Congenital Myenteric Hypoganglionosis: Erratum
No abstract available (Source: The American Journal of Surgical Pathology)
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Erratum Source Type: research

GTF2A1-NCOA2-Associated Uterine Tumor Resembling Ovarian Sex Cord Tumor (UTROSCT) Shows Focal Rhabdoid Morphology and Aggressive Behavior
No abstract available (Source: The American Journal of Surgical Pathology)
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Letters to the Editor Source Type: research

Adenocarcinoma of the Rete Testis: Seven Additional Cases, Including Exclusively and Predominantly Intrarete Tumors
No abstract available (Source: The American Journal of Surgical Pathology)
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Letters to the Editor Source Type: research

Clinicopathologic and Genomic Characterization of Inflammatory Myofibroblastic Tumors of the Head and Neck: Highlighting a Novel Fusion and Potential Diagnostic Pitfall
Inflammatory myofibroblastic tumor (IMT) is a distinctive fibroblastic and myofibroblastic spindle cell neoplasm with an accompanying inflammatory cell infiltrate and frequent receptor tyrosine kinase activation at the molecular level. The tumor may recur and rarely metastasizes. IMT is rare in the head and neck region, and limited information is available about its clinicopathologic and molecular characteristics in these subsites. Therefore, we analyzed a cohort of head and neck IMTs through a multi-institutional approach. Fourteen cases were included in the provisional cohort, but 1 was excluded after molecular analysis ...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Crohn Disease Infrequently Affects the Appendix and Rarely Causes Granulomatous Appendicitis
Data from previous studies suggest Crohn disease of the appendix accounts for ∼25% of granulomatous appendicitis cases. However, we have found that granulomatous inflammation in appendectomy specimens rarely heralds Crohn disease. We suspect that appendiceal involvement by Crohn disease is uncommon, even when patients have severe ileocolonic inflammation. We performed this study to determine the prevalence and nature of appendiceal inflammation among patients with Crohn disease. We reviewed 100 ileocolic specimens with strictures and fistulizing Crohn disease for the nature and distribution of inflammatory changes in the...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Nonampullary Duodenal Adenomas in Familial Adenomatous Polyposis and Sporadic Patients Lack the DNA Content Abnormality That Is Characteristic of the Adenoma-Carcinoma Sequence Involved in the Development of Other Gastrointestinal Malignancies
Nonampullary duodenal adenomas (NADAs) develop sporadically or in the setting of a hereditary syndrome such as familial adenomatous polyposis (FAP). Although they are thought to progress into duodenal adenocarcinomas via an adenoma to carcinoma sequence similar to colorectal cancer, limited data suggested that they may be biologically dissimilar to colorectal adenomas. The clinicopathologic features of 71 patients diagnosed with NADAs (37 FAP and 34 sporadic) were analyzed. From the 71 patients, 89 NADA biopsies (42 FAP and 47 sporadic) were evaluated by DNA flow cytometry. Eighty-two samples showed low-grade dysplasia, an...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

DEK-AFF2 Carcinoma of the Sinonasal Region and Skull Base: Detailed Clinicopathologic Characterization of a Distinctive Entity
This study aims to characterize a larger cohort of carcinomas with DEK-AFF2 fusions to assess whether they truly constitute a distinctive entity. Among 27 sinonasal and skull base nonkeratinizing squamous cell carcinoma that were negative for human papillomavirus and Epstein-Barr virus, RNA sequencing identified DEK-AFF2 fusions in 13 cases (48%). Nine were centered in the nasal cavity, 2 in the middle ear/temporal bone, 1 in the nasopharynx, and 1 in the orbit. These tumors displayed recurrent histologic features including (1) complex endophytic and exophytic, frequently papilloma-like growth, (2) transitional epithelium ...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Expanding the Spectrum of EWSR1-NFATC2-rearranged Benign Tumors: A Common Genomic Abnormality in Vascular Malformation/Hemangioma and Simple Bone Cyst
A simple bone cyst (SBC) is a cystic bone lesion predominantly affecting young males. The cyst is lined by a fibrous membrane and filled with serosanguinous fluid. EWSR1/FUS-NFATC2 rearrangements were recently identified in SBC. We here report exactly the same rearrangement in 3 lesions diagnosed as vascular malformations of 2 elderly patients. In total, through Archer FusionPlex, fluorescence in situ hybridization and/or reverse transcriptase-polymerase chain reaction the EWSR1-NFATC2 rearrangement was identified in 6 of 9 SBC, 3 of 12 benign vascular tumors, and none of 5 aneurysmal bone cyst lacking USP6 fusion. Using f...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

High-grade Papillary Early Gastric Carcinoma With High Risk for Lymph Node Metastasis and Poor Prognosis: A Clinicopathologic Study of 96 Cases Among 1136 Consecutive Radical Gastrectomies
In conclusions, high-grade papillary EGC was associated with high frequencies of LVI, LNM, and poor prognosis, and thus unsuitable for endoscopic therapy, while low-grade papillary EGC showed clinicopathologic features and prognosis similar to well-moderately differentiated tubular EGC and may be treated endoscopically in appropriate clinical settings. (Source: The American Journal of Surgical Pathology)
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Florid Foreign Body-type Giant Cell Response to Keratin Is Associated With Improved Overall Survival in Patients Receiving Preoperative Therapy for Esophageal Squamous Cell Carcinoma
While most resection specimens from patients with neoadjuvantly treated esophageal squamous cell carcinoma show therapy-related changes in the form of inflammation and fibrosis, others harbor a florid foreign body-type giant cell response to keratin debris. The purpose of our study was to perform a detailed clinicopathologic analysis of these histologic types of treatment responses and correlate these findings with patient outcome. Clinical and pathologic parameters from 110 esophagogastrectomies were recorded and analyzed. Two main types of histologic responses were observed: inflammatory-predominant response (59%) and fl...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Pediatric Hepatocellular Adenomas: The Influence of Age and Syndrome on Subtype
Hepatocellular adenomas are rare in children. A large study focused on pediatric patients has not been undertaken. A natural language search was performed at 5 institutions for hepatocellular adenomas in patients younger than 21 years old. Clinical characteristics as well as immunohistochemical staining profile was reviewed and adenomas subtyped per standard classification. Patients were divided into prepubescent and postpubescent age group. Thirty-one patients were included. Eleven (35%) were male and 10 (32%) were prepubescent. Fifteen (54%) of 28 patients with known clinical histories had adenomas associated with a synd...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Wnt Family Member 9b (Wnt9b) Is a New Sensitive and Specific Marker for Breast Cancer
Confirming the tumor origin is often a diagnostic challenge in pathology and carries significant therapeutic impacts. Cytokeratin 7, estrogen receptor, and GATA binding protein 3 (GATA3) are well-established diagnostic markers frequently used to support a tumor’s breast origin. However, their specificities still have room to improve. Many nonbreast tumors express cytokeratin 7 and estrogen receptor, and urothelial tumors frequently express GATA3. There is a practical need for a new breast lineage marker that is sensitive and specific. Wnt family member proteins play critical roles in embryo development, tissue homeostasi...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Familial Adenomatous Polyposis–associated Traditional Serrated Adenoma of the Small Intestine: A Clinicopathologic and Molecular Analysis
This study describes the clinicopathologic and molecular findings of this type of adenoma in the small bowel of patients with FAP. We reviewed small bowel adenomas in 45 consecutive FAP patients to identify adenomas with zones showing slit-like serrations, cells with eosinophilic cytoplasm, ectopic crypt formation, and vesicular nuclei. Sporadic small bowel adenomas from 51 consecutive patients were also reviewed for adenomas with the same features. Of the 177 polyps from 45 FAP patients and 60 polyps from 51 nonsyndromic patients, 18 TSAs from 9 FAP patients (20%) and 10 TSAs from the sporadic group (19.6%) were identifie...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

CD34-negative Solitary Fibrous Tumor: A Clinicopathologic Study of 25 Cases and Comparison With Their CD34-positive Counterparts
CD34-negative solitary fibrous tumors (SFTs) are rare and have not been comprehensively studied. We retrospectively reviewed all cases of SFT confirmed with STAT6 immunohistochemistry and/or STAT6 gene fusion between 2013 and 2020 and collected pertinent clinicopathologic parameters. Of a total of 244 cases, 25 (10%) lacked CD34 expression by immunohistochemistry. Compared with CD34-positive SFT, CD34-negative SFT are more likely to arise in the head and neck area (32% vs. 24%, P=0.02) and present as metastatic disease (28% vs. 1%, P (Source: The American Journal of Surgical Pathology)
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research

Clinicopathologic Analysis of Primary Adrenal Diffuse Large B-Cell Lymphoma: A Reappraisal of 23 Japanese Patients Based on EBV Association and PD-L1 Expression in Tumor Cells
Primary adrenal diffuse large B-cell lymphoma (PA-DLBCL) is rare. We investigate 23 Japanese patients with PA-DLBCL to understand the clinicopathologic features and biological behavior of this disease. The 17 males and 6 females had a median age of 74 years (range: 40 to 86 y). Tumor cells harbored Epstein-Barr virus–encoded small RNA (EBER) in 9 (39%) samples, including samples from the 2 patients with methotrexate-associated B-cell lymphoproliferative disorder. Programmed cell death ligand 1 (PD-L1) expression was detected in tumor cells of 6 (26%) samples, including 1 EBER+ and 5 EBER− samples. Four (17%) patients...
Source: The American Journal of Surgical Pathology - November 15, 2021 Category: Pathology Tags: Original Articles Source Type: research