Ectopic pheochromocytoma or paraganglioma of the ZUCKERKANDL organ: A case report and review of the literature

Abstract

Highlights EPIDEMIOLOGY : paraganglioma of the ZUCKERKANDL organ is a rare neuroendocrine tumour but must be known. DIAGNOSIS : The typical presentation which is the Menard Triad of symptoms, associating headaches, sweating and palpitation. Preoperative diagnosis can be difficult in pauci-symptomatic cases. The Clinical signs, Abdominal-pelvic CT scan and biology are the steps of the preoperative diagnosis. TREATMENT : Treatment is surgical. Preoperative medical preparation is aimed at reducing risks of peroperative hemodynamic instability. The anesthetist should therefore prepare himself to manage blood pressure peaks during the tumour’s dissection, but also the possible low blood pressure at the end of exeresis. Surgery remains the key element of treatment and consists in exeresis of the paragaglioma. PROGNOSIS : paraganglioma of the ZUCKERKANDL organ is often a benign tumor. The resuscitation determines the patient’s prognosis.

Ocular muscle metastasis as the initial presentation of a malignant pheochromocytoma: A unique case

“…In rare cases (as is discussed above), it can manifest without clinical symptoms and be detected initially as a metastatic tumor in an unusual site. [1][2][3][4] The behavior of the disease is highly variable, and the treatment should be individualized. Unfortunately, because of its rarity, there is not sufficient collective experience in the management of this condition.…”

“…[3][4][5][6] The most common metastatic sites of malignant pheochromocytoma are as follows: bone, intra-abdominal organs, lungs, and pleura. [5][6][7][8][9][10][11][12][13] Although skull metastasis is quite common, 2 there has never been a reported case of ocular…”

“…1,2 Malignant pheochromocytoma is defined as tumor cells at unusual sites, which usually do not harbor chromaffin cells. 2 The incidence rate of malignant pheochromocytoma is variable in different reports ranging from 8% to 12.5%. 3 The diagnosis of metastatic pheochromocytoma is difficult due to lack of definitive histological criteria.…”

“…Pheochromocytoma is a type of catecholamine‐releasing adrenal tumor that develops from chromaffin cells 1,2 . Malignant pheochromocytoma is defined as tumor cells at unusual sites, which usually do not harbor chromaffin cells 2 . The incidence rate of malignant pheochromocytoma is variable in different reports ranging from 8% to 12.5% 3 .…”

Metastatic pheochromocytoma to the pancreas diagnosed by endoscopic ultrasound‐guided fine needle aspiration: A case report and review of literature

“…5 Clinical signs and symptoms of functional malignant chromaffin cells are related to excess catecholamine release and may include the Menard triad of headaches, sweating, and palpitations. 6,7 In initially asymptomatic cases, symptoms may develop over time due to metastatic growth of the tumor. 8 Approximately 10% to 17% of pheochromocytomas are metastatic at initial presentation.…”