El feocromocitoma enmascaradoreporte de un caso
- Irene Ruiz Adelantado
- Clara Sáez Ibarra
- Laura Noguera Alonso
- Lorenzo Alarcón García
ISSN: 2660-7085
Año de publicación: 2024
Volumen: 5
Número: 5
Tipo: Artículo
Otras publicaciones en: Revista Sanitaria de Investigación
Resumen
Introduction: Composite pheochromocytoma is a rare pathological condition (3% of pheochromocytomas), characterized by the presence of typical elements of both pheochromocytoma and neuroblastic tumors, often presenting with subtle symptoms, with gastrointestinal and cardiovascular alterations being the most frequently reported. Case Report: A 67-year-old male referred from Urology due to radiological findings of a 3 cm renal mass with an incidental discovery of a 2 cm adrenal nodule. Histological confirmation of renal lesion malignancy leads to a decision for total nephrectomy surgery. As no functionality is detected in the adrenal tumor, surgical intervention is performed for both lesions. Microscopic examination establishes the diagnosis of composite pheochromocytoma, identifying two intermixed cellular proliferations of different nature, histology, and immunohistochemical profile. Discussion: Composite pheochromocytoma is a rare entity in which pheochromocytoma coexists with other histological components, with its pathogenesis not yet clearly understood. Radiological studies do not allow differentiation of this entity from other types of adrenal tumors, and the clinical presentation can be nonspecific as the neuroblastic component is not metabolically active. Due to its low frequency, genetic testing is advised to better understand its biological behavior and its association with hereditary syndromes.
Referencias bibliográficas
- Manger WM, Eisenhofer G (2004) Pheochromocytoma: diagnosis and management update. Curr Hypertens Rep 6:477–484.
- Brady S, Lechan RM, Schwaitzberg SD, Dayal Y, Ziar J, Tischler AS. Composite pheochromocytoma/ganglioneuroma of the adrenal gland associated with multiple endocrine neoplasia 2A: Case report with immunohistochemical analysis. Am J Surg Pathol. 1997;21:102–8
- Khan AN, Solomon SS, Childress RD. Composite pheochromocytoma-ganglioneuroma: a rare experiment of nature. Endocr Pract. 2010;16:291-99.
- Lonergan GJ, Schwab CM, Suarez ES, Carlson CL (2002) Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic–pathologic correlation. Radiographics 22(4):911–934.
- Thiel EL, Trost BA, Tower RL (2010) A composite pheochromocytoma/ ganglioneuroblastoma of the adrenal gland. Pediatr Blood Cancer. 54(7):1032–1034.
- Fujiwara T, Kawamura M, Sasou S, Hiramori K (2000) Results of surgery for a compound adrenal tumor consisting of pheochromocytoma and ganglioneuroblastoma in an adult: 5-year follow-up. Intern Med 39(1):58–62.
- Jing H, Li F, Wang L et al (2017) Comparison of the 68Ga-DOTATATA PET/CT, FDG PET/CT, and MIBG SPECT/CT in the evaluation of suspected primary Pheochromocytomas and Paragangliomas. Clin Nucl Med 42:525–529.
- Chang CA, Pattison DA, Tothill RW et al (2016) (68) Ga- DOTATATE and (18)F-FDG PET/CT in Paraganglioma and Pheochromocytoma: utility, patterns and heterogeneity. Cancer Imaging Off Publ Int Cancer Imaging Soc 16:22.
- Shawa H, Elsayes KM, Javadi S, Sircar K, Jimenez C, Habra MA (2014) Clinical and radiologic features of pheochromocytoma/ganglioneuroma composite tumors: a case series with comparative analysis. Endocr Pract 20(9):864–869.
- Galan SR, Kann PH (2013) Genetics and molecular pathogenesis of pheochromocytoma and paraganglioma. Clin Endocrinol (Oxf ) 78(2):165–175.