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Biology ◽  
2021 ◽  
Vol 10 (12) ◽  
pp. 1286
Author(s):  
Ourania Parra ◽  
Konstantinos Linos

“Cutaneous melanocytic tumor with CRTC1::TRIM11 fusion” (CMTCT) is a recently described entity belonging to the family of superficial tumors displaying melanocytic differentiation. Thirteen cases have been reported so far, on the head and neck, extremities, and trunk of adults of all ages (12 cases) and one in an 11-year-old child. Histopathologically, it is a nodular or multilobulated tumor composed of spindle and epithelioid cells arranged in nests, fascicles, or bundles that are surrounded by thin collagenous septa. By immunohistochemistry, the tumor shows variable immunoreactivity for S100-protein, SOX10, and MITF, as well as specific melanocytic markers such as MelanA and HMB-45. The neoplasm’s biologic behavior remains uncertain since the reported cases are limited and the follow-up is short (median 12 months). However, local recurrence and synchronous distant metastasis after 13 years of initial resection has been described in one case. Herein, we present a comprehensive literature review of CMTCT hoping to raise awareness among the dermatopathologists of this potentially novel entity.


2021 ◽  
Vol 22 (1) ◽  
pp. e37-e38
Author(s):  
Walid Ibn Essayed ◽  
Marcio Rassi ◽  
John H. Chi ◽  
Ossama Al-Mefty

2021 ◽  
Vol 10 (23) ◽  
pp. 5574
Author(s):  
Matthias P. Fabritius ◽  
Najib Ben Khaled ◽  
Wolfgang G. Kunz ◽  
Jens Ricke ◽  
Max Seidensticker

Intrahepatic cholangiocarcinoma is a highly aggressive malignancy with an increasing incidence in recent years. Prognosis is poor and most patients are not eligible for resection at the time of initial diagnosis due to the anatomic location, inadequate hepatic reserve, limiting comorbidities or metastatic disease. Several locoregional therapies from the field of interventional radiology exist for patients who are not amenable for surgery, or in case of local recurrence as a single treatment modality or combined with systemic treatment. To date, evidence is limited, with most conclusions drawn from single-center studies with small patient cohorts, often treated in the salvage situation or for local recurrence after initial resection. Nevertheless, the results are promising and suggest a survival benefit in selected patients. This narrative review focuses on the use of different locoregional treatment options for intrahepatic cholangiocarcinoma.


2021 ◽  
pp. 1-7
Author(s):  
Henrik Nienhüser ◽  
Markus W. Büchler ◽  
Martin Schneider

<b><i>Background:</i></b> Recurrence after resection of pancreatic cancer occurs in up to 80% of patients in the first 2 years after complete resection. While most patients are not eligible for surgical treatment due to disseminated disease, a certain group of patients can be evaluated for re-resection of local recurrence. This review summarizes the current literature on surgical treatment of recurrent pancreatic cancer and potential prognostic factors. <b><i>Summary:</i></b> Re-resection of recurrent pancreatic cancer provides a significant survival benefit to selected patients with acceptable procedure-related mortality. Median overall survival after re-resection of recurrent pancreatic cancer is up to 28 months. The most relevant clinical parameters associated with a prognostic benefit are young patient age (&#x3c;65 years), time to initial resection (&#x3e;10 months), and preoperative chemotherapy before re-resection. Molecular markers are currently under investigation and might help to improve patient selection in the future. <b><i>Key Message:</i></b> Re-resection of recurrent pancreatic cancer is safe and feasible in experienced hands. Selected patients benefit from surgical treatment, but future studies are needed to identify reliable prognostic markers predicting survival.


2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi99-vi100
Author(s):  
Michael Zhang ◽  
Cliff Wang ◽  
Zheng Pan ◽  
Benjamin Yuen ◽  
Songming Peng ◽  
...  

Abstract The prospect of using immunotherapy for IDH-mutant LGGs has been daunting given the immune-poor microenvironment and low mutational burden. We hypothesized that LGG-targeting T cells might still be present at low frequency and with limited regional infiltration into the tumor. To improve sensitivity, we combined high-density multi-region tumor sampling with high-throughput neoantigen-T cell screening for a patient with WHO Grade II diffuse astrocytoma who eventually progressed, at second recurrence, to anaplastic astrocytoma. We performed maximal-anatomic sampling from 10 distinct regions of the tumor at the initial resection, as well as single sampling at first recurrence, for exome-based prediction of clonal and subclonal expressed neoantigens, RNAseq-based estimation of regional immune cell composition, and T cell receptor (TCR) beta deep sequencing. Based on our predictions, we then generated a barcoded library of patient-specific peptide-HLA multimers loaded with predicted neopeptides. Using this library, neoantigen-specific CD8 T cells were captured and isolated from patient peripheral blood and subjected to single cell TCR sequencing. We screened patient-derived peripheral blood drawn two years after initial resection and identified five T cell clones recognizing three LGG neoepitopes. Two neoepitopes were derived from truncal, tumor-wide mutations, including a truncating splice-site mutation in TP53 and a missense mutation in MRPL46. Each of these neoepitopes were recognized by two distinct TCRs, consistent with TCR convergence. A third neoepitope was derived from a subclonal MRPL46 mutation. Using the TCR beta sequence as a molecular barcode, TCRs specific to the 2 clonal neoepitopes, but not the subclonal neoepitope, were detectable in the glioma and blood at initial resection, as well as in the recurrent glioma. In summary, we demonstrate the existence and persistence of neoantigen-targeting T cells within the blood and tumor of an IDH-mutant LGG patient. These findings suggest a feasible methodology to develop personalized T cell-based immunotherapies for IDH-mutant LGGs.


2021 ◽  
pp. 1-9
Author(s):  
Hirotaka Hasegawa ◽  
Kunal Vakharia ◽  
Lucas P. Carlstrom ◽  
Jamie J. Van Gompel ◽  
Colin L. W. Driscoll ◽  
...  

OBJECTIVE The authors’ objective was to reevaluate the role of microsurgery for epidermoid tumors by examining the associations between extent of resection (EOR), tumor control, and clinical outcomes. METHODS This was a retrospective study of patients with microsurgically treated intracranial epidermoid tumors. The recurrence-free and intervention-free rates were calculated using the Kaplan-Meier method. EOR was graded as gross-total resection (GTR) (total resection without residual on MRI), near-total resection (NTR) (a cyst lining was left in place), subtotal resection (STR) (> 90% resection), and partial resection (PR) (any other suboptimal resection) and used to stratify outcomes. RESULTS Sixty-three patients with mean clinical and radiological follow-up periods of 87.3 and 81.8 months, respectively, were included. Sixteen patients underwent second resections, and 5 underwent third resections. The rates of GTR/NTR, STR, and PR were 43%, 35%, and 22%, respectively, for the initial resections; 44%, 13%, and 44% for the second resections; and 40%, 0%, and 60% for the third resections (p < 0.001). The 5- and 10-year cumulative recurrence-free rates after initial resection were 64% and 32%, respectively. When stratified according to EOR, the 10-year recurrence-free rate after GTR/NTR was marginally better than that after STR (61% vs 35%, p = 0.130) and significantly better than that after PR (61% vs 0%, p < 0.001). The recurrence-free rates after initial microsurgery were marginally better than those after second surgery (p = 0.102) and third surgery (p = 0.065). The 5- and 10-year cumulative intervention-free rates after initial resection were 91% and 58%, respectively. When stratified according to EOR, the 10-year intervention-free rate after GTR/NTR was significantly better than that after STR (100% vs 51%, p = 0.022) and PR (100% vs 27%, p < 0.001). The 5-year intervention-free rate after initial surgery was marginally better than that after second surgery (52%, p = 0.088) and significantly better than that after third surgery (0%, p = 0.004). After initial, second, and third resections, permanent neurological complications were observed in 6 (10%), 1 (6%), and 1 (20%) patients, respectively. At the last follow-up visit, 82%, 23%, and 7% of patients were free from radiological recurrence after GTR/NTR, STR, and PR as the initial surgical procedure, respectively. CONCLUSIONS GTR/NTR seems to contribute to better disease control without significantly impairing functional status. Initial resection offers the best chance to achieve better EOR, leading to better disease control.


Author(s):  
David P. Bray ◽  
Bryan E. Buster ◽  
Joseph W. Quillin ◽  
Robert H. Press ◽  
Bree R. Eaton ◽  
...  

Abstract Introduction Atypical meningiomas (AM) are meningiomas that are more aggressive than their grade-I counterparts and have a higher rate of recurrence. The effect of adjuvant radiotherapy (ART) on AM of the skull base is not defined. Methods A retrospective review of all AM's of the skull base primarily resected at our institution from 1996 to 2018 was completed. ART was defined as radiotherapy (RT) that occurred within 6 months of initial resection, regardless of Simpson's grade. Minimum time length of follow-up after resection was 2 years. Statistical analysis was performed using SAS. Results There were a total of 59 skull base–located (SBL) AMs resected at our institution from 1996 to 2018. The average age of our cohort was 53.2 years. Gross total resection, defined as Simpson's grades I to III resection, was achieved in 36 (61%) of cases. Thirty-five of 59 (59%) patients received ART. Recurrence was observed in 14 patients (24%), and mean time to recurrence was 63.8 months. Patients who received ART had a lower observed rate of recurrence (8 vs. 46%); however, time to recurrence was not significantly different between the two populations. Conclusion We observe that AM in the skull base location have higher recurrence rates than we would expect from grade-I meningioma. These data suggest that ART may offer benefit to the overall observed frequency of recurrence of SBL AM; however, the time to recurrence between patients who received ART and those who did not was not statistically significant in survival analysis.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Joshua Byers ◽  
Hong Yin ◽  
Heather Rytting ◽  
Suzanna Logan ◽  
Mai He ◽  
...  

AbstractAngiomatoid fibrous histiocytoma (AFH) is a rare tumor of intermediate malignancy. Treatment options for unresectable and/or metastatic tumors are very limited. Immunotherapy with PD-1/PD-L1 inhibitors may be worth exploring. The aim of this study was to evaluate the expression of PD-L1 in AFHs. PD-L1 expression was assessed on 36 AFHs from 36 pediatric patients by immunohistochemical staining of PD-L1 (clone 22C3). Positivity was defined as membranous expression in ≥ 1% of either tumor or immune cells. The correlations between PD-L1 expression and clinicopathologic features were assessed. Two patients had lymph node metastasis. All patients underwent surgical resection; three of them also had systemic chemotherapy. Three patients had recurrence after initial resection; all patients were alive with a median follow-up of 2.5 years. Overall, twenty-two (61%) tumors were positively stained for PD-L1 and positivity was seen on both tumor and immune cells in eighteen of the 22 tumors. A positive correlation was found between tumor cell PD-L1 expression and CD8+ T-cell infiltration. There were no statistically significant differences between the status of PD-L1 expression and the clinicopathological features assessed. PD-L1 expression was identified in 61% of AFHs with a predominantly adaptive pattern. Our findings provide a rationale for future studies evaluating the potential of checkpoint immunotherapy for patients with unresectable and/or metastatic tumor.


2021 ◽  
Vol 9 ◽  
pp. 232470962110274
Author(s):  
Prasanth Lingamaneni ◽  
Hisham Laswi ◽  
Aleksandar Krbanjevic ◽  
Krishna Moturi ◽  
Vatsala Katiyar ◽  
...  

Merkel cell carcinoma (MCC) is a highly aggressive cutaneous neuroendocrine carcinoma, frequently associated with distant metastasis. However, recurrence of MCC manifesting with only pancreatic involvement is exceedingly rare. A 53-year-old man presented to our institution with abdominal discomfort 3 months after initial resection of chest wall MCC. Imaging revealed lesions in the pancreas and peripancreatic lymph nodes. Pathology obtained through endoscopic ultrasound confirmed recurrence of MCC. He underwent chemotherapy with cisplatin and etoposide, resulting in a complete resolution of the pancreatic lesions. Unfortunately, he passed away from sudden cardiac arrest while being in remission from MCC. Immunohistochemistry is crucial in differentiating MCC from primary pancreatic glandular and neuroendocrine tumors. While there are no definitive guidelines in the management of pancreatic lesions associated with MCC, checkpoint inhibitor immunotherapy is increasingly being used.


2020 ◽  
Vol 6 (4) ◽  
pp. 489-496
Author(s):  
Eva Sailer ◽  
Frens-Steffen Krause ◽  
Volkmar Tauber ◽  
Wolfgang Schimetta ◽  
Sebastian Alfred Graf

BACKGROUND: Repeat transurethral resection of bladder tumor is recommended when certain risk constellations are present on initial resection. Current evidence is conflicting, leading to dissenting recommendations in multinational guidelines around the world. Photodynamic diagnostics (PDD) is a tool which has been shown to increase diagnostic accuracy, but evidence is still lacking if this may permit omission of repeat resections in certain cases. OBJECTIVE: To evaluate whether the use of photodynamic diagnostics has an impact on resection quality and residual tumor rate, and to explore which parameters may have an impact on the necessity of repeat transurethral resections. METHODS: We retrospectively evaluated 373 patients in the timeframe of ten years, in whom a repeat transurethral resection of bladder tumor has been performed following initial resection at our department. About half of those resections were performed using photodynamic diagnostics. RESULTS: When PDD was used, more tumor mass was revealed and resected, but the shown trend toward a lower residual tumor rate was non-significant. Muscularis was shown more often on PDD resections. While being a rare occurrence, upstaging on repeat resection happened significantly less often after initial PDD use. Furthermore, tumor size and multifocality significantly influenced residual tumor rate in Ta high-grade stage. CONCLUSIONS: PDD use may lead to a more accurate initial staging but this may not have an impact on short-term residual tumor rate. Tumor size and multifocality should be granted more weight in the decision-making process as when to perform a repeat resection.


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