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Abbas Agaimy, MD Erlangen, Germany
The 10th Arkadi M. Rywlin International Pathology Slide Seminar Symposium in Anatomic Pathology, May 21 – 23, 2018 Split, Croatia CASE #34 Abbas Agaimy, MD Erlangen, Germany
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Objectives of this case
Recognize a distinctive tumor type (birth of a new entity!). Appreciate its association with a hereditary disease.
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AMR Case #34 Caucasian male. Multiple lipomas excised at age 26 y.
(Neck, trunk & upper arm). Then again at age 34 from similar sites. At age 35: bilateral scrotal masses (submited slides). No other special history submitted.
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Bilateral scrotal tumors
(courtesy of Dr. W. Schaschkow, Gelsenkirchen, Germany)
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At low power, well circumscribed with striking size variation
of adipocytes
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Striking fat cell size variation (anisocytosis) but little atypia
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Degenerative changes (variable staining of cell borders
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Grape-like atypical vacuolation
S100
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Chicken-wire phenomenon of cell borders/periphery
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Mild atypia & vacuolated nuclei (Lochkerne)
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Mild atypia & vacuolated nuclei (Lochkerne) are typical
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Ruptured single fat cells with
histiocytes encasing them
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Rb1 lost in most of fatty & stromal cells
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Other markers tested CD34: pos in scattered stromal cells
MDM2&CDK4 IHC: neg MDM2&CDK4 FISH: neg for amplification Rb1 FISH: no abnormality detected in this tumor
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What is your diagnosis?
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Our case ordinary lipoma
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Our case conventional ALT
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Our case pleomorphic/SCL
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What is your diagnosis? Not an ordinary lipoma.
Not a conventional ALT. No classical spindle cell lipoma component. ????
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USCAP 2015 (Boston)
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On the occasion of his scrotal lipomas, it came out that this
patient had a retinoblastoma at age 1.
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Male. Multiple lipomas excised at age 26 y. (Neck, trunk & upper arm). Then again at age 34 from similar sites. At age 35: bilateral scrotal masses (submited slides). Striking concordance between the two cases
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M: F = 7:1 Age (M: 51 y) Site: mainly upper back, shoulders or posterior neck 15% multiple (2 to 5 tumors) Rb1 loss by ICH: 69% cases. >23% Rb1 deletions by FISH Local rec: 16%.
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TP53
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Take-home-message Dysplastic (anisometric cell) lipoma is a frequent but likely misnamed subcutaneous fatty neoplasm. Distinctive from conventional ALT histolgically and by IHC. Distinctive from conventional pleomorphic/spindle cell lipoma. Has a recurrence rate in the range of 16%. No dedifferentiation reported. Tends to be frequently multifocal. Associated with childhood retinoblastoma in a subset of cases.
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29 Kilometer apart City of Berber Sudan
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Thank you for your attention
29 Kilometer apart City of Atbara Kamal G. Ishak ( ) City of Berber Sudan
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