Sipple’s Syndrome with a Malignant Pheochromocytoma Presenting as a Pericardial Effusion

Abstract

Sipple’s syndrome is a multiple endocrinopathy usually involving the adrenal medulla and the thyroid. In the present case, a malignant pheochromocytoma metastasized to the pericardium and both lungs. The patient was known to have had a calcified right suprarenal mass for 8 years. He was admitted to the emergency room with symptomatology consistent with pericardial tamponade, but despite intensive management, he expired.

Polyserositis: An Extremely Rare Life-Threatening Manifestation of Pheochromocytoma

“…Case Reports in Endocrinology with shortness of breath with demonstration of metastasis to pericardium in a histopathologic sample [6]. In our case, however, catecholamine-induced pericardial and pleural effusions were the first manifestation leading to further evaluation and prompt diagnosis of pheochromocytoma.…”

“…e pericardium is a rare site for primary tumors or the metastasis of malignant neoplasms. Few studies revealed that pericardial effusion is a very rare manifestation of pheochromocytoma [4][5][6]. It is postulated that the elevated levels of catecholamines increase the level of inflammatory factors leading to inflammatory symptoms such as fever, weight loss, and polyserositis [7][8][9][10][11].…”

Metastatic cluster 2-related pheochromocytoma/paraganglioma: a single-center experience and systematic review

“…We found 29 cases of MEN2 with metastatic PCC on literature search. The details of these cases, including our two cases, are summarized in Table 2 ( 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 ).…”

A Case of Sipple's Syndrome with Malignant Pheochromocvtoma Treated with 131I-Metaiodobenzyl Guanidine and a Combined Chemotherapy with Cyclophosphamide, Vincristine and Dacarbazine.

“…PHE in this syndrome is benign in most cases and metastatic masses are usually from the MTC. However, some case reports have described the coexistence of malignant PHE and MTC [2][3][4][5]. Recently, Sisson et al [6] have reported 5 cases of malignant PHE in which metastases were treated with a large dose of 1311-metaiodobenzyl guanidine (1311-MIBG), a structural analogue of guanethidine which is taken up by the sympathetic nerve terminals through the same pathway as norepinephrine [7].…”

“…The autopsy revealed massive invasion of PHE in the lung, liver, sacral region, right diaphragm and rectouterine pouch, all of which were histologically confirmed as The patient described here was an unusual case of Sipple's syndrome with malignant PHE, who was treated twice with large doses of 131I-MIBG and three cycles of combined chemotherapy. Although some groups have reported cases with malignant PHE and this syndrome [2][3][4][5], the majority of PHE in Sipple's syndrome is benign [19], and one might expect that the metastatic masses found in this syndrome originate from the MTC. However, Sisson et al…”