Case History
This is a fine needle aspiration of a 4.1 cm indeterminate soft tissue mass in the pancreatic head of a 41-year-old male.
What is the most likely diagnosis?
A. Leiomyosarcoma
B. Neurofibroma
C. GIST
D. Fibromyxoid sarcoma
B. Neurofibroma
C. GIST
D. Fibromyxoid sarcoma
Answer: B. Neurofibroma
Discussion:
The patient is a 41 years old male with past medical of neurofibromatosis type 1. He has a recent diagnosis of lung adenocarcinoma and imaging revealed an increased PET/CT uptake in the pancreas.
A fine needle aspiration of pancreatic head lesion/mass was performed and the specimen consists of loosely arranged small groups of spindle cells with scant cytoplasm and oval elongated and regular nuclei. Nucleoli are not seen. Ganglion cells are also present (two present in the lower half of the IHC image of the question).
Immunohistochemistry is performed for further characterization. The lesional cells are positive for S100 (shown above) and negative for CD117, DOG1 and desmin. The final diagnosis is neurofibroma.
Patients with neurofibromatosis type 1develop neurogenic-associated neoplasms, including neurofibromas. This syndrome is also associated with nonneurogenic-associated neoplasm (like pheochromocytoma) and CNS nontumoral manifestations (like occasional neuroglial hamartomas).
Case contributed by: Atif Raja, M.D., Cytopathology Fellow, UAB Pathology