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Wolf in sheep's clothing: advanced Kaposi sarcoma mimicking vulvar abscess.



Abstract: Kaposi sarcoma is a vascular neoplastic disorder that is associated with the acquired immunodeficiency syndrome acquired immunodeficiency syndrome, see AIDS.  (AIDS). The causative factor in Kaposi sarcoma is human herpes virus-8. This complication of AIDS has a predilection for homosexual males and is rarely associated with the female AIDS population. However, we present a case of Kaposi sarcoma mimicking the benign and common Bartholin gland abscess. A search of the literature including the MEDLINE The online medical database of the U.S. National Library of Medicine (NLM) whose parent is the National Institutes of Health, Bethesda, MD. MEDLINE contains millions of articles from thousands of medical journals and publications. The consumer section of the site (http://medlineplus.  database revealed a single report of Kaposi sarcoma presenting as a vulvar mass, but not as a suspected Bartholin abscess. The history, presentations, risk factors, and treatments available for Kaposi sarcoma are also discussed in this report.

Key Words: abscess, acquired immunodeficiency syndrome, human immunodeficiency virus human immunodeficiency virus
n.
HIV.


Human immunodeficiency virus (HIV)
A transmissible retrovirus that causes AIDS in humans.
, Kaposi sarcoma, vulva

**********

Kaposi sarcoma (KS) is a vascular neoplastic disorder frequently associated with human immunodeficiency virus (HIV) and acquired immunodeficiency syndrome (AIDS) that typically presents with multiple purple or red nodules or plaques. The causative factor is now thought to be human herpes virus-8 (HHV-8) or Kaposi sarcoma-associated herpes virus (KSHV). We present a case of Kaposi sarcoma that presented as a single vulvar lesion previously diagnosed as a Bartholin gland abscess. A literature search of the MEDLINE database revealed a single report in the literature of KS presenting as a vulvar mass, but not as a suspected Bartholin abscess.

Case Report

A 38-year-old female was admitted to a tertiary hospital complaining of vulvar pain and swelling. The patient relayed that the lesion was of acute onset and had become increasingly painful over the last 2 days. Three weeks previously, she had a large left inguinal lymph node measuring 5 X 3 cm excised. There was no history of fever, chills, dysuria dysuria /dys·uria/ (dis-u´re-ah) painful or difficult urination.dysu´ric

dys·u·ri·a
n.
Difficult or painful urination.
, or sexual activity in the past year. In addition, there was no abnormal vaginal discharge or odor.

Pertinent medical history included a diagnosis of HIV in 1989, Pneumocystis carinii pneumonia Pneumocystis carinii pneumonia (PCP)
A lung infection that affects people with weakened immune systems, such as people with AIDS or people taking medicines that weaken the immune system.

Mentioned in: AIDS, Antiprotozoal Drugs, Sulfonamides
, Mycobacterium avium-intracellulare pneumonia, meningococcal meningitis, gonorrhea, and syphilis treated 12 years ago. The patient had two similar episodes of vulvar lesions, 2 months and 2 years previously. The lesions had been incised and drained and treated with antibiotics with resolution of symptoms. Surgical history included bilateral tubal ligation. Her last Papanicolaou smear was several years ago, and she had no knowledge of abnormal results previously. Her social history included intravenous drug use intravenous drug use Intravenous drug abuse The habitual IV injection of drugs of abuse Epidemiology In the US ± 2.5 million–population ± 235 million have used IVDs Infections Pyogenic–eg, endocarditis, pneumonia, sepsis Common agents  and prostitution ceasing 10 years previously.

Physical examination revealed the patient to be afebrile afebrile /afe·brile/ (a-feb´ril) without fever.

a·feb·rile
adj.
Apyretic.



afebrile

without fever.

afebrile adjective Feverless
, with blood pressure of 101/62 mm Hg and a pulse of 81 beats per minute beats per minute Cardiac pacing The unit of measure for the frequency of heart depolarizations or contractions each minute–or pulse rate . Her oropharynx was clear without thrush and her neck was supple. Her cardiac, lung, and abdominal examinations were normal. Extremities were without rash, lesions, or edema. On pelvic examination, her left labia majoris was edematous (3 X 4 cm), with a necrotic area at the introitus (Figure). The edema and induration induration /in·du·ra·tion/ (in?du-ra´shun)
1. sclerosis or hardening.

2. hardness.

3. an abnormally hard spot or place.
 extended along the labia minora to the clitoral hood. Some minimal amount of drainage was noted. This area was extremely tender to palpation palpation /pal·pa·tion/ (pal-pa´shun) the act of feeling with the hand; the application of the fingers with light pressure to the surface of the body for the purpose of determining the condition of the parts beneath in physical diagnosis. .

Initial laboratory data included a white blood cell count white blood cell count,
n a diagnostic clinical laboratory test to determine the number and types of leukocytes present in a measured sample of blood. Overall the normal number of leukocytes ranges from 5000 to 10,000/mm3.
 of 4.0 X [10.sup.3]/[mm.sup.3], hemoglobin of 9.8 g/dL, and a platelet count of 146,000/[mm.sup.3]. Serum electrolyte and liver function tests Liver Function Tests Definition

Liver function tests, or LFTs, include tests for bilirubin, a breakdown product of hemoglobin, and ammonia, a protein byproduct that is normally converted into urea by the liver before being excreted by the kidneys.
 were normal. Gonorrhea and chlamydia polymerase chain reaction polymerase chain reaction (pŏl`ĭmərās') (PCR), laboratory process in which a particular DNA segment from a mixture of DNA chains is rapidly replicated, producing a large, readily analyzed sample of a piece of DNA; the process is  tests of cervical secretions were negative. Her last CD4 cell count 1 month before admission was 8/[mm.sup.3] and her HIV viral load HIV viral load AIDS A measure of the amount of HIV RNA in blood, expressed as number of copies/mL of plasma. See AIDS, HIV.  was 1,200,000/[mm.sup.3].

The lesion was incised, but no purulent drainage was found. A biopsy specimen was taken. Microscopic examination was interpreted as KS. Computed tomography of the abdomen was then performed, and multiple enlarged retroperitoneal retroperitoneal /ret·ro·peri·to·ne·al/ (-per?i-to-ne´al) posterior to the peritoneum.

ret·ro·per·i·to·ne·al
adj.
Situated behind the peritoneum.
 lymph nodes and multiple liver metastases were present. Discussion with the patient was held regarding treatment options. The patient subsequently was referred for hospice care.

Discussion and Review of the Literature

Kaposi sarcoma is a vascular neoplastic disorder that was first discovered by Moritz Kaposi in 1872. Before 1980, it was associated with elderly men of Mediterranean descent. However, it is now most closely associated with HIV or AIDS, and it has a predilection for the subpopulation of homosexual males. HHV-8, now also known as KS-associated herpes virus, is thought to be sexually transmitted and induces neoplastic changes in mesenchymal or endothelial cells. (1) The quantity of circulating HHV-8 correlates with KS stage and progression, whereas HIV viral load and CD4 cell counts do not. (2)

[ILLUSTRATION OMITTED]

The presentation may be varied, but the typical picture is that of red or purple nodules and plaques on the skin or mucocutaneous mucocutaneous /mu·co·cu·ta·ne·ous/ (-ku-ta´ne-us) pertaining to or affecting the mucous membrane and the skin.

mu·co·cu·ta·ne·ous
adj.
Of or relating to the skin and a mucous membrane.
 surfaces. The more common sites involved include the lower extremities, mucous membranes, hard palate, nose, trunk, and scalp. Involvement of the digestive tract has also been reported. Metastasis to local lymph nodes and to various body sites has been reported. (1)

Kaposi sarcoma is relatively uncommon in women, correlating with the fact that HHV-8 infection in women is also less common. Some risk factors for HHV-8 infection in HIV-positive women are intravenous drug use, sexual partners with a history of intravenous drug use, crack cocaine use, previous syphilis infection, and black race. (3,4) It is interesting to note that the subject in this case report had all of these risk factors. There is some evidence that the course of KS in women may be more aggressive or perhaps diagnosed in later stages due to more atypical presentations. Women with KS have been found to have an increased incidence of noncutaneous disease, lymphedema, lymph node disease, and visceral disease. (5,6)

The median survival time in women after diagnosis is approximately 14 months less than that of men; therefore it is of utmost importance to be aware of this disease in attempts to make an early diagnosis and begin treatment. (7) The treatments of KS vary with degree of disease progression. Disease manifestations restricted to the skin can be treated with topical alitretinoin gel, intralesional vinblastine vinblastine /vin·blas·tine/ (vin-blas´ten) an antineoplasticvinca alkaloid used as the sulfate salt in the palliative treatment of a variety of malignancies. , intralesional interferon, radiotherapy, laser therapy, or cryotherapy Cryotherapy Definition

Cryotherapy is a technique that uses an extremely cold liquid or instrument to freeze and destroy abnormal skin cells that require removal.
. Systemic chemotherapy using daunorubicin daunorubicin /dau·no·ru·bi·cin/ (daw?no-roo´bi-sin) an anthracycline (q.v.) antibiotic used as an antineoplastic; administered as the hydrochloride salt or as a liposome-encapsulated preparation of the citrate salt. , doxorubicin, and paclitaxel is necessary in more advanced cases. Experimental therapies include thalidomide and interleukin-12. (4) The initiation of highly active antiretroviral therapy Noun 1. highly active antiretroviral therapy - a combination of protease inhibitors taken with reverse transcriptase inhibitors; used in treating AIDS and HIV
drug cocktail, HAART
 (HAART HAART highly active antiretroviral therapy.
HAART Highly active antiretroviral therapy, triple combination therapy AIDS The concurrent administration of 2 nucleoside reverse transcriptase inhibitors–eg, AZT and 3TC, and a protease
) is also vital, as there are reports of KS regression with initiation of HAART. (8)

Conclusion

We present a case of a primary vulvar lesion previously diagnosed and treated with incision and drainage and antibiotics that on biopsy proved to be KS, with subsequent identification of metastatic foci. This case illustrates the atypical presentation of KS in women. Although KS may be relatively rare in women, physicians should be aware of the risk factors associated with this disease and suspect this diagnosis when any abnormal cutaneous or mucocutaneous lesion is present in this population of patients. The timely diagnosis and treatment of KS is vital to patient survival.

Accepted May 4, 2004.

References

1. Knowles DM, Cesarman E. The Kaposi sarcoma-associated human herpesvirus (human herpesvirus 8) in Kaposi sarcoma, malignant lymphoma and other diseases. Ann Oncol 1997;8:S123-S129.

2. Quinlivan EB, Zhang C, Stewart PW, et al. Elevated virus loads of Kaposi sarcoma-associated human herpesvirus 8 predict Kaposi sarcoma disease progression, but elevated levels of human immunodeficiency virus type 1 do not. J Infect Dis 2002;185:1736-1744.

3. Greenblatt RM, Jacobson LP, Levine AM, et al. Human herpesvirus 8 infection and Kaposi sarcoma among human immunodeficiency virus-infected and -uninfected women. J Infect Dis 2001;183:1130-1134.

4. Hengge UR, Ruzicka T, Tyring SK, et al. Update on Kaposi's sarcoma and other HHV8 associated diseases, I: Epidemiology, environmental predispositions, clinical manifestations, and therapy. Lancet Infect Dis 2002;2:281-292.

5. Blair JM, Kovacs A, Beall G, et al. Kaposi sarcoma in women with AIDS. Abstract 106.4. National Conference of Women and HIV, May 1997.

6. Dezube BJ. Acquired immunodeficiency syndrome-related Kaposi sarcoma: clinical features, staging and treatment. Semin Oncol 2000;27:424-430.

7. Cooley TP, Hirschhorn LR, O'Keane JC. Kaposi sarcoma in women with AIDS. AIDS 1996;10:1221-1225.

8. Parra R, Leal M, Delgrado J, et al. Regression of invasive AIDS-related Kaposi sarcoma following antiretroviral therapy. Clin Infect Dis 1998;26:218-219.

RELATED ARTICLE: Key Points

* Kaposi sarcoma is a vascular neoplastic disorder.

* The causative factor of Kaposi sarcoma is thought to be human herpes virus-8.

* This case describes Kaposi sarcoma mimicking the benign and common condition of a Bartholin gland abscess.

Brent W. Laartz, MD, Christopher Cooper, MD, Amber Degryse, and John T. Sinnott, MD

From the Division of Infectious Diseases, University of South Florida


    [
, Tampa, FL, and the College of Medicine, University of South Florida, Tampa, FL.

Reprint requests to Dr. Brent W. Laartz, 16747 Nikki Lane, Odessa, FL 33556. Email: blaartz@hsc.usf.edu
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Title Annotation:Case Report
Author:Sinnott, John T.
Publication:Southern Medical Journal
Date:Apr 1, 2005
Words:1431
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