Familial Cushing's syndrome with primary adrenocortical microadenomatosis (primary adrenocortical nodular dysplasia)

Schweizer-Cagianut, M.; Froesch, E. R.; Hedinger, Chr.

doi:10.1530/acta.0.0940529

Cited by 58 publications

(12 citation statements)

References 7 publications

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“…The familial occurrence of this disease has been demonstrated just recently (Arce et al 1978;Schweizer-Cagianut et al 1980;Donaldson et al 1981). In two siblings of one of these families we also found cardiac myxomas.…”

supporting

confidence: 63%

“…A preliminary diagnosis of von Recklinghausen's neurofibromatosis was made. At age 12, the patient's height was 1.41 m, at age 18, 1.47 m. At this age signs typical of an ACTH-independent Cushing's syndrome developed (for clinical details and laboratory findings see Schweizer-Cagianut et al 1980). Bilateral adrenalectomy was performed between the ages of 18 and 21 years.…”

Section: Patientsmentioning

confidence: 99%

“…In the following years the patient developed an ACTH-independent Cushing's syndrome (for clinical and laboratory details see Schweizer-Cagianut et al 1980). At the age of 28 she was bilaterally adrenalectomized.…”

Section: Sibling 3" 36 Year Old Female Born 1945 Died 1981mentioning

confidence: 99%

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Primary adrenocortical nodular dysplasia with Cusghing's syndrome and cardiac myxomas

Schweizer-Cagianut

Salomon

Hedinger

1982

Virchows Arch. A Path. Anat. and Histol.

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In a family with 4 children, 2 had slowly developing Cushing's syndrome and were adrenalectomized between the ages of 18 and 28 years. As in other cases with familial Cushing's syndrome, primary adrenocortical nodular dysplasia, the so-called adrenocortical adenomatosis, was demonstrated. The brother, now 39 years old, is in good health. The sister, however, died at the age of 36 years. Autopsy revealed a cardiac myxoma of the left atrium. A cardiac myxoma had been found incidentally at autopsy of these two patients' oldest brother, a boy who died at the age of 4 years. Both siblings with Cushing's syndrome presented additional Peutz-Jegher-like hyperpigmentation and myxomatous tumours of the skin. Identical observations of familial adrenocortical nodular dysplasia, Cushing's syndrome and cardiac myxomas have not been reported to date. However, the familial occurrence of cardiac myxomas and adrenocortical dysplasia in combination with bilateral large cell calcifying Sertoli cell tumours of the testis has been published recently.

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supporting

confidence: 63%

Section: Patientsmentioning

confidence: 99%

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Primary adrenocortical nodular dysplasia with Cusghing's syndrome and cardiac myxomas

Schweizer-Cagianut

Salomon

Hedinger

1982

Virchows Arch. A Path. Anat. and Histol.

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“…[35] The term PPNAD was first used by Carney in 1984 and later supported by Travis et al . [26] The other terminologies were found inappropriate because of the frequent presence of macronodules >1 cm and atrophy of intervening cortex and the absence of hyperplasia.…”

Section: Discussionmentioning

confidence: 99%

Primary pigmented nodular adrenocortical disease

Manipadam

Abraham

Sen

et al. 2011

J Indian Assoc Pediatr Surg

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“…Further studies resulted in a modification of the acronym to LAMB (lentigenes, atrial myxoma, mucocutaneous myxomas, blue nevi). The autosomaldominant syndromes were also called Swiss syndrome [15] or Carney's complex [20], describing combinations of atrial myxomas with nodular adrenal cortical hyperplasia and other multiple neoplasia in various organs (sertoli cell tumors of the testis, skin pigmentation, myxoid fibroadenomas of the breast), endocrine/pituitary hyperactivity leading to Cushing's syndrome, gigantism, and acromegaly [21,22].…”

Section: Myxoma Syndromementioning

confidence: 99%

Myxoma valvulae arteriae pulmonalis described by Rokitansky: medicohistorical and clinicopathological aspects

Kandutsch

Javor

Sedivy

2004

Wien Klin Wochenschr

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The bicentenary of the birth of Rokitansky, the first true descriptive pathologist, was celebrated in February 2004. Rokitansky gathered many and important specimens that are now displayed in the Federal Museum of Pathological Anatomy in Vienna. On examining Rokitansky's collection we found a myxoma of the pulmonary valve of a 70-year-old woman who died in 1833. Cardiac myxomas are rare but are the most frequent primary heart tumors, appearing even less frequently on cardiac valves. Modern histology has confirmed Rokitansky's diagnosis on gross pathology. The good condition of this 171-year-old specimen was surprising. Thus, Rokitansky described the myxoma 75 years before Ribbert's first description of a myxoma in this rare localization.

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Familial Cushing's syndrome with primary adrenocortical microadenomatosis (primary adrenocortical nodular dysplasia)

Abstract: Cushing's syndrome was found in 2 of 4

Cited by 58 publications

References 7 publications

Primary adrenocortical nodular dysplasia with Cusghing's syndrome and cardiac myxomas

Primary adrenocortical nodular dysplasia with Cusghing's syndrome and cardiac myxomas

Primary pigmented nodular adrenocortical disease

Myxoma valvulae arteriae pulmonalis described by Rokitansky: medicohistorical and clinicopathological aspects

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