Diseases, 3Research and Development Center and

4Urology, Urology Research Center, Sina Hospital, Medical Sciences,
University of Tehran, Iran
Kaposis sarcoma in renal transplantation

Key words
transplant Kaposis
sarcoma CMV infection

Abstract. Kaposis sarcoma (KS) is one

of the most common post transplant malignancies. A variety of factors appears to contribute to the development of KS including
genetic factors, sex hormones, immunosuppression and oncogene viruses. We present 3
cases with concurrent KS and cytomegalovirus (CMV) infection in the first year after
kidney transplantation. The suspicion on KS
due to the skin lesions was confirmed by biopsy. The diagnosis of CMV infection was
made by detecting pp65 antigen in blood. The
KS lesions were limited to the skin in 2 patients, while skin and gastrointestinal tract
were involved in 1 patient. Many factors are
reported to be involved in KS development,
but the simultaneous occurrence of KS and
CMV infection in our three cases suggested
CMV as an inducing factor for KS.

Introduction
It is well established that organ transplant
recipients receiving immunosuppressants
have an increased risk for malignancy development [Boubenider et al. 1997].

Received
June 2, 2008;
accepted in revised form
June 24, 2008
Correspondence to
E. Razeghi, MD
Associate Professor of
Nephrology, Urology
Research Center, Sina
Hospital, Imam
Khomeini St. 1136746911, Tehran, Iran
Effatl62@yahoo.com

Indeed, more than one in five patients experiences malignancy within 15 years after
kidney transplantation, this rate rises to more
than two in five patients within 20 years [London et al. 1995]. Malignancies related to viruses occur more often in patients who have
undergone kidney transplantation compared
with the general population [Brunner et al.
1995, Kasiske et al. 2000, London et al.
1995].
Kaposi sarcoma (KS) is one of the most
common post transplant malignancies. An
epidemiological study has shown a 400 -500
fold increase in the incidence of Kaposi sarcoma in this group of patients compared with

the control subjects of the same ethnic origin

[Bedan et al. 1999].
Several studies have shown that in comparison to colorectal and breast cancer, KS is
more prevalent in transplant recipients. It
should be noted that this tumor may be cured
if the immunosuppressants are discontinued
or reduced in this group of patients [Penn
2001].
KS has a multi-centric origin and is characterized by vascular and fibroblastic proliferation [Bedan et al. 1999]. Skin, conjunctiva
or oropharyngolaryngeal mucosa are involved in 58% of transplant patients with KS;
as for visceral disease mainly involving the
gastrointestinal tract, lung and lymph nodes,
they are reported to be present in about 42%
of the patients [Bedan et al. 1999, Penn 2001].
The cause of post transplant KS remains
unknown. Oncogenic viruses of the herpes
type are believed to play an important etiological role [Brunner et al. 1995]. In kidney recipients, the definite associating role of other
viruses such as cytomegalovirus, the most
common source of opportunistic infection, is
not yet clear [Moosa 2005].
We present 3 cases of kidney transplant
recipients who developed KS concurrently or
shortly after cytomegalovirus infection in the
first year after transplantation.

Case 1
A 50-year-old man with end stage renal
disease secondary to autosomal dominant
polycystic kidney disease received a living
unrelated renal allograft. The CMV antibody
state prior to transplantation was positive in
both recipient (R+) and donor (D +).

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Kaposis sarcoma in renal transplantation

He was prescribed a triple immunosuppressive regimen including cyclosporine

(Neoral), mycophenolate mofetil (MMF) and
prednisolone.
Three months after transplantation, the
patient presented with fever, fatigue, weakness and diarrhea. The diagnosis of CMV was
made by detecting PP65 antigen and ganciclovir was prescribed. In the following
month, he developed diffuse reddish blue
plaques and papules on the skin, with no
lymphadenopathy. Biopsy of the skin lesions
confirmed the diagnosis of KS. Thoracic Gallium Scan was negative. Gastrointestinal endoscopy and colonoscopy showed Kaposilike mucosal lesions which were confirmed
by biopsy.
Cyclosporine and mycophenolate mofetil
were stopped but KS did not regress and so
chemotherapy was started.

Case 2
A 53-year-old man with endstage renal
disease secondary to diabetes mellitus received a living unrelated renal allograft. The
CMV state before transplantation was documented to be R+, D+.
He received an immunosuppressive regimen consisting of cyclosporine (Neoral),
MMF and prednisolone. He returned to hospital with diffuse purple skin lesions and constitutional complaints after 5 months. Biopsy
of the lesions confirmed KS and immunohistological assay for PP65 confirmed CMV
infection. Thoracic computerized tomography and gastrointestinal endoscopy were both
negative. Antiviral therapy was started and
immunosuppressive drugs were reduced.
Recovery to some extent was first reported however the skin lesions reappeared
after a few months. So chemotherapy was
recommended.

Case 3
A 43-year-old woman with endstage renal
disease of unknown etiology received a renal
allograft. She received the immunosuppressive drugs including cyclosporine, azathioprine and prednisolone. The CMV state was
reported to be R+, D+ before transplantation.

At the 8th month after transplantation, she

presented with weakness, fatigue, thrombocytopenia, anemia and purpuric lesions.
During the necessary work-up, the laboratory data supported possible CMV infection
and as a result ganciclovir was prescribed.
One month later, she presented with purple
lesions on the anterior side of both legs. Biopsy of the lesions confirmed KS. Cyclosporine maintenance therapy was reduced to
2 mg/kg/day and radiotherapy was indicated.
After a few months the lesions regressed.

Discussion
Kaposis sarcoma secondary to an immunosuppressed state was first identified in 1969 in
kidney recipients. Since then, several cases of
KS were reported in patients receiving immunosuppressives. This disease was also reported as the most frequent cancer following
kidney transplant in developing countries
[Moosa 2005].
Two histologically characteristic features
of KS are known to be proliferation of
angiomatous lesions and of spindle shaped
cells [Itkura et al. 1990]. The cause of post
transplant KS remains unknown. Some factors such as genetic predisposition and oncogenic viruses particularly herpes viruses are
reported to be involved in addition to the immunosuppression therapy [Regumey et al.
1998].
Genetic differences are based on ethnical
differences; for instance, compared with the
normal population, HLA-A2 is more frequent
in KS patients of Saudi Arabia because of the
higher prevalence of HHV-8 in this group
[Moosa 2005].
According to epidemiologic, serologic
and geographic studies and the histological
findings, CMV is identified as a risk factor for
Kaposi sarcoma [Itkura et al. 1990]. In another study carried out to reveal the relation
between CMV infection and Kaposis sarcoma in 64 patients with classic, endemic and
epidemiologic KS, CMV -DNA was only
identified in 10 of the patients who had AIDS
and in neither of classic or endemic cases.
This study did not confirm the distribution
and location of infected CMV cells as a major
pathologic stimulant factor for KS. In other
words, similar to other immuno-suppressed

Razeghi, Hadadi, Khashayar and Pourmand

patients, CMV was identified as an opportunistic infection in these patients [Chakalarouski et al. 1992].
In our patients, KS developed concurrently or shortly after CMV infection which
was similar to other studies [Seigal et al.
1990].
In a study reactivation of cytomegalovirus
was reported in the serologic tests when KS
was diagnosed [Vlasic-Matas et al. 1994]. It
is possible that CMV infection secondary to
the considerable pharmacological immunosuppression was the cause making our patients more susceptible to develop KS.
In neither of the patients presented in this
article, there was no sign of regression following the immunosuppressive medication.
It is possible that the unresponsiveness of our
patients was due to the severity of disease or
other factors. In addition, the presence of
other factors (particularly HHV8) could
neither be excluded nor discussed in order to
define their hypothetical pathogenic role.
The association of CMV and KS suggests
CMV as an inducing factor in KS. However it
is not possible to completely rule out other
factors especially herpes Type 8 and their
pathologic role. Association of KS and CMV
infection in these three patients suggests the
role of CMV in developing Kaposi sarcoma.
Finally, simultaneous CMV infection as the
most important opportunistic infection indicates the severe immunosuppressive condition in patients. As a result it is recommended
to check the CMV state in patients with KS
lesions, because the infection is treatable.

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