Annals of Hematology

https://doi.org/10.1007/s00277-020-04011-8

LETTER TO THE EDITOR

Subcutaneous marginal zone lymphoma: an unusual

lipoma-like presentation
Magda Zanelli 1 & Maurizio Zizzo 2,3 & Francesca Sanguedolce 4 & Alessandra Soriano 5 & Giovanni Martino 6 &
Valerio Annessi 7 & Stefano Ascani 8

Received: 16 February 2020 / Accepted: 27 March 2020

# Springer-Verlag GmbH Germany, part of Springer Nature 2020

Dear Editor, diagnosed. No lymphadenopathy, spleen enlargement, or oth-

A 50-year-old, hepatitis C virus (HCV)-negative, man de-
veloped multiple, palpable, soft subcutaneous nodules under-
lying a normal appearing skin and clinically resembling lipo-
mas. A painful well-circumscribed gluteal nodule, hyperdense
on computed tomography scan (Fig. 1), was surgically ex-
cised. Grossly, the yellow subcutaneous nodule, 5 cm in di-
ameter, closely resembled a lipoma (Fig. 2, left). Histology
documented a dense and diffuse lymphoid infiltrate restricted
to the subcutaneous tissue (Fig. 2, right) and sparing dermis
and epidermis. The infiltrate was composed of small-sized
lymphoid cells with a B-phenotype, positive for CD20 and
CD79a and BCL2 and negative for germinal center markers
(BCL6, CD10, LMO2) and for CD23, CD5, cyclin D1, and
CD3. Some mature plasma cells monotypic for immunoglob-
ulin kappa light chain were present. The proliferative index
was low (Ki67 10%). A marginal zone lymphoma (MZL) was
er sites of disease were detected at staging evaluation. The
patient was treated with radiotherapy and different schemes
of chemotherapy, including R-CHOP (rituximab, cyclophos-
phamide, adriamycin, vincristine, prednisone), rituximab plus
bendamustine, R-CVP (rituximab, cyclophosphamide, vin-
cristine, prednisone), and zanubrutinib. Despite radiotherapy
and chemotherapy, patient developed multiple subcutaneous
recurrences with the same histology between 2008 and 2018.
Extracutaneous spread to an inguinal lymph node was detect-
ed in 2016. Patient is currently alive with disease.
MZL is a low-grade non-Hodgkin B cell lymphoma frequent-
ly affecting extranodal sites, spleen, and lymph nodes [1]. In the
current WHO classification, primary cutaneous MZL is included
within extranodal MZL of the mucosa-associated lymphoid

* Maurizio Zizzo
zizzomaurizio@gmail.com

1
Pathology Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio
Emilia, Reggio Emilia, Italy
2
Surgical Oncology Unit, Azienda Unità Sanitaria Locale-IRCCS di
Reggio Emilia, Reggio Emilia, Italy
3
Clinical and Experimental Medicine PhD Program, University of
Modena and Reggio Emilia, Modena, Italy
4
Pathology Unit, Azienda Ospedaliera Universitaria “Ospedali
Riuniti” di Foggia, Foggia, Italy
5
Gastroenterology Unit, Azienda Unità Sanitaria Locale-IRCCS di
Reggio Emilia, Reggio Emilia, Italy
6
Hematology Unit, CREO, Azienda Ospedaliera di Perugia,
University of Perugia, Perugia, Italy
7
General and Emergency Surgery Unit, Azienda Unità Sanitaria
Locale-IRCCS di Reggio Emilia, Guastalla, Reggio Emilia, Italy
Fig. 1 Abdomen computed tomography scan: a 5-cm well-circumscribed
8
Pathology Unit, Ospedale di Terni-University of Perugia, Terni, Italy hyperdense gluteal nodule (red arrow)
 Ann Hematol

Fig. 2 (Left) Macroscopic

histological examination: yellow
subcutaneous nodule, 5 cm in
diameter, closely resembled a
lipoma. (Right) Microscopic his-
tological examination: dense and
diffuse lymphoid infiltrate re-
stricted to the subcutaneous tis-
sue, sparing dermis, and
epidermis

tissue (MALT) [1]. MALT lymphomas are frequently associated
with infections and autoimmune diseases [1]. Borrelia
Burgdorferi is often involved in cutaneous cases [1]. The asso-
ciation of HCV with splenic MZL is well known and it has been
documented in primary cutaneous MZL. In 2010, a rare subcu-
taneous lipoma-like presentation of primary cutaneous MZL,
associated with HCV infection was reported [2]. Paulli et al.
described a series of 12 HCV-positive patients presenting with
subcutaneous nodules resembling lipomas and with an indolent
clinical course despite local recurrences [2]. Unlike primary cu-
taneous MZL lymphoma which involves the dermis with a nod-
ular/perivascular/periadnexal pattern with sparing of epidermis
and subcutis, lipoma-like variant is strictly limited to the subcu-
taneous tissue [2]. Since its first description, similar cases were
described only in two other HCV-positive patients by De Luca
et al. in 2019 [3]. Our patient developed multiple subcutaneous
nodules over a period of 10 years. Extracutaneous spread with
lymph node involvement occurred only 8 years after disease
onset. Unlike the previously reported cases, our patient was
HCV-negative. The subcutaneous tissue is rarely the primary site
of lymphoma presentation, whereas it can be involved second-
arily by systemic lymphomas [1, 2]. Clinicians should be aware
of the unusual lipoma-like presentation of primary cutaneous
MZL, as the benign clinical appearance of the nodules may
cause a delay in diagnosis and treatment.
Compliance with ethical standards
Ethical approval Local ethics committee (Comitato Etico dell’Area
Vasta Emilia Nord, Italy) ruled that no formal ethics approval was re-
quired in this particular case.
Informed consent Written informed consent was obtained from patient
for publication of this case report and any accompanying images. A copy
of the written consent is available for review by the Editor-in-Chief of this
journal.
References
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classification of tumours of haematopoietic and lymphoid tissues,
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Merli M, Rattotti S, Rossi D, Riboni R, Berti E, Magrini U, Bruno R,
Gaidano G, Lazzarino M (2010) Subcutaneous ‘lipoma-like’ B-cell
lymphoma associated with HCV infection: a new presentation of
primary extranodal marginal zone B-cell lymphoma of MALT. Ann
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3. De Luca ML, Lombardi L, Tartaglia G, Fazio F, Di Prima A, Serrao
A, Canichella M, Pulsoni A (2019) Response to interferon-free direct
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10.4084/MJHID.2019.053
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Conflict of interest The authors declare they have no conflict of interest.