Adrenal multiple cystic lesions as a morphological expression of bilateral cystic phaeochromocytoma in the context of Sipple’s syndrome (multiple endocrine neoplasia type 2a): presentation of a case

Iván I Gómez-Castro; Pilar A Ibarra-Vivas; Raúl Barreda-Escalante; Loudwin O Piña-Moneda; Enrique Granados-Sandoval

2019, Number 3

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Anales de Radiología México 2019; 18 (3)

Adrenal multiple cystic lesions as a morphological expression of bilateral cystic phaeochromocytoma in the context of Sipple’s syndrome (multiple endocrine neoplasia type 2a): presentation of a case

Gómez-Castro II, Ibarra-Vivas PA, Barreda-Escalante R, Piña-Moneda LO, Granados-Sandoval E

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Language: Spanish
References: 23
Page: 193-200
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ABSTRACT

Cystic pheochromocytomas are infrequent adrenal lesions. Multiple endocrine neoplasia type 2a. characterized by medullary thyroid carcinoma (100% of cases), pheochromocytoma (50%), and adenomas or parathyroid hyperplasia (25%). We present a case of a patient 51-year-old female who, during a subclinical state of the desease, is observed by abdominal computed tomography incidental image suggestive of bilateral cystic pheochromocytoma. Latr, were identified elevation of catecholamines and calcitonin, as well as, by imaging studies (multidetector computed tomography, magnetic resonance and scintigraphy [MCDT, MR]), was corroborated initial finding, also identifying bilateral medullary throid carcinoma with lympho-nodal metastasis to the neck, with subsequent histopathological correlation, framed in the context of multiple endocrine neoplasia type 2a.

REFERENCES

Madson Q, Almeida CS. Solid Tumors Associated with Multiple Endocrine Neoplasias. Cancer Genet Cytogenet. 2010;203(1):30-6.
Carney JA. Familial Multiple Endocrine Neoplasia, The First 100 Years. Am J Surg Pathol. 2005;29(2):254-74.
Monsalve J, Kapur J, Malkin D, Babyn PS. Imaging of cancer predisposition syndromes in children. Radiographics [Internet]. 2011;31(1):263-80.
Murakumo Y, Jijiwa M, Asai N, Ichihara M, Takahashi M. RET and neuroendocrine tumors. Pituitary. 2006;9(3):179-92.
Wohllk GN. Neoplasias endocrinas múltiples. desde el laboratorio al paciente. Rev Medica Clin Condes. 2013;24(5):778-83.
Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-peccoz P, Bordi C, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86(12):5658-71.
Kouvaraki MA, Shapiro SE, Perrier ND, Cote GJ, Gagel RF, Hoff AO, et al. RET proto-oncogene:A review and update of genotype-phenotype correlations in hereditary medullary thyroid cancer and associated endocrine tumors. Thyroid. 2005;15(6):531-44.
Frank-Raue K, Rybicki LA, Erlic Z, Schweizer H, Winter A, Milos I, et al. Risk profiles and penetrance estimations in multiple endocrine neoplasia type 2A caused by germline RET mutations located in exon 10. Hum Mutat. 2011;32(1):51-8.
Scarsbrook AF, Thakker RV, Wass JA, Gleeson F V, Phillips RR. Multiple endocrine neoplasia:spectrum of radiologic appearances and discussion of a multitechnique imaging approach. Radiographics [Internet]. 2006;26(2):433-51.
Blake MA, Kalra MK, Maher MM, Sahani DV, Sweeney AT, Mueller PR, et al. Pheochromocytoma:an imaging chameleon. Radiographics. 2004;24 Suppl 1:S87-99.
Ahualli J, Méndez-Uriburu L, Ravera ML, Méndez-Uriburu M. Feocromocitoma quístico:a propósito de un caso. Rev Argentina Radiol. 2008;72(2):177-80.
Lee TH, Slywotzky CM, Lavelle MT, Garcia RA. Cystic Pheochromocytoma. Radiographics. 2002;22(4):935-40.
Pacak K, Ilias I, Adams KT, Eisenhoffer G. Biochemical diagnosis, localization and management of pheochromocytoma:Focus on multiple endocrine neoplasia type 2 in relation to other hereditary syndromes and sporadic forms of the tumor. J Intern Med. 2005;257(1):60-8.
Mayo-Smith WW, Boland GW, Noto RB, Lee MJ. State-of-the-Art Adrenal Imaging. Radiographics. 2001;21(4):995-1012.
Binda M, Añone C, Miklaszewski J, Pardes E. Optimización del uso de las imágenes en la patología suprarrenal (parte 1). Rev Argentina Radiol. 2014;77(3):218-25.
Binda M, Añone C, Miklaszewski J, Pardes E. Optimización del uso de las imágenes en la patología suprarrenal (parte 2). Rev Argentina Radiol. 2014;77(4):255-66.
Blake MA, Cronin CG, Boland GW. Adrenal imaging. AJR Am J Roentgenol. 2012;63(1):71-81.
Bellantone R, Ferrante A, Raffaelli M, Boscherini M, Lombardi CP, Crucitti F. Adrenal cystic lesions:report of 12 surgically trated cases and review of the literature. J Endocrinol Invest. 1998;21(2):109-14.
Joya Vázquez R, Vecino Bueno C, Bengochea Cantos JM, Gómez Garcia O, López López MÁ, Molina Sánchez A, et al. Feocromocitoma quístico gigante:reporte de un caso. Rev Gastroenterol Perú. 2017;37(4):374-8.
Andreoni C, Krebs RK, Bruna PC. Cystic pheochromocytoma is a distinctive subgroup with special clinical, imaging and histological features that might mislead the diagnosis. BJU Int. 2008;101(3):345-50.
Johnson PT, Horton KM, Fishman EK. Adrenal mass imaging with multidetector CT:pathologic conditions, pearls, and pitfalls. Radio Graphics. 2009;29:1333-51.
Rosenblit A, Morehouse HT, Amis ES. Cystic adrenal lesions:CT features. Radiology. 1996;201:541-8.
Montoya-Martínez G, Moreno-Palacios J, Serrano-Brambila E. Adrenalectomía lumboscópica en un paciente con feocromocitoma y neoplasia endocrina múltiple tipo 2b. Cir. 2009;77(2):131-3.