We describe four patients with lymphoproliferative lesions confined to the skin for several years. They presented with recurrent necrotic papulovesicles of the face. Latent Epstein-Barr virus (EBV) infection was detected in the lymphoid cells from the skin lesions by in situ hybridization. The disease in three patients progressed to T-cell lymphoma. We believe that these patients represent a subset of peripheral T-cell lymphoma with a tendency to localize in the skin.
"Hydroa vacciniforme-like lymphoma (HVLL) has also been called angiocentric cutaneous T-cell lymphoma of childhood , and hydroa-like cutaneous T-cell lymphoma [4-6]. Most reports come from Asian area, including Japan, China and Korea . "
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Hydroa vacciniforme-like lymphoma (HVL) is a rare type of Epstein-Barr virus (EBV)-positive lymphoma of cytotoxic T-cell or natural killer cell origin that mainly affect children, characterized by a vesicopapular skin eruption that clinically resemble hydroa vacciniforme (HV). In current study, we report an adult patient with the tumor. The patient presented similar morphologic, immunophenotypic and genotypic changes of the disease with that occurred in children, whereas clinically, he showed a prolonged clinical course without hepatosplenomegaly or generalized lymphadenopathy. Whether there are some differences in biologic behavior between children and adults still remains unknown and it is necessary to collect more data to observe and to investigate in the future.
[Show abstract][Hide abstract] ABSTRACT: T and NK cell proliferative diseases associated with acute and chronic Epstein–Barr virus (EBV) infection are uncommon and raise several clinical issues regarding diagnostic criteria and terminology. This is a summary report of the consensus meeting held in the 4th Asian Hematopathology Workshop. The umbrella term “EBV-positive T/NK lymphoproliferative disease in childhood-type” covers the entire spectrum of EBV-associated lesions in childhood, ranging from reactive to neoplastic processes. Systemic T/NK cell lymphoproliferative disease (LPD) of childhood type is defined as a fulminant disease associated with the proliferation of polyclonal, oligoclonal, or monoclonal T or NK cells and includes aggressive NK cell-associated leukemia in children. Chronic active EBV disease-type T/NK cell LPD is divided into three groups—polymorphic/polyclonal, polymorphic/monoclonal, and monomorphic/monoclonal—based on the histology and clonality of T or NK cells. A monoclonal EBV-positive T/NK cell type of proliferation with the clinical features of chronic active EBV disease rather than the fulminant course of systemic EBV-positive T/NK cell LPD of childhood is considered “chronic active EBV disease-type T/NK cell LPD”. Hydroa vacciniforme (HV)-like T cell LPD is defined as a spectrum of EBV-infected T cell proliferative diseases with homing to the skin and is further classified into a classic type, a severe type, and malignant lymphoma. Criteria to define each category of HV-like T cell LPD remain to be clarified.
[Show abstract][Hide abstract] ABSTRACT: Solid ovarian tumors are uncommon in the pediatric population, but when they occur, they are a major source of anxiety for the patient and her family. The pediatric surgeon will be relied on to diagnose these tumors because they usually present as abdominal pain with a mass. The diagnostic evaluation consists of a carefully obtained history and physical examination, ultrasound examination, serum assay for tumor markers, and further radiographic evaluation as indicated. Two thirds of malignant tumors in children are germ cell tumors, and most of these are dysgerminomas or endodermal sinus tumors. A multimodal, team-oriented approach to therapy is crucial. Reproductive organ-sparing surgery with salpingo-oophorectomy, ascites sampling, nodule biopsy, omentectomy, and contralateral ovarian biopsy as indicated, may be curative for stage I tumors; more advanced or highly aggressive tumors should be treated with cytoreduction surgery and will require platinum-based chemotherapy. Postinduction surgery is indicated for progressive or recurrent disease. One third of tumors are physiologically active stromal tumors that often become apparent because of hormonal effects. Epithelial tumors, common in the adult, are uncommon in children and are of mild to moderate malignant potential. Other miscellaneous tumors and benign lesions are less common.
Seminars in Pediatric Surgery 03/1998; 7(1):29-34. DOI:10.1016/S1055-8586(98)70003-4 · 2.22 Impact Factor
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