International Journal of Surgical Pathology最新文献

{"title":"The \"Pigmented Side\" of Nerve Sheaths: Malignant Melanotic Nerve Sheath Tumor.","authors":"Raduan Ahmed Franca, Rosa Maria Di Crescenzo, Lorenzo Ugga, Rosa Della Monica, Elena D'Avella","doi":"10.1177/10668969241295689","DOIUrl":"10.1177/10668969241295689","url":null,"abstract":"<p><p>Malignant melanotic nerve sheath tumor (MMNST) represents a highly aggressive neoplasm arising both in peripheral and cranial nerves. It accounts for < 1% of all nerve sheath tumors, but the real incidence may not be well defined yet because of the evolution of its nomenclature. To date, it is considered a distinct tumor type, no longer as the pigmented variant of schwannoma, with a different clinical course and biological behavior. MMNSTs exhibit a specific genetic hallmark related to the <i>PRKAR1A</i> gene, which explains the major incidence in Carney Complex-affected patients. One of the more frequent localizations is the paravertebral region, where it poses diagnostic concerns with both primary tumors arising from soft tissues and the meningeal covering, as well as metastatic ones (ie, melanoma). Herein we present a patient with an MMNST accompanied by the main clinical, radiological, histopathological, and molecular findings, stressing the need for a multidisciplinary diagnostic approach. To the best of our knowledge, this is the first report of proton beam therapy for MMNST. We also performed a literature review to collect and compare the more recent data in English literature and to highlight the \"keep-in-mind\" concepts to apply in a multidisciplinary diagnostic algorithm, with a focus on histopathology and related pitfalls.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1068-1081"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142675741","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Rare Neck Lesion in Children: Bilateral Chondrocutaneous Branchial Remnant.","authors":"Tunç Tığlı, Diclehan Orhan, Tutku Soyer","doi":"10.1177/10668969241291898","DOIUrl":"10.1177/10668969241291898","url":null,"abstract":"<p><p>Cervical chondrocutaneous branchial remnant is a rare congenital developmental anomaly typically located on the lateral neck. Histologically, it has the appearance of an accessory tragus demonstrating a central cartilaginous core with surrounding fibrosis located in the subcutaneous tissue. In order to address the clinical and therapeutic aspects of cervical chondrocutaneous branchial remnants in children, a 6-year-old boy who presents with bilateral hard cervical masses at the lower region of the neck is presented. Chondrocutaneous branchial remnant is a very rare cervical lesion in children, and surgical excision is the only choice of treatment. Cervical chondrocutaneous branchial remnant has been associated with a variety of congenital anomalies, particularly involving the auditory, cardiovascular, and visual systems. Despite bilateral occurrence of cervical chondrocutaneous branchial remnant, our patient had no cardiac or genitourinary anomalies.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"951-955"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142620343","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Olıgosarcoma: A Rare Case Report Wıth Dıstınct Features.","authors":"Begum Calim Gurbuz, Tuce Soylemez Akkurt, Fatmagul Kusku Cabuk, Fahir Sencan, Bekir Tugcu, Ayca Ersen Danyeli","doi":"10.1177/10668969241291891","DOIUrl":"10.1177/10668969241291891","url":null,"abstract":"<p><p>Oligosarcoma is a recently identified entity characterized by sarcomatous changes originating from oligodendroglioma. As of our current understanding, sarcomatous components are infrequent in glial tumors. The World Health Organization (WHO) classification describes sarcomatous features as a rare pattern in grade 3 oligodendrogliomas. In this report, we present a 42-year-old man diagnosed with oligosarcoma. The patient initially presented with a lesion in the right parietotemporal area 9 years ago, and the pathological diagnosis was oligodendroglioma. Nine years later, a recurrent lesion in the same area was observed. Histomorphological evaluation of the recurrent lesion revealed distinct glial and sarcomatous components. The diagnosis of oligosarcoma was made based on histologic assessment; however, additional histochemical (reticulin-rich sarcomatous area), immunohistochemical, and molecular evaluations were also conducted. Immunohistochemical marker expression patterns in oligosarcoma have been reported variably in the literature. In our patient, the sarcomatous component exhibited p53 and OLIG2 immunohistochemical expression. Molecular analysis revealed IDH and TERT mutations, as well as 1p/19q and CDKN2A deletions.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"964-969"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142675724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Malignant Esophageal Glomus Tumor With <i>CARMN::NOTCH2</i> Fusion: An Additional Tumor Underscoring the Frequent Tendency of Esophageal Glomus Tumors for Aggressive Behavior.","authors":"Tamás Pancsa, Petr Martínek, Michael Michal","doi":"10.1177/10668969241291887","DOIUrl":"10.1177/10668969241291887","url":null,"abstract":"<p><p>Malignant glomus tumors are rare tumors of pericytic origin with a propensity to develop in the upper gastrointestinal tract. Hereby we demonstrate a tumor of a 20-year-old man, who presented with dysphagia and an exophytic esophageal mass. Histologic examination of the resected mass revealed a multinodular tumor in the esophageal wall composed of epithelioid cells showing nesting and monomorphic atypia, staghorn vessels and scanty stroma. Immunohistochemically, the neoplastic cells were positive for SMA, and H-caldesmon, while desmin was negative. Collagen IV and laminin decorated a dense intercellular basal membrane meshwork. RNA-sequencing using TruSight RNA Pan-Cancer Panel revealed a <i>CARMN::NOTCH2</i> fusion, that is a recurrent, frequently described and so far specific genetic alteration in glomus tumors. In spite of the adjuvant chemotherapy regimens, the patient died of disseminated metastatic disease 2 years after the diagnosis. Our patient presentation and the previous reports in the literature highlight the frequently aggressive behavior of glomus tumors arising in the esophagus.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"987-991"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142620643","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"BCOR-ITD Rearranged Sarcoma in the Mandible of a 5-Year-Old Child: A Case Report Highlighting Diagnostic Distinction From Odontogenic Fibromyxoma.","authors":"Sunayana Misra, Seema Rao, Sonia Badwal, Manas Kalra","doi":"10.1177/10668969241291897","DOIUrl":"10.1177/10668969241291897","url":null,"abstract":"<p><p>The 5th edition of the World Health Organization (WHO) classification of soft tissue and bone has recognized three distinct groups among Ewing-like sarcomas, namely, CIC-rearranged sarcoma, sarcomas with <i>BCOR</i> genetic alterations, and round cell sarcomas with EWSR1:: non-ETS fusions. Sarcomas with <i>BCOR</i> genetic alterations are a distinct clinicopathological group of high-grade tumors, representing 5% of small round cell tumors. BCOR-ITD rearranged tumors commonly manifest as spindle cell sarcomas and many of them display low cellularity with monomorphous cell morphology and myxoid background resembling benign fibroblastic tumors. We present an intriguing case report of a 5-year-old boy diagnosed with a moderately cellular mandibular spindle cell tumor exhibiting BCOR-ITD rearrangement which was initially misdiagnosed as an odontogenic fibromyxoma. This case report illustrates the histological and immunophenotypic findings of BCOR-ITD rearranged sarcoma, requiring a comprehensive immunohistochemical panel and additional molecular tests for accurate diagnosis.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"976-981"},"PeriodicalIF":0.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142620458","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"In Situ Encapsulated/Solid Papillary Breast Carcinoma With Lobular Differentiation.","authors":"Mariel Molina, Gustavo Moreno, Julie M Jorns","doi":"10.1177/10668969251343222","DOIUrl":"https://doi.org/10.1177/10668969251343222","url":null,"abstract":"","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969251343222"},"PeriodicalIF":0.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191815","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuroendocrine Tumor as a Somatic-Type Malignancy in Metastatic Teratoma.","authors":"Benjamin Chu, Jiaoti Huang","doi":"10.1177/10668969251343220","DOIUrl":"https://doi.org/10.1177/10668969251343220","url":null,"abstract":"<p><p>Testicular germ cell tumors represent less than 1% of all cancer diagnoses in men. Postpubertal-type teratomas are a form of nonseminomatous germ cell tumor derived from 2 or more embryonic germ cell layers. Rarely, these tumors have been shown to develop somatic-type malignancy, where a nongerm cell malignancy such as carcinoma or sarcoma arises in a germ cell tumor. Neuroendocrine tumors as a somatic-type malignancy in teratomas are extremely rare. Here, we report a high-grade neuroendocrine tumor arising within a metastatic teratoma in a 25-year-old man.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969251343220"},"PeriodicalIF":0.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191824","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Revisiting the Role of <i>Epstein-Barr Virus</i> (EBV) in Gastric Cancer: A Case Study of EBV in Normal, Dysplastic, and Malignant Gastric Epithelium.","authors":"Juhi Devendra Mahadik, Charles E Middleton, David Saulino, Michael Feely, Tiffany Chua, Ansley Hong, Ibrahim Nassour, Naziheh Assarzadegan","doi":"10.1177/10668969251343218","DOIUrl":"https://doi.org/10.1177/10668969251343218","url":null,"abstract":"<p><p>Gastric carcinoma represents a significant global health challenge, with <i>Epstein-Barr virus</i> (EBV) playing a key role in certain subtypes of the disease. However, the exact mechanisms by which EBV contributes to gastric carcinogenesis remain poorly understood. This case report presents a 78-year-old female patient with a history of chronic atrophic gastritis, intestinal metaplasia, and multiple hyperplastic gastric polyps, exhibiting both low- and high-grade dysplasia, who ultimately developed multifocal intramucosal adenocarcinoma and underwent subtotal gastrectomy. The gastrectomy specimen revealed numerous polyps of varying sizes throughout the gastric mucosa. Epstein-Barr encoding region in-situ hybridization testing demonstrated EBV expression not only within the neoplastic gastric epithelium but also in the nonneoplastic epithelium and gastric polyps, an unusual finding further confirmed by polymerase chain reaction testing for EBV DNA. While the role of EBV in some gastric carcinomas is well established, the detection of EBV in nonneoplastic gastric epithelium highlights the possibility that latent EBV infection may contribute to gastric carcinogenesis, particularly in the setting of chronic gastritis and intestinal metaplasia. This case report underscores the need for a deeper understanding of EBV's potential impact on cellular processes, including genome-wide hypermethylation, and calls for further research into the mechanisms underlying EBV-associated gastric carcinoma.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969251343218"},"PeriodicalIF":0.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Crystal-Storing Histiocytosis of the Duodenum in a Patient With IgG-Lambda Multiple Myeloma.","authors":"Shunsuke Koga, Zhaohai Yang, Zahra Alipour","doi":"10.1177/10668969251343639","DOIUrl":"https://doi.org/10.1177/10668969251343639","url":null,"abstract":"<p><p>Crystal-storing histiocytosis is a rare condition characterized by the accumulation of crystalline immunoglobulins within histiocytes, often associated with plasma cell disorders such as multiple myeloma. We report a 70-year-old man with a history of IgG-lambda multiple myeloma, systemic amyloidosis, and end-stage renal disease managed with peritoneal dialysis and long-term lanthanum carbonate therapy for hyperphosphatemia, who presented with gastrointestinal bleeding. Endoscopy revealed nodular erythematous mucosa in the duodenum, and biopsies showed histiocytosis in the lamina propria. The histiocytes showed abundant cytoplasm containing periodic acid-Schiff-positive diastase-resistant intracellular material. Immunohistochemical staining revealed diffuse CD68 positivity and lambda light-chain restriction of the storage material in the histiocytes, supporting a diagnosis of crystal-storing histiocytosis. Differential diagnoses, including Whipple disease, mycobacterial infections, lanthanum deposition, amyloidosis, and xanthoma, were excluded based on histological findings and clinical history. This report highlights the importance of recognizing crystal-storing histiocytosis in uncommon sites such as the duodenum, especially in the context of plasma cell dyscrasias.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969251343639"},"PeriodicalIF":0.9,"publicationDate":"2025-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144181639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Simple Nephrectomy for Non-Functioning Kidney: An Institutional Experience with Non-Neoplastic and Incidentally Detected Neoplastic Lesions.","authors":"Maitrayee Roy, Akshay Bali, Ayushi Kediya, Vishesh Dhawan, Vijay Shrawan Nijhawan","doi":"10.1177/10668969251339803","DOIUrl":"https://doi.org/10.1177/10668969251339803","url":null,"abstract":"<p><p><i>Introduction.</i> Chronic kidney diseases are attributed to a variety of etiologies, including diabetes mellitus, hypertension, vascular diseases, cystic kidney diseases, chronic tubulo-interstitial diseases, chronic pyelonephritis, and glomerular diseases. Simple nephrectomy is indicated when the loss of renal function in a unilateral non-functional kidney is permanent in a persistently symptomatic patient. Uncommonly, a non-functional kidney may develop neoplasm unsuspected pre-operatively and be diagnosed only during histopathological examination of nephrectomy specimen. <i>Material and methods.</i> We examined 275 simple nephrectomies performed between 2019 and 2024 in patients with non-functional kidneys confirmed by DMSA scan. All nephrectomies done for diagnosed or even suspected tumors by pre-operative imaging were excluded from the study. <i>Results.</i> Chronic pyelonephritis (79.3%) was the most common histopathological diagnosis, followed by xanthogranulomatous pyelonephritis (8.7%) and granulomatous pyelonephritis (5.4%). In five (1.8%) patients, microscopy revealed non-specific chronic interstitial inflammation and interstitial fibrosis. One patient (0.4%) was diagnosed with large solitary pyelogenic cyst and renal nocardiosis. Eleven patients (4%) were diagnosed with neoplasms. Clear cell renal cell carcinoma was the most common diagnosis (27.2%), followed by urothelial carcinomas of renal pelvis (18.2%), one patient each of collecting duct carcinoma, squamous cell carcinoma of renal pelvis, and multilocular cystic renal neoplasm of low malignant potential. Three patients had papillary adenomas. <i>Conclusion.</i> Diligent histopathological examination of simple nephrectomy is crucial in establishing the final diagnosis with important further therapeutic implications. This is especially important in neoplastic pathologies developing in non-functional kidneys that may rarely be unsuspected by pre-operative imaging and diagnosed only during histopathological evaluation.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"10668969251339803"},"PeriodicalIF":0.9,"publicationDate":"2025-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144142121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}