Giant Cystic Pheochromocytoma with Low Risk of Malignancy: A Case Report and Literature Review

Liu

et al. 2020

Preprint

Introduction: Pheochromocytoma (PCC) is a rare tumor which derives from adrenal medulla, when maximum diameter of pheochromocytoma is greater than 10CM, it is divided into Giant pheochromocytoma(GPCC), which is extremely rare and usually asymptomatic.Clinically, a huge and asymptomatic adrenal pheochromocytoma was usually misdiagnosed as other types of tumors, which result in notable increase of complication rates and death rates.Case presentation:In this case report, we described a clinically asymptomatic GPCC patient. Diagnosis:According to computed tomography (CT) scan, nuclear magnetic resonance(MR) scan, the patient is initially diagnosed as liposarcoma.After laparotomy, biochemical detection of catecholamine (CA) intermediate metabolites methoxyepinephrine (MN) and methoxy norepinephrine (NMN) and the pathological examination , diagnosis of GPCC was obtained. Intervention：The laparotomy was suspended and a diagnosis of pheochromocytoma was confirmed, because of unstable blood pressure and hypertension during separating and moving the mass during surgery. After consultation of multi-disciplinary team, adequate preoperative preparation was conducted according to the procedure of PCC surgical preparation. By the end of the regular phenoxybenzamine and intravenous fluids treatment for three weeks, the blood pressure of the patient was kept at an acceptable average. Therefore, the patient underwent the operationagain for radical resection. Outcome: After the operation, CA was basically normal in rechecking process, and the tumor was successfully removed. Pathological diagnosis of the mass after operation: Immunohistochemical resulted conformed to (epigastrium) pheochromocytoma. Immunohistochemistry: CgA(+), Inhibin-α(-), ki-67(<1%+), Syn(+). Pheochromocytoma had the definite pathological diagnosis. Conclusion: GPCC has the high diagnostic identification difficulty, it should be combined with imaging examination and biochemical measurement to identify. Before operation, the detailed imageological examination provides important reference for excision and surgical planning. Individualized and multi-disciplinary cooperation of management strategy in perioperative period should be recommended. Patients should conduct long-term follow-up.

Section: Discussionmentioning

confidence: 96%

Case report: A Re-operation Case of Asymptomatic Left Giant Pheochromocytoma and Literature Review

Liu

et al. 2020

Preprint

“…Surgery for the present case was considered challenging because the tumor was extremely large (>20 cm) and adjacent to surrounding organs including the liver, kidney, and inferior vena cava. The tumor reached a maximal diameter of 27 cm at the time of surgery, being one of the largest reported cases of pheochromocytomas . Before the surgery, we held a joint conference with related departments (e.g.…”

Section: Discussionmentioning

confidence: 99%

Acute hypotension induced by suction of cystic fluid containing extremely high concentrations of catecholamines during resection of giant pheochromocytoma

et al. 2019

Introduction: Since pheochromocytomas present with various complications due to catecholamine hypersecretion, their perioperative management needs special attention. Case presentation: A 45-year-old man visited our hospital with a complaint of abdominal swelling. Radiological and endocrinological assessments determined the tumor as a giant (>20 cm) cystic pheochromocytoma. After administration of doxazosin, the patient underwent radical surgery. Since the tumor was extremely large and fixed to surrounding structures, we punctured it and aspirated cystic fluid to improve the tumor's mobility. However, during the aspiration, the patient developed acute hypotension, which could be reversed by suction withdrawal and vasopressor administration. A similar event occurred during a second aspiration. Eventually, the tumor was successfully excised with negative surgical margin. The cystic fluid proved to contain extremely high concentrations of catecholamines, which might result in the hypotension. Conclusion: We report the first case who developed acute hypotension due to aspiration of cystic fluid from giant pheochromocytoma. Keynote messagePheochromocytomas present with various complications due to catecholamine hypersecretion, and thus their perioperative management needs special attention. We report a case who developed acute hypotension due to aspiration of tumor cystic fluid during resection of giant pheochromocytoma. The cystic fluid proved to contain extremely high concentrations of catecholamines, which might result in the hypotension. The present report would promote physicians' awareness of this possible intraoperative complication and lead to safer pheochromocytoma surgery.

“…A literature search yielded a case report by Maharaj et al which provided a summary of all giant pheochromocytomas (defined as tumors >10 cm) reported in the literature (9). The largest tumor reported by Maharaj et al was 45×20 cm originally described by Grissom et al in 1979 (10).…”

Section: Discussionmentioning

confidence: 99%

Surgical Management of a Giant Pheochromocytoma

Afaneh

Yang

Hamza

et al. 2018

Pheochromocytomas are rare tumors arising from chromaffin cells of the adrenal medulla and extramedullary sympathetic ganglia. The incidence of asymptomatic disease is rising due to increased detection rates from widespread use of computed tomography. We describe a case of one of the largest documented pheochromocytomas resected in the United States, an 18-cm tumor in a patient who presented with exertional dyspnea, abdominal pain, constipation, weight loss, and intermittent hypertension. After biochemical and appropriate imaging workup, the patient underwent an open resection of the mass.