An Update of the Benign Proliferative Monocyte‐Macrophage and Dendritic Cell Disorders

Moschella, Samuel L.

doi:10.1111/j.1346-8138.1996.tb02704.x

Cited by 8 publications

(6 citation statements)

References 50 publications

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“…2 Adult xanthogranulomatous disease falls within class II of this categorisation. In the World Health Organization ICD-10 classification of diseases, it falls within the category of 'other histiocytosis syndromes' (category D76.…”

Section: Discussionmentioning

confidence: 99%

Necrobiotic xanthogranuloma of the parotid gland

et al. 2009

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Necrobiotic xanthogranuloma may clinically mimic commoner tumours such as Warthin's tumour. Once diagnosed, the clinician should be wary of extracutaneous manifestations and paraproteinaemias. Because of the variability of presentation, there is no consensus on the best treatment for necrobiotic xanthogranuloma, which may include surgery, chemotherapy, interferon, plasmapheresis and radiation therapy.

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Section: Discussionmentioning

confidence: 99%

Necrobiotic xanthogranuloma of the parotid gland

et al. 2009

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“…According to the classification of the Histiocyte Society Writing Group in 1987, 4 AOXGD is a type of class II histiocytic disorder, composed of mononuclear histiocytes, 4,5 responsible for ocular eyelid and periorbital involvement. 6 The other major category is class I histiocytic disorder, composed of Langerhans histiocytes, a nonphagocytic, antigenprocessing accessory cell line, which is typically responsible for isolated eosinophilic granulomas of the orbital bones.…”

Section: Discussionmentioning

confidence: 99%

“…Touton giant cells with an annulus of nuclei encircling central eosinophilic cytoplasm and an outer rim of vacuolated cytoplasm are also seen. 2,4 In NXG, subcutaneous ulcerative lesions because of devitalized collagen are seen in the skin and periocular tissues. Adipophilin demonstrates the presence of cytoplasmic lipids.…”

Section: Discussionmentioning

confidence: 99%

Bilateral Adult Epibulbar Xanthogranulomas Suspicious for Erdheim–Chester Disease

Aggarwal

Hamrah

2014

Cornea

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Adult-onset xanthogranuloma can present as a solitary conjunctival mass without periocular or orbital involvement. The clinical, histopathologic, and radiologic findings in this case are suggestive of Erdheim-Chester disease without displaying any life-threatening lesions to date. Histopathologic and imaging studies can help in obtaining a diagnosis. Ophthalmologists should be aware that xanthogranulomatous conditions may have potential systemic implications, and a thorough systemic evaluation is recommended for lesions that initially seemed to be isolated in nature.

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“…13 Adult xanthogranulomatous disease, which falls within class II, is uncommon, generally affects middle aged individuals without sex predilection (table 1), and can present as subcutaneous, subconjunctival and periocular xanthochromic infiltrates with varying mass formation.…”

Section: Discussionmentioning

confidence: 99%

Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review

Sivak-Callcott

Rootman²,

Rasmussen³

et al. 2006

British Journal of Ophthalmology

164

156

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Background/aims: Adult xanthogranulomatous disease involving the ocular tissues is rare and poorly understood. Adult onset xanthogranuloma (AOX), adult onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim-Chester disease (ECD) are the four syndromes within this disorder, which is diagnosed by characteristic histopathology. Experience with eight cases prompted a multi-institutional effort to study the histopathology, immunohistochemistry, clinical findings, and systemic associations in this disorder. Methods: 22 cases, including histopathological slides, were compiled. Published reports were identified by an English language Medline search and review of reference citations. Each case in this series and the literature was classified as one of four syndromes and then analysed for age onset, sex, skin xanthoma, orbital location, immune dysfunction, internal organ and bone lesions, treatment, and outcome. The histopathology in each of these cases was reviewed by two pathologists. Immunhistochemical stains (CD3, CD4, CD8, L26) were performed in 14 cases where unstained slides were available. Results: 137 cases were compiled. There was no sex or age difference between syndromes. AOX, AAPOX, NBX affect the anterior orbit, ECD tends to be diffuse and intraconal. Skin lesions are found in all the syndromes. Immune dysfunction was noted in all cases of AAPOX and NBX; 11% of NBX and all ECD patients had internal organ disease. Treatment included surgery, corticosteroids, other chemotherapeutic agents, radiotherapy, and combinations of these. No AOX or AAPOX deaths occurred; 66% of ECD patients died. All 22 cases had xanthoma cells; most had Touton giant cells. Lymphocytes were present in all cases and occurred as aggregates (mostly in AAPOX) or diffuse populations mixed with fibroblasts (mostly in ECD). Immunohistochemistry revealed the majority of these to be CD8+. Necrosis was most marked in NBX. Conclusion: Adult xanthogranuloma of the orbit is rare, making prospective evaluation or meta-analysis impossible. The best treatment is unknown but seems to be with multiagent chemotherapy guided by histopathological, immunohistochemical, and systemic findings.

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An Update of the Benign Proliferative Monocyte‐Macrophage and Dendritic Cell Disorders

Cited by 8 publications

References 50 publications

Necrobiotic xanthogranuloma of the parotid gland

Necrobiotic xanthogranuloma of the parotid gland

Bilateral Adult Epibulbar Xanthogranulomas Suspicious for Erdheim–Chester Disease

Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review

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