Gastric glomus tumor: a case report and review of the literature.

INTRODUCTION: Gastric glomus tumor is a rare mesenchymal neoplasm. There are only a few cases of the tumor showing malignancy, and there are no specific guidelines for the management of this entity. CASE PRESENTATION: We present the case of a 53-year-old Caucasian male who was hospitalized for anemia. Computerized tomography of the abdomen depicted a mass between the pylorus of the stomach and the first part of the duodenum. Preoperative diagnosis was achieved with pathology examination of the biopsies taken via endoscopic ultrasound and upper gastrointestinal endoscopy. An antrectomy with Roux-en-Y anastomosis and appendicectomy, due to suspicion of appendiceal mucocele, were performed. The patient had an uneventful postoperative recovery and was discharged 5 days later. DISCUSSION: Preoperative diagnosis of a gastric glomus tumor is difficult owing to the location of the tumor and the lack of specific clinical and endoscopic characteristics. Furthermore, it is exceptional to establish diagnosis with biopsies taken through endoscopic ultrasound or upper gastrointestinal endoscopy, prior to surgical resection. Although most glomus tumors are benign and are not known to metastasize, there are rare examples of glomus tumors exhibiting malignancy. Treatment of choice is considered wide local excision with negative margins. However, long-term follow-up is required as there is the possibility of malignancy. CONCLUSION: The aim of this report is to enlighten doctors about this uncommon pathologic entity. Surgical resection is considered the golden standard therapy to establish a diagnosis and evaluate the malignant potential.

IgG4-related sclerosing cholangitis: not always an obvious entity.

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a distinct type of cholangitis, currently recognized as a biliary manifestation of IgG4-related disease. We present a case of type 3 IgG4-SC in a patient with normal IgG4 serum levels, surgically treated for suspicion of cholangiocarcinoma. This case highlights that differentiating between isolated IgG4-SC and cholangiocarcinoma can present a challenging diagnostic dilemma.

Could Mismatch Repair Status Serve as a Biomarker for Immunotherapy in Endometrial Carcinoma?

AIM: To study whether mismatch repair (MMR) status is related to the expression of programmed cell death-ligand 1 (PD-L1) and CD8 counts in a series of grade 3 endometrial carcinomas. MATERIALS AND METHODS: The expression of MMR protein PD-L1 and CD8+ cell count were evaluated by immunohistochemistry and related to several clinicopathological parameters. RESULTS: Among 105 endometrial carcinomas, 40% were of endometrioid and 60% of non-endometrioid histology. MMR deficiency was observed in 28.6% of cases and was related to endometrioid histology (p<0.001), positive PD-L1 expression (p=0.047) and high CD8+ cell count (p=0.022). When examined by histotype, endometrioid MMR-deficient tumors were related only to PD-L1 expression (p=0.032) but not to high CD8+ cell count (p=0.231), whereas non-endometrioid MMR-deficient carcinomas were not related to either of these markers. MMR deficiency was associated with PD-L1+/CD8high status (p=0.006), whilst MMR proficiency was associated with PD-L1-/CD8low status. In MMR-proficient tumors, high CD8+ cell infiltration alone and combined with PD-L1- status was associated with better progression-free survival (p=0.013 and p=0.04, respectively). CONCLUSION: MMR-deficient high-grade endometrioid tumors might be more likely to benefit from immunotherapy compared to other grade 3 endometrial carcinomas.

Endoscopic full-thickness resection of colorectal lesions with the full-thickness resection device: clinical experience from two referral centers in Greece.

BACKGROUND: Endoscopic full-thickness resection (EFTR) using the full-thickness resection device (FTRD®) is an invasive treatment for colorectal lesions not resectable by conventional endoscopic techniques. This study presents the first Greek experience of the FTRD® procedure, assessing the efficacy and safety of EFTR. METHODS: We conducted a retrospective analysis of 17 consecutive patients treated with the FTRD® at 2 referral centers from October 2015 through December 2018. The indications included difficult adenomas (non-lifting and/or at difficult locations), early adenocarcinomas and subepithelial tumors. Primary endpoints were technical success and R0 resection. RESULTS: Technical success and R0 resection were achieved in 82.3% procedures (14/17) and in 87.5% of those with difficult adenomas (8 patients). In the subgroup with carcinomas (n=3), the rate of technical success and R0 resection was 66.6%, while in the subgroup with subepithelial tumors (n=6) the rate was 83.3%. Technical success and R0 resection were significantly lower for lesions >20 mm vs. ≤20 mm (P=0.0429). In the 17 patients a total of 3 adverse events occurred (17.6%) and one of the patients underwent laparoscopic appendectomy because of EFTR around the appendix. CONCLUSIONS: Our study showed favorable results concerning EFTR feasibility, efficacy and safety, especially for lesions ≤20 mm, non-lifting adenomas, and subepithelial tumors. Technical success, R0 resection, and adverse events rates were comparable with previously published data. Larger randomized studies are needed to better define the clinical benefit and long-term outcomes of EFTR in selected patients.

The impact of programmed cell death-ligand 1 (PD-L1) and CD8 expression in grade 3 endometrial carcinomas.

BACKGROUND: To evaluate the expression of programmed cell death-ligand 1 (PD-L1) and CD8 in high-grade endometrial carcinomas and relate it to several clinicopathological parameters. METHODS: One hundred and one (101) patients with high-grade endometrial carcinomas who were completely surgically staged were included in this study. PD-L1 and CD8 + expression was evaluated by immunohistochemistry. RESULTS: In our cohort, 47 women (46.5%) had endometrioid carcinomas and 54 patients (53.5%) were diagnosed with non-endometrioid cancers. In endometrioid carcinomas, there was a significantly higher rate of positivity for PD-L1 expression (p = 0.042) and of intraepithelial CD8 + cell counts (p = 0.004) as opposed to non-endometrioid cancers. There were no significant relationships with any of the other clinicopathological features under study. Univariate and multivariate analysis revealed that only high intraepithelial CD8 + counts (p = 0.01) was associated with longer progression-free survival. Tumors positive for PD-L1 and high intraepithelial CD8 expression were mainly of endometrioid histology, whilst PD-L1-positive/CD8 low and PD-L1-negative/CD8 low tumors were mostly non-endometrioid carcinomas (p = 0.01). PD-L1 negative/CD8 high tumors had the longest progression-free survival (p = 0.032). CONCLUSIONS: In grade 3 endometrial carcinomas, both of endometrioid and non-endometrioid type, high intraepithelial CD8 + counts represent an independent favorable prognostic factor and when related to PD-L1-negative tumors, a longer progression-free survival can be predicted. Immunotherapy could probably be considered for PD-L1-positive/CD8 + high tumors, which were mostly of endometrioid histology.

Comparison of digital versus fiberoptic cholangioscopy in patients requiring evaluation of bile duct disease or treatment of biliary stones.

BACKGROUND: Recently, the introduction of the novel digital SpyGlass™ DS Direct Visualization system (Boston Scientific Corp., Natick, MA, USA) has signaled the transition into the era of digital single-operator cholangioscopy (D-SOC). We sought to compare the clinical utility between fiberoptic single-operator cholangioscopy (F-SOC) and D-SOC in a tertiary-care referral center in Greece. METHODS: This was a retrospective analysis of a prospective database of single-operator cholangioscopy (SOC) procedures performed over an 8-year period (2009-2017) at a single tertiary-care referral center. The study population consisted of consecutive adults referred for cholangioscopy for a variety of clinical indications, including biliary strictures, difficult biliary stones and migrated or occluded pancreatic or biliary stents. RESULTS: A total of 2763 endoscopic retrograde cholangiopancreatography procedures were performed during the study period. Overall, SOC was performed in 68 (2.46%) procedures (F-SOC=39, D-SOC=29), showing a significant increase in the utilization of cholangioscopy during the D-SOC (29/599; 4.84%) compared with the F-SOC (39/2124; 1.83%) period (P=0.0001). The overall technical success of diagnostic SOC was 69.1% (38/55), being marginally higher for D-SOC (83.3%) than for F-SOC (58.1%), although not reaching statistical significance (P=0.07). CONCLUSIONS: D-SOC was utilized more frequently in our tertiary-care non-academic referral center, demonstrating a favorable safety profile and a trend towards a marginally higher technical success rate for the diagnosis of biliary strictures compared with F-SOC.

Pilomatrix carcinoma: More malignant biological behavior than was considered in the past.

Pilomatrix carcinoma is a very rare malignancy, with ~130 cases reported in the literature. In the past, pilomatrix carcinoma was considered to be a low-grade malignant tumor. Currently, however, its significant recurrence and metastatic potential has been well documented. Lymph node and systemic metastases are frequently observed. Wide surgical excision of the primary lesion is the principal modality of treatment, whereas adjuvant radiotherapy may be beneficial in local tumor control. Lymph node metastases may be treated surgically or with radiotherapy. Systemic disease is not responsive to chemotherapy, and is hence associated with a poor prognosis. Since the majority of nodal and systemic metastases present after the initial diagnosis and treatment, follow-up examinations of these patients may be warranted, despite the currently inadequate treatment options. In the present study, a case of pilomatrix carcinoma of the parotid region with early local recurrence only 2 months after complete excision with negative surgical margins is reported. The local recurrence was treated by excision and radiotherapy. The associated literature is also discussed.

Cold snare polypectomy with pull technique of flat colonic polyps up to 12 mm: a porcine model.

BACKGROUND: Most colonoscopic complications are polypectomy-related and occur more frequently during the removal of numerous small polyps. Therefore, it is important to have the best polypectomy approach of small polyps. The aim of the present animal study was to investigate the effectiveness and safety of cold snare polypectomy (CSP) technique combined with light pull of the snare in order to peel the mucosal and upper submucosal layer, namely pull CSP (PCSP), for the removal of polyps sized up to 12 mm in porcine models. METHODS: We performed a series of polypectomies in the pig colon with a double-channel experimental gastroscope using PCSP technique. RESULTS: Thirty cases of "polyps" larger than 7 mm and up to 12 mm were treated using PCSP technique. No sign of perforation or bleeding was observed in all cases performed with PCSP. CONCLUSIONS: According to our preliminary results in this animal model, PCSP could be a safe and effective technique for flat colonic polyp removal up to 12 mm in size consistent with the basic polypectomy principles.

A giant pregnancy-associated intra-abdominal desmoid tumour: not necessarily a contraindication for subsequent pregnancy.

Desmoid tumours are rare mesenchymal tumours, often locally invasive and characteristically associated with a high local recurrence rate after resection. A potential aetiological role for female hormones is indicated. Pregnancy-associated desmoid tumours are almost exclusively located in the abdominal wall. An essential issue is how to counsel women who have had a pregnancy-associated desmoid tumour and subsequently wish to bear a child. A considerably rare case of a patient with a resection of a giant pregnancy-associated, 33 cm in diameter, intra-abdominal desmoid tumour is presented. After a subsequent pregnancy, the patient delivered healthy twins 26 months later. Fifty-four months after treatment, there are no signs of recurrent or second desmoid tumour. Although rarely located in the abdomen, pregnancy-associated desmoid tumours should be included in the differential diagnosis of intra-abdominal tumours detected during or shortly after pregnancy. Based on this case and a few others reported in the literature, subsequent pregnancy does not necessarily seem to be a risk factor for recurrent or new disease.

Prehistological evaluation of benign and malignant pigmented skin lesions with optical computed tomography.

Discrimination of benign and malignant melanocytic lesions is a major issue in clinical dermatology. Assessment of the thickness of melanoma is critical for prognosis and treatment selection. We aimed to evaluate a novel optical computed tomography (optical-CT) system as a tool for three-dimensional (3-D) imaging of melanocytic lesions and its ability to discriminate benign from malignant melanocytic lesions while simultaneously determining the thickness of invasive melanoma. Seventeen melanocytic lesions, one hemangioma, and normal skin were assessed immediately after their excision by optical-CT and subsequently underwent histopathological examination. Tomographic reconstructions were performed with a back-propagation algorithm calculating a 3-D map of the total attenuation coefficient (AC). There was a statistically significant difference between melanomas, dysplastic nevi, and non-dysplastic nevi, as indicated by Kruskal-Wallis test. Median AC values were higher for melanomas compared with dysplastic and non-dysplastic nevi. No statistically significant difference was observed when thickness values obtained by optical-CT were compared with histological thickness using a Wilcoxon sighed rank test. Our results suggest that optical-CT can be important for the immediate prehistological evaluation of biopsies, assisting the physician for a rapid assessment of malignancy and of the thickness of a melanocytic lesion.

Development of lepromatous leprosy following etanercept treatment for arthritis.

We present a case of a patient treated with etanercept (TNF-a antagonist) for psoriatic arthritis, who then developed clinical symptoms of lepromatous leprosy. She presented with multiple erythematous plaques on trunk, face and extremities, saddle nose deformity, alopecia, articular deformities of the feet and peroneal neuropathy. The clinical suspicion of Hansen's Disase was confirmed by the biopsy findings (lepromatous leprosy). On further questioning, the patient stated that her father was diagnosed with leprosy 70 years ago and had spent some years in a leper colony in Spinalonga island in Southern Greece in the 1940s. This first report of Hansen's disease after administration of etanercept highlights the need of careful risk assessment of patients for whom antiTNF treatment is planned.

Successful treatment of cutaneous leishmaniasis with intralesional aminolevulinic acid photodynamic therapy.

Leishmaniasis is a protozoan infectious disease that often affects the skin and may acquire a chronic and difficult to treat course. Topical photodynamic therapy (PDT) is a novel treatment which involves the selective uptake of a photosensitizing agent. Exposure to an appropriate light source in the presence of oxygen leads to formation of reactive oxygen species and destruction of the target cells. We report on the successful treatment of a 69-year-old patient with a relapse of long-standing cutaneous leishmaniasis using intralesional aminolevulinic acid-PDT.

Factors associated with the prevalence of atypical nevus in a Mediterranean pigmented skin lesion clinic.

Atypical melanocytic nevi constitute central risk factor and are precursor lesions for cutaneous melanoma. Data regarding factors associated with their prevalence are mainly derived from fair-skinned populations, whereas little is known regarding their epidemiological associations in darker-skinned, chronically sun-exposed populations. The aim of this study was to identify risk factors for the occurrence of at least one atypical nevus on Crete, the southernmost island of Greece. This hospital-based case-control study included 143 patients and 189 controls with at least one atypical nevus presented at the pigmented skin lesion clinic of the University of Crete. All participants were interviewed and underwent complete skin examination by the same two experienced dermatologists. Multivariate logistic regression analysis was used to adjust for potential confounders. In the multivariate analysis, common melanocytic nevi [odds ratio (OR): of 2.2, 7.5, and 58.9 for the presence of 11-25, 26-100, and >100 common nevi, respectively] and recreational sun exposure (OR: 4.4) increased significantly the risk of the presence of atypical nevus. A decreased risk for atypical nevi was related to an increasing age (OR: 0.96/age), and professional sun exposure (OR: 0.5). Intermittent, recreational sun exposure is mainly associated with the prevalence of atypical nevi in our sample and this effect does not depend on skin phototype. Promotion of sun protection, especially in patients with high numbers of common nevi, might serve as a measure to prevent the development of atypical nevi.

Pseudomembranous colitis with presence of signet - ring cells: report of two cases and review of the literature.

WE DESCRIBE TWO CASES WITH PSEUDOMEMBRANOUS COLITIS: the first case concerns a 73-year-old male patient with clinical history of cardiovascular disease and pulmonary insufficiency, admitted to the hospital for urinary tract infection and was treated with broad-spectrum antibiotics for a long period of time. During his hospitalization he developed abdominal pain and haematochezia. The second case concerns a 64-year-old woman treated with antibiotics for community-acquired pneumonia. After the treatment she developed abdominal pain and diarrhea. In both cases the colonic biopsy showed pseudomembranous colitis with presence of signet-ring cells within dilated crypts of the colonic mucosa. The presence of signet-ring cells is a rare finding in pseudomembranous colitis and may lead to misdiagnosis of signet-ring carcinoma of the colon.

Pelvic hemangiopericytoma: the role of diffusion weighted imaging in targeting the biopsy site and in monitoring the tumour response to radiotherapy.

BACKGROUND: Despite advances in imaging, the accurate characterization of soft tissue tumours remains a challenging task. Furthermore, the interpretation of post treatment changes and evaluation of tumour response to therapy is another complicating issue regarding soft tissue tumour imaging. CASE REPORT: Herein, a patient with a pelvic hemangiopericytoma, by whom different diagnostic imaging methods were used, is presented. CONCLUSIONS: Diffusion weighted imaging (DWI) might provid useful information in guiding biopsy and enabled monitoring of the radiation therapy results.

Melanoma of the vulva: a pigmented lesion is also significant in a non-sun-exposed area.

PURPOSE: The foremost important aetiological factor for malignant melanoma is considered to be sunlight exposure. However, primary lesions are also seen in non-sun-exposed areas. Vulvar melanoma is rare and associated with impaired outcome. Herein, we attempt to increase physicians' awareness for early diagnosis in order to improve prognosis. CASE REPORT: A 64-year-old female presented with pruritus and irritation at her external genitalia. At examination a pigmented lesion of the vulva 3 cm in diameter was seen. Incisional biopsy revealed melanoma. Clinical examination and imaging studies did not show evidence for metastatic disease. She underwent wide excision of the melanoma with primary wound closure and biopsy of sentinel lymph nodes, which were free of disease. After a follow-up period of 43 months, she remains free of disease. CONCLUSIONS: Literature review shows that prognosis is poorer than at other sites, most probably due more advanced disease at diagnosis. This case demonstrates that early diagnosis by physicians aware of this malignancy at this relatively rare site is of crucial importance since it may result in improved outcome, similar to that of melanoma at more common sites.