BILATERAL ADRENAL KAPOSI’S SARCOMA IN AN HIV SERONEGATIVEPATIENT

THIERRY LAZURE, FRANCOISE PLANTIER, ISSAM ABD ALSAMAD, PATRICK CABANIS,EMMANUELLE MALAURY AND JEAN-ROBERT BLONDEAU

From the Departments of Pathology, Visceral Surgery, Oncology and Radiology, Hopital Intercommunal de Creteil, France

KEY WORDS: sarcoma, Kaposi; adrenal glands; herpesvirus, Kaposi sarcoma-associated

Visceral Kaposi’s sarcoma in the absence of mucocutane-ous lesions is uncommon. Asymptomatic adrenal gland in-volvement associated with many other localizations has beenreported occasionally in AIDS related Kaposi’s sarcoma.1 Wereport a case of apparently exclusive bilateral adrenalKaposi’s sarcoma in an HIV seronegative black patient.

CASE REPORT

A 17-year-old black man living in Congo was hospitalizedfor weight loss, asthenia and vague abdominal discomfort.Abdominal computerized tomography (CT) revealed 2 massesmeasuring 3.5 cm. and 6 cm. in the right and left adrenalglands, respectively (fig. 1). Blood and urinary corticosteroidand catecholamine levels were within normal limits but ad-renocorticotropic hormone (ACTH) increased at 300 ng./ml.(normal 9 to 52) and adrenocorticotropic hormone stimulatedcortisol level was decreased at 176 ng./ml. (normal greaterthan 200), revealing latent adrenal cortical insufficiency.Left adrenalectomy was performed to disclose the nature ofthe lesion. Excision was incomplete because the mass washemorrhagic and locally adherent to the left diaphragmaticpillar.

Macroscopically, the adrenal lesion had ill-defined out-lines, and was soft, brown and hemorrhagic on cut section.Histologically, the tumor was composed of intersecting blandand uniform spindled cells fascicles with intervening slit-likespaces that contained extravasated erythrocytes and hemo-siderin (fig. 2). No hyaline globule could be found. Someplasma cells and lymphocytes were scattered throughout thetumor. The spindled cells expressed CD34. Human herpesvi-rus type 8 detection by in situ hybridization was positive ontumor paraffin block. All findings were consistent with Ka-posi’s sarcoma.

Investigations for other localizations and predisposing fac-

tors were unsuccessful. Indeed, clinical examination revealedneither cutaneous lesions nor adenopathies. Whole body CTdemonstrated no other lesion. Laryngeal, bronchial, esogas-troduodenal and colonic endoscopies were normal. No evi-dence of immunosuppression was found (malnutrition, asso-ciated malignancy, and parasitic, bacteriological andvirological infections). In particular, serologic tests for HIV1–2 were negative.

The patient underwent 3 courses of chemotherapy withbleomycin, etoposide, vinblastine and doxorubicin. Partialregression of the right adrenal tumor occurred. The residualmass was completely removed. Histopathological evaluationdisclosed foci of viable Kaposi’s sarcoma with large areas offibrosis. Three additional chemotherapy courses similar tothe first were performed with the addition of 30 Gy. radio-therapy. Complete remission occurred 5 months after begin-ning treatment.

Physical examination and abdominal CT were performedevery 6 months. At followup 41⁄2 years later he had no recur-rence and was well with substitutive hormone therapy (hy-drocortisone and 9 �-fluorohydrocortisone). Serologic testsfor HIV 1–2 were negative.

DISCUSSION

The lack of overt immunosuppression in our patient sup-ports the endemic Kaposi’s sarcoma hypothesis but it doesnot adhere to any of the 4 well-known clinical subtypes ofcutaneous indolent nodular form; aggressive localized type,characterized by large and exophytic cutaneous lesions in-vading the underlying soft tissues and bone; disseminated,florid, mucocutaneous and visceral form; and lymphadeno-pathic type occurring mainly in children.2 Visceral endemicKaposi’s sarcoma is most often widespread (liver, spleen,lymph nodes, digestive tract, lungs), which is why we couldnot rule out the possibility of occult neoplastic foci elsewhere

Accepted for publication June 29, 2001.

FIG. 1. Abdominal CT shows heterogeneous masses in right andleft adrenal glands.

FIG. 2. Spindle cell proliferation with blood-filled spaces, infiltrat-ing adrenal tissue (lower left) and scanty lymphoplasmacytic infil-trate. H & E, reduced from �250.

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that could have been eradicated by the first courses of che-motherapy. Human herpesvirus type 8 is involved in thepathogenesis of Kaposi’s sarcoma, and detection of the virusby in situ hybridization is a highly sensitive and specificdiagnostic test, particularly in cases with unusual presenta-tion.3

Depending on the clinical situation, single and multiagentcytotoxic chemotherapy, interferon �, radiotherapy and localtherapies as well as surgery have all been used successfullyfor Kaposi’s sarcoma.4 In patients with visceral Kaposi’s sar-coma, systemic chemotherapy is indicated. The combinationof doxorubicin, bleomycin, vinblastine and etoposide has pro-duced high overall response rates in previous studies.4 Sur-gery is often used for localized cutaneous lesions,4 and wastried in our case because Kaposi’s sarcoma was apparentlyconfined to the adrenal glands. Moreover, right adrenal

gland excision was useful to differentiate between cicatricialtissue and neoplastic remnants after chemotherapy.

REFERENCES

1. Tappero, J. W., Conant, M. A., Wolfe, S. E., et al: Kaposi’ssarcoma. Epidemiology, pathogenesis, histology, clinical spec-trum, staging criteria and therapy. J Am Acad Dermatol, 28:371, 1993

2. Stein, M. E., Spencer, D., Ruff, P. et al: Endemic African Kaposi’ssarcoma: clinical and therapeutic implications. 10-year expe-rience in the Johannesburg Hospital (1980–1990). Oncology,51: 63, 1994

3. Cathomas, G.: Human herpes virus 8: a new virus discloses itsface. Virchows Arch, 436: 195, 2000

4. Northfelt, D. W.: Treatment of Kaposi’s sarcoma. Current guide-lines and futures perspectives. Drugs, 48: 569, 1994

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