Familial Classic Kaposi Sarcoma in Two Siblings: Case Report and Literature Review

Almohideb, Mohammad; Ak, Watters; Gerstein, William

doi:10.2310/7750.2013.12082

Cited by 7 publications

(9 citation statements)

References 23 publications

(39 reference statements)

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“…Dear Editor , Kaposi sarcoma (KS) is an angioproliferative malignancy, subclassified into classic, African (endemic), AIDS‐related and iatrogenic type . A new subgroup of patients with KS has been increasingly recognized – men who have sex with men (MSM), but who are HIV‐seronegative and have no identifiable immunodeficiency.…”

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Kaposi sarcoma in HIV ‐negative men who have sex with men

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DEAR EDITOR, Kaposi sarcoma (KS) is an angioproliferative malignancy, subclassified into classic, African (endemic), AIDS-related and iatrogenic type. 1 A new subgroup of patients with KS has been increasingly recognizedmen who have sex with men (MSM), but who are HIV-seronegative and have no identifiable immunodeficiency. Herein, we describe a series of patients who do not fit into the current classification system for KS.Between 1997 and 2011, eight HIV-seronegative MSM patients with histologically confirmed KS were identified from the personal archives of the authors (one patient from Lewisham Hospital; seven from Chelsea and Westminster Hospital). The median age of the patients was 53 years (range 37-65). Seven (88%) patients were white British, and one patient was of Hong Chinese descent. Four (50%) patients were skin type I, three (38%) were skin type II and one (12%) was skin type IV. The number of KS lesions varied from one to 50, with a median of four lesions. Two (25%) patients had only one KS lesion. Four (50%) patients had KS lesions confined to their lower limbs, and three (38%) patients had both upper and lower limb involvement. One patient had a single lesion located on the temple.At the time of KS diagnosis, two (25%) patients had KS-associated herpesvirus (KSHV) viraemia, with detectable plasma KSHV DNA (570 and 700 copies mL À1 , respectively). Immunohistochemistry for KSHV latent nuclear antigen 8 was positive for all cases. The histological grade of KS for six (75%) patients was of nodular grade; one patient had angiomatoid grade KS; and one patient had an unspecified grade of KS. The primary therapy for six (75%) patients was surgical excision. For the remaining two patients, the KS was treated with radiotherapy (n = 1) and systemic chemotherapy (n = 1). After a median follow-up of 3Á7 years (0Á2-15), all patients were alive.Only a few sporadic reports regarding KS in HIV-seronegative MSM have been published. In 1986, Marquart et al. 2 described the case of a 44-year-old HIV-seronegative bisexual man who developed a slowly progressing KS nodule on the glans penis. Garc ıa-Muret et al. reported the case of a 42-year-old white bisexual HIV-seronegative man with disseminated KS limited to the skin and gastrointestinal tract. The CD4/CD8 ratio was normal, and the KS remained indolent over a 30-month follow-up period. 3 Kua et al.reported the case of 53-year-old man who had sex with men and who had KS of the buccal mucosa. The patient was HIV-seronegative with no other cause of immunodeficiency identified. 4 Six HIV-seronegative MSM patients were described by Friedman- Kien et al. in 1990. 5 The mean age of the group was 45 years (range 32-62). Three of the patients had KS lesions limited to the legs only, with two patients developing a single KS lesion on the penis. 5 Lanternier et al. 6 published the largest case series thus far. The series, covering a 12-year period, consisted of 28 HIV-seronegative MSM patients with histologically proven KS. 6 The mean age at diagnosis in this cohort was 55 y...

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“…The disease has a viral etiology and a multifactorial pathogenesis hinged on an immune dysfunction. Changes are multifocal, with a course ranging from indolent, with only skin manifestations, to fulminant, with extensive visceral involvement [ 1 , 2 ]. An increased incidence of Kaposi's sarcoma has been described in organ transplant recipients receiving immunosuppressive therapy.…”

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“…Treatment guidelines for each form of Kaposi's sarcoma are outlined, because a unique standard therapy for all of them cannot be considered due to heterogeneity of the disease. Management, which may depend on a variety of factors including the clinicopathologic type of Kaposi's sarcoma and results of staging, ranges from no treatment to local measures such as intralesional vinblastine or systemic administration of cytotoxic chemotherapy for disseminated disease [ 1 , 7 – 10 ].…”

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“…Kaposi's sarcoma is a low-grade angioproliferative tumor associated with Kaposi's sarcoma herpesvirus/human herpesvirus 8 (KSHV/HHV-8) infection. Its multifactorial pathogenesis hinges on an immune dysfunction [ 1 , 2 , 11 ].…”

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“…Latency-associated nuclear antigen (HHV-8) is the most specific immunohistochemical marker available to help distinguish Kaposi's sarcoma from its mimics [ 2 , 12 ]. The disease is multifocal, with a course ranging from indolent, with only skin manifestations, to fulminant, with extensive visceral involvement [ 1 , 13 , 14 ]. In our case only the skin was involved.…”

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