Bulky malignant peripheral nerve sheath tumour of the left thigh in a pregnant woman presenting with a pathological fracture of the proximal femur.

Malignant peripheral nerve sheath tumour (MPNST) is an aggressive soft tissue sarcoma with a poor prognosis, affecting most commonly the extremities. The lungs constitute the most frequent location for distant metastases. Half of all MPNSTs arise in patients with neurofibromatosis type 1, while approximately 10% are radiation induced and the rest are sporadic.The authors present a pregnant woman in her 40s with a sporadic MPNST of the lower limb and with lung metastases at diagnosis. Treatment consisted of interilioabdominal amputation, followed by adjuvant chemotherapy. Partial response and disease stabilisation were achieved with chemotherapy.Surgical resection with negative margins is the only potentially curative therapy, while radiation therapy and chemotherapy might be useful in the neoadjuvant or adjuvant setting, but their advantage in survival is not demonstrated. In the reported case, chemotherapy permitted the achievement of partial response and stabilisation of the disease.

The skin as a window to the gut: A case of carcinoid syndrome.

Neuroendocrine tumors (NETs) are a group of uncommon neoplasms derived from enterochromaffin or Kulchitsky cells (that secrete serotonin or other molecules into the bloodstream), which can manifest with symptoms of hormonal overproduction, namely carcinoid syndrome (CS). This can be the presenting feature in patients with advanced disease. We report the case of a 66-year-old woman presenting with chronic diarrhea, facial venous telangiectasia and elevated urinary 5-hydrocyindoleacetic acid levels. A 68-Ga DOTATOC PET/CT scan revealed an ileal mass and lesions consistent with liver, ovary and bone metastasis. A liver biopsy confirmed the diagnosis of well-differentiated NET G1. Therapy with somatostatin analogs achieved symptom control, but the liver disease progressed and the patient passed away after 2 years of follow-up. The challenge of diagnosing CS resides in its heterogeneous manifestations, which may range from mild to life-threatening conditions. In this case, the cutaneous findings of venous telangiectasia strongly pointed to the correct diagnosis. Treatment can also be difficult due to refractory symptoms and inevitable progression of disease, highlighting the importance of early detection and thorough disease staging.

Basaloid Follicular Hamartoma of the Eyelid: A Case Report and Literature Review about an Unusual Lesion in the Ocular Region.

Basaloid follicular hamartoma (BFH) is a normally benign, uncommon, malformative lesion involving the hair follicles, which usually poses challenges in the differential diagnosis with other benign and malignant tumours, especially basal cell carcinoma, due to significant clinical and morphological overlap. Here, we report the case of a 53-year-old male who presented with a mass in the upper left eyelid evolving for one year. The patient had a previous history of total colectomy and an abdominal desmoid tumour within the context of Familial Adenomatous Polyposis (FAP), with a documented germline mutation in the Adenomatous Polyposis Coli (APC) gene. The eyelid lesion was biopsied and the histological analysis of the three small tissue fragments received revealed fragments with cutaneous-conjunctival lining displaying a subepithelial proliferation of basaloid nests with peripheral palisading, compatible with primitive hair follicles. There were images of anastomosis between different basaloid nests, which had their connection to the epithelial lining preserved. The stroma had high cellularity and sometimes primitive mesenchymal papillae were evident. Pleomorphism was absent, mitotic figures were barely identified, and no necrosis was seen. The basaloid nests did not have epithelial-stromal retraction nor mucin deposits. A diagnosis of BFH was proposed, which was later confirmed after surgical excision of the whole eyelid lesion. No evidence of carcinoma was present. This case illustrates the main features of the rare benign eyelid BFH. The standard medical or surgical approach of these lesions remains to be firmly established. Nearly nine months after surgical excision our patient remains well without signs of disease recurrence.

Generalised papular variant of elastolytic giant cell granuloma.

Elastolytic giant cell granuloma (EGCG), also known as actinic granuloma, is an uncommon granulomatous dermatosis usually characterised by asymptomatic annular plaques on sun-exposed skin. Its aetiology is not fully elucidated, but actinic damage has been considered the main causal factor. Atypical variants with lesions in a non-photodistributed pattern are rare and often related to a systemic disorder, suggesting a more complex pathogenesis and demanding for a screening work-up. Herein, we report a case of an adult obese, diabetic woman presenting with a generalised pruritic papular eruption, histologically revealing an elastolytic giant cell granuloma, with a good response to treatment. In this case, the dermatosis was probably associated with her metabolic comorbidities.

Solidão, depressão e utilização das TIC em idosos portugueses / Loneliness, Depression, and ICT use in Portuguese Elderly / Soledad, depresión y uso de las TIC en adultos mayores portugueses

Resumo Contexto: A população idosa enfrenta dificuldades crescentes decorrentes de solidão, depressão e isolamento social. A literatura tem evidenciado o potencial da utilização das TIC para atenuar o sofrimento psicológico e melhorar a qualidade de vida. Objetivos: O presente estudo pretendeu analisar as relações entre sentimentos de solidão, sintomatologia depressiva e impacto da utilização e atitude face às TIC, em idosos. Metodologia: Trata-se de um estudo transversal que incluiu 669 sujeitos, com idades entre os 60 e 94 anos. Através de instrumentos de autorresposta avaliou-se a presença de sentimentos de solidão (UCLA-LS), sintomatologia depressiva (GDS-15) e a utilização e atitude face às TIC. Resultados: As mulheres evidenciaram níveis mais elevados de sintomatologia depressiva e sentimentos de solidão. Os idosos com mais de 80 anos relataram maiores níveis de solidão (na subescala Afinidades) e a faixa etária entre os 60-69 anos apresentou uma maior taxa de utilização das TIC. A presença de sentimentos de solidão relacionou-se com maiores níveis de sintomatologia depressiva e a utilização das TIC associou-se com menos sintomatologia depressiva. Conclusões: O envelhecimento pode ser vivido com menos sintomatologia depressiva se os idosos se sentirem menos isolados. A utilização das TIC, como ferramenta de comunicação, pode ter um impacto positivo no seu bem-estar psicológico, pois associa-se a menores níveis de depressão.

Abstract Context: The elderly population faces increasing difficulties arising from loneliness, depression and social isolation. The literature has shown the potential of using ICT to alleviate psychological distress and improve quality of life. Objective: To analyze the relationships between feelings of loneliness, depressive symptoms, and the impact of the use of and attitude towards ICT in the elderly. Methodology: This is a cross-sectional study which included 669 subjects aged between 60 and 94 years. Through self-report instruments, feelings of loneliness (UCLA-LS), depressive symptomatology (GDS-15) and the use of and attitude towards ICT were assessed. Results: Women evidenced higher levels of depressive symptomatology and feelings of loneliness. The elderly aged over 80 years reported higher levels of loneliness (in the UCLA-LS Affinities subscale) and the 60-69 age group showed the highest rate of ICT use. The presence of feelings of loneliness was associated with higher levels of depressive symptoms, and ICT use was associated with fewer depressive symptoms. Conclusions: Aging can be lived with less depressive symptomatology if the elderly feels less isolated. The use of ICT, as a communication tool, may have a positive impact on their psychological well-being, as it is associated with lower levels of depression.

Resumen Contexto: La población anciana se enfrenta a crecientes dificultades derivadas de la soledad, la depresión y el aislamiento social. La literatura ha demostrado el potencial del uso de las TIC para aliviar el malestar psicológico y mejorar la calidad de vida. Objetivos: Analizar las relaciones entre los sentimientos de soledad, la sintomatología depresiva y el impacto del uso y la actitud hacia las TIC en las personas mayores. Metodología: Se trata de un estudio transversal en el que participaron 669 sujetos con edades comprendidas entre los 60 y los 94 años. Mediante instrumentos de autoinforme se evaluó la presencia de sentimientos de soledad (UCLA-LS), la sintomatología depresiva (GDS-15) y el uso y la actitud hacia las TIC. Resultados: Las mujeres mostraron mayores niveles de sintomatología depresiva y sentimientos de soledad. Los mayores de 80 años fueron los que declararon niveles más altos de soledad (en la subescala de Afinidades) y el grupo de edad de 60-69 años fue el que más utilizó las TIC. La presencia de sentimientos de soledad se asoció con niveles más altos de síntomas depresivos y el uso de las TIC se asoció con menos síntomas depresivos. Conclusiones: Se puede vivir el envejecimiento con menos sintomatología depresiva si las personas mayores se sienten menos aisladas. El uso de las TIC, como herramienta de comunicación, puede tener un impacto positivo en su bienestar psicológico, ya que se asocia a niveles más bajos de depresión.