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. 2016 Nov;5(6):89-97.
doi: 10.1530/EC-16-0086. Epub 2016 Nov 16.

Predictors of malignancy in patients with pheochromocytomas/paragangliomas: Asian Indian experience

Kranti Khadilkar  1 , Vijaya Sarathi  2 , Rajeev Kasaliwal  3 , Reshma Pandit  4 , Manjunath Goroshi  4 , Gaurav Malhotra  5 , Abhay Dalvi  6 , Ganesh Bakshi  7 , Anil Bhansali  8 , Rajesh Rajput  9 , Vyankatesh Shivane  4 , Anurag Lila  4 , Tushar Bandgar  4 , Nalini S Shah  4
Affiliations

Predictors of malignancy in patients with pheochromocytomas/paragangliomas: Asian Indian experience

Kranti Khadilkar et al. Endocr Connect. 2016 Nov.

Abstract

Background and aims: Malignant transformation of pheochromocytomas/paragangliomas (PCC/PGL) is a rare occurrence, and predictive factors for the same are not well understood. This study aims to identify the predictors of malignancy in patients with PCC/PGL.

Materials and methods: We performed a retrospective analysis of 142 patients with either PCC or PGL registered at our institute between 2000 and 2015. Records were evaluated for clinical parameters like age, gender, familial/syndromic presentation, symptomatic presentation, biochemistry, size, number and location of tumours and presence of metastases and mode of its diagnosis.

Results: Twenty patients were found to have metastases; 13 had metastases at diagnosis and seven during follow-up. Metastases were detected by radiology (CT-neck to pelvis) in 11/20 patients (5/13 synchronous and 6/7 metachronous), 131I-metaiodobenzylguanidine in five (2/12 synchronous and 3/6 metachronous) patients and 18F-flurodeoxyglucose PET/CT in 15 (12/12 synchronous and 3/3 metachronous) patients. Malignant tumours were significantly larger than benign tumours (8.3 ± 4.1 cm, range: 3-22 cm vs 5.7 ± 2.3 cm, range: 2-14 cm, P = 0.0001) and less frequently metanephrine secreting. On linear regression analysis, tumour size and lack of metanephrine secretion were the independent predictors of malignancy.

Conclusions: Patients with primary tumour size >5.7 cm and lack of metanephrine secretory status should be evaluated for possible malignancy not only at diagnosis but also in the postoperative period. As compared to CT and 131I-MIBG scan, 18F-flurodeoxyglucose PET/CT analyses are better (sensitivity: 100%) for the diagnosis of metastases in our study.

Keywords: 18F-flurodeoxyglucose PET/CT; clinical predictors; malignant paraganglioma; malignant pheochromocytoma.

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Figures

Figure 1
Figure 1
Case 11: 35/F, abdominal sPGL. (A) CT scan showing sPGL; (B) MIBG positive only in the primary; (C) positive FDG PET in primary and skeletal metastasis; (D) on follow-up, MIBG did not detect any lesions, but FDG detected multiple metastases.
Figure 2
Figure 2
Benign right PCC. (A) CT showing primary tumour; (B) MIBG showing uptake in primary; (C) FDG PET showing uptake only in primary.
See this image and copyright information in PMC

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