Dermatology Online Journal

Letter: Primary cutaneous marginal zone B cell lymphoma of the face: A challenging diagnosis
Ana Oliveira MD¹, Mónica Caetano MD¹, Rosário Alves MD², Margarida Lima PhD³, Manuela Selores MD¹
Dermatology Online Journal 18 (2): 12

1. Department of Dermatology, Centro Hospitalar do Porto – Hospital de Santo António, Porto, Portugal
2. Department of Dermatology, Centro Hospitalar do Porto – Hospital de Santo António and of the Multidisciplinary Consultation of Cutaneous Lymphomas, Centro Hospitalar do Porto – Hospital de Santo António, Porto, Portugal
3. Department of Haematology, Centro Hospitalar do Porto – Hospital de Santo António and of the Multidisciplinary Consultation of Cutaneous Lymphomas, Centro Hospitalar do Porto – Hospital de Santo António, Porto, Portugal

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Abstract

The primary cutaneous marginal zone B cell lymphoma is a small B cell lymphoma, including cells of the marginal zone, lymphoplasmacytic cells, and plasma cells. Clinically it manifests as erythematous or erythematous-violaceous papules, plaques, or nodules, single or multiple, most often located to the extremities. Its course is usually indolent, with a survival at 5 years of approximately 97 percent. The tumor exhibits a tendency towards local recurrence, but spread to locations outside the skin is extremely rare. We present a case report of a man, 80 years of age, with a primary cutaneous marginal B cell lymphoma of the chin, an atypical location.

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Primary cutaneous B cell lymphomas represent a proliferation of aberrant immunophenotype B cells localized to the skin, without evidence of extracutaneous disease at the time of diagnosis. They must be distinguished from systemic lymphomas, which may involve the skin secondarily and have a completely different clinical behavior and prognosis.

The World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification divides cutaneous B cell lymphomas (CBCL) into two major groups: lymphomas with indolent clinical behavior and lymphomas with intermediate clinical behavior [1]. Primary cutaneous marginal zone B cell lymphomas (PCMBCL) are included in the first category.

An 80-year-old male was referred to our department in 2009 because of an asymptomatic growth on his chin. The nodule appeared one year before and had gradually enlarged over time. An ultrasound performed two months before suggested the diagnosis of a vascular tumor. The patient was otherwise asymptomatic and denied any constitutional symptoms. The medical history was positive for hypertension and benign prostatic hyperplasia, for which he was medicated with enalapril and finasteride, respectively.

Figure 1	Figure 2
Figure 1. Tumor on patient’s chin Figure 2. Tumor on patient’s oral mucosa

On physical examination, an erythematous-violaceous, slightly quadrangular, infiltrated and non-pulsatile nodule of 6 cm x 5 cm appeared localized to the chin (Figure 1) and corresponding oral mucosa (Figure 2) was noted. No lymphadenopathy, abdominal masses, or organomegaly were detected.

Figure 3	Figure 4
Figure 3. Unremarkable epidermis overlying a dermis with a nodular infiltration (H&E x4) Figure 4. Lymphocytic infiltration (H&E x40)

A punch biopsy revealed an unremarkable epidermis overlying a dermis with a nodular infiltration of atypical lymphocytes, histiocytes, and eosinophils (Figures 3 and 4). The immunohistochemistry showed that the lymphocytic infiltrate was positive for CD20 (Figure 5) and BCL-2 (Figure 6) and negative for CD10, CD5, and BCL-6. It also demonstrated a kappa light chain restriction. A diagnosis of cutaneous marginal zone B cell lymphoma was made.

Figure 5	Figure 6
Figure 5. CD20 positive (x4) Figure 6. BCL-2 positive (x4)

Borrelia burgdorferi serology and PCR on the tissue sample were negative. Laboratory tests were within normal ranges. CT scan of the neck, chest, abdomen, and pelvis showed no lymphadenopathy, hepatomegaly, or splenomegaly and the flow cytometry immunophenotyping of peripheral blood lymphocytes demonstrated no B cell monoclonality, establishing a definitive diagnosis of primary cutaneous marginal zone B cell lymphoma.

The patient was treated with 3 million units of subcutaneous interferon α2a three times a week during six months.

Twelve months after the beginning of the treatment, there is no evidence of local recurrence or extracutaneous disease.

PCMBCL accounts for approximately 8 percent of all non-Hodgkin lymphomas [2] and is one of the most frequent types of CBCL, representing, along with follicle center cell lymphoma, 75 percent of the cases [3].

It usually presents in the fifth and sixth decades as red to violaceous papules, plaques or nodules preferentially located to the trunk (46%) and arms (17%) [2]. In this case, the atypical location on the face and the presentation as a subcutaneous nodule probably led to an incorrect clinical suspicion of a vascular tumor. This hypothesis was also supported by the ultrasound, an exam that can be subjective. The correct diagnosis was made on the basis of the clinical history, physical examination, and histological and immuno-histochemical analysis.

In 2008, a consensus recommendation for treatment of CBCL was published by the EORTC and the International Society for Cutaneous Lymphomas [4]. According to these, patients presenting with solitary lesions can be treated conveniently by surgical excision or radiation therapy. These options may not be the best for young patients or in lesions, as in the present report, located on the face, where these treatments may cause disfiguration [5]. In these conditions, the alternative therapies are subcutaneous or intralesional interferon alpha or rituximab. Given the extent of the tumor, intralesional injection would have been a painful and lengthy procedure. Therefore, we performed subcutaneous instead of intralesional interferon α2a and this produced an excellent response.

References

1. Willemze R, Jaffe ES, Burg G et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105(10):3768-85. [PubMed]

2. Dalle S, Thomas L, Balme B, Dumontet C, Thieblemont C. Primary cutaneous marginal zone lymphoma. Crit Rev Oncol Hematol 2010;74(3):156-62. [PubMed]

3. Kyrtsonis MC, Siakantaris MP, Kalpadakis C et al. Favorable outcome of primary cutaneous marginal zone lymphoma treated with intralesional rituximab. Eur J Haematol 2006;77(4):300-3. [PubMed]

4. Senff NJ, Noordijk EM, Kim YH et al. European Organization for Research and Treatment of Cancer and International Society for Cutaneous Lymphoma consensus recommendations for the management of cutaneous B-cell lymphomas. Blood 2008;112(5):1600-9. [PubMed]

5. Rosmaninho A, Alves R, Lima M et al. Red nose: primary cutaneous marginal zone B-cell lymphoma. Leuk Res. 2010;34(5):682-4. [PubMed]

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