Clinicopathological characteristics of 5 cases of adult orbital xanthogranulomatous disease / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To examine the clinicopathological characteristics of adult orbital xanthogranulomatous disease(AOXGD).<p> METHODS: From January 2015 to January 2021, the researchers collected postoperative pathological diagnoses cases of AOXGD from Xi'an People's Hospital(Xi'an Fourth Hospital), retrospectively analyzed clinical pathological data, and reviewed related literature. <p>RESULTS: A total of five AOXGD cases were collected, including three cases of adult-onset xanthogranuloma(AOX), one case of necrobiotic xanthogranuloma(NBX), and one case of Erdheim-Chester disease(ECD). The five patients were composed of middle-aged and elderly patients with local orbital lesions were treated. Microscopic examination revealed that the orbital skin and subcutaneous tissue had foam-like non-Langerhans histiocytes that exhibited diffused or nested infiltration. Immunohistochemistry revealed that the cells were positive for CD68, CD163, FXⅢa, lysozyme, negative for S100, CD1a, Langerin, HMB-45, MDM2. One ECD patient had orbital lesions as the first symptom and exhibited a combination of retroperitoneal lesions, right atrium pseudotumor, and long bones, heart, kidney lesions. The diagnosis of AOXGD depends on clinical manifestations and pathological features. The treatment was based on subtypes and clinical manifestations to select corresponding strategies, which mainly include the administration of glucocorticoids, immunosuppressants, and surgical treatment. <p>CONCLUSION: AOXGD is relatively rare in clinical practice. AOXGD can manifest as a local orbital disease or an orbital disease combined with a systemic disease. In the clinical and pathological work of ophthalmology, the understanding and identification of this group of diseases should be strengthened, and correct diagnosis and standard treatment should be promoted.

Necrobiotic Xanthogranuloma Coexists with Diffuse Normolipidemic Plane Xanthoma and Multiple Myeloma

Necrobiotic xanthogranuloma associated with smoldering multiple myeloma: satisfactory response to cyclophosphamide, dexamethasone, and thalidomide

Abstract: Necrobiotic xanthogranuloma is a rare chronic condition, belonging to the group C non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly hematologic malignancies. The case reported here was only diagnosed after nine years of evolution and was associated with plasma cell dyscrasia. After treatment with cyclophosphamide, dexamethasone, and thalidomide, there was a reduction of cutaneous lesions and serum levels of monoclonal protein.

Primary Necrobiotic Xanthogranulomatous Sialadenitis with Submandibular Gland Localization without Skin Involvement

Necrobiotic xanthogranulomatous reaction is a multiorgan, non-Langerhans cell histiocytosis with an unknown etiology. Occurrence in the salivary gland is extremely rare. We recently identified a case of necrobiotic xanthogranulomatous sialadenitis in a 73-year-old Korean woman who presented with a painless palpable lesion in the chin. There was no accompanying cutaneous lesion. Partial resection and subsequent wide excision with neck dissection were performed. Pathological examination showed a severe inflammatory lesion that included foamy macrophages centrally admixed with neutrophils, eosinophils, lymphocytes, plasma cells, and scattered giant cells, as well as necrobiosis. During the 12-month postoperative period, no grossly remarkable change in size was noted. Necrobiotic xanthogranulomatous inflammation may be preceded by or combined with hematologic malignancy. Although rare, clinicians and radiologists should be aware that an adhesive necrobiotic xanthogranuloma in the salivary gland may present with a mass-like lesion. Further evaluation for hematologic disease and close follow-up are needed when a pathologic diagnosis is made.

Clinical advances in adult orbital xanthogranulomatous disease / 国际眼科杂志(Guoji Yanke Zazhi)

·Adult orbital xanthogranulomatous disease is a group of rare orbital and ocular adnexal disorders, which is classified as class II non - Langerhans histiocytic proliferations. This disease can be classified into 4 subtypes based mainly on systemic involvement: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, Erdheim-Chester disease and adult-onset asthma and periocular xanthogranuloma. Diagnosis depends on its characteristic clinical manifestations and pathologic features. Therapeutic approaches are derived from anecdotal evidences, which include corticosteroid, immunosuppressive agent, surgical debulking and chemotherapy. The management of this disease varies with different subtypes, as well as associated systemic presentations. This review summarizes advances of etiopathogenesis, clinical features, diagnosis and treatment.

Necrobiotic Xanthogranuloma with Monoclonal Gammopathy / 대한피부과학회지

Necrobiotic xanthogranuloma (NXG) is a rare, progressive, histiocytic disease characterized by destructive cutaneous lesions, a close association with paraproteinemia, multiple myeloma, hypertension, and multiple extracutaneous manifestations. Multiple indurated yellow-red plaques or nodules are commonly present in this disorder. The pathogenesis of NXG is poorly understood. Histopathologically, NXG features with hyaline necrosis or necrobiosis are present. We describe a patient with typical cutaneous and histologic findings of NXG with an associated monoclonal gammopathy.

A Case of Post-traumatic Necrobiotic Xanthogranuloma of the Orbit

PURPOSE: To investigate the role of nitric oxide (NO) on the migration of cultured human Tenon's capsule PURPOSE: Necrobiotic xanthogranuloma is a very rare disease characterized by the presence of yellow subcutaneous skin lesions and systemic paraproteinemia. We report a case of post traumatic necrobiotic xanthograuloma that presented with a preseptal mass. CASE SUMMARY: A 31-year-old man presented with ptosis and a mass on the left superior orbit that developed after trauma 3 weeks prior to admission. A computed tomography scan showed a contrast-enhancing preseptal mass, and surgical exploration was performed. CONCLUSIONS: Histopathological examination revealed a tumor that had all the major features of necrobiotic xanthogranuloma, including fat necrosis with granulomatous inflammation, giant multinucleated cells, foamy cells, and several lymphoid follicles with germinal centers. Hematologic evaluation revealed polyclonal gammopathy.

Necrobiotic Xanthogranuloma with Multiple Myeloma and No Periorbital Involvement

Necrobiotic xanthogranuloma (NXG) is a rare multisystemic disease that manifests as cutaneous inflammatory lesions and has a strong association with paraproteinemia and an increased risk of plasma cell dyscrasias and other lymphoproliferative disorders. Its general skin manifestation is multiple, asymptomatic, indurated nodules or plaques with a yellowish hue which frequently affect the periorbital regions. This case is unusual in that cutaneous lesions developed only at the extremities without periorbital involvement, accompanied by multiple myeloma.

Necrobiotic Xanthogranuloma with Paraproteinemia: A case report

Necrobiotic xanthogranuloma(NXG) is a characteristic cutaneous manifestation associated with paraproteinemia. A case of NXG associated with an IgG (lambda) monoclonal gammopathy occurred in a 48-year-old man. Skin lesions were dome-shaped, hard palpable nodules, 2x3 cm to 4x4 cm sized, on both arms and forearms. They were arranged in a linear pattern. Also, hard palpable tumors, 1x2 cm to 2x3 cm in size, were present on the left leg and the dorsum of the left foot. They were violaceous, slightly protruded, and hard on palpation. Histologically, the lesion was characterized by inflammatory xanthogranuloma with broad hands of hyaline necrobiosis. Many foreign body type of bizarre giant cells, Touton type of giant cells and foamy histiocytes were infiltrated into the dermis and subcutaneous fat tissue. Three days after medication with ledercort and immuran, the cutaneous masses decreased in size and became soft.