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  • 1
    In: European Journal of Case Reports in Internal Medicine, SMC Media, ( 2022-02-04)
    Abstract: Rosai-Dorfman-Destombes disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare non-Langerhans cell histiocytosis of unknown cause. The disease often manifests as bilateral painless cervical lymphadenopathy associated with systemic symptoms such as fever and weight loss. Extranodal disease is also frequent and can involve any organ, mostly skin, nasal cavity, bone, and retro-orbital tissue. Swelling of cartilaginous tissues such as ear helix or laryngeal structures may mimic the entity known as relapsing polychondritis. Although spontaneous remission is the most expected evolution, some cases require systemic treatment with prednisone, methotrexate or cytotoxic agents, with variable success rates. In this respect, since somatic variants in genes involved in the mitogen-activated protein kinase (MAPK) pathway have been observed to play a pathogenic role in RDD, the use of therapies targeting these pathogenic variants seems to be a reasonable strategy. Here we present a case of RDD with extensive extranodal involvement that showed a rapid and complete response to the MEK inhibitor cobimetinib
    Type of Medium: Online Resource
    ISSN: 2284-2594
    Language: English
    Publisher: SMC Media
    Publication Date: 2022
    detail.hit.zdb_id: 3016175-7
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  • 2
    Online Resource
    Online Resource
    SMC Media ; 2020
    In:  European Journal of Case Reports in Internal Medicine , No. LATEST ONLINE ( 2020-10-29)
    In: European Journal of Case Reports in Internal Medicine, SMC Media, , No. LATEST ONLINE ( 2020-10-29)
    Abstract: Spontaneous haemothorax (SH) is a rare subcategory of haemothorax that involves the accumulation of blood within the pleural space in the absence of trauma. SH especially occurs in middle-aged or elderly patients, but data are usually limited to case reports and case series. Coagulopathy, aneurysm or aortic dissection, Rendu-Osler-Weber syndrome and malignancy have to be considered among the causes of SH. Aim: We describe a case of primary angiosarcoma of the spleen presenting as relapsing haemothorax. Results: An 81-year-old woman was referred to our hospital because of a 2-month history of relapsing haemothorax after the performance of urgent splenectomy due to active bleeding from large spleen cysts. No evidence of neoplasm was seen after pathological examination of the spleen. On admission, left haemothorax and 2 new cystic masses in the thoracic wall were documented, both in close relation to the scars of previous surgery and chest tubes. After excision of 1 mass, histological examination revealed angiosarcoma, and a final diagnosis of primary angiosarcoma of the spleen with postsurgical metastatic dissemination to the thorax and pleura was made. Conclusion: Primary angiosarcoma should be included in the differential diagnosis of haemorrhagic spleen cysts. Clinical diagnosis and management usually requires splenectomy, but it should be carefully planned in order to avoid local metastatic dissemination or haematogenous spread of the tumour.
    Type of Medium: Online Resource
    ISSN: 2284-2594
    Language: English
    Publisher: SMC Media
    Publication Date: 2020
    detail.hit.zdb_id: 3016175-7
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
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