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*

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

Diagnosis, Differential ; Esophageal Neoplasms ; pathology ; surgery ; Granuloma, Pyogenic ; pathology ; surgery ; Hemangioma, Capillary ; pathology ; Hemangiosarcoma ; pathology ; Humans ; Male ; Middle Aged ; Sarcoma, Kaposi ; pathology

Female patients, 41 years old, with the left nasal cavity bleeding intermittently and left nasal congestion 20 days as the chief complaint to the hospital. Physical examinationindicated dark red mass was at the front-end of left nasal cavity, which has not smooth surface with blood vessels and hemorrhagic secretions, and back up to the middle turbinate. Sinus enhancement 3D-CT showed soft tissue density can be found in the left nasal cavity,Scan CT value is 37-47 HU, and enhanced and delay is about 69-78 HU. Nasal septum,middle turbinate and inferior turbinate bonewas visible damage. The pathologic biopsy of left nasal cavity lesions results conform to the Kaposiform hemangioendothelioma.
Adult ; Female ; Hemangioendothelioma ; pathology ; Humans ; Kasabach-Merritt Syndrome ; pathology ; Nasal Septum ; Paranasal Sinuses ; Sarcoma, Kaposi ; pathology ; Tomography, X-Ray Computed ; Turbinates ; pathology

OBJECTIVETo study the relationship between Kaposi's sarcoma (KS) and human herpes virus 8 (HHV8; Kaposi's sarcoma-associated herpes virus), and to develop an in situ hybridization (ISH) technique effective for clinical pathological diagnosis.

METHODSPolymerase chain reaction (PCR) technique was used to synthesize a digoxigenin-labeled single stranded DNA probe specific for HHV8 open reading frame 72 cyclin D homolog gene encoded mRNA as the probe accompanying with a tyramide signal amplification system (TSA) for ISH assay. Totally 20 formalin-fixed and paraffin-embedded samples from 14 cases of KS from Danish patients were collected for HHV8 detection. In order to compare the result obtained, all of these cases were checked simultaneously using PCR technique.

RESULTSHHV8 was detected in 10 of 14 (71%) KS cases, of which 8 cases were positive for HHV8 by both ISH and PCR. In ISH, HHV8 hybridization signal was seen as a dot or patch located in the nuclei of both the neoplastic spindle cells and the endothelial cells. HHV8 was found in lesions in all the stages of KS including early patch, plaque and late nodular or tumor lesions, as well as in the primary and metastatic lesions. All the control cases showed a relevant positive or negative results.

CONCLUSIONSThe results further confirmed that there is a strong association between Kaposi's sarcoma and HHV8. The ISH technique with single stranded probe and TSA would be helpful in clinical pathological diagnosis for HHV8 infected diseases, such as KS, primary effusion lymphoma and multicentric Castleman's disease.

Adolescent ; Adult ; Female ; Herpesvirus 8, Human ; isolation & purification ; Humans ; In Situ Hybridization ; methods ; Male ; Middle Aged ; Polymerase Chain Reaction ; methods ; Sarcoma, Kaposi ; pathology ; virology ; Tyramine

Acquired Immunodeficiency Syndrome ; complications ; pathology ; Antigens, CD34 ; metabolism ; Gastrectomy ; methods ; Humans ; Male ; Middle Aged ; Sarcoma, Kaposi ; metabolism ; pathology ; surgery ; virology ; Stomach ; pathology ; Stomach Neoplasms ; metabolism ; pathology ; surgery ; virology ; Vimentin ; metabolism

Actins ; metabolism ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; pathology ; Humans ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Adult ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Frontal Bone ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangioma, Cavernous ; pathology ; Humans ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; metabolism ; pathology ; Skull Neoplasms ; metabolism ; pathology ; surgery

Abdominal Cavity ; Adult ; Anemia, Aplastic ; therapy ; Antigens, CD34 ; metabolism ; Carcinoma ; metabolism ; pathology ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Ki-67 Antigen ; metabolism ; Lymph Nodes ; pathology ; Male ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; etiology ; metabolism ; pathology ; Viral Proteins ; metabolism

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