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doi: 10.1007/s11102-005-5348-y.

Pituitary pathology in Carney complex patients

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Pituitary pathology in Carney complex patients

Sotirios G Stergiopoulos et al. Pituitary.2004.

Abstract

Carney complex (CNC) is a familial multiple neoplasia syndrome with features overlapping those of McCune-Albright syndrome (MAS) and multiple endocrine neoplasia (MEN) type 1 (MEN 1). Like MAS and MEN 1 patients, patients with CNC develop growth hormone (GH)-producing pituitary tumors. Occasionally, these tumors are also prolactin-producing, but there are no isolated prolactinomas or other types of pituitary tumors. In at least some patients with CNC, the pituitary gland is characterized by hyperplastic areas; hyperplasia appears to involve somatomammotrophs only. Hyperplasia most likely precedes the formation of GH-producing adenomas in CNC, as has been suggested in MAS-related somatotropinomas, but has never been seen in MEN 1 patients. In at least one case of a patient with CNC and advanced acromegaly, a GH-producing macroadenoma showed extensive genetic changes at the chromosomal level. So far, half of the patients with CNC have germline inactivating mutations in the PRKAR1A gene; in their pituitary tumors, the normal allele of the PRKAR1A gene is lost. Loss-of-hererozygosity suggests that PRKAR1A, which codes for the regulatory subunit type 1alpha of the cAMP-dependent protein kinase A (PKA) may act as a tumor-suppressor gene in CNC somatomammotrophs. These data provide evidence for a PRKAR1A-induced somatomammotroph hyperpasia in the pituitary tissue of CNC patients; hyperplasia, in turn may lead to additional genetic changes at the somatic level, which then cause the formation of adenomas in some, but not all, patients.

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Figures

Fig. 1
Fig. 1
Hyperplasia (and tumor tissue) in the pituitary gland from a CNC patient with acromegaly and a GH-producing pituitary adenoma (case 3,Table 2): immuno-stainings with antibodies specific for (A) reticulin; (B) GH; (C) PRL; and (D) LH β-subunit (all at ×20).
Fig. 2
Fig. 2
Panels A and B are from case 1 (Table 1): (A) Abnormally expanded and irregular reticulin pattern, consistent with adenohypophesial cell hyperplasia (×100); (B) disrupted reticulin pattern in the adenomatous tissue from the same patient (×40).
Fig. 2
Fig. 2
Panels A and B are from case 1 (Table 1): (A) Abnormally expanded and irregular reticulin pattern, consistent with adenohypophesial cell hyperplasia (×100); (B) disrupted reticulin pattern in the adenomatous tissue from the same patient (×40).
Fig. 3
Fig. 3
Electron microscopy images from the same patient (case 3, Table 2) with GH- and LH-producing tumorlets [A and B] (7,682× and 5,380× respectively). Secretory granules are identified by GH-specific antibody [C] (14,200×) and double immunostaining for both GH and LH [D] (18,200×).
Fig. 3
Fig. 3
Electron microscopy images from the same patient (case 3, Table 2) with GH- and LH-producing tumorlets [A and B] (7,682× and 5,380× respectively). Secretory granules are identified by GH-specific antibody [C] (14,200×) and double immunostaining for both GH and LH [D] (18,200×).
Fig. 3
Fig. 3
Electron microscopy images from the same patient (case 3, Table 2) with GH- and LH-producing tumorlets [A and B] (7,682× and 5,380× respectively). Secretory granules are identified by GH-specific antibody [C] (14,200×) and double immunostaining for both GH and LH [D] (18,200×).
Fig. 3
Fig. 3
Electron microscopy images from the same patient (case 3, Table 2) with GH- and LH-producing tumorlets [A and B] (7,682× and 5,380× respectively). Secretory granules are identified by GH-specific antibody [C] (14,200×) and double immunostaining for both GH and LH [D] (18,200×).
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References

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