Carcinoid Tumor

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Carcinoid Tumor

• Victor Ghobrial, MDTemple UniversityConemaugh
  Memorial Hospital

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HPI

• 80 yrs WF admitted to hospital C/O abdominal pain
  and weakness
• Pain started 12 months ago and was at max on
  admission
• Located mid abdomen but radiates to lower
  quadrants bilaterally
• Pain is colicky, waxes and wanes over the course
  of days
• Pain associated with nausea and diarrhea

3
PMH

• No significant history
• Pt is non smoker, non drinker, widow and has
  three children
• Cholecystectomy
• Appendectomy

4
History

• Social Hx worked in multiple jobs, retired 25
  yrs ago
• ROS abdominal pain, nausea and vomiting, hot
  flashes, night sweats
• No other symptoms

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Physical Exam

• Vitals WNL
• HEENT unremarkable
• Neck supple, no lymphadenopathy
• Ht RRR, no murmurs
• Lungs CTAs B/L
• Abdomen soft, moderate tenderness to palpation,
  no masses
• Exter no E,C,C
• Neuro no focal deficits

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Labs

• Hgb Hct WBC Plat
  
  9.5 29 11.1 301
• Na K Cr BUN Cl CO2
  127 3.9 0.4 13
  93 29
• Alka Phos
  149

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Our Patient

• 80 yrs c/o colicky abd pain for one yr
• Admitted with increasing discomfort and
  weakness with inability to eat
• Diffusely tender abdomen
• Hyponatremic

8
Hospital Course

• Upper GI small-bowel follow through sliding
  hiatal hernia
• EGD antral gastritis
• Colonoscopy sever diverticulosis with stricture
  of sigmoid colon
• Sigmoid colectomy did not relieve abd pain
• Mesinteric angiography was negative

9
Colonic Bx

• Diverticulosis and tumour nests in the wall
  including serosa and muscularis propria
• Cells strongly ve for NSE and Cytokeratin highly
  suggestive of neuroendocrine tumour such as
  carcinoid tumour

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Hospital Course

• CT of chest small nodules non diagnostic
• 5-HIAA in urine was 14 (Normal 6)
• PIC line inserted for TPN
• Primary of a carcinoid tumour was not found
• Pt placed on Sandostatin, Kytril and Diludid
• Eventually pt expired two months later.

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Carcinoid tumors

• Rare, but are the most common gastrointestinal
  neuroendocrine tumors.
• Symptoms of the carcinoid syndrome are infrequent
  occurring in approximately 10 of patients with
  small bowel carcinoid.

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Age

• the age distribution of carcinoid tumors ranges
  from the 2nd to the 9th decade
• peak incidence occur between the ages of 50 and 70

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PATHOLOGY

• Carcinoid tumors were so named because they
  seemed morphologically different and clinically
  less aggressive than the more common intestinal
  adenocarcinoma
• Carcinoids arise from enterochromaffin cells of
  the gastrointestinal tract. The term
  enterochromaffin refers to the ability to stain
  with potassium chromate (chromaffin), a feature
  of cells that contain serotonin

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ORGAN-RELATED FEATURES

• The clinical characteristics of carcinoid tumors
  vary with the location of the tumor.

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Appendix

• Carcinoid tumors are the most common neoplasms in
  the appendix.
• Approximately 1 in 300 appendixes contain a
  carcinoid tumor.
• They arise from endocrine cells in the lamina
  propria and submucosa.
• And are detected most commonly in the patients in
  their 40s or 50s.

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Prognosis of appendiceal carcinoids

• It is best predicted by the size of the tumor.
  Tumors ? 2 cm in size (found in approximately 95
  of patients) are unlikely to have metastasized
  when diagnosed
• In contrast, up to 30 of larger tumors have
  already metastasized at diagnosis
• Five year survival in patients with distant
  metastasis is approximately 30

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Small intestine

• Carcinoid tumors of the small intestine represent
  approximately one-third of small intestine
  neoplasms .
• The tumors are felt to arise from intraepithelial
  endocrine cell, in contrast to appendiceal
  carcinoids, which arise from subepithelial
  endocrine cells.
• Small intestinal carcinoid tumors are most
  commonly located in the ileum within 60 cm of
  ileocecal valve. Multiple tumors are present in
  up to 30 percent of patients

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Clinical Presentation

• Patients usually present in their 60s or 70s with
  abdominal pain or small bowel obstruction, and
  often with metastases to lymph nodes or the liver
• Bowel obstruction or abdominal pain may be due to
  intussusception, the mechanical effect of the
  tumor, or mesenteric ischemia due to local
  fibrosis or angiopathy

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Colon

• Colonic carcinoids are usually detected in
  patients who are in their 70s during evaluation
  for abdominal pain, anorexia, or weight loss.
• The majority of tumors are located in the right
  colon, particularly the cecum
• Because of the high capacitance of the right
  colon, most patients do not become symptomatic
  until the tumors are large.

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Rectum

• The vast majority of rectal carcinoids are found
  incidentally by rectal examination or endoscopy.
  They are most commonly diagnosed in patients in
  their 60s.
• The size of rectal carcinoids correlates closely
  with the likelihood of metastases. Tumors smaller
  than 2 cm are rarely metastatic, while 10 percent
  of larger tumors metastasize, most often to local
  lymph nodes or the liver

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Stomach

• Gastric carcinoids are relatively uncommon. They
  are grouped into three categories
• Type 1 gastric carcinoids are associated with
  chronic atrophic gastritis and often pernicious
  anemia they account for 70 to 80 percent of all
  gastric carcinoids.

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Type 2

• Type 2 gastric carcinoids occur in association
  with gastrinomas (Zollinger-Ellison syndrome) or
  multiple endocrine neoplasia (MEN) type 1.
• They account for less than 5 percent of gastric
  carcinoids. Similar to carcinoids in atrophic
  gastritis, the tumors are thought to arise from
  ECL cells.

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Type 3

• Type 3 gastric carcinoids are known as sporadic
  carcinoids because they occur in the absence of
  atrophic gastritis or the Zollinger-Ellison or
  MEN-1 syndromes.
• They account for 20 percent of gastric
  carcinoids, and are the most aggressive local or
  hepatic metastases are present in up to 65
  percent of patients who come to resection

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Lung

• Pulmonary carcinoids are classified among other
  pulmonary neuroendocrine tumors such as small
  cell lung cancer.
• The tumors are thought to arise from Kulchitsky's
  cells within the bronchial mucosa Most tumors are
  perihilar.

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Clinical Presentation

• Patients usually present in their 50s with
  recurrent pneumonia, cough, hemoptysis or chest
  pain
• The tumors are capable of secreting several
  different hormones, which can produce a number of
  paraneoplastic syndromes including Cushing's
  syndrome, acromegaly, and the carcinoid syndrome,
  which occurs in less than 5 of patients

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Ovary

• Rare carcinoids arising in ovarian teratomas are
  noteworthy because they can produce the carcinoid
  syndrome without hepatic metastases due to their
  direct drainage into the systemic circulation

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CLINICAL FEATURES

• The majority of patients with carcinoid tumors
  are asymptomatic, and the tumor is diagnosed
  incidentally at endoscopy, surgery, or autopsy.
• The initial symptoms of carcinoid may be
  nonspecific. When present, symptoms correlate
  with the location and extent of the tumor

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Carcinoid Syndrome

• This is the term applied to a constellation of
  symptoms mediated by various humoral factors
  elaborated by some carcinoid tumors.
• These tumors synthesize, store, and release a
  variety of polypeptides, biogenic amines, and
  prostaglandins

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Tryptophan metabolism

• In normal subjects, one percent of dietary
  tryptophan is converted to serotonin however,
  this value may increase to 70 percent or more in
  patients with the carcinoid syndrome .
• Serotonin is then metabolized to
  5-hydroxyindoleacetic acid (HIAA)

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Amine products

• Histamine responsible for the atypical flushing
  associated with these tumors
• Kallikrein a protein that cleaves kinin from
  plasma kininogens, vasodilator, stimulates
  intestinal motility and increases vascular
  permeability.
• Prostaglandins Prostaglandins E and F stimulate
  intestinal motility and fluid secretion in the
  normal gastrointestinal tract
• Polypeptides insulin, corticotropin (ACTH),
  gastrin, vasoactive intestinal peptide, or
  somatostatin,

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CLINICAL FEATURES

• Cutaneous flushing Episodic flushing is the
  clinical hallmark of the carcinoid syndrome, and
  occurs in 85 percent of patients.
• Diarrhea Secretory diarrhea occurs in 80
  percent of patients and is often the most
  debilitating component of the syndrome.

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CLINICAL FEATURES

• Bronchospasm Ten to twenty percent of patients
  with the carcinoid syndrome have wheezing and
  dyspnea, often during flushing episodes
• Cardiac valvular lesions Pathognomonic
  plaque-like deposits of fibrous tissue develop in
  the heart in approximately 3 to 4 percent of all
  patients with carcinoid tumors

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BIOCHEMICAL TESTING

• Urinary excretion of 5-HIAA The most useful
  initial diagnostic test for the carcinoid
  syndrome is to measure 24-hour urinary excretion
  of 5-hydroxyindoleacetic acid (HIAA), which is
  the end product of serotonin metabolism
• This test has a sensitivity of 75 percent and
  specificity of up to 100 percent

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Medical Rx

• .Somatostatin analog Octerotide Flushing and
  diarrhea are initially relieved in 75 to 80
  percent of patients treated with 50 µg three
  times daily subcutaneously . A longer acting
  somatostatin analogue (Sandostatin LAR Depot) is
  easier to use than the short acting preparation.
• Patients with diarrhea could be treated with
  cyproheptadine, a serotonin antagonist, while
  those with gastric carcinoids that elaborate
  histamine would be treated a histamine blocker.