Table of ContentsDefinition / general | Essential features | Terminology | ICD coding | Radiology images | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Electron microscopy description | Differential diagnosis | Board review style question #1 | Board review answer #1
Cite this page: Gonzalez RS. Neuroendocrine tumor with sarcomatous differentiation. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreaspensarcomatous.html. Accessed July 5th, 2020.
Definition / general
- Well differentiated neuroendocrine tumor with poorly differentiated myosarcomatous component
- Extremely rare lesion (two cases reported in English literature)
- Lesion may transition from neuroendocrine to sarcomatous component or may have two discrete admixed components
- Both reported cases use outdated term islet cell tumor, as they were published in 1989 and 2001
- ICD-10: C25.4 - malignant neoplasm of endocrine pancreas
Microscopic (histologic) description
- Lesion is primarily composed of typical well differentiated neuroendocrine tumor
- Sarcomatous component may have rhabdomyosarcomatous features or may show spindled cells with enlarged nuclei arranged in bundles
- Sarcomatous component is admixed with neuroendocrine component but transition may be gradual or abrupt (with sarcomatous component appearing as discrete nodules)
Microscopic (histologic) images
Electron microscopy description
- Z lines and thick filaments (if rhabdomyosarcomatous component)
- Leiomyosarcoma: most common sarcoma of pancreas; would not demonstrate neuroendocrine component
Board review style question #1
A neuroendocrine tumor of the pancreas with a sarcomatous component would be most likely to show what sort of differentiation within that component?
Board review answer #1
B. Leiomyosarcomatous. Additionally, leiomyosarcoma is the most common primary sarcoma of the pancreas.