Epithelioid angiomyolipomas (EAMLs) are mesenchymal tumors that are part of the family of the perivascular epithelioid cell neoplasms (PEComas). These tumors portray a potential aggressive behavior with metastatic lesions found in around 30% of reported cases. EAMLs might present sporadically or in association with the tuberous sclerosis complex (TSC). They typically involve the kidneys, liver, and lungs. It is extremely rare for these tumors to arise from other organs. The present report describes an unusual case of an adult patient with a history of TSC who developed EAML of the adrenal gland. Moreover, he presented with metastatic disease to the liver, a feature rarely described. The diagnosis of EAMLs can be challenging as they are hard to distinguish from other adrenal or renal tumors without a thorough histopathologic and immunohistochemical evaluation. Due to the potential aggressive behavior of these malignancies, timely diagnosis is extremely important and has significant therapeutic and prognostic implications.
The testicular seminomas are germ-cell tumors which account for approximately 50% of all testicular tumors. Most primary testicular germ cell tumors metastasize through a lymphatic system in a predictable pattern with the retroperitoneal lymph nodes being the most common initial metastatic site. Hematological metastasis to the distant organs is less common, and except for pulmonary metastasis, changes the classification from good to intermediate prognosis. Metastasis of testicular seminoma to the prostate is an extremely rare entity with only five reported cases in the literature. In this report, we present a 63-year-old male with recurrent testicular seminoma presenting in prostate.
The management of functional adrenocortical carcinoma (ACC) is challenging as it involves treating the underlying endocrinological abnormalities such as hypercortisolism. Competitive glucocorticoid receptor antagonist such as mifepristone (Korlym®) can be used to treat dysglycemia secondary to hypercortisolism. A 59-year-old male was admitted for dizziness and lightheadedness. Past medical history was significant for hypertension, and obesity. During evaluation, patient was found to have a random blood glucose of 663 mg/dL and a hemoglobin A1c of 14.4% with a previous of 5.8%. After admission, patient required 62.9 units of IV insulin over 9 hours to stabilize blood glucose. Due to the high insulin requirement and clinical presentation, a concern for hypercortisolism was considered and workup revealed an 8am cortisol level of 28.3 mcg/dL, and dexamethasone suppression test with cortisol of 50.4 mcg/dL, dexamethasone level 209 ng/dL and ACTH of <5 pg/mL. Patient was discharged on glargine 40 units SC BID and aspart 10 units SC TID AC. Seventeen days after discharge, patient was admitted with hyperglycemia (233 mg/dL), hypokalemia (2.5 mg/dL), abdominal pain, nausea, and vomiting. CT of abdomen revealed a 9.3 x 10.8 x 9 cm retroperitoneal mass arising from the left adrenal. Patient was started on mifepristone (Korlym®) 600mg PO daily while inpatient as his acute hyperglycemia was insufficiently controlled for consideration to open adrenalectomy. Within 24 hours of starting therapy, patient’s insulin requirement was de-escalated, and euglycemia was maintained for 20 days until surgery. Postoperative pathology demonstrated a 12.1cm resected tumor consistent with adrenocortical carcinoma. No clinical signs of adrenal insufficiency occurred postoperatively despite not stopping mifepristone (Korlym®) prior to surgery. Discussion: Adrenocortical carcinomas (ACC) are rare malignancies with an annual incidence of 0.7–2 cases per million population. They carry a poor prognosis with 5-year survival between 16–47%. The clinical presentation varies on the functional activity of these tumors and approximately 60% of them are cortisol secreting. The management of functioning-ACCs is challenging. Our patient presented with hyperglycemia secondary to hypercortisolism that was recurrent and difficult to control. He was started on mifepristone (Korlym®), a FDA approved potent competitive glucocorticoid receptor antagonist for the treatment of dysglycemia secondary to all forms of Cushing disease. In our patient, mifepristone provided a significant and rapid glycemic control, requiring to decrease the dose of insulin. Conclusion: This case demonstrates a safe and acute use of mifepristone (Korlym®) without initial titration in an inpatient monitored setting making this medication a viable option for management and optimization of hyperglycemia secondary to hypercortisolism. This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the authors and do not necessarily express the official views of the HCA Healthcare or any of its affiliated entities.
INTRODUCTION: Myelodysplastic syndromes (MDS) are a group of myeloid clonal disorders that have a heterogeneous spectrum of disease. The clinical presentation arises from cytopenias with potential to evolve into acute myeloid leukemia. Definitive cure may be attained via allogenic hematopoietic stem cell transplantation, however hypomethylating agents, such as Azacitadine, are commonly used as treatment. Common side effects include inhibition of hematopoietic cells, nausea, liver and kidney injury. Pan-serositis is a rare side effect and infrequently reported. Moreover, acute pericardial tamponade is a side effect not previously described in the literature. CASE PRESENTATION: A 58-year-old man with untreated high risk MDS presented with one week of worsening dyspnea. He was hemodynamically stable with an unremarkable physical exam. Laboratory testing revealed a hemoglobin of 6.2g/dl and symptoms were attributed to acute on chronic anemia. Computed tomography (CT) angiography of the chest showed normal perfusion, normal lung parenchyma and heart size with no pericardial effusion. Symptoms and anemia resolved with multiple transfusions of packed red blood cells. Bone marrow biopsy showed 10-15% myeloblasts with dysplastic changes, and he was started on cytotoxic therapy with Azacitidine as a bridge to potential transplant. On day five of treatment, he developed a dry cough and dyspnea that progressed over the next days. One day after finishing the 7-day course of Azacitatine, CT of the chest revealed a moderate to severe pericardial effusion with no right heart strain and large bilateral pleural effusions. Vital signs were notable for borderline hypotension with mild tachycardia. He had muffled heart sounds and diminished bilateral lower lobe breath sounds. Echocardiogram showed signs of impending cardiac tamponade. Emergency pericardial window with subsequent bilateral chest tube insertion was performed. Fluid studies showed no evidence of underlying malignant infiltration or infectious etiology. Further treatment with Azacitabine was held and symptoms resolved. DISCUSSION: Azacitidine causes demethylation of genes involved in cell-cycle regulation, apoptosis and cell adhesion, which are associated with disease progression in MDS. Pericardial effusions have rarely been reported as a side effect of this medication, and to our knowledge, there are no reports in the literature describing the development of acute pericardial tamponade. The Naranjo drug reaction probability scale was 6 indicating probable adverse reaction. Possible mechanisms suggest an underlying cascade of immune mediated delayed hypersensitivity caused by the chemotherapy. CONCLUSIONS: Further analysis is needed to determine the cardiotoxic side-effect profile of hypomethylating agents in order to improve awareness and therefore prompt recognition of a potentially life-threatening event such as acute pericardial tamponade.
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