[Giant bilateral adrenal myelolipoma associated with congenital adrenal hyperplasia].

Abstract

BACKGROUND:

Myelolipoma is a rare benign tumor formed by mature fat tissue with strata of haematopoiesis. It is mainly asymptomatic, only large myelolipomas manifest themselves by abdominal discomfort, bleeding or by symptoms of oppressing adjacent organs. It is usually localized in the region of the adrenal gland. Myelolipomas are mostly clinically inert, only a small number of them are associated with Cushing's type of endocrine disorders, Conn's syndrome, Addison's disease, etc.

PATIENT AND METHODS:

The authors present a rare case of a giant bilateral myelolipoma emerging out of the adrenal gland cortex in a congenital adrenal hyperplasia, with steroid 21-hydroxylase deficiency, in a woman with pronounced virilism. The principal diagnostic methods include sonography and computer tomography. The therapy available is either a surgical extirpation in the case of large or growing myelolipomas or observation in the case of small ones (< 5 cm).

RESULTS:

The condition was resolved by bilateral adrenalectomy with extirpation of both myelolipomas and subsequent hormonal substitution treatment during which there was a gradual regression of virilising symptoms.

CONCLUSIONS:

The coincidence of myelolipoma and congenital disorder with subsequent overproduction of the adrenocorticotropin hormone and androgens, might be explained by the incipient of myelolipoma through chronic hormonal stimulation of the adrenal gland cortex. However, the etiopathogenesis of myelolipoma is still unclear.

• Authors: V Treska, M Wirthová, S Hadravská, P Mukensnábl, V Kuntscher, B Kreuzberg, L Lisá, K Kozák
• Journal: Zentralblatt fur Chirurgie (Zentralbl Chir) Vol. 131 Issue 1 Pg. 80-3 (Feb 2006) ISSN: 0044-409X [Print] Germany
• Vernacular Title: Ein bilaterales Riesenmyelolipom der Nebennieren in Kombination mit kongenitaler adrenaler Hyperplasie.
• PMID: 16485216 (Publication Type: Case Reports, Journal Article)

Chemical References

• Steroid 21-Hydroxylase

Topics

• Adrenal Gland Neoplasms (complications, diagnosis, pathology, surgery)
• Adrenal Glands (pathology)
• Adrenal Hyperplasia, Congenital (complications, diagnosis, pathology, surgery)
• Adrenalectomy
• Female
• Humans
• Middle Aged
• Myelolipoma (complications, diagnosis, pathology, surgery)
• Neoplasms, Multiple Primary (complications, diagnosis, pathology, surgery)
• Steroid 21-Hydroxylase (blood)
• Tomography, X-Ray Computed