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CP Case Conference 9-9-11 Steven Smith

“Thicker than Blood”. CP Case Conference 9-9-11 Steven Smith. 29 yo F with left neck mass. PMHx : HTN, Asthma, OSA, “Large benign ovarian tumor” SurgHx : USO FHx :   Heart disease, DM, thyroid Father deceased, “bile duct cancer” Uncle deceased, “stomach cancer” SHx :  

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CP Case Conference 9-9-11 Steven Smith

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  1. “Thicker than Blood” CP Case Conference 9-9-11 Steven Smith

  2. 29 yo F with left neck mass

  3. PMHx: • HTN, Asthma, OSA, “Large benign ovarian tumor” • SurgHx: • USO • FHx:   • Heart disease, DM, thyroid • Father deceased, “bile duct cancer” • Uncle deceased, “stomach cancer” • SHx:   • Grand Rapids, from Texas 2 years ago • No ETOH, No tobaccoLives at home with school aged daughter • ROS: Increased “fatigue” denies weight loss, fevers, night sweats. + Depression

  4. 22 Months Prior: L neck swelling, fatigue, "heat flashes", chills, pleuritic chest and back pain PCP ordered CXR c/f "bulky" mediastinal LAD. CT scan  Pan-mediastinal mass encasing great vessels and trachea. No obvious organ involvement or LAD in the abdomen or pelvis Biopsy  NS Hodgkin Lymphoma, Stage IIB 6 Cycles of ABVD Chemotherapy  CR

  5. 3 Months Prior: Headache, presented to ED On PE, left submandibular > left neck LAD Surveillance and Re-Staging CT ordered

  6. Neoplasm

  7. Dx/Tx Considerations U of M Lymphoma Clinic: Presumptive recurrence of disease Induction chemotherapy, followed by autologous SCT Biopsy

  8. Received fresh is a 120 gm, 8 x 5.5 x 3.5 cm soft tissue excision consisting predominantly of a 7 cm, encapsulated, soft tissue mass with surrounding dense fibroadipose tissue.

  9. DDX • Hodgkin recurrence – Syncytial? • Metastatic Carcinoma • Lymphoepithelial carcinoma • Poorly diff. squamous adeno • Anaplastic large cell lymphoma • Metastatic melanoma

  10. Negative: AE1/AE3, CAM 5.2, Melan A, EMA, CD30/15 Patchy: S100

  11. CD68

  12. CD21

  13. Clusterin

  14. Fascin

  15. Dx: Follicular Dendritic Cell Sarcoma

  16. Follicular Dendritic Cell Sarcoma 1 • Neoplasm thought to be derived from follicular dendritic cells • Stromal-derived (as opposed to myeloid-derived LC, IDC, and PDC) • Present on follicles where they present antigen to B-cells • Stable, non-migrating cells form a meshwork attached by desmosomes • Ancillary • CD21, CD23, CD35, Fascin, Clusterin, EGFR • CD68, FcR, S100+/- (but not strong), Factor XIIIa+/- • Negative for LC markers (CD1a)

  17. Follicular Dendritic Cell Sarcoma 2 • Epi: • Young, middle aged, slight F>M • May arise with FDC dysplasia in Castleman’sdz

  18. Biopsy Interp. of Soft Tissue, 1st Ed, Fisher et al. 2011

  19. Biopsy Interp. of Soft Tissue, 1st Ed, Fisher et al. 2011

  20. Follicular Dendritic Cell Sarcoma 3 • Presentation: Painless Mass • Lymph nodes • Neck, mediastinum, spleen, tonsil • 1/3 extranodal, GI, soft tissue, H&N

  21. Follicular Dendritic Cell Sarcoma 4 • Gross: Firm white mass, rare hemorrhage/necrosis • Micro: • Ovoid to spindled cells, whorls • Long cellular processes • Nuclei distinctive, speckled to vesicular chromatin • Multinucleated cells

  22. Biopsy Interp. of Soft Tissue, 1st Ed, Fisher et al. 2011

  23. Biopsy Interp. of Soft Tissue, 1st Ed, Fisher et al. 2011

  24. Inflammatory Pseudotumor-like Variant Biopsy Interp. of Soft Tissue, 1st Ed, Fisher et al. 2011

  25. FDC – Myxoid Variant Biopsy Interp. of Soft Tissue, 1st Ed, Fisher et al. 2011

  26. FDC – Myxoid Variant Biopsy Interp. of Soft Tissue, 1st Ed, Fisher et al. 2011

  27. Follicular Dendritic Cell Sarcoma 5 • Tx • Surgery with adequate local excision • Chemotx for recurrence mets • Prognosis: 40% recur locally, late • 7% DOD

  28. Returning to the Case • Monitored by CT, then PET-CT for ensuing 3 years without recurrence • AWOD

  29. Thanks • Dr. Jonathan McHugh • Dr. Elizabeth Wey • Michigan Pathology Imaging Core

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