Esofagitis disecante supeEsofagitis disecante superficial: dos casos de una misma entidad poco frecuente / Esophagitis dissecans superficialis: two cases of the same rare entity

La esofagitis disecante superficial (EDS) es una entidad infrecuente que se caracteriza endoscópicamente por el desprendimiento de las capas superficiales del epitelio esofágico e, histológicamente, por el aspecto bitonal del epitelio escamoso esofágico secundario a la necrosis de los estratos más superficiales. La etiología es desconocida, aunque se ha asociado con la ingesta de determinados fármacos, enfermedades autoinmunes, estasis esofágica y procedimientos endoscópicos. Presentamos dos casos: uno de ellos acontece en una mujer tras un episodio de disfagia abrupta y el segundo en un varón con comorbilidades y clínica de dolor epigástrico. La EDS es una patología que hay que considerar en su adecuado contexto clínico y endoscópico, ya que su curso es autolimitado en comparación con otras entidades de evolución tórpida o que precisan un tratamiento específico. (AU)

Esophagitis dissecans superficialis (EDS) is a rare disease characterized by sloughing of the superficial esophageal mucosa and, histologically, by the bitonal appearance of the squamous epithelium secondary to necrosis of the most superficial layers. Etiology is uncertain, however, it has been associated with some medications, autoimmune diseases, esophageal stasis and endoscopic procedures. Here, two cases are presented, one of them which appeared in a woman after an episode of dysphagia and another one which occurred to a man with comorbidities and epigastric pain. This entity should be considered due to its self-limiting clinical course, compared to other entities with a more torpid evolution or that require more specific treatment. (AU)

[Esophagitis dissecans superficialis: two cases of the same rare entity]. / Esofagitis disecante superficial: dos casos de una misma entidad poco frecuente.

Esophagitis dissecans superficialis (EDS) is a rare disease characterized by sloughing of the superficial esophageal mucosa and, histologically, by the bitonal appearance of the squamous epithelium secondary to necrosis of the most superficial layers. Etiology is uncertain, however, it has been associated with some medications, autoimmune diseases, esophageal stasis and endoscopic procedures. Here, two cases are presented, one of them which appeared in a woman after an episode of dysphagia and another one which occurred to a man with comorbidities and epigastric pain. This entity should be considered due to its self-limiting clinical course, compared to other entities with a more torpid evolution or that require more specific treatment.

Mixed crawling-type gastric adenocarcinoma.

Crawling-type gastric carcinoma is a rare variant of early gastric carcinoma. Endoscopically, it is a poorly defined tumor with a flat appearance and histologically, especially in a small biopsy, mimic intestinal metaplasia with reactive atypia. We present a case in which, due to the finding of foci of signet ring cell carcinoma, correct management of the patient was carried out.

Tratamiento quirúrgico de estenosis duodenal secundaria a enfermedad de Crohn / Duodenal stenosis surgical treatment in Crohn’s disease

We present the case of a 34-year-old man with daily vomiting and 20% weight loss in a year. A gastroduodenoscopy was performed, noticing 2nd and 3rd duodenal portion dilatation and inflammatory involvement of the 3rd and 4th portion, causing luminal stenosis. These findings are the same than in the magnetic resonance . The biopsy proves the histological diagnosis of Crohn's disease. At the beginning the patient was treated with Prednisone, Adalimumab and Ustekinumab. After 9 months, surgery was decided because the disease was refractory to treatment and there was corticosteroid dependence. A partial resection of 3rd and 4th portion of the duodenum and the first loop of jejunum was performed, with duodenojejunal anastomosis. The patient presents good postoperative evolution and after 1 year he remained asymptomatic under treatment with Ustekinumab.(AU)

An incidental traditional serrated adenoma of the gallbladder: A case report / Un adenoma serrado tradicional incidental de la vesícula biliar: informe de un caso

Serrated lesions outside the low digestive tract are scarce, with only two traditional serrated adenomas (TSA) reported in the gallbladder, with limited information about the serrated pathway outside the colon. Our case was an incidental finding in a patient undergoing surgery to treat a cholecystitis, when a polypoid lesion was observed. The epithelium formed gland structures with ectopic crypts, serrated slits and eosinophilic cytoplasm. MUC4 and MUC5A were positive, but mismatch repair proteins (MSI) retained nuclear staining. BRAF showed a not mutated profile and NRAS/KRAS was inconclusive due to the absence of remaining tissue. MSI and CpG island (CIMP), the most common genetic hallmarks of the serrated pathway, have been proven in gallbladder carcinomas, although serrated polyps are not recognized as premalignant precursors. Hereby we report one TSA of the gallbladder without the usual genetic drivers. A larger evidence is needed to improve the diagnosis and management.(AU)

Las lesiones serradas no suelen localizarse fuera del tracto digestivo bajo, con solo 2 adenomas serrados tradicionales (TSA) descritos. Por ello, la información sobre la vía serrada fuera del colon es limitada. Nuestro caso trata de un hallazgo incidental en un paciente al que se le realizó una colecistectomía y en el que se observó una lesión polipoide. Esta formaba estructuras glandulares con criptas ectópicas, serración y citoplasma eosinófilo. MUC4 y MUC5A eran positivos, pero las proteínas implicadas en la inestabilidad de microsatélites (MSI) conservaban tinción nuclear. BRAF no estaba mutado y NRAS/KRAS no fue concluyente. La MSI y la metilación de CpG (CIMP) son las vías oncogénicas más comunes de la vía serrada y se ha demostrado en carcinomas de vesícula biliar. Sin embargo, los pólipos serrados no se reconocen como precursores premalignos. Nuestro caso trata de un adenoma serrado tradicional de vesícula biliar sin rasgos genéticos habituales. Se necesita mayor casuística en la literatura.(AU)

An incidental traditional serrated adenoma of the gallbladder: A case report.

Serrated lesions outside the low digestive tract are scarce, with only two traditional serrated adenomas (TSA) reported in the gallbladder, with limited information about the serrated pathway outside the colon. Our case was an incidental finding in a patient undergoing surgery to treat a cholecystitis, when a polypoid lesion was observed. The epithelium formed gland structures with ectopic crypts, serrated slits and eosinophilic cytoplasm. MUC4 and MUC5A were positive, but mismatch repair proteins (MSI) retained nuclear staining. BRAF showed a not mutated profile and NRAS/KRAS was inconclusive due to the absence of remaining tissue. MSI and CpG island (CIMP), the most common genetic hallmarks of the serrated pathway, have been proven in gallbladder carcinomas, although serrated polyps are not recognized as premalignant precursors. Hereby we report one TSA of the gallbladder without the usual genetic drivers. A larger evidence is needed to improve the diagnosis and management.

Duodenal stenosis surgical treatment in Crohn's disease.

We present the case of a 34-year-old man with daily vomiting and 20% weight loss in a year. A gastroduodenoscopy was performed, noticing 2nd and 3rd duodenal portion dilatation and inflammatory involvement of the 3rd and 4th portion, causing luminal stenosis. These findings are the same than in the magnetic resonance . The biopsy proves the histological diagnosis of Crohn's disease. At the beginning the patient was treated with Prednisone, Adalimumab and Ustekinumab. After 9 months, surgery was decided because the disease was refractory to treatment and there was corticosteroid dependence. A partial resection of 3rd and 4th portion of the duodenum and the first loop of jejunum was performed, with duodenojejunal anastomosis. The patient presents good postoperative evolution and after 1 year he remained asymptomatic under treatment with Ustekinumab.

Intestinal obstruction secondary to Brunner's glands hyperplasia.

Brunner's gland hyperplasia constitutes 10.6% of benign tumors of the duodenum, with an incidence of 0.008%. It is usually an incidental finding during endoscopy or imaging tests as they are small and asymptomatic. In the case of symptomatic tumors, resection of the lesion is indicated. In lesions ≤2 cm, endoscopic resection can be chosen, reserving surgery for larger lesions or endoscopically inaccessible ones. We present the case of a patient with a history of vomiting and hyporexia of months of evolution who presented peptic ulcer perforation and underwent surgery. During follow-up, she presented intestinal obstruction due to pyloric stenosis. Given the impossibility of ruling out a neoplastic process with certainty in diagnostic tests, surgical resection (antrectomy) was decided with an anatomopathological finding of Brunner's gland hyperplasia.

Hepatitis autoinmune seronegativa, descripción de 2 casos en edad pediátrica / Seronegative autoimmune hepatitis: Description of two paediatric cases

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Porocarcinoma: ¿infrecuente o infradiagnosticado? Estudio retrospectivo de 6 casos y revisión bibliográfica / Porocarcinoma: Uncommon or underdiagnosed? A retrospective study of 6 cases and a review of the literature

El porocarcinoma es un tumor maligno infrecuente, habitualmente originado sobre un poroma de larga evolución. Normalmente se localiza en extremidades inferiores, cabeza y cuello, en la sexta y séptima décadas de la vida. Debido a la escasa cantidad de casos documentados, realizamos un estudio retrospectivo durante 12 años consecutivos entre los diagnósticos registrados en la base de datos de nuestro Servicio de Anatomía Patológica. Recogimos 6 casos de dicho tumor sobre un total de unas 33.000 biopsias de piel, y analizamos sus características clínicas e histopatológicas. Observamos una incidencia 4 veces superior a la descrita en otros estudios, predominio en mujeres, y una media de edad y tamaño tumoral superiores. Su presentación clínica variada, larga evolución asintomática, y sus características arquitecturales, citológicas e inmunohistoquímicas, sí son concordantes con la literatura previa (AU)

Porocarcinoma is a rare malignant sweat gland tumour, usually developing from a long standing poroma. It is frequently found on the lower extremities or head and neck, and most cases occur in elderly patients. Due to the limited infrequency of documented cases, we performed a retrospective study over 12 consecutive years from the database of our department of pathology. We collected 6 cases of this tumour from a total of 33,000 skin biopsies and reviewed the main clinical and histopathological findings. In comparison to previous studies, we found a 4-times-higher incidence of porocarcinoma, female predominance, a higher average age and a larger tumour size. However, their diverse clinical presentation, longstanding asymptomatic evolution, morphological features and immunohistochemical profile are consistent with previous literature (AU)

Tumor desmoplásico de células pequeñas y redondas en pared torácica / Desmoplastic small round cell tumor of the thoracic wall

El tumor desmoplásico de células pequeñas y redondas es un tumor agresivo poco frecuente que surge generalmente en el peritoneo abdominal o pélvico. Afecta sobre todo a varones jóvenes y se caracteriza por la presencia de células pequeñas e indiferenciadas con núcleos hipercromáticos. Su principal característica diagnóstica es la co-expresión de marcadores epiteliales, musculares y neuronales, así como la presencia de la translocación recíproca t (11; 22)(p13; q12), que afecta a los genes EWS y WT1. Presentamos un caso de localización y clínica atípicas, con un perfil inmunohistoquímico peculiar y cuyo diagnóstico pudo ser filiado de forma concluyente tras realizar el estudio molecular. Se trata de una niña de 7 años con un tumor localizado en la pared torácica. Tras la cirugía se inicia tratamiento con quimioterapia. La paciente continúa viva a los 7 meses de la intervención (AU)

Desmoplastic small round cell tumour is a rare, aggressive neoplasm usually found in young males in the abdominal or pelvic peritoneum. It is characterized by the presence of small undifferentiated cells with hyperchromatic nuclei. Its main diagnostic feature is the co-expression of epithelial, muscular and neural markers in addition to the reciprocal translocation t (11; 22)(p13; q12), which affects the WT1 and EWS gene. We present a case of a 7 year old girl with a tumour in the thoracic wall. The unusual location, together with an atypical IHC profile meant that diagnosis was only possible after molecular studies. The patient underwent postoperative chemotherapy and is alive and well 7 months after surgery (AU)