Castleman disease (CD) is a rare lymphoproliferative disorder with unique clinicopathological features, including two distinct clinical subtypes categorized as unicentric (UCD) and multicentric (MCD). UCD usually involves a single lymph node site presenting with no or minimal local symptoms. Histologically, most UCD cases exhibit regressive hyaline vascular germinal centers, characterized by penetrating vessels, dendritic hyperplasia/dysplasia, and increased interfollicular vascularity.
View Article and Find Full Text PDFBackground: Small bowel adenocarcinomas (SBAs) are rare and aggressive cancers. About one-fifth of SBA patients have predisposing conditions; among them, there are also genetic tumor syndromes, including Lynch syndrome, familial adenomatous polyposis, and Peutz-Jeghers syndrome. Although BRCA2 mutations, both somatic and germline, have been recently described in SBAs, direct evidence of BRCA2 inactivation in SBA tumor tissue of patients with BRCA2-related hereditary cancer syndrome is still very limited.
View Article and Find Full Text PDFAdenomyoma/adenomyomatosis (AM) of the gallbladder is generally considered an incidental and innocuous finding; however, neoplastic lesions, including intracholecystic neoplasms (ICNs), flat-type dysplasia, and carcinomas, may arise within AM. AM-associated ICNs, composed of mural cystically dilated glands containing florid papillary proliferations lined by mucinous and/or overtly dysplastic epithelium, are very rare and poorly characterized. This study aimed at investigating the clinico-radiologic, phenotypic/immunophenotypic, and molecular features of a mono-institutional case series of four AM-ICNs (0.
View Article and Find Full Text PDFIntroduction: Castleman disease (CD) represents a spectrum of heterogeneous lymphoproliferative disorders sharing peculiar histopathological features, clinically subdivided into unicentric CD (UCD) and multicentric CD (MCD) and presenting with variable inflammatory symptoms. Interleukin (IL)-6 and other cytokines play a major role in mediating CD inflammatory manifestations. Although the local microenvironment seems to be among the major sources of hypercytokinemia, the precise cellular origin of IL-6 production in CD is still debated.
View Article and Find Full Text PDFClin J Gastroenterol
December 2024
A 74-year-old woman in good general health presented with a 5-year history of progressive hair loss over several years, interpreted as female androgenetic alopecia (AGA), and was treated with topical 5% Minoxidil without improvement. The patient's relevant medical history revealed infiltrating, triple-negative apocrine carcinoma of the right breast four years before, treated by quadrantectomy, radiation, lymphadenectomy and chemotherapy, with no recurrence at the last follow-up. On examination, there was an asymptomatic 15 × 15 cm firm and whitish area of scarring alopecia on the central scalp.
View Article and Find Full Text PDFIntroduction: Early-onset small bowel adenocarcinoma (EO-SBA) is a rare and poorly characterized entity.
Methods: This retrospective study conducted on an international multicenter cohort of 208 patients with SBA aimed at comparing clinicopathologic features of EO-SBA (age younger than 50 years at SBA diagnosis) and late-onset SBA (age 50 years or older at SBA diagnosis).
Results: The presence of predisposing pathologic conditions was significantly more common in the EO-SBA group compared with that in the late-onset SBA group ( P = 0.