Primary cutaneous follicle center lymphoma of the medial canthus
of the eye in an eleven year old
Paul R. D’Alessandro, MD, MSc1, Andrea C. Lo,
MD2, Martin H. Spencer, MD3, Pedro
Farinha, MD, PhD4, Linlea Armstrong,
MD5, Peter J. Dolman, MD6, Sylvia
Cheng, MD1
1Division of Pediatric Hematology/Oncology/BMT,
British Columbia Children’s Hospital, University of British Columbia,
Vancouver, British Columbia, Canada
2Division of Radiation Oncology and Developmental
Radiotherapeutics, Department of Surgery, University of British
Columbia, British Columbia Cancer Agency, Vancouver, British Columbia,
Canada
3 Department of Ophthalmology, Nanaimo Regional
General Hospital, Nanaimo, British Columbia, Canada
4Department of Pathology and Laboratory Medicine,
University of British Columbia, British
Columbia Cancer Agency, Vancouver, British Columbia, Canada
5Department of Medical Genetics, University of British
Columbia, Children’s and Women’s Health Centre of BC, Vancouver, British
Columbia, Canada
6Department of Ophthalmology & Visual Sciences,
University of British Columbia, Vancouver General Hospital, Vancouver,
British Columbia, Canada
Corresponding Author:
Dr. Sylvia Cheng
Room B315, 4480 Oak Street, Vancouver, British Columbia, V6H 3V4
Phone : 604.875.2406, Fax : 604.875.2911, Email :
sylvia.cheng@cw.bc.ca
Word Count: Main Text: 462, not including Figures or References
Key Words: primary cutaneous follicle center lymphoma; pediatric;
ophthalmology
Abbreviations
PCFCL: primary cutaneous follicle center lymphoma, HHV8: human herpes
virus 8
To the Editor:
We present the fifth case of primary cutaneous follicle center lymphoma
(PCFCL) documented in a patient under age twenty-one. PCFCL is a
low-grade lymphoma of the follicle center B cells limited to skin
without systemic or nodal involvement at diagnosis.1,2Patients typically present with localized, non-ulcerated, erythematous
lesions on the scalp, forehead, or trunk.1,3,4 PCFCL
presenting on the lower extremity carries a poorer
prognosis.1,4 The etiology remains unknown. AntecedentBorrelia burgdorferi , hepatitis C, and human herpes virus 8
(HHV8) infections have been proposed.2,4
Neoplastic lymphocytes in PCFCL are composed of centrocytes and
centroblasts derived from germinal center B cells.3These cells express B-cell and follicle center markers, usually BCL6
and/or CD10.3,4 BCL2 expression is variable, though
usually absent.1,3 Ki-67 proliferation index may be
low.1 Reactive T lymphocytes are often
seen.1,2,4 PCFCL is not usually associated with BCL2
t(14;18.)1,2,3,4
In adults, five-year progression free survival exceeds
95%.2 Locally aggressive disease has been described
in untreated patients.1 Treatment for adult patients
includes surgical resection and/or radiation therapy.2Rituximab has been used for extensive lesions.2,4Transformation to diffuse large B-cell lymphoma is
uncommon.1 Recurrences, mostly extracutaneous, occur
in 20-30% of patients.1,2,4 The specific prognosis of
pediatric PCFCL is unknown due to its rarity.3
The affected individual is an eleven-year-old male who presented with a
twelve-month history of an enlarging mass arising from the plica
semilunaris and caruncle in the medial canthus of his right eye (five mm
maximal diameter.) It was associated with keratoconjunctivitis sicca and
incomplete eyelid closure. The lesion was pink with central erythema. An
ophthalmologist excised the lesion with topical anesthetic without
complication. Pathology demonstrated PCFCL. Resection margin was
negative (Figure 1.) Chest x-ray, bone marrow aspirate/biopsy, MRI
brain/orbits, lumbar puncture with CSF analysis, and whole body
f-fluorodeoxyglucose (FDG) positron emission tomography were negative
for distant disease. Serologies for B burgdoferi and hepatitis C
were negative. Imaging identified anatomic variants: fused C2-C3
vertebral bodies and posterior elements, right-sided aortic arch,
horseshoe kidney, bifid ribs, and thoracic hemivertebrae with levoconvex
curve. This patient was reviewed by a medical geneticist. There was no
clinical evidence of hereditary cancer syndrome. The family declined
whole exome sequencing.
Institutional multidisciplinary case conference recommended adjuvant
radiation therapy or observation. We elected for observation. The
patient remains disease-free at two years, with clinical exams by a
pediatric oncologist and ophthalmologist (every six months) and
pediatric dermatologist (annually.) This represents the fifth documented
case of pediatric PCFCL. Clinical features are consistent with the four
prior published cases.3,5,6,7,8 Those cases had
variable treatments, including surgery, with or without chemotherapy or
radiation therapy, and follow-up ranging from 13 to 48
months.5,6,7,8 Ceppi et al advocate for
complete surgical resection of single lesions followed by observation
due to late effects of radiation.3 This patient’s
outcome supports this conservative approach in a pediatric setting.
Figure 1: Polypoid fragment lined with non-keratinized squamous
epithelium with an atypical lymphoid infiltrate with nodular pattern
(Figure 1A, HE 2x.) The atypical lymphoid cells are variable in size
with small to medium centrocyte-like cells admixed with scattered larger
centroblast-like cells. Single cell necrosis and mitotic figures are
noted (Figure 1B, HE 40x.) The atypical cells are B-cells that express
strong CD20, CD10 and BCL6 as well as uniform BCL2. Fewer CD3+ T cells
are noted surrounding the nodular areas. The CD21 staining highlights
expanded and disrupted follicular dendritic cell meshwork consistent
with atypical follicles. The proliferation index of the B-cells (Ki-67)
is moderate, approximately 60%. DNA PCR analysis using BIOMED2
immunoglobulin heavy chain gene consensus primers confirmed the presence
of a clonal B cell population (Figure 1C.) FISH analysis using Vysis
break-apart probes for the rearrangements of BCL2 and BCL6was negative.
CONFLICT OF INTEREST
The authors have no conflicts of interest to declare.
ACKNOWLEDGEMENTS
The authors gratefully acknowledge the patient and his family for
supporting this publication.
ETHICS STATEMENT
The patient and his parents have consented to publication of this
clinical case.
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