|Year : 2018 | Volume
| Issue : 2 | Page : 134-137
Primary cutaneous B-cell lymphoma: A single-center 5-year experience
Linu Abraham Jacob, Vikas Asati, KC Lakshmaiah, Babu K Govind, Dasappa Lokanatha, Suresh MC Babu, KN Lokesh, AH Rudresh, LK Rajeev, Nikita J Mulchandani, Abhishek Anand, Deepak Koppaka, Suma Narayana Mysore
Department of Medical Oncology, Kidwai Cancer Institute, Bengaluru, Karnataka, India
|Date of Web Publication||31-Dec-2018|
Dr. Vikas Asati
Department of Medical Oncology, Kidwai Cancer Institute, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
BACKGROUND: Skin is the second most common site for extranodal non-Hodgkin's lymphoma (NHL). Most primary cutaneous NHLs are of T-cell origin (70%). Primary cutaneous B-cell lymphoma (PCBCL) is a rare entity. MATERIALS AND METHODS: Patients diagnosed with PCBCL between January 2012 and July 2017 at our center were retrospectively analyzed. RESULTS: Eight patients of PCBCL were diagnosed. Three patients (37.5%) were males while 5 patients (62.5%) were females. The median age at diagnosis was 45 years (range, 18–60 years). Scalp was the most common site of involvement (50% of the patients). Diffuse large B-cell lymphoma (DLBCL) was the most common histology (63%), with leg-type DLBCL diagnosed in 1 patient. Two patients had primary cutaneous follicle center lymphoma, whereas the remaining 1 patient had precursor B-lymphoblastic lymphoma. All 5 DLBCL cases were treated with CHOP chemotherapy, and rituximab was given to 3 patients. Of the primary cutaneous follicle center lymphomas, 1 patient with stage II disease was treated with CHOP and is alive without recurrence for the past 5 years, whereas the other patient is on observation alone. The patient with precursor B-lymphoblastic lymphoma was started on MCP-841 protocol; however, the patient did not complete the treatment and died after 11 months. CONCLUSIONS: PCBCL is a heterogeneous group of diseases and dividing them into subtypes, based on morphology and immunophenotype, has therapeutic implications.
Keywords: Cutaneous B-cell lymphoma, cutaneous diffuse large B-cell lymphoma, cutaneous lymphoma prognosis, skin lymphoma
|How to cite this article:|
Jacob LA, Asati V, Lakshmaiah K C, Govind BK, Lokanatha D, Babu SM, Lokesh K N, Rudresh A H, Rajeev L K, Mulchandani NJ, Anand A, Koppaka D, Mysore SN. Primary cutaneous B-cell lymphoma: A single-center 5-year experience. Indian J Cancer 2018;55:134-7
|How to cite this URL:|
Jacob LA, Asati V, Lakshmaiah K C, Govind BK, Lokanatha D, Babu SM, Lokesh K N, Rudresh A H, Rajeev L K, Mulchandani NJ, Anand A, Koppaka D, Mysore SN. Primary cutaneous B-cell lymphoma: A single-center 5-year experience. Indian J Cancer [serial online] 2018 [cited 2019 Mar 25];55:134-7. Available from: http://www.indianjcancer.com/text.asp?2018/55/2/134/249198
| » Introduction|| |
Lymphomas are a heterogeneous group of hematological cancers that arise from cells of the lymphoid system. Lymphoma is divided into two distinct categories – non-Hodgkin's lymphoma (NHL) and Hodgkin's lymphoma. Approximately 85% of lymphomas are of B-cell origin and the remaining are of T-cell origin. The stomach is the most common site for extranodal involvement. According to surveillance, epidemiology, and end results data, the skin is the second most common site of extranodal NHL. Among primary cutaneous lymphomas, T-cell lymphomas are more common than B-cell lymphomas. The Dutch and Austrian Cutaneous Lymphoma registries reported that approximately 70% of all cutaneous lymphomas are of T-cell origin and 22% are of B-cell origin. The World Health Organization and European Organization for Research and Treatment of Cancer (WHO–EORTC) classify primary cutaneous lymphomas into three groups –cutaneous T-cell and natural killer lymphomas, cutaneous B cell lymphomas, and precursor hematologic neoplasms [Table 1]. Indian data on primary cutaneous B-cell lymphoma (PCBCL) is lacking with most publications being case reports. In this article, we report our 5-year experience of cutaneous B-cell lymphomas from a tertiary care center in south India.
|Table 1: Clinical features, pathology, and treatment of primary cutaneous B-cell lymphoma patients|
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| » Materials and Methods|| |
This was a retrospective observational study carried out at Kidwai Cancer Institute, Bengaluru India, from January 2012 to July 2017.
Objectives of the study
There were two main objectives of the study:
- To study the epidemiological, pathological, and clinical characteristics of PCBCL
- To study the treatment pattern and outcome among these patients.
Newly diagnosed patients of B-cell lymphoma, wherein the disease was confined to the skin, with or without its draining lymph node(s), were designated as PCBCL.
Data of lymphoma patients managed at the Department of Medical Oncology, Kidwai Cancer Institute was reviewed. Patients diagnosed with B-cell lymphoma confined to the skin, with or without involvement of the draining lymph nodes, between January 2012 and June 2017 were selected for further analysis. The diagnosis was confirmed by morphology and immunohistochemistry (IHC).
Patient information such as age, sex, presenting complaints, HIV status, treatment received, response to treatment, and outcome was recorded. All patients underwent all the relevant investigations such as hemogram with peripheral smear, biochemistry including lactate dehydrogenase, bone marrow examination, and computed tomography (CT) scan of the neck, thorax, abdomen, and pelvis. Overall survival (OS) was defined as the time (in months) from the diagnosis of disease to death due to any cause. Progression-free survival was defined as the time (in months) from the date of diagnosis to the date of documented disease progression or death.
| » Results|| |
A total of eight cases of PCBCL (confirmed by IHC) were diagnosed during the 5-year study period. The median age of diagnosis was 45 years (range 18–60 years). Three patients (37.5%) were males, whereas 5 patients (62.5%) were females. Scalp was the most common site of involvement (50% of the patients).
Based on morphology and IHC, three different subtypes could be identified:
- Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) – most common histology (5 patients) with leg-type DLBCL diagnosed in one patient
- Primary cutaneous follicle-center lymphoma – two patients
- Precursor B-lymphoblastic lymphoma – one patient.
Primary cutaneous diffuse large B-cell lymphoma
PCDLBCL was the most common histology (63%). Three patients were males, while two patients were females. Scalp was the primary site in three patients. One patient presented with lesions on the legs (leg-type DLBCL) whereas the other had diffuse cutaneous and subcutaneous nodules with perilesional erythema, involving the chest, abdomen, and back [Figure 1]. Based on Hans algorithm, three cases were classified as germinal-center B-cell (GCB) type whereas the other two were non-GCB type. From January 2016, our center started performing c-Myc IHC marker in all newly diagnosed DLBCL cases to identify “Double Expressor DLBCL,” and both cases diagnosed after January 2016 were positive for c-Myc along with positivity for bcl-2. All patients received CHOP-based chemotherapy; three received anti-CD20 antibody (rituximab) along with CHOP [Table 1]. One patient is still on treatment. Two patients are alive without recurrence (follow-up period 31 months and 18 months respectively), one patient who presented with diffuse skin involvement is alive with recurrence (receiving salvage chemotherapy [R-GDP], PFS-11 months), and one patient died after 4 months of completion of treatment. Four out of five patients are still surviving; and hence, the median OS is not reached after a median follow-up of 11 months.
|Figure 1: Clinical images of primary cutaneous diffuse large B-cell lymphoma patient presenting as diffuse subcutaneous nodules with skin involvement before and after the treatment|
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Primary cutaneous follicle-center lymphoma
Two patients were diagnosed with primary cutaneous follicle-center cell lymphoma (PCFCCL). Both patients were females. Left thigh was involved in one patient whereas the left cheek was the primary site in the other. After excision biopsy of the left cheek lesion, a positron emission tomography-CT scan revealed no evidence of disease elsewhere in the body. Hence, the patient was kept on follow-up with clinical examination every 3 months and is alive without any recurrence after follow-up of 65 months. The patient with left thigh involvement was treated with six cycles of CHOP chemotherapy and achieved complete response. She is alive without recurrence after 45 months follow-up.
Precursor B-lymphoblastic lymphoma
One patient was diagnosed with precursor B-lymphoblastic lymphoma of the scalp. Biopsy of the scalp showed infiltration of the dermis by a monotonous population of blast-like cells which were positive for CD20 and Tdt [Figure 2]. Bone marrow and peripheral blood were negative for blasts. The patient was initiated on MCP-841 protocol. She completed the induction phase of the protocol but discontinued further treatment and died after 11 months.
|Figure 2: Histopathological (H and E staining) and immunohistochemistry (CD20 and Tdt positive) images of a patient of primary cutaneous precursor B-lymphoblastic lymphoma|
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| » Discussion|| |
Cutaneous lymphoma is a rare dermatological condition. Two important obstacles to accurate diagnosis (especially in India) is the lack of clinical suspicion by the primary care physician or dermatologist and availability of IHC markers at most centers treating dermatological conditions. Only the tip of the iceberg is often detected at tertiary care centers. In this article, we focused only on cutaneous B-cell lymphomas which are one-fourth in incidence compared to the T-cell counterpart., As per the EORTC-WHO classification (updated in 2008), cutaneous B-cell lymphoma is further divided into 3 subgroups, primary cutaneous marginal zone lymphoma (PCMZL); PCFCCL; and PCDLBCL, leg type (PCDLBCL, LT). PCMZL and PCFCCL belong to the indolent variety while PCDLBCL belongs to the aggressive variety. Based on the histology and site (favorable skin site – head/neck and arm, unfavorable skin site – trunk and leg) cutaneous B-cell lymphoma patients can be divided into four prognostic groups – IA, IB, II, and III, with OS of 81, 72, 48, and 27 months, respectively. This clearly indicates that the cutaneous B-cell lymphoma is a heterogeneous group of diseases, and correct diagnosis based on morphology, IHC, gene rearrangement studies for clonality is of paramount importance.
One of the largest series (a retrospective analysis) on cutaneous B-cell lymphoma was published by the Italian Study Group for Cutaneous Lymphomas involving 467 patients. PCFCL and PCMZL accounted for 57% and 31% of the cases, respectively, whereas PCDLBCL, LT was reported in only 11%. In our series, PCDLBCL was the most common histology.
The clinical course and prognosis of PCBCL is different from nodal lymphoma. The treatment of PCBCL depends on the histology and stage. Various options available in the absence of any randomized control studies include excision, localized treatment in the form of radiotherapy, steroids, and systemic chemotherapy with rituximab.
For indolent histology, radiotherapy and excision are associated with better response rates than chemotherapy (98%, 97%, and 76%–86%), especially for the more limited disease. In our series, also both patients of primary cutaneous follicle-center lymphoma are long-term disease-free posttherapy. As per an Italian study group for cutaneous lymphomas, the CR rate, 5-year OS, and 10-year OS in patients with primary cutaneous follicle center lymphoma, and PCMZL were 92%–95%, 96%–97% and 89%–90.5%, respectively, with RT in 52.5% (dose of 35–45 Gy); chemotherapy in 25% (mainly CHOP) and surgery in 23%. The NCCN guidelines recommend local RT (24–30 Gy; alone or in combination with excision) or excision alone as the initial treatment options for patients with solitary lesions or regional disease (T1-2). Local RT is the preferred initial treatment. In patients with very extensive or symptomatic disease combination, chemotherapy regimens recommended for the treatment of follicular lymphoma may be used.
For aggressive histology such as PCDLBCL, prognosis remains poor with 5-year survival of 73%. Radiotherapy alone is less effective with poor- and short-lived response. Anthracycline-containing chemotherapy with rituximab has better short-term outcome. Among 12 patients treated with anthracycline-based chemotherapy with rituximab, the CR rate was 92% compared with 62% for patients who received other therapies. The 2-year OS rate for these two groups was 81% and 59%, respectively. Moreover, there are several other reports (including ours) that highlight from India the importance of adding rituximab to anthracycline-containing chemotherapy.,
No large data has been published till date on PCBCL from India. The largest series of 8 patients (out of 141 patients of cutaneous lymphoma) was published from KEM Mumbai by Doshi and Khopkar. IHC testing was performed in only 5 cases. To our knowledge, this is the first report from India regarding PCBCL describing various subtypes of PCBCL along with treatment and outcome. For better understanding and improved management of PCBCL, multicenter cooperative studies across the country is needed, as suggested by T. Raychaudhury.
| » Conclusions|| |
PCBCL is a heterogeneous group of diseases and dividing them into subtypes based on morphological and immunophenotyping has therapeutic implications.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
We would like to thank Dr. Abhilasha Goyal for helping in writing this manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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