Monday, August 27, 2012

Lymphangioma-Like Kaposi Sarcoma.

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.

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

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).

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