Endocrine studies in a patient with functioning adrenal rest tumor of the liver

Wallace, Eleanor Z.; Leonidas, J R; Stanek, Albert; Avramides, A.

doi:10.1016/0002-9343(81)90886-x

Cited by 41 publications

(34 citation statements)

References 6 publications

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“…However, at autopsy, the left adrenal cortex was partially involved by the tumor, and therefore, it is possible that the tumor may have arisen from an extra-adrenal nodule. To our knowledge, there have been so far only four case reports in which steroidogenic capability was found in an adrenal rest tumor [7,[21][22][23]. The present case, however, is the first to confirm steroidogenic capability in a malignant tumor by immunohistochemistry.…”

Section: Discussionsupporting

confidence: 40%

“…Whether these criteria can be applied to adrenal rest tumors has never been assessed. In the present case, supraclavicular lymph node metastasis was found from the time of first admission confirming malignancy, and the histopathologic findings including the MIB1 index (about 10%) were consistent with a malignant diagnosis according to Weiss. To our knowledge, only 11 cases of malignant adrenal rest tumors (with the exception of the present case) have been so far reported [2][3][4][5][6][7][8][9][10][11][12]. In these cases, the primary sites of malignant adrenal rest tumor were the testis, liver, retroperitoneum, kidney, and spinal cord, and seven cases showed hormonal symptoms.…”

Section: Discussionmentioning

confidence: 79%

“…The adrenal rest rarely persists until adulthood as it generally undergoes atrophy and disappears within a few years after birth [2]; however, a neoplasm can arise from the adrenal rest. Their malignant counterpart, however, is extremely rare, and only a few sporadic cases have been reported [2][3][4][5][6][7][8][9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

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Malignant Adrenal Rest Tumor of the Retroperitoneum Producing Adrenocortical Steroids

et al. 2011

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We present a case of a malignant adrenal rest tumor arising from the retroperitoneum with Cushing's syndrome in a 31-year-old female. Her serum cortisol and dehydroepiandrosterone sulfate levels were elevated, while adrenocorticotropic hormone levels were low. Computed tomography scans and magnetic resonance imaging revealed a retroperitoneal tumor with no visible lesions in the adrenal glands and ovaries. From those results and the histopathologic findings following biopsy of an enlarged supraclavicular lymph node, the patient was diagnosed as a malignant adrenal rest tumor of the retroperitoneum. Despite chemotherapy, the patient died of rapid development of multiple metastases. Autopsy revealed a large tumor that extended around the abdominal aorta from the levels of the left kidney to the aortic bifurcation with generalized metastases. Tumor cells were characterized by clear and eosinophilic cytoplasm and atypical nuclei that exhibited frequent and atypical mitoses. Immunohistochemistry regarding steroidogenesis was performed and revealed that the tumor cells were immunopositive for adrenal 4 binding protein/steroidogenic factor-1, cholesterol side-chain cleavage enzyme, 17α-hydroxylase, and 21-hydroxylase. We thus elucidated the adrenocortical steroid production in the tumor cells causing Cushing's syndrome. This case report first demonstrates the steroidogenic capacity in a malignant adrenal rest tumor.

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Section: Discussionsupporting

confidence: 40%

Section: Discussionmentioning

confidence: 79%

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Malignant Adrenal Rest Tumor of the Retroperitoneum Producing Adrenocortical Steroids

et al. 2011

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“…We are aware of only three cases of functioning adrenal rest tumor of the liver reported in the literature (7)(8)(9). Our conclusion that the excised tumor was the source of the cortisol excess is supported by the following findings: 1) the tumor contained about 20 times more cortisol per milligram wet tissue than did the surrounding liver; 2) after the operation the elevated steroid levels in serum and urine fell to normal or subnormal values; 3) significant steroidogenic capability was found in tumor minces; 4) apparent autonomy of the tumor as evidenced by suppressed gonadotropins and undetectable ACTH levels in serum, and 5) the adrenals were atrophic on exploration and on histopathological examination.…”

Section: Discussionmentioning

confidence: 99%

Adrenal Rest Tumor of the Liver Causing Cushing's Syndrome: Treatment withKetoconazole preceding an Apparent Surgical Cure*

Contreras

Altieri

Liberman

et al. 1985

The Journal of Clinical Endocrinology & Metabolism

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Ketoconazole, an imidazole derivative known to inhibit cytochrome P450-dependent adrenal enzymes was given to a patient with a functioning adrenal rest tumor of the liver in preparation for surgery. The drug was administered in a stepwise manner for 42 days starting with 400 mg and reaching 1 g the last 4 weeks of the trial. Clear clinical improvement was evident early in the trial and was associated with evidence of amelioration of her hypercortisolism and striking changes in serum and urinary levels of steroid hormones and metabolites. Sex steroids in serum and urine fell dramatically from the first day to the end of the trial. Urinary 17-ketosteroid excretion fell from a basal average of 139 mg/24 h to near normal levels within a week of therapy; serum testosterone fell from a basal level of 2.4 to 0.18 ng/ml; serum 17 beta-estradiol fell likewise from 1096 to 150 pg/ml. In contrast, cortisol levels in serum and urine increased in the first 2 weeks of the trial and subsequently fell to values below the basal levels. Similarly, serum 17 alpha-OH-progesterone levels increased 63% above the basal levels by day 6 of the trial and declined afterwards. Nine months after successful tumor resection the patient is apparently cured as judged by steroid hormone levels and physical appearance. We conclude that ketoconazole was effective in blocking tumoral steroidogenesis which resulted in clinical benefit.

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“…A non-ACTH-mediated development of a neoplasm in such tissues is uncommon and the clinical picture depends on the steroid producing profile of the tumour (Conn's syndrome, virilisation or Cushing's syndrome). So far only 9 cases of Cushing's syndrome caused by functional ectopic adrenal neoplasms have been reported (31)(32)(33)(34)(35)(36)(37)(38). The ectopic locations involved the vicinity of normal adrenals, the liver, the pararenal region, and the ovary.…”

Section: Unusual Causes Of Acth-independent Cushing's Syndrome (Tablementioning

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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Endocrine studies in a patient with functioning adrenal rest tumor of the liver

Cited by 41 publications

References 6 publications

Malignant Adrenal Rest Tumor of the Retroperitoneum Producing Adrenocortical Steroids

Malignant Adrenal Rest Tumor of the Retroperitoneum Producing Adrenocortical Steroids

Adrenal Rest Tumor of the Liver Causing Cushing's Syndrome: Treatment withKetoconazole preceding an Apparent Surgical Cure*

Unusual causes of Cushing's syndrome

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