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CP Case Conference 9-9-11
Steven Smith
“Thicker than Blood”
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29 yo F with left neck mass
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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 agoNo ETOH, No tobaccoLives at home with school aged daughter
ROS: Increased “fatigue” denies weight loss, fevers, night sweats. + Depression
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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
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3 Months Prior:
Headache, presented to ED
On PE, left submandibular > left neck LAD
Surveillance and Re-Staging CT ordered
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Neoplasm
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Dx/Tx Considerations
U of M Lymphoma Clinic: Presumptive recurrence of disease
Induction chemotherapy, followed by autologous SCT
Biopsy
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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.
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DDX
• Hodgkin recurrence – Syncytial?• Metastatic Carcinoma• Lymphoepithelial carcinoma• Poorly diff. squamous adeno
• Anaplastic large cell lymphoma• Metastatic melanoma
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Negative: AE1/AE3, CAM 5.2, Melan A, EMA, CD30/15Patchy: S100
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CD68
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CD21
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Clusterin
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Fascin
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Dx: Follicular Dendritic Cell Sarcoma
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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)
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Follicular Dendritic Cell Sarcoma 2
• Epi:• Young, middle aged, slight F>M• May arise with FDC dysplasia in Castleman’s dz
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Bio
psy
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2011
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Bio
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Follicular Dendritic Cell Sarcoma 3
• Presentation: Painless Mass• Lymph nodes• Neck, mediastinum, spleen, tonsil• 1/3 extranodal, GI, soft tissue, H&N
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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
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Bio
psy
Inte
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2011
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Bio
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Inte
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2011
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Inflammatory Pseudotumor-like Variant
Bio
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Inte
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2011
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FDC – Myxoid Variant
Bio
psy
Inte
rp. o
f S
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2011
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FDC – Myxoid Variant
Bio
psy
Inte
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f S
oft T
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2011
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Follicular Dendritic Cell Sarcoma 5
• Tx• Surgery with adequate local excision• Chemotx for recurrence mets
• Prognosis: 40% recur locally, late • 7% DOD
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Returning to the Case
• Monitored by CT, then PET-CT for ensuing 3 years without recurrence
• AWOD
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Thanks
• Dr. Jonathan McHugh• Dr. Elizabeth Wey• Michigan Pathology Imaging Core