An unusual carcinoid tumor in a case of Cushing syndrome
ZT Turki, FA Aouididi, HS Smida, LBS Ben Salem, RB Bouguerra & CBS Ben Slama
The overall incidence of carcinoid tumors has been estimated to be 1 to 2 cases /100000 people in USA. Carcinoid tumors habitually accounts for 1% of all cases of Cushings syndrome. The authors report an unusual case of ectopic secretion of corticotropin from pulmonary carcinoid tumor.
A. F. is a 29 years old patient who has been referred for hypercorticism with patent features including trophical signs and severe depression.
Laboratories findings revealed an elevated ACTH (ACTH=322 ng/ml) and an abnormal response during the low dose Dexamethasone suppression test (cortisolemia after the test =89.1 nmol/l). The MRI of the sella was normal. An ectopic secretion of corticotropin was suspected. The CT Scan of the chest and the abdomen showed a left basal pulmonary nodule and hilar lymph nodes.
The Patient underwent to surgical resection of the tumor. Histopathologic examination of the tumor defined it as a typical bronchopulmonary carcinoid with metastatic lymph nodes.
After surgery the evolution was marked by the disappearance of the Cushing syndrome. But the psychiatric signs persisted. A Treatment by chemotherapy and radiation therapy are envisaged.
This observation presents several atypies. The patient is young whereas the disease usually presents in the fifth decade of life. The majority of the tumors are perihilar in location. Atypical carcinoids have an aggressive clinical course, metastazing to mediastinal lymph nodes, in 30 to 50% of cases. The five year survival rate is between 40 and 60%.