Marginal zone B-cell lymphoma: lessons from Western and Eastern diagnostic approaches.

Immunoglobulin G4 Thymic Tumor.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by infiltration of extensive IgG4-positive plasma cells and lymphocytes. Although IgG4-RD has been observed in almost all organs, it rarely affects the myocardium. Cardiovascular lesions of IgG4-RD appear as aortic (aortic aneurysm and aortitis) and pericardial (constrictive pericarditis) lesions as well as pseudotumors around the coronary arteries. We herein report a case of IgG4-RD with a cardiac mass in the right atrium involving a sinus node. This condition caused arrhythmia and repeated strokes. We successfully treated the patient through resection of the cardiac mass, catheter ablation and immunosuppressive therapy. 

IgG4-related Disease with a Cardiac Mass Causing Cerebral Infarction

Among mediastinal masses, about half are primary tumors in the anterior mediastinum, both benign and malignant. A variety of nonneoplastic lesions (developmental, inflammatory, reactive) can present as a localized mass in this compartment involving the thymus. Ectopic/embryonic remnants also occur in the prevascular compartment of the mediastinum. Rare tumors may develop from each of the multiple tissue types located in this vital area. The purpose of this chapter is to provide a short morphologic/photographic overview of lymphomas and of other rare tumors of mesenchymal type and of germ cell tumors occurring in the thymus or in the anterior mediastinum area. Selected examples of pseudotumors will also be shown. Specific differential diagnostic points are provided, both for “solid” tumors and for hematological malignancies. The main immunohistochemical characteristics of neoplastic/nonneoplastic pathology and updated specific references are discussed.

Lymphomas and Other Rare Tumors of the Thymus

CD30, also known as TNFRSF8 (tumor necrosis factor receptor superfamily member 8), is a protein receptor that is heavily glycosylated inside the Golgi apparatus, as well as a tumor marker that is found on the surface of specific cells in the body, including certain immune cells and cancer ones. This review aims to shed light on the critical importance of CD30, from its emergence in the cell to its position in diagnosing various diseases, including Hodgkin lymphoma, where it is expressed on Hodgkin and Reed–Sternberg cells, as well as embryonal carcinoma, anaplastic large cell lymphoma (ALCL), and cutaneous T-cell lymphoma (CTCL). In addition to its role in positive diagnosis, targeting CD30 has been a promising approach treating CD30-positive lymphomas, and there is ongoing research into the potential use of CD30-targeted therapies for autoimmune disorders. We aim to elaborate on CD30’s roles as a tumor marker, supporting thus the hypothesis that this receptor might be the aim of cytostatic treatment.

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The Polyvalent Role of CD30 for Cancer Diagnosis and Treatment

IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lymphoplasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally. The epidemiology remains poorly studied, but it has been noted that in the majority of recorded instances, patients are middle-aged men. IgG4-RD could mimic conditions other than tumors, such as infection, inflammation, or other systemic disorders. To ensure accuracy of diagnosis, an exhaustive histopathological analysis is required, together with clinical, radiological, and serological data. Thymic fibrosis in the absence of other primary thymic lesions is a very rare occurrence; in English literature only 1 case has been reported with scattered IgG4 plasma cells infiltrate and focal obliterative phlebitis. We will describe, for the first time, the case of a 49-year-old man displaying an anterior mediastinic, hilar, and intramyocardial mass simulating a sarcoidosis, with a definitive diagnosis of IgG4-related thymic fibrosis extending to the mediastinum and the heart. At the histological examination, we found many features of IgG4-RD in the thymic tissue, such as diffused storiform fibrosis, dense lymphoplasmacytic infiltrate with abundant plasma cells IgG4 positive (ratio IgG/IgG4: 40%), obliterative phlebitis, eosinophilic infiltrate, and Castleman-like lymphoid follicles. We discussed the differential diagnosis and reviewed the literature and the other cases of IgG4-related diseases that had been diagnosed in our department.

IgG4-related disease in thymus. A very rare case of chronic fibrosis mimicking sarcoidosis.

IgG4-related disease with possible myocardial involvement.

Resumen La enfermedad relacionada con el deposito de IgG4 es una entidad caracterizada por lesiones tumefactas, infiltrado denso linfoplasmocitico con positividad inmunohistoquimica para IgG4, fibrosis estoriforme y, frecuentemente, niveles sericos elevados de IgG4. Puede cursar con afectacion multisistemica; sin embargo, la afectacion miocardica, objetivada mediante pruebas de imagen, no ha sido descrita en la literatura medica. Se presenta el caso clinico de un varon afecto de enfermedad relacionada con IgG4 con posible afectacion miocardica, detectada a traves de cardiorresonancia magnetica, que plantea el diagnostico diferencial con otras enfermedades como la sarcoidosis y la enfermedad de Fabry, cuyo diagnostico diferencial es de gran importancia por su repercusion terapeutica.

Nelson Leal Bohorquez

Papers

IgG4-related disease in thymus. A very rare case of chronic fibrosis mimicking sarcoidosis.

IgG4-related disease with possible myocardial involvement.

Enfermedad relacionada con IgG4 con posible afectación miocárdica