Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas

'Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations (AU)

Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations.

Prognostic significance of sentinel node biopsy status in cutaneous melanoma: a 21-years prospective study from a single institution.

AIM: To analyze the accuracy of the sentinel lymphatic node biopsy (SLNB) and to investigate predictive factors for sentinel node (SN) status and prognostic factors for recurrence-free survival (RFS) and disease-specific survival (DSS) in patients with melanoma. MATERIAL AND METHODS: Between June 1997 and June 2017, 440 consecutive patients, who underwent SLNB by a single surgical team, were prospectively included. Descriptive and survival analysis were performed. RESULTS: 119 of 440 patients (26%) had positive SN. SLNB's false-negative rate was 6.3%. Breslow thickness, Clark´s level, ulceration and histological subtype were statistically significant predictive factors of SN metastases. In a multivariate analysis, positive SN (HR = 2.21, p = 0.01), deeper Breslow thickness (HR = 2.05, p = 0.013), male gender (RR = 2.05, p = 0.02), and higher Clark's level (HR = 2.30, p = 0.043) were significantly associated with decreased RFS; and positive SN (HR = 2.58, p < 0.001), deeper Breslow thickness (HR = 2.57, p = 0.006) and male gender (HR = 1.93, p = 0.006) were associated with lower DSS. CONCLUSION: SLNB is a reliable and reproducible procedure with high sensitivity (93.7%). Positive SN metastases, Breslow thickness and male gender were statistically associated with poorer outcomes. Male gender was an independent prognostic factor of tumor thickness or SN status.

Utility of PET/CT in patients with stage I-III melanoma.

PURPOSE: To study the utility of positron emission tomography with computerized tomography (PET/CT) in patients with a stage I-III melanoma. PATIENTS AND METHODS: PET/CT findings from all patients with a stage I-III melanoma attended at our hospital from September 2011 to November 2015 were reviewed. RESULTS: Data from 83 patients with a stage I-III melanoma, 39 patients with a positive sentinel lymph node biopsy (SLNB) and 35 patients with locoregional recurrences were analyzed. Sensitivity of PET/CT in clinical stage I-III patients was 5%, with a 14% of false positives. In patients with a positive SLNB, PET/CT previous to complete lymph node dissection had a 23% of false negatives. In patients with clinical locoregional recurrences, PET/CT findings revealed asymptomatic visceral distant metastasis in 25.7%. CONCLUSIONS: PET/CT has a significant rate of false positive and negative results in patients with a stage I-III melanoma. Utility in patients with nodal locoregional recurrences seems higher than in patients with skin metastases.

Why do patients with thick melanoma have different outcomes? A retrospective epidemiological and survival analysis

No disponible

Absceso retroperitoneal secundario a espondilodiscitis tuberculosa simulando una hernia inguinal incarcerada.

Tuberculosis is the commonest cause of spinal infection worldwide (9-46%). Tuberculosis spondylodiscitis causes multifocal thoracic and lumbar spinal disease, and big paraspinal and psoas abscesses. It is more frequent in people under 40 who had previous tuberculosis infection and from countries where the illness is endemic. Clinic is non-specific and sub-acute. We report the clinical case of a 29-year-old patient who suffered from pericardic tuberculosis in her childhood and who presents a bilateral retroperitoneal abscess due to tuberculosis spondylodiscitis. Her clinical debut began with left inguinal pain and an irreducible mass at this level that simulated an incarcerated inguinal hernia, which is why surgery was indicated. Due to discrepancies between intraoperative findings and the initial diagnosis, the diagnosis and treatment strategy were changed. The purpose of this case report is to emphasize the challenge that the diagnosis of this pathology represents, due to low incidence in our environment and poor clinical features, which results in late diagnosis and late management.