Diagnosis & Surgical Management of Carcinoid Tumors
Download
Report
Transcript Diagnosis & Surgical Management of Carcinoid Tumors
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?