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Pediatric Dermatopathology Paul K. Shitabata, M.D. Dermatopathology Institute Wednesday, May 22, 13

Pediatric Dermatopathology - dermpathmd.comdermpathmd.com/cases/dermatopathology_general/Pediatric... · Histopathology Cellular tumor with diffuse infiltration into fat Minimal pleomorphism

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Page 1: Pediatric Dermatopathology - dermpathmd.comdermpathmd.com/cases/dermatopathology_general/Pediatric... · Histopathology Cellular tumor with diffuse infiltration into fat Minimal pleomorphism

Pediatric Dermatopathology

Paul K. Shitabata, M.D.Dermatopathology Institute

Wednesday, May 22, 13

Page 2: Pediatric Dermatopathology - dermpathmd.comdermpathmd.com/cases/dermatopathology_general/Pediatric... · Histopathology Cellular tumor with diffuse infiltration into fat Minimal pleomorphism

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Lichen Striatus

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Histopathology

Lichenoid interface dermatitis

Superficial and Deep perivascular and periadnexal lymphocytic infiltrate

Variable epidermal changes

Wednesday, May 22, 13

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Male FetusCoccygeal Mass

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Infantile Fibrosarcoma

Wednesday, May 22, 13

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Wednesday, May 22, 13

Page 22: Pediatric Dermatopathology - dermpathmd.comdermpathmd.com/cases/dermatopathology_general/Pediatric... · Histopathology Cellular tumor with diffuse infiltration into fat Minimal pleomorphism

Histopathology

Cellular tumor with diffuse infiltration into fat

Minimal pleomorphism Mitotically active Scattered chronic

inflammatory cells common t(12;15)(p13;q25) Fusion of

ETV6 with neurotrophin-3 receptor gene

VIM+

Wednesday, May 22, 13

Page 23: Pediatric Dermatopathology - dermpathmd.comdermpathmd.com/cases/dermatopathology_general/Pediatric... · Histopathology Cellular tumor with diffuse infiltration into fat Minimal pleomorphism

3 month old GirlAnus

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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ES/PNET (Ewing’s Sarcoma/

Wednesday, May 22, 13

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Small Round Blue Cell Tumors of Childhood

Wednesday, May 22, 13

Page 38: Pediatric Dermatopathology - dermpathmd.comdermpathmd.com/cases/dermatopathology_general/Pediatric... · Histopathology Cellular tumor with diffuse infiltration into fat Minimal pleomorphism

Histopathology

Small round blue cells Rarely show neural

rosettes IPOX-CD99 t(11;22)(q24;q12) DDX: Small round blue

cell tumors

Wednesday, May 22, 13

Page 39: Pediatric Dermatopathology - dermpathmd.comdermpathmd.com/cases/dermatopathology_general/Pediatric... · Histopathology Cellular tumor with diffuse infiltration into fat Minimal pleomorphism

CD99 Positive TumorsDiagnosis Positive %

ES/PNET 95T-lymphoblastic lymphoma 92Poorly differentiated synovial sarcoma 50Small cell osteosarcoma 23Rhabdomyosarcoma 21Desmoplastic small round blue cell tumor

16Small cell carcinoma 9Merkel cell carcinoma 9Neuroblastoma 0

Wednesday, May 22, 13

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t(11;22)(q24;q12) Tumors

Skeletal and extra-skeletal Ewing’s sarcoma PNET Askin’s tumor Olfactory neuroblastoma Small-cell osteosarcoma Mesenchymal chondrosarcoma

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Giant Cell Fibroblastoma

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Histopathology

Upper trunk and extremities Median age 3 yrs Variable cellularity Pseudovascular spaces lined

by discontinuous giant cells CD34+ Related to DFSP Recurrences in 50% No mets to date Wide local excision

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Langerhans Cell Histiocytosis

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Histopathology

Effacement of DEJ by atypical mononuclear cells

Reniform nuclei Variable eosinophils Abundant cytoplasm IPOX: CD1a+ EM: Birbeck granules

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Nodular Fasciitis

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Histopathology

Typical clinical scenario Tissue culture-like

proliferation of bland spindle cells

Infiltrative into surrounding soft tissue

Variable inflammatory cells

MF common, no atypical MF

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Epidermal Nevus

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Histopathology

Beware diagnosis of SK in child

Multiple skin tag lesions with SK features

ILVEN cases with psoriasiform changes

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Wednesday, May 22, 13

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Spitz Nevus

Wednesday, May 22, 13

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Wednesday, May 22, 13

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Histopathology

Circumscribed peripheral borders

Vertically oriented nests of epithelioid and spindled melanocytes

Streaming pattern Vertical clefting Increased vascularity Mitotic figures but none at

base Kamino bodies

Wednesday, May 22, 13

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Wednesday, May 22, 13