(Chest. 2000;117:410-414.)
© 2000
American College of Chest Physicians
Intrathoracic Kaposi’s Sarcoma in Women With AIDS*
Linda B. Haramati, MD, FCCP and
Julie Wong, MD
*
From the Department of Radiology, Albert Einstein College of Medicine, Montefiore Medical Center and Jacobi Medical Center, Bronx, NY.
Correspondence to: Linda B. Haramati, MD, FCCP, Department of Radiology, Albert Einstein College of Medicine and Montefiore Medical Center, 111 East 210th St, Bronx, NY 10467; e-mail: lharamati{at}aecom.yu.edu
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Abstract
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Study objective: To describe the radiographic features
of intrathoracic Kaposi’s sarcoma in women with AIDS.
Subjects and methods: From 1987 to 1998, we identified
seven women with biopsy-proven (n = 4) or autopsy-proven (n = 3)
pulmonary Kaposi’s sarcoma. Charts were reviewed for HIV risk factors,
cutaneous and/or oropharyngeal Kaposi’s sarcoma, CD4 cell count, and
differential diagnosis of pulmonary disease prior to the diagnosis of
pulmonary Kaposi’s sarcoma. Chest radiographs (n = 6), chest CT
scans (n = 3), and reports of unavailable chest radiograph (n = 1)
closest to the time of diagnosis of pulmonary Kaposi’s sarcoma were
reviewed for the following: nodular and peribronchovascular opacities;
thickened interlobular septa; pleural effusions; lymphadenopathy; and
radiographic stage.
Results: Mean patient age was 33
years (range, 27 to 42 years). HIV risk factors were IV drug use
(n = 2), heterosexual contact (n = 3), and both (n = 2). All
patients had prior opportunistic infections. The median CD4 cell
count was 18 /µL (mean, 63/µL; range, 5 to 210/µL).
Cutaneous Kaposi’s sarcoma was diagnosed prior to pulmonary Kaposi’s
sarcoma in four patients, subsequently in two patients, and not
identified in one patient. Oropharyngeal Kaposi’s sarcoma was
diagnosed prior to pulmonary Kaposi’s sarcoma in three patients. Only
infection was considered in the differential diagnosis of the
patients’ pulmonary disease in five patients. One patient presented
with acute hemoptysis and died, and one patient recently received a
diagnosis of pulmonary Karposi’s sarcoma at another hospital. Chest
radiographic findings were the following: nodular opacities in five of
seven patients (71%); peribronchovascular opacities in six of seven
patients (86%); thickened interlobular septa in two of seven patients
(29%); pleural effusion in three of seven patients (43%); and
lymphadenopathy in two of seven patients (29%). Five of seven patients
(71%) were determined to be in radiographic stage 3, one patient in
stage 1, and one patient in stage 2. CT demonstrated additional
lymphadenopathy in three of three patients, thickened interlobular
septa in two of three patients, and pleural effusion in one of three
patients, but it did not change the staging of disease in any
patient.
Conclusion: Pulmonary Kaposi’s sarcoma can
cause diffuse lung disease in women with AIDS. The disease is usually
mistaken clinically for pulmonary infection.
Key Words: AIDS • Kaposi’s sarcoma • thorax • radiology
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Introduction
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An
epidemic of disseminated Kaposi’s sarcoma in homosexual men in
1981 was the initial impetus to investigate and describe the disease
now known as AIDS. Since then, in the United States, AIDS-associated
Kaposi’s sarcoma has continued to be a disease that predominantly
affects homosexual and bisexual men.1
In those men who are
not severely immunocompromised, Kaposi’s sarcoma may remain an
indolent cutaneous disease. However, in patients with advanced AIDS,
Kaposi’s sarcoma will often disseminate to involve the oropharynx,
larynx, tracheobronchial tree, lungs, and other
viscera.1
2
The literature describing the findings of intrathoracic Kaposi’s
sarcoma consists of several series of men3
4
5
and includes
an occasional woman.6
7
In our inner-city population, we
have encountered a number of HIV-infected women with diffuse lung
disease who ultimately received a diagnosis of pulmonary Kaposi’s
sarcoma. The purpose of this study is to describe the radiographic
findings of intrathoracic Kaposi’s sarcoma in these women.
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Materials and Methods
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We retrospectively identified seven HIV-infected women with
pulmonary Kaposi’s sarcoma between 1987 and 1998. The diagnosis was
made by transbronchial biopsy in three women, by autopsy in three, and
by CT-guided needle biopsy in one. All charts were reviewed for HIV
risk factor, CD4 cell count, prior opportunistic infections,
differential diagnosis for the patients’ pulmonary disease prior to
the tissue diagnosis of pulmonary Kaposi’s sarcoma, and the presence
of cutaneous and oropharyngeal Kaposi’s sarcoma. Chest radiographs
(n = 6) or reports of unavailable chest radiograph (n = 1) and
chest CT scans (n = 3) were reviewed using the criteria and
radiographic staging of Gruden et al3
for the presence of
nodular opacities, peribronchovascular opacities, thickened
interlobular septa, pleural effusions, and hilar or mediastinal
lymphadenopathy. Radiographic stages of disease are defined as follows:
stage 1, isolated peribronchial cuffing; stage 2, small nodules; and
stage 3, large nodules or areas of consolidation.
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Results
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The mean patient age was 33 years (range, 27 to 42 years). HIV
risk factors were heterosexual contact in three patients, IV drug use
in two, and both in two. The median CD4 cell count was 18/µL (range,
5 to 210/µL) with a mean of 63/µL. Five patients were Hispanic, one
was African American, and one was African. All patients had prior
opportunistic infections including the following: four instances of
Mycobacterium avium complex; four instances of
Pneumocystis carinii pneumonia; three instances of recurrent
bacterial pneumonia; two instances of Salmonella sepsis; and one
instance each of tuberculosis (TB) Mycobacterium xenopi, GI
cytomegalovirus, GI cryptosporidiosis, esophageal candidiasis,
toxoplasmosis of the brain, and disseminated herpes zoster. In five
patients, only infection was considered in the differential diagnosis
of their pulmonary disease prior to the diagnosis of Kaposi’s sarcoma.
The differential diagnosis in these patients included bacterial
pneumonia (n = 3), P carinii pneumonia (n = 3), and
M avium complex (n = 1). One patient had a recent proven
diagnosis of pulmonary Kaposi’s sarcoma by bronchoscopy at another
institution. Her pulmonary disease was attributed to her known
diagnosis. One patient presented with acute hemoptysis and died, with
the diagnosis of Kaposi’s sarcoma established at autopsy. Six patients
had proven cutaneous Kaposi’s sarcoma. The diagnosis of cutaneous
Kaposi’s sarcoma was made prior to the diagnosis of pulmonary
Kaposi’s sarcoma in four patients, subsequent to the diagnosis of
pulmonary Kaposi’s sarcoma in two, and was not diagnosed in one.
Oropharyngeal Kaposi’s sarcoma was diagnosed prior to pulmonary
Kaposi’s sarcoma in three patients.
The chest radiographs (Table 1
) demonstrated stage 3 disease (Fig 1
) in five patients, stage 1 disease in one patient, and stage 2 disease
in one patient (Fig 2
). In comparison, chest CT scans performed in three patients
demonstrated additional peribronchovascular opacities (n = 1),
thickened interlobular septa (n = 2; Fig 3
), pleural effusion (n = 1), and lymphadenopathy (n = 3). However,
all three patients examined with CT had stage 3 disease demonstrated on
both chest radiographs and CT scans.
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Table 1.. Chest Radiographic and CT (n = 3) Findings and
Radiographic Stages in Seven HIV-Infected Women With Pulmonary
Kaposi’s Sarcoma*
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Figure 1.. Chest radiograph and CT scan of a 42-year-old
African-American woman (patient 3) with stage 3 intrathoracic Kaposi’s
sarcoma. Top: Posteroanterior chest radiograph
demonstrates ill-defined nodular opacities with confluence in the left
mid and lower lung. Thickened interlobular septa are evident in the
right lung. There is a small left pleural effusion.
Bottom: CT scan through the lower lung demonstrates
confluent consolidation in the left lower lobe and lingula. In the
right lung, there are ill-defined large nodules with a
peribronchovascular distribution. Thickened interlobular septa are
present bilaterally. Emphysema is noted.
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Figure 2.. Posteroanterior chest radiograph of a 30-year-old
African woman (patient 5) with a CD4 cell count of 5/µL and stage 2
intrathoracic Kaposi’s sarcoma demonstrating bilateral small nodular
opacities and peribronchial cuffing.
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Figure 3.. Chest radiograph and CT scan of a 31-year-old
Hispanic woman (patient 2) with a CD4 cell count of 18/µL and stage 3
intrathoracic Kaposi’s sarcoma. Top: Posteroanterior
chest radiograph demonstrates nodular opacities of > 1 cm
diameter with perihilar confluence and small bilateral pleural
effusion. Focal right upper lobe opacity is due to prior TB infection.
Bottom: CT image through the mid lung demonstrates
ill-defined nodules with a peribronchovascular predominance and a small
bilateral pleural effusion. Thickened interlobular septa are present
and were noted only on the CT scan.
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Discussion
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In the United States, 90 to 95% of cases of Kaposi’s
sarcoma occur in HIV-infected homosexual and bisexual men.
Intrathoracic involvement is seen in about one third of men with
cutaneous Kaposi’s sarcoma and, consequently, should be strongly
considered when these patients develop diffuse lung
disease.8
To our knowledge, this is the first series
describing the radiographic findings of pulmonary Kaposi’s sarcoma in
women with AIDS. The majority of these patients were initially
suspected of having an infectious etiology for their diffuse pulmonary
disease. In fact, in three patients, the correct diagnosis was
established only at autopsy. Two of the six women with proven cutaneous
disease had that diagnosis made after the diagnosis of pulmonary
Kaposi’s sarcoma prompted a careful skin examination. This can be
attributed to a low index of suspicion for diagnosing Kaposi’s sarcoma
in women with AIDS. Gender bias in the differential diagnosis of
pulmonary disease in women with AIDS has been previously described.
HIV-infected women with P carinii pneumonia9
10
and with TB11
have been reported to be both underdiagnosed
and undertreated compared with men receiving the diagnosis in
the same population. This also seems to be the case for pulmonary
Kaposi’s sarcoma.
The women in this series had chest radiographic and CT scan findings
that were similar to those findings previously described for
intrathoracic Kaposi’s sarcoma.3
4
5
6
7
Chest radiographic
findings included the following: lung nodules (71%);
peribronchovascular opacities (86%); thickened interlobular septa in
(29%); pleural effusion (41%); and lymphadenopathy (29%).
Seventy-one percent of the patients in this series had stage 3 disease
(advanced intrathoracic disease) compared with 50% of patients in the
series of men by Gruden et al.3
The diagnosis of Kaposi’s
sarcoma at an advanced stage in women was also reported by Cooley et
al,12
who described 12 women who received a diagnosis of
Kaposi’s sarcoma at the Boston City AIDS Clinic over a 7-year period.
Eighty-three percent of these women had advanced disease (not confined
to the skin) compared with 25% of men who received a diagnosis of
Kaposi’s sarcoma during the same time period.
Epidemiologic evidence has suggested a sexually transmitted infectious
etiology for Kaposi’s sarcoma for some time, and recently the human
herpesvirus 8 (HHV-8) has been described as the likely etiologic
infection.13
14
15
16
The prevalence of HHV-8 varies among
different populations. It is endemic in Africa. In the United States,
the prevalence of serum antibody to HHV-8 is 1% in the general blood
donor population, 35% among HIV-infected homosexual men, and 4% among
HIV-infected women.17
This correlates closely with the
development of Kaposi’s sarcoma in HIV-infected persons. In the United
States, Kaposi’s sarcoma develops in 20 to 30% of HIV-infected
homosexual and bisexual men and in only 1% of HIV-infected
women.17
Heterosexual contact is the primary mode of HIV transmission
worldwide.14
National AIDS surveillance data indicates
that heterosexual contact is the risk factor for HIV infection that is
increasing most rapidly in the United States.18
19
Heterosexual transmission of HIV is occurring more frequently and in
disproportionate numbers among black and Hispanic women. While HHV-8 is
still most prevalent in the male homosexual and bisexual population,
there is a crossover into the heterosexual population. Eleven percent
of AIDS patients with Kaposi’s sarcoma from a recent Boston City AIDS
Clinic series were women.12
The high prevalence of
Kaposi’s sarcoma in these women was attributed to sexual transmission
of both HIV and HHV-8 by contact with bisexual men, prostitution, or
promiscuous heterosexual activity. In the present series of pulmonary
Kaposi’s sarcoma in seven Hispanic and black women from an inner-city
population, the HIV risk factors were heterosexual contact, IV drug
use, or both. We postulate that HHV-8 was sexually transmitted in all
cases, as IV drug use is closely associated with sexual promiscuity.
Although Kaposi’s sarcoma remains predominantly a male disease at the
present time, a heightened awareness of the occurrence of this disease
in women may lead to diagnosis and treatment at an earlier stage.
A limitation of the present study is the small number of women
comprising the study population. We believe this to be a reflection of
the rarity of the diagnosis of pulmonary Kaposi’s sarcoma in women
with AIDS in the United States. This also may reflect the fact that
bronchoscopic visualization of typical violaceous endobronchial lesions
in patients with cutaneous Kaposi’s sarcoma is usually considered
diagnostic of intrathoracic Kaposi’s sarcoma. Patients with the
endobronchial lesions often do not undergo biopsy due to the risk of
hemorrhage. Therefore, our strict inclusion criteria may have excluded
some women with pulmonary Kaposi’s sarcoma. The radiologic literature
includes two series of patients with intrathoracic Kaposi’s sarcoma in
which one woman is described in each.6
7
The largest
clinical series, to our knowledge, of women with AIDS and cutaneous
Kaposi’s sarcoma, visceral Kaposi’s sarcoma, or both12
was composed of only 12 patients. Therefore, our series of seven women
with AIDS and intrathoracic Kaposi’s sarcoma is, to our knowledge, the
largest series described in the literature to date.
In summary, we describe a group of women with AIDS and diffuse lung
disease who ultimately received a diagnosis of pulmonary Kaposi’s
sarcoma. In most cases, despite the presence of cutaneous lesions and
typical radiographic findings, the presumptive clinical diagnosis was
pulmonary infection. This can be attributed to the low index of
suspicion for the diagnosis of Kaposi’s sarcoma in women with AIDS,
which reflects a gender bias. Although uncommon, pulmonary Kaposi’s
sarcoma should be considered in the differential diagnosis of diffuse
lung disease in women with AIDS.
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Acknowledgements
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We thank Eleanor Murphy for her assistance
in manuscript preparation.
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Footnotes
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Abbreviations: HHV-8 = human herpesvirus 8;
TB = tuberculosis
Received for publication March 12, 1999.
Accepted for publication July 7, 1999.
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Abstract
Introduction
