Composite Pheochromocytoma/Paraganglioma: Case Report and Analysis of the Literature

Presentation Number: SUN 410
Date of Presentation: April 2nd, 2017

Alejandro Roman-Gonzalez*1, Joanna Marquez2, Juan Pablo Dueñas3, Gabriel Varela3, Camilo Jimenez4 and Johnayro Gutierrez5
1Hospital Uiversitario San Vicente Fundacion-Universidad de Antioquia, Medellin, Colombia, 2Universidad de Antioquia, Colombia, 3Hospital Pablo Tobon Uribe, Colombia, 4The University of Texas MD Anderson Cancer Center, Houston, TX, 5Hospital Pablo Tobon Uribe, Medellin Antioqua, Colombia

Abstract

Introduction: Composite pheochromocytomas (Pheos) and paragangliomas (PGs) represent less than 3% of all Pheos/PGs. These tumors are a mixture between a Pheo/PG and another neural crest derived tumor such as a ganglioneuroma (GN), ganglioneuroblastoma (GNB), neuroblastoma (NB) or malignant peripheral nerve sheath tumor (MPNST).

Methods: We report a 57-year-old woman with paroxysms of headache, palpitations and hypertension with elevated plasma metanephrines. Abdominal MRI showed a heterogeneous 12 cm right adrenal mass encasing the inferior vena cava. The patient underwent an open adrenalectomy; pathology report confirmed a composite Pheo/GN. Also, all reported cases were searched. PubMed, LILACs and EMBASE databases were searched for the terms "composite or mixed Pheo/PG". 83 references were obtained and data analysis was made using SPSS 22.

Results: 113 patients were found with a composite tumor. Mean age was 48±18 years, 61(54%) were women. The median size of the primary tumor was 6.06 cm (range 0.6-27cms). 21.23% of patients had a composite tumor in the context of a genetic syndrome: 17 had neurofibromatosis type 1 (NF1), 3 had multiple endocrine neoplasia type 2A (MEN2A) and one patient had MEN2B 1, 2 had Von Hippel-Lindau disease (VHL), and 1 patient had a paraganglioma syndrome type 4. Composite Pheo frequencies were: Pheo/GN 53.1%, Pheo/GNB 10.6%, Pheo/NB 7.1%, Pheo/MPNST 1.8%, Pheo/GN/adrenal adenoma 1.8%, Pheo/Neuroendocrine adrenal carcinoma 1.14%, Pheo/GN/NB 1.14%, Pheo/MPNST/rhabdomyosarcoma 1.14% and Pheo/GNB/squamous cell carcinoma 1.14%. PGs were: PG/GN 76.92%, PG/NB 11.5%, PG/GNB 7.69%. The most frequent clinical presentations included: incidentaloma 32.6%, adrenergic symptoms 31.6%, and abdominal mass/pain 21%. Metastatic disease was found in 11.5%. There was no association between malignancy and tumor size or genetic syndrome. Median follow-up was 22 months (0-336). 24.32% died because of disease. We found that composite Pheo/Pg are more common in women. Tumor size was variable (0.6-27cms). NF-1 was the most frequent associated genetic syndrome. The frequency of incidentaloma (31%) was similar to other cohorts of Pheo/PG.

Conclusions: Composite Pheo/PG are a heterogeneous group of tumors arising from the paraganglia with diverse clinical presentation.

 

Nothing to Disclose: AR, JM, JPD, GV, CJ, JG