Sildenafil citrate-aided radiotherapy for the treatment of Kaposi''s sarcoma of the penis

We report a case of classical Kaposi's sarcoma involving multiple sites of the penis in a 62-year-old male who was treated with sildenafil citrate-aided electron beam therapy, resulting in complete resolution of the lesions.     

Kaposi''s sarcoma: aetiopathogenesis, histology and clinical features

Kaposi's sarcoma (KS) represents today one of the most common skin cancers in transplanted Mediterranean subjects and, since the epidemic of human immunodeficiency virus/acquired immune deficiency syndrome, in young unmarried single men. The disease has been associated with the recent identified human herpesvirus (HHV)-8 or KS herpesvirus and its incidence in the general population shows a north to south gradient that parallels the HHV-8 increasing prevalence from Nordic countries to sub-Saharan regions. The identification of the aetiopathogenetic mechanisms (viral agents and immunodeficiency) involved in the pathogenesis of KS, are relevant for identifying susceptible subjects (HHV-8 seropositive subjects), monitoring the immune levels in iatrogenic immune suppressed patients, and developing new therapeutic approaches based on antiviral and immune modulators. Learning objective: This article should enable the reader: (i) to learn about the clinical and molecular aspects of KS in order to have a multidisciplinary approach to a tumour that shows unique features; (ii) to consider the role of viral agents and immunity; and (iii) to recognize properties of an opportunistic neoplasm. The identification of the HHV-8 role in KS pathogenesis should establish a relevant tool in the clinical management of KS patients.     

Iatrogenic Kaposi''s sarcoma and HCV infection

Iatrogenic Kaposi's sarcoma develops in patients undergoing immunosuppressive treatment and is considered to be induced by activation of latent HHV8. In most cases the first manifestation of Kaposi's sarcoma develops after 1 year from when the drug was first administered. In a recent study from Italy on HHV8 positivity in patients with Kaposi's sarcoma, it was found that 52% of the control group were positive (Masini C., et al. G Ital Dermatol Venereol 1999; 134: 315-320). For this reason we could expect a larger number of cases of iatrogenic Kaposi's sarcoma given the number of patients who undergo immunosuppressive treatment for one reason or another. Thus, we have to look to a contemporaneous presence of other factors that co-operate with the HHV8. We present a case of a 49-year-old woman, HHV8 and HCV positive, who develops a Kaposi's sarcoma after 9 months of steroid therapy (methylprednisolone 16 mg/die). The low dose of steroids prescribed to our patient and the fact that the first skin manifestation developed after a shorter period than average from the start of therapy do not explain the acute onset of an extensive Kaposi's sarcoma even taking into account the HHV8 positive status. Both HHV8 and HCV produce proteins, such as IL6 and IL8 which are able to control cell growth. It can be supposed that the contemporaneus presence of the two viruses created a sinergy for the onset of the Kaposi's sarcoma.     

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.     

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.     

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.     

Lymphangioma-like pattern and anaplastic evolution in a case of classic Kaposi's sarcoma

Background Usually, classic Kaposi's sarcoma (KS) is clinically characterized by maculae, plaques and nodules and histologically by a proliferation of irregularly-shaped blood vessels, spindle cells and extravasated erythrocytes. The course is indolent. Very rarely, cases have been reported in which malignant transformation occurred with subsequent visceral diffusion. Observations A case of classic KS in an 82-year-old man is reported. Clinically, the lesion appeared lo be a nodule which grew rapidly to become an exophytic tumour mass. Histopathologically, it was characterized at the beginning by a lymphangioma-like pattern with subsequent frank anaplasia. The diagnosis of KS was based on lymph node examination which showed typical features. The patient died after 1 year. Conclusions Taking into account these unusual patterns we emphasize their importance in the differential diagnosis of KS from other angiomatous and anaplastic lesions.     

Exclusive penile Kaposi''s sarcoma: report of an HIV-negative man successfully treated with radiotherapy

Kaposi's sarcoma (KS) is a rare neoplasm of endovascular cells with multifocal origin. The exact nature of the disease is not clear, but current data support the notion that KS is a vascular hyperplasia with a tight link to human herpesvirus 8 (HHV-8) infection. Classic KS occurs primarily on the lower extremities in elderly men living in the Mediterranean region. Penile involvement has rarely been reported in non-human immune deficiency virus (HIV) patients. Herein we present a 71-year-old HIV-negative man with isolated KS on the penis who was treated successfully with radiotherapy. A review of the literature is also presented.     

Epidemiological study of classic Kaposi's sarcoma: a retrospective review of 125 cases from Northern Israel

BACKGROUND: The morphology of Kaposi's sarcoma is clinically and histologically the same in all clinical forms of the disease. However, there is a difference in the clinical and biological behaviour of the different forms of the disease. The behaviour also differs among individuals with the same form. The factors involved in the initiation and prognosis of the disease are still unknown. The classical form is more common in middle-aged Jews of East European or Mediterranean origin, people of Italian and southern Greek origin. Classic Kaposi's Sarcoma is seen relatively more frequently in Israel than in many other countries. OBJECTIVE: The aim of this study was to examine risk factors that influence the development and course of the disease. METHODS: This retrospective study includes 125 patients with Kaposi's sarcoma, all diagnosed and followed in the Department of Dermatology at Rambam Medical Center in Haifa. RESULTS: The group included 85 (68%) men and 40 (32%) women. Fourteen subjects received corticosteroid therapy and three were kidney transplant recipients. Age at onset of the disease was 21-87 years, with a mean age of 67. A total of 121 patients (96.8%) were Jews and four (3.2%) were non-Jews. A majority (61.6%) were of East European origin. The number of new cases each year was constant in relation to the general population, except for two peaks, one in 1970 and another in 1986-89. The lower limbs were involved in most patients. Extracutaneous involvement was present in 18.4%. Of all the subjects, 28 (22.4%) had diabetes mellitus and 21 (16.8%) had a second primary malignancy. The malignancies were of lymphoreticular origin in 10 patients, four in the urinary bladder, three had carcinoma of the large bowel and one of the pancreas. CONCLUSION: Our study shows similar clinical findings to those described in other series. The relatively high frequency of carcinomas of the colon and urinary bladder was not reported elsewhere. We observed a consistent rate of new cases each year with two peaks in 1970 and 1986-1989, the cause of which deserves explanation. Of interest is the relative rise in the number of females with Kaposi's sarcoma. A relative high risk for developing Kaposi's sarcoma has been found among Jews of Ashkenazi origin compared to those of other ethnic groups. Israeli-born subjects presented a relatively more aggressive course of disease than others.     

Kaposi肉瘤p53蛋白的免疫光镜和电镜观察

应用免疫组化和免疫电镜方法研究了新疆Ｋａｐｏｓｉ肉瘤（ＫＳ）组织内ｐ５３蛋白的表达情况，结果表明１６例ＫＳ组织内均存在着ｐ５３蛋白的异常表达，而１０例皮肤纤维瘤仅有２例阳性，正常皮肤均为阴性。作者推测ｐ５３蛋白基因突变可能在ＫＳ发病机制上起一定作用     

包皮假性阿洪病1例

报告1例包皮假性阿洪病,患者男,33岁。包皮缩空环进行性加重2年,龟头淤血伴龟头麻木感2个月,体格检查见包皮前端有一完整环形缩空带。完约0.5cm,正常肤皮,质地略硬。组织病理检查见真皮胶原纤维轻度增生,诊断为假性阿洪病。可能是由于包皮嵌顿,局部长期摩擦引起。     

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.     

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.     

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肉瘤患者血清人类疱疹病毒8型的检测

目的：了解干扰素治疗Kaposi肉瘤近期疗效及其对血清HHV-8 DNA检测的影响。方法：主要应用干扰素治疗4例无明显内脏损害的新疆经典型Kaposi肉瘤患者，同时在治疗前后采用PCR法检测血清HHV-8 DNA特异性片段。结果：4例患者经干扰素治疗45d后，均有不同程度的近期疗效，主要表现在皮损色泽变暗、结节和斑块变软、变平，部分小结节消退，且均无新皮损发生。但淋巴水肿无明显改观。血清HHV-8 DNA特异性片段经PCR检测，治疗后全部转阴。结论：干扰素治疗可有效地清除Kaposi肉瘤患者血清中HHV-8感染，能缓解病情，防止Kaposi肉瘤多灶病状的发生。     

Initial lesions of HIV-related Kaposi's sarcoma — a histological,immunohistochemical, and ultrastructural study

Summary Kaposi's sarcoma (KS) in human immunodeficiency virus infection (HIV) has become a rather frequent manifestation of the previously rare disease with fatal outcome. Initial lesions of KS were studied by means of histopathology, immunohistology, and electron microscopy in order to define the earliest alterations. The histopathological changes of initial lesions were distinct, consisting of (1) discrete proliferation of capillary vessels, (2) dissection of collagen by proliferating spindle cells which formed slits, (3) atypical spindle cells arranged in an Indian file pattern, and (4) the lack of any inflammatory cellular infiltrate. Double staining with antibodies against vimentin and immunohistochemical markers for endothelial cells revealed that slits forming vimentin-positive spindle cells displayed laminin, factor VIII, and PAL-E. Atypical vimentinpositive spindle cells arranged in an Indian file pattern inconsistently expressed laminin and factor VIII, but not PAL-E. KS cells rarely stained with the lectin UEA I, not even in case of less advanced dedifferentiation. Electron microscopy showed gradual transformation between spindle cells forming slits and those having lost the ability to form incomplete vessel walls. The present findings support the view that KS develops from the endothelial cells of the blood vessels. The proliferation of atypical endothelial cells as early as in initial lesions and the lack of inflammation favors the primary neoplastic genesis of KS.Dedicated to Professor Dr. Dr. h. c. Otto Braun-Falco on the occasion of his 65th birthday     

Tinea of the glans penis: report of a case presenting as a crop of papules

A 27-year-old Japanese male student with tinea of the glans penis was described. The lesion on the glans penis was unique; it developed as a crop of papules.     

Nasal and nasal type CD56+ natural killer cell/T-cell lymphoma: a case with rapid progression to bone marrow involvement

We describe herein a case of CD56+ natural killer cell lymphoma (NKCL) that showed unusual clinical manifestations and a fulminant course of disease. A 70-year-old male patient presented with rapidly growing skin lesions and fever; a nasal lymphoma was subsequently detected and bone marrow involvement developed within a few weeks. Although the time relationship is not clear, bone marrow involvement could be dissemination from localized disease of nasal and nasal type NKCL. As seen in our case, the course can be excessively aggressive and fulminant even when it first appeared as a localized disease, and CD56 positivity, which is a specific characteristic of NKCL, may serve as a factor showing a poor prognosis for a malignant lymphoma.