Clinicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1.
Thymic carcinoid is part of the multiple endocrine neoplasia type 1 (MEN1) syndrome occurring predominantly in male patients who were heavy smokers, presenting most commonly in middle age. In contrast with metastatic midgut carcinoids, MEN1-related thymic carcinoid is not associated with carcinoid syndrome, nor is it associated with Cushing syndrome, in contrast with sporadic thymic carcinoids. Local invasion and metastasis are common. Prognosis is poor because of late detection, lack of effective treatment, and the aggressive nature of the tumor. All patients with thymic carcinoids should be investigated for MEN1, including thorough clinical evaluation and family studies. Anterior mediastinal lesions in MEN1 male patients should be considered thymic carcinoids until proven otherwise. All male MEN1 patients and asymptomatic gene carriers should be warned of the risk of thymic carcinoids and the possible link to smoking. Computed tomography (CT) of the chest is recommended on first screening for MEN1 in male patients more than 25 years of age, followed by yearly chest X-rays and chest CT every 3 years. Prophylactic thymectomy should be carried out during subtotal or total parathyroidectomy on MEN1 patients.
Teh
B T
BT
Department of Molecular Medicine, Karolinska Hospital, Stockholm, Sweden.
McArdle
J
J
Chan
S P
SP
Menon
J
J
Hartley
L
L
Pullan
P
P
Ho
J
J
Khir
A
A
Wilkinson
S
S
Larsson
C
C
Cameron
D
D
Shepherd
J
J
D000328:Adult; D002276:Carcinoid Tumor / Q000235:genetics / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D003480:Cushing Syndrome / Q000473:pathology; D005500:Follow-Up Studies; D006579:Heterozygote; D006801:Humans; D008297:Male; D008303:Malignant Carcinoid Syndrome / Q000473:pathology; D008479:Mediastinal Neoplasms / Q000235:genetics / Q000473:pathology; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics / Q000473:pathology*; D009361:Neoplasm Invasiveness; D016105:Parathyroidectomy; D012189:Retrospective Studies; D012307:Risk Factors; D012907:Smoking / Q000009:adverse effects; D013934:Thymectomy; D013953:Thymus Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome