Neuroendocrine Carcinoid Tumor

Eric Richard Handley, MD - Case Coordinator
Tammy Sung, MD - Radiology Discussion
Michael W Bennett, MD - Pathology Discussion
Philip Costello, MD - Attending Radiologist
Steven E Seltzer, MD - Attending Radiologist
Pablo R Ros, MD, MPH - Attending Radiologist

September 8, 2003

Presentation

Imaging Findings

On posteroanterior and lateral radiographs, the cardiac silhouette is normal. There are several opacities in the left lobe superior to the hilum as well as an area of consolidation in the right lung base. Given the patient's history, the differential diagnosis should include tuberculosis, aspergillosis, and (less likely) carcinoma.

High-resolution axial CT images of the chest confirm the area of consolidation in the right lung base. On the image depicting mediastinal windowing, there appears to be a soft tissue mass in the right hilum. Peripheral opacities are also visible. The soft tissue lesion appears to enhance with contrast and to be situated within an airway. The bronchi are dilated.

Differential Diagnosis

Diagnosis

Discussion

The gross specimen is from a right lower lobectomy. The tumor is visible in the bronchus (cut section, bottom right of the first image; central on second image). The bronchial margin was frozen, and the tumor became unattached during this process. The specimen was bisected along the obstructed bronchus. The tumor mass (2.9 cm) is contiguous with the visceral pleura. Mucoid obstruction of the bronchus is evident distal to the mass. Low power microscopy illustrates the tumor arising from the bronchus and spreading into the luminal space. Normal bronchial mucosa and the cartilaginous wall of the bronchus are also visible. At slightly higher power, the trabecular pattern of the tumor cells is clear. Additional magnification shows that the chromatin in the nuclei has a ‘salt-and-pepper’ quality, indicating granularity. These features suggest neuroendocrine differentiation. Special stains were used to confirm the presence of neurosecretory granules or their components. This tumor is positive for both chromagranin and synaptophysin.

Radiology Discussion:

The differential diagnosis of an endobronchial opacity includes broncholithiasis, foreign body, and neoplasm. The most likely neoplasms include hamartoma, carcinoid tumor, and mucoepidermoid tumor. There are three types of endobronchial neuroendocrine tumor. Typical carcinoid is characterized by a round, smooth, well-defined nodule. The vast majority (90%) are central, and metastases are uncommon. It typically strikes patients between the ages of 40 and 50 years, and is associated with a 92% 5-year survival rate. Atypical carcinoid typically manifests in larger nodules. The masses may appear centrally or peripherally, and metastatic spread to the hilar and mediastinal lymph nodes is common. This is most common among slightly older patients (50-60 years) and is associated with a lower 5-year survival rate (69%). Small-cell carcinoma is generally associated with a small, peripheral lung nodule and bulky adenopathy of the hilum and/or mediastinum. Extrathoracic metastases are common. It typically affects patients between the ages of 60 and 70 years.

The radiologic features of carcinoid depend somewhat on whether the mass is central or peripheral. Central masses appear in the lobar, segmental, or subsegmental bronchi or in the hilum. Typical signs include obstructive pneumonia and segmental or lobar collapse. Peripheral masses tend to grow slowly and may calcify (30%). Regardless of location, the masses enhance avidly with contrast (iodine or gadolinium).

References

Rosado de Christenson ML, Abbott GF, Kirejczyk WM, Galvin JR, Travis WD. From the Archives of the AFIP: Thoracic Carcinoids: Radiologic-Pathologic Correlation. RadioGraphics, May 1, 1999; 19(3): 707-736.

Weissleder R et al. Primer of Diagnostic Imaging, 2nd Ed. Mosby, St Louis, 1997.

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