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BACKGROUND The subtonsillar transcerebellomedullary approach (STA) has been well established as one of the surgical options to access lesions in the foramen of Luschka. The middle cerebellar peduncle has been defined as the superior limit of this corridor, and tumors extending beyond this landmark were often approached through combined accesses. METHOD We illustrate the access to the suprasellar cistern, from a purely STA for a large tumor arising from the foramen of Luschka. CONCLUSION This manuscript demonstrates the STA as a valid alternative for certain tumors of the foramen of Luschka, which extend into crural and suprasellar cisterns.

Epithelioid hemangioendothelioma is a rare malignant vascular sarcoma. Here we present a patient with a very large tumor arising from the superior vena cava, in whom a resection with negative margins was accomplished using venovenous bypass and bovine pericardial patch reconstruction of the superior vena cava.

Primary sarcomas of the vulva are rare, with an incidence of 1.5% to 5% of all vulvar malignancies. There are only 41 cases described in the English literature of proximal epithelioid sarcoma of the vulva, which also constitutes a highly aggressive neoplasm with a high rate of recurrence and metastasis. We are reporting the first case of vulvar epithelioid sarcoma reported in our country, Peru, and Latin America. We present the case of a 53-year-old woman with no significant history who presented a large tumor mass of 25 cm in the left hemivulva of 2 years of evolution, associated with intense pain. She underwent radical surgical resection with left inguinopelvic lymphadenectomy and reconstruction of Rectus Abdominal Muscle Myocutaneous Flaps (TRAM). Keywords: Epithelioid sarcoma, vulva, sarcoma

While benefits of neurosurgical intraoperative ultrasound (IOUS) are reported frequently, this method still has some significant pitfalls, which are described less often. However, sufficient knowledge on dealing with IOUS drawbacks, particularly various image artifacts, is important for successful surgery. We report a case of failed IOUS-guided pediatric cerebellar pilocytic astrocytoma resection, incorrectly evaluated as gross total resection according to IOUS. A large tumor residuum was left in place. Successful IOUS-guided reoperation using new IOUS technology and appropriate ultrasound imaging technique are described. The most probable reasons for initial resection failure and crucial points of reoperation, predominantly dealing with IOUS artifacts, are discussed. Neurosurgeons should be aware of IOUS limitations and have sufficient knowledge about how to overcome them before adopting routine use of this intraoperative imaging modality.

Histoid leprosy is a rare form of multibacillary leprosy, characterized by the presence of papules, plaques, or nodules whose appearance is keloid-like, skin colored, or erythematous. Fusiform cells are the main histopathological feature. Due to the fact that it can simulate other dermatological lesions, for example, dermatofibroma and neurofibroma, it constitutes a diagnostic challenge for clinicians and pathologists. It is a bacilliferous form of leprosy, and it plays an important role in disease transmission. A case of a patient with histoid leprosy living in the Northeast Region of Brazil is reported.

Diarrhea is a common condition seen among soldiers in both garrison and deployed environments. Although the vast majority of soldiers with diarrhea will recover uneventfully with supportive care, clinicians should also maintain suspicion for less common causes and perform a thorough physical exam. We report the case of a young, healthy soldier with chronic diarrhea and progressively worsening abdominal distention that began during his deployment to Honduras who was subsequently found to have a large intra-abdominal desmoid tumor. Desmoid tumor is a rare and benign neoplasm that typically appears on the extremity, abdominal wall, intra-abdominal space, and occasionally in the chest wall. This tumor may be associated with abdominal distension and gastrointestinal complaints. A large tumor can compress organs, causing local tissue damage and, in rare cases, death.

Lymphocyte-predominant Hodgkin’s lymphoma (LPHL) that involving the breast and axillary tissue is very rare. We report a case of this subtype of Hodgkin’s lymphoma (HL) in right breast mass of a 48-year-old man, who presented with a 20 years history of this breast painless mass; but over the past year there has been an increase in the size of the mass, with pain but no discharge. Pathology report after immunohistochemistry (IHC) confirmed the LPHL.

Solid pseudopapillary neoplasms (SPNs) of the pancreas have low malignant potential. However, malignant SPNs are not fully understood.To evaluate risk factors affecting malignant potential, the clinicopathologic features of 375 surgically resected SPNs were compared.Fifty (13.3%) had malignant histologic features. Twenty-seven and 22 had perineural and lymphovascular invasions, respectively. Adjacent organ invasion was noted in 9 cases. Recurrence occurred in 8 cases. The median recurrence time after surgical resection was 67 months and was associated with a higher pT category (P = 0.001), lymphovascular invasion (P 0.001), and synchronous metastasis (P 0.001). SPN patients with malignant histologic features had worse recurrence-free survival (RFS; 10-year survival rate, 73.2%) than those without malignant histologic features (96.3%; P = 0.01). Patients with a higher pT category (P = 0.04), synchronous metastasis (P 0.01), and lymphovascular invasion (P 0.01) had worse RFS. Lymphovascular invasion (P = 0.042) and a higher T category (P = 0.002) were poor prognostic factors for recurrence.Lymphovascular invasion and a higher T category were worse prognostic factors for recurrence in SPN patients with malignant histologic features. For SPN patients with malignant histologic features, a longer follow-up may be required.

Thyroid cancer (TC) is a common malignant tumor of the endocrine system. Currently, it is an urgent problem to be solved to explore the relevant molecular targets and molecular mechanisms for the occurrence and development of TC. miR-93 plays a role in regulating tumors in multiple cancers, but its expression in TC and its effect on proliferation,migration and apoptosis of TC cells have not been reported so far. In this paper, RT-qPCR was used to detect the expression of miR-93 and LATS2. Cell transfection techniques was used to interfere with or overexpress miR-93 and interfere with the expression of LATS2, and RT-qPCR was used to detect transfection efficiency. CCK-8 technique and clone formation assay were used to detect cell survival. Transwell and wound healing assay was used to detect cell migration and invasion, and western blot was used to detect the expression of invasion and migration related proteins MMP2 and MMP9. The apoptosis rate was detected by flow cytometry, and the expression of apoptotic proteins bcl-2, Bax, caspase3/9 and pro-caspase-3/9 was detected by western blot. The results showed that miR-93 was significantly increased in TC cell lines, especially in TPC1 cells. Interference with the expression of miR-93 inhibited proliferation, invasion, and migration, and promoted the apoptosis of TPC1 cells. In addition, we also found that interfering with the expression of miR-93 can promote the expression level of LATS2 in TPC1 cells. Interference with the expression of LATS2 can inhibit the effect on the proliferation, invasion, migration and apoptosis of TPC1 cells after miR-93 interference. In conclusion, interference with the expression of miR-93 inhibits the proliferation and migration and promotes apoptosis of TC cells through targeting LATS2.

INTRODUCTION Ventricular tachycardia (VT) normally occurs from an abnormal structural substrate. CASE We report a case in which VT was caused by a large tumor in the interventricular septum. Surgical intervention was not an option due to the location of the tumor and its proximity to the coronary arteries. CONCLUSION The patient underwent ablation and upgrade to CRT before ultimately receiving a heart transplant.

The purpose of this study was to evaluate the prognostic values of pathological tumor size and preoperative blood-based inflammation biomarkers, including the neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and monocyte-lymphocyte ratio (MLR), in upper tract urothelial carcinoma (UTUC).From 2007 to 2017, retrospective data of 449 patients with UTUC who underwent radical nephroureterectomy were assessed. Use of Kaplan-Meier and univariable/multivariable analyses evaluated the effect of preoperative blood-based inflammation biomarkers on overall (OS), cancer-specific (CSS), and progression-free survival (PFS) in pathological tumor sizesand ≤3 cm.Kaplan-Meier analyses showed that high-level NLR, PLR, or MLR had significantly shorter OS, CSS, and PFS for tumor sizes3 cm (all P .05), but not for ≤3 cm. For UTUCs with tumor sizes3 cm, multivariable analyses showed simultaneously high-level PLR and MLR to be independent predicators of poor OS, CSS, and PFS (all P .05). Moreover, receiver operating characteristic (ROC) analyses revealed that the predictive accuracy of the combination of PLR and MLR for OS, CSS, and PFS with the area under the ROC curve of 0.836, 0.871, and 0.806, respectively, in tumor sizes3 cm (all P .001).Our study demonstrated that a high-level PLR and MLR can serve as an independent predicator of worse outcomes in UTUCs with tumor sizes3 cm. This combination can clinically help enhance the prognostic discrimination of UTUCs with tumor sizes3 cm and further may guide physicians in selecting patients for postoperatively systemic chemotherapy.

Background/objectives A number of therapeutic agents have been reported to be clinically useful for the management of the patients with unresectable pancreatic neuroendocrine tumors (PanNETs) including somatostatin analogues, molecular-targeted agents and cytotoxic agents. However, the optimal strategy for selection among those treatment modalities above in these patients has remained unexplored. Methods Japanese experts for PanNET discussed and determined the optimal treatment strategies according to the results of previously reported studies. Results The tumor volume of liver metastases and the Ki-67 labeling index were unanimously accepted as indicators of the tumor burden and tumor aggressiveness, respectively, which are two most clinically pivotal factors for determining the strategy of systemic treatment for unresectable PanNETs. In addition, for those with a relatively small tumor burden and slow disease progression, somatostatin analogues were selected as the first-line treatment agents. For those with a relatively large tumor burden and rapid tumor progression, cytotoxic agents were selected, possibly aiming at tumor shrinkage. For those of intermediate tumor volume and/or growth rate, molecular-targeted agents were selected as the first choice. Based on this strategy discussed among the experts, we tentatively prepared a MAP for proposing optimal treatment strategy and examined its validity in some patients with unresectable PanNETs. Results validated the usefulness of this MAP proposed for patients harbouring unresectable PanNETs. Conclusion We herein propose a tentative MAP for optimal treatment selection for the patients harbouring unresectable PanNETs. Further large scale studies are, however, warranted to validate the usefulness of this MAP proposed in this study.

Objective The aim of this study was to analyze the incidence of and risk factors for adrenocortical carcinoma (ACC) in adrenal incidentaloma (AI). Summary of background data AI guidelines are based on data obtained with old-generation imaging and predominantly use tumor size to stratify risk for ACC. There is a need to analyze the incidence and risk factors from a contemporary series. Methods This is a retrospective review of 2219 AIs that were either surgically removed or nonoperatively monitored for ≥12 months between 2000 and 2017. Multivariate logistic regression was performed to define risk factors. ROC curves constructed to determine optimal size and attenuation cut-offs for ACC. Results 16.8% of AIs underwent upfront surgery and rest initial nonoperative management. Of conservatively managed patients, an additional 7.7% subsequently required adrenalectomy. Overall, ACC incidence in AI was 1.7%. ACC rates by size were 0.1%, 2.4%, and 19.5% for AIs of 6 cm, respectively. The optimal size cut-off for ACC in AI was 4.6 cm. ACC risks by Hounsfield density were 0%, 0.5%, and 6.3% for lesions of 20 HU, with an optimal cut-off of 20 HU to diagnose ACC. 15.5% of all AIs and 19.2% of ACCs were hormonally active. Male sex, large tumor size, high Hounsfield density, and >0.6 cm/year growth were independent risk factors for ACC. Conclusion This contemporary analysis demonstrates that ACC risk per size in AI is less than previously reported. Given these findings, modern management of AIs should not be based just on size, but a combination of thorough hormonal evaluation and imaging characteristics.

Objective Tumors harboring a POLE pathogenic variant, associated with high tumor mutational burden, are good candidates for immunotherapy. However, POLE pathogenic variants are not currently screened in routine clinical practice. Can these tumors be identified by means of an already available test? Methods We describe seven tumors harboring a POLE pathogenic variant, among eight patients with tumors harboring multiple BRCA1/2 variants (from 4 to 20). All patients were managed at Institut Curie, Paris. Five patients were selected because of unexpected tumor BRCA testing results with multiple variants and another three patients were selected because of a POLE pathogenic variant detected by large tumor testing. We looked for other tumor variants by Next-Generation Sequencing in tumors harboring multiple BRCA1/2 variants, and for multiple BRCA1/2 variants in tumors harboring a POLE pathogenic variant. Results Four of the five tumors selected because of multiple BRCA1/2 variants exhibited a POLE pathogenic variant, and all three tumors selected for POLE pathogenic variants exhibited multiple BRCA1/2 variants. Conclusions Tumor BRCA testing could be a way to detect tumors harboring a highly mutagenic POLE pathogenic variant.

OBJECTIVES To investigate long-term chronological changes in functional renal volume and renal function after nephron-sparing surgery, and factors that contribute to the progression of postoperative chronic kidney disease. METHODS A total of 80 patients who underwent nephron-sparing surgery were enrolled in this prospective observational study. The renal function deterioration group was defined as patients whose estimated glomerular filtration rate at 5 postoperative years decreased by ≥20% relative to that before surgery. RESULTS The predicted estimated glomerular filtration rate, calculated based on the functional renal volume at 5 postoperative years, was strongly correlated with the postoperative estimated glomerular filtration rate (Spearman's ρ = 0.89, P

Objective: The aim of this study was to determine the sensitivity and specificity of the MOLES scoring system in differentiating choroidal melanomas from nevi according to Mushroom shape, Orange pigment, Large tumor size, Enlarging tumor, and Subretinal fluid (SRF). Methods: Color photographs, fundus-autofluorescence images, and optical coherence tomography of 222 melanocytic choroidal tumors were reviewed. Each MOLES feature was retrospectively scored between 0 and 2 and tumors categorized as “common nevus,” “low-risk nevus,” “high-risk nevus,” and “probable melanoma” according to the total score. MOLES scores were compared with the experts’ diagnosis of melanoma. Results: The MOLES scoring system indicated melanoma in all 81 tumors diagnosed as such by ocular oncologists (100% sensitivity) and nevus in 135 of 141 tumors given this diagnosis by these experts (95.7% specificity). Of the 6 tumors with discordant diagnoses, 4 had basal diameters exceeding 6 mm, all with SRF and/or orange pigment, and 2 small tumors showed either significant SRF with traces of orange pigment, or vice versa. Conclusions: The MOLES system for diagnosing melanocytic choroidal tumors compares well with expert diagnosis but needs to be evaluated when deployed by ophthalmologists and community optometrists in a wide variety of working environments.

Pelvic tumors are rare entities. The rates of cure for malignant tumors involving the pelvis are often lower than those involving the extremities, with significant morbidity and mortality. This may be due to the complexity of the anatomy of the pelvis and the usually large tumor volume, making resection with adequate margins very difficult. Management of these patients requires a multidisciplinary approach and should be treated at centers specialized in musculoskeletal oncology. This chapter will focus on the surgical principles and approaches in the management of pelvic tumors.

The diagnosis and treatment of intracardiac mass are challenging for emergency physicians. Dyspnea is one of the most common chief complaints in ER among patients with rapidly progressed heart failure without significant medical history. Establishing such an unusual diagnosis based on dyspnea patients requires more evidence. We present a case of a patient who was diagnosed with a rapidly progressing right atrial mass.

OBJECTIVEAcromegaly is a rare neuroendocrine condition that can lead to significant morbidity; therefore, large studies are invaluable for understanding the disease burden. Despite China’s vast population size, studies on acromegaly remain sparse. In this report, we aimed to investigate clinical characteristics and predictors of biochemical remission after surgery for acromegaly.METHODSA retrospective nationwide study was conducted using patient-reported data from the China Acromegaly Patient Association (CAPA) from 1998 to 2018. Univariate analyses were performed using Wilcoxon rank-sum tests, two-sample t-tests, and chi-squared tests. Using the purposeful selection method, multivariate logistic regression analysis was employed to determine independent predictors of biochemical remission at 3 months in patients after surgery.RESULTSOf the 575 cases (mean age: 37.3 years; 59% female), macroadenomas and invasive tumors (Knosp score 3-4) were 87% and 61%, respectively. Ninety-five percent of patients were treated first with surgery (5.1% transcranial and 94.9% endonasal) and 38.3% exhibited biochemical remission at 3-months postoperatively. The following independent predictors of biochemical remission were identified: preoperative growth hormone (GH) levels between 12 and 28 μg/L [odds ratio (OR)=0.61; 95% confidence interval (CI), 0.39-0.96; p=0.031], preoperative GH levels >28 μg/L (OR=0.56; 95% CI, 0.35-0.90; p=0.016), macroadenoma (OR=0.57; 95% CI, 0.33-0.97; p=0.041), giant adenomas (OR=0.17; 95% CI, 0.06-0.44; p=0.0005), Knosp score 3-4 (OR=0.39; 95% CI, 0.25-0.59; pCONCLUSIONSIn this nationwide study spanning over two decades, we highlight that higher preoperative GH levels, large tumor size, and greater extent of tumor invasiveness are associated with a lower likelihood of biochemical remission at 3-months after surgery, while preoperative medical therapy increases the chance of remission.

Introduction A novel CD74-NRG2α fusion has recently been identified in NSCLC. We surveyed a large tumor database comprehensively profiled by whole transcriptome sequencing to investigate the incidence and distribution of NRG2 fusions among various solid tumors. Methods Tumor samples submitted for clinical molecular profiling at Caris Life Sciences (Phoenix, AZ) that underwent whole transcriptome sequencing (NovaSeq [Illumina, San Diego, CA]) were retrospectively analyzed for NRG2 fusion events. All NRG2 fusions with sufficient reads (> three junctional reads spanning ≥ seven nucleotides) were identified for manual review, characterization of fusion class, intact functional domains, EGF-like domain isoforms, breakpoints, frame retention, and co-occurring alterations by next-generation sequencing (NextSeq [Illumina, San Diego, CA], 592 genes). Results Seven inframe functional (containing the intact EGF-like domain) NRG2α fusions were identified, namely, the following: (1) NSCLC (two of 9600, 0.02%: CDH1-NRG2α [C11, N2], F11R-NRG2α [F1, N4]); (2) endometrial (two of 3060, 0.065%: CPM-NRG2α [C2, N2], OPA3-NRG2α [O1, N2]); (3) ovarian (one of 5030, 0.02%: SPON1-NRG2α [S6, N2]); (4) prostate (one of 1600, 0.063%: PLPP1-NRG2α [P1, N2]); and (5) carcinoma of unknown origin (one of 1400, 0.07%: CYSTM1-NRG2α [C2, N2]). No NRG2β fusions were identified. Both NSCLC samples contained the reciprocal NRG2 fusions (NRG2-CDH1, NRG2-F11R). Almost all inframe NRG2α fusions have no (N = 6, 85.7%) or low (N = 1, 14.3%) programmed death-ligand 1 expression. No additional known driver mutations were identified in these seven NRG2α fusion-positive tumor samples. Conclusions Similar to NRG1 fusions, NRG2α fusions are recurrent and rare ligand-fusions in NSCLC and other multiple tumor types, especially gynecologic malignancies.

We investigate the feasibility of an Electron Tracking Compton Camera (ETCC) for an imaging in a clinical situation. The ETCC is an emergence technology which can trace the track of Compton recoiled electrons. This makes possible to reconstruct images with fewer detected events compared with a conventional Compton camera. The ETCC was originally developed for astronomical purposes. In this study, we applied the ETCC in a clinical situation, especially for imaging of an At-211 therapy. This is because the At-211 has a high therapeutic efficiency and provides considerable recent research interest. In order to investigate the feasibility of the ETCC, we performed a simulation with a point source of At-211 in a water sphere with a radius of 10 cm. We found that the ETCC system can reconstruct the images with only about 1000 events. In addition, we improved the spatial resolution of the reconstructed image using a bootstrapping method. The spatial resolution was good enough to resolve a large tumor. These results suggest that the ETCC system can be used for an imaging in At-211 radiation therapy.