Case Study

Recognizing and Diagnosing the Subtleties of Carcinoid Syndrome

This case and the associated images have been provided by Rodney Pommier, MD (Professor of Surgery, Division of Surgical Oncology, Oregon Health & Science University, Portland, OR.)

Patient Background

  • 54-year-old postmenopausal woman
  • Married with 2 adult children
  • History of hypertension
  • Previously diagnosed with mild irritable bowel syndrome (IBS) with diarrhea
  1. – Family history is negative for both

Case History

  • Presents to internist for care of hot flashes that have returned 2 years after menopause
  1. – Occur mostly after meals, when she drinks wine and when she goes running
  • Referred to gynecologist
  1. – Physical examination is entirely normal
  2. – Experiences 2 hot flashes with no sweating during the examination
  • Hormone-replacement therapy (HRT) is prescribed
  • Referred to gastroenterologist for IBS treatment

3-Month Follow-up

  • Patient has experienced no improvement of hot flashes
  • A different formulation of HRT is prescribed

12-Month Follow-up

  • Returns to gastroenterologist with worsening of her IBS-like symptoms
  1. – Reports cramping
  2. – Abdominal pain
  3. – Increased episodes of diarrhea
  • Wakes up at night with diarrhea
  • Patient discontinued her HRT medication, feeling they were ineffective
  • Patient is prescribed an antidiarrheal and antispasmodics, for control of diarrhea

21-Month Follow-up

Patient is in need of emergency care

  • Patient presents with acute bowel obstruction and is taken to operating room
  1. – There is marked fibrosis of the terminal ileum with multiple hairpin turns of the bowel and a small tumor in the terminal ileum (Figure 1)

Figure 1. Small-Bowel Obstruction

Small Bowel Obstruction
  • Tumor is surgically resected (Figure 2)

Figure 2. Bowel Resection Surgical Specimen

Small Bowel Obstruction
  • Surgical investigation revealed multiple metastatic tumors in lobes of the liver (Figure 3).

Figure 3. Liver Metastases

Small Bowel Obstruction
  • Biopsies of the liver tumors were taken

Pathological Report

Small bowel and cecal resection reveals:

  • Carcinoid tumor, 1.5 cm, with intense peritoneal sclerosis
  • Metastatic carcinoid in 10 of 23 mesenteric lymph nodes
  • Liver masses determined to be metastatic carcinoid tumors

Diagnostic Conclusion

  • The patient had carcinoid syndrome

Oncologist Consulted

Postsurgical follow-up with an oncologist included:

  • OctreoScan™*
  • 5-hydroxyindoleacetic acid (5-HIAA) and chromogranin A (CgA) level evaluation
  1. – Both levels were markedly elevated
  • Patient was advised to maintain a record of symptom frequency and severity
  • Follow-up appointment scheduled

*OctreoScan is a trademark of Mallinckrodt Pharmaceuticals

Decipher the Evidence Early

Evidence

  • The patient was experiencing nocturnal diarrhea

Conclusion

  • IBS-associated diarrhea stops during sleep, so this patient may not be experiencing IBS

Evidence

  • Hot flashes were not associated with sweating and did not improve with HRT

Conclusion

  • Menopausal hot flashes and most other causes of flushing are associated with sweating, so the patient may not be experiencing menopausal hot flashes
  • Carcinoid-associated flushing has no sympathetic component, and thus is "dry"

Evidence

  • The patient's diarrhea was exacerbated by eating, drinking wine, and running

Conclusion

  • Carcinoid syndrome is exacerbated by:
  1. – Spicy food
  2. – Epinephrine
  3. – Stress
  4. – Alcohol
  5. – Physical exertion
  • This patient may be experiencing carcinoid syndrome

Carcinoid Tumors—Diagnose early to help gain control over
debilitating symptoms

  • Many patients with carcinoid tumors remain misdiagnosed for up to 5 to 7 years1–3
  • The 5-year survival rate decreases based on the extent of metastatic disease4*
  1. – 78.2% survival rate with localized tumor
  2. – 71.7% survival rate with regional metastases
  3. – 38.5% survival rate with distant metastases
  • Carcinoid syndrome can be commonly misdiagnosed as IBS or menopause

If Carcinoid Syndrome is Suspected:

  • 5-HIAA and serum CgA level testing can help confirm the diagnosis
  • OctreoScanTM is helpful for imaging and tumor staging

* These data are from The National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) 1992-1999 Registry.

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References
  1. Modlin IM, Moss SF, Chung DC, Jensen RT, Snyderwine E. Priorities for improving the management of gastroenteropancreatic neuroendocrine tumors. J Natl Cancer Inst. 2008;100:1282-1289.
  2. Toth-Fejel S, Pommier RF. Relationships among delay of diagnosis, extent of disease, and survival in patients with abdominal carcinoid tumors. Am J Surg. 2004;187:575-579.
  3. Oberg K, Castellano D. Current knowledge on diagnosis and staging of neuroendocrine  tumors. Cancer Metastasis Rev. 2011;30(suppl 1):3-7.
  4. Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003;97:934-959.