RESUMO
We present the case of multiple sclerosing pneumocytomas (SPs) associated with ACTH-secreting carcinoid tumorlets responsible for an ectopic Cushing syndrome (ECS). SP is a rare benign tumor originating from pulmonary epithelial cells. An 18-year-old male presented with shortness of breath and right-sided chest pain after exercise. Chest radiograph indicated right pneumothorax and bilateral lung nodules. CT imaging showed innumerable bilateral hypodense pulmonary nodules and a wedge resection gave the definitive diagnosis of SP with associated carcinoid tumorlets. Two years later, he presented with severe back pain in context of thoracic vertebral compression fracture. He had central fat accumulation, violaceous striae, proximal muscle weakness, hypertension, and diabetes. MRI of the pituitary gland showed a 7-mm adenoma. Inferior petrosal sinus sampling with no central-to-periphery gradient suggested an ectopic origin of ACTH, which was confirmed by ACTH expression in a subset of tumorlet cells in the lung lesions. The patient was started on ketoconazole and subsequently underwent a bilateral adrenalectomy. During follow-up, CT scans showed no growth of the lesions, except for the most recent CT scan, in which an increase in the size of the largest nodule was described. Ten years after the diagnosis, the patient remains asymptomatic of his pulmonary lesions. This article provides a case of ECS in the setting of multiple SP with associated carcinoid tumorlets.
RESUMO
Objective: Diabetes myonecrosis, also called diabetic muscle infarction (DMI), is a rare complication of diabetes. Given its rarity, our understanding of the underlying causes or the optimal management of DMI cases remains unclear. Methods: We report on a patient who experienced 2 episodes of DMI and we also review the literature. Results: A 46-year-old male with longstanding type 2 diabetes mellitus with multiple microvascular complications presented with acute-onset painful right thigh induration. On physical examination, he had right thigh swelling, tenderness, and crepitus. Blood tests showed leukocytosis, elevated creatine phosphokinase, and elevated acute-phase reactants. Microbiological cultures were negative. Glycated hemoglobin was 6.4% (46 mmol/mol). Magnetic resonance imaging demonstrated T2 hyperintensity involving the quadriceps group. The clinical and laboratory signs suggested a muscle infection. A muscle biopsy was suggestive of DMI. Eleven months later, the patient presented again with a 4-week history of left thigh pain and weakness in both legs. On examination, he had bilateral thigh anterior tenderness without evidence of swelling or induration. He also had marked bilateral proximal motor deficiency and inability to stand or ambulate. Despite a different clinical presentation, imaging features were consistent with DMI. The patient was managed with conservative therapy. His strength improved significantly after 3 months of follow up. Conclusion: The typical clinical presentation of DMI includes unilateral acute-onset pain in the quadriceps, local swelling, and the appearance of a palpable painful mass. The second episode in our patient illustrates an atypical clinical presentation of DMI and shows the importance of the correlation of clinical and imaging findings for the diagnosis of DMI.