A case of malignant pheochromocytoma of the adrenal gland in a young female

Background Pheochromocytoma is a tumour of the adrenal medulla, derived from catecholamine producing chromaffin cells. Malignant pheochromocytomas constitute 10–25% of all cases. These are difficult to diagnose microscopically. Therefore, malignant pheochromocytomas are diagnosed by the presence of local invasion or metastatic disease. Case presentation We present a case of malignant Pheochromocytoma in a 20-year-old woman from south India with classic symptoms whose urinary metanephrines levels were elevated. After controlling the blood pressure preoperatively and laparoscopic right-sided adrenalectomy was performed. The Postoperative period was uneventful. Histopathology proved to be malignant pheochromocytoma with a PASS score of 16/20 and immunohistochemical staining was positive. DOTATATE PET/CT showed no evidence of disease anywhere else in the body. Conclusion Malignant pheochromocytomas are rare tumor, so they pose a significant diagnostic and therapeutic challenge. Surgery is the mainstay of treatment. DOTATATE PET/CT helps in the localization of metastatic disease.

18F-FDG PET/CT in a Patient With Malignant Pheochromocytoma Recurrence and Bone Metastasis After Operation—Case Report and Review of the Literature

Introduction: Bone metastasis of malignant pheochromocytoma is a rare disease. We report a patient with a 10-year history who underwent 18F-FDG PET/CT to detect bone metastasis and receive radiotherapy and chemotherapy with complete response for bilateral iliac pain.Case presentation: A 48-year-old male patient complained of dizziness, hypertension, and bilateral iliac pain for 2 months. The patient had a history of resection of bilateral malignant adrenal pheochromocytoma 10 years earlier, and all complaints were relieved immediately after operation. 18F-FDGPET/CT showed abdominal lymph node uptake and multiple bone uptake, as well as multiple brown fat uptake. A biopsy of the left ilium confirms the metastasis of malignant pheochromocytoma.Discussion: In our literature review, we discuss the metastasis of pheochromocytoma reported by some scholars, and the role of radionuclides such as 18F-FDG PET/CT, 18F-DOPA PET/CT, I-123MIBG, and 68Ga-DOTATATE PET, in the diagnosis of malignant pheochromocytoma. The patient above is a good case for clinicians in the diagnosis and treatment of metastatic pheochromocytoma, especially in some hospitals with only 18F-FDG imaging agents.Conclusion: A review of this case and similar rare cases in the literature illustrates the importance of 18F-FDG PET/CT in the diagnosis of malignant pheochromocytoma.

Case Report: Malignant Pheochromocytoma Without Hypertension Accompanied by Increment of Serum VEGF Level and Catecholamine Cardiomyopathy

IntroductionPheochromocytoma is a catecholamine-producing tumor in the adrenal medulla and is often accompanied by hypertension, hyperglycemia, hypermetabolism, headache, and hyperhidrosis, and it is classified as benign and malignant pheochromocytoma. In addition, persistent hypertension is often observed in subjects with malignant pheochromocytoma.Case PresentationA 52-year-old Japanese male was referred and hospitalized in our institution. He had a health check every year and no abnormalities had been pointed out. In addition, he had no past history of hypertension. In endocrinology markers, noradrenaline level was as high as 7,693 pg/ml, whereas adrenaline level was within normal range. Abdominal contrast-enhanced computed tomography revealed a 50-mm hyper-vascularized tumor with calcification in the right adrenal gland and multiple hyper-vascularized tumors in the liver. In 131I MIBG scintigraphy, there was high accumulation in the right adrenal gland and multiple accumulation in the liver and bone. In echocardiography, left ventricular ejection fraction was as low as 14.3%. In coronary angiography, however, there was no significant stenosis in the coronary arteries. Based on these findings, we finally diagnosed him as malignant pheochromocytoma accompanied by multiple liver and bone metastases and catecholamine cardiomyopathy. However, blood pressure was continuously within normal range without any anti-hypertensive drugs. Right adrenal tumor resection was performed together with left hepatic lobectomy and cholecystectomy. Furthermore, serum levels of vascular endothelial growth factor (VEGF) and parathyroid (PTH)-related protein were very high before the operation but they were markedly reduced after the operation.ConclusionsThis is the first report showing the time course of serum VEGF level in a subject with malignant pheochromocytoma, clearly showing that malignant pheochromocytoma actually secreted VEGF. In addition, this case report clearly shows that we should bear in mind once again that malignant pheochromocytoma is not necessarily accompanied by hypertension.

Prognostic Nomograms for Predicting Overall Survival and Cancer‐Specific Survival of Patients With Malignant Pheochromocytoma and Paraganglioma

BackgroundMalignant pheochromocytoma and paraganglioma (PPGL) are rare tumors with few prognostic tools. This study aimed to construct nomograms for predicting 3- and 5-year survival for patients with malignant PPGL.MethodsThe patient data was retrieved from the Surveillance, Epidemiology, and End Results (SEER) database. A total of 764 patients diagnosed with malignant PPGL from 1975 to 2016 were included in this study. The patients were randomly divided into two cohorts; the training cohort (n = 536) and the validation cohort (n = 228). Univariate analysis, Lasso regression, and multivariate Cox analysis were used to identify independent prognostic factors, which were then utilized to construct survival nomograms. The nomograms were used to predict 3- and 5-year overall survival (OS) and cancer-specific survival (CSS) for patients with malignant PPGL. The prediction accuracy of the nomogram was assessed using the concordance index (C-index), receiver operating characteristic (ROC) curves and calibration curves. Decision curve analysis (DCAs) was used to evaluate the performance of survival models.ResultsAge, gender, tumor type, tumor stage, or surgery were independent prognostic factors for OS in patients with malignant PPGL, while age, tumor stage, or surgery were independent prognostic factors for CSS (P <.05). Based on these factors, we successfully constructed the OS and CSS nomograms. The C-indexes were 0.747 and 0.742 for the OS and CSS nomograms, respectively. In addition, both the calibration curves and ROC curves for the model exhibited reliable performance.ConclusionWe successfully constructed nomograms for predicting the OS and CSS of patients with malignant PPGL. The nomograms could inform personalized clinical management of the patients.

Malignant pheochromocytoma: pain, palpitation, perspiration and perplexities

A 60-year-old man presented with headache, giddiness, abdominal pain and palpitation. When evaluated outside for the same, the patient was diagnosed to have hypertension and started on antihypertensives for which he did not respond. ECG was suggestive of non-ST elevation myocardial infarction. The patient was subjected to a coronary angiogram, which was normal. Patient had multiple episodes of fluctuating blood pressures. CT of the abdomen showed a 7.1×5.6×8.2 cm mass in the left adrenal gland suggestive of a pheochromocytoma. Serum, urine metanephrines and normetanephrines were elevated. After discussing with the multidisciplinary team, the patient was stabilised with alpha blockers and taken up for laparoscopic left adrenalectomy. Histopathology was reported as pheochromocytoma with a Pheochromocytoma Adrenal Scaled Score of 10/20 suggestive of malignancy. This is one such case of a malignant pheochromocytoma, which was managed successfully despite the perplexities faced in stabilising the crisis followed by laparoscopic resection in a moribund patient.