Alveolar capillary dysplasia with misalignment of the pulmonary veins: A surgical lung biopsy and autopsy in a full-term newborn.

Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACD/MPV) is a rare and lethal interstitial lung disorder, caused by a congenital abnormality affecting the development of the parenchyma and pulmonary vessels. We report the case of a newborn at the end of 40 weeks of pregnancy, who showed no cardiopulmonary anomalies in prenatal control ultrasounds. However, after delivery, pulmonary hypertension and hypoxemic respiratory failure became apparent. She died after 12 days from refractory hemodynamic and respiratory failure despite intensive therapy. A surgical lung biopsy and clinical autopsy were performed, both revealing the same histopathological signs consistent with this disorder. In our case, the findings of digestive and genital malformations, together with the genetic result of the alteration in the FOXF1 gene, led us to conclude the definitive diagnosis of alveolar capillary dysplasia.

Extended distal pancreatectomy with thoracic wall resection after neoadjuvant FOLFIRINOX: Is there a limit of resection for pancreatic cancer after downstaging?

Indications and outcomes of extended pancreatectomies have been recently appraised by the International Study Group for Pancreatic Surgery. However, no definitive conclusions have been drawn, particularly in the setting of neoadjuvant treatments. We present here a case of 53-year-old man diagnosed with a bulky adenocarcinoma of the tail of the pancreas and infiltrating the adjacent organs and the thoracic wall. The patient was sent to neoadjuvant chemotherapy and he underwent 12 cycles of FOLFIRINOX. Since a significant radiological response was observed after chemotherapy, the patient was scheduled for extended distal pancreatectomy with en bloc resection of the thoracic wall, in order to achieve a radical resection. The surgery is herein described with all technical details. The patient was discharged after an uneventful early post-operative course and subsequently readmitted for a late grade B post-operative pancreatic fistula, which was ultimately treated successfully. Pathology showed complete response. When performed in centers with ample experience in pancreatic surgery, extended pancreatic resections represent a viable curative option with acceptable surgical outcomes. In this setting, challenging tailored resections should be considered to achieve negative margins, particularly following maximized effective downstaging strategies.

[The value of alpha-methylacyl-CoA racemase expression in the progression of colonic carcinoma]. / Valor de la expresión inmunohistoquímica de alfa-metilacil-CoA racemasa en la progresión del carcinoma de colon.

Alpha-methylacyl-CoA racemase (AMACR) expression has been demonstrated in several normal tissues and in diverse types of carcinoma. Our aim was to analyze the immunohistochemical expression of AMACR in the sequence-progression of colonic cancer. We studied 237 cases, including samples of normal mucosa of the colon, adenomas with different degrees of dysplasia, colonic carcinomas, lymph nodes and liver metastases of colonic carcinomas. A scale of intensity and percentage of expression was used to analyze the AMACR immunohistochemical profile. The expression was nearly absent in samples of normal mucosa, increased in both adenomas and carcinomas, decreased in lymph node metastases but was significantly increased in liver metastases.

[SDHB expression in Warthin's tumour]. / Expresión de SDHB en el tumor de Warthin.

INTRODUCTION: Succinic dehydrogenase subunit B (SDHB) is an enzyme belonging to the mitochondrial complex II. The aim of this study is to analyse SDHB expression in a series of Warthin's tumours, studying its relationship with oncocytic changes, constantly present in this form of tumour. MATERIAL AND METHODS: In resection tumour specimens from a series of ten Warthin's tumours (all from the parotid gland), immunohistochemical expression of SDHB was analysed using a commercially-available monoclonal antibody. RESULTS: The Warthin's tumours studied affected 10 men (mean age: 64.2 yrs, range 40-80), all with smoking habits, and 2 with metachronous bilateral involvement. Two patients presented associated urothelial carcinoma. Our SDHB study showed marked reactivity (++/+++) in all cases in the oncocytic epithelial component and also in striated duct cytoplasm (+) from non-tumorous parotid tissue. Expression was not influenced by age, smoking intensity or bilateral character. One of the tumours showed squamous metaplasia foci with SDHB-negativity at this level. DISCUSSION AND CONCLUSIONS: Due to the constant and intense SDHB reactivity in oncocytic cells in our observations, oncocytic changes are not considered to be associated with defective enzyme activity in the mitochondrial complex II. Strong SDHB reactivity is an additional marker of oncocytic changes in Warthin's tumour. Neither of these facts has been described previously.

Linitis plástica como presentación de un carcinoma lobulillar de mama / Linitis plastica as a presentation of lobular breast carcinoma

El estómago es un lugar infrecuente de metastatización del cáncer de mama. Presentamos un caso de linitis plástica metastásica de un carcinoma lobulillar de mama localmente avanzado. La distinción entre el origen primario gástrico del secundario es fundamental, y se basa en estudios inmunohistoquímicos de las biopsias (AU)

The stomach is an infrequent site of breast cancer metastasis. We report a case of metastatic linitis plastica from locally advanced lobular breast carcinoma. The distinction between primary and secondary gastric origin is essential and is based on immunohistochemical studies of biopsies (AU)

Valor de la expresión inmunohistoquímica de alfa-metilacil-CoA racemasa en la progresión del carcinoma de colon / The value of alpha-methylacyl-CoA racemase expression in the progression of colonic carcinoma

La expresión inmunohistoquímica de la alfa-metilacil-CoA racemasa (AMACR) ha sido demostrada en varios tejidos normales y en diferentes tipos de carcinomas. Nuestro objetivo fue analizar la expresión inmunohistoquímica de la AMACR en la secuencia-progresión del carcinoma de colon. Se estudiaron 237 casos, que incluían muestras de mucosa colónica normal, adenomas con distintos grados de displasia, carcinomas de colon, y metástasis ganglionares y hepáticas de carcinoma colónico. Para el análisis de la expresión inmunohistoquímica de la AMACR se utilizó una escala que valoraba la intensidad y el porcentaje de su expresión en el tejido. La expresión fue casi nula en las muestras de mucosa normal, se incrementó en los adenomas y carcinomas, disminuyó de forma significativa en las metástasis ganglionares, pero volvió a aumentar su expresión en las metástasis hepáticas (AU)

Alpha-methylacyl-CoA racemase (AMACR) expression has been demonstrated in several normal tissues and in diverse types of carcinoma. Our aim was to analyze the immunohistochemical expression of AMACR in the sequence-progression of colonic cancer. We studied 237 cases, including samples of normal mucosa of the colon, adenomas with different degrees of dysplasia, colonic carcinomas, lymph nodes and liver metastases of colonic carcinomas. A scale of intensity and percentage of expression was used to analyze the AMACR immunohistochemical profile. The expression was nearly absent in samples of normal mucosa, increased in both adenomas and carcinomas, decreased in lymph node metastases but was significantly increased in liver metastases (AU)

Diagnóstico diferencial inmunomorfológico de las lesiones quísticas maxilares con queratinización / Immunomorphological differential diagnosis of maxillary cystic lesions with keratinization

Objetivos. Los quistes maxilares con queratinización son formas lesionales de carácter controvertido y de relevancia clínica, dada la implicación clínico-evolutiva del llamado tumor odontogénico queratoquístico (TOQ). En el presente estudio nos planteamos valorar la utilidad de las técnicas inmunohistoquímicas en la identificación de estas lesiones. Material y métodos. Se analizan de forma retrospectiva las lesiones quísticas maxilares dotadas de fenómenos de queratinización interna, diagnosticadas en un mismo centro hospitalario, a lo largo de un periodo de 4 años, realizando un estudio inmunohistoquímico mediante la aplicación de un panel de cinco anticuerpos (Ki67, Bcl-2, p53, CK19, D2-40). Resultados. De un total de 410 lesiones quísticas maxilares, se seleccionaron 22 casos (5,36%) en los que existían rasgos morfológicos de queratinización interna. Aplicando los criterios morfológicos de la clasificación histológica de la OMS (2005) se diagnosticaron 15 TOQ y 4 quistes odontogénicos ortoqueratósicos (QOO), existiendo 3 observaciones con rasgos morfológicos híbridos de TOQ y de QOO. El estudio inmunohistoquímico llevado a cabo permitió realizar un certero diagnóstico diferencial entre el TOQ y el QOO, y además su empleo posibilitó adscribir de forma correcta las formas híbridas a cada uno de estos dos tipos lesionales. Conclusiones. El análisis inmunohistoquímico, con la aplicación de un panel de cinco anticuerpos, permite un diagnóstico certero de las lesiones quisticas maxilares con queratinización, permitiendo la diferenciación del TOQ frente al QOO, así como la correcta identificación de las lesiones de carácter morfológico híbrido(AU)

Objectives. Maxillary cystic lesions with keratinization are controversial lesions that are clinically relevant due to the prognostic implication of the so-called keratocystic odontogenic tumor (KOT). The aim of this study was to assess the usefulness of immunohistochemistry in the identification of KOTs. Material and methods. Retrospective study of all maxillary cystic lesions exhibiting keratinization diagnosed over a 4-year period in one hospital center. Immunohistochemical study using a panel of five antibodies (Ki67, Bcl-2, p53, CK19, D2-40) was performed. Results. Of a total of 410 maxillary cystic lesions, 22 cases (5.36%) showing morphological features of internal keratinization were selected. Using WHO-2005 histological criteria, 15 KOTs and 4 orthokeratotic odontogenic cysts (OOC) were diagnosed, as well as 3 cases with hybrid morphological characteristics. Immunohistochemical results accurately differentiated between KOT and OOC and allowed the hybrid forms to be correctly identified. Conclusions. Immunohistochemical analysis with a panel of five antibodies allows an accurate diagnosis of maxillary cystic lesions with keratinization, enabling the differentiation between KOT and OOC and the correct identification of cases of hybrid morphological characteristics(AU)

Expresión de SDHB en el tumor de Warthin / SDHB expression in Warthin’s tumour

Introducción: El objetivo del presente estudio es analizar la expresión de la succinodeshidrogenasa B (SDHB), enzima perteneciente al complejo mitocondrial II, en el tumor de Warthin analizando su relación con los cambios oncocíticos, un dato morfológico constante en este tipo tumoral. Material y métodos: En una serie de 10 tumores de Warthin, todos parotídeos, se analizó en los especímenes de resección tumoral la expresión de SDHB utilizando un anticuerpo monoclonal comercializado. Resultados: Los 10 tumores afectaron a 10 varones (edad media: 64, 2 años; rango: 40-80), todos ellos con antecedentes de hábito tabáquico, y 2 con afectación bilateral metacrónica. Dos pacientes presentaron de forma asociada carcinomas del tracto urotelial. El estudio de la SDHB mostró en todos los casos acusada reactividad (++/+++) del componente epitelial oncocítico, así como del citoplasma de los conductos estriados del tejido parotídeo no tumoral. Esta expresión no se vio influida por el rango de edad, la intensidad del hábito tabáquico, ni por el carácter bilateral de los tumores. Uno de los tumores presentó focos de metaplasia escamosa con negatividad a la SDHB en esa localización. Discusión y conclusiones: La constante e intensa reactividad de la SDHB encontrada en nuestras observaciones, en las células oncocíticas, orienta a considerar que los cambios oncocíticos en el tumor de Warthin no se asocian a una actividad enzimática defectiva en este componente del complejo mitocondrial II. La reactividad de SDHB se conforma como un marcador adicional de la diferenciación oncocítica existente en el caso del tumor de Warthin, aspectos ambos previamente no descritos (AU)

Introduction: Succinic dehydrogenase subunit B (SDHB) is an enzyme belonging to the mitochondrial complex II. The aim of this study is to analyse SDHB expression in a series of Warthin’s tumours, studying its relationship with oncocytic changes, constantly present in this form of tumour. Material and methods: In resection tumour specimens from a series of ten Warthin’s tumours (all from the parotid gland), immune histochemical expression of SDHB was analysed using a commercially-available monoclonal antibody. Results: The Warthin’s tumours studied affected 10 men (mean age: 64.2 yrs, range 40-80), all with smoking habits, and 2 with metachronous bilateral involvement. Two patients presented associated urothelial carcinoma. Our SDHB study showed marked reactivity (++/+++) in all cases in the oncocytic epithelial component and also in striated duct cytoplasm (+) from non-tumorous parotid tissue. Expression was not influenced by age, smoking intensity or bilateral character. One of the tumours showed squamous metaplasia foci with SDHB-negativity at this level. Discussion and conclusions: Due to the constant and intense SDHB reactivity in oncocytic cells in our observations, oncocytic changes are not considered to be associated with defective enzyme activity in the mitochondrial complex II. Strong SDHB reactivity is an additional marker of oncocytic changes in Warthin’s tumour. Neither of these facts has been described previously (AU)