Laparoscopic Resection is Inappropriate in Patients with Known or Suspected Adrenocortical Carcinoma

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Laparoscopic Resection is Inappropriate in Patients with Known or Suspected Adrenocortical Carcinoma

B. S. Miller¹ , J. B. Ammori¹, P. G. Gauger¹, J. T. Broome³, G. D. Hammer² and G. M. Doherty¹

(1)	Division of Endocrine Surgery, University of Michigan, 2920F Taubman Center, 1500 E. Medical Center Drive, Ann Arbor, MI 48109, USA

(2)	Division of Metabolism, Endocrinology, and Diabetes, University of Michigan, Ann Arbor, MI, USA

(3)	Division of Surgical Oncology and Endocrine Surgery, Vanderbilt University, Nashville, TN, USA

Published online: 7 April 2010

Abstract

Background

Complete surgical resection is the mainstay of treatment for patients with adrenocortical cancer (ACC). Use of laparoscopy has been questioned in patients with ACC. This study compares the outcomes of patients undergoing laparoscopic versus open resection (OR) for ACC.

 

Methods

A retrospective review (2003–2008) of patients with ACC was performed. Data were collected for demographics, operative and pathologic data, adjuvant therapy, and outcome. Chi-square analysis was performed.

 

Results

Eighty-eight patients (66% women; median age, 47 (range, 18–81) years) were identified. Seventeen patients underwent laparoscopic adrenalectomy (LA). Median tumor size of those who underwent LA was 7.0 (range, 4–14) cm versus 12.3 (range, 5–27) cm for OR. Recurrent disease in the laparoscopic group occurred in 63% versus 65% in the open group. Mean time to first recurrence for those who underwent LA was 9.6 months (±14) versus 19.2 months (±37.5) in the open group (p < 0.005). Fifty percent of patients who underwent LA had positive margins or notation of intraoperative tumor spill versus 18% of those who underwent OR (p = 0.01). Local recurrence occurred in 25% of the laparoscopic group versus 20% in the open group (p = 0.23). Mean follow-up was 36.5 months (±43.6).

 

Conclusions

ACC continues to be a deadly disease, and little to no progress has been made from a treatment standpoint in the past 20 years. Careful and complete surgical resection is of the utmost importance. Although feasible in many cases and tempting, laparoscopic resection should not be attempted in patients with tumors suspicious for or known to be adrenocortical carcinoma.

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B. S. Miller Email: barbram@umich.edu

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Adrenal Insufficiency

Clinical Background

Adrenal insufficiency is defined as hypofunction of the adrenal gland with decreased or absent cortisol secretion.

Epidemiology

• Incidence – estimated at 5/100,000
• Sex – M:F, equal

Classification

• Primary or secondary insufficiency

Etiology

• Causes of primary adrenal insufficiency
  • Autoimmune  (Addison disease)
    • Frequent association with other endocrine diseases
      • Autoimmune polyendocrine syndromes
        • Epidemiology
          • Incidence – 1-2/100,000
          • Age – usually in 30s
          • Sex – M<F, 1:3
        • Most frequent endocrine abnormality is adrenal insufficiency
        • Type 1
          • Addison disease
          • Chronic mucocutaneous candidiasis
          • Hypoparathyroidism
        • Type 2
          • Diabetes mellitus type 1 (DM1)
          • Addison disease with hypothyroidism – Schmidt syndrome
      • Both types 1 and 2 may also include:
        • DM1
        • Other autoimmune disorders – vitiligo, chronic atrophic gastritis, alopecia
    • Autoantibodies to 21-hydroxylase are frequently present
  • Anatomic destruction of the gland
    • Surgical removal
    • Bilateral hemorrhage into the gland
      • Associated with anticoagulant therapy
    • Invasion of the gland
      • Metastatic cancer is extremely rare
  • Infection
    • Bacterial
      • Tuberculosis
      • Meningococcus (Waterhouse-Friderichsen) – rare
      • Pseudomonas – rare
    • Fungal – histoplasmosis, coccidioidomycosis
    • Viral – cytomegalovirus (CMV), AIDS
  • Congenital adrenal hyperplasia (CAH)
• Causes of secondary adrenal insufficiency
  • Hypopituitarism
    • Postpartum hemorrhage – Sheehan syndrome
    • Pituitary radiation
    • Pituitary surgery
    • Acute interruption of prolonged corticosteroids
    • Pituitary infiltrative disease – tuberculosis, sarcoidosis, Wegener granulomatosis
  • Exogenous glucocorticoid administration
  • Lymphocytic hypophysitis – may be part of autoimmune polyendocrine syndrome

Pathophysiology

• Primary – glucocorticoid and mineralocorticoid deficiency
• Secondary – only glucocorticoid deficiency

Clinical Presentation

• Insidious onset of fatigue, weakness, anorexia, nausea and emesis
• Cutaneous hyperpigmentation – diffuse tan, brown or bronzing
  • Does not occur in secondary insufficiency
• Orthostatic hypotension
• Diarrhea, abdominal pain
• Acute presentation may occur in patients with mild adrenal insufficiency who are stressed (eg, critical illness, surgery)
  • Mainly attributable to mineralocorticoid deficiency
  • Hypotension which is unresponsive to fluids

Treatment

• Primary – glucocorticoids and mineralocorticoids in primary
• Secondary – glucocorticoids only
• Increase glucocorticoid dosing during acute illness

Diagnosis

• Indications for testing – presence of disease process associated with adrenal insufficiency and other symptoms consistent with adrenal insufficiency
• Laboratory testing
  • Initial testing
    • First, measure early morning serum cortisol
      • Cortisol ≥5 µg/dL makes primary adrenal insufficiency less likely; however, if serious consideration is given to this diagnosis, perform stimulation testing
    • Follow with adrenocorticotropic hormone (ACTH) testing
      • If ACTH >300 pg/dL – adrenal failure likely
      • If ACTH <10 pg/dL – pituitary failure likely
      • If ACTH between 10 pg/dL and 300 pg/dL – administer ACHT (cosyntropin) stimulation test
  • Stimulation
    • ACTH stimulation test – cortisol response to cosyntropin (250 µg followed by serial cortisol measures at 30 and 60 minutes)
      • Cortisol <5 µg/dL – adrenal failure
      • Cortisol >20 µg/dL – normal
      • Cortisol ≤20 µg/dL but ≥5 µg/dL – evaluate for pituitary failure
    • Pituitary failure stimulation testing
      • Insulin tolerance testing (ITT)
      • Metyrapone overnight testing
      • CT/MRI anatomic location based on cortisol ratios (see Adrenal Insufficiency Testing Algorithm)
• Imaging studies
  • MRI/CT based on stimulation testing
  • If stimulation testing or absolute cortisol suggests adrenal failure – MRI/CT of adrenal glands
  • If stimulation testing suggests pituitary failure – MRI/CT of pituitary

Differential Diagnosis

• Bronchogenic carcinoma
• Hemochromatosis
• Peutz-Jeghers syndrome
• Tuberculosis
• Congenital adrenal hyperplasia
• Malnutrition

 

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