Disseminated Juvenile Xanthogranuloma Associated With Follicular Lymphoma in an Adult: Successful Treatment With Chemotherapy and Rituximab. A Review of the Literature

Narváez-Moreno, Basilio; Pulpillo-Ruiz, Águeda; Zulueta‐Dorado, Teresa; Conejo‐Mir, Julián

doi:10.1016/j.adengl.2012.02.009

Cited by 10 publications

(12 citation statements)

References 14 publications

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“…Such overlapping features between LCH and Erdheim-Chester disease, for example, with identical genetic properties, have led to the re-grouping of these diseases in the classification by Emile et al [73]. There are also descriptions of an association to acute leukemias [20,132], lymphomas [152], and sarcomas [131], and some rare histiocytoses have been related to autoimmune diseases, in particular to lupus erythematosus [38,131]. While the malignant histiocytoses are of clonal origin, the nature of the benign diseases is still not quite clear [61,223].…”

Section: Introduction ▼mentioning

confidence: 99%

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

2016

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Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Here, we follow the classification recently published by Jean Francois Emile and an international expert panel, defining subgroups of histiocytoses described as L-Group, C-Group, M-Group, R-Group, and H-Group, which stands for LCH-like, cutaneous or mucocutaneous, malignant, Rosai-Dorfman-Disease like and HLH like. Some of the diseases have an excellent prognosis after resection or even disappear spontanously, others progress rapidly, requiring intensive systemic therapies. The malignant non-Langerhans cell histiocytoses in general have a poor prognosis, here, complex chemotherapy protocols are usually applied, with inconsistant results. An interesting perspective in non-malignant rare histiocytoses might be small molecular inhibitors, in particular BRAF inhibitors, since BRAF mutations have been found in some subtypes of non-Langerhans cell histiocytoses. By prospective and retrospective collection of experiences in a new registry (the "International Rare Histiocytic Disorders Registry", IRHDR), knowledge about these rare diseases might hopefully be improved.

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Section: Introduction ▼mentioning

confidence: 99%

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

2016

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“…JXG is a non‐LCH of usually benign cutaneous presentation, most commonly seen in children as self‐limited yellow‐brown solitary lesions. Disseminated disease is rare and has been associated with blood malignancies such as chronic lymphocytic leukemia (CLL), acute lymphocytic leukemia (ALL), follicular lymphoma, and monoclonal gammopathy . Lesions can manifest before, during, or even after the diagnosis of the malignancy with some speculation that JXG may represent a neoplastic process .…”

Section: Discussionmentioning

confidence: 99%

“…Disseminated disease is rare and has been associated with blood malignancies such as chronic lymphocytic leukemia (CLL), acute lymphocytic leukemia (ALL), follicular lymphoma, and monoclonal gammopathy . Lesions can manifest before, during, or even after the diagnosis of the malignancy with some speculation that JXG may represent a neoplastic process . Lesion distribution in eyes, lungs, bones, testes, and gastrointestinal tract has been described .…”

Section: Discussionmentioning

confidence: 99%

Juvenile xanthogranuloma: A herald to the diagnosis of Erdheim‐Chester disease in an adult with acute leukemia

Sanchez‐Petitto¹,

Duke²,

Swaby

et al. 2018

J Cutan Pathol

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“…However, there are unusual cases with extracutaneous dissemination, most notably to the eye, and rarer cases associated with neurofibromatosis type 1 and juvenile myelomonocytic leukemia. Although much less prevalent, xanthogranuloma has also been identified in adults, where most lesions tend to be solitary and do not regress spontaneously . Histologically, classic juvenile and adult xanthogranulomas are similar and characterized by xanthomatized, vacuolated histiocytes and Touton‐type multinucleated giant cells.…”

Section: Introductionmentioning

confidence: 99%

“…Although much less prevalent, xanthogranuloma has also been identified in adults, where most lesions tend to be solitary and do not regress spontaneously. 1 Histologically, classic juvenile and adult xanthogranulomas are similar and characterized by xanthomatized, vacuolated histiocytes and Touton-type multinucleated giant cells. Early JXG and fibrohistiocytic JXG have been described, and are characterized by small to intermediate mononuclear histiocytes and spindled histiocytes, respectively, and generally display fewer, or absent, Touton giant cells when compared to classic JXG.…”

Section: Introductionmentioning

confidence: 99%

Emperipolesis and S100 expression may be seen in cutaneous xanthogranulomas: A multi‐institutional observation

et al. 2018

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Cutaneous Rosai-Dorfman disease (RDD) can be difficult to distinguish from other non-Langerhans cell histiocytoses, particularly xanthogranuloma (XG). Pathologists use S100 immunoreactivity, abundant plasma cells, and the presence of emperipolesis to distinguish RDD from XG. However, S100 expression has been reported in XG and, in practice, we have occasionally observed emperipolesis in cases that were otherwise clinically and pathologically consistent with XG. We present 10 cases of XG with emperipolesis and variable S100 immunoreactivity. Histologically, 7 cases were most in keeping with XG, and a histologic differential of XG versus RDD was raised in the remaining 3 cases. All 10 cases were clinically consistent with XG. Notably, none of these cases showed abundant plasma cells. Nine cases showed variable S100 immunostaining, ranging from focal/weak expression, to focal/strong, diffuse/moderate, and diffuse/strong expression. Histiocytes in all cases were CD68 positive and CD1a negative. We conclude that emperipolesis and S100 expression in a skin biopsy cannot reliably distinguish XG from cutaneous manifestations of RDD. Clinical correlations are essential, as are histologic clues to a diagnosis of classic XG that include an abundance of foamy mononuclear cells, Touton giant cells, and an absence of pale-stained histiocytes, abundant plasma cells, fibrosis, or vascular proliferation.

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Disseminated Juvenile Xanthogranuloma Associated With Follicular Lymphoma in an Adult: Successful Treatment With Chemotherapy and Rituximab. A Review of the Literature

Cited by 10 publications

References 14 publications

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

Juvenile xanthogranuloma: A herald to the diagnosis of Erdheim‐Chester disease in an adult with acute leukemia

Emperipolesis and S100 expression may be seen in cutaneous xanthogranulomas: A multi‐institutional observation

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