Find information on thousands of medical conditions and prescription drugs.

Carcinoid syndrome

Carcinoid syndrome refers to the array of symptoms that occur secondary to carcinoid tumors. Carcinoid tumors are discrete, yellow, well-circumscribed tumors that can occur anywhere along the gastrointestinal tract (GI). They most commonly affect the appendix, ileum, and rectum. These tumors are unique in that they are endocrine in nature. They secrete hormones into the blood stream, which then travel to end organs and act upon them via appropriate receptors. Although quite rare, 15 cases/1,000,000 population, carcinoid tumors account for 75% of GI endocrine tumors. more...

Home
Diseases
A
B
C
Angioedema
C syndrome
Cacophobia
Café au lait spot
Calcinosis cutis
Calculi
Campylobacter
Canavan leukodystrophy
Cancer
Candidiasis
Canga's bead symptom
Canine distemper
Carcinoid syndrome
Carcinoma, squamous cell
Carcinophobia
Cardiac arrest
Cardiofaciocutaneous...
Cardiomyopathy
Cardiophobia
Cardiospasm
Carnitine transporter...
Carnitine-acylcarnitine...
Caroli disease
Carotenemia
Carpal tunnel syndrome
Carpenter syndrome
Cartilage-hair hypoplasia
Castleman's disease
Cat-scratch disease
CATCH 22 syndrome
Causalgia
Cayler syndrome
CCHS
CDG syndrome
CDG syndrome type 1A
Celiac sprue
Cenani Lenz syndactylism
Ceramidase deficiency
Cerebellar ataxia
Cerebellar hypoplasia
Cerebral amyloid angiopathy
Cerebral aneurysm
Cerebral cavernous...
Cerebral gigantism
Cerebral palsy
Cerebral thrombosis
Ceroid lipofuscinois,...
Cervical cancer
Chagas disease
Chalazion
Chancroid
Charcot disease
Charcot-Marie-Tooth disease
CHARGE Association
Chediak-Higashi syndrome
Chemodectoma
Cherubism
Chickenpox
Chikungunya
Childhood disintegrative...
Chionophobia
Chlamydia
Chlamydia trachomatis
Cholangiocarcinoma
Cholecystitis
Cholelithiasis
Cholera
Cholestasis
Cholesterol pneumonia
Chondrocalcinosis
Chondrodystrophy
Chondromalacia
Chondrosarcoma
Chorea (disease)
Chorea acanthocytosis
Choriocarcinoma
Chorioretinitis
Choroid plexus cyst
Christmas disease
Chromhidrosis
Chromophobia
Chromosome 15q, partial...
Chromosome 15q, trisomy
Chromosome 22,...
Chronic fatigue immune...
Chronic fatigue syndrome
Chronic granulomatous...
Chronic lymphocytic leukemia
Chronic myelogenous leukemia
Chronic obstructive...
Chronic renal failure
Churg-Strauss syndrome
Ciguatera fish poisoning
Cinchonism
Citrullinemia
Cleft lip
Cleft palate
Climacophobia
Clinophobia
Cloacal exstrophy
Clubfoot
Cluster headache
Coccidioidomycosis
Cockayne's syndrome
Coffin-Lowry syndrome
Colitis
Color blindness
Colorado tick fever
Combined hyperlipidemia,...
Common cold
Common variable...
Compartment syndrome
Conductive hearing loss
Condyloma
Condyloma acuminatum
Cone dystrophy
Congenital adrenal...
Congenital afibrinogenemia
Congenital diaphragmatic...
Congenital erythropoietic...
Congenital facial diplegia
Congenital hypothyroidism
Congenital ichthyosis
Congenital syphilis
Congenital toxoplasmosis
Congestive heart disease
Conjunctivitis
Conn's syndrome
Constitutional growth delay
Conversion disorder
Coprophobia
Coproporhyria
Cor pulmonale
Cor triatriatum
Cornelia de Lange syndrome
Coronary heart disease
Cortical dysplasia
Corticobasal degeneration
Costello syndrome
Costochondritis
Cowpox
Craniodiaphyseal dysplasia
Craniofacial dysostosis
Craniostenosis
Craniosynostosis
CREST syndrome
Cretinism
Creutzfeldt-Jakob disease
Cri du chat
Cri du chat
Crohn's disease
Croup
Crouzon syndrome
Crouzonodermoskeletal...
Crow-Fukase syndrome
Cryoglobulinemia
Cryophobia
Cryptococcosis
Crystallophobia
Cushing's syndrome
Cutaneous larva migrans
Cutis verticis gyrata
Cyclic neutropenia
Cyclic vomiting syndrome
Cystic fibrosis
Cystinosis
Cystinuria
Cytomegalovirus
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
D
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

Pathophysiology

Carcinoid tumors secrete vasoactive substances such as serotonin, histamine, catecholamines, and prostaglandins. These hormones acts upon many tissues of the body including the central nervous system, blood vessels of the intestinal tract, and platelets, ultimately altering blood flow. Prior to metastasis, the liver is capable of metabolizing most of the hormones elaborated by such tumors, rendering patients asymptomatic. Only about 5% of patients with carcinoid tumors ever develop symptoms of the carcinoid syndrome which includes, flushing, diarrhea, hypotension, edema, asthma-like bronchoconstriction attacks, and valvular heart disease. The onset of these symptoms suggests that the tumor has metastasized to the liver, allowing serotonin and its various by-products to reach systemic circulation.

Diagnosis

With a certain degree of clinical suspicion, diagnosis is made by measuring the 24 hour urine levels of 5-HIAA (5-hydroxyindoleacetic acid), a breakdown product of serotonin. Patients with carcinoid syndrome usually excrete >25 mg of 5-HIAA per day.

Treatment

For symptomatic relief of carcinoid sydrome:

  • Octreotide (somatostatin analogue- neutralizes serotonin and decreases urinary 5-HIAA)
  • Methysergide maleate (antiserotonin agent but not used because of serious side effect of retroperitoneal fibrosis)
  • Cyproheptadine (antihistamine)

Alternative treatment for qualifying candidates:

  • Surgical resection of tumor and chemotherapy (5-FU and doxorubicin)

Prognosis

Prognosis varies from individual to individual. It ranges from a 95% 5 year survival for localized disease to a 20% 5 year survival for those with liver metastases. However, median survival for patients with carcinoid sydrome is 2.5 years from the first episode of flushing.

Synonyms

Thorson-Bioerck syndrome, argentaffinoma syndrome, Cassidy-Scholte sydrome, flush syndrome

Read more at Wikipedia.org


[List your site here Free!]


Intraoperative OctreoScan and management of bronchial carcinoid - Letter to the Editor
From CHEST, 10/1/02

To the Editor

We read with interest the article of Rodriguez et al (March 2002), (1) in which they pointed out the importance of [sup.111]Indiethylenetriamine pentaacetic acid pentetreotide (OctreoScan; Mallinckrodt-Tyco; St. Louis, MO) scintigraphy in the preoperative diagnosis and intraoperative management of bronchial carcinoid. In 1997, we (2) published our preliminary results about our experience with OctreoScan in the preoperative diagnosis and postoperative follow-up of neuroendocrine and nonneuroendocrine tumors of the lung, OctreoScan is effective in detecting both primary neuroendocrine bronchial tumors and their mediastinal metastases with higher accuracy than thoracic CT scan. Positive scintigraphy results in nonneuroendocrine lung tumors (especially in undifferentiated large cell carcinomas) are probably due to the presence of activated lymphocytes surrounding the surface of the tumor. (2) We demonstrated how OctreoScan was effective in the diagnosis of liver metastases of resected atypical carcinoid with carcinoid syndrome (3); a positive OctreoScan result can, in addition, influence medical therapy with octreotide or lanreotide (two current generations of somatostatin analogs) because of the expression of the tumor tissue receptors for somatostatin. (3)

The authors used OctreoScan to obtain a preoperative diagnosis of neuroendocrine lung tumor, (1) and they performed a fight thoracotomy with a wedge resection of the lower and the middle lobes, guided by intraoperative octreotide scintigraphy. (1) They did not perform an anatomic resection or a systemic mediastinal lymphadenectomy. (1) Further, no information about the definitive histologic type of the resected bronchial carcinoid, clinical staging, and adjuvant therapy is present in the article.

It is well known that bronchial carcinoids are a part of the spectrum of neuroendocrine tumors of the lung (4); they are low-grade malignant tumors, which may be locally invasive or may spread to mediastinal lymph nodes, or distantly. (5) Mediastinal and distant metastases are more frequent in the atypical form. (5,6) Thus, preoperative diagnosis is mandatory inselecting the extent of surgical resection of the tumor. For a large neoplastic lesion such as this (6.5 cm in size), bronchoscopy or fine-needle aspiration biopsy is usually effective. The authors describe a nondiagnostic bronchoscopy, but they did not perform a transthoracic biopsy. The preoperative diagnosis of neuroendocrine tumor was obtained only by the positivity of the OctreoScan finding and the presence of high serotonin and 5-hydroxyindolacetic acid levels.

The current surgical management is influenced by the histologic type, the recurrence rate, and the survival patterns of neuroendocrine tumors, as described in literature (6-9); anatomic resections (lobectomy if pulmonary function test results are adequate, or segmentectomy in other cases) with systematic lymphadenectomy are mandatory, to reduce the risk of recurrence.

The authors performed a first wedge resection in the lower lobe, completed by another in the middle one, because of the presence of residual tumor in the scintigraphic intraoperative procedure. They did not perform an intraoperative histologic examination of a specimen of the tumor to confirm its neuroendocrine nature and to distinguish it from a small cell carcinoma. or a lymphadenectomy. (1)

We think that intraoperative OctreoScan might be effective in detecting micrometastases of neuroendocrine tumors, in lung tissue or in the mediastinum, but the surgeons must operate to achieve an oncologic radical resection of the lesion and avoid its recurrences. We suggest to the authors to use OctreoScan in the follow-up of these patients, because of its effectiveness in early detection of metastases or possible recurrences.

Correspondence to: Pier Luigi Filosso, MD, University of Torino, San Giovanni Battista Hospital, Department of Thoracic Surgery, Via Genova, 3 10126 Torino, Italy: e-mail: pierluigifilosso@tiscalinet.it

REFERENCES

(1) Rodriguez JA, Meyers MO, Jacome TH, et al, Intraoperative detection of a bronchial carcinoid with a radiolabeled somatostatin analog. Chest 2002; 121:985-988

(2) Oliaro A, Filosso PL, Bello M, et al. Use of [sup.111]In-DTPA-octreotide scintigraphy in the diagnosis of neuroendocrine and non-neuroendocrine tumors of the lung: preliminary results. J Cardiovasc Surg 1997; 38:313-315

(3) Filosso PL, Ruffini E, Rena O, et al. Long-term survival of atypical bronchial carcinoids with liver metastases, treated with octreotide. Eur J Cardiothorac Surg 2002; 21:91:3-917

(4) Travis WD, Colby TV, Corrin B, et al. Histological typing of lung and pleural tumours. 3rd ed. Berlin, Germany: Springer. 1999

(5) Filosso PL, Rena O, Donati G, et al. Bronchial carcinoid tumors: surgical management and long-term outcome. J Thorac Cardiovasc Surg 2002; 123:303-309

(6) Marty-Ane CH, Costes V, Pujol JL, et al. Carcinoid tumors of the lung: do atypical features require aggressive management? Ann Thorac Surg 1995; 59:78-83

(7) Travis W, Rush V, Flieder D, et al, Survival analysis of 200 pulmonary, neuroendocrine tumors with classification of criteria for atypical carcinoid and its separation from typical carcinoid. Am J Surg Pathol 1995; 22:934-944

(8) Ferguson MK, Landreneau RJ, Hazelrigg SR. et al. Long-term outcome after resection for bronchial carcinoid tumors. Eur J Cardiothorac Surg 2000; 18:156-161

(9) El Jamal M, Nicholson AG, Goldstraw P. The feasibility of conservative resection for carcinoid tumours: is pneumonectomy ever necessary for uncomplicated cases? Eur J Cardiothorac Surg 2000; 18:301-306

To the Editor:

Thanks to Filosso and associates for their comments on our article on bronchial carcinoid that appeared in CHEST (March 2002). (1) They refer to their report in 1997 in the Journal of Cardiovascular Surgery, (2) which we did not reference, as we acknowledged the work of Krenning and associates, (3) which antedated the work of Filosso and colleagues by a decade. Dr. Filosso points out that we did not perform an anatomic bilobectomy or a systematic mediastinal lymphadenectomy. It should be recalled that the patient had moderately severe mitral regurgitation with elevated pulmonary artery pressures. In addition, the intraoperative OctreoScan findings indicated no activity over the lymphatic drainage beds of the middle and lower lobes, and in our experience this is substantially more sensitive than the routine histologic examination of these nodes. (4) Though useful for staging, I am aware of no evidence suggesting that mediastinal lymphadenectomy affects survival in patients with lung cancer or lung carcinoid tumors. Although not reported in our article, the patient underwent mitral valve replacement after recovering from her thoracic procedure in order to correct her mitral insufficiency. We felt that the equivalent of a segmentectomy was the appropriate intraoperative management for this patient. A frozen section was, of course, obtained during that original operative procedure.

The patient has been observed at intervals of 6 months with total body OctreoScans, the findings of which have been negative for recurrent tumor to date.

Correspondence to: Lynn H. Harrison, MD, FCCP, LSU Health Sciences Center, 1542 Tulane Ave, New Orleans, LA 70112

REFERENCES

(1) Rodriguez JA, Meyers MO, Jacome TH, et al, Intraoperative detection of a bronchial carcinoid with a radiolabeled somatostatin analog. Chest 2002; 121:985-988

(2) Oliaro A, Filosso PL, Bello M, et al. Use of [sup.111]In-DTPA-octreotide scintigraphy in the diagnosis of neuroendocrine and non-neuroendocrine tumors of the lung: preliminary results. J Cardiovasc Surg 1997; 38:313-315

(3) Krenning EP, Bakker WH, Breeman WA, et al. Localization of endocrine-related tumours with a radioiodinated analogue of somatostatin. Lancet 1989; 1:242-244

(4) Woltering EA, Barrie R, O'Dorisio TM, et al. Detection of occult gastrinomas with [sup.125]I-labeled lanreotide and intraoperative gamma detection. Surgery 1994; 116:1139-1146

COPYRIGHT 2002 American College of Chest Physicians
COPYRIGHT 2003 Gale Group

Return to Carcinoid syndrome
Home Contact Resources Exchange Links ebay