|Year : 2020 | Volume
| Issue : 1 | Page : 61-63
Cutaneous mantle cell lymphoma: A rare case report
Salwa Bano, Veena Ramaswamy, BN Tejaswini, Chaitra Chandrashekar
HCG Cancer Centre, Bengaluru, Karnataka, India
|Date of Submission||18-Dec-2018|
|Date of Acceptance||27-Mar-2019|
|Date of Web Publication||06-Jan-2020|
No. 120, F2, Emaar Paradise, 4th Main, 2nd Cross, BTM 1st Stage, Bengaluru - 560 029 Karnataka
Source of Support: None, Conflict of Interest: None
Cutaneous mantle cell lymphoma (MCL) is a very rare entity with very few cases reported till date. We report a case of 69-year-old man with generalized lymphadenopathy who was diagnosed with mitotically active B-cell lymphoma and a year later developed multiple large maculopapular skin rashes which on biopsy showed infiltration of large atypical blast-like cells in the skin and on immunohistochemistry was in favor of MCL-blastoid variant. Skin involvement is rare in MCL, although in some cases, cutaneous manifestations are the first symptom. Skin involvement suggests a disseminated disease and is often associated with blastoid cytological features. Awareness of skin manifestations of MCL is crucial for dermatologists and hematologists for prompt diagnosis and timely treatment. Moreover, it has a dismal prognosis and shows a poor response to standard treatment.
Keywords: Cutaneous lymphoma, Cyclin D1, mantle cell lymphoma
|How to cite this article:|
Bano S, Ramaswamy V, Tejaswini B N, Chandrashekar C. Cutaneous mantle cell lymphoma: A rare case report. Clin Dermatol Rev 2020;4:61-3
| Introduction|| |
Mantle cell lymphoma (MCL) accounts for approximately 5%–10% of non-Hodgkin's lymphoma (NHL) cases. Typically, MCL presents in the fifth to sixth decades of life with generalized lymphadenopathy and extranodal involvement. Cutaneous MCL is a very rare entity with only a handful of cases reported till date. MCL presenting with skin lesions as the primary manifestation is common. However, primary skin involvement is extremely rare and controversial  and is not included in the World Health Organization (WHO)–European Organisation for Research and Treatment of Cancer classification for cutaneous lymphomas. MCL is characterized by immunohistochemistry(IHC) expression of CD5, Cyclin-D1 and SOX-11 and negative expression of CD23. This pattern of reactivity distinguishes from follicular lymphoma, diffuse large B-cell lymphoma (BCL), and chronic lymphocytic leukemia (CLL). The prognosis is generally poor, with a median survival of only 3–4 years; blastoid variant has an even dismal prognosis.
| Case Report|| |
A 69-year-old male, known case of diabetes mellitus and hypertension, presented with complaints of neck swelling for 2 months. Positron emission tomography–computed tomography showed an active bilateral cervical, axillary, deep pectoral, retroperitoneal, pelvic, and bilateral inguinal lymphadenopathy. Metabolically active soft tissue in the nasopharynx, bilateral superior palatal, and lingual tonsil was also seen, which was suggestive of Stage III disease. He underwent bilateral tonsillectomy, which on histopathology showed large atypical mitotically active cells arranged in a diffuse pattern. IHC profile was of BCL (CD20(+), leukocyte common antigen(+), BCL2(+), and Ki67 – 80–90%). The patient was started on R-CHOP regimen. Post six cycles of chemotherapy, the patient showed a complete metabolic response. A year later, the patient developed lesions on the back which was progressive. He also developed nodular swelling and plaque-like erythematous lesions over the trunk [Figure 1]a and [Figure 1]b and extremities. Biopsy of the skin lesion showed large atypical blast-like cells proliferating in the dermis and epidermis in sheets diffusely [Figure 2]a and [Figure 2]b. On IHC, these atypical cells were immunoreactive for CD20 [Figure 3]a, CD5 [Figure 3]b, BCL2 [Figure 3]c, and CD43 [Figure 3]d. Cyclin-D1 [Figure 4] IHC stain was performed which showed a diffuse and strong nuclear positivity in the neoplastic cells. CD23 [Figure 5]a, BCL6 [Figure 5]b, and Tdt [Figure 5]c were negative while Ki67 index was very high (80%) [Figure 5]d. On the basis of clinical presentation, the morphology of tumor cells, and IHC pattern, the patient was diagnosed as disseminated MCL-blastoid variant, involving the skin.
|Figure 1: Nodular and plaque-like cutaneous swelling over the trunk (a) and lower back (b) of a 69-year-old male postremission phase of non-Hodgkin's lymphoma|
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|Figure 2: H and E, ×10 (a) showing the proliferation of neoplastic cells in the dermis and subcutis. High-power view (b) of the tumor cells showing blastic morphology and mitotic activity|
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|Figure 3: Tumor cells showing a strong and diffuse positivity for CD20 (a), CD5 (b), B-cell lymphoma 2 (c), and CD43 (d)|
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|Figure 4: Cyclin-D1 stain by immunohistochemistry showing a diffuse nuclear positivity in the neoplastic cells infiltrating the dermis and subcutis|
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|Figure 5: Neoplastic cells were negative for CD23 (a), B-cell lymphoma-6 (b), and Tdt (c) ruling out lymphoblastic lymphoma and show a very high Ki67 proliferative index (d)|
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Bone marrow transplant was deferred in view of old age, and the patient was started on bendamustine along with rituximab. Gradually, he has attained remission of skin lesions. The patient is now on maintenance therapy with rituximab.
| Discussion|| |
MCL is one of the small B-cell NHLs and usually involves lymph nodes, bone marrow, spleen, liver, gastrointestinal tract, and Waldeyer's ring but rarely skin. Histopathologic examination of a typical MCL demonstrates a homogeneous population of small to medium-sized lymphoid cells with slightly to markedly irregular nuclear contours that resemble centrocytes of a germinal center; however, the blastoid variant reveals tumor cells infiltrating diffusely and having a resemblance to lymphoblasts. Because of the very high mitotic rate seen in these tumors, they sometimes give a starry-sky pattern look on low power.
Some authors  have suggested that MCL can be observed primarily in the skin without systemic involvement; however, mostly cutaneous involvement is a manifestation of systemic disease.
Skin lesions may be the only manifestation of MCL for an extended period.
It is important to differentiate between primary and secondary involvements because it is postulated that patients without extracutaneous disease should not be treated with systemic chemotherapy unlike the latter.
Strict follow-up is necessary to detect new lesions or systemic involvement and to define whether it is a systemic lymphoma or a cutaneous one exclusively.
Patients who develop cutaneous disease with widespread MCL typically have a poor prognosis and poor response to treatment.,
The WHO describes the immunoprofile of MCL as BCL2-expressing, CD10, and BCL6-negative tumors, usually positive for CD5 and CD43, sometimes positive for IRF4/MUM1, and negative or weakly positive for CD23. Nuclear cyclin-D1 is expressed by >95% of MCLs, including the minority of cases that are CD5 negative. Blastoid variant may show aberrant phenotypes including the absence of CD5, expression of CD10 and BCL6, and positivity for LEF1 (CLL associated antigen).
The immunohistochemical profile of MCL of our patient was consistent with that of typical MCL. Furthermore, we got a strong and diffuse positivity for cyclin-D1, which is an indirect evidence of translocation t(11:14), which is pathognomonic for MCL.
Mutation of the t(11;14)(q13;q32) translocation causes the juxtaposition of the CCND-1 gene on chromosome 11 with the immunoglobulin heavy chain gene on chromosome 14 leading to the overexpression of cyclin-D1. This protein acts as a positive signal for the transition from G1 to S phase and stimulates cells to proliferation.
MCL relapsing into blastoid variant and vice versa has been described. It has a dismal prognosis and shows a poor response to standard treatment.
| Conclusion|| |
Cutaneous BCLs are rare and usually results as secondary involvement of underlying disseminated disease. We present one such case where the patient relapsed with skin manifestations of MCL blastoid variant. Awareness of skin manifestations of MCL is crucial for dermatologists and hematologists for prompt diagnosis and timely treatment.
Declaration of patient consent
The informed consent was obtained for participation in the study and publication of data and images for research and educational purposes.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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