AL Amyloidoma of the Skin/Subcutis: Cutaneous Amyloidosis, Plasma Cell Dyscrasia or a Manifestation of Primary Cutaneous Marginal Zone Lymphoma?

The American Journal of Surgical Pathology
Noreen M WalshL Cerroni

Abstract

It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Nine patients had a solitary nodule or plaque on the lower extremity (n=7), upper extremity (n=1), or chin (n=1). One patient had an AL amyloidoma on the right thigh and a second lesion on the right arm showing histopathologic features of cMZL without amyloid deposits. Clinical investigations excluded relevant systemic disease in all cases. Microscopically, dermal/subcutaneous deposits of amyloid were associated with sparse to moderate perivascular infiltrates of lymphocytes and monotypic plasma cells (7 with kappa and 3 with lambda light chain restriction). The plasma cells expressed CD56 in one of 9 studied cases. One case was characterized by a t(14;18)(q32;q21)/IGH-MALT1 translocation. Follow-up was available in 8 cases. All remain systemically well after a median time of 86.5 months (range: 40 to 144 mo). Local recurrence of disease was observed in...Continue Reading

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Citations

Mar 7, 2019·The American Journal of Dermatopathology·Stefan UeberdiekChristina Mitteldorf
May 17, 2019·Journal of Cutaneous Pathology·Noreen M WalshLorenzo Cerroni
Mar 7, 2019·The American Journal of Dermatopathology·Cynthia M MagroShabnam Momtahen
Oct 22, 2019·The American Journal of Dermatopathology·Andrea SagginiCarlo Cota
Nov 30, 2019·Virchows Archiv : an International Journal of Pathology·Ilske OschliesJohn R Goodlad
Jul 25, 2020·Skeletal Radiology·Sohil S DesaiMotasem Al Maaieh
Jan 27, 2019·Virchows Archiv : an International Journal of Pathology·Christiane Stuhlmann-LaeiszChristoph Röcken
Mar 18, 2021·Clinical, Cosmetic and Investigational Dermatology·Andrea RonchiRenato Franco

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