A case of classic Kaposi's sarcoma with multiple organ involvement

Kaposi's sarcoma is a systemic, multifocal, angiomatous tumor which can be classified into four clinical variants. Among them, classic Kaposi's sarcoma is rare in Oriental people, and the involvement of internal organs in this variant is reported to be very rare. We present a case of classic Kaposi's sarcoma in a 67-year-old Korean male, who had multiple organ involvement in the skin, lymph node, and stomach. Lesions of Kaposi's sarcoma cleared after treatment with etoposide, but the skin lesions recurred after a three-month symptom-free period. He was treated with doxorubicin, bleomycin, and vincristine, and all the skin lesions were cleared.     

Classic Kaposi's sarcoma treated with topical rapamycin

Kaposi's sarcoma (KS) is an angioproliferative disorder caused by human herpesvirus 8 (HHV‐8). Current research efforts have focused on the study of the relative role of KSHV‐encoded genes in Kaposi's sarcomagenesis in order to identify novel mechanism‐based therapies for patients suffering from this tumor. Although several viral genes have potential for KS pathogenesis, compelling data point to the KSHV‐encoded G protein‐coupled receptor (vGPCR) as a leading candidate viral gene for the initiation of KS. Interestingly, the oncogenic potential of vGPCR seems to correlate with its capacity to activate the mammalian target of rapamycin (mTOR) signaling pathway. Rapamycin, the prototypical inhibitor of the mTOR signaling pathway, has recently emerged as an effective treatment for KS when administered orally. In this case report, we present an immunocompetent patient with KS lesions treated with topical rapamycin achieving clinical and histologic healing after 16 weeks of treatment. The topical application of rapamycin could be a novel therapeutic option for the treatment of KS.     

A case of Kaposi's sarcoma associated with pemphigoid nodularis

Pemphigoid nodularis is a rare variant of bullous pemphigoid. Bullous pemphigoid complicated by internal malignancy has been well documented. There have been only a few reported cases of bullous pemphigoid associated with Kaposi's sarcoma. We described a case of pemphigoid nodularis who later developed Kaposi's sarcoma that might be related to immunosupressive therapy, a situation that has not been reported previously.     

Combination of vinblastine and bleomycin as first line therapy in advanced classic Kaposi''s sarcoma

BACKGROUND: Classic KS (CKS) mainly affects elderly people, has an irregular course, and is relatively benign for years. However, sometimes the disease may progress rapidly and spread to internal organs, thus necessitating systemic chemotherapy. We therefore decided to carry out a prospective trial using vinblastine and bleomycin, which are active, easy to administer and control, and low cost. METHODS: We treated 29 patients affected by CKS with vinblastine i.v., up to 10 mg in combination with bleomycin i.m., 15 IU every 3 weeks. We administered a median of seven cycles of therapy. RESULTS: All the 29 enrolled patients were evaluated: 21% reached a complete response and 76% had an intermediate response. Toxicity was limited. The maximal response was attained in a median of 5 months, with a mean duration of 4 months. CONCLUSION: The combination of vinblastine and bleomycin achieved a high rate of objective responses in a subgroup of elderly and symptomatic patients, without considerable toxicity. We recommend the combination as first line chemotherapy for advanced CKS.     

A case of classic Kaposi's sarcoma in a Japanese man: detection of human herpes virus 8 (HHV-8) infection by means of polymerase chain reaction and immunofluorescence assay

The recently discovered human herpes virus 8 (HHV-8) has been implicated in the pathogenesis of Kaposi's sarcoma (KS). Because classic KS in Japan is rare and the detection of HHV-8 DNA by polymerase chain reaction (PCR) has been successful only in limited cases, the frequency and role of HHV-8 infection in KS in Japan remain unclear. Herein we report a case of classic KS in a Japanese man whose HHV-8 infection was confirmed by the detection of lesional viral DNA and serum antibodies against lytic antigen.     

Pediatric Kaposi's sarcoma associated with immune reconstitution inflammatory syndrome

Immune reconstitution inflammatory syndrome (IRIS) represents paradoxical immune-mediated inflammation in response to an infecting pathogen, occurring after initiation of antiretroviral therapy (ART), concomitantly with immune system recovery. It has also been described in Kaposi's sarcoma (KS). We report a case of a 9-year-old Guinean girl, who developed Kaposi's sarcoma, following introduction of ART. KS associated with immune reconstitution inflammatory syndrome is rare, especially in children, but with the increased use of ART is becoming more prevalent.     

Kaposi's sarcoma: a light and electron microscopic study

A patient with diabetes mellitus who developed the typical classic lesions of Kaposi's sarcoma is described. Our patient presented with a reddish-purple papulonodular lesion on the right foot of five months' duration. A skin biopsy specimen showed a proliferation of spindle cells forming numerous vascular slits and a diffuse extravasation of erythrocytes. The patient's sera was negative for human immunodeficiency virus (HIV) antibodies and cytomegalovirus (CMV) antibodies. Ultrastructural examination demonstrated fibroblast-like spindle cells phagocytosing and digesting red blood cells to form vascular spaces. The patient died, due to gastrointestinal hemorrhage, and the autopsy revealed an extensive visceral involvement of Kaposi's sarcoma.     

Classic Kaposi's sarcoma: a review of 90 cases.

A series of 90 cases of classic Kaposi's sarcoma observed and treated over the last 25 years was examined retrospectively. Fifty-one were the localized and 39 the disseminated form. Outpatient surgery for the localized and vinblastine chemotherapy for the disseminated form are probably the best treatments. Radiotherapy may be a valid alternative.     

Intralesional human leukocyte interferon treatment alone or associated with IL-2 in non-AIDS related Kaposi's sarcoma.

Patients with classical European Kaposi's sarcoma were treated by intra- and peritumoral injections of human alpha leukocyte interferon (IFN) (12 cases) or, alternatively, with IFN and naturally synthesized IL-2 (8 cases). All the patients were HIV negative with tumors which had been present for at least six months. In each patient, one tumor received 1 ml (50,000 IU) IFN alone or alternatively associated with 1 ml IL-2 twice a week for 4-6 weeks; another nodule situated 10-12 cm away was considered as a control and remained uninjected. The clinical follow-up revealed that, in the same patient in the same anatomical area, the treated nodule was cured in all the investigated cases; the untreated one was not. These data strongly suggest that IFN is the factor responsible for the involution and final cure of these Kaposi tumors treated by perilesional inoculations. Association with IL-2 (and certainly also other interleukins) increases the beneficial clinical activation of the tumor involution. Histological examination showed that important histopathological changes occur in the treated nodules: complete disappearance of the Kaposi's aspect, fibrosclerous modifications progressively replacing the fibroblasts characteristic of Kaposi's sarcoma, abundant infiltrations of leukocytes, especially lymphocytes and necrotic patches, often with hemorrhagic centers. IL-2 association seems to especially induce this last type of histological phenomenon.     

Radiotherapy of classic and human immunodeficiency virus-related Kaposi's sarcoma: results in 1482 lesions

Background The lesions of the various forms of Kaposi's sarcoma (KS), which are relatively radiosensitive, have been treated with different modalities of radiotherapy, with heterogeneous aims and results. Objective To verify the effectiveness and safety of radiotherapy on a large number of lesions endowed (classic KS) with a prolonged follow‐up. Methods A retrospective study was done on 711 lesions of classic KS and 771 lesions of human immunodeficiency virus (HIV)‐related KS, treated with traditional X‐ray therapy. Results In classic KS, a cure rate of 98.7% resulted after 13.5 years from the end of radiotherapy. In three lesions (0.42%) in the same patient, an acute radiodermatitis occurred after traumatic action. In HIV‐related KS, a complete remission was obtained in 91.43% of the lesions, partial remission in 6.74% and non‐response in 0.51% at 1 to 46 months from the end of radiotherapy. Conclusion Radiotherapy showed to be a safe and effective method, with relevant importance in the therapeutic strategy of KS.     

Detection of human herpesvirus 8 in Korean Kaposi's sarcoma cases by polymerase chain reaction and in situ polymerase chain reaction.

Several infectious agents, including herpesvirus-like particles, had been suggested as possible candidates for the development of Kaposi's sarcoma (KS), and a new herpesvirus, human herpesvirus 8 (HHV-8), was recently identified in the vast majority of KS lesions, irrespective of their association with human immunodeficiency virus (HIV) infection. However, the etiologic role of HHV-8 in KS remains controversial. We undertook this study to screen for and localize the presence of HHV-8 in KS in Korea. A total of 46 paraffin-embedded specimens were studied, including KS, hemangioproliferative disorders, and 10 non-KS lesions from HIV-positive patients. We performed nested polymerase chain reaction (PCR) and in situ PCR with HHV-8 specific primers. HHV-8 DNA sequences were detected in 8 of 11 KS specimens. All specimens of hemangioproliferative disorders, non-KS lesions from HIV-positive patients, and other skin samples were negative for HHV-8. When sequencing PCR products, the sequences were almost identical with the prototypic sequence for HHV-8. In PCR-positive tissues, in situ PCR staining of HHV-8 localized to nuclei of endothelial cells and perivascular spindle-shaped tumor cells. The results of this study suggest that HHV-8 is not widespread and has a certain causative role in the development of KS. Further studies, including serological and animal studies, will be helpful to appreciate an epidermiological link and pathogenetic mechanism between HHV-8 and KS.     

Radiotherapy in the management of classic Kaposi's sarcoma: A single institution experience from Northeast Turkey

Kaposi sarcoma is a rare adult neoplasm and there has been no certain consensus on standard treatment, either local or systemic. Radiotherapy is an effective, suitable treatment modality. Between 1996 and 2016, patients who were diagnosed with Kaposi sarcoma and referred to our clinic for radiotherapy were included in this retrospective study. Ninety‐two patients were examined in total and it was diagnosed that all the patients had non‐HIV associated Kaposi's sarcoma. There were 36 (39%) females and 56 (61%) males and female to male ratio was 2/3. Median age at presentation was 72 (30–93) years. Sixty‐eight patients (77%) were treated with 8 Gy (1 fraction), 15 patients (16%) were treated with 20 Gy (2 Gy/fraction), four patients (4%) were treated 25 Gy (2.5 Gy/fraction), and five patients (6%) were treated 30 Gy (3 Gy/fraction). The median follow‐up time was 72 (5–192) months. The complete response at 5 years was 91.6% with >20 Gy and 89.6% with 8 Gy. Radiotherapy is an effective, suitable treatment modality of Classic Kaposi sarcoma and usually, radiotherapy is well tolerated with minimal side effects.     

Recurrent Classical Type of Kaposi's Sarcoma Treated by Interferon-alpha

Kaposi''s sarcoma (KS) is a lympho-angioproliferative disease, with four variants; one of which is classical KS (CKS). Classical KS is clinically characterized by single or multiple pea-sized bluish-red macules on the distal portions of the lower extremities. A 60-year-old man presented with an asymptomatic, solitary patch on the left sole. He had been successfully treated for the classical type of Kaposi sarcoma on the right sole with interferon-alpha 3 years before. The patient was treated with six million units of interferon-alpha three times per week for 6 months. The lesion showed complete resolution and there has been no report of recurrence for 1 year after treatment.     

A case of classical Kaposi's sarcoma of the penis showing a good response to high-energy pulsed carbon dioxide laser therapy.

Isolated Kaposi's sarcoma (KS) of the glans penis is rare; only 37 cases are reported in the literature. Treatment of KS consists mostly of old modalities, some old methods in updated improved versions, and some new and experimental therapies. We report a case of isolated classical Kaposi's sarcoma involving multiple sites of the penis in a 54-year-old man who was treated with high-energy pulsed carbon dioxide (CO2) laser therapy, resulting in complete resolution of the lesions and no evidence of recurrence.     

Kaposi's Sarcoma Associated with Lung Cancer and Immunosuppression

We report a 65-year-old Japanese woman with Kaposi's sarcoma (KS). The eruption first occurred on the legs while she was admitted for treatment of poorly differentiated lung cancer. Approximately eight months after the evolution, cutaneous tumors rapidly spread to the forearms, trunk, and pharynx. At that time, the patient had received systemic corticosteroid (10–40 mg/day of prednisolone) for about three months to reduce pulmonary inflammation. The laboratory data showed anemia, lymphopenia, hypogammaglobulinemia, and a decreased T cell count, although the serological test for HIV infection was negative. The patient was treated with radiation (X-ray for KS of pharynx and electron beam for KS of lower legs) and local intralesional injection of vinblastine. Although both therapies were very effective and well tolerated, she died of bacterial pneumonia and sepsis. Autopsy revealed KS tumors, unknown before death, in both lungs, the esophagus, and the stomach. The left lung cancer had disseminated and metastasized to the right lung, pleura, mediastinum, and abdominal cavity. It is suspected that chronic respiratory distress and systemic use of corticosteroids might have induced the rapid extension of KS.     

Intralesional vincristine as first‐line therapy for nodular lesions in classic Kaposi sarcoma: a prospective study in 151 patients

Background Classic Kaposi sarcoma is a rare angioproliferative neoplasm with varying biological behaviour. Depending on the clinical stage, local or systemic therapy can be used. Vincristine has proven to be effective as systemic chemotherapy and in very few reports as intralesional treatment. Objectives Our aim was to determine the efficacy and safety of intralesional vincristine in the treatment of classic Kaposi sarcoma nodular lesions. Methods We conducted a prospective, open‐label, single‐centre clinical trial in 151 patients with stage IB classic Kaposi sarcoma. Vincristine was injected in a single nodule (0·3–0·8 mm) on the lower limb. Another similar lesion on the same limb, at a distance of ≥ 10 cm, or on the contralateral limb, was kept under clinical observation as control. Adverse effects were evaluated after 1 week, and efficacy after 4 and 12 weeks. Results One hundred and fifty‐one patients were enrolled. At final evaluation, 115 patients presented complete response (76·1%), 28 had partial response (18·5%), six had improvement (4%), one had stable disease (0·7%) and only one patient had tumour progression (0·7%). Therefore the total response rate was 98·7% (149 patients). Therapy was generally well tolerated. The most frequently registered adverse events, observed in 21 patients (13·9%), were erythema and itching. Conclusions Intralesional vincristine is an effective and safe treatment for nodular lesions in classic Kaposi sarcoma and can be recommended as first‐line therapy in initial stages and as support therapy in advanced stages.     

Classic Kaposi's sarcoma: A review of 156 cases

Background

Kaposi's sarcoma (KS) is a reactive, multifocal, multicentric, angiogenic neoplastic proliferation that is thought to originate from endothelial cells that are infected with human herpesvirus-8 (HHV-8). This report examines a cohort of patients with classic Kaposi's sarcoma (KS) evaluated at the national institute of oncology over the 13-year period.