COPPS, a composite score integrating pathological features, PS100 and SDHB losses, predicts the risk of metastasis and progression-free survival in pheochromocytomas/paragangliomas

Pierre, Charlie; Agopiantz, Mikaël; Brunaud, Laurent; Battaglia-Hsu, Shyue-Fang; Max, Antoine; Pouget, Celso; Nominé, Claire; Lomazzi, Sandra; Vignaud, Jean-Michel; Weryha, G.; Oussalah, Abderrahim; Gauchotte, Guillaume; Busby-Venner, Hélène

doi:10.1007/s00428-019-02553-5

Cited by 47 publications

(51 citation statements)

References 57 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Several histopathological scoring systems have been proposed, including PASS and GAPP scores, but it is also true that those above could by no means precisely predict the clinical outcome and/or the degree of aggressive clinical behavior in PPGL patients (5,(79)(80)(81). As a basis for these two established representative histological scoring systems, several combined scoring systems with genetic abnormalities and immunohistochemical findings have also been recently proposed, such as M-GAPP (Modified-GAPP) score (82), ASES (Age, Size, Extra-adrenal location, and Secretory type) score (83) and COPPs (Composite Pheochromocytoma/ paraganglioma Prognostic score) (84). However, further investigations are needed to clarify the practical value of such systems in discerning the clinical behavior of patient tumors.…”

Section: Challenges Of Predictive Clinicopathological/ Histopathologimentioning

confidence: 99%

Section: Copps (Composite Pheochromocytoma/ Paraganglioma Prognostic mentioning

confidence: 99%

“…COPPs (Composite Pheochromocytoma/paraganglioma Prognostic score) was recently proposed by Pierre et al in 2019, integrating morphological features and immunohistochemical findings of S-100 and SDHB (84). They examined a total of 147 PPGLs and performed multivariate analysis, including incorporation of the morphological features listed in PASS, immunohistochemical findings of S-100, Ki-67, and MCM6, clinicopathological factors (tumor size, age, and hypertension) and genotype (84). Finally, COPPs were defined according to the following criteria: three clinicopathological parameters (tumor size > 7 cm, necrosis, and vascular invasion), loss of S-100 immunoreactivity (loss of intervening sustentacular cells), and loss of SDHB immunoreactivity (suggesting SDHB mutation) (84) ( Figure 1E).…”

Section: Copps (Composite Pheochromocytoma/ Paraganglioma Prognostic mentioning

confidence: 99%

“…Both S-100 and Ki-67 are well-known and widely used markers for evaluation of the malignant potential of PPGLs (80,81,84). S-100 is generally immunolocalized in sustentacular cells surrounding tumor cells (91).…”

Section: Conventional Markersmentioning

confidence: 99%

“…S-100 is generally immunolocalized in sustentacular cells surrounding tumor cells (91). Absence or attenuation of S-100 immunoreactivity (sustentacular cells) is generally considered to reflect diffuse growth patterns that deviate from the structure of Zellballen, possibly resulting in the aggressive clinical behavior of tumors (84,91). S-100 positive sustentacular cells have recently been reported as non-neoplastic cells because SOX-10 and SDHB are both positive only in sustentacular cells in the cases of SDHB-mutated PPGLs (91).…”

Section: Conventional Markersmentioning

confidence: 99%

See 4 more Smart Citations

Recent Advances in Histopathological and Molecular Diagnosis in Pheochromocytoma and Paraganglioma: Challenges for Predicting Metastasis in Individual Patients

et al. 2020

View full text Add to dashboard Cite

show abstract

Section: Challenges Of Predictive Clinicopathological/ Histopathologimentioning

confidence: 99%

Section: Copps (Composite Pheochromocytoma/ Paraganglioma Prognostic mentioning

confidence: 99%

Section: Copps (Composite Pheochromocytoma/ Paraganglioma Prognostic mentioning

confidence: 99%

Section: Conventional Markersmentioning

confidence: 99%

Section: Conventional Markersmentioning

confidence: 99%

See 3 more Smart Citations

Recent Advances in Histopathological and Molecular Diagnosis in Pheochromocytoma and Paraganglioma: Challenges for Predicting Metastasis in Individual Patients

et al. 2020

View full text Add to dashboard Cite

show abstract

Metastatic sporadic paraganglioma with EWSR1::CREM gene fusion: A unique molecular profile that expands the phenotypic diversity of the molecular landscape of the EWSR1::CREM gene fusion positive tumors

Javaid

Patton

Tinoco

et al. 2022

Genes Chromosomes & Cancer

View full text Add to dashboard Cite

Chromosomal translocations with gene fusions are uniquely rare events in paraganglioma, mostly involving UBTF::MAML3 gene fusion. Precedent literature suggests that tumors involving MAML3 gene fusion correlate with poor clinical outcomes. Herein, we report a case of metastatic sporadic paraganglioma harboring EWSR1::CREM gene fusion in a 36‐year‐old male, that has not been previously described. The patient presented with large paraspinal mass that was resected the same year. Tumor recurred 3‐years later and on further work‐up, patient was found to have metastases involving both lungs. Histopathologic evaluation of the original primary tumor showed tightly packed irregular nests and cords of cells containing palely eosinophilic cytoplasm. Features considered atypical included: areas of solid growth pattern, coagulative tumor necrosis, focal cellular atypia and angiolymphatic invasion were also identified. By immunohistochemistry, the tumor cells were positive for synaptophysin and chromogranin and negative for keratin. The S100 stain highlights the sustentacular cells and the Ki‐67 proliferation index of 15%. The recurrence specimen was similar but showed increased cellularity, atypia, necrosis, and proliferative activity (Ki‐67 proliferation index of 35%). CT guided biopsy of the right lung lesion was consistent with metastasis. Next generation sequencing identified EWSR1::CREM fusion. The breakpoints were found in chromosome 22: 29683123 for EWSR1 exon 7 (NM_005243.3) and at chromosome 10:35495823 for CREM exon 6 (NM_001267562.1). Fluorescence in situ hybridization for EWSR1 gene rearrangement was positive. In summary, we report a case of metastatic paraganglioma with EWSR1::CREM gene fusion, not previously described in this entity, and expands on the phenotypic diversity within the genetic landscape of EWSR1::CREM gene fusion positive tumors.

show abstract

Analysis of clinical and pathological characteristics of retroperitoneal paraganglioma and associated prognostic factors

Chun,

Song,

et al. 2024

Journal of Surgical Oncology

View full text Add to dashboard Cite

Background and ObjectivesThe aim of this study is to explore the long‐term prognostic risk factors associated with patients diagnosed with retroperitoneal paraganglioma (RPGL) and examine their clinical and pathological characteristics.MethodsExpressions of biomarkers were identified using immunohistochemistry (IHC) and case databases were retrospectively searched. Survival analysis was performed using Kaplan‐Meier and Cox risk regression to identify the factors that influence the postoperative progression‐free survival of patients with RPGL.ResultsA total of 105 patients, most of whom had tumors situated in the paraaortic region, and whose average tumor size was 8.6 cm, were enrolled in this study. The average follow‐up duration was 51 months, with a mortality rate of 19% and a recurrence and metastasis rate of 41.9%. Tumors were assessed using the modified Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP), and SDHB, S‐100, and Ki‐67 were stained using IHC in all cases. Out of the total cases examined, negative in SDHB expression were observed in 18.1% of cases, S‐100 expression was negative in 36.2% of cases, and endovascular tumor enboluswas present in approximately 25.7% of cases. The results of the univariate analysis indicated that several factors significantly influenced the progression‐free survival of patients with PGL as follow: maximum tumor diameter (>5.5 cm), tumor morphological features, tumor grading (modified GAPP score > 6), SDHB negative, S‐100 negative, and expression of proliferation index Ki‐67 (>3%) (X2 = 4.217−27.420, p < 0.05). The results of the multivariate analysis indicated that negative of S‐100 (p = 0.021) and SDHB (p = 0.038), as well as intravascular tumor thrombus (p = 0.047) expression were independent risk factors for progression‐free survival in patients.ConclusionRPGL is characterized by diverse biological features and an elevated susceptibility to both recurrence and metastasis. Both SDHB and S‐100 can be employed as traditional IHC indicators to predict the metastatic risk of PGL, whereas the tumor histomorphology‐endovascular tumor enbolus assists in determining the metastasis risk of RPGL.

show abstract

COPPS, a composite score integrating pathological features, PS100 and SDHB losses, predicts the risk of metastasis and progression-free survival in pheochromocytomas/paragangliomas

Cited by 47 publications

References 57 publications

Recent Advances in Histopathological and Molecular Diagnosis in Pheochromocytoma and Paraganglioma: Challenges for Predicting Metastasis in Individual Patients

Recent Advances in Histopathological and Molecular Diagnosis in Pheochromocytoma and Paraganglioma: Challenges for Predicting Metastasis in Individual Patients

Metastatic sporadic paraganglioma with EWSR1::CREM gene fusion: A unique molecular profile that expands the phenotypic diversity of the molecular landscape of the EWSR1::CREM gene fusion positive tumors

Analysis of clinical and pathological characteristics of retroperitoneal paraganglioma and associated prognostic factors

Contact Info

Product

Resources

About