Giovanni Bartoloni

Leiomyomatosis with vascular invasion. A unified pathogenesis regarding leiomyoma with vascular microinvasion, benign metastasizing leiomyoma and intravenous leiomyomatosis.

Three uterine leiomyomas with vascular invasion (LWVI), two of which were associated with pulmonary leiomyomatous nodules, and a case of intravenous leiomyomatosis (IVL) invading the vena cava and extending to the right atrium, are described. Despite their histological benignity, these lesions have a strong tendency to metastasize and are closely related to the socalled benign metastasizing leiomyoma (BML). From a clinical point of view, the pulmonary nodules of LWVI are stable or slowly-growing. The IVL was a “wormlike” tumour that presented as a cardiac mass. On the basis of their histological and immunohistological features, a unified histogenetic view of LWVI, IVL and BML of the uterus is proposed. LWVI and BML may be the same pathological entity and microscopic vascular invasion may represent the metastatic mechanism of BML. Alternatively, LWVI may be the initial stage of IVL. In rare instances, IVL may be associated with distant parenchymal (pulmonary) metastases. LWVI seems to be the precursor of both BML and IVL.

Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases

Inflammatory diseases of the aorta include routine atherosclerosis, aortitis, periaortitis, and atherosclerosis with excessive inflammatory responses, such as inflammatory atherosclerotic aneurysms. The nomenclature and histologic features of these disorders are reviewed and discussed. In addition, diagnostic criteria are provided to distinguish between these disorders in surgical pathology specimens. An initial classification scheme is provided for aortitis and periaortitis based on the pattern of the inflammatory infiltrate: granulomatous/giant cell pattern, lymphoplasmacytic pattern, mixed inflammatory pattern, and the suppurative pattern. These inflammatory patterns are discussed in relation to specific systemic diseases including giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis (Wegeners), rheumatoid arthritis, sarcoidosis, ankylosing spondylitis, Cogan syndrome, Behçets disease, relapsing polychondritis, syphilitic aortitis, and bacterial and fungal infections.

Cardiac iron overload in thalassemic patients: An endomyocardial biopsy study

Secondary heart failure induced by organ siderosis is the main cause of death in patients affected by thalassemia major. At present it cannot be predicted whether heart siderosis is correlated with iron overload and little is known about the real cardiac histological pattern of post transfusional hemochromatosis in patients with thalassemia major and intermedia. The study aim was to evaluate cardiac iron overload by non invasive and invasive techniques. Fifteen thalassemic patients were investigated and endomyocardial biopsy performed in ten revealed different grades of endomyocardial iron overload with histochemical positivity. Non invasive techniques are not able to furnish an exact picture of the cardiac hemochromatosis. There was a significant correlation between serum ferritin and myocardial iron grade.Patients with elevated ferritin levels and poor compliance to chelating therapy are at high risk of severe heart hemochromatosis. It was seen that endomyocardial biopsy is a useful tool in studying myocardial iron.

Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: II. Noninflammatory degenerative diseases - Nomenclature and diagnostic criteria

Surgical aortic specimens are usually examined in Pathology Departments as a result of treatment of aneurysms or dissections. A number of diseases, genetic syndromes (Marfan syndrome, Loeys-Dietz syndrome, etc.), and vasculopathic aging processes involved in vascular injury can cause both distinct and nonspecific histopathologic changes with degeneration of the media as a common denominator. Terminology for these changes has varied over time leading to confusion and inconsistencies. This consensus document has established a revised, unified nomenclature for the variety of noninflammatory degenerative aortic histopathologies seen in such specimens. Older terms such as cystic medial necrosis and medionecrosis are replaced by more technically accurate terms such as mucoid extracellular matrix accumulation (MEMA), elastic fiber fragmentation and/or loss, and smooth muscle cell nuclei loss. A straightforward system of grading is presented to gauge the extent of medial degeneration and synoptic reporting tables are provided. Herein we present a standardized nomenclature that is accessible to general pathologists and useful for future publications describing these entities.

Cytokeratin profile and neuroendocrine cells in the glandular component of cardiac myxoma

Glandular cardiac myxomas are very rare tumors of uncertain histogenesis that display glandular structures within otherwise typical myxomatous tissue. The origin of the glands has been attributed to epithelial differentiation of a totipotent cardiomyogenic precursor cell or to entrapped embryonal rests in the tumor. We studied four cases of glandular myxomas (three sporadic and one familial) to define the immunophenotypic profile of the glandular elements. The glands were either single and located within the myxoma cell islands (three cases) or in groups embedded in the myxomatous matrix. In the latter case, the glands featured villous projections, irregular profile, active inflammation or focal reactive cellular atypia (case 3) and had acidic and neutral mucins (mostly sialomucins). The cytokeratin expression profile (cytokeratin 7 and 20 co-expression) was similar to that of foregut derivatives. Scattered chromogranin-positive neuroendocrine cells were observed in case 3. Our findings indicate that the glandular component in cardiac myxoma is morphologically heterogeneous. In some cases, the scattered glands may derive from a divergent (epithelial) differentiation of myxoma cells; in others, entrapment of embryonic gastrointestinal rests (with mature neuroendocrine and mucous cell populations) could be the case.

Expression of αvβ3 and α4β1 integrins throughout the putative window of implantation in a cohort of healthy fertile women

Background.  Blastocyst implantation is a dynamic process requiring a specific cascade of cellular interactions and endometrial changes. The aim of this prospective observational study was to investigate the endometrial expression of αVβ3 and α4β1 integrins throughout the window of implantation in healthy fertile women.

Ultrastructural features and ICSI treatment of severe teratozoospermia: report of two human cases of globozoospermia

Abnormal sperm morphology is associated with male infertility. We describe two human cases of globozoospermia (round-headed spermatozoa) together with fine diagnosis and proposed treatment. Scanning and transmission electron microscopy (SEM and TEM) were performed to identify the ultrastructural features. Female partners underwent ovarian hyperstimulation and intracytoplasmic sperm injection (ICSI). Fertilized oocytes were transferred 36 h later. One couple had a healthy live-birth. Ultrastructural analysis may help to diagnose sperm morphology and identify those which will respond to treatment.

Expression of α6 and β4 integrin subunits on human endometrium throughout the menstrual cycle and during early pregnancy

Abstract Objective: To evaluate the expression of α6 and β4 integrin subunits on surface and glandular endometrium throughout the menstrual cycle and during early pregnancy. Setting: Second Department of Obstetrics and Gynecology, University of Catania, Catania, Italy. Patient(s): Thirty-two women. Nineteen of the women regularly menstruated in different phases of the cycle, and 13 were in the sixth to ninth week of gestation and required voluntary abortion. Intervention(s): Endometrial specimens collected during endometrial biopsy, hysterectomy, or voluntary abortion. Main Outcome Measure(s): Immunohistochemical staining for α6 and β4 integrin subunits in endometrial tissues. Result(s): Both subunits (poorly expressed in preimplantation days) reached a significant peak on the endometrial surface during the implantation window, which tended to disappear in the postimplantation phase. On glandular endometrium they exhibited an opposite trend, showing high levels in the preimplantation and postimplantation days, whereas their expression decreased during the implantation window. The two subunits tended to disappear in early pregnancy. Conclusion(s): α6 and β4 integrin subunits are uniformly distributed and highly expressed on the endometrial surface during the implantation window; they decreased dramatically in the postimplantation phase. These results could suggest involvement of integrin-extracellular matrix components in blastocyst-endometrium interaction during the early stages of implantation.

Apoptosis in temporomandibular joint disc with internal derangement involves mitochondrial-dependent pathways. An in vivo study

Abstract Objective. Two main apoptosis pathways have been identified: an extrinsic (or death receptor-mediated) and an intrinsic (or mitochondrial) pathway. Apoptotic cell death through the extrinsic pathway has just been described in temporomandibular joint disc (TMJ) with internal derangement (ID); in contrast, no data are available on the involvement of the intrinsic pathway in this tissue. The aim of this work was to investigate whether the intrinsic pathway participates in apoptosis activation in patients with TMJ ID and anterior disc displacement without reduction. Materials and methods. Apoptosis activation was studied in TMJ discs from 15 patients with ID and in six unaffected discs using bcl-2–associated X protein (bax), B-cell lymphoma 2 (bcl-2), cytochrome c and caspase 9 immunohistochemistry. A correlation was sought between immunohistochemical findings and degree of disc damage. Results. None of the pathological TMJ disc sections were immunopositive for bcl-2; negative bcl-2 immunostaining was detected in affected discs; cytochrome c and caspase 9 immunoreactivity was greater in pathological compared to unaffected discs; the difference was significant and correlated with histopathological degeneration score data (Spearmans rho = 0.617). Conclusion. The present findings suggest that in-human TMJ with ID and anterior disc displacement without reduction of cell apoptosis occurs, at least partly, via the mitochondrial pathway, which contributes to the subsequent disc degeneration. These data may have clinical implications and could help devise improved treatment strategies.

Functional mitral regurgitation: from normal to pathological anatomy of mitral valve.

Mitral valve (MV) is composed of several structures working in synchrony to open during diastole and close in systole within the high-pressure systemic environment. Its morphological features ensure a normal leaflet closure that prevents regurgitation of blood back into the left atrium causing loss of ventricular pressure and forward flow. The complex interactions of the normal MV are reliant on each component playing a complete role for the efficient working of the valve. In this review we firstly discuss the overall MV structure in terms of a complex make up of the annulus, the leaflets, their tendinous cords, and the supporting papillary muscles, and then the anatomical changes of each MV components due to left ventricular geometry and function alterations, underlying functional mitral regurgitation.