CARCINOID SYNDROME
CARCINOID SYNDROME
- Carcinoid syndrome occurs in 10% of carcinoid tumour.
- Carcinoid syndrome consist of following features-
a) Carcinoid tumour is malignant
b) Secondaries in the liver
c) Symptoms are- red- blue cyanosis of skin, flushing and asthmatic attacks, intestinal hyperperistalsis causing diarrhea, pulmonary and tricuspid stenosis with CCF.
- Common in small bowel carcinoid (midgut carcinoid)
- Syndrome releases 5 HT, 5 HTP, histamine, dopamine, bradykinin, prostaglandin, kallikrein, substance P.
- Precipitating factors- stress, alcohol, large meal, sexual intercourse
- Rare manifestations of Carcinoid syndrome includes retroperitoneal fibrosis causing urethral obstruction, Peyronie’s disease of the penis, intraabdominal fibrosis, and occlusion of the mesenteric arteries or veins.
TYPES-
- Foregut Carcinoid (Bronchial, thymic, gastroduodenal, pancreatic)
- Mostly argyrophilic
- MC seen in stomach
- Secrets low levels of serotonin
2. Midgut Carcinoid (Jejunal, ileal, appendiceal, right colon)
- Argentaffin
- Secrets high level of serotonin
3. Hindgut Carcinoid (digital colon, rectum)
- Do not stain with silver
- Secrets somatostanin
ATYPICAL CARCINOID SYNDROME
- Occur in patients with gastric carcinoid tumors (foregut carcinoid).
- These patients experience cutaneous flushes that are patchy and highly pruritic.
- Diarrhea, bronchospasm, and cardiac lesions are rare.
- Normal serotonin levels
- The syndrome is due to large release of histamine from the tumor rather than serotonin
CLINICAL FEATURES-
- Vasomotor
- Flushing- is the MC manifestation (cutaneous flushing)
- Cyanotic violaceous flush with watery eyes
- Red patchy flush seen in carcinoid stomach
2. GIT-
- Diarrhea
- Hepatomegaly
3. Cardiac-
- Pulmonary stenosis > tricuspid insufficiency > tricuspid stenosis features of right valvular fibrosis (inflow)
- Bronchospasm
INVESTIGATION-
- Increase urinary levels of 5 HIAA
TREATMENT-
- Biological agents- somatostanin analogue and interferon α
- Chemotherapy- 5- FU
- Octreotide- decreases flushing and diarrhea
- Treatment of hepatic secondaries- emobolization, chemotherapy, TACE, hepatic artery ligation, radiofrequency ablation
Exam Important
- Carcinoid syndrome consist of following features-
a) Carcinoid tumour is malignant
b) Secondaries in the liver
c) Symptoms are- red- blue cyanosis of skin, flushing and asthmatic attacks, intestinal hyperperistalsis causing diarrhea, pulmonary and tricuspid stenosis with CCF.
- Common in small bowel carcinoid (midgut carcinoid)
- Syndrome releases 5 HT, 5 HTP, histamine, dopamine, bradykinin, prostaglandin, kallikrein, substance P.
- Precipitating factors- stress, alcohol, large meal, sexual intercourse
- Rare manifestations of Carcinoid syndrome includes retroperitoneal fibrosis causing urethral obstruction, Peyronie’s disease of the penis, intraabdominal fibrosis, and occlusion of the mesenteric arteries or veins.
TYPES-
- Foregut Carcinoid (Bronchial, thymic, gastroduodenal, pancreatic)
- Mostly argyrophilic
- MC seen in stomach
- Secrets low levels of serotonin
2. Midgut Carcinoid (Jejunal, ileal, appendiceal, right colon)
- Argentaffin
- Secrets high level of serotonin
3. Hindgut Carcinoid (digital colon, rectum)
- Do not stain with silver
- Secrets somatostanin
ATYPICAL CARCINOID SYNDROME
- Occur in patients with gastric carcinoid tumors (foregut carcinoid).
- These patients experience cutaneous flushes that are patchy and highly pruritic.
- Diarrhea, bronchospasm, and cardiac lesions are rare.
- Normal serotonin levels
- The syndrome is due to large release of histamine from the tumor rather than serotonin
CLINICAL FEATURES-
- Vasomotor
- Flushing- is the MC manifestation (cutaneous flushing)
- Cyanotic violaceous flush with watery eyes
- Red patchy flush seen in carcinoid stomach
2. GIT-
- Diarrhea
- Hepatomegaly
3. Cardiac-
- Pulmonary stenosis > tricuspid insufficiency > tricuspid stenosis features of right valvular fibrosis (inflow)
- Bronchospasm
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