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\begin{document}

\title{An unexpected case of primary rectal diffuse large B-cell lymphoma}



\author[1]{Sondes Bizid}%
\author[1]{Fatma Ben Farhat}%
\author[1]{Hela Ghedira}%
\author[1]{Sonia Bennaser}%
\author[1]{Beya Chelly}%
\author[1]{Hela Stambouli}%
\author[1]{Fehmi Msadek}%
\affil[1]{Military Hospital of Instruction of Tunis}%


\vspace{-1em}



  
  \date{May 25, 2024}


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\selectlanguage{english}
\begin{abstract}
Primary rectal lymphoma (PRL) is the rarest primary rectal cancer.
Clinical presentation is similar to rectal carcinoma. The role of
surgery is still controversial and the benefit of chemotherapy and
radiotherapy is not well established. We here report the case of female
patient suffering from PRL completely disappeared with chemotherapy.%
\end{abstract}%



\sloppy


\textbf{Case Report}

\textbf{An unexpected case of primary rectal diffuse large B-cell
lymphoma}

\textbf{Author list:}

Sondes BIZID \textsuperscript{1,5}, Fatma BEN
FARHAT\textsuperscript{1,5}, Hela GHEDIRA\textsuperscript{2,5}, Sonia
BENNASER\textsuperscript{3,5},

Beya CHELLY\textsuperscript{4,5}, Hela STAMBOULI \textsuperscript{2,5},
Fehmi MSADEK \textsuperscript{2,5}

1.Gastro-enterology Department , Military hospital of Tunis, Tunis,
Tunisia

2.Hematology Department, Military hospital of Tunis, Tunis, Tunisia

3.Oncology Department, Military hospital of Tunis, Tunis, Tunisia

4.Pathology Department, La Rabta, Tunis, Tunisia

5.University of Tunis El Manar, Faculty of Medicine, 1007, Tunis,
Tunisia

\selectlanguage{greek}\textsuperscript{\textbf{µ}}\selectlanguage{english}\textbf{Corresponding author:}

Fatma Ben Farhat,

University of Tunis El Manar, Faculty of Medicine of Tunis, 1007, Tunis,
Tunisia

The military hospital of Tunis, Department of Gatroenterology,
Montfleury 1008, Tunisia

\textbf{Email:\emph{Fatmabf8@gmail.com}}

\textbf{Consent:}

Written informed consent was obtained from the patient to publish this
report in accordance with the journal's patient consent policy.

\textbf{Keywords:}

Case report, diffuse large B-cell lymphoma, rectal, chemotherapy.

\textbf{Introduction:}

Primary colorectal lymphoma (PRL) accounts for 1.4\% of all cases of
non-Hodgkin's lymphoma (NHL) and less than 1\% of all colorectal
malignancies. Although the gastrointestinal (GI) tract is the most
common site of extranodal lymphoma, colorectal lymphoma is rare {[}1{]}.
Dawson~and al~established criteria for the diagnosis of PRL in 1961
including~the absence of clinically enlarged lymphadenopathy in physical
examination, the absence of enlarged mediastinal lymph nodes on chest
x-ray, normal white blood cell count and bone marrow biopsy, and the
absence of abnormalities of the liver and spleen {[}2{]}. While
treatment strategies in nodal NHL are well established, management of
PRL is still unclear {[}3{]}. ~We present the case of a 38-year-old
woman, diagnosed with a stage IIE PRL

\textbf{Patient and observation:}

\textbf{Patient information:} A 38-year-old woman presented with
constipation, a feeling of incomplete evacuation and weight loss.

\textbf{Clinical findings:} A fixed mass was identified on digital
rectal examination. No palpable superficial lymph nodes were identified
and chest x-ray was normal.

\textbf{Diagnostic Assessment:} The colonoscopy revealed a
circumferential ulcerated non obstructive mass about 5 cm from the anal
verge extended up to 10 cm (Figure 1).~Rectal biopsy concluded in
non-Hodgkin's diffuse large B-cell lymphoma (DLBCL) CD20(+), CD5(+),
CD3(+), Bcl-2(--), CD56(--), CD117 (--), cyclin D1 (-), and Ki-67 at
80\% (Figure 2). Cervical and thoraco-abdomino-pelvic computed
tomography (CT-scan) showed irregular circumferential non-stenosing
parietal thickening of the rectum located at 4 cm from the anal verge
and extended over 9cm. Mesorectal and bilateral hypogastric lymph nodes
were identified, the largest one measured 26 x 20 mm (Figure 3). CEA,
LDH, white blood cell count and the bone marrow biopsy were normal.
According to the Ann Arbor staging classification, tumor was classified
stage IIE.

\textbf{Therapeutic intervention:} The patient received six cycles of
R-CHOP chemotherapy including Rituximab, cyclophosphamide, doxorubicin,
vincristine and prednisolone with good tolerance.

\textbf{Follow-up and outcomes} : Radiological and endoscopic evaluation
concluded in a complete remission confirmed with rectal biopsy. The
patient is monitored each 3 months and is still in complete remission
after 18 months of follow up.

\textbf{Discussion:}

The most common histologic subtype of NHL affecting the GI tract and
colon is DLBCL. PRL usually presents with signs and symptoms suggestive
of primary rectal carcinoma: weight loss, abdominal pain and lower
gastro-intestinal bleeding {[}4{]}. Whatever the presentation, endoscopy
with biopsy are the most valuable diagnostic tests. The majority of
colorectal lymphomas are localised in the cecum or ascending colon
{[}5{]}. As reported in the literature, colonoscopy showed in our case a
budding rectal mass, and biopsy confirmed the diagnosis of DLBCL.
Immunohistochemistry usually used to confirm diagnosis is CD20, CD79a,
and CD10~{[}6{]}. CT scan is usually used to study tumor extension.
Concentric rectal wall thickening with or without a regional lymph node
involvement are frequently reported. Positron emission tomography (PET)
is currently indicated in the diagnosis and disease follow-up {[}7{]}.
The Ann Arbor staging system modified by Musshoff is widely used in
tumor classification {[}8{]}. Given the rarity of the rectal
localization, there is no standard treatment protocol. Chemotherapy
remains the the main therapeutic modality {[}9{]}.R-CHOP protocol
including Rituximab, cyclophosphamide, doxorubicin, vincristine and
prednisone remains the most used protocol {[}10{]}. Several studies
reveal a trend toward improved survival when surgery is used in
combination with chemo- or radiotherapy {[}11{]}. In our case, we have
treated patient with chemotherapy with a complete response. Jeong and al
noted a significant difference in survival between patients with stages
I-II and stage IV disease {[}5{]}.

\textbf{Conclusion:}

Management of rectal lymphoma is not well codified because of its
rarity. Chemotherapy +/- radiotherapy is currently the standard
treatment. Surgery is reserved for extensive post-chemotherapy stenosis.
Individualized treatment strategies need to be developed based on
specific clinical situations. Currently we could not make formal
conclusions apart from the effectiveness of chemotherapy and anti CD20
treatment.

\textbf{Competing interests}

The authors declare no competing interest.

\textbf{Figures:}

\textbf{Figure1:} Rectal circumferential ulcerated tumor

\textbf{Figure2:} Histopathology showing~an~H\&E stained section of a
rectal biopsy\selectlanguage{ngerman}×\selectlanguage{english} 40 (A); IHC showing CD20 + rectal cells (B) and CD3+
rectal cells (C); IHC analysis of Ki-67 expression (Ki-67 =80\%) (D).

\textbf{Figure3:} Pelvic CT-scan: parietal thickening of the rectum and
mesorectal and bilateral hypogastric lymph nodes

\textbf{Authors' contributions:}

I confirm that all the authors of the manuscript have read and agreed to
its content.

Sondes BIZID , Fatma BEN FARHAT, Hela GHEDIRA, Sonia BENNASER,

Beya CHELLY, Hela STAMBOULI wrote the paper.

Fehmi Msadek contributed to the quality control and correction of the
manuscript before submission.

\textbf{References}

1. Weingrad DN, Decosse JJ, Sherlock P, Straus D, Lieberman PH, Filippa
DA. Primary gastrointestinal lymphoma: a 30-year review. Cancer. 15 mars
1982;49(6):1258\selectlanguage{english}-65.

2. Dawson IMP, Cornes JS, Morson BC. Primary malignant lymphoid tumours
of the intestinal tract. Report of 37 cases with a study of factors
influencing prognosis. BJS Br J Surg {[}Internet{]}. 1961 {[}cite 26
fevr 2021{]};49(213):80\selectlanguage{english}-9.

3. Doolabh N, Anthony T, Simmang C, Bieligk S, Lee E, Huber P, et al.
Primary colonic lymphoma. J Surg Oncol {[}Internet{]}. 2000 {[}cite 26
fevr 2021{]};74(4):257\selectlanguage{english}-62.

4. J Z, Mv L. Primary colonic lymphoma. Clinical presentation,
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Clin Gastroenterol {[}Internet{]}. 1 juin 1994 {[}cite 27 fevr
2021{]};18(4):291\selectlanguage{english}-7.

5. Jeong JH, Kim S, Kim JE, Yoon DH, Lee S-W, Huh J, et al. Clinical
characteristics, treatment, and outcome of primary rectal lymphoma: a
single center experience of 16 patients. Blood Res {[}Internet{]}. 22
juin 2017 {[}cite 27 fevr 2021{]};52(2):125\selectlanguage{english}-9.

6. Unal B, Karabeyoglu M, Erel S, Bozkurt B, Kocer B, Cengiz O. Primary
rectal lymphoma: an unusual treatment for a rare case. Postgrad Med J.
juin 2008;84(992):333\selectlanguage{english}-5.

7. Rohren EM, Turkington TG, Coleman RE. Clinical Applications of PET in
Oncology. Radiology {[}Internet{]}. mai 2004 {[}cite 27 fevr
2021{]};231(2):305\selectlanguage{english}-32.

8. Carbone PP, Kaplan HS, Musshoff K, Smithers DW, Tubiana M. Report of
the Committee on Hodgkin's Disease Staging Classification. Cancer Res.
nov 1971;31(11):1860\selectlanguage{english}-1.

9. Quayle FJ, Lowney JK. Colorectal Lymphoma. Clin Colon Rectal Surg
{[}Internet{]}. mai 2006 {[}cite 27 fevr 2021{]};19(2):49\selectlanguage{english}-53.

10. Devita VincentT, Canellos GeorgeP, Chabner B, Schein P, Hubbard
SusanP, Young RobertC. ADVANCED DIFFUSE HISTIOCYTIC LYMPHOMA, A
POTENTIALLY CURABLE DISEASE. The Lancet {[}Internet{]}. fevr 1975
{[}cite 28 fevr 2021{]};305(7901):248\selectlanguage{english}-50.

11. Perry PM, Cross RM, Morson BC. Primary Malignant Lymphoma of the
Rectum (22 Cases). Proc R Soc Med {[}Internet{]}. 1 janv 1972 {[}cite 28
fevr 2021{]};65(1):72\selectlanguage{english}-72.

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