Metaplastic Breast Carcinoma in a 37-Year-Old Female: A Case Report.

Metaplastic breast carcinoma (MBC) is a rare and aggressive histologic subtype of cancer. Because of its rarity and heterogeneity, the management of these patients is challenging. Here, we present the case of a rapidly progressive MBC with mesenchymal differentiation in a 37-year-old female, treated with trimodal therapy consisting of neoadjuvant chemotherapy with paclitaxel and carboplatin, followed by dose-dense cyclophosphamide and doxorubicin (ddAC), modified radical left mastectomy, and adjuvant radiotherapy. Despite the need to anticipate the surgery after the first cycle of ddAC, because of a life-treating adverse event, there was a pathologic complete response. Nevertheless, 6.2 months after completing adjuvant radiotherapy, the patient had a recurrence on the central nervous system (CNS) (two lesions), which was managed with excisional biopsy and stereotactic body radiation therapy. The patient also started "complementary" chemotherapy with capecitabine. Still, 18 months after being diagnosed, she died due to CNS disease progression.

Surviving Extreme Anaemia.

Before the development of transfusion medicine, severe anaemia was an important cause of morbidity and mortality. The discovery of haematopoietic mechanisms and essential nutrients made it possible to easily treat and prevent this condition. Nevertheless, it is often fatal in patients presenting with extreme anaemia (haemoglobin levels <2 g/dl). We report the rare case of a 54-year-old woman who presented with profound megaloblastic anaemia (haemoglobin of 1.7 g/dl) due to vitamin B12 deficiency, and was successfully treated. LEARNING POINTS: The discovery of vitamin B12 in the 20th century led to the successful and easy treatment of thousands of patients with anaemia.Focus on patient adherence to treatment and medical advice is essential in order to manage chronic conditions such as post-gastrectomy nutritional deficiencies.Extreme anaemia is very rare and associated with high mortality; treatment should be tailored to acute or chronic anaemia and in cases where haemodynamic stability is guaranteed, a restrictive blood transfusion strategy should be considered to reduce the risk of complications.

Mesalazine-induced Hypersensitivity Pneumonitis.

A 57-year-old woman with Crohn's disease (ulcerative proctitis) treated with mesalazine (5-ASA) developed worsening respiratory distress and cough. The lack of response to antibiotics and the results of bronchoalveolar lavage led to the diagnosis of mesalazine-related hypersensitivity pneumonitis, an infrequent entity. Symptoms improved after discontinuation of mesalazine and the administration of corticosteroid therapy. The authors discuss the diagnosis and management of this rare condition. LEARNING POINTS: A diagnosis of mesalazine-related hypersensitivity pneumonitis should be considered when unexplained respiratory symptoms develop during treatment with mesalazine.It is important to distinguish pulmonary manifestations in patients with inflammatory bowel disease secondary to drug-related toxicity from the disease process itself.Amelioration of symptoms and improvement in imaging and lung function seem to occur only upon abrupt discontinuation of the drug; severe symptoms such as respiratory failure may justify corticosteroid therapy.

Non-ketotic hemichorea-hemiballismus presenting as generalised tonic-clonic convulsive state in uncontrolled diabetes.

We report the case of a 70-year-old diabetic woman who presented to the emergency department with multiple seizure episodes and coma, prompting the need for sedation and mechanical ventilation. She was transferred to our institution for neurosurgical evaluation as the initial CT scan identified hyperdense lesions in the left basal ganglia, interpreted as acute intracranial haemorrhage. On admission, laboratory tests were mostly normal except for blood glucose of 413 mg/dL. Medical records revealed a history of poorly controlled diabetes mellitus and non-adherence to therapy. After seizure control and lifting sedation, right-sided ataxia/involuntary movements were observed. Considering the patient's history and these findings, the CT scan was reviewed and the striatal region hyperdensities interpreted as lesions typical of non-ketotic hemichorea-hemiballismus. MRI was latter performed and confirmed the diagnosis, even though the unusual presentation. Levetiracetam initiation and glycaemic control optimisation led to great neurological improvement without seizure recurrence.

Brugada Pattern: Unraveling Possible Cardiac Manifestation of SARS-CoV-2 Infection.

We report the case of a 41-year-old patient with no family history of sudden cardiac death. The patient presented with high fever and vomiting and was diagnosed with acute pyelonephritis. Screening for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection was positive. An electrocardiogram (ECG) performed during a fever episode revealed a Brugada pattern. Fever can be a trigger for induction of the electrocardiographic Brugada pattern but it is still unknown if the cardiac involvement by coronavirus disease 2019 (COVID-19) can interfere with myocardial ion channels.

Cladosporium herbarum Hot-Tub Lung Hypersensitivity Pneumonitis in a Greenhouse Worker.

Hypersensitivity pneumonitis is understood to be a delayed allergic reaction to the repeated exposure of a usually innocent inhaled agent, causing inflammatory damage to the pulmonary parenchyma, alveoli and terminal bronchioles. With ongoing exposure, it can cause respiratory compromise and pulmonary fibrosis. Recognizing the heterogeneity of the causative agents and the low incidence of the disease, we considered it important to report the case of a greenhouse worker who developed a Cladosporium sp. related hot-tub lung hypersensitivity pneumonitis. We believe it to be the first reported case of a greenhouse occupational hypersensitivity pneumonitis due to Cladosporium sp. LEARNING POINTS: Enquiring about environmental and occupational exposure during anamnesis is fundamental, particularly when addressing respiratory tract symptoms.The mainstay management of hypersensitivity pneumonitis is based on eviction of the offending agent and control of inflammation, currently with corticosteroids.Antigen source removal may entail important psychological, social and economic consequences for the patient and will need a multidisciplinary approach.

Deep Venous Thrombosis and a Very Rare Finding: Inferior Vena Cava Infra-renal Segment Agenesis.

Bilateral deep vein thrombosis (DVT) should prompt investigation for pro-thrombotic conditions and the exclusion of vascular compromise due to intrinsic and extrinsic factors. The authors present the case of a 47-year-old man admitted with bilateral DVT and diagnosed with inferior vena cava (IVC) infra-renal segment agenesis, and discuss the management of this rare condition. LEARNING POINTS: Bilateral deep vein thrombosis in young and otherwise healthy individuals should prompt investigation of the aetiology and risk factors in case life-long treatment and anticoagulation therapy is required.Although rare, anatomical variations and congenital malformations should be considered even in adult patients.Rare medical cases should prompt a multidisciplinary approach to investigation and treatment, as prognosis will depend on both therapeutic procedures and anticipation of complications.

Perianal tuberculosis in an immunocompetent patient: exploring the uncommon manifestation

As it is an infrequent etiology, the diagnosis of perianal tuberculosis is challenging, especially in the absence of a pulmonary focus. TB should be considered in the differential diagnosis of perianal ulcers, fistulas, abscesses, mainly in non-healing and recurrent anal lesions. Treatment with anti-TB agents can provide complete recovery. Furthermore, these lesions are often diagnosed later after complete histopathological and mycobacterial results, where the benefit of avoiding morbid multiple surgeries by effective anti-TB treatment is lost. We reported a rare case of an immuno-competent patient with perianal TB, which was the first manifestation of the disease. A fit-and-well man in his 20s presented a large perianal abscess. Unexpectedly, his chest X-ray showed a rounded hyper-transparency in the left lung. The abscess was drained. Posterior investigation with culture analysis from pus swabs and sputum revealed the presence of Mycobacterium tuberculosis complex infection. After completing the 6 months of oral administration of anti-TB drugs, the patient was asymptomatic. By highlighting this unusual manifestation, we aim to improve clinicians' awareness of perianal TB, facilitating early recognition and appropriate management. (AU)

[Congenital absence of inferior vena cava associated to polispleenism. Case report]. / Agenésia da veia cava inferio associada a poliesplenismo. Casa clínico.

Interruption of the inferior vena cava with azygos continuation is an uncommon vascular anomaly that results from aberrant development during embryogenesis. We report a case of this anomaly in a young boy of 19 years old sent for our observation for presenting an hipotransparency in the chest x-ray, which study revealed a prominence of the azygos cross. Further evaluation by CT-scans disclosed the diagnosis of congenital absence of the inferior vena cava, associated to multiple spleens (polispleenism).

Visceral Angioedema Induced by Angiotensin Converting Enzyme Inhibitor: Case Report.

INTRODUCTION: Intestinal angioedema is a rare adverse effect of angiotensin converting enzyme inhibitors. CLINICAL CASE: A 42-year old woman presented to the Emergency Department complaining of diffuse abdominal pain, predominantly in the right quadrants, with no other associated symptoms. She had been started on perindopril plus indapamide 72 h before the admission for arterial hypertension. There was no other relevant background. Physical examination suggested peritoneal irritation on the lower quadrants of the abdomen and right flank. Laboratory tests were relevant for mild leukocytosis. Abdomen ultrasound and contrast-enhanced computed tomography scan showed moderate amount of fluid in the pelvic excavation and small intestine wall thickening. She was admitted for observation. Once the hypothesis of intestinal angioedema was admitted, angiotensin converting enzyme inhibitor was withheld and no other-directed therapy was instituted. Within 24 h she showed clinical, analytic and imaging improvement, thus supporting this diagnosis. CONCLUSION: The diagnosis of intestinal angioedema induced by angiotensin converting enzyme inhibitor can be challenging and time consuming due to its rarity and nonspecific symptoms, which may lead to underdiagnosis of this entity.

INTRODUÇÃO: O angioedema intestinal é um efeito adverso raro dos inibidores da enzima de conversão da angiotensina. CASO CLÍNICO: Sexo feminino, 42 anos, recorreu ao Serviço de Urgência por dor abdominal difusa predominante nos quadrantes direitos, sem outra sintomatologia. Tinha iniciado perindopril e indapamida 72 horas antes por hipertensão arterial. Sem outros antecedentes de relevo. Ao exame objetivo salientava-se apenas sinais de irritação peritoneal à palpação da fossa ilíaca e flanco direitos. Analiticamente evidenciava discreta leucocitose, sem outras alterações. A ecografia e tomografia computorizada contrastada abdominal revelaram líquido moderado na escavação pélvica e espessamento das paredes do delgado. Foi internada para vigilância. Admitindo-se angioedema intestinal, o inibidor da enzima de conversão da angiotensina foi suspenso e não foi instituída qualquer outra terapêutica dirigida. Em 24 horas, verificou-se melhoria clínica, analítica e imagiológica, corroborando a hipótese de diagnóstico. CONCLUSÃO: O diagnóstico de angioedema intestinal por inibidor da enzima de conversão da angiotensina é, por vezes, difícil e moroso pela sua raridade e inespecificidade dos sintomas, podendo conduzir ao subdiagnóstico desta entidade.

Cystic duct carcinoma mimicking a middle bile duct tumour.

Cystic duct carcinoma was defined by Farrar as a tumour restricted to the cystic duct, making it a rare disease. The authors describe a case of a cystic duct carcinoma that fulfils Farrar's strict diagnostic criteria and that became clinically relevant by compressing the common hepatic duct, thus causing cholestasis. A cholecystectomy was performed with en bloc resection of the cystic and extrahepatic bile duct with a regional lymphadenectomy.

Neuroendocrine breast cancer.

Neuroendocrine breast cancer is thought to account for about 1% of all breast cancers. This rare type of breast malignancy is more common in older women and presents as a low-grade, slow-growing cancer. The most definitive markers that indicate neuroendocrine carcinoma are the presence of chromogranin, synaptophysin or neuron-specific enolase, in at least 50% of malignant tumour cells. The authors present a case report of an 83-year-old woman, admitted to their institution with right breast lump. Physical examination, mammography and ultrasonography showed a 2.4 cm nodule, probably a benign lesion (BI-RADS 3). A fine needle aspiration biopsy was performed and revealed proliferative epithelial papillary lesion. She was submitted to excisional biopsy and histology showed endocrine breast cancer well differentiated (G1). Immunohistochemically, tumour cells were positive for synaptophysin. These breast cancers are characterised for their excellent prognosis and conservative treatment is almost always enough to obtain patient cure.

[Abdominal compartment syndrome: survey on the awareness of Portuguese general surgeons]. / Síndrome de compartimento abdominal: questionário sobre a sensibilidade dos cirurgiões gerais Portugueses.

The Abdominal Compartment Syndrome (ACS) is a clinical entity recognized for over a century, but only recently its risk criteria, monitorization and treatment have been defined by the World Society of the Abdominal Compartment Syndrome (WSACS). The general surgeon's involvement is vital since this syndrome is common in surgical patients and because its treatment may culminate in a laparostomy. 250 questionnaires of 17 questions were distributed among general surgeons attending the XXVIII Portuguese Congress of Surgery. The data were analyzed using SPSS® v16. We received 36,4% (91) of the delivered questionnaires, most of which from male surgeons (63,7%), from central hospitals (75,8%), working 42 h per week (70.3%), whose average of age was 38 years. About half of the respondents received training in Intensive Care Units. All surgeons had already heard about measuring the Intra- abdominal Pressure (IAP), which was being performed at 89% of their hospitals. About 40% of surgeons only admitted intra-abdominal hypertension above 20 mmHg (only 22% indicated the correct value of 12 mmHg). 36,3% of surgeons suggested that a decompressive laparostomy must be carried out for primary ACS if IAP greater then 20 mmHg with new organ failure; 36.3% favoured the "Vacuum-pack"-like system, and 56% only re-operate the patients "as needed". 48,4% of surgeons had already performed decompressive laparostomy, 66% of which had residence training in a ICU (p = 0,005). Respondents also pointed an average mortality related to ACS of 81% without laparostomy, and a reduction to 38,5% after performing that procedure. Only 26% of the surgeons were aware of the WSACS consensus definitions and recommendations, of those, 83% had already performed a laparostomy (P<0,001). It can be concluded that, in spite of recognizing the ACS as a clinical entity, portuguese general surgeons are quite unaware of the WSACS definitions and treatment guidelines, urging the need for its divulgation.

Simultaneous gastric and colic laparoscopic adjustable gastric band migration. Complication of bariatric surgery.

Laparoscopic adjustable gastric banding (LAGB) is a minimally invasive treatment for morbid obesity, which has proved its safety, efficiency, and reversibility. Postoperative complications are rare and might be related with the reservoir, connecting tube, or with the band itself. The lack or unspecificity of clinical signs and symptoms makes the diagnosis of gastric band erosion difficult. The authors present the case of a 54-year-old female, submitted to laparoscopic adjustable gastric banding in April 2004 (BMI = 40 kg/m(2)). During the first year, she reduced her body mass index to 30 and remained stable thereafter. In August 2008, while investigating a moderate colicky abdominal pain, she was submitted to a colonoscopy that showed part of the band inside the transverse colon. Two exams were performed: the abdominal CT scan, which showed the connecting tube inside the transverse colon lumen and the lap band which was apparently well positioned around the stomach, and an upper digestive endoscopy that revealed band migration to the stomach lumen. The patient underwent laparoscopic band removal and closure of both stomach and colon walls, thus treating the fistula. LAGB erosion and migration is a late complication of this surgery that frequently needs surgical removal. LAGB migration to colon or stomach is described in literature isolated. Simultaneous erosion to stomach and colon lumen, with a gastrocolic fistula formation, has never been described before, making this case a unique one.

A rare cause of lower abdominal and pelvic mass, primary tuberculous psoas abscess: a case report.

INTRODUCTION: Tuberculous psoas abscess was usually associated to complicate Pott's disease, but it can also be secondary to direct extension from other adjacent structures or haematogenous spread from an occult source. However, the occurrence of this entity as the presenting manifestation of tuberculosis, without evidence of active infection elsewhere, has been seldom reported. CASE PRESENTATION: We report a clinical case of a 64-year-old immunocompetent female that presented with left lower abdominal pain and a soft tissue mass over the left iliac fossa and inguinal regions due to a primary tuberculous psoas abscess. Early diagnosis and prompt treatment with percutaneous drainage guided by ultrasound along with antituberculous drugs, lead to a satisfactory outcome. CONCLUSION: The purpose of this case report is to point out attention to the diagnostic challenge of tuberculous psoas abscess in the absence of tuberculosis in other organs or a predisposing condition. A brief review of the literature about its epidemiology, etiology, clinical features and management is discussed over the text.