Temporal headache and jaw claudication may be the key for the diagnosis of giant cell arteritis.

BACKGROUND: Temporal artery biopsy (TAB) is a surgical procedure with a low positive yield. The purpose of this study is to determine which variables are the most important in the giant cell arteritis (GCA) diagnosis. The objective of this evaluation is to improve the percentage of positive temporal artery biopsy and if possible, avoid the biopsy in some cases. MATERIAL AND METHODS: A retrospective clinical study consisted of 90 patients who had undergone TAB at the Río Hortega Hospital (Spain) from January 2009 to December 2016. Clinical findings, erythrocyte sedimentation rates (ESR) and other laboratory parameters, American College of Rheumatology (ACR) criteria for GCA score and biopsy results were recorded. RESULTS: Nineteen (21.1%) biopsies were positive for GCA. The mean age in positive TAB was 78.6 years old (SD 7.93), and 73.7% were female. Presence of temporal headache (p = 0.003), jaw claudication (p = 0.001), abnormal artery exploration (p = 0.023), elevated erythrocyte sedimentation rate (p = 0.035), CRP (p = 0.018) and platelets (p = 0.042), were significantly associated with GCA. Multivariate logistic regression revealed that the best predictors for the diagnosis of GCA are headache and jaw claudication, adjusted by sex, age, and temporal exploration. CONCLUSIONS: TAB has benefit only for patients who score a 2 or 3 on the ACR criteria for GCA without biopsy. These findings highlight the need for a better diagnostic strategy for patients with suspected temporal arteritis.

The Endoscopic Reference Score shows modest accuracy to predict either clinical or histological activity in adult patients with eosinophilic oesophagitis.

BACKGROUND: Conflicting results have been recently reported for the accuracy of the Endoscopic Reference Score (EREFS), an standardised endoscopic classification, to predict the histological activity of eosinophilic oesophagitis (EoE). AIM: To evaluate the accuracy of the EREFS to predict either histological or clinical activity of EoE. METHODS: Prospective multicentre study conducted in eight Spanish centres evaluating adult EoE patients, either naïve or after treatment. Symptoms were evaluated before upper endoscopy through the Dysphagia Symptom Score, whereas researchers scored the EREFS immediately after the endoscopic procedure, unaware of the histological outcome. RESULTS: One hundred and forty-five EoE patients undergoing 240 consecutive endoscopic procedures were included. Exudates (P = 0.03), furrows (P = 0.03) and a composite score of inflammatory signs (exudates, furrows and oedema) (P < 0.001) accurately predicted histological activity. Exudates were the only endoscopic sign showing a good correlation with histological outcome after therapy. Furrows and oedema persisted in 50% and 70% of patients despite histological remission. No endoscopic feature exceeded 70% accuracy to predict histological activity. Likewise, no endoscopic finding could adequately predict dysphagia severity. Crepe paper mucosa, diffuse exudates and severe rings correlated with higher symptom scores. CONCLUSIONS: Endoscopic findings assessed by the Endoscopic Reference Score did not correlate with histological or clinical disease activity in adult EoE patients. Only exudates correlated with peak eosinophil count and histological outcome, whereas furrows and oedema persisted in over half of patients despite histological remission.

Cutaneous mucormycosis infection by Absidia in two consecutive liver transplant patients.

Mucormycosis, although an infrequent fungal infection, has a high mortality in patients undergoing orthotopic liver transplantation. We present two cases of cutaneous Absidia mucormycosis in two successive patients undergoing liver transplantation in our hospital. In our literature search, we encountered only one published case of Absidia infection in liver transplantation.

[External ear melanoma]. / Melanoma de conducto auditivo externo.

Cutaneous melanomas are the tumours that have increased more their incidence in the last fifty years. Melanomas arising from the external auditory canal are extraordinariously unfrequent. These tumours show an aggressive and silent behaviour, and due to this the diagnosis is frequently made in an advanced stage. A male with a malignant melanoma arising from his left external auditory canal was attended in our department, suspecting an epidermoid carcinoma. The clinical findings and the extension of the lesion required a lateral temporal bone resection, parotidectomy and neck dissection to achieve a total resection. We present a review of the literature about this entity and an analysis of the incidence, significance of the lymph node metastases and value of the elective neck dissection.

Melanoma de conducto auditivo externo / Melanoma in the external auditory canal

Los melanomas cutáneos son los tumores cuya incidencia más ha aumentado en los últimos cincuenta años. Los tumores originados en el conducto auditivo externo son extraordinariamente raros. Estos tumores muestran un comportamiento agresivo y silente. Debido a esto el diagnóstico se realiza en estadios avanzados habitualmente. Un varón con un melanoma originado en el conducto auditivo externo izquierdo fue atendido en nuestro Servicio sospechándose inicialmente un carcinoma epidermoide. Los hallazgos de la lesión y la extensión requirieron una resección lateral del temporal, parotidectomía y disección cervioganglionar para completar una resección total. Presentamos una revisión de la literatura sobre este caso y un estudio de la incidencia, importancia de las metástasis ganglionares y valoración del vaciamiento cervical electivo (AU)

Cutaneous melanomas are the tumours that have increased more their incidence in the last fifty years. Melanomas arising from the external auditory canal are extraordinariously unfrequent. These tumours show an aggressive and silent behaviour, and due to this the diagnosis is frequently made in an advanced stage. A male with a malignant melanoma arising from his left external auditory canal was attended in our department, suspecting an epidermoid carcinoma. The clinical findings and the extension of the lesion required a lateral temporal bone resection, parotidectomy and neck dissection to achieve a total resection. We present a review of the literature about this entity and an analysis of the incidence, significance of the lymph node metastases and value of the elective neck dissection (AU)

[Idiopathic retroperitoneal fibrosis]. / Fibrosis retroperitoneal idiopática.

OBJECTIVE: A case of idiopathic retroperitoneal fibrosis in a 34-year-old man with anemia and elevated erythrocyte sedimentation rate is presented. METHODS/RESULTS: Ultrasound, CT and MRI evaluation and pathological study were performed. These diagnostic imaging techniques demonstrated an infiltrating mass in the upper portion of the abdomen which was resected. Histological and immunohistochemical studies of the surgical specimen demonstrated sclerosing retroperitonitis. CONCLUSIONS: Idiopathic retroperitoneal fibrosis (Ormond's disease) is a rare disease entity whose etiology is obscure and is characterized by retroperitoneal fibrosis with inflammatory infiltrates composed of plasma cells, lymphocytes and eosinophils. This case is of interest due to its atypical location, and because the patient was young with no remarkable clinical history and only mild anemia and an elevated erythrocyte sedimentation rate.

[Adrenal gland myelolipoma: radiological view]. / Mielolipoma suprarrenal: una visión radiológica.

OBJECTIVE: To report a case of adrenal myelolipoma in a female patient with a history of arterial hypertension and increased plasma aldosterone levels. METHODS: Evaluation by CT and ultrasound, and anatomopathological analysis were performed. RESULTS: The CT and ultrasound scans showed a fat-containing adrenal mass. The patient underwent surgery. Histological analysis of the surgical specimen demonstrated a myelolipoma, a benign tumor composed in varying proportions of adipose and hematopoietic tissue. CONCLUSIONS: A diagnosis of adrenal myelolipoma must be considered when a fat-containing adrenal mass is demonstrated by ultrasound, CT or MRI.

[Retroperitoneal ganglioneuroma in childhood]. / Ganglioneuroma retroperitoneal en la infancia.

OBJECTIVE: An incidentally discovered retroperitoneal ganglioneuroma in a 4-year-old child is presented. METHODS: Ultrasonography and CT were performed. The surgical specimen was analyzed by macroscopic, histological and immunohistochemical techniques. RESULTS: US and CT localized a retroperitoneal mass independent from the left kidney and adrenal gland. The histological study showed a fascicular proliferation with myxoid and fibrillar areas mixed with mature ganglion cells. These cells were positive for neurofilament and neuron specific enolase. The patient had a favorable outcome with no signs of tumor recurrence. CONCLUSIONS: Ganglioneuroma is a rare, completely mature tumor that has to be differentiated from neuroblastoma.

Invaginación ileocecal causada por un pólipo fibroide inflamatorio / Ileocecal intussusception caused by an inflammatory fibroid polyp

El pólipo fibroide inflamatorio es una lesión poco frecuente del tracto gastrointestinal caracterizada por una proliferación celular no neoplásica compuesta por fibroblastos, vasos sanguíneos y células inflamatorias en el seno de una estroma edematosa y colágena. Presentamos el caso de una mujer de 78 años de edad en la que la enfermedad se manifestó inicialmente con un cuadro de suboclusión intestinal. El estudio histológico puso de manifiesto una estructura polipoide constituida por una proliferación de vasos de variado tamaño, algunos congestivos, y de fibroblastos, que se disponían en áreas de forma concéntrica. La estroma se acompañaba de un intenso infiltrado inflamatorio con abundantes eosinófilos. El pólipo fibroide inflamatorio podría considerarse una lesión no neoplásica de carácter reactivo e inflamatorio, y explicarse como una cicatrización hipertrófica del tejido conectivo submucoso, a modo de queloide (AU)

[Persistent leucocytosis as initial manifestation of Whipple's disease and development of gastric cancer in the follow up]. / Leucocitosis persistente como debut de la enfermedad de Whipple con desarrollo de cáncer gástrico en el seguimiento.

We report the case of a 57 year old male with Whipple's disease. The patient was asymptomatic and an unexplained peripheral leucocytosis was found in a routine examination. It persisted as the only abnormality for one year and then he developed articular symptoms, diarrhoea and weight loss. The diagnosis was confirmed by duodenal biopsy five years later. The leucocyte count ranged between 14,000 and 22,000 leuc/mm3. Response to cotrimoxazole was favourable with disappearance of all signs and symptoms, including leucocytosis. In the last endoscopic control, eight years after initial manifestations, an intramucosal gastric adenocarcinoma was diagnosed.

[An intimal sarcoma of the pulmonary artery. An immunohistochemical study]. / Sarcoma intimal de la arteria pulmonar. Estudio inmunohistoquímico.

Pulmonary artery intimal sarcomas tend to be presented with symptoms of pulmonary thromboembolism and grow regionally, with little capacity to metastasize. They probably originate from subendothelial cells, that become myofibroblasts. Knowledge of it is important to establish a presurgery diagnosis, with the possibility of a total resection, the only useful treatment until now. We report a case of a pulmonary artery primary sarcoma, in a 73 year old woman, admitted with hemoptysis and pleuritic chest pain, who died ten days after. Autopsy revealed an intraluminal mass at the pulmonary artery trunk, without regional nor distance involvement. Microscopic study showed a pleomorphic tumor with spindle and epithelioid cells, positive for actin, desmin and vimentin. All these data support the diagnosis of primary intimal sarcoma of the pulmonary artery. We want to emphasize the myogenic differentiation of the tumor, uncommon in previously reported cases.

[Granulomatous prostatitis. Review of 22 cases]. / Prostatitis granulomatosa. Revisión de 22 casos.

Granulomatous prostatitis is an uncommon inflammatory process of the prostate, which can be caused by a variety of infectious agents or be part of a systemic allergic condition. In most cases, origin is unspecific. Clinical presentation, treatment and evolution are similar to those of benign prostatic hypertrophy and, in many cases, can only be distinguished from prostate carcinoma in the histological study. The authors present in this paper the results and conclusions of a retrospective study of 22 cases of granulomatous prostatitis.

[Extramedullary anaplastic plasmacytoma]. / Plasmocitoma anaplásico extramedular.

A case of extramedullary plasmacytoma of the maxillary sinus, locally aggressive, in a 65 years old man is presented. Clinical diagnosis of adenocarcinoma was suspected but the pathological study showed an anaplastic morphology, with little plasmacytic differentiation and lack of stain with cytokeratins and epithelial membrane antigen (EMA), immunohistochemical marker of plasmatic cells, among others. Definitive diagnosis was based on light chain restriction and lack of multiple myeloma.

[Mucinous adenocarcinoma of the prostate. Report of 3 cases and review of the literature]. / Adenocarcinoma mucinoso de próstata. Presentación de tres casos y revisión de la literatura.

OBJECTIVES: To describe 3 cases of mucinous adenocarcinoma, an uncommon variant of prostatic adenocarcinoma, and review the literature. METHODS: 3 cases of mucinous adenocarcinoma of the prostate are described. The histological and immunohistochemical features, diagnostic and therapeutic aspects of this variant of prostatic adenocarcinoma are presented and the literature briefly reviewed. RESULTS/CONCLUSIONS: This variant of prostatic adenocarcinoma is characterized by the presence of extra and intracellular mucus formation. It is generally seen in the advanced stages and responds poorly to any form of therapy.