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\begin{document}

\title{Primary cutaneous precursor B-cell lymphoblastic lymphoma with
concomitant skin and nodal involvement in a child}



\author[1]{Houda Hammami}%
\author[1]{Amal Chamli}%
\author[1]{Raja Jouini}%
\author[1]{Rym Benmously}%
\author[1]{Ashraf Debbiche}%
\author[1]{Anissa Zaouak}%
\author[1]{Samy Fenniche}%
\affil[1]{University of Tunis El Manar Faculty of Medicine of Tunis}%


\vspace{-1em}



  
  \date{May 25, 2024}


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\selectlanguage{english}
\begin{abstract}
Primary cutaneous B-cell lymphoblastic lymphoma (PB-LBL) is an
exceedingly rare malignant neoplasm in children. Skin lesions may
represent the initial sign. Herein, we report a case of a PB-LBL with
cutaneous lesions as a primary extranodal involvement, occurring in a
5-year-old infant.%
\end{abstract}%



\sloppy


\emph{Title} : Primary cutaneous precursor B-cell lymphoblastic lymphoma
with concomitant skin and nodal involvement in a child

\emph{Running title}\textbf{:} B-cell lymphoblastic lymphoma

\emph{Authors} Houda Hammami\textsuperscript{1,2}, MD, Amal
Chamli\textsuperscript{1,2}, MD, Raja Jouini\textsuperscript{2,3}, MD,
Rym Benmously\textsuperscript{1,2}, MD, Ashraf
Debbiche\textsuperscript{2,3}, PhD, Anissa Zaouak\textsuperscript{1,2},
MD, Samy Fenniche\textsuperscript{1,2}, PhD

\emph{Affiliations:}

1-Dermatology Department, Habib Thameur Hospital, Tunis, Tunisia

2-University of Tunis El Manar, Faculty of medicine of Tunis, Tunisia

3-Pathology Department, Habib Thameur Hospital, Tunis, Tunisia

\emph{Corresponding author:} Amal Chamli, MD

Address: Habib Thameur Hospital, 8 Ali Ben Ayed,

Montfleury, 1008, Tunis, Tunisia

\textbf{E-mail:\emph{amal.fmt@gmail.com}}

\emph{ORCID~: https://orcid.org/0000-0002-7927-9088}

\textbf{Tel:} 0021654085688

\emph{Funding}\textbf{:} none

\emph{Conflict of interest:} none

\emph{Acknowledgements}\textbf{:} none

\emph{Authorship:} All authors had access to the data and a role in
writing this manuscript.

\emph{Table count}\textbf{:} 0

\emph{Figures:} \textbf{1}

\emph{Word count}\textbf{: 506}

\emph{References}\textbf{: 4}

\emph{Key words:} Dermatology, Hematology, Paediatrics and adolescents
medicine

\textbf{Primary cutaneous precursor B-cell lymphoblastic lymphoma with
concomitant skin and nodal involvement in a child}

\textbf{Abstract:}

Primary cutaneous B-cell lymphoblastic lymphoma (PB-LBL) is an
exceedingly rare malignant neoplasm in children. Skin lesions may
represent the initial sign. Herein, we report a case of a PB-LBL with
cutaneous lesions as a primary extranodal involvement, occurring in a
5-year-old infant.

\textbf{Key words:} lymphoma, non-Hodgkin lymphoma, lymphoblastic,
B-cell lymphoma, pediatric

\textbf{Key Clinical Messages:}

\textbf{-} Skin lesions may represent the initial sign of primary
cutaneous B-cell lymphoblastic lymphoma (PB-LBL).

- PB-LBL should be considered as a potential differential diagnosis for
head and neck tumors especially in the pediatric population.

\textbf{Case report}

A 5-year-old previously healthy boy presented to our dermatology
department with a 2-month history of skin tumor on his scalp associated
with cervical nodes. On physical examination, there was a 4 cm,
dome-shaped, firm, tender, erythematous tumor of the left parietal
region of the scalp (figure 1). It was associated with multiple,
bilateral, asymmetric cervical nodes. Blood cell count showed normal
values for blood cell count and platelet count. Skin biopsy of the scalp
lesion showed a diffuse infiltrate of atypical lymphoid cells in the
entire dermis (figure 2A). The neoplastic lymphoid cells appeared
medium-sized with inconspicuous nucleoli, scant cytoplasm, and a high
mitotic rate (figure 2B). A grenz zone was present below a
normal-appearing epidermis. Immunohistochemical (IHC) studies revealed
that neoplastic cells expressed terminal deoxynucleotidyl transferase
(TdT) (figure 2C), CD10, CD34, CD79a, and Bcl2, but were negative for
CD3, and CD20. Peripheral blood and bone marrow aspiration smear,
cerebrospinal fluid analysis revealed no evidence of any neoplastic
populations. Whole-body computed tomography revealed involvement of the
cervical, axillary and mesenteric lymph nodes associated with
hepatomegaly. Based on these findings a diagnosis of primary cutaneous
precursor B-cell lymphoblastic lymphoma was made. The patient was
treated with polychemotherapy GFAL B2001 protocol including 6 courses of
vincristine, oncovin, methotrexate, and prednisone. He showed partial
remission after a 14-month follow-up.

\textbf{Discussion:}

Lymphoblastic lymphoma (LBL) is an uncommon non-Hodgkin lymphoma and
accounts for only 5\% of all non-Hodgkin's lymphoma. According to the
WHO classification, LBL is distinguished from acute lymphoblastic
leukemia (ALL) by a bone marrow blast count of less than 20\% of
nucleated cells \textsuperscript{1}. It can be of T-cell or B-cell
lineage. B-cell LBL (B-LBL), the rarer subtype, is a high-grade neoplasm
of immature B cells that demonstrate lymphoblastic morphology and
express precursors and B-cell markers \textsuperscript{2}. It accounts
for less than 10\% of cases of lymphoblastic lymphoma. In contrast to
T-cell LBL, B-LBL tends to involve skin and bone and to spare
mediastinal lymph nodes. Primary cutaneous precursor B-LBL (PB-LBL) is
exceedingly uncommon in children and seems to occur more frequently in
young females \textsuperscript{3}. It usually presents as solitary red
to purple nodules with a propensity for the head and neck
area\textsuperscript{4}.

Histologically, there is an overlap between B-LBL and other lymphoid
malignant neoplasms mainly precursor T-cell LBL, and `small blue round
cell' neoplasms such as Ewing's sarcoma, rhabdomyosarcoma, and
neuroblastoma, rendering histopathological diagnosis difficult. Thus,
IHC is crucial and very useful to rule out differential diagnoses.
Indeed, the expression of TdT and CD34 and the absence of CD20 markers
on B-lineage lymphomas suggest their precursor B-cell origin, as in our
case \textsuperscript{3}.

Although PB-LBL is a highly aggressive malignant neoplasm, its prognosis
is dramatically improved with the use of aggressive multi-agent
chemotherapy ALL-regimens with up to 90\% of 5-year EFS
rate\textsuperscript{3}. Partial remission, as seen in our case despite
an aggressive multi-agent systemic chemotherapy, is rare.

In conclusion, this case serves as an important reminder for clinicians
to consider this entity as a potential differential diagnosis for head
and neck tumors especially in the pediatric population.

\textbf{Conflicts of Interest: None declared}

\textbf{Funding sources: None}

\textbf{Ethics statement:} Appropriate consent has been obtained, prior
to submission, for the publication of images and data.

\textbf{Data availability statement:} Data sharing was not applicable to
this article as no datasets were generated or analyzed during the
current study.

\textbf{Author Contributions:}

Drs Houda Hammami and Amal Chamli contributed to the first draft of the
manuscript. Drs Houda Hammami, Amal Chamli, Raja Jouini, and Rym
Benmously, contributed to the literature search, analysis, and
interpretation of the data. Drs Ashraf Debbiche, Anissa Zaouak, and Samy
Fenniche critically revised the manuscript and gave final approval. All
authors read and approved the final manuscript and agree to be finally
accountable for ensuring the work's integrity and accuracy.

\textbf{Acknowledgements: None}

\textbf{References}

1. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the
World Health Organization classification of lymphoid
neoplasms.\emph{Blood.} 2016;127(20):2375-2390.

2. Kawakami T, Kimura S, Kinoshita A, Kondo K, Soma Y. Precursor B-cell
Lymphoblastic Lymphoma with only Cutaneous Involvement. \emph{Acta Derm
Venerol} . 2009;89(5):540-541.

3. Ceppi F, Pope E, Ngan B, Abla O. Primary Cutaneous Lymphomas in
Children and Adolescents: Cutaneous Lymphomas in Children and
Adolescents. \emph{Pediatr Blood Cancer} . 2016;63(11):1886-1894.

4. Lee WJ, Moon HR, Won CH, et al. Precursor B- or T-lymphoblastic
lymphoma presenting with cutaneous involvement: A series of 13 cases
including 7 cases of cutaneous T-lymphoblastic lymphoma. \emph{J Am Acad
Dermatol} . 2014;70(2):318-325.

\textbf{Figure legend:}

\textbf{Figure 1:} a 4 cm, dome-shaped erythematous tumor of the left
parietal region of the scalp in a 5-year-old child.

\textbf{Figure 2:} \textbf{A} and \textbf{B,} skin biopsy showed a
diffuse infiltrate in the entire dermis, composed of medium-sized
lymphoid cells with inconspicuous nucleoli, scant cytoplasm, and a high
mitotic rate (Haematoxylin and Eosin \selectlanguage{ngerman}×200 and ×600,
respectively)\textbf{, C,} Immunohistochemical staining revealed
positive terminal deoxynucleotidyl transferase cells (TdT, ×200).\selectlanguage{english}
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