From ACTH-Dependent to ACTH-Independent Cushing's Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells

Claudia Ramírez-Rentería; Ana Laura Espinosa-De-Los-Monteros; Espinosa-Cardenas Etual; Daniel Marrero-Rodríguez; Guillermo Castellanos; Rocio Arreola-Rosales; Guillermo Montoya-Martinez; Keiko Taniguchi-Ponciano; Paola Briseño-Díaz; Mayte Lizeth Padilla-Cristerna; María Del Pilar Figueroa-Corona; Mercado Moises

doi:10.1155/2020/4768281

From ACTH-Dependent to ACTH-Independent Cushing's Syndrome from a Malignant Mixed Corticomedullary Adrenal Tumor: Potential Role of Embryonic Stem Cells

Case Rep Endocrinol. 2020 May 4:2020:4768281. doi: 10.1155/2020/4768281. eCollection 2020.

Authors

Claudia Ramírez-Rentería^{1

2}, Ana Laura Espinosa-De-Los-Monteros¹, Espinosa-Cardenas Etual¹, Daniel Marrero-Rodríguez^{2

3}, Guillermo Castellanos⁴, Rocio Arreola-Rosales⁴, Guillermo Montoya-Martinez⁵, Keiko Taniguchi-Ponciano⁶, Paola Briseño-Díaz⁷, Mayte Lizeth Padilla-Cristerna⁷, María Del Pilar Figueroa-Corona⁷, Mercado Moises^{1

2}

Affiliations

¹ Endocrinology Service, UIM Enfermedades Endocrinas, Hospital de Especialidades, Centro Médico Nacional, S.XXI, Instituto Mexicano del Seguro Social, Av Cuauhtemoc 330, Col. Doctores, CP 06720, Mexico City, Mexico.
² UIM Enfermedades Endocrinas, Hospital de Especialidades, 23 Centro Médico Nacional, S.XXI, Instituto Mexicano del Seguro Social, Mexico City, Mexico.
³ Molecular Biomedicine Department, Center for Research and Advanced Studies, Instituto Politécnico Nacional, Av Cuauhtemoc 330, Col. Doctores, CP 06720, Mexico City, Mexico.
⁴ Pathology Department, Hospital de Especialidades, Centro Médico Nacional, S.XXI, Instituto Mexicano del Seguro Social, City. Av Cuauhtemoc 330, Col. Doctores, CP 06720, Mexico City, Mexico.
⁵ Urology Department, Hospital de Especialidades, Centro Médico Nacional, S.XXI, Instituto Mexicano del Seguro Social, Av Cuauhtémoc 330, Col. Doctores, CP 06720, Mexico City, Mexico.
⁶ Laboratorio de Neuroendocrinología Comparada, Departamento de Ecología y Recursos Naturales, Biología, Facultad de Ciencias, Universidad Nacional Autónoma de México, Exterior Circuit S / N, C.U. Coyoacán 04510, Mexico City, Mexico.
⁷ Departamento de Biomedicina Molecular Centro de Investigacion y 49 Estudios Avanzados, Av Instituto Politécnico Nacional 2508, San Pedro Zacatenco, Gustavo A. Madero, 07360 Ciudad de México, Mexico City, Mexico.

Abstract

Objective: To report the immunohistochemical and molecular evaluation of a patient with ectopic ACTH syndrome (EAS) from a MCAT which has single cells with features of both 96 medullary and cortical differentiation. Case Description and Methods. A 16-year-old woman presented with severe EAS and a large right MCAT composed of ACTH-secreting cells resembling pheochromocytoma and another lineage similar to adrenal carcinoma. Immunohistochemistry (IHC) showed positivity for medullary (ACTH, chromogranin A, synaptophysin, and PS-100) and epithelial components (inhibin, melan-A, and calretinin). Embryonic stem cell markers were evaluated using RT/PCR and immunofluorescence. After initial surgery, the tumor recurred shifting to rapidly progressive ACTH-independent liver metastasis.

Results: Histopathology and IHC revealed two distinct and intermingled cellular patterns, while some cells immunostained for both medullary and cortical markers. Demonstration of all stem cell biomarkers by RT/PCR and immunofluorescence was predominantly localized to the nucleus, whereas SOX2 immunoreactivity was evident in the cytoplasm as well.

Conclusion: The expression of cancer stem cell biomarkers points towards the involvement of primitive embryonic cells as the origin of this neoplasm and maybe to the clinically aggressive and biochemically changing behavior.

Publication types

Case Reports