Keywords: Gastrointestinal system; Kaposi
KSHV, a member of the gamma herpes virus family,
is the etiological agent of the disease but is not capable
of causing KS alone. Although it is not fully clear which
factors trigger the oncogenic transformation of KSHV,
iatrogenic or acquired immunosuppression appears to
be an important factor for KS development. The oncogenic
genes of KSHV induce genes that stimulate cellular
proliferation, transformation, cellular signaling,
cytokine production, immune escape, antiapoptotic,
and angiogenenic action. Although the origin of spindle
cells, the real neoplastic cells of KS, is still controversial, it has been shown that they develop from uncommitted
endothelial progenitors, with the lymphatic
endothelial type being most predominant.[
KS is a neoplastic model where cancer and inflammation
are intertwined and has different epidemiological
forms with variable clinical course despite a common
etiological agent. Classic KS is usually characterized by
slowly progressive cutaneous lesions in extremities in
older men. Visceral organ involvement is rare in classic
KS.[
In this study, a 72-year-old male classic KS patient
with skin, gastrointestinal system, and lymph node involvement
was discussed in terms of clinicopathologic
features.
The patient was found to be HIV-negative. On endoscopy,
a hemorrhagic, hyperemic nodular lesion was
found in the postbulbar region. Diagnostic biopsies
were taken from lesional skin, duodenum, and lymph
node regions. Histopathologic examination showed
infiltrative, extravascular erythrocyte-bearing spindle
cell proliferation, which removed the usual tissue in all
three biopsy specimens. Typical and atypical mitotic
figures were present in the tumor. In neoplastic cells,
diffuse immunoreactions with CD31, CD34, vimentin, and HHV-8 (LNA-1) were detected (Fig.
Lymphadenopathic KS is a symptomatic manifestation
of clinical lymph node enlargement resulting in
neoplastic infiltration of KS in single or multiple lymph
nodes. In classic KS, lymph node involvement is rare.
In an epidemiological study in Morocco, the visceral
organ involvement rate was 16% in classic KS cases
and about half of these cases were lymphadenopathic
KS cases.[
In classic KS, gastrointestinal system involvement is the second most common extra cutaneous localization
after lymph node involvement. The colon, small
intestine, and stomach are among the most common
intestinal localizations. Lower gastrointestinal system
KS may cause bleeding, perforation, and occasionally
obstruction. Upper gastrointestinal system KS cases
are usually asymptomatic and sometimes can be detected
with hemoptysis.8 Gastrointestinal KS differential
diagnosis includes a wide range of diseases. It
can be confused clinically/endoscopically with nonneoplastic
lesions such as ulcerative colitis, amebiasis,
and conditions with massive lesions with spindle cell,
vascular-rich tumors.[
Classic KS is generally characterized by slow-onset
cutaneous lesions, and the mortality rate varies between
0.6% and 9% in various series. However, in older
patients, many factors (i.e., chronic systemic diseases,
immunosuppressive treatments, severe infections) that
disrupt the general condition of the individual may reveal
visceral organ involvement. It has been reported
that cutaneous or visceral KS develops during immunosuppressive
treatment regimens among individuals
without a history of KS. KS may develop during rituximab
treatment, which is used for severe autoimmune
disorders, chronic inflammatory disorders, and prevention
of organ rejection, and may regress following
its cessation.[
Our case, similar to the general characteristics of
classic KS, was of advanced age and male sex. For many
years, localized cutaneous KS was diagnosed without
visceral organ involvement. We think that the development
of visceral organ involvement in our case was
highly likely due to steroids used in the treatment of
interstitial lung disease and severe pulmonary infection.
Our idea is supported by several previous studies
reporting that steroids and other immunosuppressive
agents cause the emergence of de novo KS lesions or
the progression of pre-existing KS lesions.
In our case, upper gastrointestinal system KS involvement
was asymptomatic and detected during
endoscopic examination in accordance with the literature.
It has been reported that systemic involvement
may occur in classic KS cases, rarely without cutaneous
lesions. A more detailed clinical examination of
visceral organ involvement in classic KS cases may provide more accurate information in determining the
frequency of systemic involvement of this disease.
Disclosures Statement
The authors declare no conflicts of interest.
Ethics Committee Approval: This study was conducted inaccordance
with local ethical rules.
Peer-review: Externally peer-reviewed.
Conflict of Interest: None declared.
Authorship contributions: Concept - N. O. K; Design - N.
O. K, F. B; Supervision - N. O. K, G. Y; Materials - N. O. K,
B. D. G; Data collection &/or processing - N. O. K, İ. E. B;
Analysis and/or interpretation - N. O. K, Ş. O. Ö; Literature
search - N. O. K, F. B; Writing - N. O. K, Ş. O. Ö; Critical
review - N. O. K, F. B, G. Y, B. D. G, İ. E. B, Ş. O. Ö.