Low-grade dysplasia in Barrett's esophagus: A problematic diagnosis / Displasia de bajo grado en el esófago de Barrett: un diagnóstico problemático

Although low-grade dysplasia (LGD) in Barrett's esophagus (BE) is a histopathological diagnosis based on different histological abnormalities, it is still problematic for different reasons. Patients without confirmed diagnosis of LGD undergo unnecessary and intensified follow-up where the risk of progression is low in the majority of cases. In contrast, the presence of confirmed LGD indicates a high risk of progression. In this article we try to address these reasons focusing on re-confirmation of LGD diagnosis, interobserver agreement, and persistent confirmed LGD. The progression risk of LGD to high-grade dysplasia and esophageal adenocarcinoma will also be reviewed. (AU)

Aunque la displasia de bajo grado (DBG) en el esófago de Barrett (EB) es un diagnóstico histopatológico basado en diferentes anomalías histológicas, este no deja de ser problemático por diferentes razones. Los pacientes sin diagnóstico confirmado de DBG se someten a un seguimiento innecesario e intensificado donde el riesgo de progresión es bajo en la mayoría de los casos. Por el contrario, la presencia de DBG confirmada indica un alto riesgo de progresión. En este artículo tratamos de abordar estas razones centrándonos en la reconfirmación del diagnóstico de la DBG, la concordancia entre observadores y la DBG confirmada y persistente. También se revisará el riesgo de progresión de la DBG a displasia de alto grado y adenocarcinoma esofágico. (AU)

Validation of a Pathological Score for the Assessment of Bronchial Biopsies in Severe Uncontrolled Asthma: Beyond Blood Eosinophils

Background: Blood eosinophil count (BEC) is currently used as a surrogate marker of T2 inflammation in severe asthma but its relationship with tissue T2-related changes is elusive. Bronchial biopsy could add reliable information but lacks standardization. Objectives: To validate a systematic assessment of the bronchial biopsy for the evaluation of severe uncontrolled asthma (SUA) by standardizing a pathological score. Methods: A systematic assessment of submucosal inflammation, tissue eosinophilic count/field (TEC), goblet cells hyperplasia, epithelial changes, basement membrane thickening, prominent airway smooth muscle and submucosal mucous glands was initially agreed and validated in representative bronchial biopsies of 12 patients with SUA by 8 independent pathologists. In a second phase, 62 patients with SUA who were divided according to BEC≥300cells/mm3 or less underwent bronchoscopy with bronchial biopsies and the correlations between the pathological findings and the clinical characteristics were investigated. Results: The score yielded good agreement among pathologists regarding submucosal eosinophilia, TEC, goblet cells hyperplasia and mucosal glands (ICC=0.85, 0.81, 0.85 and 0.87 respectively). There was a statistically significant correlation between BEC and TEC (r=0.393, p=0.005) that disappeared after correction by oral corticosteroids (OCS) use (r=0.170, p=0.307). However, there was statistically significant correlation between FeNO and TEC (r=0.481, p=0.006) that was maintained after correction to OCS use (r=0.419, p=0.021). 82.4% of low-BEC had submucosal eosinophilia, 50% of them moderate to severe. Conclusion: A standardized assessment of endobronchial biopsy is feasible and could be useful for a better phenotyping of SUA especially in those receiving OCS. (AU)

Gingival and alveolar mucosal reactive hyperplastic lesions: a retrospective clinical and histological study of 996 cases

Background: gingival/alveolar mucosal reactive hyperplastic lesions (GRHL), including fibrous hyperplasia (FH), pyogenic granuloma (PG), peripheral ossifying fibroma (POF) and peripheral giant cell lesion (PGCL), are a common group of oral diseases. The aim of the present study was to access the frequency and distribution of the clinical and histological features of these disorders in a Brazilian population. Material and methods: all specimens diagnosed as GRHL in three Oral Pathology laboratories were selected for the study. Clinical information was retrieved from the laboratory biopsy forms and hematoxylin and eosin stained histological slides were reviewed for analysis of the histological characteristics. Results: final sample was composed of 996 specimens, including 463 FH (47%), 280 PG (28%), 183 POF (18%) and 70 PGCL (7%). Females were more affected by FH, PG, and POF, and most cases affected adults with mean ages ranging from 40 to 53 years. FH, PG, and POF were more common in the upper gingiva/alveolar mucosa. Most PG, POF and PGCL were pedunculated, in contrast with FH (p<0.001). PG, FH and POF were mostly red or normal mucosal in color, while PGCL were mostly red/purple (p<0.001). PGCL were larger, followed by POF, FH and PG (p<0.001). Some histological features were characteristically found in some conditions, but they were also encountered in other lesions with variable frequencies. Conclusions: Oral medicine specialists, oral pathologists and periodontists are usually the professionals in contact with patients presenting GRHL and it is of upmost relevance that they should be familiarized with their clinical and histological profile. (AU)

Actinic cheilitis: Proposal of a clinical index

Background: Actinic cheilitis is a potentially malignant lesion most commonly found in the lower lip of individuals with chronic exposure to ultraviolet radiation. The aim of this study was to develop and to test a clinical indexthat can be used to assess the severity of actinic cheilitis.Material and Methods: The clinical index of actinic cheilitis was applied to 36 patients. An incisional biopsy wasobtained to grade oral epithelial dysplasias following the World Health Organization (WHO) and binary systems,and to evaluate their association with clinical characteristics by Fisher’s exact test (P<0.05). The accuracy of theindex was evaluated based on sensitivity, specificity, positive and negative predictive values, and receiver operating curve.Results: The blurring between the border of the lip and the skin was significantly associated with cases withoutdysplasia/mild epithelial dysplasia (P=0.041) and with low risk of malignancy (P=0.005). Ulcers and crusts weresignificantly associated with moderate/severe epithelial dysplasia (P=0.002 and P=0.012, respectively) and highrisk of malignancy (P=0.005 and P=0.045, respectively). Erosion showed a significant association only with highrisk cases of malignancy (P=0.024). The cut-off values of the diagnostic test showing the best performance were10 for the WHO grading system and 11 for the binary system.Conclusions: The index cut-offs with the highest accuracy were considered indicators for a biopsy. Erosion, ulceration and crusts were associated with more severe oral epithelial dysplasias. (AU)

Hiperplasia endotelial papilar intravascular (tumor de Masson) de la conjuntiva. Ubicación inusual de un tumor vascular endotelial raro / Intravascular papillary endothelial hyperplasia (Masson's tumor) of the conjunctiva. Unusual location of a rare vascular tumor

La hiperplasia endotelial papilar intravascular (IPEH) se caracteriza por la proliferación de células endoteliales generalmente producida dentro de una malformación vascular previa o un tumor. La IPEH suele afectar cuello, cabeza y extremidades inferiores, se han publicado pocos casos en la órbita ocular y párpados. Presentamos un caso único, un hombre de 48 años con una lesión conjuntival púrpura, elevada y multilobular de tres semanas de duración que se sometió a una biopsia excisional. Las características patológicas revelaron una hiperplasia endotelial papilar intravascular (tumor de Masson) (AU)

Intravascular papillary endothelial hyperplasia (IPEH) is characterized by proliferation of endothelial cells usually occurring within a long-standing vascular malformation or tumor. IPEH usually affects neck, head and lower extremities, and few orbital and eyelid cases have been reported. We present a unique case, a 48-year-old man with a purple, elevated, multilobular conjunctival lesion of three-weeks duration that underwent an excisional biopsy. Pathological features revealed intravascular papillary endothelial hyperplasia (Masson's tumor) (AU)

Esófago de Barrett, hacia la mejora de la práctica clínica / Barrett's esophagus, towards improved clinical practice

La principal relevancia clínica del esófago de Barrett (EB), resultado de la exposición crónica al reflujo gastroesofágico, es su potencial progresión a adenocarcinoma esofágico (ACE). Aunque no se recomienda el cribado de EB en la población general, tras su diagnóstico es beneficiosa una estrategia de seguimiento para la detección precoz de displasia o neoplasia. El patrón oro para el diagnóstico y seguimiento es la endoscopia oral de alta definición con toma de biopsias aleatorias. Además, toda lesión visible debe resecarse de forma completa preferentemente mediante resección endoscópica mucosa, que se considerará curativa en presencia de displasia de bajo grado (DBG), displasia de alto grado (DAG) o ACE confinado a la mucosa (T1a), tras lo cual se debe erradicar el EB residual mediante ablación endoscópica. En ausencia de lesión visible, la ablación por radiofrecuencia es el tratamiento de elección para erradicar el EB con DBG, DAG o ACE intramucoso. (AU)

The main clinical relevance of Barrett's esophagus (BE), a result of chronic exposure to gastroesophageal reflux, is its potential progression to esophageal adenocarcinoma (EAC). Although screening for BE is not recommended in the general population, after diagnosis of BE, a surveillance strategy for early detection of dysplasia or neoplasia is needed. The gold standard for diagnosis and surveillance is high-definition oral endoscopy with random biopsies. In addition, any visible lesion should be completely resected, which will be considered curative in the presence of low grade dysplasia (LGD), high-grade dysplasia (HGD) or EAC confined to the mucosa (T1a), followed by eradication of residual BE by endoscopic ablation. In the absence of a visible lesion, radiofrequency ablation should be performed to eradicate BE with LGD, HGD or intramucosal EAC. (AU)

Rebound Thymic Hyperplasia Secondary to Surgical Therapy of Parotid Tumor

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Hiperplasia endotelial papilar intravascular (tumor Masson) de localización ginecológica / Intravascular papillary endothelial hyperplasia (Masson's tumour) of gynaecological origin

La hiperplasia papilar endotelial intravascular o tumor de Masson es una lesión vascular no neoplásica de carácter reactivo. Se caracteriza por ser un tumor poco frecuente, clínicamente inespecífico y con localizaciones descritas muy diversas. Resulta fundamental tenerlo en consideración dentro del diagnóstico diferencial de los tumores vasculares malignos como el angiosarcoma, cuyo pronóstico es muy diferente. Para lograr su diagnóstico definitivo el estudio anatomopatológico resulta primordial. El tratamiento de elección es la exéresis quirúrgica asegurando márgenes libres de enfermedad. El presente caso reportado supone un hecho excepcional, al ser un tumor de Masson de localización pélvica, cuyo diagnóstico fue un hallazgo casual propiciado por el estudio de estadificación quirúrgica de un adenocarcinoma de ovario.

Intravascular papillary endothelial hyperplasia or Masson's tumour is a non-neoplastic vascular lesion of reactive character. It is a rare diagnosis, clinically non-specific and with diverse locations. It is essential to take it into consideration and make a differential diagnosis with malignant vascular tumours such as angiosarcoma. Pathological study is fundamental for diagnosis. Treatment consists of complete resection of the tumour, including sufficiently wide margins to avoid recurrence.The case reported is an exceptional event, because of the pelvic location of the Masson's tumour that was diagnosed as part of the surgical staging of an ovarian cancer.

Hiperplasia endotelial papilar intravascular, el inusual diagnóstico de una masa orbitaria / Intravascular papillary endotelial hiperplasia, the unusual diagnosis of an orbital mass

La hiperplasia endotelial papilar intravascular es una lesión vascular benigna, infrecuente en la región periocular, pero que debe ser tenida en cuenta en el diagnóstico diferencial de una masa periorbitaria.Histopatológicamente se caracteriza por la proliferación intravascular de proyecciones papilares de tejido conectivo recubiertas por células endoteliales. La diferenciación anatomopatológica con el angiosarcoma puede ser difícil, pero es muy importante para evitar tratamientos agresivos. Generalmente, la escisión quirúrgica completa es curativa, mientras que una escisión incompleta puede causar recurrencias.Describimos un caso clínico de hiperplasia endotelial papilar intravascular en una mujer de 72 años que presentaba una masa palpebral inferior izquierda de 15 años de evolución que le provocaba hiperglobo del ojo izquierdo. El estudio histopatológico mostró la presencia de calcificación intralesional, característica infrecuente en esta patología.La masa fue extirpada completamente y tras 5 meses de seguimiento no mostró signos de recurrencia.

Intravascular papillary endotelial hyperplasia is a bening vascular lesion that rarely involves the periocular region, nevertheless, it should be considered in the differential diagnosis of a periorbital mass.It is histopatologically characterized by intravascular fibrous papillary fronds covered by endothelial cells. Histopathological differentiation from angiosarcoma can be challenging but is very important to avoid aggressive treatments. Complete surgical excision is frequently curative. Uncompleted excision can lead to recurrences.We describe the clinical and histological findings of intravascular papillary endotelial hyperplasia in the left lower lid of a 72-year-old woman who had a painless mass since 15 years ago, that caused hypertropia of her left eye. Histopathological examination revealed intralesional calcification, which is not an usual feature in this entity.The patient underwent complete surgical resection of the mass and there was no evidence of recurrence after 5 months of follow-up after surgery.

Hiperplasia muscular aberrante de la mano y síndromes de sobrecrecimiento relacionados con el PIK3CA. Serie de casos / Aberrant muscular hyperplasia of the hand and PIK3CA related overgrowth spectrum disorders. A case series study

Introducción: La hiperplasia muscular congénita de la mano esuna entidad rara, caracterizada por la presencia de músculos aberranteso accesorios en la eminencia hipotenar, que siempre se ha descrito enla literatura médica como una anomalía esporádica. El objetivo de esteartículo es informar de una serie de casos con fenotipo de hiperplasiamuscular aberrante asociada a mutaciones del PIK3CA. Material y métodos: Serie retrospectiva de casos atendidos ennuestro centro entre 2008 y 2020, con un fenotipo único en el contextode las mutaciones del PIK3CA. Resultados: Se incluyeron un total de 6 pacientes (4 masculinos y2 femeninos) con ensanchamiento de la eminencia hipotenar de la manoy arrugamiento peculiar de la piel suprayacente, asociado a desviaciónulnar de las articulaciones metacarpofalángicas. En todos los casos seobservaron grados variables de sobrecrecimiento congénito del miembroipsilateral. Se realizó biopsia cutánea por punch (5 mm de diámetro y 3-5mm de profundidad) en 4 pacientes con hiperplasia muscular y presenciade fibras musculares estriadas en dermis e hipodermis. Los estudiosgenéticos revelaron mutación del PIK3CA en 3 de los 4 pacientes, cuyotejido afectado fue enviado a analizar, con mosaicismo de entre el 3 y el8% (His1047Arg, Glu542Lys y Cys420Arg respectivamente). Conclusión: La hiperplasia muscular aberrante de la mano es unaentidad reconocida pero escasamente descrita en asociación con síndromes relacionados con el PIK3CA en la población pediátrica. A día dehoy sigue desconociéndose el papel del PIK3CA en el sobrecrecimientomuscular.(AU)

Introduction: Congenital muscular hyperplasia of the hand is ararely described entity, characterized by the presence of aberrant oraccessory muscles in the hypothenar eminence, which has been alwaysreported as sporadic anomaly in the medical literature. The aim of thispaper is to report a series of cases with a phenotype of aberrant musclehyperplasia associated with PIK3CA mutations. Material and Methods: We describe a retrospective series of casesfollowed at our institution between 2008 and 2020, with a unique phe-notypein the context of PIK3CA mutations. Results: A total of 6 patients were included (4 males and 2 females),who presented with an enlargement of the hypothenar eminence ofthe hand and peculiar wrinkling of the overlying skin, associated withulnar deviation of the metacarpo-phalangeal joints. In all cases, variabledegrees of congenital overgrowth of the ipsilateral limb were observed.Skin punch biopsy (5 mm diameter and 3-5 mm depth) was performedon 4 patients, where striated muscle hyperplasia and the presence ofstriated muscular fibers in the dermis and hypodermis were observedin all of them. Genetic studies revealed PIK3CA mutation in 3 of the 4patients whose affected tissue was analyzed, in a mosaic state rangingfrom 3 to 8% (His1047Arg, Glu542Lys, and Cys420Arg, 1 case each). Conclusion: Aberrant muscular hyperplasia of the hand is a well-recognized entity scarcely described in association with PIK3CA spec-trum disorder in the pediatric population. The role of PIK3CA in muscleovergrowth is not yet well understood.(AU)

Hiperplasia endotelial papilar intravascular en la región supraclavicular / Intravascular papillary endothelial hyperplasia in the supraclavicular region

La hiperplasia endotelial papilar intravascular (IPEH) es una lesión vascular benigna poco frecuente que se presenta habitualmente como una neoformación subcutánea eritemato-violácea inespecífica. El estudio histopatológico, necesario para el diagnóstico de confirmación, muestra proliferación papilar de células endoteliales asociada con material trombótico. La IPEH puede simular otras lesiones como el angiosarcoma, por lo que el diagnóstico correcto de esta entidad es esencial para evitar tratamientos agresivos. La resección con márgenes amplios suele ser suficiente

Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign vascular lesion that usually presents as a nonspecific erythematous-violaceous subcutaneous neoformation. The histopathological study, necessary for the confirmatory diagnosis, shows papillary proliferation of endothelial cells associated with thrombotic material. IPEH can simulate other lesions such as angiosarcoma, so the correct diagnosis of this entity is essential to avoid aggressive treatments. The resection with wide margins is usually enough