|Year : 2020 | Volume
| Issue : 1 | Page : 177-179
Human herpes virus-8-negative kaposiform sarcoma in a HIV-positive male: Novel variant
Department of Dermatology, Saham Hospital, Saham, Oman
|Date of Submission||18-Jan-2017|
|Date of Decision||15-Jun-2017|
|Date of Acceptance||10-Mar-2018|
|Date of Web Publication||23-Jul-2018|
Department of Dermatology, Saham Hospital, P O Box.582, Pc-319
Source of Support: None, Conflict of Interest: None
Human herpes virus (HHV)-8 is associated with causation of Kaposi's sarcoma (KS). An HIV-positive male with multiple nodules on the body was clinically and histopathologically diagnosed as a case of KS; however, immunohistochemistry was negative for HHV-8. This peculiar scenario of HHV-8-negative tumor with typical clinical and histopathologic findings was labeled as “Atypical spindled endothelial proliferation suspicious of Kaposi sarcoma”, hitherto unreported novel entity.
Keywords: Human herpes virus-8, HIV, Kaposi's sarcoma, kaposiform, sarcoma
|How to cite this article:|
Kumar P. Human herpes virus-8-negative kaposiform sarcoma in a HIV-positive male: Novel variant. J Can Res Ther 2020;16:177-9
| > Introduction|| |
Kaposi's sarcoma (KS) is a multifocal, endothelial proliferation predominantly involving the skin and other organs and associated with formation of vascular channels and proliferation of spindle-shaped cells. KS has become the most common neoplasm in AIDS patients.
KS poses problems in histologic diagnosis because of its broad morphologic spectrum and similarity to many benign vasoproliferative lesions, for example, pyogenic granuloma, bacillary angiomatosis, microvenular hemangioma, and tumors with a prominent spindle cell component, for example, spindle cell hemangioma (SCH), spindle cell angiosarcoma, and dermatofibrosarcoma protuberans.,
KS associated with HIV infection was first identified when an epidemic of this tumor was identified in the homosexual community in New York. It is more common in homosexual and bisexual males than in others at risk.,
We present the curious case of a young homosexual man with nodules scattered on the limbs and trunk. His clinical and histologic features were typical of KS; however, stain for human herpes virus (HHV-8) was negative. The male tested HIV positive with a high viral load and low CD4 count.
This unusual situation previously unreported is presented, and we also discuss implications of such novel findings.
| > Case Report|| |
A 33-year-old unmarried male presented with multiple skin-colored nodules on the body for 3 months' duration. The nodules were 1–1.5 cm in length and 0.5 cm (approximately) in width, scattered on the dorsum of hands, forearms, arms, thighs, legs and trunk, sparing the face, scalp, palms, soles, and orogenital areas. The nodules were firm to hard in consistency and nontender.
He was a heavy smoker, moderate alcoholic, and a homosexual. Systemic examination ruled out any lymph node enlargement, hepatomegaly, or splenomegaly. Pitting edema was evident on the feet.
Routine investigations, including complete blood count, liver functions, renal functions and urine, were all within normal limits. Venereal disease research laboratory, hepatitis B, and hepatitis C were all negative. A nodule from the right forearm was excised for histopathology. He was tested positive for HIV which was confirmed by Western blot; other parameters were as follows: CD4 count was 79 cells/μL, viral load was 8966 copies/ml, log10 was 3.95, CD percentage was 15.66%, and lymphocytes was 505 cells/μL. His chest X-ray, computerized tomography scan of the chest and abdomen did not show involvement of any internal organ.
The histopathology showed an unremarkable epidermis and dermis showed a diffuse infiltrate of small slit-like blood vessels, intimately associated with spindle cells displacing the collagen [Figure 1] and [Figure 2]. Focal intracytoplasmic inclusion intraluminal red blood cells were noted.
|Figure 1: Slit-like blood vessels in histopathology. Stain H and E, ×40 magnification|
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|Figure 2: Same slide showing spindle cells and blood vessels. Stain H and E, ×100 magnification|
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Immunohistochemistry revealed spindle cells positive for CD31 negative for HHV-8 however.
The interim laboratory report labeled it as a vascular spindle cell lesion typical of KS. In view of negative HHV-8 report, second opinion was sought from a sarcoma expert and the final opinion has been added – the spindle cell component is highlighted by stains for CD31 and erythroblast transformation specific-related gene (ERG). Stains for smooth muscle actin (SMA) and HHV-8 are negative. Thus, on morphological grounds alone, the appearance and clinical description would fit well with KS, but negativity for HHV-8 is extremely unusual; hence, it was termed as “atypical spindled endothelial proliferation suspicious for KS.”
| > Discussion|| |
KS is a malignant neoplasm of the vascular endothelium that is multifocal in origin involving the skin and other organs. KS associated with HIV is more common in homosexuals and bisexuals than in others at risk. Our patient was a homosexual and was diagnosed HIV-positive during workup.
KS may pose problems in histologic diagnosis with benign vasoproliferative lesions, for example, pyogenic granuloma, bacillary angiomatosis, and microvenular hemangioma, and tumors with a prominent spindle cell component, for example, SCH, spindle cell angiosarcoma, and dermatofibrosarcoma protuberans.
The tumorous nodules in KS are compartmentalized by dense bands of fibrocollagenous tissue. The characteristic feature is a honeycomb-like network of blood-filled slits that are closely associated with interweaving spindle cells. Flattened endothelial cells lining the vascular clefts are easily recognized with immunohistochemical markers CD31 and CD34. The endothelial cells of the capillary walls are more prominent in the vascular spaces of pyogenic granuloma, and most angiomas and the vessels are set farther apart by intervening stroma.
SCH, kaposiform hemangioendothelioma, and moderately differentiated angiosarcomas with spindle cell differentiation are the most important histologic mimickers of nodular KS lesions. SCH is distinguished by cavernous or widely dilated vascular spaces and collections of epithelioid cells with or without intracytoplasmic lumina.
Kaposiform hemangioendothelioma is mainly a disease of children that usually affects deep soft tissues although the skin can be affected. Its histology shows intermediate features of capillary hemangioma and has lobular growth pattern which is absent in KS. Furthermore, the capillaries at the periphery of tumor lobules in kaposiform hemangioendothelioma often show focal thrombosis; there may also be focal epitheloid endothelial cells, and eosinophilic globules may be absent.
Occasionally, spindle cell differentiation is prominent in angiosarcoma, but usually markedly atypical cells are present, allowing differentiation from KS.
Spindle cell fascicles with a bland cytologic appearance are not a prominent component of bacillary angiomatosis and pyogenic granuloma. Demonstration of HHV-8 with immunohistochemistry is of great help in histologic differential diagnosis of KS as all vascular and nonvascular entities mentioned above are usually not associated with this virus.
Our patient had distinct features of KS histologically with CD31 and erythroblast regulatory gene (ERG) positivity, however, negative for HHV-8 and SMA. It has been established that HHV-8 is the primary and necessary factor in the development of KS. Hence, this case was labeled as “atypical spindled endothelial proliferation suspicious for KS” which is short of KS and makes it unique.
Although classic KS has been reported from Oman, KS with HIV has not been documented earlier. Aggressive and widespread lesions are typical of epidemic KS, and these patients are younger than those affected by classic KS.
The absence of HHV-8 in this patient despite clinical and histological evidence of KS raises pertinent questions. It is plausible that this is a novel entity which mimics KS and is yet unreported. The patient was alcoholic, homosexual, and also had HIV infection; there is a possibility that he had all cofactors associated to induce tumors of KS sans HHV-8, which hypothetically indicates that HHV-8 may not be an essential trigger for KS.
Epidemic KS lesions resolve with institution of highly active antiretroviral therapy, yet the traces of HHV-8 remain in the body. In contrast, our case was de novo who was not on any antiviral therapy. In addition, it does not seem possible that overwhelming immunosuppression due to HIV may have overshadowed or removed traces of HHV-8 from the body.
Thus, we present an interesting case of atypical spindled endothelial proliferation suspicious for KS in male with HIV.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]