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Rare Cancer Presentations
Published in Debjani Sahni, Adam Lerner, Bilal Fawaz, Advanced Skin Cancer, 2022
Bilal Fawaz, Heather A. Edwards, Monica Rosales Santillan, Debjani Sahni, Connor O’Boyle, Daniel L. Faden
Histologically, DFH is a well-circumscribed subcutaneous spindle-cell neoplasm with a thick fibrous pseudo-capsule. It displays a storiform pattern of plump spindle cells with intermixed lymphocytes and entrapment of collagen bundles, in addition to a fascicular growth pattern with frequent extension into subcutaneous tissue. Foam cells, multinucleate giant cells, including Touton giant cells, and osteoclastic giant cells have also been reported. Immunohistochemical staining can demonstrate positivity for Factor XIIIa, CD34, and smooth muscle actin; it is sometimes focally positive for desmin. Staining is often negative for EMA.1,2
Benign tumors
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
These lesions are classified as fibrohistiocytic tumors due to their histopathology. It consists of many spindle-shaped and banana-shaped mononuclear cells in a whorled pattern, which may be fibroblast-derived, and there is a variable amount of new collagenous dermal connective tissue. There are also many histiocytic cells present, which often contain lipid or iron pigment, both of which may derive from the large number of small blood vessels also contained in the lesion. The presence of Touton giant cells loaded with hemosiderin is thought to be pathognomonic of dermatofibroma.
Histiocytic lesions
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Histopathology is identical with hyperlipidemic xanthomatosis elsewhere. The nodules contain masses of foamy cells and Touton giant cells. They were negative for protein S100 and CD68 in one case.8
IgG4-related ophthalmic disease in association with adult-onset asthma and periocular xanthogranuloma: a case report
Published in Orbit, 2023
Brigitte M. Papa, H. Miles Prince, Alan A. McNab, Penny McKelvie
IgG4-RD is a systemic fibroinflammatory condition, not uncommonly presenting with an ophthalmic tumefactive lesion or lesions, but can affect almost any organ.4 Diagnosis is made by integration of clinical, radiologic, serologic and histopathological data, with reference to consensus criteria.5 The term IgG4-related ophthalmic disease (IgG4-ROD) is preferred for ocular adnexal and orbital involvement of IgG4-RD.4 Histological findings show significant overlap between XG and IgG4-RD: common features include fibrosis; reactive lymphoid follicles; eosinophils; plasma cells; and diffuse infiltrates of T cells.6 Characteristics specific to IgG4-RD include storiform and/or eosinophilic angiocentric patterns of fibrosis, obliterative venulitis and a consistent IgG4+/IgG+ plasma cell ratio greater than 40%. Xanthoma cells and Touton giant cells do not comprise part of the histological pattern.6
Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence
Published in Orbit, 2022
Aleza A Andron, Akshay Gopinathan Nair, David Della Rocca, Robert C Della Rocca, Harsha S Reddy
Adult xanthogranulomatous diseases of the orbit and ocular adnexa are rare disorders that are characterized as class II non-Langerhans histiocytic proliferations.1 The disease is diagnosed histopathologically and is characterized by foamy histiocytes, Touton giant cells and varying degrees of fibrosis. The disease has four distinct subtypes; adult onset xanthogranuloma (AOX), Erdheim–Chester disease (ECD), necrobiotic xanthogranuloma (NBX), and adult onset asthma and periocular xanthogranuloma (AAPOX).1–3 Eyelid and orbital lesions can appear in all subtypes. After diagnosis, a systemic workup is warranted including ruling out asthma and a full rheumatology workup. Many treatments have been tried, such as corticosteroids, intralesional steroids, steroid-sparing agents, low dose radiation, debulking surgery, and even chemotherapy.3,4 There is little consensus on the preferred treatment owing to its rarity. IgG4-ROD is a systemic inflammatory disease first diagnosed in a case of autoimmune pancreatitis.5,6 This disease involves different organs with many different manifestations thus presenting often as a diagnostic challenge.7,8 The currently accepted criteria for diagnosis are:9-11
Adult xanthogranulomatous disease of the orbit: case report of spontaneous regression and review of treatment modalities
Published in Orbit, 2020
Michelle M. Maeng, Kyle J. Godfrey, Sanjai Jalaj, Michael Kazim
Magnetic resonance imaging (MRI) demonstrated an enhancing soft tissue lesion of both upper eyelids contiguous with the lacrimal gland, larger on the right [Figures 2a and 3a]. An incisional biopsy was performed. Histopathology demonstrated a mixed reactive lymphoplasmacytic infiltrate forming lymphoid nodules containing foamy histiocytes with scattered Touton giant cells. Immunohistochemical staining demonstrated a mixture of CD20 + B cells and CD3 + T cells and a mixture of polytypic kappa and lambda positive plasma cells [Figure 4]. Adult-onset xanthogranuloma (AOX) was diagnosed.