Overlapping Case of Advanced Systemic Sclerosis and IgG4-Related Disease after Autologous Hematopoietic Stem Cell Transplantation.

Both scleroderma and immunoglobulin G4-related disease (IgG4-RD) are systemic fibro-inflammatory diseases characterised by lymphoplasmacytic infiltrates. IgG4-RD and systemic sclerosis (SSc) may share common pathophysiological mechanisms, but no examples of co-occurrence of the diseases have been found. Autologous haematopoietic stem cell transplantation (AHSCT) is implemented in selected rapidly progressive SSc with a high risk of organ failure. However, existing guidelines are based on clinical trials that do not represent the entire patient population and exclude critically ill patients with no therapeutic alternatives. Examples of AHSCT in IgG4-RD are absent. We report the case of a 44-year-old female patient with overlapping progressive diffuse SSc and sinonasal IgG4-RD. After 11 years of ineffective SSc treatment, AHSCT was performed. The 63-month follow-up showed a regression of SSc symptoms. AHSCT was not intended as treatment in the case of IgG4RD, although the first symptoms of the disease developed before transplantation. The sinus lesions progressed after AHSCT and remained indolent only after surgical treatment (bilateral ethmoidectomy, sphenoidotomy, intranasal buccal antrostomy), which allowed histopathological confirmation of IgG4-RD.

Late Recurrence of a Rare Middle Ear Neuroendocrine Tumor With an Intracranial Extension to the Temporal Fossa: A Case Report.

We report a clinical case of an extremely rare neuroendocrine tumor of the right middle ear (MeNET) that recurred after 13 years with a local extension into the right temporal fossa. In the current medical literature, there are approximately 150 cases of MeNETs and even fewer cases with more than 10 years of follow-up, recurrence, and intracranial tumor progression. Therefore, we believe that this paper can make an important contribution to the existing and future knowledge about this disease. The purpose of this article is to present our experience in treating such a rare neoplasm in a 35-year-old woman. The patient initially complained of worsening hearing in her right ear over the past year. The final diagnosis was made based on the findings of computed tomography (CT), magnetic resonance imaging (MRI), and histological and immunohistochemical evaluation of excisional biopsies of the original and recurrent tumors. The primary tumor masses were removed with clear resection margins, and the ossicular chain was reconstructed. The patient has been monitored clinically and radiologically with temporal bone CTs every year and MRIs three times in general since then. A postoperative audiogram showed remaining mixed hearing loss in the right ear that eventually worsened as the tumor grew. Tumor recurrence and progression after 156 months (13 years) were seen on CT and MRI, requiring further treatment. After resection of the recurrent tumor, paresis of the right facial nerve developed, which was treated with dexamethasone. The surgical treatment caused the initial symptoms to disappear, but the facial nerve paresis persisted with mild functional improvement. The patient is not receiving adjuvant radiotherapy and is being monitored closely because the tumor may recur in the future.

Hybrid intercalated duct lesion of the parotid: A case report.

BACKGROUND: Intercalated duct lesions (IDLs) are considered relatively benign and rare tumors of salivary glands, that were only described recently. Their histopathological appearance may range from ductal hyperplasia to encapsulated adenoma with hybrid patterns of both variants. It is thought that IDLs may be the precursor for malignant proliferations, therefore their correct diagnosis remains crucial for proper lesion management. It is the first reported IDL case arising from the accessory parotid gland (APG), which stands for less frequent but higher malignancy rate tumor developmental area. CASE SUMMARY: A 24-years-old male with no accompanying diseases was referred to the hospital with a painless nodule on the right cheek. On physical examination, the stiff, immobile, and painless mass was palpable in the anterior portion of the right parotideomasseteric region, just superior to the parotid duct. Ultrasound examination demonstrated 1.5 cm × 1.0 cm hypoechogenic mass on the anterior part of the right parotid gland. Ultrasound-guided fine needle aspiration cytology, followed by liquid-based fine needle aspiration biopsy were performed. However, the results were uninformative. A contrast-enhanced magnetic resonance imaging (MRI) of the parotid was obtained, demonstrating a 1.5 cm × 1.0 cm × 0.5 cm tumor with high intensity capsule together with low intensity core in the very anterior part of right superficial lobe, situated in the APG. An MRI features were uncharacteristic to common parotid tumors, therefore surgical resection followed up. After histopathological examination, the final diagnosis of hybrid IDL was confirmed. CONCLUSION: Fine needle aspiration biopsy might not always be diagnostic, and given the malignant potential, the surgical resection of such lesion remains the treatment of choice.

A Case Report of Rare Sacral Solitary Fibrous Tumor.

Huge primary epidural solitary fibrous tumors in the sacrum are a rare clinical entity. The purpose of this article is to present our experience in treating such large and complex neoplasms in a 31-year-old woman. The patient complained of constant nocturnal bilateral hip and lower back pain and unilateral radicular symptoms (numbness, paresthesias) in the left S1/S2 dermatomal distribution. Diagnostic imaging, biopsy, preoperative endovascular embolization, two-staged tumor resection, and lumbosacroiliac fusion were performed. The treatment resolved the patient's neurological symptoms and resulted in overall good postoperative functionality. The patient has been in remission for more than five years despite her refusal of adjuvant radiotherapy.

Acquired nasolacrimal duct obstruction: clinical and histological findings of 275 cases.

BACKGROUND: Acquired nasolacrimal duct obstruction is a blockage of the lacrimal outflow system usually caused by local nonspecific inflammation of the lacrimal sac and the nasolacrimal duct. However, cases exist where the primary nasolacrimal system obstruction is caused by malignancies. Our aim was to investigate lacrimal sac pathologies in patients with acquired nasolacrimal duct obstruction and compare their clinical manifestations. METHODS: This retrospective clinical study included 275 patients with acquired nasolacrimal duct obstruction who underwent external dacryocystorhinostomy and lacrimal sac biopsy. Cases were classified into tumor or nonspecific pathology groups and subdivided according to the level of inflammation. Histological and clinical data were analyzed. RESULTS: Three tumors (1.1%) (an adenoid cystic carcinoma, an eccrine spiradenoma and small B cell lymphoma) were diagnosed. Chronic nongranulomatous inflammation was the most common histological finding, corresponding to 194 cases (70.5%). The other 81 (29.5%) were subacute, acute forms of nongranulomatous inflammation, tumors and fibrosis cases. Epiphora with continuous purulent discharge was the most common clinical sign reported by 144 (52.4%) patients, and two (0.7%) patients had a palpable mass near the medial canthal tendon, which was identified as an eccrine spiradenoma and small B cell lymphoma. There was no significant difference in the clinical symptoms, duration or case history between the nonspecific pathology and tumor groups (p = 0.292). CONCLUSIONS: Chronic nongranulomatous inflammation of the lacrimal sac was the most common finding among acquired nasolacrimal duct obstruction cases. There were no associations between the histological findings and clinical presentation. The authors recommend a lacrimal sac biopsy only in cases when a tumor is clinically suspected.

Associations between Physical Function, Bone Density, Muscle Mass and Muscle Morphology in Older Men with Sarcopenia: A Pilot Study.

Background and Objectives: It is thought that muscle and bone interact only on a biomechanical level, however, some research is now emerging that links bone and muscle on a cellular level. The aim of this study was to explore associations between physical function, muscle mass and bone density in community-dwelling elderly men with sarcopenia. A secondary goal was to analyze if muscle morphology was associated with bone density and physical functioning. Materials and Methods: Body composition was measured by dual-energy X-ray absorptiometry (DXA). Bone density was evaluated according to WHO criteria. Sarcopenia was diagnosed according to European Working Group on Sarcopenia in Older People (EWGSOP) criteria: low muscle mass and low muscle strength or low physical performance. Microbiopsy of musculus vastus lateralis was performed with a disposable muscle microbiopsy system. The perimeter and cross-sectional area of muscle fibers were calculated using image analysis software in whole slide images; type of fibers and their distribution were evaluated as well. Results: A total of 151 men, 60 years or older were included in this study. Mean age of the subjects was 72.9 ± 8.02 years. Sarcopenia was diagnosed in 45 (29.8%) men. Multiple significant correlations were found between bone mineral density, lean mass, appendicular lean mass, arm and leg lean mass, gait speed, balance test and handgrip strength in sarcopenic men. Lean mass was associated with femoral neck BMD (bone mineral density; r = 0.418, p = 0.006) and handgrip strength (r = 0.553, p < 0.001). In the sarcopenia group, 25 muscle biopsies were examined. In 9 sarcopenic men with T-scores equal or below -2.5, the muscle fiber area had a significant correlation with the balance test (r = 0.73, p = 0.025). Conclusions: In men with sarcopenia, low lean muscle mass was associated with low femoral neck BMD and low muscle strength. In sarcopenic men with osteoporosis, low muscle fiber area was associated with low scores in a balance test.

Systematic and MRI-Cognitive Targeted Transperineal Prostate Biopsy Accuracy in Detecting Clinically Significant Prostate Cancer after Previous Negative Biopsy and Persisting Suspicion of Malignancy.

Background and objectives: Overdiagnosis, overtreatment, and the need for repeated procedures caused by transrectal ultrasound guided prostate biopsies and their related complications places a heavy burden on healthcare systems. This was a prospective cohort validating study to access the clinical accuracy of systematic and MRI-cognitive targeted transperineal prostate biopsies in detecting clinically significant prostate cancer after a previous negative biopsy and persistent suspicion of malignancy. The primary goal was to assess the ability of multiparametric magnetic resonance imaging (mpMRI) to detect clinically significant prostate cancer with an additional goal to assess the diagnostic value of systematic and MRI-cognitive transperineal biopsies. Materials and Methods: In total, 200 patients were enrolled who had rising serum prostate specific antigen (PSA) levels for at least 4 months after a previous negative transrectal ultrasound (TRUS) biopsy. All eligible men underwent 1.5T prostate mpMRI, reported using the Prostate Imaging Reporting and Data System version 2 (PI-RADS v2), followed by a 20-region transperineal prostate systematic biopsy and additional targeted biopsies. Results: Systematic 20-core transperineal prostate biopsies (TPBs) were performed for 38 (19%) patients. Systemic 20-core TPB with additional cognitive targeted biopsies were performed for 162 (81%) patients. Clinically significant prostate cancer (csPC) was detected for 31 (15.5%) patients, of which 20 (64.5%) cases of csPC were detected by systematic biopsy, eight (25.8%) cases were detected by targeted biopsy, and three (9.7%) both by systematic and targeted biopsies. Conclusions: Cognitive mpMRI guided transperineal target biopsies increase the detection rate of clinically significant prostate cancer after a previously negative biopsy. However, in a repeat prostate biopsy setting, we recommend applying a cognitive targeted biopsy with the addition of a systematic biopsy.

ECHO-7 oncolytic virus Rigvir® in an adjuvant setting for stage I uveal melanoma; A retrospective case report.

PURPOSE: To describe a case of choroidal melanoma treated with Rigvir® virotherapy in an adjuvant setting. OBSERVATIONS: A female patient born in 1956 presented with a small choroidal melanoma in October 2007. 34 months after transpupillary thermotherapy the state of her eye worsened until tumor growth was visualized. Despite photodynamic therapy and transpupillary thermotherapy the tumor continued to grow locally. In October 2016 enucleation was performed. Since gene expression profile testing disclosed a tumor (class 2) with a high risk of metastasis formation in 5 years, the patient sought options to prevent progression of the disease. In December 2016 virotherapy with Rigvir® was started with 3 administrations for 3 consecutive days. Therapy was continued once per week until March 2017, when the administrations were changed to once per month. The patient is being monitored by an ophthalmologist. She is stable with the virotherapy ongoing and magnetic resonance cholangiopancreatography (7 May 2018) and abdominal ultrasound (23 March 2019) imaging excludes metastasis formation. The quality of life is high. CONCLUSIONS: To the best of our knowledge, this is the first documented case of uveal melanoma treatment with virotherapy as an adjuvant therapy. Considering the few if any available treatments and the encouraging results of the present treatment, virotherapy should be evaluated more extensively as a potential treatment of uveal melanoma.

Combination treatment with nivolumab and Rigvir of a progressive stage IIC skin melanoma patient.

A 35-year-old male patient was diagnosed with stage IIC skin melanoma that rapidly progressed after surgery. Treatment was continued with radiotherapy, which did not stop further spread of disease and the patient was put on a combination of nivolumab and Rigvir. Subsequently, the progression has slowed.

Diagnosis, treatment, and outcomes of encapsulated papillary carcinoma: a single institution experience.

BACKGROUND: Encapsulated papillary carcinoma (EPC) is a rare entity of breast cancer accounting for approximately 1-2% of all breast tumours. There are no evidence-based guidelines for the treatment of EPC. MATERIALS AND METHODS: From the database of the National Centre of Pathology (NCP), we obtained pathology reports of 19 patients with histologically confirmed EPC, who were treated at the National Cancer Institute (NCI) in Vilnius, Lithuania, between July 2009 and July 2015. Demographic, diagnostic and treatment data were collected from medical records retrospectively. RESULTS: During the indicated period, 19 patients with EPC were treated at the NCI. Three of them had pure EPC, they were 74 to 81 years of age at the time of diagnosis (mean 76.7 years, median 75 years); all of them are still alive and no disease progression has been observed. Seven patients had EPC associated with carcinoma in situ. Nine patients had EPC associated with invasive breast ductal carcinoma. All patients underwent surgery, in most cases - wide local excision. Only one patient died. CONCLUSIONS: EPC is a rare form of breast cancer and usually presents with an invasive breast carcinoma or carcinoma in situ in postmenopausal women. Tumours have an excellent prognosis in the cases of pure EPC and in both EPC associated with carcinoma in situ (CIS) and invasive carcinoma.

Decreased expression of MT1E is a potential biomarker of prostate cancer progression.

Differentiation of indolent and aggressive prostate carcinoma (PCa) at the time of diagnosis is currently one of the major challenges. This study aimed at identification of prognostic biomarkers to aid in predicting biochemical recurrence (BCR) of the disease. Microarray-based gene expression profiling in tissues of 8 BCR and 8 No-BCR cases revealed expression differences of 455 genes, most of which were down-regulated in BCR cases. Eleven genes were selected for validation by real-time PCR in the first PCa cohort (N = 55), while seven of them were further validated in the second, independent, PCa cohort (N = 53). Down-regulation of MT1E (p < 0.001) and GPR52 (p = 0.002) expression and up-regulated levels of EZH2 (p = 0.025) were specific biomarkers of BCR in at least one of the two PCa cohorts, but only MT1E expression retained the independent prognostic value in a multivariate analysis (p < 0.001). DNA methylation analysis (114 PCa and 24 non-cancerous tissues) showed frequent MT1E methylation in PCa (p < 0.001) and was associated (p < 0.010) with the down-regulated expression in one PCa cohort. The results of our study suggest MT1E down-regulation as a potential feature of aggressive PCa.

Inflammatory myopathy in a patient with Aicardi-Goutières syndrome.

Aicardi-Goutières syndrome (AGS) is an inflammatory disorder belonging to the recently characterized group of type I interferonopathies. The most consistently affected tissues in AGS are the central nervous system and skin, but various organ systems and tissues have been reported to be affected, pointing to the systemic nature of the disease. Here we describe a patient with AGS due to a homozygous p.Arg114His mutation in the TREX1 gene. The histologically proven inflammatory myopathy in our patient expands the range of clinical features of AGS. Histological signs of muscle biopsies in the proband, and in two other AGS patients described earlier, are similar to those seen in various autoimmune myositises and could be ascribed to inapproapriate IFN I activation. In view of signs of possible mitochondrial damage in AGS, we propose that mitochondrial DNA could be a trigger of autoimmune responses in AGS.

Death Caused by Hepatodiaphragmatic Interposition of the Colon.

Hepatodiaphragmatic interposition of the colon is a rare, usually asymptomatic, anomaly and is typically an incidental radiologic finding. There are few cases in the literature describing the symptomatic form of the condition, known as Chilaiditi syndrome. In some cases, it may be accompanied by various severe complications. If symptoms are present, usually conservative treatment is given. However, conservative treatment only addresses the symptoms but does not prevent their recurrence and possible complications. Our present report shows that this anomaly may not only cause symptoms, but may also progress and cause severe complications, in our case-megacolon leading to right heart failure and, ultimately, death. To date, however, there have been no literature reports of death caused by colonic interposition. Therefore, it is important to draw attention to the importance of this anomaly and its appropriate diagnosis and treatment to ensure the most favorable patient outcomes.

Heterogeneity of DNA methylation in multifocal prostate cancer.

Most prostate cancer (PCa) cases are multifocal, and separate foci display histological and molecular heterogeneity. DNA hypermethylation is a frequent alteration in PCa, but interfocal heterogeneity of these changes has not been extensively investigated. Ten pairs of foci from multifocal PCa and 15 benign prostatic hyperplasia (BPH) samples were obtained from prostatectomy specimens, resulting altogether in 35 samples. Methylation-specific PCR (MSP) was used to evaluate methylation status of nine tumor suppressor genes (TSGs), and a set of selected TSGs was quantitatively analyzed for methylation intensity by pyrosequencing. Promoter sequences of the RASSF1 and ESR1 genes were methylated in all paired PCa foci, and frequent (≥75 %) DNA methylation was detected in RARB, GSTP1, and ABCB1 genes. MSP revealed different methylation status of at least one gene in separate foci in 8 out of 10 multifocal tumors. The mean methylation level of ESR1, GSTP1, RASSF1, and RARB differed between the paired foci of all PCa cases. The intensity of DNA methylation in these TSGs was significantly higher in PCa cases than in BPH (p < 0.001). Hierarchical cluster analysis revealed a divergent methylation profile of paired PCa foci, while the foci from separate cases with biochemical recurrence showed similar methylation profile and the highest mean levels of DNA methylation. Our findings suggest that PCa tissue is heterogeneous, as between paired foci differences in DNA methylation status were found. Common epigenetic profile of recurrent tumors can be inferred from our data.

Quantitative histopathological assessment of ocular surface squamous neoplasia using digital image analysis.

The aim of this retrospective pilot study was to evaluate the Aperio nuclear V9 algorithm as an image analysis tool to observe the histopathological changes of ocular surface squamous neoplasia (OSSN). A histopathological assessment, including the Ki-67 proliferative index (PI) of immunohistochemically-stained tumor conjunctiva (TC) and healthy conjunctiva (HC) tissues, was performed in six cases of OSSN. The Aperio V9 algorithm was applied to digital images of the tissue specimens to count the Ki-67 PI and to measure the nuclear area indices. This digital algorithm was validated using stereological and visual analysis methods. The visual scoring of Ki-67 PI ranged from 22 to 60% (mean, 38.5%), and from 5 to 20% (mean 9.5%) in TC and HC tissue, respectively. The computer-aided analysis, using the Aperio nuclear V9 algorithm, revealed that the Ki-67 PI ranged from 21.5 to 43.5% (mean, 33.6%), and from 1.9 to 21.0% (mean, 11.8%) in the TC and HC tissue, respectively. The stereological method demonstrated that the Ki-67 PI ranged from 30.1 to 51.5% (mean, 41.0%), and from 3.2 to 30.1% (mean, 15.1%) in the TC and HC tissues, respectively. The strongest association in the collinearity of regression analysis was observed between the Aperio nuclear V9 algorithm/stereological models in the TC tissue (r2=0.7; P=0.04) and the HC tissue (r2=0.7; P=0.03), and the visual/stereological models in the TC tissue (r2=0.7; P=0.04) and the visual/Aperio nuclear V9 algorithm models in the HC tissue (r2=0.7; P=0.04). A weak and statistically insignificant association was identified between the visual/Aperio nuclear V9 algorithm analysis in the TC tissue (r2=0.4; P=0.2) and the visual/stereological models in the HC tissue (r2=0.5; P=0.13). No significant difference was observed between the nuclear area of the TC (mean, 36.5 µm2) and HC (mean, 35.7 µm2; P=0.88) tissues. It was concluded that the Aperio nuclear V9 algorithm is a useful tool for the reliable analysis of histopathological changes of OSSN. The results of this computer-aided algorithm correlate strongly with the stereological method when assessing the Ki-67 PI.

Prognostic value of RASSF1 promoter methylation in prostate cancer.

PURPOSE: Patients with prostate cancer who have biochemical recurrence after curative therapy are at higher risk for distant metastasis and cancer specific death. Assessment of aberrant DNA methylation in urine might complement currently used clinical prognostic factors and serve as a noninvasive tool for early prediction of biochemical recurrence after radical prostatectomy. MATERIALS AND METHODS: Promoter methylation of 7 genes was evaluated by methylation sensitive polymerase chain reaction in 149 prostate cancer tissues, 37 noncancerous prostate tissues and 17 benign prostatic hyperplasia samples. Quantitative polymerase chain reaction was used for DNA methylation analysis of the urine of 253 patients with prostate cancer and 32 with benign prostatic hyperplasia. RESULTS: In prostate cancer tissue the most frequently methylated genes were RASSF1, GSTP1 and RARB, which combined were positively identified in 85% of cases. These genes were also methylated in the urine of 60% of patients with prostate cancer. RASSF1 was methylated in 45% of prostate cancer urine samples with methylation intensity significantly higher in prostate cancer than in benign prostatic hyperplasia cases (p = 0.018). In a univariate model RASSF1 methylation and the total number of methylated genes in prostate cancer tissue were predictive of time to biochemical recurrence (p = 0.019 and 0.043, respectively). On multivariate analysis RASSF1 methylation together with pathological stage was the most significant predictor of biochemical recurrence in patients with Gleason score 6 tumors when analyzed in tissue and urine (p ≤0.001). CONCLUSIONS: Hypermethylation of RASSF1 in cancerous tissue and urine from patients with prostate cancer correlated with biochemical recurrence after radical prostatectomy. The prognostic potential of this biomarker deserves further investigation.

Combined analysis of TMPRSS2-ERG and TERT for improved prognosis of biochemical recurrence in prostate cancer.

Prostate cancer (PCa) is a heterogeneous disease with diverse clinical outcomes. TMPRSS2-ERG is the most common gene fusion in PCa, whereas activation of telomerase is a common feature of various malignancies. The aim of our study was to explore the combined utility of these and some other biomarkers in predicting biochemical recurrence after radical prostatectomy. Prostate specimens and urine sediments from 179 previously untreated patients with pT2-pT3 stage PCa were analyzed for expression of telomerase (TERT and TR) and the TMPRSS2-ERG fusion gene by means of reverse transcription PCR. Real-time PCR was used for quantification of ERG and SPINK1 expression. In total, 74% (117/158) of the prostate adenocarcinomas were positive for the TMPRSS2-ERG and/or TERT expression. Noninvasively, these transcripts were identified in 31% (19/61) of catheterized urine specimens. Significantly higher expression of ERG was detected in TMPRSS2-ERG-positive tumors (P<0.0001), whereas more intense expression of SPINK1 was characteristic for the TMPRSS2-ERG-negative tumors (P=0.003). TERT-positive cases also had elevated levels of ERG (P=0.016), suggesting a possible link between aberrant expression of ERG and reactivation of TERT in prostate tumors. The cases negative for both transcripts, TMPRSS2-ERG and TERT, rarely recurred (P=0.014) and showed significantly longer biochemical recurrence-free period (P=0.022) as compared to the TMPRSS2-ERG and/or TERT-positive cases. The results of our study suggest that combined analysis of TMPRSS2-ERG and TERT expression can be a valuable tool for early prediction of biochemical recurrence of PCa after radical prostatectomy.

Tumor suppressor gene ZAC/PLAGL1: altered expression and loss of the nonimprinted allele in pheochromocytomas.

ZAC/PLAGL1 is a novel imprinted tumor suppressor gene encoding an important inducer of cell cycle arrest and apoptosis, and found to be lost during tumorigenesis. We analyzed the significance of ZAC in the development of a rare, usually benign tumor of the adrenal gland: pheochromocytoma (PCC). Twenty-four PCCs were analyzed for the loss of the active nonimprinted allele of ZAC, and nine of the twenty-four PCCs were also assayed for expression of the protein. In thirteen of the cases, a paired nonmalignant tissue was available for analysis. Methylation-specific polymerase chain reaction revealed frequent (15 of 23, 65%) loss of unmethylated DNA in the imprinting control region of ZAC. Immunohistochemistry identified reduced ZAC expression in 56% (5 of 9) of the subset cases. Four of the five PCC cases where reduced expression of ZAC was observed were also positive for the loss of the active ZAC allele. Additionally, the loss of ZAC expression was also found to be frequent in a series of capillary hemangioblastomas and gliomas (6 of 6, 100%, and 17 of 27, 63%, respectively) examined for comparison. In conclusion, our study suggests the involvement of the imprinted ZAC gene in the pathogenesis of PCC.

Ocular surface reconstruction using amniotic membrane following excision of conjunctival and limbal tumors.

PURPOSE: To report the clinical results of patients treated by preserved human amniotic membrane transplantation (AMT) following the removal of conjunctival and limbal tumors. METHODS: Retrospective noncomparative interventional case series of 9 patients (9 eyes) who underwent AMT after removal of conjunctival and limbal tumors with lesion-free margins and perilesional cryotherapy. RESULTS: The excised tumors were histopathologically examined and included 2 squamous cell carcinomas, 2 papillomas, and 5 nevi. Bulbar conjunctiva was involved in all of the eyes, limbus and cornea in 7 and 3 eyes, respectively. The mean extent of the limbal involvement was 4 clock hours (range 2-9, SD 2.4); the average diameter of the base of the tumor was 12.8 mm (range 10-20, SD 4.4). The mean follow-up time was 38 months (range 13-60, SD 15). No surgical or early postoperative complications were observed. In all eyes, complete healing of the tissue defect occurred, resulting in a stable, wet, and noninflamed epithelium. All eyes demonstrated a smooth ocular surface except one with a clinically insignificant symblepharon after the excision of a squamous cell carcinoma. Superficial peripheral corneal vascularization and opacification as a sign of partial limbal stem cell deficiency developed in 2 eyes. In one case, a recurrence of conjunctival papilloma was diagnosed after a 3-year follow-up. CONCLUSIONS: Amniotic membrane transplantation is an effective method of reconstruction following a conjunctival and limbal tumor excision and cryotherapy of surgical wound margins. In most cases, complete healing of an ocular surface can be achieved without any clinically significant complications.

Dementia and art: neuronal intermediate filament inclusion disease and dissolution of artistic creativity.

The paper presents a new case of neuronal intermediate filament inclusion disease (NIFID), a recently described new variant of early-onset frontotemporal dementia. Documented with repetitive brain images, morphologically proven cases additionally endorse evolving the clinical and pathological phenotype of NIFID. For the first time the paper describes the probable influence of NIFID on the artistic creativity of an accomplished artist showing rapid dissolution of artistic talent.