Black cardiac paraganglioma in a multiple paraganglioma syndrome

Abstract

We describe a unique case of a patient with a 'pigmented' cardiac paraganglioma in a multiple paraganglioma syndrome. She was symptomatic for arrhythmias, hypertensive crises and dyspnoea due to a cardiac tumour, which was richly vascularised from the right coronary artery and was partially obstructing the right atrioventricular inflow. She was operated on, but the mass was not completely resectable due to its relationship with cardiac structures. The histological findings were paraganglioma with abundant, dark granular pigment. To our knowledge, pigment presence has never been cited in surgical cases of cardiac paragangliomas.

Cardiac Paragangliomas

“…SDHB mutation was reported in four patients (one had family history, one had local invasion, one suffered metastasis, and the other one had a non‐pheochromocytoma adrenal mass) . SDHC mutation was reported in two patients (no special clinical history), and SDHD deletion and mutation was found in two patients (one was black PGL, and the other one was accompanied with adrenal PGL) . RET (rearranged during transfection) gene and von Hippel‐Lindau gene abnormality was undetectable in all patients.…”

“…The tumor weight was documented in 14 patients in the range between 12.5 and 137 g with a mean value of 76.0 g. On microscopy, CPGLs showed the characteristics of common PGLs, composed mainly of so‐called chief cells, grouped together in cell clusters or organoid (“Zellbellen”) surrounded by a capillary network. Pigmented CPGLs were reported in three patients . It is well known that the reliable diagnostic criterion for malignancy is the presence of metastasis rather than histology features.…”

“…In such cases, it was difficult to clarify which one was the primary tumor. Familial PGLs history was recorded in four patients . The patients with multiple tumors or family histories were described in Table S1.…”

“…Familial PGLs history was recorded in four patients. 3,[5][6][7] The patients with multiple tumors or family histories were described in Table S1.…”

Pigmented paraganglioma of the kidney: a case report

“…They are located in the uterus, retroperitoneum, lumbar spine, urinary bladder, orbit, heart, and mediastinum [2,4-9]. To the best of our knowledge, none have been described in the kidney.…”