Cutaneous Plasmacytoma

 Must exclude cutaneous involvement in multiple myeloma and plasmacytoid B-cell lymphomas, such as primary cutaneous extranodal marginal zone lymphoma






Etiology/Pathogenesis




• Cell of origin is immunoglobulin-secreting, heavy-chain class-switched, terminally differentiated B cell


Clinical Issues




• Skin is rare site for plasmacytomas; most occur in respiratory tract

• Cutaneous lesions may be found anywhere on body

• Serum protein electrophoresis and urinary protein electrophoresis show low level of monoclonal gammopathy in 20%

• Usually local radiation or surgery

• Local recurrence may happen


Microscopic




• Diffuse or nodular collections of dermal plasma cells

• Oval-shaped plasma cells with eccentric nucleus, clock face or spoke wheel chromatin, without prominent nucleoli

• Increased pink cytoplasm with perinuclear hof

• Mature plasma cells, plasmablasts, and pleomorphic forms may be identified


Ancillary Tests




• Immunohistochemistry shows CD38(+), CD138(+), CD45rb (-), CD20(-), pax-5(-), CD79a(+)

• Flow cytometry may underestimate number of plasma cells

• Plasmacytomas may show same molecular changes as seen in plasma cell myeloma


Top Differential Diagnoses




• Extranodal marginal zone lymphoma

• Reactive plasma cell infiltrates

image
Gross Image
A single red solitary plaque on the forearm is shown, which microscopically is a cutaneous plasmacytoma (CP).


image
Low-Power of Cutaneous Plasmacytoma
Low-magnification examination of a CP shows a diffuse, dermal-based lymphoid infiltrate with deep extension.

image
Higher Magnification of Cutaneous Plasmacytoma
This CP shows a prominent plasmacytoid infiltrate surrounding multiple adnexal ducts image and infiltrating between collagen bundles image.

image
Plasma Cell Morphology
This CP shows multiple mature-appearing plasma cells with prominent perinuclear hofs image. Scattered small lymphocytes image are also present.


TERMINOLOGY


Abbreviations




• Cutaneous plasmacytoma (CP)


Synonyms




• Extraosseous plasmacytoma of skin


Definitions




• Neoplasm of monoclonal plasma cells involving skin
image Must exclude cutaneous involvement in multiple myeloma and plasmacytoid B-cell lymphomas, such as primary cutaneous extranodal marginal zone lymphoma

image No clinical features of plasma cell myeloma and no evidence of bone marrow plasmacytosis


ETIOLOGY/PATHOGENESIS


Cell of Origin




• Clone of immunoglobulin-secreting, heavy-chain class-switched, terminally differentiated B cells that usually secrete single monoclonal immunoglobulin


CLINICAL ISSUES


Epidemiology




• Incidence
image Extremely rare

– Only 3-5% of all plasma cell neoplasms

– Many cases previously reported as primary CPs would now be reclassified as extranodal marginal zone lymphomas

image Rarely involve skin, more common in respiratory tract (80% of extraosseous plasmacytomas in oropharynx, nasopharynx, and nasal sinuses)

image Rarely, may be seen as posttransplant lymphoproliferative disorder (PTLD)

– Morphologically identical to other plasmacytomas, but PTLD plasmacytoma-like tumors are often EBV(+)

• Age
image Median age: 55 years

• Sex
image Mostly in men; M:F = 2:1


Site




• Skin (by definition)

• Lesions may occur anywhere on body


Presentation




• Usually single or rarely multiple lesions
image Upper airway/nasal lesions may present with rhinorrhea, nasal obstruction, epistaxis


Laboratory Tests




• Serum protein electrophoresis
image 20% reported to show monoclonal gammopathy

image There is usually low level of M protein in serum &/or urine

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cutaneous Plasmacytoma

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