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

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 tobacco Lives 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.

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

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

14. CD68

15. CD21

16. Clusterin

17. Fascin

18. Dx: Follicular Dendritic Cell Sarcoma

19. 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)

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

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

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

24. 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

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

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

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

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

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

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

32. Thanks Dr. Jonathan McHugh Dr. Elizabeth Wey Michigan Pathology Imaging Core