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Malignant pheochromocytoma: new malignancy criteria

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Abstract

Purpose

The pathological diagnosis of malignancy in pheochromocytomas remains a controversial issue. According to the WHO, malignancy is defined in the presence of metastasis. Multiparameter scoring systems such as PASS (Pheochromocytoma of Adrenal gland Scaled Score) have been used but remain controversial. The aim of this study was to search for new immunohistologic elements allowing determination of pheochromocytoma malignancy.

Methods

Among 53 patients operated for pheochromocytoma between 1993 and 2009, we selected pheochromocytomas with proven metastasis, seven cases in group 1 (G1) and paired two others groups: group 2 (G2), patients who had “benign” pheochromocytoma with PASS ≥4 and group 3 (G3), patients who had “benign” pheochromocytoma with PASS <4. We retrospectively analysed PASS criteria, size, weight, tumour necrosis, Ki-67 and pS100 staining.

Results

The size and weight of the lesion were directly and significantly correlated to malignancy in all three groups: respectively 9.7 cm and 292.0 g (G1), 6.2 cm and 83.8 g (G2) and 3.8 cm and 37.1 g (G3) (p < 0.005 for both). Tumour necrosis (TN) was present in all G1 (p < 0.005) and respectively at 0% and 37.5% in G2 and G3. Ki-67 is directly correlated to presence of TN (p < 0.005) and malignancy (G1 14.1%, G2 1.8%, G3 2.6%; p < 0.001). All G1 had a Ki-67 index >4%, although one G3 presented an 11% Ki-67 index. There was an inverse statistically significant correlation between the three groups in staining using pS100 (p < 0.01).

Conclusions

Size and weight of the pheochromocytoma are directly related to PASS and malignancy. The presence of tumour necrosis, Ki-67 index >4% and pS100 absence impose a close histopathological evaluation and follow-up with regard to cases presenting a high risk of malignancy/recurrence.

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Conflicts of interest

No competing interest is declared for this paper.

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Authors and Affiliations

  1. Service de Chirurgie Digestive et Endocrinienne, CHU La Milétrie, 86021, Poitiers, France

    Pierre de Wailly, Luigi Oragano, Francois Radé, Anthony Beaulieu, Vincent Arnault & Jean Louis Kraimps

  2. Service d’Anathomopathologie, CHU La Milétrie, Poitiers, France

    Pierre Levillain

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  1. Pierre de Wailly
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  2. Luigi Oragano
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  3. Francois Radé
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  4. Anthony Beaulieu
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  5. Vincent Arnault
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  7. Jean Louis Kraimps
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Correspondence to Pierre de Wailly.

Additional information

This paper is based on the work that is the subject of an oral presentation at the ESES Workshop 2011, 12–14 May 2011, Lyon (France).

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de Wailly, P., Oragano, L., Radé, F. et al. Malignant pheochromocytoma: new malignancy criteria. Langenbecks Arch Surg 397, 239–246 (2012). https://doi.org/10.1007/s00423-011-0850-3

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