Florian N Loch, Carsten Kamphues, Freschta Rieger, Katharina Beyer, Wael Rayya, Christian Schineis, Frederick Klauschen, David Horst, Simon Schallenberg, Mihnea P Dragomir
{"title":"Stepwise Analysis of Resection Margin Impact on Survival and Distant Metastasis in Pancreatic Head Ductal Adenocarcinoma.","authors":"Florian N Loch, Carsten Kamphues, Freschta Rieger, Katharina Beyer, Wael Rayya, Christian Schineis, Frederick Klauschen, David Horst, Simon Schallenberg, Mihnea P Dragomir","doi":"10.1177/10668969241229342","DOIUrl":"10.1177/10668969241229342","url":null,"abstract":"<p><p>The prognostic role of tumor cells in pancreatic ductal adenocarcinoma (PDAC) of the pancreatic head with direct microscopic infiltration (DMI) or in close proximity (≤1 mm) to the resection margin (RM) remains unclear. This single-center, retrospective study included specimens from 75 patients who underwent oncological resection of pancreatic head PDAC between February 2013 and July 2020. Two pathologists independently re-measured the distance between tumors and the multiple RMs. The impact of RM involvement for DMI, tumor cells within ≤1 mm, in general, and for individual RMs on overall survival (OS) and development of distant pulmonary (PM) and hepatic (HM) metastasis was analyzed. DMI of RMs was significantly associated with a shorter OS (median 5 vs 19 months, <i>P</i> = .02). The presence of tumor cells within ≤1 mm of RMs yielded a negative impact on OS with a trend toward significance (median 9 vs 21 months, <i>P</i> = .09). DMI and tumor cells within ≤1 mm of the pancreatic transection margin (PRM), individually, had a significant negative impact on OS (median 4 vs 19 months and 6 vs 19 months, <i>P</i> < .05), but not for any other individual RM. RM involvement of ≤1 mm of only the vascular circumferential resection margin (VCRM) resulted in a shorter time to HM development (<i>P</i> = 0.05). DMI of the posterior circumferential resection margin (PCRM) and VCRM, individually, showed shorter time to PM (<i>P</i> < .05). Potential clinical considerations include extended intraoperative evaluation of the PRM (1 mm) and intensified preoperative prediction of R1 resection as a basis for neoadjuvant therapy.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1429-1440"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11528963/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139671756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Metastatic Granular Cell Tumor: A Rare Entity.","authors":"Dimosthenis Zylis, Stefania Kokkali, Michail Sofopoulos, Eirini Zografaki, Christine Vourlakou, Evangelia Skafida","doi":"10.1177/10668969241229347","DOIUrl":"10.1177/10668969241229347","url":null,"abstract":"<p><p>Granular cell tumor, which is thought to recapitulate a Schwann cell phenotype, is a very rare neoplasm that belongs to soft tissue tumors. It can be classified as benign, atypical or malignant, based on specific histological criteria, with the majority of cases exhibiting an indolent behavior. Its biology and clinical course are poorly understood and its optimal management is yet to be defined, given the rarity of cases. Here we describe an atypical granular cell tumor in the upper middle back skin that evolved after a thirty-year indolent period. Despite complete surgical removal, the patient experienced a recurrence, both local and in the lungs, following an aggressive clinical course. Data on management of metastatic disease are extremely scarce, comprised exclusively of case reports. Therefore, we administered to the patient systemic therapy according to soft tissue sarcoma guidelines, which led to disease progression, with fatal outcome. In conclusion, recurrent and/or metastatic granular cell tumor is a rare disease that can be life-threatening, for which response to different therapies is unknown. The biologic behavior of atypical and malignant granular cell tumor is quite different from its benign counterpart, evoking soft tissue sarcomas, and its diagnosis should alert clinicians. The role of adjuvant chemotherapy and radiation therapy in this setting should be explored, to limit disease recurrence.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1514-1519"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139697368","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":"Retroperitoneal Sarcomatoid Yolk Sac Tumor in a Chemotherapy-Naive Patient With Testicular Postpubertal Type Teratoma: A Rare Case Report With Emphasis on Molecular Features.","authors":"Bo Zhang, Payal Kapur, Prasad R Koduru, Liwei Jia","doi":"10.1177/10668969241231973","DOIUrl":"10.1177/10668969241231973","url":null,"abstract":"<p><p>Sarcomatoid yolk sac tumor is a very rare histologic type of testicular germ cell tumor and is mainly reported in testicular germ cell tumor patients who receive chemotherapy. Herein, we report an extremely rare concurrent retroperitoneal sarcomatoid yolk sac tumor in a man with a testicular postpuberal teratoma before he received chemotherapy. A 37-year-old man initially presented with a persistent abdominal pain. Subsequent imaging studies revealed a 9.6-cm retroperitoneal mass, and 2 testicular masses (3.1 cm and 0.9 cm in greatest dimension, respectively). His serum tumor markers were within normal ranges. His radical orchiectomy demonstrated a postpubertal type teratoma with an adjacent scarring nodule. Later, his retroperitoneal tumor showed spindle tumor cells embedded in predominantly myxoid and focally fibrous stroma with diffuse and strong immunoreactivity for keratin AE1/AE3, SALL4 and glypican 3. No tumor necrosis or brisk mitotic figures were observed. A diagnosis of sarcomatoid yolk sac tumor was rendered. Fluorescence in situ hybridization analysis of his retroperitoneal sarcomatoid yolk sac tumor revealed polysomy 12 and <i>MYC</i> amplification, whereas no evidence of isochromosome 12p [i(12p)], and DNA sequencing showed 6 mutations per megabase (muts/Mb), and the somatic alterations included <i>ARAF</i> amplification and <i>ATR</i> I774Yfs*5. Considering its rarity, sarcomatoid yolk sac tumor may pose diagnostic challenges. Therefore, relevant clinicoradiologic information and ancillary work up, including immunohistochemistry and molecular studies, may be helpful for the accurate classification. Our tumor further raises awareness of this rare event, expands the spectrum of its clinical presentation, and explores the molecular features.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1537-1543"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139912572","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}
Isidro Machado, Yanming Zhang, Meera Hameed, Sinchun Hwang, Aarti E Sharma, Mark H Bilsky, Konstantinos Linos
{"title":"<i>GRM1</i>-Rearranged Chondromyxoid Fibroma With FGF23 Expression: A Potential Pitfall in Small Biopsies.","authors":"Isidro Machado, Yanming Zhang, Meera Hameed, Sinchun Hwang, Aarti E Sharma, Mark H Bilsky, Konstantinos Linos","doi":"10.1177/10668969241229345","DOIUrl":"10.1177/10668969241229345","url":null,"abstract":"<p><p>The clinical, radiological, and histopathological features of chondromyxoid fibroma can sometimes resemble those of other benign or malignant tumors. Recently, recurrent <i>GRM1</i> rearrangements have been identified in chondromyxoid fibroma, and GRM1 positivity by immunohistochemistry has emerged as a dependable surrogate marker for this molecular alteration. Phosphaturic mesenchymal tumor is a rare tumor that often exhibits overexpression of fibroblastic growth factor 23 (FGF23) through various mechanisms. In this report, we present a case of <i>GRM1</i>-rearranged chondromyxoid fibroma that also exhibited FGF23 expression via in situ hybridization, posing significant diagnostic challenges during workup of the initial core biopsy. We hope that this case can serve as an educational resource, shedding light on a rare diagnostic pitfall.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1500-1507"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139671677","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":"Palmar Nodular Fasciitis Harboring a Novel <i>SREBF1::USP6</i> Fusion Gene.","authors":"Haider A Mejbel, Gene P Siegal, Shi Wei","doi":"10.1177/10668969241229330","DOIUrl":"10.1177/10668969241229330","url":null,"abstract":"<p><p>The diagnosis of low-grade fibroblastic/myofibroblastic tumors of acral sites can be challenging. These tumors encompass a diverse group of neoplasms with a spectrum of biologic potential ranges from benign to overtly malignant. They often demonstrate significant clinical, radiologic, and immunophenotypic overlap, in which the molecular phenotype may play an important diagnostic role to arrive at the final diagnosis. Herein, we report a case of soft tissue mass lesion presented on the palm of an adult patient for four months. Histologically, the tumor consisted of primarily low-grade spindle cells expressing smooth muscle actin. Molecular testing revealed a novel <i>SREBF1::USP6</i> fusion gene, confirming the final diagnosis of nodular fasciitis and ultimately expanding its molecular profile. This case highlights the diagnostic value of single, cost-effective, targeted molecular panel to arrive at an accurate diagnosis and provide helpful therapeutic information.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1494-1499"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139671751","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":"Testicular Primary Well-Differentiated Neuroendocrine Tumor: Clinicopathologic, Immunohistochemical, and Molecular Characterization of Two Patients.","authors":"Liwei Jia, Bo Zhang, Daniel Shen, Prasad R Koduru","doi":"10.1177/10668969241235315","DOIUrl":"10.1177/10668969241235315","url":null,"abstract":"<p><p>Well-differentiated neuroendocrine tumor rarely occurs as a testicular primary tumor, accounting for less than 1% of all testicular cancers, and is rarely reported with sufficient molecular profiles. After searching our departmental database (2003-2023), two testicular primary well-differentiated neuroendocrine tumors were identified in a 35-year-old man and a 23-year-old man, respectively, both of whom had normal serum level of tumor markers. Both tumors grossly exhibited solid, yellow-tan, and homogeneous appearance and histologically displayed a mixture of growth patterns, including organoid, tubular, cribriform, nests, cords, and single cells, were composed of eosinophilic tumor cells with salt-and-pepper chromatin and indistinct cell borders. Immunoreactivity for chromogranin and synaptophysin were detected, with Ki-67 labeling 9% and 2% of tumor cells on counting of 500 tumor cells, respectively. There was no germ cell neoplasia in situ in the background testicular parenchyma. Furthermore, fluorescence in situ hybridization failed to identify the presence of isochromosome 12p in both tumors. A panel-based next-generation sequencing was done in one of tumors and showed no reportable pathogenic variants with a mutation burden of 0.5 mutations per megabase. Although elevated mitotic figures (up to 6 per 10 high power fields), lymphovascular invasion and marked nuclear pleomorphism were present in this tumor, there was no evidence of disease detected in this patient via Dotatate positron emission tomography/computed tomography scan after the surgery. This report expands the spectrum of testicular primary well-differentiated neuroendocrine tumor. Considering its rarity, it may pose a diagnostic challenge or pitfall in certain clinical circumstances. In addition, the literature pertaining to this entity is herein reviewed.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1574-1581"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140174658","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}
Fnu Sonam, Miglena K Komforti, James W Jakub, Lauren F Cornell, Kristin A Robinson
{"title":"Rare Presentation of Papillary Carcinoma Within a Sentinel Lymph Node in a Patient With Ductal Carcinoma in Situ of the Breast: A Challenging but Useful Case Report Discussion Regarding Displacement or True Metastasis?","authors":"Fnu Sonam, Miglena K Komforti, James W Jakub, Lauren F Cornell, Kristin A Robinson","doi":"10.1177/10668969241235316","DOIUrl":"10.1177/10668969241235316","url":null,"abstract":"<p><p>Papillary carcinoma of the breast is a rare histologic subtype of invasive breast cancer. In contrast, ductal carcinoma in situ (DCIS) is a noninvasive, nonobligate precursor to invasive mammary carcinoma, confined within the terminal duct lobular unit of the breast and surrounded by an intact myoepithelial cell layer at the stromal/epithelial interface. We present an unusual case in which a patient with only DCIS of the breast exhibited papillary carcinoma within a sentinel lymph node, leading to questions about the origin of the lymph node lesion-whether it represents a true metastasis or iatrogenic displacement during diagnostic procedures. This case underscores the diagnostic challenges and clinical implications associated with such rare presentations.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1588-1594"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140158137","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}
João Costa, Sara Alves, Fernanda Silva, Filipa Leite, Carla Bartosch
{"title":"Gynecologic Adnexal Tumors and Tumor-Like Lesions in Children and Adolescents: Experience at a Cancer Center.","authors":"João Costa, Sara Alves, Fernanda Silva, Filipa Leite, Carla Bartosch","doi":"10.1177/10668969241232698","DOIUrl":"10.1177/10668969241232698","url":null,"abstract":"<p><p><i>Introduction.</i> Tumors and tumor-like lesions of the uterine adnexa in children and adolescents are uncommon but may carry devastating consequences. <i>Methods.</i> We conducted an observational retrospective cohort study, to describe patients aged 0 to 19 years diagnosed with tumors and tumor-like lesions of the uterine adnexa at our institution between 2000 and 2018. <i>Results.</i> Eighty-nine patients with 105 adnexal lesions were included. Thirty-seven (42%) patients presented with benign tumors, 13 (15%) with borderline tumors, 25 (28%) with malignant tumors and 14 (16%) with tumor-like lesions. Germ cell tumors (n = 45|43%) were the most frequent, followed by epithelial tumors (n = 30|29%). No significant differences were found in the age distribution of the lesions by malignant potential or histologic group. Most patients (n = 80|90%) were treated primarily with conservative surgery, including cystectomy (n = 25|28%) and unilateral oophorectomy/adnexectomy (n = 54|61%). Thirty-four (38%) underwent surgical staging (partial omentectomy and peritoneal biopsies). Twenty (23%) patients with borderline and malignant tumors were submitted to chemotherapy. Four (5%) patients with borderline or malignant tumors relapsed, one of whom died from disease. <i>Conclusion.</i> Gynecological lesions in children and adolescents encompass a wide range of rare histological tumor subtypes, requiring evaluation by experienced pathologists. Most tumors were diagnosed at early stages, with low relapse and mortality rates. Conservative management, with fertility sparing surgery and limited use of adjuvant chemotherapy, is of utmost importance.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1459-1469"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139912570","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}
Adrienne A Workman, Donald C Green, Edward G Hughes, Parth S Shah, Jeffrey M Cloutier, Jonathan D Marotti
{"title":"Chondrolipoma of the Breast: A Myofibroblastoma Variant or a Distinct Lesion?","authors":"Adrienne A Workman, Donald C Green, Edward G Hughes, Parth S Shah, Jeffrey M Cloutier, Jonathan D Marotti","doi":"10.1177/10668969241231975","DOIUrl":"10.1177/10668969241231975","url":null,"abstract":"<p><p>The entity commonly referred to as chondrolipoma is a rare and enigmatic breast lesion with unclear histogenesis and a complete lack of molecular characterization. It is uncertain whether it represents a hamartoma, choristoma, or a distinct neoplasm, including possibly a variant of mammary-type myofibroblastoma. We report two additional chondrolipomatous lesions of the breast. The lesions had varying histologic and immunohistochemical features similar to myofibroblastoma, including the loss of retinoblastoma (Rb) protein expression in one lesion. Molecular analysis by chromosomal microarray analysis performed on a second lesion did not demonstrate a loss of 13q14 or 16q typical of myofibroblastoma. Our findings further support the concept that at least a subset of breast lesions that historically have been classified as chondrolipoma are related to myofibroblastoma. However, the lack of myofibroblastoma-specific molecular alterations in one lesion suggests chondrolipomas may also have varying origins.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1525-1530"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139735139","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}
Sarvenaz Karamooz, Paula D Binsol, Jaya Ruth Asirvatham, Anjali Pargaonkar
{"title":"Metastasis of Clear Cell Renal Cell Carcinoma to Uterine Leiomyoma: First Case Report and Review of Literature.","authors":"Sarvenaz Karamooz, Paula D Binsol, Jaya Ruth Asirvatham, Anjali Pargaonkar","doi":"10.1177/10668969241231983","DOIUrl":"10.1177/10668969241231983","url":null,"abstract":"<p><p>Metastasis of clear cell renal cell carcinoma (clear cell RCC) to the gynecologic tract is infrequent, and involvement of the uterus is extremely rare. A review of the literature identified a total of 12 reported examples with metastasis to the uterine serosa (1), endometrium (5), cervix (5) and only one with metastasis to the myometrium. This report represents the first case of tumor-to-tumor metastasis involving a clear cell RCC with metastasis to a uterine leiomyoma. The patient was a 50-year-old woman status post-radical nephrectomy for newly diagnosed unilateral clear cell RCC (stage pT3a) with negative margins, who subsequently underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy for the incidental finding of multiple uterine masses measuring up to 14.5 cm suggestive of fibroid on pelvic ultrasound. The pathologic exam of the specimen was consistent with metastatic clear cell RCC (1.2 cm) to uterine leiomyoma, confirmed with keratin, vimentin, CD10, CA9, and PAX8 immunohistochemistry. The patient's postoperative course was uneventful, and no new lesions were identified at follow-up during the past 6 months.</p>","PeriodicalId":14416,"journal":{"name":"International Journal of Surgical Pathology","volume":" ","pages":"1552-1556"},"PeriodicalIF":0.9,"publicationDate":"2024-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139971855","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}