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Acromegaly: Differential Diagnoses & Workup

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Santiago A Centurion, MD, Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey
Contributor Information and Disclosures

Updated: Nov 9, 2009

Differential Diagnoses

Pachydermoperiostosis

Other Problems to Be Considered

Pseudoacromegaly is defined as the presence of acromegaloid features in the absence of elevated levels of GH or IGF-I in patients with severe insulin resistance.

Pachydermoperiostosis syndrome is manifested clinically by finger clubbing, extremity enlargement, hypertrophic skin changes, and periosteal bone formation.

Cancer risk is controversial in acromegalic patients.7 Patients with acromegaly may have an increased risk of developing colorectal, breast, and prostate tumors.8  However, the prevalence of cancers in patients with acromegaly remains controversial. A 2008 study suggested that patients with acromegaly have an increased risk of colon cancer and polyps, and the risk for thyroid cancer is increased in males. Patients with acromegaly might be advised to undergo screening colonoscopy and thyroid ultrasonography.

Workup

Laboratory Studies

  • Patients with active acromegaly have abnormal dynamics of GH secretion. A simple diagnostic approach is to measure serum GH 1 hour after oral administration of 100 g of glucose. Clearly elevated GH levels (>10 ng/mL) after oral glucose, combined with the clinical picture, secure the diagnosis of acromegaly, while a normal GH level (<5 ng/mL) after oral glucose essentially excludes the diagnosis.
    • Only a small percentage of patients investigated for acromegaly have a postglucose GH level that is intermediate (5-10 ng/mL). In these patients, other tests can be used to define their status.
    • Measurements of SM-C reflect the integrated production of GH. This marker also serves as a useful screening test to exclude acromegaly.
    • Since IGF-I has a long half-life, its measurement is useful to gauge integrated GH secretion, to screen for acromegaly, and to monitor the efficacy of therapy.
    • IGF-I concentrations vary with age. An assay is required in which the normal ranges have been stratified to account for this discrepancy.
    • Starvation, obesity, and diabetes mellitus decrease IGF-I concentration. Pregnancy increases IGF-I concentration.
    • Before immunoassays for IGF-I, GH measurement was the only method used in the biochemical assessment of acromegaly.9 IGF-I has been the most reliable biochemical indicator of acromegaly. The availability of supersensitive GH has changed many aspects of the interpretation of the GH value. Hypersecretion and abnormal neuroregulation characterize acromegaly. GH can be measured in many ways to give useful information on diagnosis, therapy, and prognosis. Measuring GH in the management of acromegaly complements the information IGF-I values provide.
    • Random GH measurements often are not diagnostic because of the episodic secretion of GH, its short half-life, and the overlap between GH concentration in individuals with acromegaly and individuals without the condition.
  • The definitive test for the diagnosis of acromegaly is the oral glucose tolerance test. A paradoxic rise in GH concentration is seen in 15-20% of patients with acromegaly following oral glucose administration.
  • Measurement of IGF binding protein 3, the primary binding protein for circulating IGF, is increased in acromegaly and may be useful in the diagnosis of acromegaly. It also may be helpful for monitoring the activity of the disease during treatment.
  • GHRH concentration may be obtained if clinically indicated. Levels higher than 300 pg/mL usually indicate an ectopic source of GHRH. In pituitary disease (GHRH independent), GHRH concentration is normal or suppressed.
  • Since as many as 20% of GH-secreting pituitary adenomas co-secrete prolactin, the prolactin level also may be elevated; however, a rise in prolactin may result from stalk compression or from co-secretion from a pituitary adenoma. Pituitary adenomas may be associated with deficiencies of other pituitary hormones. Consider evaluation of the adrenal, thyroid, and gonadal axes.

Imaging Studies

  • Obtain imaging studies only after a firm biochemical diagnosis of acromegaly has been made because of the relatively high incidence of nonfunctioning incidentally discovered pituitary adenomas.
  • Image the sella turcica first, since GH-secreting pituitary adenoma is the most common cause.
  • MRI is more sensitive than CT scan.
    • MRI provides detailed information about surrounding structures (eg, optic chiasm, cavernous sinuses).
    • If MRI findings of the sella are negative, appropriate studies to localize tumors causing ectopic secretion of GH or GHRH may be obtained.
    • Use CT scan of the abdomen/pelvis to evaluate for pancreatic, adrenal, and ovarian tumors secreting GH/GHRH.
    • Use chest CT scan to evaluate for bronchogenic carcinoma secreting GH/GHRH.
  • Radiographic studies show the following:
    • Increase in length and thickness of the mandible is noted.
    • Underbites can be recognized as a result of mandible enlargement.
    • Calvaria are thickened.
    • Bony ridges and muscle attachments are exaggerated.
    • Frontal, mastoid, and ethmoid sinuses are enlarged.
    • Ribs are elongated because of proliferation at the cartilage-bone junction.
    • Deep barrel chest often is pronounced in long-standing acromegaly as a result of continued costal growth.
    • Periosteal growth of the vertebrae occurs, and osteophytic proliferation of the articular margins of joints frequently is recognized.
    • Cartilage proliferation of the larynx is noted.
    • Cortical thickening and distal tufting are noted.
    • Deformities of the skull are noted.

Histologic Findings

Surgical specimens from pituitary tumor demonstrate a variety of histologic findings as follows:

  • Densely granulated somatotrope adenoma
  • Sparsely granulated somatotrope adenoma
  • Mixed somatotrope-lactotrope adenoma
  • Acidophilic stem cell adenoma
  • Mammosomatotroph adenoma
  • Plurihormonal adenoma producing GH and 1 or more glycoprotein hormones, principally alpha
  • Somatotrope carcinoma
  • Somatotrope hyperplasia
  • No distinct morphologic change

Skin biopsy may demonstrate histologic findings as follows:

  • Slight thinning of the epidermis
  • Papillary and upper reticular dermis (may appear edematous and myxoid)
  • Separation of the collagen fibers
  • Slightly increased number of fibroblasts
  • Fibrous component normal, both qualitatively and quantitatively
  • Observation of a dense glycosaminoglycan deposit (most consistent abnormality)
  • Infiltration by glycosaminoglycans (most prominent in papillary, upper reticular dermis, and in vicinity of sweat glands)

More on Acromegaly

Overview: Acromegaly
Differential Diagnoses & Workup: Acromegaly
Treatment & Medication: Acromegaly
Follow-up: Acromegaly
References
[ CLOSE WINDOW ]

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Further Reading

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Keywords

acromegaly, growth hormone, GH, gigantism, GH-secreting pituitary adenomas, somatomedins, SMs, somatotropin-mediating hormones, insulinlike growth factors, IGFs

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Santiago A Centurion, MD, Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey
Santiago A Centurion, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Shyam Verma, MBBS, DVD, FAAD, Adjunct Clinical Assistant Professor, Department of Dermatology, University of Virginia, State University of New York at Stonybrook, Penn State University
Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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