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Subcutaneous panniculitis-like T-cell lymphoma is a rare subtype of cutaneous T-cell lymphomas and represents less than 1% of non-Hodgkin's lymphomas. Currently, the diagnosis is based on clinical and histological findings although clinical features may be nonspecific. Often, it is localised to subcutaneous tissue without lymph node involvement.

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Background: Mesenteric fibromatosis is a benign locally-aggressive mesenchymal neoplasm that lacks the potential for metastasis. It is related to Gardner's Syndrome, previous trauma, abdominal surgery, and prolonged intake of oestrogen. Differentially diagnosing this from similar tumours is crucial in order for establishing the appropriate treatment and only immunohistochemical features can be used for a definitive diagnosis.

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Article Synopsis
  • This report talks about a patient with a rare type of cancer called subcutaneous panniculitis-like T-cell lymphoma (SPTCL) that was linked to a gene mutation known as HAVCR2.
  • The patient had some serious issues related to his immune system and later had to get a special treatment called stem cell transplantation.
  • Scientists found that the problems with his immune system, due to the gene mutation, made him more likely to get this type of lymphoma again, and they suggest a possible test to help find similar cases.
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Background: Panniculitis-like T-cell lymphoma is an uncommon type of non-Hodgkin lymphoma, occurring usually in the form of nodules within the subcutaneous fat tissue of the extremities or trunk. In the literature, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is described as a distinct type of T-cell lymphoma with a variable clinical behavior, depending on molecular phenotype of T-cell receptor (TCR) and on the presence or absence of hemophagocytic syndrome.

Case Presentation: We present a bioptic and autoptic case of a 65-years old Caucasian man with panniculitic T-cell lymphoma with morphological and immunohistochemical features of SPTCL, limited to the retroperitoneal and mesenteric mass, i.

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[Imaging of abdominal tumors in childhood and adolescence : Part II: relevant intra-abdominal and retroperitoneal tumor entities].

Radiologe

July 2018

Sektion Pädiatrische Radiologie, Institut für Diagnostische und Interventionelle Radiologie, Universitätsklinikum Jena, Am Klinikum 1, 07747, Jena, Deutschland.

Abdominal and pelvic tumors in children and adolescents can be manifested intra-abdominally and also often in the retroperitoneum. The most important pediatric primary retroperitoneal space-occupying lesions are nephroblastoma (Wilms' tumor) and neuroblastoma, whereby imaging plays a decisive role in the diagnostics and differentiation of the two entities. Benign tumors of the mesentery and gastrointestinal tract occur more frequently in children than malignant lesions.

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