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

CP Case Conference 9-9-11

Steven Smith

“Thicker than Blood”

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:  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

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

3 Months Prior:

Headache, presented to ED

On PE, left submandibular > left neck LAD

Surveillance and Re-Staging CT ordered

Neoplasm

Dx/Tx Considerations

U of M Lymphoma Clinic: Presumptive recurrence of disease

Induction chemotherapy, followed by autologous SCT

Biopsy

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.

DDX

• Hodgkin recurrence – Syncytial?• Metastatic Carcinoma• Lymphoepithelial carcinoma• Poorly diff. squamous adeno

• Anaplastic large cell lymphoma• Metastatic melanoma

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

CD68

CD21

Clusterin

Fascin

Dx: Follicular Dendritic Cell Sarcoma

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)

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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Follicular Dendritic Cell Sarcoma 3

• Presentation: Painless Mass• Lymph nodes• Neck, mediastinum, spleen, tonsil• 1/3 extranodal, GI, soft tissue, H&N

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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Inflammatory Pseudotumor-like Variant

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FDC – Myxoid Variant

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FDC – Myxoid Variant

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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

Returning to the Case

• Monitored by CT, then PET-CT for ensuing 3 years without recurrence

• AWOD

Thanks

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