Primordial odontogenic tumour of the maxilla: case report and review of the literature.

Primordial odontogenic tumour (POT) is a relatively newly described entity with well-defined clinicopathological features. Since its initial description in 2014, 22 cases of POT have been reported in the literature. Only five cases of POT have arisen in the maxilla. This article describes an additional patient with a POT of the maxilla and provides a review of the literature on POT.

[Appendicular actinomycosis as a cause of ileocecal mass in children: Case report]. / Actinomicosis apendicular como causa de masa ileocecal en la edad pediátrica: Caso clínico.

INTRODUCTION: Appendicular actinomycosis is a rare disease produced by cecal appendix invasion by Actinomyces gender bacteria after a predisposing cause that disrupts mucosal barrier, such as acute appendicitis. The highest frequency appears in adults; however, there are cases in paediatric age described in literature. The aim of this article is to introduce a case of appendicular actinomycosis in a child with atypical presentation and to review the literature. CLINICAL CASE: 10-year-old boy transferred to our hospital due to intraoperative finding at a different centre of a right flank tumor, without identification of cecal appendix. After completing the study with image tests, the patient was re-intervened with the diagnosis of complicated acute appendicitis. Intraoperatively, an organized plastron lacking of acute inflammatory signs was found. Post-surgical evolution was positive with conventional antibiotic treatment. Histological study of the specimen revealed an appendicular actinomycosis, so antibiotic treatment was widened in an outpatient basis and predisposing diseases were ruled out. COMMENTS: Appendicular actinomycosis is an infrequent cause of appendicitis that can simulate an intra-abdominal tumor in children and adults. It is necessary to rule out predisposing diseases and to administer an adequate antibiotic treatment.

INTRODUCCION: La actinomicosis apendicular es una patología rara producida por la invasión del apéndice cecal por bacterias del género Actinomyces al alterarse la barrera mucosa después de una causa predisponente, como podría ser una apendicitis aguda. Se presenta con mayor frecuencia en adultos, aunque en la literatura se han descrito algunos casos en edad pediátrica. En este artículo se presenta un nuevo caso pediátrico de actinomicosis apendicular de presentación atípica y se revisa la literatura al respecto. CASO CLINICO: Paciente varón de 10 años de edad derivado a nuestro hospital por el hallazgo intraoperatorio en otro centro de una tumoración en flanco derecho, sin identificación del apéndice cecal. Tras completar el estudio con pruebas de imagen, el paciente fue reintervenido bajo la orientación diagnóstica de apendicitis aguda complicada. Intraoperatoriamente, se identifica un plastrón organizado y con escasos signos inflamatorios agudos. La evolución postquirúrgica fue favorable con tratamiento antibiótico convencional. El estudio anatomopatológico de la pieza reveló una actinomicosis apendicular, por lo que se amplió el tratamiento antibiótico de forma ambulatoria y se descartó patología predisponente. COMENTARIOS: La actinomicosis apendicular es una causa infrecuente de apendicitis que puede simular una tumoración intraabdominal en niños y adultos. Es necesario descartar patología predisponente y realizar un tratamiento antibiótico adecuado.

Alveolar capillary dysplasia with misalignment of pulmonary [corrected] veins: concordance between pathological and molecular diagnosis.

We report the case of a newborn with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV), a rare condition of unknown etiology presenting in the neonatal period with significant persistent pulmonary hypertension. The diagnosis was made by lung biopsy and confirmed at autopsy. Specific genetic analysis demonstrated defects in the FOXF1 gene. The diagnosis of ACD/MPV requires a high level of suspicion and is made by lung biopsy or necropsy examination by a pediatric pathologist with experience in this condition. The availability of genetic testing has led to increasing diagnosis of patients with this lethal disorder and can influence their management, specifically by indicating the need for lung biopsy in a critically ill newborn.

Novel histologic scoring system for long-term allograft fibrosis after liver transplantation in children.

The existing systems for scoring fibrosis were not developed to evaluate transplanted livers. Our aim was to design and validate a novel fibrosis scoring system specifically adapted to assess liver allograft fibrosis (LAF). Clinical data, histology, transient elastography (TE) and AST/platelet ratio index (APRI) were reviewed in 38 pediatric liver transplant (LT) recipients. Protocol liver biopsies performed at 6 months and 7 years post-LT were reviewed by three pathologists who assessed LAF using the METAVIR and Ishak systems. LAF was also scored separately in portal (0-3), sinusoidal (0-3) and centrolobular areas (0-3). Scoring evaluations were correlated with fibrosis quantification using morphometry, and also with TE and APRI. Statistical correlations between morphometry and METAVIR were 0.571 (p < 0.000) and 0.566 (p < 0.000) for the Ishak system. The novel score (0-9) for separate assessment of portal, sinusoidal and centrolobular fibrosis showed a better correlation with morphometry (0.731; p < 0.000) and high intra-/interobserver agreement (0.966; p < 0.000 and 0.794; p < 0.000, respectively). No correlation was found between TE or APRI and morphometry or the three histologic scores. In conclusion, this novel semiquantitative fibrosis scoring system seems to more accurately reflect LAF than the existing scoring system and may become a practical tool for staging fibrosis in LT.

[Submucous urethral angioma]. / Angioma uretral submucoso.

OBJECTIVES: Urethral hemangioma is a rare benign vascular tumor. The clinical features of this tumor type and the therapeutic options are discussed. METHODS/RESULTS: We report on a 55-year-old male with urethral hemangioma who consulted for urethral hemorrhage. CONCLUSIONS: To our knowledge, approximately 40 cases of this rare tumor type have been reported in the literature. Urethroscopy is the best diagnostic procedure. Treatment depends on tumor site, size and number. Transurethral resection of the tumor, urethrectomy, arterial embolization, radiotherapy or ablation with Nd:YAG laser can be utilized.