Cushing's syndrome induced by hypersecretion of cortisol from only one of bilateral adrenocortical tumors

Kato, S; Masunaga, Rumi; Kawabe, Takao; Nagasaka, Akio; Miyamoto, T.; Itoh, Motoko; Nakai, Asami; Iwase, Katsumi; Tsujimura, Tohru; Ohtani, Susumu; Inagaki, Asako; Miura, Kaoru; Chikamatsu, Hitoshi; Mizuno, Yasushi

doi:10.1016/0026-0495(92)90268-f

Cited by 14 publications

(5 citation statements)

References 12 publications

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“…Cases of full blown Cushing's syndrome due to bilateral adrenal tumours have been reported [13][14][15][16][17][18][19], but not in those with preclinical Cushing's syndrome as in this case. In addition, the case reported here is also the first reported case of unilateral adrenocortical carcinoma associated with contralateral adenoma.…”

Section: Discussionmentioning

confidence: 87%

A Patient With Preclinical Cushing's Syndrome and Excessive DHEA-S Secretion Having Unilateral Adrenal Carcinoma and Contralateral Adenoma.

et al. 1999

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Abstract. We report a case of preclinical Cushing's syndrome in a 54-year-old male associated with bilateral adrenocortical tumours. Physical findings and general laboratory data were unremarkable except for mild hypertension (158/90 mmHg) and impaired glucose tolerance. Endocrinological evaluation revealed the presence of autonomous cortisol secretion including unsuppressible serum cortisol by 8 mg dexamethasone test (11,ug/dl), high serum DHEA-S (3580 ng/ml, normal: 400-3500) and increased urinary 17-KS excretion (31.0-35.8 mg/day, normal: 5.8-21.3). CT scan demonstrated the presence of tumours in both adrenals and bilateral adrenalectomy was subsequently performed. Histological examination of the resected specimens revealed an adrenocortical carcinoma on the right side and an adenoma on the left side with noticeable cortical atrophy in non-neoplastic adrenals. Immunohistochemical study of steroidogenic enzymes demonstrated that all the steroidogenic enzymes involved in cortisol biosynthesis were expressed in both right and left adrenal tumours. Enzymatic activities of 21, f3-hydroxylases were detected in both right and left adrenals except for the absence of 11 f3-hydroxylase activity in the left adrenal adenoma.Results of in vitro tissue steroidogenesis examined in short-term tissue culture of the specimens revealed no significant differences between carcinoma and adenoma in cortisol production, but the production of adrenal androgens in carcinoma was significantly higher than that in adenoma, which may indicate the importance of evaluating adrenal androgen levels in patients with adrenocortical neoplasms.

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Section: Discussionmentioning

confidence: 87%

A Patient With Preclinical Cushing's Syndrome and Excessive DHEA-S Secretion Having Unilateral Adrenal Carcinoma and Contralateral Adenoma.

et al. 1999

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“…In bilateral adenomas there is usually only one single nodule in each gland with surrounding atrophy of the adrenal cortex. However, in one case (43) multiple nodules in each adrenal gland were observed. Typically in AIMAH there is massive enlargement of the adrenals with multiple macronodules measuring up to 5 cm while in PPNAD the adrenal glands appear normal to slightly hyperplastic with micronodules not exceeding 5 mm.…”

Section: Bilateral Adrenal Adenomasmentioning

confidence: 85%

“…Bilateral adrenalectomy necessitating lifelong steroid replacement is the treatment of choice in PPNAD and AIMAH while in bilateral adenomas an effort to preserve the adrenocortical function could be made through bilateral partial adrenalectomy. It is important to document functionality of both adenomas before undergoing bilateral adrenalectomy, since there is also the possibility of the coexistence of a functional and a contralateral nonfunctioning adenoma, as has been reported in a few cases (43,44). In this case adrenal scintigraphy and/or adrenal vein sampling might be of value.…”

Section: Bilateral Adrenal Adenomasmentioning

confidence: 99%

Unusual causes of Cushing's syndrome

Vassiliadi

Tsagarakis

2007

Arq Bras Endocrinol Metab

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Although in the majority of the patients with Cushing's syndrome (CS), hypercortisolism is due to ACTH hypersecretion by a pituitary tumour or to ectopic ACTH secretion from an extrapituitary neoplastic lesion or to autonomous cortisol secretion by an adrenal tumour, in occasional patients a much rarer entity may be the cause of the syndrome. Herein, we attempted to summarise and categorise these unusual causes according to their presumed aetiology. To this end, we performed a comprehensive computer-based search for unusual or rare causes of CS. The following unusual forms of CS were identified: (i) ACTH hyperesecretion due to ectopic corticotroph adenomas in the parasellar region or the neurohypophysis, or as part of double adenomas, or gangliocytomas; (ii) ACTH hypersecretion due to ectopic CRH or CRH-like peptide secretion by various neoplasms; (iii) ACTH-independent cortisol hypersecretion from ectopic or bilateral adrenal adenomas; (iv) glucocorticoid hypersensitivity; (v) iatrogenic, due to megestrol administration or to ritonavir and fluticasone co-administration. Such unusual presentations of CS illustrate why Cushing's syndrome represents one of the most puzzling endocrine syndromes. RESUMOCausas Incomuns da Síndrome de Cushing. Embora na maioria dos pacientes com síndrome de Cushing (SC), o hipercortisolismo se deva à hipersecreção de ACTH resultante de um tumor hipofisário ou de uma fonte ectópica de ACTH por uma lesão neoplásica extra-hipofisária, ou ainda pela secreção autônoma de cortisol por um tumor adrenal, ocasionalmente uma entidade muito mais rara pode ser a causa da síndrome. Nesta revisão, tentaremos sumarizar e categorizar essas causas incomuns de acordo com sua pressuposta etiologia. Para isso, fizemos uma ampla pesquisa por computador em busca dessas causas raras ou não usuais de SC. As seguintes formas não usuais de SC foram identificadas: (i) hipersecreção de ACTH devida a adenomas corticotróficos ectópicos na região parasselar ou na neurohipófise, ou como parte de adenomas duplos, ou gangliocitomas; (ii) hipersecreção de ACTH devida à secreção ectópica de CRH ou peptídeo CRH-símile por várias neoplasias; (iii) hipersecreção de cortisol ACTHindependente por adenomas adrenais ectópicos ou bilaterais; (iv) hipersensibilidade aos glicocorticóides; (v) iatrogênica, devida à administração de megestrol ou à co-administração de ritonavir e fluticasona. Essas apresentações incomuns da SC ilustram por que essa síndrome é considerada uma das mais desafiadoras da endocrinologia.

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“…Finalmente, o cateterismo seletivo das veias supra-renais demonstrou que os adenomas bilaterais eram funcionantes. A ocorrência de adenoma adrenal bilateral é rara, havendo apenas descrições de casos isolados (12)(13)(14). O diagnóstico diferencial das massas adrenais benignas bilaterais associadas a hipercortisolismo inclui a Síndrome de Cushing ACTHdependente, a hiperplasia adrenal macronodular e a displasia adrenal nodular pigmentada primária (PPNAD).…”

Section: Discussionunclassified

Incidentaloma adrenal bilateral associado à síndrome de cushing sub-clínica

Leal

Freitas

Tucci

et al. 1998

Arq Bras Endocrinol Metab

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RESUMOApresentamos um caso de paciente com achado de massa adrenal bilateral à tomografia computadorizada e ausência de sinais ou sintomas associados à hipersecreção adrenal (incidentaloma). A investigação laboratorial revelou perda do ritmo circadiano do cortisol plasmático e salivar, não supressibilidade do cortisol plasmático e salivar a doses crescentes de dexametasona (1 mg, 2mg, 8mg, 24mg), ACTH plasmático basal baixo e não responsivo à injeção de CRH ovino, caracterizando assim autonomia da secreção adrenal e o quadro de síndrome de Cushing sub-clínica. Os achados cirúrgico e anátomo-patológico e o cateterismo seletivo de veias renais e supra renais foram compatíveis com o diagnóstico de adenomas adrenais bilaterais funcionantes. A paciente foi submetida à adrenalectomia com boa evolução clínica. São discutidos dados clínicos e laboratoriais pertinentes ao caso. (Arq Bras Endocrinol Metab 1998;42/6:489-492). Unitermos: Incidentaloma adrenal; Adenoma adrenal; Síndrome de Cushing ABSTRACTWe present the case of a patient with bilateral adrenal mass on CT and no symptoms related to adrenal hypersecretion (incidentaloma). Laboratory investigation revealed loss of arcadian rhythm of plasma and salivary cortisol, no supression of plasma and salivary cortisol to increasing dexamethasone doses (1mg, 2mg, 8mg, 24mg), low basal plasma ACTH and no response of ACTH to ovine CRH. These data characterize autonomous adrenal secretion and sub-clinical Cushing's Syndrome. Surgical and anatomopathological findings and selective catheterization of adrenal and renal veins indicated the diagnosis of functional bilateral adrenal adenomas. The patient was adrenalectomized and had good clinical evolution. We discuss clinical and laboratory data related to the case. (Arq Bras Endocrinol Metab 1998;42/6:489-492).Keywords: Adrenal incidentaloma; Adrenal adenoma; Cushing's syndrome APRESENTAÇÃO DO CASO P ACIENTE FEMININA DE 65 ANOS, natural e procedente de São Tomás de Aquino (MG) referia há aproximadamente 7 anos, dor lombar direita, com irradiação para flanco e fossa ilíaca direitos, tipo latejante, com freqüência diaria, que se acentuava com atividade física e melhorava com o repouso. Há 8 meses ao realizar exames radiográficos (CT e USG abdominais) foram detectados "massa adrenal à direita e cistos renais". Negava alteração do peso, do facies ou da pilificação corpórea e coloração da pele, acne e estrias violáceas, hipotensão postural, vômitos, polifagia, polidipsia ou poliuria, alteração da genitália e da libido. Ultima menstruação há 20

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Cushing's syndrome induced by hypersecretion of cortisol from only one of bilateral adrenocortical tumors

Cited by 14 publications

References 12 publications

A Patient With Preclinical Cushing's Syndrome and Excessive DHEA-S Secretion Having Unilateral Adrenal Carcinoma and Contralateral Adenoma.

A Patient With Preclinical Cushing's Syndrome and Excessive DHEA-S Secretion Having Unilateral Adrenal Carcinoma and Contralateral Adenoma.

Unusual causes of Cushing's syndrome

Incidentaloma adrenal bilateral associado à síndrome de cushing sub-clínica

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