OBJECTIVETo explore the clinicopathologic features of kaposiform hemangioendothelioma (KHE).

METHODSThe clinicopathologic data were studied in three cases of KHE and review the literatures.

RESULTSTwo cases were female and one was male. All cases occurred in infancy. Two tumor located in axillary chest wall and one in lumbar region. All of the three patients had Kasabach-Merritt syndrome. Histologically, the tumor was composed of spindle-shaped cells. in all cases nodular growth pattern was seen. Immunohistochemically, Neoplastic spindled cells expressed CD34 and CD31. Associated lymphangiomatosis was present in two cases. Two tumors were resected completely, one was resected partly. the follow-up period ranged from 6 months to 3 years, and all were alive.

CONCLUSIONSKaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that mainly occurred in early infancy. It is frequently complicated by Kasabach-Merritt syndrome, and it has features common to both capillary hemangioma and Kaposi sarcoma. The prognosis of KHE is determined by the size, location and the hemorrhage degree of vascular tumor. Better outcome might be achieved in patients with KHE of the skin and in the soft tissues under the skin. It appears that the main treated measure should be wide local excision.

Diagnosis, Differential ; Female ; Hemangioendothelioma ; complications ; pathology ; Humans ; Infant ; Male ; Purpura, Thrombocytopenic ; etiology ; Sarcoma, Kaposi ; complications ; pathology ; Skin Neoplasms ; complications ; pathology

Kaposi's sarcoma is a rare and slowly progressive disease that primarily affects the skin but has an associated visceral involvement. It can occur in the HIV-positive patients or patients treated with immunosuppressants. However, it is extremely rare in the patients receiving the treatment for inflammatory bowel disease. We used corticosteroid for the treatment of ulcerative colitis in 60-year-old woman. Then, Kaposis's sarcoma occured in the skin and colon of the patient. Since she was HIV-negative, we believed that it was developed from the condition of corticosteroid-induced immunosuppression. We present a case of skin and colonic Kaposi's sarcoma in a HIV-negative woman following treatment with corticosteroid for ulcerative colitis.
Aged ; Colitis, Ulcerative/*complications/drug therapy ; Colonic Neoplasms/*complications/diagnosis ; English Abstract ; Female ; Humans ; Sarcoma, Kaposi/*complications/diagnosis ; Skin Neoplasms/*complications/diagnosis

Kaposi sarcoma herpes virus (KSHV), also known as human herpesvirus-8, plays an important role in the pathogenesis of Kaposi sarcoma (KS), multicentric Castleman disease (MCD) of the plasma cell type, and primary effusion lymphoma. KSHV is rarely associated with the hemophagocytic syndrome (HPS), but when it does occur, it most occurs in immunocompromised patients. We report herein an unusual case of KSHV-associated HPS in an immunocompetent patient. A previously healthy 62-yr-old male was referred for evaluation of leukocytopenia and multiple lymphadenopathies. After a lymph node biopsy, he was diagnosed with MCD of the plasma cell type. KSHV DNA was detected in the lymph node tissue by polymerase chain reaction. Following a short-term response of the leukocytopenia to prednisolone, mental change, left side weakness, fever, thrombocytopenia, hemolytic anemia, and renal failure developed. Despite intravenous immunoglobulin therapy and plasmapheresis, he expired. The lymph nodes were infiltrated by hemophagocytic histiocytes in the sinuses. Pulmonary nodules and gastric erosions were shown to be KS. KSHV DNA was detected in the stomach, lung, and liver. This is the first case of multiple KSHV associated diseases including MCD and KS with KSHV-associated hemophagocytic syndrome in an HIV-negative, non-transplant, immunocompetent patient.
Autopsy ; Giant Lymph Node Hyperplasia/complications/*diagnosis/pathology ; HIV Seronegativity ; Herpesviridae Infections/*diagnosis ; Herpesvirus 8, Human/*isolation & purification ; Humans ; Immunocompetence ; Lymphohistiocytosis, Hemophagocytic/*diagnosis/etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Polymerase Chain Reaction ; Sarcoma, Kaposi/complications/*diagnosis

Kaposi sarcoma (KS) is a vascular neoplasm, which is fairly prevalent in acquired immunodeficiency syndrome (AIDS) patients. Mucocutaneous and lymph node involvements are characteristic features of KS in AIDS patients. The involvement of gastrointestinal tract occurs in 40% of KS patients and leads to significant morbidity and mortality. In the highly active antiretroviral therapy (HAART) era, the rate of AIDS related KS has fallen with control of human immunodeficiency virus (HIV) viremia. However, it is still recognized as the primary AIDS-defining illness, and the proportion of AIDS diagnoses made due to KS ranged from 4.1% to 7.5%. In Korea, AIDS-related KS has been report in low rate incidence. Its gastrointestinal involvements are rarely reported. To date, five cases have been recorded in Korea. Herein, we present an additional case of gastrointestinal KS as the AIDS-defining illness and review of the Korean medical literature.
Acquired Immunodeficiency Syndrome/complications/*diagnosis ; Anti-HIV Agents/therapeutic use ; Endoscopy, Digestive System ; HIV Infections/complications/diagnosis/drug therapy ; Humans ; Male ; Middle Aged ; Republic of Korea ; Sarcoma, Kaposi/*diagnosis/etiology/pathology ; Tomography, X-Ray Computed

OBJECTIVETo improve the knowledge of Kaposi sarcoma and the relationship between Kaposi sarcoma and human immunodeficiency virus (HIV) infection, and to improve the ability to diagnose and treat Kaposi sarcoma and acquired immune deficiency syndrome (AIDS).

METHODSSymptoms, signs and results of 121 patients encountered in the department of otorhinolaryngology head and neck surgery in Tanzania, who was diagnosed as Kaposi sarcoma actually with HIV infection and AIDS, were retrospectively analyzed in this study.

RESULTSThere were 46 males and 75 females with age ranged from 5 to 65 years, medium 30 year. The mucous membranes and skin lesions was the most commonly seen clinical manifestation in 121 cases, these lesions appeared as raised blotches or lumps that might be purple, brown, or red, early stages typical lesions began as flat or slightly raised colored spots. Among the cases reported here, 25 patients (20.66%) showed progressive nose blockage and nose bleeding and the purple-red new-grows were found in the nose of these patients. Fifteen patients (12.40%) had flat or slightly raised colored spots in their mucous membrane of mouth (palate or tongue), and in other 7 patients, purple small lumps were found in the gums of the patients. There were same lesions in their pharynx in 9 cases. In 10 patients (8.26%), Kaposi sarcoma was found in tonsil looked like tonsillitis with enlarged tonsils by two to three degree. Twelve patients (9.92%) had masses in the neck with no pain. Thirty-five patients (28.92%)had lesions of purple black nodules, including 10 patients who had the same lesions with ulcer formation in the nodules. All patients had been followed-up for at least two-years. Eighty-five patients passed away in one year, survival rate of one year was 21.48% (26/121), only 12 patients survived from the disease over two years, two years' survival rate was 9.92% (12/121).

CONCLUSIONSKaposi sarcoma is the characteristic disease for AIDS, mainly found on the membranes and skin. These lesions appears as raised blotches or lumps that may be purple, brown, or red, early stages typical lesions begin as flat or slightly raised colored spots. Patients who had kaposi sarcoma often died in a short-time without treatment.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; HIV Infections ; complications ; diagnosis ; Head ; pathology ; Humans ; Male ; Middle Aged ; Neck ; pathology ; Retrospective Studies ; Sarcoma, Kaposi ; complications ; diagnosis ; Survival Rate ; Tanzania ; Young Adult