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Polymorphous adenocarcinoma is a slow growing malignant tumor of minor salivary glands. The most common location of these tumors is palate, buccal mucosa, upper lip, floor of mouth and retromolar region. This malignant neoplasm is characterized by cytological uniformity, architectural diversity and infiltrative growth pattern. This is a case report of polymorphous adenocarcinoma occurring in lacrimal gland. They are seldom reported in this location and propose unique diagnostic challenges.

[Background]: Acantholytic cutaneous squamous cell carcinomas (aCSCCs) have been classically considered as a high-risk variant of CSCC. However, more recent studies show that aCSCC does not confer more aggressiveness. This study aims to establish whether the prognosis of the aCSCC is worse than that of the non-acantholytic (naCSCC) or not. [Methods]: Retrospective case-control study with 50 aCSCCs and 50 naCSCCs. For each aCSCC, an naCSCC with similar high-risk features to the aCSCC but with no acantholysis was selected. Prognosis between both groups was compared. [Results]: The mean age was 86 years (SD 9.61). Sixty-one patients were men. Thirty-nine CSCCs were located in high-risk head and neck areas. Twenty CSCCs exhibited a poor degree of differentiation, and 36 showed an infiltrative growth pattern. The tumor diameter was 18.71 mm (interquartile range, IQR 35), and the tumor thickness was 6.72 mm (IQR 15.50). Twelve CSCCs exhibited perineural infiltration, and eight CSCCs exhibited invasion beyond the subcutaneous fat. Positive margins after excision of the tumor in 22 aCSCCs vs eight naCSCCs (P, Instituto de Salud Carlos III, Grant/Award Number: PI18/000587

Signet Ring Rectal Cancer (SRRC) of rectum is rare high-grade subtype with poor prognosis and characteristic histopathology. We evaluated its imaging appearance and correlated its outcomes. We conducted a retrospective review of the rectal MRIs of 97 patients with rectal SRRC, evaluating tumor morphology, T2 signal, length, location, pattern of tumor growth, nodal status and location, EMVI (extramural vascular invasion), site of metastases, and response to chemotherapy. The tumor signal on T2W images was categorized into intermediate, T2 hyperintense, and fluid/mucin bright. Imaging findings were correlated with risk of metastatic/ recurrent disease, disease-free survival, and overall survival. The median age of patients of SRRC in our study was 35 years and more frequently found in male patients. The common imaging features of SRRC were T2-hyperintense signal (63%), infiltrative growth pattern (76%), positive MR CRM (Circumferential Resection Margin on MRI) (84%), presence of EMVI (51%), and advanced T and N stage (97% and 84%, respectively). Peritoneum and nodes were the most common sites of metastases. Raised serum CEA (Carcino-embryonic Antigen) levels, positive MR CRM status, extramesorectal adenopathy, and advanced N stage had statistically significant predictive value for recurrence or metastases. Elevated serum CEA levels (p = 0.019) and intermediate T2 signal (p = 0.012) demonstrated significant independent association with poor overall survival, while advanced N stage (p = 0.033) demonstrated significant independent association with worse disease-free survival in multivariate analysis. SRRC affected young patients and demonstrated T2-hyperintense signal and subepithelial spread in an infiltrative pattern. Elevated CEA levels and T2-intermediate signal intensity are independent predictors for worse overall survival and advanced nodal stage is independent prognostic factor of poor disease-free survival. MRI rectum can pinpoint the pathology given the distinct MRI morphology and age of presentation.

Purpose Prostate-specific membrane antigen (PSMA-) PET has become a promising tool in staging and restaging of prostate carcinoma (PCa). However, specific primary tumour features might impact accuracy of PSMA-PET for PCa detection. We investigated histopathological parameters and immunohistochemical PSMA expression patterns on radical prostatectomy (RPE) specimens and correlated them to the corresponding 68Ga-PSMA-11-PET examinations. Methods RPE specimens of 62 patients with preoperative 68Ga-PSMA-11-PET between 2016 and 2018 were analysed. WHO/ISUP grade groups, growth pattern (expansive vs. infiltrative), tumour area and diameter as well as immunohistochemical PSMA heterogeneity, intensity and negative tumour area (PSMA%neg) were correlated with spatially corresponding SUVmax on 68Ga-PSMA-11-PET in a multidisciplinary analysis. Results All tumours showed medium to strong membranous (2–3 +) and weak to strong cytoplasmic (1–3 +) PSMA expression. Heterogeneously expressed PSMA was found in 38 cases (61%). Twenty-five cases (40%) showed at least 5% and up to 80% PSMA%neg. PSMA%neg, infiltrative growth pattern, smaller tumour area and diameter and WHO/ISUP grade group 2 significantly correlated with lower SUVmax values. A ROC curve analysis revealed 20% PSMA%neg as an optimal cutoff with the highest sensitivity and specificity (89% and 86%, AUC 0.923) for a negative PSMA-PET scan. A multiple logistic regression model revealed tumoural PSMA%neg (p p = 0.0497, OR = 306.537) as significant predictors for a negative PSMA-PET scan. Conclusions We describe PSMA%neg, infiltrative growth pattern, smaller tumour size and WHO/ISUP grade group 2 as parameters associated with a lower 68Ga-PSMA-11 uptake in prostate cancer. These findings can serve as fundament for future biopsy-based biomarker development to enable an individualized, tumour-adapted imaging approach.

Carcinoma cuniculatum (CC) is a rare variant of squamous cell carcinoma (SCC) that is characterized by minimal cytologic atypia and a unique deeply infiltrative growth pattern resembling rabbit burrows (cuniculi). With less than 75 cases reported in the head and neck, the clinical and pathologic spectrum of this entity remains poorly understood. A retrospective review of the clinical and pathologic features of archival cases of oral CC was performed. A total of six cases of oral CC were identified. Age ranged from 25-77 years; the male-to-female ratio was 5:1. All patients had a long-standing history of tobacco and betel-quid consumption. The tumors were distributed in the gingivobuccal sulcus (n = 2), the tongue (n = 2), buccal mucosa (n = 1), and the palate (n = 1). Histology in all cases typically revealed a tumor composed of well-differentiated squamous epithelium, devoid of atypia, lining deeply infiltrative, large-sized, branching, keratin-filled cavities, resembling rabbit-burrows. Dense lymphocytic infiltrates and discharging micro-abscesses were regular features. Underlying bone invasion and lymph node metastasis were observed in 1 patient. One patient with a tongue tumor developed locoregional recurrence at 10 months while none developed distant metastasis. Oral CC is a rare and under-recognized variant of SCC with locally aggressive behavior. Lack of familiarity with this variant exacerbated by the absence of cytologic anaplasia makes CC susceptible to multiple negative biopsies and erroneous diagnoses. Awareness of this clinicopathologic entity is essential to allow its accurate diagnosis and optimal management.

Histologic distinction between melanoma ex-blue nevus and cellular blue nevus (CBN) can often be difficult, but features supporting melanoma include infiltrative growth pattern, frequent mitoses, cytologic atypia and pleomorphism, cell crowding, and tumor necrosis. Unfortunately, these features are not constantly dependable and frequently borderline lesions exist, so-called atypical CBN, which lack explicit malignant features. Furthermore, some CBN and atypical CBN show an assortment of features, which may lead to their misdiagnosis as melanoma, but to date necrosis is generally absent. We present an unusual case of an atypical cellular blue nevus with extensive necrosis mimicking melanoma ex-blue nevus.

In rats, chondrosarcomas have been reported to occur both spontaneously and secondary to chemical induction. In a rare case, a spontaneous chondrosarcoma was identified in the deformed femur of a young male Wistar rat. After gross examination of the femur and knee joint, tissue was collected and preserved. The formalin-fixed tissue was decalcified, embedded in paraffin, sectioned, and stained with hematoxylin and eosin. Microscopic examinations revealed a large, highly proliferative, noncapsulated growth of chondrocytic or chondroblastic origin in the femoral bone, with proliferating chondrocytes invading the bone and surrounding tissues in an infiltrative growth pattern. Based on its histomorphological features, the lesion was diagnosed as a malignant cartilaginous neoplasm of spontaneous origin.

Cutaneous squamous cell carcinoma (CSCC) is one of the most common skin cancers. Prognosis is favorable following surgical resection of early-stage disease, but the management of the metastatic disease is challenging. Several prognostic risk factors have been described in the American Joint Committee on Cancer/the Union for International Cancer Control (UICC) 8th edition staging and the Brigham and Women's Hospital T classification system. However, their clinical validity in Asian populations is unclear because of racial differences in the clinical characteristics of CSCC. This study aimed to identify factors that could predict lymph node metastasis in Asian patients.This retrospective single-center study evaluated 540 patients with primary CSCC between 1989 and 2013. Five factors were evaluated for their ability to predict lymph node metastasis: maximum tumor diameter, tumor thickness, depth of invasion, degree of differentiation, and infiltrative growth pattern (INF).Tumor diameter 2 cm (p 0.0001), tumor thickness 6 mm (p 0.0001), invasion beyond the subcutaneous fat (p 0.0001), poor differentiation (p = 0.042), and INFc infiltration (p 0.0001) were associated with lymph node metastasis in the univariate analyses. In the multivariate analysis, lymph node metastasis was independently associated with tumor size 2 cm [hazard ratio (HR) 2.9, 95% confidence interval (CI) 1.4-6.2; p = 0.006], tumor thickness 6.0 mm (HR 2.9, 95% CI 1.3-6.4; p = 0.007), and invasion beyond the subcutaneous fat (HR 2.3, 95% CI 1.0-5.1; p = 0.045).Larger tumor diameter, greater tumor thickness, and deeper invasion included in the UICC T classification system are associated with increased risks of lymph node metastasis from CSCC in Japanese patients.

Introduction: Diffuse midline glioma (DMG), H3K27M mutant is an infiltrative midline high grade glioma with predominantly astrocytic differentiation and K27M mutation in either H3F3A or HIST1H3B/C. Case Report: A 45-year-old female presented with complaints of headache and memory loss for 3 months. MRI was suggestive of an infiltrative mass lesion in the quadrigeminal plate cistern suggestive of pineal neoplasm. Squash and histomorphology showed a low-grade astrocytic tumour with infiltrative growth pattern. Microvascular proliferation and necrosis were absent. Immunohistochemistry showed loss of ATRX protein, focal positivity for p53 proteinand IDH1R132H negativity. On molecular analysis, H3K27M mutation was noted and the case was labelled as DMG H3K27Mmutant (WHO IV) Conclusion: DMG (H3K27M) is a newly added entity in the WHO 4th revised editionof 2016. It presents with a diagnostic challenge as it has varied histomorphology, not requiring atypia, mitosis, endothelial hyperplasia and necrosis for diagnosis as Grade IV.

Malignant gliomas are primary brain tumours with an infiltrative growth pattern, often with contrast enhancement on magnetic resonance imaging (MRI). However, it is well known that tumour infiltration extends beyond the visible contrast enhancement. The aim of this study was to investigate if there is contrast enhancement not detected visually in the peritumoral oedema of malignant gliomas by using relaxometry with synthetic MRI. 25 patients who had brain tumours with a radiological appearance of malignant glioma were prospectively included. A quantitative MR-sequence measuring longitudinal relaxation (R1), transverse relaxation (R2) and proton density (PD), was added to the standard MRI protocol before surgery. Five patients were excluded, and in 20 patients, synthetic MR images were created from the quantitative scans. Manual regions of interest (ROIs) outlined the visibly contrast-enhancing border of the tumours and the peritumoral area. Contrast enhancement was quantified by subtraction of native images from post GD-images, creating an R1-difference-map. The quantitative R1-difference-maps showed significant contrast enhancement in the peritumoral area (0.047) compared to normal appearing white matter (0.032), p = 0.048. Relaxometry detects contrast enhancement in the peritumoral area of malignant gliomas. This could represent infiltrative tumour growth.

Background/aim Myxofibrosarcoma (MFS) is characterized by an infiltrative growth pattern. This study aimed to determine the correlation between overall survival (OS) and morphological features of MFS as well as examine the reproducibility of these findings on preoperative magnetic resonance imaging (MRI). Patients and methods Fifty-eight MFS patients underwent preoperative MR imaging with the following features analysed: i) tumour size, ii) localization, iii) margins, iv) morphology, v) signal characteristics, vi) contrast enhancement, vii) presence and extent of perilesional oedema, and viii) presence of the tail sign. Results Only circumscribed perilesional oedema was associated with a significantly better survival compared to diffuse oedema (p=0.010), which was found in the majority of cases. The tail sign was found in less than 50% of the cases. Cohen's kappa coefficients confirmed a relatively high interrater variability. Conclusion Perilesional diffuse oedema on MR imaging of MFS is significantly correlated with a poor overall survival. The interrater variability in interpretation of MR examinations varies from slight to substantial agreement. Preoperative MR imaging with detailed planning of the resection seem to be a logical approach to achieve negative resection margins and recurrence-free survival.

Desmoplastic fibroma (DF) is a rare, benign yet locally aggressive tumor of the bone. It traditionally presents as an intra-osseous lesion with a high recurrence rate and infiltrative growth pattern, affecting the mandible, long bones, and pelvis. We present a rare occurrence of DF in the maxilla, managed successfully under our care, and followed up uneventfully for three years. Treatment efficiency relies on wide local excision with an adequate safety margin to avoid recurrence.

Syringomatous adenoma of the nipple (SAN) is a rare benign lesion which often presents as a subareolar mass with associated nipple shape changes including pruritus, crusting, and discharge It is thought that syringomatous tumors arise from eccrine glands of the nipple and areola. Due to its locally infiltrative growth pattern the main differential is with low-grade adenosquamous carcinoma which requires more aggressive surgical treatment. Syringomatous adenoma of the nipple can recur, and therefore complete local excision is recommended. We present a case of a syringomatous adenoma of the nipple diagnosed after nearly 5 years of observation with microcalcifications noted on mammography.

Lipoblastoma (LB) and lipoblastomatosis (LBS) are uncommon benign mesenchymal tumors of embryonal fat, occurring almost exclusively in infancy and early childhood. These fast-growing tumors have an excellent prognosis if properly treated. Eight consecutive children having pathologically demonstrated LB treated by the same surgical team were retrospectively reviewed. There were 5 boys and 3 girls between 7 to 36 months (median age 22 months). The localization of the tumors was on the thigh (1 case), abdomen (2 cases), axillary and pectoral region (1 case) paragluteal region (1 case), lumbar area (1 case), inguinal-scrotal (1 case), and in one case, presacral, gluteal and perirectal region (1 case). Five were focal and in 3 cases an infiltrative growth pattern was observed. One case exhibited a gross appearance resembling sacrococcygeal teratoma, with associated Dravet syndrome. No recurrence was noted in our series, after a mean follow-up of 28 months post operatory. Despite its rareness, LB must be kept in mind when diagnosing a rapidly growing fatty mass in children. Even when dealing with very large abdominal LB, complete surgical excision is possible, with an excellent prognosis. Due to the relatively high recurrence rate noted in the literature, particularly in LBS, follow-up is extremely important.

Peritumoral edema (PE) of breast cancer at T2-weighted MR images is considered a poor prognostic sign and may represent the microenvironment surrounding the tumor; however, its histopathological mechanism remains unclear. The purpose of the study was to identify and describe detailed histopathological characteristics associated with PE at preoperative breast MRI in breast cancer patients. This retrospective study included breast cancer patients who had undergone preoperative MRI and surgery between January 2011 and December 2012. Two radiologists determined the presence of PE in consensus based on the signal intensity surrounding the tumor at T2-weighted images. The following detailed histopathological characteristics were reviewed by two breast pathologists using four-tiered grades; lymphovascular invasion, vessel ectasia, stromal fibrosis, growth pattern, and tumor budding. Tumor necrosis and tumor infiltrating lymphocytes were assessed using a percent scale. Baseline clinicopathological characteristics, including age and histologic grade, were collected. The associations between detailed histopathologic characteristics and PE were examined using multivariable logistic regression with odds ratio (OR) calculation. A total of 136 women (median age, 49 ± 9 years) were assessed; among them 34 (25.0%) had PE. After adjustment of baseline clinicopathological characteristics that were significantly associated with PE (age, T stage, N stage, histologic grade, and subtype, all Ps P = 0.009), vessel ectasia (P = 0.021), stromal fibrosis (P = 0.024), growth pattern (P = 0.036), and tumor necrosis (P

Background Evaluation of response to neoadjuvant radiotherapy (NART) does not consider soft tissue sarcoma (STS) heterogeneity. We aimed to investigate radiological and pathological response of 4 major histotypes. Methods Extremity or trunk STS patients who received 50 Gy NART between 2009 and 2020 were retrospectively included. Relative variation in tumor size (RVTS) and pathological response were reported in the overall population and in undifferentiated pleomorphic sarcoma (UPS), myxofibrosarcoma (MFS), myxoid liposarcoma (MLS) and synovial sarcoma (SS) patients to identify response modalities of each histotype. Results Among the 121 included patients, 49, 19, 13 and 11 presented UPS, MFS, MLS and SS. Median RVTS were 0% (IQR -18-+18), +8% (IQR 0-+24), −12% (IQR -20–3) and −11% (IQR -15–9), respectively (p = 0.001). Median viable cells were 10%, 60%, 20% and 70% (p = 0.007). In overall population, pathological complete response and median necrosis were 27.7% and 10% without significant correlation to histotype (p = 0.18 and 0.06). Nineteen (38.8%) UPS specimens presented cysts that were emptied during the sampling process and distorted the microscopic response evaluation. Infiltrative growth pattern was observed in 28% and 38.9% UPS and MFS patients. Five (38.5%) MLS presented mature adipocytes without proven prognostic value. Cysts were observed in 36% of SS specimens. In the absence of initial tumor limits, the great viable cellularity of SS may be overestimated by their nodular aspect. Conclusion After NART, we highlighted disparate response of UPS, frequent progression of MFS, and confirmed MLS and SS radiosensitivity. Response must be interpreted with caution and consider the histotype-specific patterns.

Background: Little is known about the clinicopathological characteristics of superficial spreading-type esophageal carcinoma extending ≥5 cm along the long axis of the esophagus. This study was aimed at investigating the frequency of lymph node metastasis (LNM) in patients with superficial spreading-type esophageal carcinoma. Methods: We reviewed the data of 320 patients with superficial esophageal squamous cell carcinoma who had undergone esophagectomy with lymph node dissection at our hospital between 1986 and 2010. The incidence of LNM was compared between the spreading (≥5 cm) and nonspreading (< 5 cm) types. Results: The multivariate analysis revealed significant differences in the likelihood of LNM depending on the lymphovascular invasion, the infiltrative growth pattern (INF)-c, and the depth. There was no difference in the LNM frequency between nonspreading and spreading type in the patients with epithelium (EP)-lamina propria, muscularis mucosa (MM)-submucosa (SM)1 and SM2/3 lesions. The frequencies of LNMs (nonspreading-type vs. spreading-type tumors) in the patients with MM-SM1 lesions were 7/47 (14.9%) versus 4/25 (16%) and those in the patients with SM2/3 lesions were 22/58 (37.9%) versus 4/14 (28.9%), when the lesions did not have lymphovascular invasion and INF-c. Conclusions: Endoscopic resection can be selected for ­EP-SM1 lesions, regardless of whether the lesions are of the spreading type or nonspreading type.

Normal breast tissue consists of branching ductal networks that are lined by an outer layer of myoepithelial cells and an inner layer of polarized ductal epithelium.1 Breast myoepithelial cells are usually located between the basement membrane and breast ductal epithelium.2 Based on the World Health Organization classification of tumors of the breast, the myoepithelial lesions are composed of a pure or dominant population of myoepithelial cells, while the epithelial‐myoepithelial lesions are derived from a dual population of epithelial and myoepithelial cells.3 Epithelial‐myoepithelial lesions include pleomorphic adenomas, adenomyoepitheliomas (AMEs), AME with carcinoma, and adenoid cystic carcinomas.3 Adenomyoepitheliomas is characterized by the bicellular proliferation of glands that consist of an inner epithelial and an outer myoepithelial layer of cells.4 It can have a spindle, tubular, or lobulated histological pattern.5 One or both of the components of the tumor may exhibit malignant transformation, which is called AME with carcinoma and is characterized by a higher mitotic rate, infiltrative growth pattern, marked cytologic atypia, necrosis, and/or metastasis of cells.5 Adenomyoepitheliomas with myoepithelial carcinoma of the breast is rare, and its biological behavior and treatment are not well characterized. This report presents a case of AME with myoepithelial carcinoma of the breast.

Superficial CD34 positive fibroblastic tumor (SCPFT) is a recently recognized, unique neoplasm with distinctive histomorphological features such as high pleomorphism, low mitotic rate, and diffuse CD34 reactivity. Hereby we present three cases of our experience with clinicopathological, morphological, and immunohistochemical characteristics. The patients were a 31-year-old female, 53-year-old female, and 33-year-old male. The tumors were all superficially located; left forearm, medial aspect of the left ankle, and left thigh, respectively. Histomorphologically they had expansile and focal infiltrative growth pattern consisting of highly pleomorphic spindle cells with intranuclear inclusions, yet low mitotic rate. Tumoral cells showed strong and diffuse reactivity for CD34. One of our cases showed focal and weak reactivity for pancytokeratin. Unlike the other two tumors, one case was positive for desmin. During the clinical follow-up, one case showed local recurrence four times. SCPFT is a newly recognized, borderline mesenchymal neoplasm of soft tissues that can show local recurrence or even rarely metastasize. To the best of our knowledge, this three case series is the first to be reported from Turkey. Our aim to report these three cases was to make contribution to the literature about this rare entity and increase awareness.

Bevacizumab, a VEGF‐targeting monoclonal antibody, may trigger an infiltrative growth pattern in glioblastoma. We investigated this pattern using both a human specimen and rat models. In the human specimen, a substantial fraction of infiltrating tumor cells were located along perivascular spaces in close relationship with endothelial cells. Brain xenografts of U87MG cells treated with bevacizumab were smaller than controls (p = 0.0055; Student t‐test), however, bands of tumor cells spread through the brain farther than controls (p < 0.001; Student t‐test). Infiltrating tumor Cells exhibited tropism for vascular structures and propensity to form tubules and niches with endothelial cells. Molecularly, bevacizumab triggered an epithelial to mesenchymal transition with over‐expression of the receptor Plexin Domain Containing 1 (PLXDC1). These results were validated using brain xenografts of patient‐derived glioma stem‐like cells. Enforced expression of PLXDC1 in U87MG cells promoted brain infiltration along perivascular spaces. Importantly, PLXDC1 inhibition prevented perivascular infiltration and significantly increased the survival of bevacizumab‐treated rats. Our study indicates that bevacizumab‐induced brain infiltration is driven by vascular endothelium and depends on PLXDC1 activation of tumor cells., What's new? Bevacizumab, a VEGF‐targeting monoclonal antibody, has been observed to trigger an infiltrative growth pattern in glioblastoma as an escape mechanism. The mechanisms underlying this gliomatosis‐like growth pattern, however, remain unclear. Here, the authors found that the infiltrative growth pattern occurs mostly along perivascular spaces and relies on the over‐expression of PLXDC1 by tumor cells and on the restoration of the endothelial component of blood brain barrier. Altogether, the data show that the brain infiltration induced by bevacizumab is mainly driven by the vascular endothelium. Importantly, inhibition of PLXDC1 prevents bevacizumab‐induced infiltrative growth, resulting in significant increase of survival.

To determine if changes in surrounding tissues of soft-tissue sarcomas (STS) evaluated by MRI during neoadjuvant chemotherapy (NAC) are associated with the histological response and satellite tumorous cells beyond the pseudocapsule on surgical specimen, disease-free survival (DFS) and overall survival (OS).Fifty-seven adult patients with locally advanced high-grade STS of extremities and trunk wall were included in this single-centre retrospective study. All were uniformly treated by 5-6 cycles of anthracycline-based NAC, curative surgery and adjuvant radiotherapy and had available MRI with a gadolinium-chelates administration at baseline and after 2 cycles. Thirty-seven patients also had a pre-operative MRI. Two senior radiologists evaluated MRI growth pattern, oedema, contrast-enhanced oedema, aponeurotic enhancement, and their qualitative changes during NAC. An expert pathologist reviewed all surgical specimens. A good histological response was defined as10% viable cells. Associations with pathological findings were estimated with Fisher and Chi-square tests and multivariate analysis with binary logistic regression. Survival analyses included log-rank tests.Forty-two patients had poor responses and 25 had satellite tumorous cells on surgical specimen. Changes in surrounding oedema and in contrast-enhanced oedema were associated with responses (p = 0.008 and 0.011, respectively). Diffuse infiltrative growth pattern (IGP) on baseline MRI, changes in margin definition and in contrast-enhanced oedema at early evaluation were associated with satellite tumorous cells (p = 0.039, 0.011 and 0.009, respectively). Diffuse IGP on baseline MRI and stable or increased oedema during treatment were predictors of DFS (p = 0.009 and 0.040, respectively).Surrounding tissues of STS during NAC should be carefully evaluated as they may steer treatment efficacy and patient prognosis.

Background: Plexiform Fibromyxoma (PF) of the stomach is a very rare mesenchymal tumor of the gastrointestinal tract. We report the first case of PF which was confirmed after surgical resection pathologically. Case summary: The tumor was characterised microscopically infiltrative and diffuse growth into the smooth muscle bundles of the muscularis propria by multinodular and plexiform within myxoid stroma. Immunohistochemistry analysis revealed that the tumor cells were positive or weakly positive for smooth muscle actin (SMA), vimentin and H-caldesmon, and negative for desmin, CD117, CD34, CK-20, Pan-CK, Dog1, S100, ER, PR, and CD10. No mutations of C-kit and platelet-derived growth factor receptor alpha (PDGFRA) were detected in our case. No genetic disruption of glioma-associated oncogene homolog 1 (GLI1) was detected by fluorescence in situ hybridization (FISH). Conclusion: So we mainly relied on the morphological and immunohistochemistry findings and made the final diagnosis of PF.

Development of effective treatments for high-grade glioma (HGG) is hampered by (1) the blood–brain barrier (BBB), (2) an infiltrative growth pattern, (3) rapid development of therapeutic resistance, and, in many cases, (4) dose-limiting toxicity due to systemic exposure. Convection-enhanced delivery (CED) has the potential to significantly limit systemic toxicity and increase therapeutic index by directly delivering homogenous drug concentrations to the site of disease. In this review, we present clinical experiences and preclinical developments of CED in the setting of high-grade gliomas.

Yes-associated protein (YAP) and TEA domain-containing sequence-specific transcription factors 4 (TEAD4) are essential components of the Hippo pathway. Abnormal regulation of the Hippo pathway contributes to the progression and metastasis of many cancer types. However, their clinicopathologic and prognostic significances have not been studied in gallbladder cancers. Here, we systematically evaluated the YAP and TEAD4 immunolabelings and their association with clinicopathologic characteristics and survival outcomes using 212 specimens of surgically resected gallbladder cancers. High YAP and TEAD4 immunolabelings were identified in 70 (33%) cases and were associated with infiltrative growth pattern, poor differentiation, perineural invasion, and advanced pT classification and AJCC stage. High YAP immunolabeling was significantly associated with high TEAD4 immunolabeling (p < 0.001). High immunolabeling levels of YAP or TEAD4 alone and the combined YAPhigh TEAD4high group were significantly associated with poor survival in both univariate (p < 0.001) and multivariate analyses (HR = 2.358; 95% CI, 1.369-4.061; p = 0.002). Therefore, the YAP and TEAD4 immunolabelings are associated with aggressive behavior of gallbladder cancers and may be useful as a prognostic indicator in patients with surgically resected gallbladder cancer.

Myxofibrosarcoma is a common soft-tissue sarcoma in elderly patients, characterized by an infiltrative growth pattern and a high risk for persistent local recurrence. A 35-years-old woman was diagnosed with myxofibrosarcoma on the right upper arm and the tumor is surgically resected. The tumor relapsed 7 months later. Then the patient received five cycles of low power cumulative high-intensity focused ultrasound (HIFU) treatments, which completely ablated the tumor without complications. Now the patient is disease free with a high quality of life more than 30 months. This case indicates HIFU ablation might be a novel, promising therapy for recurrent myxofibrosarcoma.

Desmoid-type fibromatosis (DF) is a rare soft tissue lesion with an annual incidence of 2 to 4 per million population and peak incidence occurring at approximately 4.5 years of age. While benign, the tumor has a locally aggressive infiltrative growth pattern and a high rate of recurrence. Given the functional and aesthetic implications of excision and reconstruction in the facial skeleton, novel medical treatment options are highly desirable. We describe the case of a 3-year-old boy who presented with an enlarging, asymptomatic mass involving the left mandible. Biopsy revealed an immunohistochemical profile consistent with DF. Despite the high likelihood of recurrence, conservative, mandible-sparing en bloc resection and limited mandibulectomy were performed. Pathological and immunohistochemical analysis of the resection specimen revealed DF with grossly positive margins and elevated expression of angiotensin II type 1 receptor. Postoperative medical treatment with the angiotensin receptor blocker losartan was initiated. The patient remains medically stable and disease progression-free on repeat imaging at 20 months post-resection. We describe for the first time the successful use of the angiotensin blocker losartan following conservative surgery for management of DF.

Trichoblastomas are rare dermal neoplasms usually found on the scalp and face. Histology shows a proliferation of small basaloid cells arranged in cords or fields surrounded by cellular stroma. Trichoblastomas are usually not aggressive, but trichoblastic carcinomas arising from preexisting trichoblastomas have been described and have been linked to basal cell carcinoma. We found a tumor with features of trichoblastoma with presence of Merkel cells, but with a deeply infiltrative growth pattern into the fat and muscle tissue, without significant architectural or cellular atypia. Tumors with similar growth patterns were previously described as deeply invasive trichoblastic neoplasms. It appears to be a new entity that has been described before but has not been fully characterized: low-grade trichoblastic carcinoma. This malignancy seems to show only locally aggressive growth. Radical excision was accomplished with Mohs micrographic surgery.

Some studies state that laryngeal squamous cell carcinoma (LSCC) is associated with possible direct tumor cell seeding to percutaneous endoscopic gastrostomy (PEG) site. However, there is a lack of experimental proof that LSCC tumor tissue can adhere and grow in distant sites. Therefore, we aimed to investigate the growth pattern of LSCC implants on chicken embryo chorioallantoic membrane (CAM) and evaluate possible associations between clinical course of the disease and behavior of experimentally implanted LSCC tumors. Our results show that implanted LSCC tissue survives on CAMs in 95% of cases while retaining essential morphologic characteristics and proliferative capacity of the original tumor. We identified the increased CAM vascularization, an infiltrative growth pattern of the implant and formation of distant isolated metastatic nodes on the CAMs. LSCC tumors with worse differentiation degree (G2 or G3) adhered to the experimental CAMs significantly better than G1. These results facilitate the understanding of tumor biology and allow presuming that dissemination and direct implantation of LSCC cells into the stomal wall during the pull PEG procedure might be possible.

Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLPS) is a locally aggressive mesenchymal neoplasm composed either entirely or partly of an adipocytic proliferation showing at least focal nuclear atypia in both adipocytes and stromal cells. ALT most frequently occurs in deep soft tissue of proximal extremities (thigh and buttock) and usually presents as a deep-seated, painless mass that can slowly attain a very large size, which is one of the most common sarcomas of extremity. In the patients who presented with deep-seated tumor, distinction between intramuscular lipoma and well-differentiated liposarcoma is important due to the differences in treatment and prognosis. However, atypical well-differentiated liposarcoma with intramuscular lipoma-like component of the thigh is extremely rare. Moreover, the infiltrative growth pattern or intramuscular component may lead to a misinterpretation as intramuscular lipoma on a small biopsy. We present an unusual case of a female who presented with symptomatic mass at the thigh which has rarely been reported in English literature as an infiltrative intramuscular lipoma-like growth pattern of well-differentiated liposarcoma. Therefore, preoperative diagnosis is necessary, and correlation with imaging studies is required when one encounters with a large deep-seated mass. Atypical lipomatous tumor or well-differentiated liposarcoma should be kept in mind in the patient who presents with abnormal thigh mass.

Pancreatic ductal adenocarcinoma (PDAC) is characterized by an infiltrative growth pattern with intense desmoplastic stroma comprised of cancer-associated fibroblasts (CAFs). Additionally, the histological characteristics are considered to play a vital role in the poor prognosis of PDAC. However, the density of cancer cells, degree of desmoplasia and vascular proliferation varies in individual cases. We hypothesized that preoperative radiological images would reflect histological characteristics, such as cancer cell density, CAF density and microvessel density. To clarify the association between the histological characteristics and radiological images of PDAC, the cancer cell density, CAF density and microvessel density from surgical specimens were measured with immunostaining, and the time density curve of dynamic contrast-enhanced computed tomography (CECT) was analyzed. Overall, the initial slope between non-enhanced and arterial phases was correlated with microvessel density, and the second slope between arterial and portal phases was correlated with CAF and cancer cell densities. In conclusion, the present study suggested the possibility of estimating cancer cell, CAF and microvessel densities using the TDC of dynamic CECT.

Diffuse intrinsic pontine gliomas (DIPGs) are a pontine subtype of diffuse midline gliomas (DMGs), primary central nervous system (CNS) tumors of childhood that carry a terrible prognosis. Because of the highly infiltrative growth pattern and the anatomical position, cytoreductive surgery is not an option. An initial response to radiation therapy is invariably followed by recurrence; mortality occurs approximately 11 months after diagnosis. The development of novel therapeutics with great preclinical promise has been hindered by the tightly regulated blood–brain barrier (BBB), which segregates the tumor comportment from the systemic circulation. One possible solution to this obstacle is the use of convection enhanced delivery (CED), a local delivery strategy that bypasses the BBB by direct infusion into the tumor through a small caliber cannula. We have recently shown CED to be safe in children with DIPG (NCT01502917). In this review, we discuss our experience with CED, its advantages, and technical advancements that are occurring in the field. We also highlight hurdles that will likely need to be overcome in demonstrating clinical benefit with this therapeutic strategy.

Background Because immunosuppression is necessary for kidney transplant recipients, malignant tumorigenesis of recipient organs is a concern; however, few studies have discussed the malignant alteration of transplanted grafts that have been functional for a long time. In addition, a urothelial carcinoma (UC) in transplanted kidney graft is a rare disease. Case report A 62-year-old man had end-stage renal failure 31 years ago and received a kidney transplant from his father. Acute renal failure due to obstruction of the transplanted ureter was diagnosed. Ultrasound, ureterogram, and non-enhanced computed tomography scans revealed no obvious evidence of any neoplastic lesion. We treated the obstruction and hydronephrosis with transplant ureter stenting. However, the regional lymph nodes enlarged, and it became necessary to change the ureteral stent frequently because of stent stenosis; therefore, he underwent lower transplant ureteral resection and reconstruction. Histopathology confirmed a UC with a flat and infiltrative growth pattern. The patient then underwent graftectomy including right external iliac vein resection and reconstruction; however, because of numerous metastatic nodules, radical surgery could not be performed. The patient subsequently died because of septic shock after the second surgery. Conclusion We report a case of an invasive UC with a flat and infiltrative growth pattern derived from a transplant kidney graft that occurred 31 years after a living-donor transplant that could not be treated immediately and was difficult to diagnose.

In this retrospective study, we analyzed the association between the tumor infiltrative growth pattern (INF) and tumor immune environment and its predictive value for lymph node metastasis and overall survival (OS) in stage T1 esophageal squamous cell carcinoma (ESCC). In total, 593 patients with a diagnosis of stage T1 ESCC who underwent esophagectomy and regional lymphadenectomy between 2009 and 2018 were included. The INF type and elements of the tumor immune microenvironment, including tumor infiltrative lymphocytes (TILs) and tertiary lymphoid structures (TLSs), were microscopically evaluated within the tumor invasive margin with hematoxylin and eosin (HE)-stained slices. The infiltrative-type INF (INFc) was associated with low-grade TILs and the absence of TLSs, deep tumor invasion, poorly differentiated phenotype. Multivariate logistic regression identified INFc as one of the independent risk factors for lymph node metastasis. INFc and low-grade TILs were independent inferior predictive factors for OS. A novel histologic risk stratification model was classified as INFa/b and high-grade TILs, INFa/b and low-grade TILs, INFc and high-grade TILs, and INFc and low-grade TILs. The Kaplan-Meier curves showed that INFa/b and high-grade TILs were associated with the best prognosis, and INFc and low-grade TILs were associated with the worst prognosis, and there was significant difference between groups. In conclusion, INFc is an independent risk factor for lymph node metastasis and an independent inferior prognostic factor for stage T1 ESCC. Furthermore, INFc is associated with immunosuppression, and the combination of the INF and TILs is useful for the risk stratification of prognosis.

Purpose To study the spectrum of imaging findings in hepatic alveolar echinococcosis (HAE) and to evaluate the potential role of diffusion-weighted imaging (DWI) in its characterisation. Material and methods Two radiologists with more than seven years of experience retrospectively studied ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) findings in 23 histopathologically proven cases of HAE with emphasis on the appearance and extent of disease. DWI characteristics of lesions were noted, and their apparent diffusion values (ADC) were calculated. Results Ultrasonography features of HAE included heterogeneous, hyperechoic hepatic mass with or without calcification (n = 20), or heterogeneous mass with solid-cystic appearance (n = 2). CT revealed heterogeneous density infiltrative hepatic mass with no contrast enhancement in 19 patients or thick-walled cystic mass (n = 4). Following Kodama classification one type 1, six type 2, two type 3, eight type 4, and two type 5 lesions were identified on T2-weighted MRI. No enhancement was seen on post-contrast T1-weighted images. Mean ADC values were 1.74 ± 0.48 × 10-3 mm2/s (range: 1.39 × 10-3 mm2/s to 2.3 × 10-3 mm2/s). Conclusions HAE by virtue of its infiltrative growth pattern with a tendency to involve biliary, vascular, and extra hepatic structures can be easily misdiagnosed as malignant hepatic neoplasm. Knowledge of varied imaging appearances of HAE is essential to suspect the condition and to make an appropriate diagnosis. Diffusion-weighted imaging is a useful adjunct with relatively high diffusivity (high ADC values) suggesting diagnosis of alveolar hydatid.

Less than 60 cases of haemangiomas of the testis (HOTs) have been reported sporadically in different parts of the world in diverse age groups including in children and adults even though it has been more commonly reported in individuals who are younger than 20 years. HOTs may present with lump/mass in testis/scrotum, scrotal enlargement with or without associated pain/discomfort. The results of serum germ cell tumour marker levels tend to be normal always. The clinical examination does reveal a discrete lump/mass in the testis which may or may not be tender. Radiology imaging does illustrate a discrete mass which usually tends to be well-demarcated but at times the lesion could be abutting the tunica albuginea testis. Doppler scan would tend to show increased vascularity and blood flow within the lesion which may also contain calcifications and at times radiology imaging could illustrate some degree of mild contrast enhancement and these features at times may suggest the possibility of benign disease but malignancy cannot be excluded. An incidental lesion of HOT was identified incidentally in radiology imaging in the absence of a palpable mass/lump. The ideal treatment for HOT is testis-sparing partial orchidectomy which has been undertaken in about a third of cases of HOT reported and radical orchidectomy has been undertaken in two-thirds of cases of HOT under a provisional diagnosis of malignant testicular tumour and the final diagnosis has been based upon the post-operative pathology reports. Diagnosis of HOT tends to be based upon the histopathology examination findings of small tubules with red blood cells that are lined by mesothelial like cells that have uniform vesicular nuclei but they can have focally infiltrative growth pattern with entrapment of seminiferous tubules. Immunohistochemistry staining studies of HOTs do show positive staining for Factor VIII, CD31, CD34, and FLI1 but negative staining for Pancytokeratin AE1/AE3, Keratin 8 / 18, EMA, placental herpesvirus 8, human chorionic gonadotrophin, c-kit, and p53. No recurrence has been reported following either testis-sparing partial orchidectomy after frozen section pathology examination or after radical orchidectomy. If urologist and radiologists have a high index of suspicion for the disease, and intraoperative frozen section excision biopsies are undertaken in all cases of testicular tumours in all age groups with the knowledge of normal levels of germ cell tumour markers then testis-preserving surgery can be undertaken and unnecessary radical orchidectomy can be avoided.

The classification of the distinct group of mesenchymal neoplasms, first described as 'Xp11 translocation perivascular epithelioid cell tumor (PEComa)' and for which the term 'melanotic Xp11 neoplasm' or 'Xp11 neoplasm with melanocytic differentiation' has recently been proposed, remains challenging and controversial. We collected 27 melanotic Xp11 neoplasms, the largest series to date, for a comprehensive evaluation. Fourteen of the cases, together with eight alveolar soft part sarcomas (ASPS), nine conventional PEComas and a control group of seven normal tissues were submitted to RNA sequencing. Follow-up available in 22 patients showed 5-year overall survival and 5-year disease-free survival of 47.6 and 35.7%, respectively, which were similar to ASPS and significantly worse than conventional PEComa. Univariate analysis of location (occurring in the kidney versus not kidney), infiltrative growth pattern, nuclear pleomorphism, mitotic activity ≥2/50 high-power fields (HPF), necrosis and lymphovascular invasion were found to be associated with overall survival and/or disease-free survival. Multivariate analysis identified that location was the only factor found to independently correlate with disease-free survival. More importantly, RNA sequencing-based clustering analysis segregated melanotic Xp11 neoplasm and ASPS from other tumors, including conventional PEComa and Xp11 translocation renal cell carcinoma, and formed a compact cluster representative of the largely similar expression signature. Here we clearly define the true biologic nature of melanotic Xp11 neoplasms which are distinctive malignant mesenchymal tumors, rather than simply PEComa variants with occasionally unpredictable behavior. Meanwhile, melanotic Xp11 neoplasm and ASPS more likely represent phenotypic variants of the same entity, which is distinct from conventional PEComa and Xp11 translocation renal cell carcinoma. Based on these important findings, melanotic Xp11 neoplasm might be reclassified into a distinctive entity together with ASPS, independent from PEComa, in future revisions of the current WHO categories of tumors of soft tissue and bone for the improved reclassification. © 2020 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Low-grade neuroepithelial tumors (LGNT) show a broad histopathological spectrum and may be difficult to classify using current World Health Organization (WHO) criteria. A 57-year-old man came to medical attention because of headaches. The patient medical history was otherwise unremarkable. Magnetic resonance imaging (MRI) revealed a 2.5 cm lesion, partially cystic, with an increased signal on T2-weighted imaging, located in the right frontal lobe. The patient underwent right frontal craniotomy and the surgical specimen was entirely evaluated. Microscopic examination showed a tumor arranged predominantly in sheets and nests, with an infiltrative growth pattern and oligodendroglioma-like appearance. Tumor cells were round to oval with cytoplasmic clearing, hyperchromatic nuclei and inconspicuous nucleoli. Only one mitosis was identified. Necrosis was absent. Differential diagnostic considerations included oligodendroglioma, clear cell ependymoma, polymorphous low-grade neuroepithelial tumor of the young (PLNTY) and long-term epilepsy-associated tumor with clear cell morphology. Neoplastic cells showed positivity for glial fibrillary acidic protein (GFAP), oligodendrocyte transcription factor 2 (OLIG2), α-thalasemia X-linked mental retardation syndrome (ATRX) (retained nuclear expression) and CD34. Epithelial membrane antigen (EMA), neuronal nuclear antigen, microtubule-associated protein-2e, cyclo-oxygenase-2, chromogranin A and isocitrate dehydrogenase 1 (IDH1) (R132H) were negative. Ki-67 labeling index was 2-3%. Molecular analysis identified neither IDH1/IDH2 mutations nor 1p19q codeletion. Rapidly accelerated fibrosarcoma homolog B1 (BRAF) V600E mutation was also absent by both molecular and immunohistochemical testing. Polymerase chain reaction analysis revealed the presence of fibroblast growth factor receptor 3 (FGFR3)-transforming acidic coiled-coil (TACC) fusion. Taken together, the morphological, immunohistochemical and molecular findings supported the final diagnosis of PLNTY.

Ependymomas of the spinal cord remain confined to the CNS and vary in presentation, depending on WHO grade. Higher-grade lesions usually cannot be surgically removed due to their infiltrative growth pattern. Spinal cordectomy has been proposed as a rescue treatment to improve survival in patients with high-grade as well as recurrent lesions. This report details an instructive and unique case of long-term follow-up of a patient who underwent cordectomy from T-4 through S-5 for what was initially diagnosed as a high-grade glial neoplasm of the spinal cord in 1993. The patient lived symptom free for 13 years after spinal cord resection and then presented with numerous bilateral extraspinal (intraabdominal and intrathoracic) tumors, which eventually led to her death 15 years after the cordectomy. In this case, spinal cordectomy was effective in preventing the ascending spread of the neoplasm, but ultimately not effective in preventing recurrence in the plicated distal dural sac.

Cellular neurothekeomas (CNTs) are rare, benign cutaneous tumors that arise primarily on the head and neck, with a slight female predominance. CNTs with atypical features have been described, including those with an infiltrative growth pattern. Although CNTs with atypical features are benign, recognition of this entity can pose diagnostic challenges. Here, we report a case of CNT with an unusual clinical presentation on the left second digit, and with atypical histological features including an infiltrative growth pattern, which could have been mistaken for features of malignancy.