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Solid Pseudopapillary Neoplasm
- 1. Rawa Muhsin Solid PseudopapillaryNeoplasm of Pancreas
- 2. Etiology Low-grade malignantneoplasm of uncertain cellular differentiation Electron microscopy shows evidence of epithelial differentiation 90-100% harbor mutations in CTNNB1 gene
- 3. Clinical Most patientsin 20s and 30s Female predominance (10-20:1) Evenly distributed throughout pancreas Vague abdominal symptoms Indolent 10-15% metastasize (liver, peritoneum, lymph nodes) Prognosis excellent
- 6. Gross Large solitarymass Well circumscribed Solid to cystic, usually mixed White-gray to yellow cut surface Evenly distributed throughout pancreas
- 11. Microscopic Solid monomorphicsheets Delicate vessels with hyalinized or myxoid stroma Low mitotic rate Perineural and true vascular invasion quite rare Marked degenerative changes (pseudopapillae, foamy macrophages, cholesterol clefts, hemorrhage, lipofuscin or melanin pigment, calcification, areas of infarction) Infiltration of adjacent parenchyma is common "Blood lakes" common at periphery of neoplasm
- 14. Cytologic Nuclei orientedaway from vessels with zone of cytoplasm separating nuclei from capillaries Uniform and round to oval with finely dispersed nuclear chromatin Often with longitudinal nuclear grooves Moderate amount of eosinophilic cytoplasm but can be clear with vacuoles Intracytoplasmic eosinophilic hyaline globules
- 20. Immunohistochemistry Antibody Pattern %Cases Beta-catenin Nuclear 99% 524 CD10 Membranous 93% 181 CD56 Membranous 98% 152 CD99 Membranous 98% 110 Progesterone Nuclear 100% 96 Cyclin D1 Nuclear 78% 162 E-cadherin Membranous 8% 319
- 21. Immunohistochemistry Antibody % Cases CD5698% 152 NSE 92% 76 Synaptophysin 68% 288 Chromogranin 12% 161 INSM1 9% 11
- 25. Molecular Missense mutationsin exon 3 of CTNNB1 in nearly all cases Prevents destruction of beta-catenin
- 27. Differential diagnoses Pancreaticneuroendocrine tumor Nuclear features IHC panel Acinic cell carcinoma Pattern IHC panel Pseudocyst Serous neoplasms
Editor's Notes
- #5 Well-circumscribed neoplasm with solid and cystic components Calcifications in ~ 30%
- #6 Well-circumscribed neoplasm with solid and cystic components Calcifications in ~ 30%
- #8 This well-demarcated tumor has a soft and friable solid surface with hemorrhagic areas.
- #9 Hemorrhagic tumor of the head of the pancreas without involvement of the Wirsung.
- #10 Encapsulated solid hemorrhagic tumor.
- #11 The tumor can present as a well-demarcated, hemorrhagic cystic mass mimicking a pseudocyst.
- #13 Solid sheets of tumor cells become dyscohesive and result in a characteristic pseudopapillary appearance with a central fibrovascular-like core surrounded by neoplastic cells.
- #14 The delicate vessels can have myxoid stroma or may be hyalinized.
- #16 The sheets of tumor cells have overlapping, round to oval nuclei that are oriented away from the vessels with a rim of cytoplasm toward the capillary.
- #17 Tumor cells have round to oval nuclei and sometimes exhibit longitudinal nuclear grooves . These intra- and extracytoplasmic eosinophilic hyaline globules stain positive for PASD and α-1-antitrypsin.
- #18 Typically the neoplastic cells are polygonal with eosinophilic cytoplasm, but these tumors can also have clear cytoplasm or, rarely, be composed of monomorphic spindle cells.
- #19 Diff-Quik-stained FNA smear of a solid pseudopapillary tumor shows tumor cells with round to oval, eccentrically placed nuclei , condensed nuclear chromatin, and a moderate amount of delicate cytoplasm.
- #20 Diff-Quik-stained FNA smear of a solid pseudopapillary tumor shows pseudopapillae comprised of a capillary channel bordered by tumor cells . Pap-stained FNA smear of solid pseudopapillary tumor shows "naked" branched capillaries with a background of dispersed tumor cells that have slender cytoplasmic processes .
- #23 Immunohistochemistry for β-catenin shows nuclear and cytoplasmic staining in > 90% of tumors.
- #24 Immunohistochemistry for progesterone receptor shows nuclear staining in nearly 80% of solid-pseudopapillary tumors. Staining for estrogen receptor is generally negative.
- #25 Immunohistochemistry for CD10 shows cytoplasmic staining in solid-pseudopapillary tumors in nearly all the cases. Perinuclear staining may be seen in some instances.
- #27 Figure 7-31 The role of APC in regulating the stability and function of β-catenin. APC and β-catenin are components of the WNT signaling pathway. A, In resting colonic epithelial cells (not exposed to WNT), β-catenin forms a macromolecular complex containing the APC protein. This complex leads to the destruction of β-catenin, and intracellular levels of β-catenin are low. B, When normal colonic epithelial cells are stimulated by WNT molecules, the destruction complex is deactivated, β-catenin degradation does not occur, and cytoplasmic levels increase. β-catenin translocates to the nucleus, where it binds to TCF, a transcription factor that activates genes involved in cell cycle progression. C, When APC is mutated or absent, as frequently occurs in colonic polyps and cancers, the destruction of β-catenin cannot occur. β-catenin translocates to the nucleus and coactivates genes that promote entry into the cell cycle, and cells behave as if they are under constant stimulation by the WNT pathway.