Extraskeletal myxoid chondrosarcoma: p53 and Ki-67 offer prognostic value for clinical outcome - an immunohistochemical and molecular analysis of 31 cases.

Fecha de publicación: 14/11/2022 Fecha Ahead of Print: 01/11/2022

Autores de IIS La Fe

Francisco Giner Segura

Autor

Participantes ajenos a IIS La Fe

Lopez-Guerrero, Jose Antonio
Machado, Isidro
Rubio-Martinez, Luis Alberto
Espino, Monica
Navarro, Samuel
Agra-Pujol, Carolina
Ferrandez, Antonio
Llombart-Bosch, Antonio

Grupos

Cirugía digestiva y Cuidados perioperatorios

Leader

Matteo Frasson

Abstract

Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft tissue tumor of unpredictable clinical behavior. The morphological spectrum of EMC based on histology alone can be difficult. There is no precise immunohistochemical (IHC) profile that together with the clinical parameters is able to predict the clinical outcome. We studied 31 cases confirmed as EMC. Clinical and follow-up data were recorded. Histopathological, molecular, and IHC studies were performed. Association among histopathological parameters was assessed using a chi-square test to determine homogeneity or linear trend for ordinal variables. The Kaplan-Meier proportional risk test (log rank) was used to study the impact of the histological, IHC, and molecular factors on progression-free survival (PFS) and disease-specific survival (DSS). Most EMCs showed a typical architectural pattern. Only a few cases presented an atypical histology (higher cellularity and solid pattern). IHC positivity (focal or diffuse) was present for CDK4 (100%), STAT-6 (90%), CD117 (84%), HNK-1 (81%), SATB2 (68%), and S-100 (58%). Synaptophysin and INSM1 were expressed in 22.6% and 38.7% of cases respectively. The EWSR1::NR4A3 rearrangement was found in 19 cases and 7 tumors presented the TAF15::NR4A3 fusion. Positive surgical margins together with atypical histology and expression of p53 and Ki67 correlated with worse clinical prognosis. EMCs express several IHC markers which are also seen in other soft tissue sarcomas. The molecular detection of NR4A3 rearrangement supports the differential diagnosis. Positive surgical margins together with atypical histology and positive expression of p53 and Ki-67 seem to predict a poor clinical outcome with worse prognosis, increased rate of recurrence, metastasis, and poor overall survival.

Datos de la publicación

ISSN/ISSNe:: 0945-6317, 1432-2307
Tipo:: Article
Páginas:: 407-417
DOI:: 10.1007/s00428-022-03453-x
PubMed:: 36376703
Factor de Impacto:: 1,184 SCImago ℠
Cuartil:: Q1 SCImago ℠

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Métricas

Filiaciones

Giner, Francisco:: 1 Hosp Univ & Politecn La Fe Valencia, Pathol Dept, Valencia, Spain, España.

6 Univ Valencia, Pathol Dept, Avd Blasco Ibanez 15, Valencia 46010, Spain, España.
Lopez-Guerrero, Jose Antonio:: 3 Inst Valenciano Oncol, Mol Biol Dept, Valencia, Spain, España.

4 Catholic Univ Valencia, Dept Pathol, Valencia, Spain, España.

5 Ctr Invest Principe Felipe CIPF, Joint Canc Res Unit, Valencia, Spain, España.
Machado, Isidro:: 2 Inst Valenciano Oncol & Patol, Pathol Dept, Lab Hosp QuironSalud, Valencia, Spain, España.

6 Univ Valencia, Pathol Dept, Avd Blasco Ibanez 15, Valencia 46010, Spain, España.
Rubio-Martinez, Luis Alberto:: 1 Hosp Univ & Politecn La Fe Valencia, Pathol Dept, Valencia, Spain, España.
Espino, Monica:: 6 Univ Valencia, Pathol Dept, Avd Blasco Ibanez 15, Valencia 46010, Spain, España.
Navarro, Samuel:: 6 Univ Valencia, Pathol Dept, Avd Blasco Ibanez 15, Valencia 46010, Spain, España.

7 Hosp Clin Univ, Pathol Dept, Valencia, Spain, España.
Agra-Pujol, Carolina:: 8 Hosp Gregorio Maranon, Pathol Dept, Madrid, Spain, España.
Ferrandez, Antonio:: 6 Univ Valencia, Pathol Dept, Avd Blasco Ibanez 15, Valencia 46010, Spain, España.

7 Hosp Clin Univ, Pathol Dept, Valencia, Spain, España.
Llombart-Bosch, Antonio:: 6 Univ Valencia, Pathol Dept, Avd Blasco Ibanez 15, Valencia 46010, Spain, España.