Clinically Silent Corticotroph Tumors of the Pituitary Gland

Author:

Scheithauer Bernd W.1,Jaap Alan J.2,Horvath Eva3,Kovacs Kalman3,Lloyd Ricardo V.1,Meyer Fredric B.4,Laws Edward R.5,Young William F.2

Affiliation:

1. Departments of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, Minnesota

2. Departments of Internal Medicine, Mayo Clinic, Rochester, Minnesota

3. Department of Laboratory Medicine, St. Michaels Hospital, University of Toronto, Toronto, Canada

4. Departments of Neurosurgery, Mayo Clinic, Rochester, Minnesota

5. Department of Neurosurgery, University of Virginia, Charlottesville, Virginia

Abstract

ABSTRACT OBJECTIVE To determine the clinical presentation, imaging characteristics, microscopic and ultrastructural characteristics, and treatment outcomes of patients with clinically silent pituitary corticotroph adenomas. METHODS All silent corticotroph adenomas diagnosed at the Mayo Clinic during the years 1975 through 1997 were selected from the files of the Mayo Tissue Registry. RESULTS We studied 23 cases, occurring in 16 male and 7 female patients (age range, 11–79 yr; mean age, 48 yr), who presented with headaches (50%), visual field defects (61%), extraocular muscle paresis (13%), hypopituitarism (26%), and galactorrhea/amenorrhea (43%/29% of the female patients). No patients exhibited clinical hypercortisolism. All tumors were macroadenomas (2.4 ± 0.8 cm; range, 1.5–4.0 cm) and exhibited suprasellar extension in 87% of the cases and hemorrhage, necrosis, and/or cystic changes in 61%. All tumors stained were variably periodic acid-Schiff-, adrenocorticotropic hormone-, and β-endorphin-positive, particularly Subtype I lesions. Ultrastructural classification was performed in 19 cases. In a comparison of Subtype I and II tumors, differences were observed with respect to sex (male/female, 1.4:1 versus 6:1), preoperative hyperprolactinemia (5 of 16 versus 0 of 6 cases), preoperative hypopituitarism (9 of 16 versus 5 of 7 cases), radiographic or gross invasion (7 of 16 versus 5 of 7 cases), and partial or total postoperative pituitary failure (6 of 16 versus 6 of 6 cases). The overall median postoperative follow-up period was 4.9 years (range, 0.3–16.6 yr); 54% of the patients had persistent or recurrent tumors. CONCLUSION Clinically silent corticotroph adenomas behave in an aggressive manner and are characterized by the following: lack of clinical signs or symptoms of Cushing's syndrome and normal cortisol levels; no or only minor elevations of serum adrenocorticotropic hormone levels; macroadenomas with hemorrhagic infarction; and presentation dominated by mass effect symptoms. The high persistence/recurrence rate underscores the need for long-term follow-up.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Neurology (clinical),Surgery

Reference20 articles.

1. Silent corticotroph adenoma: Case report and literature review;Braithwaite;Endocr Pract,1997

2. Endocrine diseases of the dog associated with hair loss, Sertoli cell tumor of testis, hypothyroidism, canine Cushing's syndrome;Coffin;J Am Vet Med Assoc,1953

3. A syndrome of multiorgan hyperplasia with features of gigantism, tumorigenesis, and female sterility in p27Kip1-deficient mice;Fero;Cell,1996

4. Experimental pituitary tumors;Furth;Recent Prog Horm Res,1955

5. Silent corticotropic adenomas of the human pituitary gland;Horvath;Am J Pathol,1980

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