Ectopic Cushing's Syndrome in Pediatric Age.

BACKGROUND: Cushing's syndrome due to ectopic ACTH secretion is a rare clinical condition resulting from a dysregulated ACTH secretion by neuroendocrine tumors, which can have various localizations and different histological differentiations. The overall incidence of endogenous Cushing's syndrome is 0.7-2.4 per million people per year. Children account for just 10% of all new cases that are reported each year. CASE REPORT: When the patient first presented clinically, she was a 17-year-old girl who displayed symptoms of schizophrenia (delirium, psychotic episodes, and hallucinations). Blood tests showed diabetes mellitus and hypokalemia. She was also affected by high blood pressure and osteoporosis complicated by D9-D10 and L1-L5 vertebral collapses. For these reasons, she was treated with aripiprazole, insulin glargine, potassium chloride, spironolactone, enalapril, and calcium carbonate. After two months of treatment, she was referred to the pediatrician endocrinologist, who diagnosed hypercortisolism after prescribing hormone tests (Table 1). After receiving her diagnosis, she began taking 1000 mg of metyrapone and had a whole-body CT scan, which revealed bilateral adrenal hyperplasia. The results of the 68Ga-PET/DOTATOC and 18FDG-PET scans were negative. The clinical course was intermittent in the months that followed, with hypercortisolism and eucortisolism alternating. After one year of treatment, a 68Ga-PET/DOTATOC showed a nodule in the thymic lodge (Fig. 1). The patient underwent a thymectomy. Unfortunately, after surgery, she continued to have high levels of cortisol, for which she continued metyrapone 750 mg/die. 68Ga-PET/DOTATOC repeated three months after surgery showed again an uptake corresponding to the thymic lodge (Fig. 2). In order to remove the neuroendocrine lesion, she had a new surgery, which resulting a finally resolutive. ACTH levels were monitored before, during, and post-surgery (Table 2). The laboratory provided the ACTH results very quickly and thoracic surgeons waited for hormonal results before concluding the procedure. The adopted strategy permitted us to monitor the outcome of the surgery. CONCLUSION: The heterogeneity of ectopic Cushing's syndrome makes diagnosis difficult. Treatment of ectopic Cushing's syndrome requires close clinical, biochemical, and instrumental observation. Metyrapone is a drug able to control hypercortisolism in a lasting way with a good level of safety. 68Ga-PET/DOTATOC proves to be a tracer with good sensitivity and specificity for the identification of ACTH-secreting neuroendocrine lesions. The short half-life of ACTH is found to be a strategy to monitor the complete surgical resection of the neuroendocrine lesion. A multidisciplinary approach improves therapeutic success and reduces the risk of recurrence.

Bilateral adrenal hemorrhage: learning notes from clinical practice and literature review.

Adrenal hemorrhage is a rare, but important, diagnosis to recognize, in particular when there is involvement of both adrenal glands. Bilateral adrenal hemorrhage can in fact lead to adrenal insufficiency, with dramatic consequences if not promptly recognized and treated. It is normally caused by systemic conditions that lead to the vasoconstriction and thrombosis of the adrenal vein. Oftentimes, the clinical diagnosis of this condition can be very challenging, as its signs and symptoms are generalized and nonspecific (abdominal pain, nausea, and fatigue). Here, we present the cases of two patients admitted to the Emergency Department in 2016 and 2022 with acute abdominal pain, having recently undergone surgery and subsequently prescribed low-molecular-weight heparin. In both cases, laboratory results revealed neutrophilic leukocytosis and an unexplained anemia. Due to the persistence of abdominal pain despite medication, a CT scan was performed, showing an enlargement of both adrenal glands suggestive of bilateral adrenal hemorrhage. Adrenal function was tested that correlated with a diagnosis of adrenal insufficiency, and both patients were promptly treated with parenteral hydrocortisone as a result. On 5 years' follow-up from the acute event, the second patient's adrenal function had returned to normal, and he has not needed further adrenal replacement therapy; the first patient however demonstrated persistence of adrenal failure requiring replacement therapy. In this paper, through our experience and a literature analysis, we will aim to outline some clues to identify patients at potential risk of bilateral adrenal hemorrhage.

Condrossarcoma em paciente com osteocondromatose múltipla: relato de caso e revisão da literatura / Chondrosarcoma in a patient with multiple osteochondromatosis: a case report and review of the literature

Os autores relatam um caso de condrossarcoma em uma paciente de 14 anos portadora de osteocondromatose múltipla, que procurou assistência médica com a queixa de tumoração na coxa esquerda. Cortes tomográficos do membro afetado evidenciaram lesão osteodestrutiva no terço superior do fêmur esquerdo. O diagnóstico foi feito através da revisão da lâmina do serviço de origem da paciente, confirmando o achado de condrossarcoma grau I em lesão condromatosa. Foi realizada desarticulação do colo femoral devido ao extenso comprometimento de grupos musculares, de tecido celular subcutâneo e de pele. A literatura mostra ampla variação nas taxas de transformação maligna das exostoses em pacientes portadores desta doença, sendo esta uma importante complicação nestes pacientes.

The authors report a case of chondrosarcoma in a 14-year-old female patient with multiple osteochondromatosis who has sought medical assistance complaining of a tumor on the left coxa. CT images of the affected limb have demonstrated osteodestructive lesion on the superior third of the left femur. The diagnosis has been made through a review of the biopsy slide from the service of origin of the patient, confirming the finding of a grade I chondrosarcoma in a chondromatous lesion. Disarticulation of the femoral neck was made because of the large extent of the lesion in muscular groups, subcutaneous cellular tissue and skin. The literature shows a wide variation in rates of malignant transformation of exostosis in patients with this disease, and this is a significant complication in patients affected by this disease.

Violência e saúde: uma perspectiva psicanalítica / Violence and health: a psychoanalytical perspective

O artigo investiga o fenômeno da violência em suas manifestações individuais e coletivas e sua relação com as características constitutivas do psiquismo humano. Uma vez destacados os funcionamentos psíquicos e ideológicos que levam ao ocultamento da violência, o autor analisa como o fenômeno é abordado por Freud na carta dirigida a Einstein. Nela, Freud analisa a inevitabilidade da violência, uma vez que se estabelece o estado de direito a partir da estrutura psíquica que envolve os instintos de vida e de morte. A teoria psicanalítica sobre a agressividade é aprofundada com as constribuições de Melaine Klein e Donald W. Winnicot, que introduzem algumas mudanças significativas de perspectiva, apontando para a importância da agressividade no desenvolvimento saudável do psiquismo humano.

Avaliação por tomografia computadorizada do envolvimento loco-regional do carcinoma espinocelular de corda vocal / Computed tomography evaluation of loco-regional involvement of squamous cell carcinoma of the vocal cord

No período de 1992 a 1998, foram avaliados, retrospectivamente, exames de tomografia computadorizada e prontuários de 22 pacientes com carcinoma espinocelular de corda vocal. Avaliou-se a concordância entre observadores para todos os casos e a acurácia e concordância entre os métodos para os casos operados, utilizando-se o índice kappa. A concordância foi excelente para o comprometimento tumoral das cartilagens tireóide, cricóide, extensão extralaríngea e estadiamento linfonodal; ótima para o envolvimento tumoral das cordas vocais, comissura posterior e espaço paraglótico; boa para o envolvimento tumoral da supraglote, subglote e estadiamento tumoral; regular para o envolvimento tumoral da comissura anterior e cartilagem aritenóide. A utilização simultânea da avaliação clínica e tomográfica para o estadiamento T obteve acurácia e concordância com achados patológicos de 89,47 por cento e 84,9 por cento, respectivamente, sendo superior à análise clínica isolada ou tomográfica. A acurácia e concordância patológica da tomografia computadorizada para o estadiamento N foi de 100 por cento, sendo superior à avaliação clínica

We reviewed the case notes and computed tomography scans of 22 patients with squamous cell carcinoma of the vocal cord, in the period between 1992 and 1998. Interobserver concordance for all cases and accuracy and concordance between the methods for the operated patients were evaluated using the kappa test. Concordance was classified as follows: excellent for evaluating tumoral involvement of the thyroid and cricoid cartilages, extralaryngeal extension and lymph node staging; very good for tumoral involvement of vocal cords, posterior commissure and paraglottic space; good for tumoral involvement of the supraglottic and subglottic regions and tumoral staging; regular for tumoral involvement of the anterior commissure and arytenoid cartilage. The combination of clinical and computed tomography evaluation for T staging resulted in an accuracy and concordance with the pathological results of 89.47% and 84.9%, respectively, which were superior to isolated clinical or computed tomography analysis. The accuracy and pathological concordance of computed tomography for N staging was 100%, which was superior to clinical evaluation.