Kaposi sarcoma (KS) is a lymphoangioproliferative condition linked to human herpesvirus-8. KS presents in four clinical variants: classic, iatrogenic, endemic, and AIDSrelated. The classic type has a chronic course and primarily affects people of Eastern European Jewish or Mediterranean heritage, with a higher incidence in males." Approximately 70% of patients respond partially or satisfactorily to treatment, 20% experience recurrence, and 10% show progression despite treatment.? Furthermore, there have been documented cases of self-regression in the classic type of KS.2
Sarcoma, Kaposi ; Iatrogenic Disease

Kaposi Sarcoma (KS) is a vascular tumor commonly associated with HIV/AIDS. There is unusual presentation of KS in a non-HIV patient, initially diagnosed as small vessel vasculitis. Early recognition and accurate diagnosis are important for the patient’s best management.

A 68-year-old male presented with spontaneous bluish-black patches on his extremities, swelling, pain, and bullae on his toes. Initial workup, including negative ANA and ANCA markers, pointed to small vessel vasculitis, with autoimmune, hematologic, and occlusive diseases considered. Peripheral vascular occlusion was ruled out, and a biopsy showed granulomatous vasculitis. Despite corticosteroid treatment, the lesions worsened. Five months later, the patient developed violaceous papules, plaques, and nodules. A second biopsy confirmed Kaposi Sarcoma (KS) with positive CD34 and HHV-8 stains. Restaining the initial biopsy also revealed KS. Treatment with Doxorubicin was initiated, but the disease progressed, affecting the gastrointestinal system. The patient’s condition deteriorated, and he died from complications of KS.

This case underscores the importance of considering Kaposi Sarcoma in HIV-negative patients with vascular lesions. The initial diagnosis of vasculitis, confirmed by granulomatous changes, delayed the KS diagnosis. Restaining the first biopsy later confirmed the presence of KS from the onset. The extensive skin and gastrointestinal involvement made management with Doxorubicin difficult, leading to a poor outcome.

Kaposi sarcoma (KS) is an angioproliferative tumor affecting the blood and lymphatic vessels. The human herpesvirus 8 (HHV8) is directly implicated in the development of the disease. Non-AIDS kaposi sarcoma is a rare clinical type of KS and must be distinguished from AIDS-associated KS as it differs in clinical presentation, course, prognosis, and management.

A 73-year old male, Filipino, rice farmer, from Quezon Province, Philippines, presented with a progressive 10-year history of violaceous to hyperpigmented plaques and nodules on all extremities, with associated pruritus and difficulty in performing fine motor tasks. Histopathology showed proliferation of vascular channels and immunohistochemical stains were positive for CD31, CD34, and human herpesvirus 8 (HHV8), consistent with KS. Work-up revealed non-reactive HIV serology with an adequate CD4 count and computed tomography (CT) scan of the chest and abdomen were unremarkable. The case was classified as non-AIDS KS. He was then referred to oncology for chemotherapy with paclitaxel.

This case highlights that KS can occur in non-endemic areas and in immunocompetent individuals. Non-AIDS KS must be differentiated from other types of KS since non-AIDS KS is less aggressive, limited to the skin, and is highly responsive to therapy. It is therefore crucial to correlate clinical and histopathologic findings, utilizing immunohistochemical stains, as histology of KS can mimic benign vascular tumors, and it is crucial to achieve an early and accurate diagnosis. There are currently no treatment guidelines for KS and management aims to decrease morbidity and improve a patient’s quality of life.

Child ; Humans ; Circulating Tumor DNA ; Lymphangioma ; Family ; Hemangioendothelioma ; Sarcoma, Kaposi

Objective: To investigate the clinical and pathologic features, diagnosis and differential diagnosis of congenital hemangioma (CH). Methods: Forty cases of CH were diagnosed from January 2017 to December 2020 in Henan Provincial People's Hospital. The clinical and pathological and immunohistochemical data were analyzed, with review of literature. Results: There were 24 male and 16 female patients. The lesions were located in the head, neck (11 cases), limbs (14 cases), and trunk (15 cases). The clinical manifestations were congenital painless plaques or masses, the larger ones protruded on the skin surface, mostly dusky purple or bright red, with surrounding white halos. Under low magnification, the tumor was lobular and well demarcated, composed of neo-microvascular lumen of different sizes. The vascular endothelial cells were cuboidal or hobnail in appearance, forming stellar drainage vessels within the lobules. Extra-medullary hematopoiesis was seen in one case of rapidly involuting CH; there were different number of tortuous and dilated vascular lumen between the lobular structures, and some non-involuting CH cases were vascular malformations, which were devoid of lobulated structures. Immunohistochemistry showed that endothelial cells were strongly positive for CD31, CD34 and ERG, while D2-40 and GLUT-1 were negative. Conclusions: CH is a benign congenital vascular tumor with characteristic lobulated growth and abnormal blood vessels in the stroma. Pathological diagnosis often needs to be differentiated from infantile hemangioma, pyogenic granuloma, kaposiform hemangioendothelioma and vascular malformation.
Endothelial Cells/pathology* ; Female ; Hemangioendothelioma/pathology* ; Hemangioma/pathology* ; Humans ; Kasabach-Merritt Syndrome/pathology* ; Male ; Sarcoma, Kaposi/pathology* ; Skin Neoplasms/pathology*

Introduction: Acquired immunodeficiency syndrome-Kaposi sarcoma (AIDS-KS) has unique clinical characteristics, often dis- seminated on presentation, a rapidly progressive course, and often fatal outcome. Describing the epidemiology and clinical characteristics of AIDS-KS in the Philippines may lead to early recognition, diagnosis, and management of this condition, which are the keys to preventing significant complications. Case Series: AIDS-KS in 11 Filipino MSM patients with a mean age of 36.55 years (SD 11.54) was described. Violaceous plaques and nodules were present for an average of 5.1 months prior to diagnosis confirmed by biopsy. Histopathologic findings from all pa- tients were consistent with KS. The median CD4+ count of patients was 44 cells/microliter (range, 4 to 181). Six patients presented with opportunistic infections (OI)/AIDS-related conditions (ARC). The most common OIs observed were pulmonary tuberculosis, oropharyngeal candidiasis, and Pneumocystis jiroveci pneumonia. Nine patients improved with highly active antiretroviral therapy (HAART). One patient required modification on his HAART regimen, which was shifted to 2 NRTI and ritonavir-boosted protease inhibitor, and one patient died due to AIDS-related complications. Conclusion This series of 11 cases of AIDS-KS showed similar demographic, clinical and histopathologic characteristics to pre- viously published studies. Findings suggest the need for earlier recognition and diagnosis. While HAART afforded clinical improve- ment in a majority of patients, other treatment options such as chemotherapy should be considered for appropriate patients.
Sarcoma, Kaposi ; Antiretroviral Therapy, Highly Active

OBJECTIVE: To study the clinical features, treatment, and prognosis of neonates with Kasabach-Merritt syndrome (KMS), and to provide a reference for optimizing the diagnosis and treatment of this disease. METHODS: A retrospective analysis was performed for the clinical and follow-up data of 16 neonates with KMS who were admitted to the Anhui Children's Hospital, Anhui Medical University, from January 2016 to December 2020. RESULTS: Of the 16 neonates, there were 13 boys (81%) and 3 girls (19%), with an age of 1 hour to 10 days on admission. Among these neonates, 13 (81%) had cutaneous hemangioma (2 in the head and face, 5 in the trunk, and 6 in the extremities) and 3 (19%) had liver hemangioma. The main clinical manifestations of bleeding tendency and scattered petechiae and ecchymosis were observed in 10 neonates (62%). All the 16 neonates had varying degrees of thrombocytopenia and coagulation disorders. They all received glucocorticoid treatment after admission and 7 (44%) of them had response, among whom 4 experienced recurrence. Among the neonates with no response to glucocorticoid treatment, 3 received sirolimus treatment, among whom 1 had the tumor volume reduced by 58.8% after 4 weeks of treatment, with platelet count and coagulation function returning to normal, while 2 had no significant reduction in tumor volume or significant increase in platelet count and achieved a tumor volume reduced by (43.7±0.4)% after 4 weeks of combined treatment with bleomycin arterial embolization, with platelet count and coagulation function returning to normal. After 4 weeks of bleomycin arterial embolization alone for 4 neonates, tumor volume was reduced by (52.0±3.4)%, and platelet count and coagulation function returned to normal. Blunt and sharp dissection was performed for 2 neonates. The tumor was removed completely during surgery in the 2 neonates, with no infection or recurrence after surgery, and platelet count and coagulation function returned to normal. The postoperative pathological examination showed Kaposiform hemangioendothelioma in 1 out of the 2 neonates. CONCLUSIONS KMS has characteristic clinical manifestations, histopathological features, and laboratory examination results. The KMS neonates who are not sensitive to glucocorticoids can achieve a good curative effect through arterial embolization and sirolimus treatment.
Child ; Female ; Hemangioendothelioma ; Humans ; Infant, Newborn ; Kasabach-Merritt Syndrome/therapy* ; Male ; Neoplasm Recurrence, Local ; Retrospective Studies ; Sarcoma, Kaposi

Kaposi's sarcoma (KS) is an unusual vascular tumor associated with human herpesvirus-8 (HHV-8) infection, which is common in immunosuppressors. Although extremely rare, iatrogenic (drug-related) KS can occur in human immunodeficiency virus (HIV)-negative patients under immunosuppressive therapy. We report a 64-year-old male diagnosed with ulcerative colitis for 1 year. He was treated with methylprednisolone because of an acute severe disease flare. He presented with several popular violet lesions on the body 4 months after steroid therapy. Histological examination of skin biopsies showed Kaposi's sarcoma associated with HHV-8. The skin lesions regressed after steroid withdrawal and chemotherapy. Two key words "Kaposi's sarcoma" and "inflammatory bowel disease" were searched in Wanfang data and CNKI, but no relevant articles were found. Thirty-eight articles in English were retrieved on PubMed with the key words of ("ulcerative colitis" OR "Crohn's disease" OR "inflammatory bowel disease") AND (Kaposi sarcoma). Twenty-five cases of Kaposi's sarcoma related to inflammatory bowel disease (IBD) were reported. Including this case, the majority of 26 Kaposi's sarcoma related IBD patients were male (80.8%, 21/26). The average age was (51.1 ± 16.4) years. Twenty cases were ulcerative colitis and 6 were Crohn's disease. All the patients received immunomodulatory therapy, including glucocorticoid, azathioprine/mercaptopurine, methotrexate, cyclosporin and anti tumor necrosis factor α antibody. Thirteen cases were positive for HHV-8. There were 18 cases involving the distal ileum and colorectum only, 3 cases involving skin only, and 5 cases involving both skin and colorectum at the same time. Overall, the prognosis was good. Three patients only stopped immunosuppressive therapy, 1 received radiotherapy, 1 received chemotherapy, and 20 received surgery. Kaposi's sarcoma could be seen in IBD patients with immunomodulatory therapy. It is very important to distinguish from the skin lesions related to IBD or drug treatment. The adverse reactions of immunomodulatory therapy should not be ignored. In addition, attention should be paid to the cooperation of multi-disciplinary team, which can diagnose and treat rare cases earlier and more accurately.
Adult ; Aged ; Colitis, Ulcerative ; Crohn Disease ; Herpesvirus 8, Human ; Humans ; Immunosuppression Therapy ; Male ; Middle Aged ; Sarcoma, Kaposi

Acquired Immunodeficiency Syndrome ; Biopsy, Fine-Needle ; China ; HIV ; Humans ; Sarcoma, Kaposi

Intravascular papillary endothelial hyperplasia or Masson's hemangioma is a rare vascular tumor. The reactive proliferation of endothelial cells in this disease mimics other benign or malignant vascular proliferation such as angiosarcoma or Kaposi's sarcoma. It is important to make an accurate distinction to avoid confusion with these malignant tumors. This would facilitate a proper diagnosis, which is essential so that the patient is not subjected to unnecessarily aggressive or inappropriate treatment.
Diagnosis ; Endothelial Cells ; Hemangioma ; Hemangiosarcoma ; Humans ; Hyperplasia ; Sarcoma, Kaposi