This article reviews the nomenclature of benign and malignant neoplasm of the heart and pericardium in the 4th edition of the World Health Organization's Classification, with emphasis on differences since the 3rd edition of 2004. The tumours are divided into benign, malignant, and intermediate tumors of uncertain behavior, with separate sections on germ cell tumours and tumors of the pericardium. There are important updates in the sarcoma classification, with emphasis on the most common site, the left atrium. The importance of the new genetic finding in cardiac myxomas, namely somatic mutations in the PRKAR1A gene underscores the importance of this alteration in the pathogenesis of these tumors. Challenges on the classification of each entity are discussed.

Context.— Controversies and uncertainty persist in prostate cancer grading. Objective.— To update grading recommendations. Data Sources.— Critical review of the literature along with pathology and clinician surveys. Conclusions.— Percent Gleason pattern 4 (%GP4) is as follows: (1) report %GP4 in needle biopsy with Grade Groups (GrGp) 2 and 3, and in needle biopsy on other parts (jars) of lower grade in cases with at least 1 part showing Gleason score (GS) 4 + 4 = 8; and (2) report %GP4: less than 5% or less than 10% and 10% increments thereafter. Tertiary grade patterns are as follows: (1) replace “tertiary grade pattern” in radical prostatectomy (RP) with “minor tertiary pattern 5 (TP5),” and only use in RP with GrGp 2 or 3 with less than 5% Gleason pattern 5; and (2) minor TP5 is noted along with the GS, with the GrGp based on the GS. Global score and magnetic resonance imaging (MRI)-targeted biopsies are as follows: (1) when multiple undesignated cores are taken from a single MRI-targeted lesion, an overall grade for that lesion is given as if all the involved cores were one long core; and (2) if providing a global score, when different scores are found in the standard and the MRI-targeted biopsy, give a single global score (factoring both the systematic standard and the MRI-targeted positive cores). Grade Groups are as follows: (1) Grade Groups (GrGp) is the terminology adopted by major world organizations; and (2) retain GS 3 + 5 = 8 in GrGp 4. Cribriform carcinoma is as follows: (1) report the presence or absence of cribriform glands in biopsy and RP with Gleason pattern 4 carcinoma. Intraductal carcinoma (IDC-P) is as follows: (1) report IDC-P in biopsy and RP; (2) use criteria based on dense cribriform glands (>50% of the gland is composed of epithelium relative to luminal spaces) and/or solid nests and/or marked pleomorphism/necrosis; (3) it is not necessary to perform basal cell immunostains on biopsy and RP to identify IDC-P if the results would not change the overall (highest) GS/GrGp part per case; (4) do not include IDC-P in determining the final GS/GrGp on biopsy and/or RP; and (5) “atypical intraductal proliferation (AIP)” is preferred for an intraductal proliferation of prostatic secretory cells which shows a greater degree of architectural complexity and/or cytological atypia than typical high-grade prostatic intraepithelial neoplasia, yet falling short of the strict diagnostic threshold for IDC-P. Molecular testing is as follows: (1) Ki67 is not ready for routine clinical use; (2) additional studies of active surveillance cohorts are needed to establish the utility of PTEN in this setting; and (3) dedicated studies of RNA-based assays in active surveillance populations are needed to substantiate the utility of these expensive tests in this setting. Artificial intelligence and novel grading schema are as follows: (1) incorporating reactive stromal grade, percent GP4, minor tertiary GP5, and cribriform/intraductal carcinoma are not ready for adoption in current practice.

Fibroblast growth factor receptors (FGFR) are transmembrane kinase proteins with growing importance in cancer biology given the frequency of molecular alterations and vast interface with multiple other signaling pathways. Furthermore, numerous FGFR inhibitors in clinical development demonstrate the expanding therapeutic relevance of this pathway. Indeed, results from early phase clinical trials already indicate that a subset of patients with advanced tumors derive benefit from FGFR targeted therapies. FGFR gene aberrations and FGFR gene rearrangements are relatively rare in solid malignancies. The recently described FGFR3-TACC3 fusion protein has a constitutively active tyrosine kinase domain and promotes aneuploidy. We summarize the prevalence data on FGFR3-TACC3 fusions among different histological tumor types and the preliminary evidence that this rearrangement represents a targetable molecular aberration in some patients with solid tumors.

Lymphoma of the heart and pericardium is usually present as one aspect of disseminated disease and rarely occurs as a primary malignancy. It accounts for 1.3% of primary cardiac tumors and 0.5% of extranodal lymphomas. Cardiac lymphomas are most commonly diffuse large cell lymphomas and frequently manifest as an ill-defined, infiltrative mass. Atrial location is typical; the right atrium is most often affected. Pericardial thickening or effusion is often a common early feature of disease. Infiltration of atrial or ventricular walls with extension along epicardial surfaces is also a notable feature. At computed tomography, the attenuation of cardiac lymphoma may be similar to or lower than that of normal myocardium. At magnetic resonance imaging, it has variable signal intensity and contrast enhancement. Clinical manifestations may include pericardial effusion, cardiac arrhythmias, and a variety of nonspecific electrocardiographic abnormalities, notably first- to third-degree atrioventricular block. Treatment most commonly includes anthracycline-based chemotherapy and anti-CD20 treatment. Chemotherapy has been used alone or combined with radiation therapy. Palliative surgery has been performed, mainly for tumor debulking. The prognosis for patients with either primary or secondary lymphomatous heart involvement is usually poor; late diagnosis is one of the major factors affecting outcome.

Vascular tumors of the bladder are rare and a subject of small series and case reports. We retrospectively identified vascular tumors of the urinary bladder from the consultation files from one of the authors. We identified 13 lesions that included 3 hemangiomas, 3 intravascular papillary endothelial hyperplasias (Masson vegetant hemangioendotheliomas), 2 arteriovenous malformations (AVMs), 1 epithelioid hemangioendothelioma (EHE), and 4 angiosarcomas. One of the angiosarcomas was associated with conventional high-grade urothelial carcinoma (sarcomatoid carcinoma). All patients were adults with a range in age from 18 to 85 years old (mean 63.3). There was no statistical difference among the various lesions in terms of age, although angiosarcomas tended to arise in older patients (mean 71 y vs. 60 y of the remainder). Hematuria was the most common presentation of both benign and malignant lesions. Other symptoms included voiding irritation, pelvic pain, and obstruction. Histologically, benign and malignant lesions were similar to their counterparts in other organ systems. Two hemangiomas were of the capillary type and a third one of the cavernous subtype. They measured 1.1, 2.4, and 3.2 cm. Both AVMs were clinically large broad-based masses measuring 5.5 and 5.8 cm in greatest diameter. One of the AVMs was associated with pseudoepitheliomatous hyperplasia of the urothelium. All 3 patients with Masson lesion had history of radiation therapy for other causes. These presented as raised lesions and were all <1.0 cm. Patients with hemangiomas, papillary endothelial hyperplasias, and AVM had an invariably benign prognosis and needed no further therapy. These benign lesions had consistent involvement of the submucosa and spared the muscularis propria of the organ. All cases of angiosarcoma and EHE involved the muscularis propria. Two of four patients with angiosarcoma had a history of prior radiation therapy and all 4 were dead of disease at 6 months. Angiosarcomas measured 3, 4.5, 5, and 5.8 cm in greatest diameter at cystoscopy. The patient with EHE had a single nodule treated by transurethral resection of the bladder and no evidence of disease at 4 years of follow-up. None of the patients experienced marked gross hematuria that resulted in morbidity or mortality. A wide spectrum of benign, intermediate malignant, and malignant vascular lesions primarily involved the bladder. Despite the potential for marked hemorrhage, none of the tumors resulted in marked hematuria. Papillary endothelial hyperplasia occurs in the bladder and must be differentiated from angiosarcoma, which has a rapidly fatal outcome.