Sclerosing Mesenteritis as an Uncommon Site of Involvement of IgG4-Related Disease: A Case Report With an Updated Review of the Literature.

Immunoglobulin G4-related disease (IgG4-RD) is an uncommon immune-mediated disorder most commonly involving the pancreas, lacrimal, and salivary glands. Immunoglobulin G4-related sclerosing mesenteritis (IgG4-RSM) is a rare site of involvement that usually mimics the imaging characteristics of mesenteric malignancies. Herein, we report a case of IgG4-RSM followed by an updated and comprehensive review of the literature. A 73-year-old woman presented with colicky abdominal pain in the right hypochondrium. The findings on contrast medium computed tomography (CMCT) showed a swelling of the mesenteric root with vascular structures surrounded by slightly contrast-impregnated tissue and irregular margins. The 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET) showed an area of inhomogeneous and intense hypermetabolism of the mesenteric root. Hence, laparoscopic resection of the mesenteric root was performed to distinguish such masses from malignant tumors, obtaining specimens for histopathologic examination. The latter exhibited tissue infiltration with lymphocytes, IgG4-positive plasma cells, and fibrosis, indicating a diagnosis of IgG4-RSM in the presence of both elevated serum IgG4 levels and the aforementioned imaging findings. With steroid therapy, no clinical signs of re-exacerbation within a one-year follow-up were observed and serum IgG4 levels returned to normality. Aiming to evaluate the real frequency of IgG4-RSM in view of the 2017 Comprehensive Diagnostic Criteria (CDC) of IgG4-RD, we undertook a complete MEDLINE, EMBASE, Web of Science, and Scopus database search of all case reports of IgG4-RSM published so far. Such criteria were met in only six cases with a definite diagnosis. This case highlights the mesentery as a rare site of involvement of IgG-RD and allows us to advance knowledge of IgG4-RSM.

Pathology of the thoracic aorta: a morphologic review of 338 surgical specimens over a 7-year period.

A total of 338 surgical specimens of the thoracic aorta were studied morphologically. These were obtained from patients (238 males, 100 females) operated on for aortic aneurysm or dissection at the Careggi Hospital, Florence, Italy, between January 1999 and June 2005. Medial degeneration was diagnosed in 299 cases (138 aneurysms, 161 dissections), atherosclerosis with extensive medial destruction was seen in 32 cases (26 aneurysms, 6 dissections), and giant cell arteritis (GCA) was found in 7 cases (all aneurysms). These data show that medial degeneration was a common nonspecific histologic diagnosis in aortic resection after the occurrence of aneurysm or dissection. Considering that this diagnosis was made in as many as 118 patients over 70 years of age and in 175 patients over 65, normal advancing age would appear as the most frequent correlation with these aortic events. Ageing is also associated with the increasing number of cases of GCA with aortic involvement. Angiogenesis, always observed next to areas of medial damage whatever the underlying pathology, may contribute to the pathogenesis of aortic dissection and aneurysm.

Malignant fibrous histiocytoma as a primary cardiac tumor.

We describe the case of a malignant fibrous histiocytoma (MFH) of the heart, presenting with an obstinate cough and clinically misdiagnosed as myxoma, in an 80-year-old woman. Fourteen months after surgical removal, an embolic episode revealed recurrence of the tumor. To plan the best surgical management, MFH, rare as it is, should be borne in mind when evaluating a cardiac intracavitary mass, especially if located in the left atrium.

Hepato-splenic lymphoma: a rare entity mimicking acute hepatitis: a case report.

We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented with markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic, autoimmune and metabolic liver diseases were excluded. Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease. Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen. Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45+, CD45RO+, CD3+, CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, thrombocytopenic purpura and blast in the peripheral blood. In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic T-cell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.

Constrictive pericarditis associated with hydrops fetalis.

We report a rare case of constrictive pericarditis coexistent with a unique heart malformation in a fetus. Cardiac abnormalities, possibly due to a transplacental viral infection, were responsible for severe hydrops fetalis.

Giant cell arteritis presenting with annuloaortic ectasia.

Four cases of giant cell arteritis causing severe aortic regurgitation secondary to an aneurysm of the ascending aorta are described. In two cases, the nature of the aortic pathology could be suspected considering the past clinical evidence of temporal arteritis and/or polymyalgia rheumatica. In the two other cases, the cardiothoracic manifestations represented the onset of Horton disease.

Secundum atrial septal defect and pulmonary hypertension in an 86-year-old woman: a case report and review of the literature.

A case of well tolerated secundum atrial septal defect in a woman who died at 86 years of age is described and the lesions responsible for pulmonary hypertension are discussed. Previous reports of over-70-year-old patients with untreated secundum atrial septal defect are also reviewed.