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Mediastinal and retroperitoneal plexiform neurofibroma with sarcomatous change

Case contributed by Chamali Athauda
Diagnosis certain

Presentation

Chronic cough, shortness of breath.

Patient Data

Age: 30 years
Gender: Male

An infiltrating hypodense mass lesion with areas of peripheral enhancement in the left paratracheal region displacing the branches of the aortic arch. The lesion extends into the prevertebral space but no bone destruction or communication with the spinal canal.

Extension into the retroperitoneal space is through the esophageal hiatus and it encases the branches of the celiac axis and superior mesenteric artery.

Few smaller separate lesions with similar characteristics are seen in the vicinity of the main mediastinal lesion.

Multiple poorly enhancing soft tissue density nodules are seen in the subcutaneous tissue plane.

Case Discussion

The patient was diagnosed with neurofibromatosis type 1 (NF1) with multiple cutaneous neurofibromas. Plexiform neurofibromatosis is pathognomic of NF1 and mediastinal location mimics lymphadenopathy. Significant risk of malignant transformation is well known in plexiform neurofibromatosis.

Histology report

Microscopy: Sections reveal fragments of tumor tissue with large areas of necrosis. There are intervening spindle cell proliferation composed of fascicular arrangement of pump and spindly cells with moderate nuclear atypia. Mitotic figures are seen. Background shows fibro myxoid stroma. Surrounding adipose tissue with possible lymphoid follicles are also seen. In addition there is well defined area of benign looking spindly cells with wavy nuclei in background of fibromyxoid stroma, favoring neurofibroma.

Contributed by Dr Sumudu Palihawadane and Dr Chamali Athauda

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