Weekly Chest CasesArchive of Old Cases

Case No : 85 Date 1999-06-12

  • Courtesy of Koun-Sik Song, MD, Tae-Hwan Lim, MD / Asan Medical Center, Seoul, Korea
  • Age/Sex 41 / F
  • Chief ComplaintEasy fatigability, bruisability and glucose intolerance
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Diagnosis With Brief Discussion

Diagnosis
Thymic neuroendocrine tumor (carcinoid tumor) with mediastinal lymph node metastasis and bilateral adrenal hyperplasia due to ectopic production of ACTH
Radiologic Findings
CT scans after administration of intravenous contrast media show elongated mass with lobulation in left side of the anterior mediastinum at the level of the aortic arch. Multiple round and oval shaped enlarged lymph nodes are seen between the superior vena cava and innominate artery, and right lower paratracheal nodal station. Adrenal glands are diffusely enlarged bilaterally and show strong enhancement. Attenuation of the liver is generally decreased.
Brief Review
Carcinoid tumor is a well-differentiated neuroendocrine tumor of low-grade malignancy occupying one extreme end of neuroendocrine tumors. Highly malignant poorly differentiated small cell carcinoma occupies the opposite end, and between these entities there are so called atypical carcinoid tumors and non-small cell(large cell) neuroendocrine carcinoma.Histologically this case was proved as non-small cell(large cell) neuroendocrine carcinoma. Average age of the patients are 42 years. About one third of the patients are asymptomatic and the tumors are found incidentally on chest radiographs. Non-specific symptoms and signs such as chest pain, cough and dyspnea resulted from the anterior mediastinal mass itself. Some cases are associated with multiple endocrine adenomatosis or ectopic ACTH production with Cushing's syndrome.Nonfunctioning thymic carcinoid tumors are often large enough to be detected incidentally on routine chest radiographs. On the other hand, thymic carcinoid tumors associated with Cushing's syndrome maybe so small that that they are undetectable by routine chest radiographs. In these cases, CT scan almost always demonstrates small thymic tumors sometimes indistinguishable from prevascular lymph node enlargement. Grossly irregular areas of coagulation necrosis or hemorrhage may be present.The tumor frequently adheres to and at times actually invades surrounding organs or tissues such as the pleura, pericardium, lung, and adventitia of the major vessels. Fibrous bands, which are often found in thymoma are not present. Lymph node metastasis is not rare. The most frequent paraneoplastic syndrome associated with thymic carcinoid tumor is Cushing's syndrome. The treatment of choice is surgical removal of the tumor. The prognosis of patients with paraneoplastic syndromes such as Cushing's syndrome is invariablely poor. Frequent site of metastasis include mediastinal lymph nodes, cervical lymph nodes, lung and bone. Bone metastasis is often osteoblastic.
References
Shimosato Y, Mukai K. Tumors of the thymus and related lesions. Atlas of Tumor Pathology, 3rd Series, Fascicle 21. Wshington, D.C.:Armed Forces Institute of Pathology, 1997
Keywords
Mediastinum, Malignant tumor,

No. of Applicants : 24

▶ Correct Answer : 11/24,  45.8%
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