ACTH-producing adrenocortical carcinoma: an exceedingly rare diagnosis.

BACKGROUND: Adrenocortical carcinoma is a very rare endocrinopathy that has a poor prognosis and is frequently associated with ACTH-independent Cushing's syndrome. Despite having an adrenocortical carcinoma, our patient surprisingly had an ACTH-dependent Cushing's syndrome. CASE REPORT: A 26-year-old female presented with Cushing's syndrome and an abdominal mass. Imaging studies revealed an adrenal mass consistent with a high-grade malignancy. Laboratory workup showed hypercortisolism, hyperandrogenism, and hypokalemia with normal levels of metanephrines. Unexpectedly, her ACTH levels were remarkably elevated. The pathological analysis of a tumor sample was conclusive for adrenocortical carcinoma with immunopositivity for ACTH. CONCLUSIONS: Our patient suffered from an adrenocortical carcinoma that was ectopically producing ACTH. This case emphasizes that physicians should have a broad-minded approach when evaluating cases of rare endocrine malignancies.

Abdominal Actinomycosis misdiagnosed as liposarcoma

Actinomycosis is an uncommon, endogenous, and chronic infection with varied and nonspecific clinical features such as abdominal, pelvic or cervical masses, ulcerative lesions, abscesses, draining fistula, fibrosis, and constitutional symptoms. The disease ensues when the bacteria disrupt the mucosal barrier, invade, and spread throughout interfascial planes. Currently, the diagnosis of actinomycosis is challenging because of its very low frequency and depending on the clinical presentation it may masquerade malignancies. Therapy consists initially in intravenous penicillin, followed by an oral regimen that may be extended until a year of treatment. A timely diagnosis is crucial to avoid extensive therapeutic attempt as surgery. However, a biopsy or drainage of abscesses and fistula's tract may be required not only as a diagnostic procedure as part of the therapy. We report the case of a 72-year-old woman with an abdominal mass initially misdiagnosed as a liposarcoma. A second biopsy of a skin lesion of the abdominal wall made the diagnosis of actinomycosis, avoiding a major surgical procedure. The patient was treated with a long-term course of antibiotics with favorable outcome. Liposarcoma was ruled out after the patient's full recovery with antibiotics and the misdiagnosis was credit to the overconfidence on the immunohistochemical positivity to MDM2.

Abdominal Actinomycosis misdiagnosed as liposarcoma.

Actinomycosis is an uncommon, endogenous, and chronic infection with varied and nonspecific clinical features such as abdominal, pelvic or cervical masses, ulcerative lesions, abscesses, draining fistula, fibrosis, and constitutional symptoms. The disease ensues when the bacteria disrupt the mucosal barrier, invade, and spread throughout interfascial planes. Currently, the diagnosis of actinomycosis is challenging because of its very low frequency and depending on the clinical presentation it may masquerade malignancies. Therapy consists initially in intravenous penicillin, followed by an oral regimen that may be extended until a year of treatment. A timely diagnosis is crucial to avoid extensive therapeutic attempt as surgery. However, a biopsy or drainage of abscesses and fistula's tract may be required not only as a diagnostic procedure as part of the therapy. We report the case of a 72-year-old woman with an abdominal mass initially misdiagnosed as a liposarcoma. A second biopsy of a skin lesion of the abdominal wall made the diagnosis of actinomycosis, avoiding a major surgical procedure. The patient was treated with a long-term course of antibiotics with favorable outcome. Liposarcoma was ruled out after the patient's full recovery with antibiotics and the misdiagnosis was credit to the overconfidence on the immunohistochemical positivity to MDM2.

Neuroendocrine tumor of the appendix-a case report and review of the literature.

Incidental appendiceal neuroendocrine tumors (ANETs) occur in 0.2-0.7% of surgical resections for suspected appendicitis (Moris, Tsilimigras, Vagios, Ntanasis-stathopoulos, Karachaliou, Papalampros, et al. Neuroendocrine neoplasms of the appendix: a review of the literature. Anticancer Res [Internet]. 2018;38:601-11.). It's a rare entity, clinically challenging and has controversy surrounding its management. A 69-year-old male presented to our clinic with acute right lower quadrant abdominal pain with less than 24 hours of evolution. The presumed clinical diagnosis was acute appendicitis. It was performed a laparoscopic appendectomy and the histopathologic result of the specimen showed neuroendocrine tumor of the appendix. ANETs are rare and typically diagnosed as incidental histopathologic finds in appendices specimens. However, they are the most common tumors arising from de appendix, between 30% and 80% of the cases (Pape, Niederle, Costa, Gross, Kelestimur, Kianmanesh, et al. ENETS consensus guidelines for neuroendocrine neoplasms of the appendix (excluding goblet cell carcinomas). Neuroendocrinology. 2016;103:144-52). Appendectomy may be sufficing for tumors <2 cm and they are associated with good long-term outcomes.

Small bowel obstruction due to intraluminal metastasis from malignant melanoma.

The most common form of presentation of malignant melanoma is cutaneous. However, it is important to notice that it can occur in any part of the body, mostly from metastatic route. Gastrointestinal forms of symptomatic metastasis are rare, making it sometimes difficult to diagnose. We present a rare case of an 84-year-old female with a small bowel obstruction due to melanoma metastasis. She was treated with an enterectomy, with no surgical or post-operative complications. Although surgery should be reserved from symptomatic patients, some authors refer to it as a palliative treatment with improvement in long-term survival.

Análise do acolhimento com classificação de risco em uma maternidade pública terciária de Fortaleza / Analysis of risk classification in a tertiary public maternity of Fortaleza

Objetivo: Analisar os atendimentos no acolhimento com classificação de risco em uma maternidade pública terciária de Fortaleza, Ceará. Metodologia: Estudo descritivo, retrospectivo, com abordagem quantitativa, que utilizou técnica de análise documental, com amostra de 365 instrumentos para classificação de risco, realizados no setor de emergência de uma maternidade pública terciária, no período de janeiro a junho de 2016. Resultados: A maioria (57,5%) das mulheres foi classificada como pouco urgente para o atendimento, sendo classificadas com a cor verde. As principais queixas nos atendimentos foram: dor (34,5%), sangramento vaginal (17,5%) e perda de líquido (14%). Conclusão: A clientela que busca atendimento precisa ser mais bem orientada sobre os sinais e sintomas do trabalho de parto, durante as consultas de pré-natal na atenção básica.

Objective: To analyze the host with risk classification in a tertiary public maternity hospital in Fortaleza, Ceará. Methodology: A descriptive, retrospective study with a quantitative approach using a documental analysis technique, with a sample of 365 instruments for risk classification, performed in the emergency sector of a tertiary public maternity hospital, from January to June, 2016. Results: The majority (57.5%) of the women were classified as not very urgent for care being classified as green. The main complaints were pain (34.5%), vaginal bleeding (17.5%) and fluid loss (14%). Conclusion: Clients seeking care need to be better informed about the signs and symptoms of labor during prenatal consultations in basic care.

Objetivo: Analizar la acogida con clasificación de riesgo en una maternidad pública terciaria de Fortaleza, Ceará. Metodología: Estudio descriptivo, retrospectivo, con abordaje cuantitativo, que utilizó técnica de análisis documental, con muestra de 365 instrumentos para clasificación de riesgo, realizados en el sector de emergencia de una maternidad pública terciaria, en el período de enero a junio de 2016. Resultados: La mayoría (57,5%) de las mujeres fue clasificada como poco urgente para la atención siendo clasificadas con el color verde. Las principales quejas en las atenciones fueron: dolor (34,5%), sangrado vaginal (17,5%) y pérdida de líquido (14%). Conclusión: La clientela que busca atención necesita ser mejor orientada sobre los signos y síntomas del trabajo de parto, durante las consultas de prenatal en la atención básica.

Parathyroid carcinoma: From a case report to a review of the literature.

INTRODUCTION: Parathyroid carcinoma is a rare endocrine malignancy. The reported incidence is from 0.5 to 5% of primary hyperparathyroidism cases in various series. Consideration of parathyroid carcinoma in the differential diagnosis of hypercalcaemic disorders is important because the morbidity and mortality are substantial and the best prognosis is associated with early recognition and surgical resection. PRESENTATION OF CASE: We report a case of parathyroid carcinoma occurring in a 60-year-old woman who presented to our institution with persistent hyperparathyroidism and hypercalcemia 3 years after being submitted to one gland parathyroidectomy in another hospital. DISCUSSION AND CONCLUSION: A review of the related literature follows.

Qualidade de Vida Familiar na Deficiência Intelectual: Revisão Sistemática de Estudos / Family Quality of Life in Intellectual Disability: A Systematic Review of Studies

Resumo A qualidade de vida familiar (QdVF) em famílias de pessoas com deficiência intelectual (DI) tem interessado investigadores, sugerindo potencialidades em termos de avaliação e intervenção. O presente artigo realizou uma revisão sistemática dos estudos empíricos que analisam a QdVF em famílias de pessoas com DI. Efetuou-se uma pesquisa bibliográfica num portal de bases de dados e recorreu-se a critérios de inclusão previamente definidos. Do total de 32 estudos selecionados, 15 recorreram ao instrumento FQOLS-2006, focando-se na análise das dimensões e domínios da mesma; 11 ao Beach Center FQOL Survey, relacionando o constructo com diversas variáveis; e seis a outras formas de avaliação, essencialmente qualitativas. Assim, a QdVF mostra-se pertinente para compreender e intervir com famílias de pessoas com DI.

Abstract Family quality of life (FQoL) for families with members who have an intellectual disability (ID) has recently captured the interest of researchers and demonstrated potential for evaluation and intervention. This paper intends to revise systematically empirical studies that analyze FQoL in families with members having ID. A bibliographic search was carried out in a database and previously defined inclusion criteria were used. From a total of 32 studies, 15 used the FQOLS-2006 that focused on the analysis of their dimensions and domains, 11 the Beach Center FQOL Survey that related the construct to other individual and family variables and six other methods to evaluate FQoL. Thus, FQoL is relevant to understand and intervene with families of persons with ID.

Inflammatory myofibroblastic tumor presenting as ileocecal intussusception-A case report.

INTRODUCTION: Inflammatory myofibroblastic tumor (IMFT) is an uncommon mesenchymal solid tumor commonly documented in children and young adults. It is usually located in lungs however, extrapulmonary involvement has also been reported. Here we report a case of IMFT presenting as an ileocecal intussusception. PRESENTATION OF CASE: A 55-year-old man presented with a two months history of colicky abdominal pain, more intense at the right inferior abdominal quadrant, and unintentional weight loss of 8kg in the previous four months. Computer tomography showed an image of intussusception at the cecum. Colonoscopy demonstrated a pediculated tubular lesion, with the base near ileocecal valve. We performed a right hemicolectomy. Histopathological examination of the tumor revealed an IMFT. DISCUSSION: IMFT usually affects lungs with rare gastrointestinal involvement. Clinical presentation is related with location of the neoplasm. Intussusception in adults presents with non-specific symptoms and classical image signs facilitate preoperative diagnosis. IMFT diagnosis is histopathological which usually implies surgical resection. Complete surgical excision, with microscopically clear margins, is the mainstain of treatment, with virtually no local recurrence or metastasis. CONCLUSION: Gastrointestinal IMFT are rare in the adult population and clinical features depend on its location. Surgical approach with total excision of the neoplasm confirms the diagnosis. Prognosis is good with the main prognostic indicator being the adequacy of the primary excision.

Expression of citrulline and homocitrulline residues in the lungs of non-smokers and smokers: implications for autoimmunity in rheumatoid arthritis.

INTRODUCTION: Smoking is a well-established risk factor for rheumatoid arthritis (RA), and it has been proposed that smoking-induced citrullination renders autoantigens immunogenic. To investigate this mechanism, we examined human lung tissue from 40 subjects with defined smoking status, with or without chronic obstructive pulmonary disease (COPD), and control tissues from other organs for citrullinated proteins and the deiminating enzymes peptidylarginine deiminase type-2 (PAD2) and -4 (PAD4). METHODS: Lung tissue samples, dissected from lobectomy specimens from 10 never smokers, 10 smokers without airflow limitation, 13 COPD smokers and eight COPD ex-smokers, and control tissue samples (spleen, skeletal muscle, liver, ovary, lymph node, kidney and heart), were analysed for citrullinated proteins, PAD2 and PAD4 by immunoblotting. Citrulline and homocitrulline residues in enolase and vimentin were analysed by partial purification by gel electrophoresis followed by mass spectrometry in 12 of the lung samples and one from each control tissues. Band intensities were scored semi-quantitatively and analysed by two-tailed Mann-Whitney T-test. RESULTS: Within the lung tissue samples, citrullinated proteins, PAD2 and PAD4 were found in all samples, with an increase in citrullination in COPD (P = 0.039), but minimal difference between smokers and non-smokers (P = 0.77). Citrullination was also detected at lower levels in the tissues from other organs, principally in lymph node, kidney and skeletal muscle. Mass spectrometry of the lung samples showed that vimentin was citrullinated at positions 71, 304, 346, 410 and 450 in non-smokers and smokers both with and without COPD. A homocitrulline at position 104 was found in four out of six COPD samples and one out of six non-COPD. Citrulline-450 was also found in three of the control tissues. There were no citrulline or homocitrulline residues demonstrated in α-enolase. CONCLUSIONS: We have shown evidence of citrullination of vimentin, a major autoantigen in RA, in both non-smokers and smokers. The increase in citrullinated proteins in COPD suggests that citrullination in the lungs of smokers is mainly due to inflammation. The ubiquity of citrullination of vimentin in the lungs and other tissues suggests that the relationship between smoking and autoimmunity in RA may be more complex than previously thought.