Although the presentation of pheochromocytoma is remarkably protean, it usually presents as sustained or paroxysmal hypertension with triad of headache, palpitation and diaphoresis. The Incidence of serendipitously discovered pheochromocytoma is approximately 4% to 8%. Perioperative management of such cases are not only controversial but also poses infallible challenges to the surgeon. This case report intends to highlight an unusual presentation of asymptomatic, normotensive pheochromocytoma as a huge abdominal mass and its management.
A 49-year-old man presented in the emergency department with altered sensorium and renal failure and was placed on a ventilator. Evaluation with MRI of the brain showed enhancing lesion in the occipital lobe. Biochemical tests revealed elevated calcium and parathyroid hormone (PTH) levels. Further evaluation revealed multiple lesions at both lobes of the lungs and nodular lesion in the right inferior pole of the thyroid. Diagnosis of metastatic parathyroid carcinoma was suspected. Patient was stabilised with bisphosphonates and haemodialysis and planned for surgery. Intraoperatively a hard lesion was found in the right inferior parathyroid infiltrating the surrounding structures. En bloc resection was done. General condition of the patient improved and he was weaned from the ventilator. Histopathology confirmed the diagnosis of parathyroid carcinoma. Postoperatively, there was persistent elevation of PTH in spite of a fall in calcium levels indicating functioning brain and pulmonary metastases. Due to extensive metastases, the patient was given palliative cinacalcet and was kept under follow-up.
Objective: A rare disease. Background: von Hippel-Lindau (VHL) disease is an autosomal dominant familial syndrome with a multitude of benign and malignant multivisceral tumors and one-third of them harbor pheochromocytoma (PCC). We intend to present one such rare case and challenges in its surgical management. Case: A 38-year-old lady presented with hypertension and episodic headache. Biochemical evaluation was suggestive of hypercatecholaminism. Magnetic resonance imaging (MRI) showed the presence of a heterogeneous intensity lesion in the left suprarenal area suggestive of PCC. Multiple cysts were seen in both the kidneys as well as in the pancreatic head and tail. Imaging of the brain and spine showed a left cerebellar and spinal cord hemangioblastoma, respectively. I-131 metaiodobenzylguanidine (MIBG) showed high uptake in the left suprarenal region. After adequate preparation, she underwent left adrenalectomy. Histopathology was consistent with adrenal PCC and the patient was biochemically cured of hypercatecholaminism. The presence of PCC and other manifestations of VHL type 2A were evident in this patient. Conclusion:The presence of multiple renal and pancreatic cysts can camouflage adrenal tumors intraoperatively. Careful identification, dissection, and differentiation of PCC from these cysts are crucial. Multicentricity and multifocality of PCC are not uncommon in VHL and these patients need lifelong close follow-up for other tumors as well.
Introduction: Papillary thyroid cancers that are less than 10mm are diagnosed as papillary thyroid microcarcinomas. There is a rising incidence of incidental microcarcinomas and the consensus regarding management and follow up is still evolving.
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