Dermoscopic Rainbow Pattern in Kaposi's Sarcoma Lesions: Our Experience

Oct 2012

Rosanna Satta, MD; Leonardo Fresi, MD; Francesca Cottoni, PhD

Arch Dermatol. 2012;148(10):1207-1208. doi:10.1001/archdermatol.2012.2204.

Cheng et al were the first to define the multicolored areas in some Kaposi's sarcoma (KS) lesions observed under polarized-light dermoscopy as rainbow pattern and to describe this dermoscopic feature as specific but not sensitive for the diagnosis of KS. Their observation prompted several interesting discussions in the dermoscopic literature. In our clinical practice, we have observed a high number of classic KS (CKS) cases. Dermoscopic examination of all the KS lesions in a group of patients with CKS was performed to evaluate the sensitivity of the rainbow pattern feature observed in the different clinical types of KS lesions.

Archives of Dermatology

Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review.

Sept 2012

Bhatia K, Shiels MS, Berg A, Engels EA.

Source

Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Rockville, Maryland, USA. bhatiak@mail.nih.gov

Abstract

PURPOSE OF REVIEW:

In immunodeficiency, an increased sarcoma risk is confirmed for Kaposi's sarcoma. Whether rates of other sarcoma subtypes are elevated in the setting of immunodeficiency is not known. We therefore reviewed published case reports on HIV and AIDS patients and organ transplant recipients with sarcomas. For comparison, we assessed sarcomas in the U.S. general population using Surveillance Epidemiology End Results (SEER) data.

RECENT FINDINGS:

A total of 176 non-Kaposi sarcoma were identified, 75 in people with HIV and AIDS and 101 in transplant recipients. Leiomyosarcomas (n = 101) were the most frequently reported sarcomas, followed by angiosarcomas (n = 23) and fibrohistiocytic tumors (n = 17). Leiomyosarcomas were reported with two age peaks, in children and young adults. Epstein-Barr virus (EBV) was detected in the tumor cells in 85 and 88% of leiomyosarcomas in HIV-infected people and transplant recipients, respectively. Angiosarcomas and fibrohistiocytic tumors were most frequently reported in men. Among kidney transplant recipients, 20% of sarcomas arose at the site of an arteriovenous fistula. In comparison, leiomyoscarcomas, angiosarcomas, and fibrohistiocytic tumors comprised 16.9, 3.8, and 18.7% of sarcomas in the U.S. general population.

SUMMARY:

Leiomyosarcoma and angiosarcoma may occur disproportionately in immunodeficiency. Leiomyosarcomas appear causatively linked to EBV, whereas angiosarcomas might be correlated with an arteriovenous fistula. Additional studies are necessary to understand the contribution of immunodeficiency to the cause of these sarcomas.

Lippincott, Williams & Wilkins

Histopathological analysis of vesicular and bullous lesions in Kaposi sarcoma.

Aug 2012

Onak Kandemir N, Barut F, Dogan Gun B, Solak Tekin N, Hallac Keser S, Oguz Ozdamar S.

Abstract

BACKGROUND: In this study, the clinical and morphological features of vesiculobullous lesions observed inKaposi sarcoma are analyzed, and the features of bullous Kaposi sarcoma cases are emphasized.

METHODS:

A total of 178 biopsy materials of 75 cases diagnosed as classic-type cutaneous Kaposi sarcoma were reviewed. Twenty-five cases showing vesiculobullous features were included in the study. Tumor, epidermis, dermis, and clinical data regarding these cases was evaluated.

RESULTS:

Vesicular changes were observed in 21 (12%) out of 178 lesions of the 75 cases, while bullous changes were present in only 4 (2%). In all cases where vesicular and bullous changes were detected, tumor, epidermis, and dermis changes were similar. All cases were nodular stage KS lesions, whereas hyperkeratosis and serum exudation in the epidermis, marked edema in the dermis, and enlarged lymphatic vessels and chronic inflammatory response were observed.

CONCLUSIONS:

Our findings suggest that changes in vascular resistance occurring during tumor progression are the most important factors comprising vesiculobullous morphology. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1646397188748474.

Diagnostic Pathology

Vaccine prospect of Kaposi sarcoma-associated herpesvirus.

Aug 2012

Wu TT, Qian J, Ang J, Sun R.

Source

Department of Molecular and Medical Pharmacology, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA 90095, United States. twu@mednet.ucla.edu

Abstract

Infection of Kaposi sarcoma-associated herpesvirus (KSHV) or human herpesvirus-8 (HHV-8) is estimated to account for 34,000 new cancer cases globally. Unlike other herpesviruses, KSHV is not ubiquitous but is highly prevalent in some areas, such as sub-Saharan Africa where Kaposi sarcoma is the leading cancer among adults. While latent infection of KSHV plays a major and direct role in tumorigenesis, viral lytic replication also makes significant contributions to this process. Efforts to develop a KSHV vaccine are limited, but studies with EBV have provided important lessons. Informative vaccine research has been conducted in the mouse infection model of a closely related rodent virus, murine gammaherpesvirus-68 (MHV-68 or γHV-68). This mouse model has generated fundamental principles for an effective vaccination strategy. KSHV vaccines designed to prevent a naïve host from infection and to boost the immune control of KSHV in persistently infected people will have major impact on individuals who are at a high risk of developing KSHV-associated diseases.

SciVerse

Lymphatic Reprogramming by Kaposi Sarcoma Herpes Virus Promotes the Oncogenic Activity of the Virus-Encoded G-protein Coupled Receptor.

Aug 2012

Aguilar B, Choi I, Choi D, Chung HK, Lee S, Yoo J, Lee YS, Maeng YS, Lee HN, Park E, Kim KE, Kim NY, Baik JM, Jung JU, Koh CJ, Hong YK.

Source

Surgery and Biochemistry and Molecular Biology, University of Southern California.

Abstract

Kaposi sarcoma (KS), the most common cancer in HIV-positive individuals, is caused by endothelial transformation mediated by the KS herpes virus (KSHV)-encoded G-protein coupled receptor (vGPCR). Infection of blood vascular endothelial cells (BECs) by KSHV reactivates an otherwise silenced embryonic program of lymphatic differentiation. Thus, KS tumors express numerous lymphatic endothelial cell (LEC)-signature genes. A key unanswered question is how lymphatic reprogramming by the virus promotes tumorigenesis leading to KS formation. In this study, we present evidence that this process creates an environment needed to license the oncogenic activity of vGPCR. We found that the G-protein regulator RGS4 is an inhibitor of vGPCR that is expressed in BECs, but not in LECs. RGS4 was downregulated by the master regulator of LEC differentiation PROX1, which is upregulated by KSHV and directs KSHV-induced lymphatic reprogramming. Moreover, we found that KSHV upregulates the nuclear receptor LRH1, which physically interacts with PROX1 and synergizes with it to mediate repression of RGS4 expression. Mechanistic investigations revealed that RGS4 reduced vGPCR-enhanced cell proliferation, migration, VEGF expression and Akt activation and to suppress tumor formation induced by vGPCR. Our findings resolve long-standing questions about the pathological impact of KSHV-induced reprogramming of host cell identity, and they offer biological and mechanistic insights supporting the hypothesis that a lymphatic microenvironment is more favorable for KS tumorigenesis.

Cancer Research

or

PubMed

Lymphangioma-Like Kaposi Sarcoma.

August 2012

We report on a recent lesion in a 75-year-old woman who

had been diagnosed with classic Kaposi sarcoma (KS) 8 years earlier, at which time she presented with plaques on the right thigh and right forearm (Fig. 1A) and no metastasis was detected. Findings from the initial biopsy of the forearm lesions were compatible with nodular KS.

During the 8-year follow-up, the patient developed 7 recurrent skin tumors on the forearm and legs. Each tumor had the same clinical appearance in the form of plaques (Fig. 1B), and no systemic involvement was detected at any time. The lesions were treated with liposomal doxorubicin. Biopsies were taken from the forearm at each recurrence and histology continued to show findings consistent with nodular KS mixed with lymphangioma-like areas. 

These areas were comprised of irregularly-dilated ectatic vascular spaces in the reticular dermis that were lined by moderately atypical endothelial cells. These spaces were greater in number and size than normal lymphatic vessels (Fig. 2). Immunohistochemistry showed strong CD34 positivity. Endothelial cells lining the lymphangioma-like areas of the tumor were

also positive on staining with human herpes virus type 8

(HHV-8) antibody (Fig. 2C) and the lymphatic endothelial

marker podoplanin (D2-40) (Fig. 2D). Based on these findings, the patient was diagnosed with lymphangioma-like KS.

Seven months after completing the most recent cycle of

chemotherapy with doxorubicin, the patient presented with a serious recurrence consisting of localized, multinodular, ulcerated lesions on both legs but more severe on the right thigh; severe lymphedema was also noted (Fig. 1C). She was given radiotherapy, second-line chemotherapy with paclitaxel (taxol), and thorough local treatments. At the time of writing the lesions remained ulcerated and had not changed in size. The patient attended scheduled follow-up visits and did not show signs of systemic spread of disease.

Full Text Article with Diagnostic Images

Elsevier

Highly active antiretroviral therapy-associated flare of oropharyngeal kaposi sarcoma.

August 2012

Tesuven K. Naidu, MBChB(Natal), FCORL(SA), MMed(ORL)(UKZN); Pratistad

Kaposi sarcoma (KS) is a human herpesvirus 8–associated multifocal, angioproliferative tumor that occurs in cutaneous, mucocutaneous, and visceral locations. The presentation of KS in the oral cavity is of prognostic importance as it may be the initial manifestation of generalized KS or even the sentinel clue to human immunodeficiency virus (HIV) infection and AIDS. Oral cavity KS is also associated with higher death rates when compared with cutaneous KS alone. Furthermore, oral cavity KS is a prognostic marker of an individual's response to highly active antiretroviral therapy (HAART). Despite the role of HAART in effecting stabilization, regression, and a significant reduction in the incidence and morbidity of KS, adverse clinical events that are temporally associated with the onset of HAART are being increasingly recognized. Immune reconstitution inflammatory syndrome (IRIS) is characterized by an exuberant immune-mediated inflammatory response to latent antigenic triggers after the initiation or resumption of HAART. It results in the appearance or recurrence of previously treated or undetected opportunistic infections, the flare of viral infections, and the development of neoplasms. To date, however, airway obstruction as a manifestation of a KS flare after HAART is undocumented in the global literature (to our knowledge).

Full Text Article

Otolaryngology - Head & Neck Surgery