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21/07/2017 1 21/07/2017 CD31 ERG 2 21/07/2017 SPINDLE CELL - - PDF document
21/07/2017 1 21/07/2017 CD31 ERG 2 21/07/2017 SPINDLE CELL - - PDF document
21/07/2017 1 21/07/2017 CD31 ERG 2 21/07/2017 SPINDLE CELL SPINDLE CELL HAEMANGIOMA HAEMANGIOMA CLINICAL FEATURES Second and third decades M = F Mainly distal extremities (hands) Red / blue nodules (more than 50% with
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21/07/2017 3 SPINDLE CELL HAEMANGIOMA SPINDLE CELL HAEMANGIOMA
CLINICAL FEATURES
Second and third decades M = F Mainly distal extremities (hands) Red / blue nodules (more than 50% with multiple lesions) Slowly progressive course Association with Mafucci, Klippel-Trenaunay (about 5%),
lymphoedema, varicose veins
Initially thought to be a low-grade malignant tumour, re-
classified as benign and a form of vascular malformation and recently proven to be a benign neoplasm (R132C IDH1 mutation demonstrated both in familial and sporadic cases)
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SPINDLE CELL HAEMANGIOMA
Spindle cell haemangioma Spindle cell haemangioma
Associations
Congenital lymphoedema
Klippel-Trenaunay syndrome
Maffuccis syndrome 5%
SPINDLE CELL HAEMANGIOMA SPINDLE CELL HAEMANGIOMA
HISTOLOGICAL FEATURES
Poorly circumscribed Cavernous thin-walled vascular spaces Solid spindle cell areas Focal epithelioid cells with cytoplasmic vacuoles Areas reminiscent of an arteriovenous
malformation
Thrombosis
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CD34
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ILLUSTRATIVE CASE Male, 50. Multiple vascular papules on lower lip ILLUSTRATIVE CASE Male, 50. Multiple vascular papules on lower lip
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CD31 ERG
PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA
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First described 1992 by Mirra et al
- Epithelioid sarcoma, Fibroma-like,
Fibrohistiocytic/Myoid Variant
2003 by Billings, Folpes and Weiss
- Epithelioid Sarcoma
Like Hemangioendothelioma
2011 Hornick and Fletcher
- Pseudomyogenic Hemangioendothelioma
Pseudomyogenic Hemangioendothelioma: A distinctive,
- ften multicentric tumor with indolent
behavior Pseudomyogenic Hemangioendothelioma: A distinctive,
- ften multicentric tumor with indolent
behavior
Hornick JL, Fletcher CD, Am J Surg Pathol 2011;35:190-201
PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA
Young male (31y mean age) Limbs (78%), Trunk (16%), Head and
Neck (4%)
Multifocality Multiple tissue levels Occasional infiltration of bone
PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA
Loosely fascicular and sheet like, rarely
focally storiform
Focally myxoid Plump spindle cells with vesicular nuclei,
prominent nucleoli and abundant bright eosinophilic cytoplasm with a rhabdomyoblast like appearance
Can have focal epithelioid areas Neutrophilic infiltrate 8 year-old male. Multiple painless nodules on the thigh
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PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA PSEUDOMYOGENIC HAEMANGIOENDOTHELIOMA
100% (+) for : AE1/AE3 and Fli1 100% (-) for : CD34, S100, desmin 100% Retention of INI1 Positive for new endothelial marker ERG Variable positivity for CD31 Focal positivity for Cam5.2, MNF116, SMA, EMA
GENETICS GENETICS
t(7;19)(q22;q13) SERPINE1-FOSB By FISH or RT-PCR
PROGNOSIS PROGNOSIS
TUMOUR OF INTERMEDIATE
MALIGNANCY
Overall indolent behaviour High Risk for Local recurrence (may lead to
amputation)
Lymph node metastasis Low risk of distant mets
EPITHELIOID SARCOMA EPITHELIOID SARCOMA
50% positive for CD34 90% loss of INI1 Negative for Fli1 Negative for CD31 EMA positive
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