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Thymic carcinoid tumor with superimposed aspergillosis due to paraneoplastic Cushing syndrome

Case contributed by Carmen Bodlak
Diagnosis certain

Presentation

Patient presented with fatigue to the emergency room.

Patient Data

Age: 50 years
Gender: Male

Anterior mediastinal mass and probably subcarinal, paratracheal, subaortic and left hilar lymphadenopathy. Right pleural effusion. 

Anterior mediastinal solid mass with cystic - necrotic degeneration. Pulmonary nodule with multilobulated margins and subpleural location in inferior right lobe. Bilateral adrenal gland enlargement.

No evidence of lymphadenopathy.

10 days later, during CT-guided biopsy

Enlargement of the pulmonary mass is seen and complete  CT - angiography is performed.

Multiple pulmonary masses and nodules. Cavitation and pseudocavitation, feeding vessel sign and halo sign are noted in some of the lesions. Distribution is multilobar and subpleural and they are of different sizes. 

The biggest one is on the previous location of the pulmonary nodule seen in first CT.

No filling defects within the pulmonary vasculature are demonstrated

Octreotide concentrates in the mediastinal mass. Adrenal and pulmonary masses and nodules are not concentrating the radiotracer. 

Hydropneumothorax with multiple air-fluid levels and cavities with thin walls. Cavitated pulmonary nodule in the middle lobe. Radiological improvement of multiple nodules. 

Radiological improvement of the multiple nodules showing progressive thin wall cavitation.

Anterior mediastinal mass with no significant size changes. 

Hydropneumothorax with locules and prominent air content. Chest drainage tube.

Extensive microbiological tests were performed. Aspergillus fumigatus was found in pleural fluid and High levels of cortisol in laboratory tests. Core biopsy of the anterior mediastinal mass was performed.

Pathology diagnosis was atypical carcinoid tumor. 

Case Discussion

Initial chest x-ray demonstrates a prevascular anterior mediastinal mass.

Contrast-enhanced CT demonstrated a mediastinal mass with no lymphadenopathy. A pulmonary nodule and bilateral adrenal enlargement are also noted and considered to be metastasic in origin.  Invasive thymoma (due to metastasic lesion), thymic carcinoma, thymic carcinoid or primary mediastinal lymphoma are the most likely diagnoses for this patient. 

During biopsy of the mediastinal mass, multiple pulmonary nodules are detected with typical features of septic pulmonary emboli.

Re-evaluation of the case was made. The carcinoid tumor was producing ACTH and the adrenal glands (enlarged on CT) are responding with excessive cortisol production thus leading to a Cushing paraneoplastic syndrome. The immunosuppression induced by the high levels of cortisol is responsible for invasive aspergillosis.

Treatment with ketoconazole failed to decrease cortisol levels and the patient's condition worsened. The decision was made by a multidisciplinary committee to perform excision of the mediastinal mass. The patient underwent surgery and unfortunately died after some postoperative complications.

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