Diagnosis & Surgical Management
of Carcinoid Tumors
PETER J. DIPASCO, MD
ASSISTANT PROFESSOR OF SURGERY
DEPARTMENT OF SURGERY – SECTION OF SURGICAL ONCOLOGY
THE UNIVERSITY OF KANSAS MEDICAL CENTER
FRIDAY, APRIL 4TH, 2014
ACOS GENERAL SURGERY
IN-DEPTH REVIEW
Disclosure
 I have no disclosures
Introduction
 Definition – “Carcinoid”
 Applied to well-differentiated neuroendocrine tumors originating in the
digestive tract, lungs, or rare sites such as kidneys or ovaries
 Is not used to describe high grade / poorly differentiated neuroendocrine
tumors
Epidemiology
 Peak incidence within
the sixth to seventh
decade
 >50% are found within
the GI tract, with the
appendix as a very
common site of origin


50% are found within 2ft
of the ileocecal valve
>30% are multifocal
 Incidence is increasing
 Carcinoid is more
common than SB adenoC
Pathology
 Carcinoids are characterized by the
ability to produce peptides and other
biologically active substances




Serotonin
Amines
Tachykinins
Prostaglandins
 Arise from enterochromaffin cells (GI
tract, bronchi)
 Usually tan, yellow, or gray-brown
intramural or submucosal lesions
 Multifocality comprises 30% of cases
Clinical Findings
 Carcinoid could be referred to as a
“malignant neoplasm in slow
motion”
 At the time of diagnosis…


40% have invaded the muscularis
45% have metastasized to the liver
 Tumors < 1cm rarely metastasize
(2%)
 Tumors > 2cm frequently
metastasize (80%)
 Massive metastatic lesions are also
encountered from minute primaries
Clinical Findings
 Small tumors are often asymptomatic
 30% cause symptoms of obstruction, pain, bleeding,
or the carcinoid syndrome
 Carcinoid syndrome




Cutaneous flushing
Diarrhea
Bronchoconstriction
Right sided heart valvular dysfunction (collagen deposition)
 Carcinoid Crisis
 Usually precipitated by anesthesia, surgery or chemotherapy
Usually refractory to fluid resuscitation or vasopressors
 Octreotide should be given to all pts with metastatic or bulky
disease prior to the above events

Clinical Presentation
 Varies depending on location and production of
biologically active substances
Foregut
Midgut
Hindgut
Clinical Presentation – Foregut
 Most commonly atypical in
presentation due to nonserotonin products (gastrin /
ACTH / GH)
 Pulmonary tumors are usually
perihilar, causing pneumonia,
cough, hemoptysis and even
chest pain
 Gastric carcinoids are mainly
associated with chronic atrophic
gastritis and pernicious anemia
(75%, type I)
Clinical Presentation - Midgut
 Typically only produce symptoms
when they are bulky or metastatic
 Most are located within the distal
one-third of the small bowel

Vast majority of appendiceal carcinoids
are found incidentally
 Symptoms are generally non-
specific colicky abdominal pain
 Mesenteric fibrosis typically
accompanies tumor growth

Can lead to obstruction or ischemia
Clinical Presentation - Hindgut
 Commonly clinically silent
until advanced
 Two-thirds are found in the
ascending colon


Average size at diagnosis is 5cm
Usual presentation is bleeding
 Rectal Carcinoid
 80% are less than 10mm –
endoscopic resection is feasible
and safe
 Muscular and lymphovascular
invasion confer an increased risk of
metastasis
Diagnosis - Laboratory
 50% of patients will have an
elevated urinary 5-HIAA
(regardless if carcinoid syndrome
is present)


This test requires restriction of multiple
food items
Levels can correlate with tumor burden
 Serum Chromogranin A carries of
sensitivity of 80% in well
differentiated tumors

Is also useful in non-functional tumors
(still positive)
Diagnosis - Imaging
 Indium (111In-penetriodide)
labeled Octreotide or
Metaiodobenzylguanidine
radiolabeled with 131I


MIBG is taken up by the tumor and
stored in neurosecretory granules
88% of tumors will be positive
 Anatomically directed
investigations (CT Chest / CXR
/ Bronchoscopy / Endoscopy)


CT typically shows a “spoke-wheel”
appearance on small bowel tumors
70% demonstrate calcifications
Special Consideration – Appendix
 Represents the most
common tumor of the
appendix
 95% less than 2cm
 Rarely metastasize
 LN involvement rare
 75% in the distal third
 Mostly unifocal
Surgery – Localized Disease
 Small bowel – wide en bloc resection including
mesenteric LNs regardless of size



70% will metastasize to LNs
Mesenteric resection may be difficult due to fibrosis
Thorough examination of the entire small bowel is necessary
as 20-40% of tumors are multicentric

40% of patients with midgut carcinoid have a second GI
malignancy
 Rectum – Endoscopic vs TEMS for subcentimeter
 1 – 2cm tumors are also candidates in the absence of LVI or
local invasion
 >2cm – total mesorectal excision
Surgery – Advanced / Metastatic Disease
 Role of surgery not well defined
 If all metastases and primary are resectable, total
extirpation should be performed


Prolongs disease free survival
Provides symptomatic relief

Mean duration for resected liver lesions is 5.3 years
 Prophylactic cholecystectomy should be performed
in all patients

Risk of cholelithiasis with ongoing octreotide treatment
Surgery – Advanced / Metastatic Disease
 Patients with mildly symptomatic disease burden
can be managed with octreotide alone

Controls symptoms

Duration of response was ~1 year

150μg subcutaneous TID

Depot sandostatin

20 – 30mg q4 weeks
 Slows down tumor growth >50% cases
 Causes mild regression 10 – 20% cases
CASE REPORT
 65F with classical findings of




acute appendicitis made on
history and physical
examination alone
Pt taken to the operating room
for laparoscopic appendectomy
Neoplastic process at the tip of
the appendix noted invading
mesoappendix. Frozen section
shows 2.5cm carcinoid tumor
Next step?
What if 4cm left lateral
segment was involved?