Sarcoma indiferenciado de células redondas con rearreglo de CIC-DUX4 de vulva: presentación de un caso.

Jorge E Lasso de la Vega Z.

doi:10.48204/medica.v45n2.8429

Submitted October 13, 2025

Published 2025-10-14

Casos de interés quirúrgico

Vol. 45 No. 2 (2025): Revista médica de Panamá

Undifferentiated round cell sarcoma with CIC-DUX4 rearrangement of the vulva: case report.

Jorge E Lasso de la Vega Z. ⁺⁻

DOI https://doi.org/10.48204/medica.v45n2.8429

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DOI: 10.48204/medica.v45n2.8429

Published: 2025-10-14

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Lasso de la Vega Z. , J. E. (2025). Undifferentiated round cell sarcoma with CIC-DUX4 rearrangement of the vulva: case report. Revista médica De Panamá, 45(2), 103–109. https://doi.org/10.48204/medica.v45n2.8429

This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

Abstract

We present an extremely rare case of vulvar sarcoma that required specialized genetic testing for its final diagnosis. Initially, it was mistaken for an infectious process. Then, the biopsy classified it as a sarcoma, and the patient was referred to the National Cancer Institute. The corresponding surgery was performed, and the tumor was reported as an undifferentiated small cell sarcoma. For the final diagnosis, paraffin blocks were sent to a center specializing in sarcomas in Italy. The patient's progress has been satisfactory.

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Vasconcelos-Prado, Oliver Daniel, López-García, Alma Edith, & García-Montalvo, Iván Antonio. (2021). Aspectos genéticos de los Feocromocitomas y Paragangliomas. JONNR. 6(4), 636-650. doi: 10.19230/jonnpr.4020
Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. (2010). J Clin Endocrinol Metab. 96(3):717-25. doi: 10.1210/jc.2010-1946
Nagore-Ancona, Jesús F., Torres-Silva, Carlos, Várgas-Ávila, Arcenio L., Añorve-Bailón, Denisse, Mendoza-Elizarraraz, Diana, & Sánchez-Lora, Juan. (2021). Feocromocitoma maligno con disfunción hemodinámica. Presentación de un caso y revisión bibliográfica. GAMO. 20(1):72-78. doi: 10.24875/j.gamo.20000084
Jurado-Gómez, Daniel Alberto, Pineda-Garcés, Catalina, Arias, Luis Fernando, & Gutiérrez-Montoya, Jorge Iván. (2022). Feocromocitoma adrenal gigante derecho. Reporte de un caso. Rev Colomb Cir. 37(3), 511-517. doi: 10.30944/20117582.905
Cascón A, Calsina B, Monteagudo M, et al., (2023). Genetic bases of pheochromocytoma and paraganglioma. J Mol Endocrinol. 70(3): e220167. doi: 10.1530/JME-22-0167
Timmers HJLM, Taïeb D, Pacak K, Lenders JWM. (2024). Imaging of Pheochromocytomas and Paragangliomas. Endocr Rev. 7;45(3):414-434. doi: 10.1210/endrev/bnae001
Fishbein L, Leshchiner I, Walter V, et al., (2017). Comprehensive molecular characterization of pheochromocytoma and paraganglioma. Cancer Cell (31):181–193. doi: 10.1016/j.ccell.2017.01.001
Saavedra T, J. S., Nati-Castillo, H. A., Valderrama Cometa, et al., (2024). Pheochromocytoma: an updated scoping review from clinical presentation to management and treatment. Front. Endocrinol. 15(1433582). doi: 10.3389/fendo.2024.1433582
A.S. Kroiss, C. Uprimny, B.L. Shulkin, et al., (2019). Comparación entre la PET/TC con 18F-DOPA y la PET/TC con 68Ga-DOTATOC para la localización del paraganglioma maligno extra-adrenal y el feocromocitoma. Rev Esp Medi Nucl Imagen Mol. 38, (2) 94-99. doi:10.1016/j.remn.2018.09.004
Sina Jasim, Camilo Jimenez. (2020). Metastatic pheochromocytoma and paraganglioma: Management of endocrine manifestations, surgery and ablative procedures, and systemic therapies. Best Pract Res Clin Endocrinol Metab. 34(2). doi: 10.1016/j.beem.2019.101354

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[1] Vasconcelos-Prado, Oliver Daniel, López-García, Alma Edith, & García-Montalvo, Iván Antonio. (2021). Aspectos genéticos de los Feocromocitomas y Paragangliomas. JONNR. 6(4), 636-650. doi: 10.19230/jonnpr.4020

[2] Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromocytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. (2010). J Clin Endocrinol Metab. 96(3):717-25. doi: 10.1210/jc.2010-1946

[3] Nagore-Ancona, Jesús F., Torres-Silva, Carlos, Várgas-Ávila, Arcenio L., Añorve-Bailón, Denisse, Mendoza-Elizarraraz, Diana, & Sánchez-Lora, Juan. (2021). Feocromocitoma maligno con disfunción hemodinámica. Presentación de un caso y revisión bibliográfica. GAMO. 20(1):72-78. doi: 10.24875/j.gamo.20000084

[4] Jurado-Gómez, Daniel Alberto, Pineda-Garcés, Catalina, Arias, Luis Fernando, & Gutiérrez-Montoya, Jorge Iván. (2022). Feocromocitoma adrenal gigante derecho. Reporte de un caso. Rev Colomb Cir. 37(3), 511-517. doi: 10.30944/20117582.905

[5] Cascón A, Calsina B, Monteagudo M, et al., (2023). Genetic bases of pheochromocytoma and paraganglioma. J Mol Endocrinol. 70(3): e220167. doi: 10.1530/JME-22-0167

[6] Timmers HJLM, Taïeb D, Pacak K, Lenders JWM. (2024). Imaging of Pheochromocytomas and Paragangliomas. Endocr Rev. 7;45(3):414-434. doi: 10.1210/endrev/bnae001

[7] Fishbein L, Leshchiner I, Walter V, et al., (2017). Comprehensive molecular characterization of pheochromocytoma and paraganglioma. Cancer Cell (31):181–193. doi: 10.1016/j.ccell.2017.01.001

[8] Saavedra T, J. S., Nati-Castillo, H. A., Valderrama Cometa, et al., (2024). Pheochromocytoma: an updated scoping review from clinical presentation to management and treatment. Front. Endocrinol. 15(1433582). doi: 10.3389/fendo.2024.1433582

[9] A.S. Kroiss, C. Uprimny, B.L. Shulkin, et al., (2019). Comparación entre la PET/TC con 18F-DOPA y la PET/TC con 68Ga-DOTATOC para la localización del paraganglioma maligno extra-adrenal y el feocromocitoma. Rev Esp Medi Nucl Imagen Mol. 38, (2) 94-99. doi:10.1016/j.remn.2018.09.004

[10] Sina Jasim, Camilo Jimenez. (2020). Metastatic pheochromocytoma and paraganglioma: Management of endocrine manifestations, surgery and ablative procedures, and systemic therapies. Best Pract Res Clin Endocrinol Metab. 34(2). doi: 10.1016/j.beem.2019.101354

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