Typical Histopathological Features Research Articles

European consensus-based interdisciplinary guideline for diagnosis, treatment and prevention of actinic keratoses, epithelial UV-induced dysplasia and field cancerization on behalf of European Association of Dermato-Oncology, European Dermatology Forum, European Academy of Dermatology and Venereology and Union of Medical Specialists (Union Européenne des Médecins Spécialistes).

A collaboration of multidisciplinary experts from the European Association of Dermato-Oncology, the European Dermatology Forum, the European Academy of Dermatology and Venereology, and the European Union of Medical Specialists was formed to develop European recommendations on AK diagnosis and treatment, based on current literature and expert consensus. This guideline addresses the epidemiology, diagnostics, risk stratification and treatments in immunocompetent as well as immunosuppressed patients. Actinic keratoses (AK) are potential precursors of cutaneous squamous cell carcinoma (cSCC) and display typical histopathologic and immunohistochemical features of this malignancy in an early stage. They can develop into cSSC insitu and become invasive in a low percentage of cases. AK is the most frequent neoplasia in white populations, frequently occurring within a cancerous field induced by ultraviolet radiation. Since it cannot be predicted, which lesion will progress to cSCC and when treatment is usually recommended. The diagnosis of AK and field cancerization is made by clinical examination. Dermatoscopy, confocal microscopy, optical coherence tomography or line-field confocal-OCT can help in the differential diagnosis of AK and other skin neoplasms. A biopsy is indicated in clinically and/or dermatoscopically suspicious and/or treatment-refractory lesions. The choice of treatment depends on patients' and lesion characteristics. For single non-hyperkeratotic lesions, the treatment can be started upon patient's request with destructive treatments or topical treatments. For multiple lesions, field cancerization treatment is advised with topical treatments and photodynamic therapy. Preventive measures such as sun protection, self-examination and repeated field cancerization treatments of previously affected skin areas in high-risk patients are advised.

COMPARISON OF GUT MICROBIOTA IN ALCOHOLIC AND METABOLIC-DYSFUNCION ASSOCIATED STEATOTIC LIVER DISEASE IN ANIMAL MODELS.

Alcoholic liver disease (ALD) and metabolic-dysfunction associated steatotic liver disease (MASLD) are common, and gut microbiota (GM) is involved with both. Here we compared GM composition in animal models of MASLD and ALD to assess whether there are specific patterns for each disease. MASLD model- adult male Sprague Dawley rats, randomized into two groups: MASLD-control (n=10) fed a standard diet; MASLD-group (n=10) fed a high-fat-choline-deficient diet for 16 weeks. ALD model- adult male Wistar rats randomized: ALD-control (n=8) fed a standard diet and water+0.05% saccharin, ALD groups fed with sunflower seed and 10% ethanol+0.05% saccharin for 4 or 8 weeks (ALC4, n=8; ALC8, n=8). ALC4/8 on the last day received alcoholic binge (5g/kg of ethanol). Afterwards, animals were euthanized, and feces were collected for GM analysis. Both experimental models induced typical histopathological features of the diseases. Alpha diversity was lower in MASLD compared with ALD (p<0.001), and structural pattern was different between them (P<0.001). Bacteroidetes (55.7%), Firmicutes (40.6%), and Proteobacteria (1.4%) were the most prevalent phyla in all samples, although differentially abundant among groups. ALC8 had a greater abundance of the phyla Cyanobacteria (5.3%) and Verrucomicrobiota (3.2%) in relation to the others. Differential abundance analysis identified Lactobacillaceae_unclassified, Lachnospiraceae_NK4A136_group, and Turicibacter associated with ALC4 and the Clostridia_UCG_014_ge and Gastranaerophilales_ge genera to ALC8. In this study, we demonstrated that the structural pattern of the GM differs significantly between MASLD and ALD models. Studies are needed to characterize the microbiota and metabolome in both clinical conditions to find new therapeutic strategies. •Changes in the composition of the intestinal microbiota are related to the development of alcoholic liver disease and metabolic-dysfunction associated steatotic liver disease. •The diversity of the intestinal microbiota was lower in animals with MASLD compared to ALD. •The structural pattern of the intestinal microbiota was significantly different among the experimental groups. •Studies are needed to characterize the composition of the intestinal microbiota and metabolome to find new therapeutic strategies.

Rosai Dorfman Disease: A Rare Case Report.

To study the presentation and plan of treatment of patient with Rosai Dorfman Disease. Rosai-Dorfman disease(RDD), is rare, non-neoplastic, multisystemic histiocytic disorder. Nodal form is more common. It's self-limiting disorder of unknown etiology. Symptomatic treatment is mainstay. Bardet-Biedl syndrome (BBS) is rare ciliopathic, autosomal-recessive disorder, affecting multiple organs. Characterized by marked central obesity, retinal dystrophy, polydactyly, mental retardation, hypogonadism and renal dysfunction. Treatment is symptomatic with hormone supplementation & regular follow-ups. 10year male presented with swelling over left side of neck and intermittent fever since 2years, diminished vision in night since 5years. History of similar complaints on right side 5years back. Fine needle aspiration cytology(FNAC)-features consistent with Rosai Dorfman Disease. Examination showed short stature, squint eyes, polydactyly. Multiple palpable neck nodes of variable sizes. Ophthalmic evaluation showed Retinitis Pigmentosa. Paediatric consultation for syndromic evaluation, features were consistent with Bardet Biedel syndrome. Since the presentation is same as that of opposite side in past, because of recurrence of symptoms even with regular antibiotic and steroid therapy, and no local recurrence of disease on right side, surgical excision is planned for the patient. Rosai-Dorfman disease and Bardet-Biedl syndrome are rare disorders presenting many diagnostic and therapeutic challenges. High degree of clinical suspicion (RDD & BBS) with typical histopathological features (RDD) are diagnostic. Symptomatic treatment is useful and surgical excision can be done for recurrent/ complicated cases of RDD while symptomatic treatment with regular follow-up for BBS.

Diagnostic criteria for Buerger's disease: International Consensus of VAS - European Independent Foundation in Angiology/Vascular Medicine.

Buerger's disease (BD) remains a debilitating condition and early diagnosis is paramount for its effective management. Despite many published diagnostic criteria for BD, selective criteria have been utilized in different vascular centers to manage patients with BD worldwide. A recent international Delphi Consensus Study on the diagnostic criteria of BD showed that none of these published diagnostic criteria have been universally accepted as a gold standard. Apart from the presence of smoking, these published diagnostic criteria have distinct differences between them, rendering the direct comparison of patient outcomes difficult. Hence, the expert committees from the Working Group of the VAS-European Independent Foundation in Angiology/Vascular Medicine critically reviewed the findings from the Delphi study and provided practical recommendations on the diagnostic criteria for BD, facilitating its universal use. We recommend that the 'definitive' diagnosis of BD must require the presence of three features (history of smoking, typical angiographic features and typical histopathological features) and the use of a combination of major and minor criteria for the 'suspected' diagnosis of BD. The major criterion is the history of active tobacco smoking. The five minor criteria are disease onset at age less than 45 years, ischemic involvement of the lower limbs, ischemic involvement of one or both of the upper limbs, thrombophlebitis migrans and red-blue shade of purple discoloration on edematous toes or fingers. We recommend that a 'suspected' diagnosis of BD is confirmed in the presence of a major criterion plus four or more minor criteria. In the absence of the major criterion or in cases of fewer than four minor criteria, imaging and laboratory data could facilitate the diagnosis. Validation studies on the use of these major and minor criteria are underway.

Fast, bedside diagnosis of toxic epidermal necrolysis using ex vivo confocal laser scanning microscopy: A retrospective study.

Toxic epidermal necrolysis (TEN) is a severe life-threatening drug eruption with rapid evolution. A fast histologic differentiation between TEN and clinically similarly looking staphylococcal scalded skin syndrome is of vital importance for relevant treatment decision. The recently developed ex vivo confocal laser scanning microscopy (CLSM) offers innovative and extremely fast histological visualization of fresh tissue specimens. To assess the diagnostic efficacy of ex vivo CLSM in comparison with standard histopathology for TEN. We performed side-by-side comparison of TEN specimens analysed with ex vivo CLSM and haematoxylin and eosin staining. Analysis focused on typical histopathological features of TEN, including epidermal cleavage in the basal layer and confluent epidermal necrosis. We retrospectively assessed the diagnostic performance of ex vivo CLSM for TEN in clinically confirmed cases. We report substantial agreement between ex vivo CLSM and classical histology for the detection of subepidermal cleavage and confluent epidermal necrosis. When considering full-thickness epidermal loss, epidermal cleavage in the basal layer showed the highest diagnostic performance, reaching 87.5% sensitivity and 100% specificity. Based on our data, ex vivo CSLM appears as a rapid, resource-optimizing, and reliable approach for morphological TEN emergency screening on fresh skin samples.

Linear syringocystadenoma papilliferum of neck: a rare variant adnexal tumour

Syringocystadenoma papilliferum is a rare benign adnexal tumour presenting as exuberant proliferating lesion showing differentiation in apocrine pattern. The three clinical variants are linear, plaque and solitary of which linear variant is the rarest. We report a case of linear variant of syringocystadenoma papilliferum, which had typical histopathological features. As there is risk of malignancy in long standing cases, early diagnosis and treatment is of utmost importance.

Completing the view – histologic insights from circular AAA specimen including 3D imaging

Abdominal aortic aneurysm (AAA) is a pathologic enlargement of the infrarenal aorta with an associated risk of rupture. However, the responsible mechanisms are only partially understood. Based on murine and human samples, a heterogeneous distribution of characteristic pathologic features across the aneurysm circumference is expected. Yet, complete histologic workup of the aneurysm sac is scarcely reported. Here, samples from five AAAs covering the complete circumference partially as aortic rings are investigated by histologic means (HE, EvG, immunohistochemistry) and a new method embedding the complete ring. Additionally, two different methods of serial histologic section alignment are applied to create a 3D view. The typical histopathologic features of AAA, elastic fiber degradation, matrix remodeling with collagen deposition, calcification, inflammatory cell infiltration and thrombus coverage were distributed without recognizable pattern across the aneurysm sac in all five patients. Analysis of digitally scanned entire aortic rings facilitates the visualization of these observations. Immunohistochemistry is feasible in such specimen, however, tricky due to tissue disintegration. 3D image stacks were created using open-source and non-generic software correcting for non-rigid warping between consecutive sections. Secondly, 3D image viewers allowed visualization of in-depth changes of the investigated pathologic hallmarks. In conclusion, this exploratory descriptive study demonstrates a heterogeneous histomorphology around the AAA circumference. Warranting an increased sample size, these results might need to be considered in future mechanistic research, especially in reference to intraluminal thrombus coverage. 3D histology of such circular specimen could be a valuable visualization tool for further analysis.

Evaluation of Lymphovascular Invasion by CD31 Expression in Gastric Adenocarcinoma.

Lymphovascular tumoral invasion is a typical histopathological feature of gastric carcinomas and supports the recognition of high-risk patients for the recurrence. We aimed to study CD31 expression in diverse subtypes of gastric carcinomas and to show its association with the histopathologic findings of the carcinoma to assess the prognosis. This cross-sectional study was conducted on 40 established patients with gastric adenocarcinoma from radical gastrectomy. The patients were classified according to the pathology assessments. Tumoral tissues were assessed by immunohistochemical staining for CD31 expression. Malignant behavior was estimated by histopathological evaluations. CD31 positivity was described in 23 (57.5%) of all evaluated patients. In assessment of CD31 expression and tumor features presented, no significant association between the CD31 expression and patients' age, sex, tumor site, size, grade and stage, subtypes of carcinoma, perineural invasion, and also lymphovascular invasion was found. (P>0.05). Lymphovascular invasion may make valuable additional evidence and may be useful to detect gastric carcinoma patients at high risk for recurrence, who could be candidates for more supplementary therapies. However, in our study, CD31 expression did not show any association with the aggressive histopathologic features of this tumor.

Collecting Duct Carcinoma of the Kidney

Purpose: Collecting duct carcinoma of the kidney (CDC) is an aggressive subtype of renal cell carcinoma with a dismal prognosis. The available knowledge concerning optimal management is still unclear. We report the largest single-institutional experience in the management of CDC. Materials and Methods: All the cases of CDC which were diagnosed at our center following a radical or partial nephrectomy in the past 15 years were included. Treatment details, radiological features, and histological features were reviewed. Descriptive statistics were used to show the clinicopathological profile and management of these patients, and Kaplan–Meier overall survival (OS) estimate was calculated. Results: Eighteen patients who were diagnosed and underwent the primary intervention at our center were included. The median age of patients was 40 years, with a male-to-female ratio of 5:1. Typical histopathological features included high-grade nuclear features, stromal desmoplasia, and tubular architecture. Surgery was the primary modality of treatment. Five (28%) patients who had metastases received adjuvant therapy, three received interferon-alpha therapy, and two received adjuvant chemotherapy with gemcitabine and cisplatin. The median follow-up was 19 months (range: 3–46 months). Kaplan–Meier OS estimate was 68% at 1 year and 48% at 3 years. Conclusion: CDC of the kidney often presents at an advanced stage, and has a poor prognosis. Survival remains poor despite surgery and adjuvant therapy.

Giant cell Fibroma of Tongue: Entity with unique histopathological features

Giant Cell Fibroma can present as a nodular growth in the oral cavity, representing approximately 2% to 5% of all oral fibrous proliferations. They can be best distinguished from other fibrous lesions by their typical histopathological features of presence of stellate shape fibroblasts. Here, we report a case of a 65-year-old female with a mass on the tongue and discuss its clinical and histopathological differential diagnosis.

Significance Of Tumor Giant Cells In Oral Squamous Cell Carcinoma: A Case Report

Background : Oral squamous cell carcinoma (OSCC) is the most common cancer of the oral cavity. OSCC presents with some typical histopathological characteristics like dysplastic epithelial cells with variable degrees of squamous differentiation. These histopathological features make it relatively easy to diagnose conventional squamous cell carcinoma. However, some cases show certain unique features that help in determining the prognosis of the disease. These features include epithelial-mesenchymal interaction, stromal hyalinization, clear cell change, stromal desmoplasia, perineural invasion, vascular invasion, tissue eosinophilia, giant cells and tertiary lymphoid follicle formation. The presence of giant cells in OSCC can be the result of foreign body reaction to keratin or due to formation of malignant tumor giant cells in poorly differentiated carcinomas. The giant cells showing pleomorphic features can be helpful in predicting the prognosis of the disease.

P11.44.A The impact of heme biosynthesis regulation on glioma aggressiveness: correlations with most recent diagnostic molecular markers

Abstract Background The prognosis of patients with diffusely infiltrating gliomas is dismal but varies greatly between individuals. While characterization of gliomas was primarily relied on typical histopathological features, specific molecular markers increasingly gained importance and play a key role in the recently published 5th edition of the World Health Organization (WHO) classification. Heme biosynthesis represents a crucial pathway due to its key role in oxygen transport, energy production or drug metabolism. Recently, we described a “heme biosynthesis mRNA expression signature” that correlates with histopathological glioma grades and patient survival. The aim of the current study was to correlate the heme biosynthesis mRNA expression signature with the most recent diagnostic molecular markers for glioma stratification. Material and Methods In this study, patient data were derived from the “The Cancer Genome Atlas” (TCGA) lower-grade glioma and glioblastoma cohorts. We identified diffusely infiltrating gliomas correlating molecular tumor diagnosis according to the most recent WHO classification with heme biosynthesis mRNA expression. The following molecular markers were analyzed: EGFR amplification, TERT promoter mutation, CDKN2A/B homozygous loss, concurrent chromosome 7 gain/10 loss, MGMT methylation, IDH mutation, ATRX loss, p53 mutation and 1p19q co-deletion. Subsequently, we calculated the heme biosynthesis mRNA expression signature and correlated this signature with distinct molecular glioma markers as well as the resulting molecular subgroups. Results A total of 649 patients with available data on up-to-date molecular markers and heme biosynthesis mRNA expression were included. According to analysis of individual molecular markers, we found a significantly higher heme biosynthesis mRNA expression signature in gliomas with IDH wildtype (p&amp;lt;0.0005), without 1p19q co-deletion (p&amp;lt;0.0005), with homozygous CDKN2A/B loss (p&amp;lt;0.0005) and with EGFR amplification (p=0.001). Furthermore, we observed that the heme biosynthesis mRNA expression signature increased with the aggressiveness of the molecular subgroups (p&amp;lt;0.0005), being lowest in WHO grade 2 oligodendrogliomas and highest in WHO grade 4 glioblastomas. Conclusion Our data demonstrate a significant correlation between diagnostic molecular markers and heme biosynthesis regulation in diffusely infiltrating gliomas. Consequently, heme biosynthesis expression is a promising biomarker for glioma aggressiveness and might constitute a potential target for novel therapeutic approaches.

Peripheral Ossifying Fibroma: A Case Report

The gingiva is often considered as one of the most common sites of localized growths that are considered to be reactive rather than malignant in nature. Clinically, the majority of these lesions are difficult to identify, and therefore histopathology is useful in diagnosing these lesions as specific entities only on the basis of typical and consistent features. Focal gingival enlargements are quite frequent lesions in the oral cavity amounting to almost 3.1% of all oral tumors and 9.6% of gingival lesions. Peripheral ossifying fibroma (POF) is one of the reactive lesions that have typical histopathological features. Since the late 1940s, intraoral ossifying fibromas have been discussed in the literature. The term “Peripheral ossifying fibroma” is usually used to describe a fibroma of the gingiva which shows areas of calcification or ossification. They are usually classified as non-neoplastic enlargement of the gingiva. Many POFs are thought to develop initially as a pyogenic granuloma that undergoes fibrous maturation and subsequent calcification. However, many authors have suggested that rather than a transitional form of pyogenic granuloma or irritation fibroma, POF represents a separate clinical entity. Different lesions with clinical presentations similar to POF make it difficult to arrive at an appropriate diagnosis. Usually, the treatment of POF includes excision of the lesion surgically with minimal chances of recurrence. This paper describes a case report of a 32 year old female patient who reported growth on gingiva in the mandibular left anterior region of the mouth for three years.

Type-A Thymoma in a 56-Year-Old Male: A Case Report

Thymoma is the epithelial neoplasm of the thymus gland that is most commonly found in the anterior mediastinum in individuals aged in the fourth to the sixth decades. Its incidence is approximately 2.5 cases per 1 million people/year with a cure rate of 100%. This case is interesting enough to be discussed because it is a very rare incidence and the case has a typical histopathological feature. This case report presents a 56-year-old man with complaints of cough and lower right chest pain. CT – Scan examination showed a solid tumor with central necrotic in the inferior lobe of the right lung.

Femtosecond laser assisted superficial lamellar keratectomy as a successful treatment of corneal opacity in a patient with Thiel Behnke corneal dystrophy

Introduction and importanceThiel Behnke corneal dystrophy (TBCD) and Reis Buckler corneal dystrophy (RBCD) are Bowman's layer dystrophies with overlapping clinical features causing diagnostic confusion. However, each entity has typical histopathological features. We describe in this case the successful use of Femtosecond laser (FSL) in the treatment of TBCD-related corneal opacity.Case presentationWe present a 54-year-old male with bilateral superficial corneal opacities consistent with TBCD based on clinical appearance, anterior segment optical coherence tomography (AS-OCT), and In vivo confocal microscopy. Management options were discussed with the patient before proceeding with Femtosecond Laser Assisted Superficial Lamellar Keratectomy (FSLASLK). The histopathological findings of the excised left anterior lamellar corneal flap were typical of TBCD and the patient had a satisfactory outcome.Clinical discussionTBCD typically affects Bowman's layer centrally with progressive opacities involving the deeper layer of the corneal stroma and the periphery with advancing age. Histopathology typically shows subepithelial fibrosis with interrupted basement membrane and totally replaced Bowman's layer by uneven fibrous tissue forming the characteristic saw tooth pattern. The treatment of such cases is challenging with variable success and recurrence rates. Our case was managed successfully using FSL.ConclusionTBCD, even though a rare type of dystrophy, should be suspected based on the appearance of the corneal opacities clinically. It can be diagnosed by typical AS-OCT findings supported by histopathological confirmation and can be successfully treated by FSASLK.

Injection of prototypic celiac anti-transglutaminase 2 antibodies in mice does not cause enteropathy.

Celiac disease is an autoimmune enteropathy driven by dietary intake of gluten proteins. Typical histopathologic features are villous flattening, crypt hyperplasia and infiltration of inflammatory cells in the intestinal epithelium and lamina propria. The disease is hallmarked by the gluten-dependent production of autoantibodies targeting the enzyme transglutaminase 2 (TG2). While these antibodies are specific and sensitive diagnostic markers of the disease, a role in the development of the enteropathy has never been established. We addressed this question by injecting murine antibodies harboring the variable domains of a prototypic celiac anti-TG2 immunoglobulin into TG2-sufficient and TG2-deficient mice evaluating for celiac enteropathy. We found no histopathologic abnormalities nor clinical signs of disease related to the injection of anti-TG2 IgG or IgA. Our findings do not support a direct role for secreted anti-TG2 antibodies in the development of the celiac enteropathy.

Sinonasal Mucosal Melanoma: An Update and Review of the Literature.

Primary sinonasal mucosal melanoma (SNMM) is an aggressive tumor with high metastatic potential and poor outcomes. Presenting symptoms are nonspecific, and the nasal cavity is the most common site of origin followed by the maxillary and ethmoid sinuses. Histopathologically, SNMMs are pleomorphic and predominantly composed of epithelioid cell type. Identifying these tumors requires a high index of suspicion for melanoma and the use of a panel of immunohistochemical markers when typical histopathological features are missing. Not infrequently, these tumors are undifferentiated and/or amelanotic. Currently, SNMM falls into 2 different staging systems proposed by the American Joint Committee on Cancer, one for carcinoma of the nasal cavity and sinuses and the other for head and neck melanoma. Although therapeutic standards do not exist, surgical resection with adjuvant radiotherapy and/or systemic therapy may offer the best outcome. Lymphadenectomy including possible parotidectomy and neck dissection should be considered in patients with regional lymph node metastasis. However, the role of elective lymph node dissection is controversial. Genetic profiling has identified a number of recurrent gene mutations that may prove useful in providing targets for novel, emerging biological treatments. In this article, we provide an update on clinicopathological features, staging, molecular discoveries, and treatment options for SNMM.

Graves\u2019 disease overlapping with chronic hepatitis B and methimazole-induced liver injury and autoimmune hepatitis: a case report

BackgroundLiver injury related to Graves’ Disease (GD) includes hepatotoxicity of thyroid hormone excess, drug-induced liver injury, and changes resulting from concomitant liver disease. Methimazole (MMI) has been shown to induce several patterns of liver injury. However, the diagnosis and treatment of autoimmune hepatitis (AIH) overlapping with either GD or chronic hepatitis B are challenging.Case presentationA 35-year-old man from China presented with a two-year history of GD and a 10-day history of progressive jaundice. He had taken MMI for two months and discontinuing treatment due to liver toxicity 1 year ago and for another 6 days 20 days prior to hospitalization. The patient was diagnosed with GD overlapping with chronic hepatitis B and MMI-induced liver injury with early stage of acute-on-chronic liver failure on admission. However, the elevated aminotransferase and bilirubin levels could not be controlled after correction of liver failure and effective control of HBV replication and hyperthyroidism by daily oral entecavir and one-time oral administration of 131-iodine. The patient underwent liver biopsy on the 43rd day of hospitalization, showing HBsAg expression on the membrane of hepatocytes and typical histopathological characteristics of AIH. He was finally diagnosed with GD overlapping with chronic hepatitis B and MMI-induced liver injury and AIH. The elevated aminotransferase and bilirubin completely returned to normal by 3-month glucocorticoid therapy and continuous entecavir treatment and there was no recurrence during a 6-month follow-up, suggesting that AIH in this patient is different from classical AIH or GD-associated AIH.ConclusionsGD together with AIH is a complex and difficult subject. It needs to be clarified whether MMI or HBV can act as a trigger for AIH in this patient.

Benign Recurrent Intrahepatic cholestasis- A case series

Background and Aim: Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive inherited disorder characterised by repeated self-limiting episode complex of cholestatic jaundice with intense pruritus, weight loss, lasting several weeks to months with normal asymptomatic phases in between episodes. Due to rarity of disease, no guideline is available for treatment of BRIC. We compared various aspects of three adult patients who were diagnosed as BRIC, including demographics, clinical signs and symptoms, investigations, treatment and outcome. Methods:Three cases of young adults presented to a tertiary care hospital in last one year with progressive cholestatic jaundice with intense pruritus. In all three cases the diagnosis of BRIC was made by a combination of clinical features, laboratory findings and typical histopathological features on liver biopsy and exclusion of other causes of cholestasis. Results: The case series includes three male patients, mean age 23.3 years with conjugated hyperbilirubinemia, high ALP levels, with normal GGTP. Two of the three patients had at least two such attacks with normal asymptomatic period between episodes. One patient had single such episode but all other causes of cholestatic hepatitis were ruled out and diagnosis was made based on liver biopsy in all patients. Genetic study was done in one patient with pending report. All of them were treated with rifampicin, UDCA, tapering steroids, anti-histamines and cholestyramine. Episodes resolved within 2-3 months. Conclusions: BRIC presents around first two decades of life with male preponderance. The diagnosis can be made by exclusion of other causes, histopathology and confirmation by generic testing. Resolution is usually slow, and treatment remains largely symptomatic. As a non-invasive treatment, rifampicin may be the first choice.

Spinal intradural endodermal cyst

Purpose: Epithelial cysts of the central nervous system, according to the type of cell origin; neuroepithelial or endodermal type. Endodermal cysts are rare forms of benign tumors. In this presentation, we present a 35-year-old male patient with typical histopathologic features and due to the presence of this rare lesion. We aimed to present this rare case and emphasize the importance of cystic epithelial cell differences. Methods: Neurenteric cysts, cyst lining epithelium of cell types were analyzed using immunohistochemical diagnostic methods. Results: The importance of the cell type in the endodermal cyst is also emphasized and the frequency of recurrence in cysts that have goblet-columnar epithelial component. This epithelium is increasing the risk of recurrences. However, the other cause of recurrence is also incomplete excision. Therefore, the complete excision of ECs is important, not only for avoiding recurrence but also for differentiating cell types for ECs and predicting recurrence risk. Conclusions: The precise pathologic diagnosis of cell type determines the risk of recurrence. Neurosurgeons should exert their best efforts to achieve complete excision of the cysts.