Unusual association of adrenal angiosarcoma and Cushing's disease

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8 Citations (Scopus)

Abstract

The occurrence of an angiosarcoma in the adrenal gland is exceedingly rare as only 21 patients have been reported in the literature since its first description 30 years ago. We report the clinicopathological features of a 34-year-old patient presenting with overt Cushing's syndrome due to a ACTH-secreting pituitary adenoma and an adrenal spindle cell angiosarcoma. This association made the diagnosis of Cushing's disease particularly puzzling in view of the results of biochemical tests which were compatible in part with adrenal and in part with paraneoplastic hypercortisolism and the negative pituitary imaging. The unique appearance of this tumor and its diagnostic implications are discussed.

Original languageEnglish
Pages (from-to)124-129
Number of pages6
JournalHormone Research
Volume56
Issue number3-4
DOIs
Publication statusPublished - 2001

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Pituitary ACTH Hypersecretion
Hemangiosarcoma
Cushing Syndrome
ACTH-Secreting Pituitary Adenoma
Adrenal Glands
Neoplasms

Keywords

  • ACTH
  • Adrenal cortex
  • Angiosarcoma
  • Cushing's disease
  • Cushing's syndrome

ASJC Scopus subject areas

  • Endocrinology

Cite this

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title = "Unusual association of adrenal angiosarcoma and Cushing's disease",
abstract = "The occurrence of an angiosarcoma in the adrenal gland is exceedingly rare as only 21 patients have been reported in the literature since its first description 30 years ago. We report the clinicopathological features of a 34-year-old patient presenting with overt Cushing's syndrome due to a ACTH-secreting pituitary adenoma and an adrenal spindle cell angiosarcoma. This association made the diagnosis of Cushing's disease particularly puzzling in view of the results of biochemical tests which were compatible in part with adrenal and in part with paraneoplastic hypercortisolism and the negative pituitary imaging. The unique appearance of this tumor and its diagnostic implications are discussed.",
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author = "Cecilia Invitti and {Pecori Giraldi}, Francesca and Francesco Cavagnini and Angelica Sonzogni",
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T1 - Unusual association of adrenal angiosarcoma and Cushing's disease

AU - Invitti, Cecilia

AU - Pecori Giraldi, Francesca

AU - Cavagnini, Francesco

AU - Sonzogni, Angelica

PY - 2001

Y1 - 2001

N2 - The occurrence of an angiosarcoma in the adrenal gland is exceedingly rare as only 21 patients have been reported in the literature since its first description 30 years ago. We report the clinicopathological features of a 34-year-old patient presenting with overt Cushing's syndrome due to a ACTH-secreting pituitary adenoma and an adrenal spindle cell angiosarcoma. This association made the diagnosis of Cushing's disease particularly puzzling in view of the results of biochemical tests which were compatible in part with adrenal and in part with paraneoplastic hypercortisolism and the negative pituitary imaging. The unique appearance of this tumor and its diagnostic implications are discussed.

AB - The occurrence of an angiosarcoma in the adrenal gland is exceedingly rare as only 21 patients have been reported in the literature since its first description 30 years ago. We report the clinicopathological features of a 34-year-old patient presenting with overt Cushing's syndrome due to a ACTH-secreting pituitary adenoma and an adrenal spindle cell angiosarcoma. This association made the diagnosis of Cushing's disease particularly puzzling in view of the results of biochemical tests which were compatible in part with adrenal and in part with paraneoplastic hypercortisolism and the negative pituitary imaging. The unique appearance of this tumor and its diagnostic implications are discussed.

KW - ACTH

KW - Adrenal cortex

KW - Angiosarcoma

KW - Cushing's disease

KW - Cushing's syndrome

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