Erdheim-chester disease: A case report with immunohistochemical and biochemical examination

Ono, Kenzo; Oshiro, Mariko; Uemura, Keiichi; Ota, Hiroyoshi; Matsushita, Yasuyuki; Ijima, Shojyu; Iwase, Tamaki; Uchida, Masao; Katsuyama, Tsutomu

doi:10.1016/s0046-8177(96)90145-8

Cited by 49 publications

(34 citation statements)

References 12 publications

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“…The initial diagnosis (cellular BFH) was reconsidered as non-LCH. The similar immunohistochemical profile in the bone -CD68 (+), CD1a (-) [2] and S100 (slightly positive) [10] -also supported the non-LCH type of bone proliferating histiocytes. The mild and focal positivity of S100 in histiocytes and the absence of emperipolesis in those histiocytes expressing S100 protein rule out the diagnosis of RDD.…”

Section: Discussionsupporting

confidence: 54%

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Complex Erdheim-Chester disease associating rectal adenocarcinoma and breast cancer

Gutu¹,

Danciu²,

Guțu³

et al. 2016

Arch Clin Cases

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Erdheim-Chester Disease (ECD) is a rare form of non-Langerhans' cell histiocytosis (non-LCH). It is characterized by the excessive production of histiocytes. We present the case of a 63 year-old Caucasian woman with ECD, with a history of breast cancer (diagnosed in 2006) and rectal cancer (diagnosed in 2013). Most probably, the initial ECD manifestation occurred within the next 5 years after mastectomy in the soft tissues adjacent to the scar. Recently it became manifest in a different localization (meta-diaphysis of long bones), while the other possible sites like the lungs, central nervous system and pituitary gland show minimal involvement with no specific clinical manifestation yet. Shortly after the bone manifestations were detected, she developed an invasive rectal adenocarcinoma. The ECD diagnosis was based on bone scintigraphy, radiography and immunohistochemistry examination.

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Section: Discussionsupporting

confidence: 54%

“…The histological changes include tissue infiltration with lipoid granulomas composed of foamy CD68-positive but CD1a-negative macrophages, in contrast to Langerhans cells. These macrophages are also usually negative for S-100 protein but may occasionally be weakly positive [9,10].…”

Section: Introductionmentioning

confidence: 99%

Complex Erdheim-Chester disease associating rectal adenocarcinoma and breast cancer

Gutu¹,

Danciu²,

Guțu³

et al. 2016

Arch Clin Cases

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“…Nowadays, much more information is available, and this concept has been hardly challenged. Evidences favoring distinct entities are the age of presentation (children or young adults in LCH against adults or elderly in ECD), the characteristic osteosclerotic symmetrical lesions of ECD as opposed to the frequent osteolytic lesions of LCH, the proliferative index of ECD lesions consistently lower than 3% as opposed to the LCH range (2% to 60%; mean value 10.5% for cytologically typical cases and 22.5% for cytologically atypical cases) [18,20,23,35]. Further, ECD histiocytes are uniformly negative for CD1a antigen, and no Birbeck granule has been demonstrated.…”

Section: Discussionmentioning

confidence: 99%

“…Histologically, ECD lesions show a cell infiltrate of xanthomatous histiocytes of non-Langerhans-cell lineage (CD68 positive; S100 protein, CD1a and Birbeck granules negative) and fibrosis with an infiltrative pattern [9,10,19,20,23,26]. Histiocytes have no nuclear atypia, and scattered lipid-laden multi-nucleated giant cells of the Touton-type can be found.…”

Section: Introductionmentioning

confidence: 99%

Erdheim?Chester disease of the breast associated with Langerhans-cell histiocytosis of the hard palate

et al. 2004

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We report a patient with Langerhans-cell histiocytosis (LCH) localized to the hard palate that was later proven to be associated with Erdheim-Chester disease (ECD), involving the right breast, skeleton, retroperitoneum and left orbit. The diagnosis was based on the symmetric osteosclerosis of the long bones diaphyses (tibias and fibulas), breast lump histopathological/immunohistochemical findings and retroperitoneum and left orbit images in magnetic resonance. Mammary involvement by ECD is an extremely rare condition, which should be differentiated from some benign and malignant mimickers, especially the histiocytoid type of breast carcinoma. Characteristic histological features plus clinical and radiographic information are needed to achieve a correct diagnosis. The ECD, its relation to the LCH and details of the breast lesion are discussed.

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“…3 Classically, the patients develop symmetric osteosclerotic lesions of the metaphyseal region of long bones with sparing of the flat bones. 1,2,4,5 Lytic bone lesions are present in approximately one-third of cases. 1,2 Typically, bone pain is the most frequent clinical symptom.…”

Section: Sommairementioning

confidence: 99%