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Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 268-271
Primary cutaneous large B-cell lymphoma of scalp: Case report of a rare variant

1 Department of Pathology, R. N. Cooper Hospital, Mumbai, Maharashtra, India
2 Department of Dermatology, R. N. Cooper Hospital, Mumbai, Maharashtra, India

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Date of Web Publication19-Jun-2017


Primary cutaneous large B-cell lymphoma (Bcl) is defined as a lymphoma composed of large cells constituting more than 80% of the infiltrate and absence of extracutaneous involvement after staging investigations. In the new World Health Organization/European Organization for Research and Treatment of Cancer classification, cutaneous Bcls with large cells are of three types - primary cutaneous large Bcl leg type (PCLBCLLT), primary cutaneous follicle center lymphoma diffuse type (PCFCLDT), and primary cutaneous large Bcls other (PCLBCLO). These three different types are distinct in terms of their clinicopathological features and survival. The PCLBCLO has intermediate features between those of PCLBCLLT and PCFCLDT. We present a case of PCLBCLO in a 57-year-old male who presented with a scalp swelling. Ultrasonography examination was suggestive of a sebaceous cyst. Computed tomography scan revealed the presence of an ill-defined hyperdense region in the soft tissue of the scalp region extending into the deeper layers of the scalp. Fine-needle aspiration cytology (FNAC) revealed the presence of atypical lymphoid cells. Diagnosis was confirmed by biopsy and immunohistochemistry. Patient received rituximab combined with doxorubicin, vincristine, cyclophosphamide, and prednisolone regimen with complete resolution of the lesion. We present this case for its rarity, the utility of FNAC in early diagnosis, and to discuss the differential diagnosis.

Keywords: Cutaneous lymphoma, diffuse large B-cell lymphoma other type, immunohistochemistry, scalp

How to cite this article:
Khatib Y, Dande M, Patel RD, Makhija M. Primary cutaneous large B-cell lymphoma of scalp: Case report of a rare variant. Indian J Pathol Microbiol 2017;60:268-71

How to cite this URL:
Khatib Y, Dande M, Patel RD, Makhija M. Primary cutaneous large B-cell lymphoma of scalp: Case report of a rare variant. Indian J Pathol Microbiol [serial online] 2017 [cited 2022 Jan 27];60:268-71. Available from: https://www.ijpmonline.org/text.asp?2017/60/2/268/208414

   Introduction Top

Primary cutaneous large B-cell lymphomas (Bcls) are a unique group of skin lymphomas characterized by the absence of extracutaneous manifestations at diagnosis. Diffuse large Bcl (DLBCL) is the most common form of non-Hodgkin's lymphoma accounting for more than one-third of all lymphomas.[1] Although it usually occurs in the lymph nodes, it can arise in other tissues such as intestine, bone, breast, liver, lung, skin, and central nervous system.[1] In the new World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification of cutaneous lymphomas, primary cutaneous Bcls (PCBCLs) with large cells are divided into three groups. Primary cutaneous large Bcl leg type (PCLBCLLT) is defined as a cutaneous Bcl with a predominance of large cells (centroblasts and immunoblasts) that are positive for Bcl-2. Cases of PCLBCL with a predominant cleaved cell morphology (large centrocytes) are included in the follicular center cell lymphoma diffuse type. Rare cases not fitting into these two categories are included in the group of PCLBCLO as the present case.[2] Recognition of the correct entity led to appropriate therapeutic strategy with favorable outcome.

   Case Report Top

A 57-year-old male presented with a gradually increasing solitary scalp swelling for 5 months. He was a known case of diabetes mellitus and hypertension on regular treatment. There were no other systemic complaints. Clinical examination revealed a single 2 cm × 2 cm skin covered nontender scalp swelling with bosselated surface, irregular margins, and firm consistency. There was no evidence of lymphadenopathy, splenomegaly, hepatomegaly, or similar lesions on the body. A clinical differential diagnosis of proliferating trichilemmal cyst versus appendageal tumor was put forth. Ultrasonography of the scalp was suggestive of a sebaceous cyst. A plain axial computed tomography (CT) of the brain was performed which revealed an ill-defined hyperdense lesion in the soft tissue of the scalp over the right parietal eminence. It had irregular margins and was extending into the deeper layers of scalp [Figure 1]a. No underlying bony erosion was noted. Fine-needle aspiration cytology (FNAC) of the lesion was carried out using a 24-gauge needle and Papanicolaou- and Giemsa-stained smears were studied. Smears were cellular showing many large atypical lymphoid cells with large nuclei arranged singly and in small groups [Figure 2]a. Based on the FNAC findings, the lesion was considered to be suspicious for non–-Hodgkin's lymphoma. A whole body positron emission tomography-CT and diagnostic contrast CT were carried out. The soft tissue lesion involving the scalp showed uptake of fludeoxyglucose (SUVmax: 6.1). It measured 3 cm × 2 cm in maximum transverse dimensions and approximately 0.7 cm in superoinferior extent. The underlying bone was not involved [Figure 1]b. For further workup, a 6 mm punch biopsy was done from the lesion. Histologically, the skin biopsy showed an unremarkable epidermis. The tumor was seen in the dermis below a grenz zone and was composed of diffuse population of large malignant cells separated by few bundles of hyaline tissue [Figure 2]b. The cells were large with scanty cytoplasm and large round hyperchromatic nuclei [Figure 2]c. No definite epidermotropism or plasma cell infiltration was identified. Occasional mitosis was seen. On immunohistochemistry (IHC), the tumor was positive for CD20 [Figure 3]a and weakly positive for Bcl-6, whereas negative for CD3, Bcl-2, CD10, CD30, and MUM-1. CD23 was negative and did not show any dendritic cell meshworks. Stain for CD 4 was equivocal. MIB-1 labeling index was approximately 80%–85% [Figure 3]b. CT scan of chest, abdomen, and pelvis was unremarkable. Bone marrow aspiration and biopsy were unremarkable. A final diagnosis of primary cutaneous large Bcl other (PCLBCLO) type was made based on morphology, IHC, and staging investigations. Other investigations such as complete blood count and peripheral smear were within normal limits. Liver function tests and renal function tests were within normal limits. Serology for HIV, hepatitis B surface antigen, and hepatitis C virus was nonreactive. The disease was staged as stage IAE DLBCL – scalp location, nonbulky disease. Patient was treated with three cycles of chemotherapy comprising rituximab, vincristine, adriamycin, cyclophosphamide, and prednisolone (R-CHOP). The skin lesion had completely regressed following the treatment.
Figure 1: (a) Computed tomography scan focal, well-defined solid scalp soft tissue mass with diffuse, homogeneous enhancement was identified on computed tomography head. The fat planes are intact and so is the underlying cortex. There is no reactive sclerosis or lysis of the adjacent bone. (b) Positron emission tomography on whole body positron emission tomography, there is no other focal area of fludeoxyglucose uptake to suggest additional area of involvement

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Figure 2: (a) Microphotograph of fine-needle aspiration cytology of scalp swelling showing loose population of atypical lymphoid cells in small clusters (H and E, ×400). (b) Microphotograph showing diffuse infiltration in dermis with sheets of large monomorphic cells separated by bands of hyaline tissue (H and E, ×400). (c) Microphotograph showing large cells with scanty cytoplasm and large, hyperchromatic nuclei (H and E, ×400)

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Figure 3: (a) Microphotograph showing positivity for CD20 (IHC, ×400). (b) Microphotograph showing positivity for Ki67, MIB-1 labeling index 80%–85% (IHC, ×400)

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   Discussion Top

PCBCL belongs to a distinct group of lymphoproliferative disorders defined by its presentation in the skin without evidence of extracutaneous spread at the time of diagnosis.[3] Extranodal involvement occurs in approximately 25% of non–Hodgkin's lymphomas with the gastrointestinal tract (GIT) being the most common site of extranodal involvement, followed by skin.[4] The annual incidence of cutaneous lymphomas is approximately 0.5–1/100,000.[3] PCBCL group represents 20%–25% of all primary cutaneous lymphomas.[5] The new WHO/EORTC classification of cutaneous lymphomas identifies three main subtypes of PCBCL primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and PCLBCL.

The pathogenesis of PCBCL is unclear. There is some speculation that PCBCL may represent a lymphoproliferative response to antigenic stimuli in the cutis, a skin-associated lymphoid tissue-related Bcl (a process similar to mucosa-associated lymphoid tissue in GIT).[6] In Europe Jelic et al. linked positive Borrelial serology to the development of PCBCL.[7] Molecular analysis of PCBCL supports the hypothesis of an antigen-driven germinal center origin based on findings of a characteristic pattern of somatic hypermutation and the presence of intraclonal diversity in B-cell immunoglobulin genes.[8]

The subtype identified in our patient, i.e., PCLBCLO is a rare variant of cutaneous lymphoma. The 2005 WHO/EORTC classification defines the category of PCLBCLO as exceptional cases of PCLBCL that present in the skin but does not meet the diagnostic criteria for PCLBCLLT or PCFC diffuse type (PCFCLDT).[9]

In a study of 93 cases of PCLBCL, there were only nine cases of PCLBCLO. There were two men and seven women in this group with a median age of 70 years. Only one case was located in the head and neck region as the present case. Lesions were solitary in 7/9 cases. Kim et al. did a study on 25 cases of primary and secondary lymphomas and found primary cases to be more frequent in the head and neck region.[10] Kim et al. reviewed 21 patients of PCLBCL in Korea. Out of 2831 cases of Bcls, only two cases of PCDLBCLO type were found.[11] Out of 79 cases of PCLBCL of skin analyzed by Plaza et al., 26 were of other type. There were 9 women and 17 men. Presentation was at varied sites such as face, leg, shoulder, abdomen, and chest; only three cases were on the head region.[12]

Definitive diagnosis is based on the combination of morphologic, histologic, immunohistochemical, and genotypic analysis of biopsy specimen. Histopathology reveals diffuse nonepidermotropic infiltrate made up of monotonous population of large noncleaved B-cells usually extending into subcutaneous tissue. The neoplastic cells express B-cell markers such as CD20 and CD79a but are negative for Bcl-2 and MUM-1. Proliferation rate index was high in the majority of the cases. PCLBCLO type had mean proliferation index of 62.7%.[2] In terms of clinicopathologic features, phenotype, and prognosis, it seems that PCLBCLO represents an intermediate group between cases of PCLBCLLT and those of PCFCLDT with more similarities to former than latter type.[2] At present, it is unclear whether PCLBCL truly represents a distinct group of PCLBCL or it should be considered a Bcl2 negative variant of PCLBCLLT or round cell variant of PCFCLDT.[2]

The differential diagnosis includes metastasis of small cell carcinoma, Merkel cell carcinoma, pseudolymphoma, and sarcoma. Morphology and IHC studies will aid in the diagnosis. Within the group of PCLBCL, a diffuse, nonepidermotropic infiltrate composed of sheets of centroblasts and immunoblasts in the dermis with an immunophenotype positive for CD20, Bcl-2, Bcl6, MUM-1 positive and negative for CD10 and CD 3 is diagnostic of PCLBCLLT. A diffuse infiltrate composed of centrocyte-like cleaved cells with intermingled centroblasts and immunoblasts positive for CD20, Bcl-6, and CD10 with Bcl-2 negativity favors a diagnosis of PCFCLDT. PCMZL is an indolent type of B lymphoma showing a nodular or diffuse nonepidermotropic infiltrate composed of small-to-medium-sized lymphoid cells or plasmacytoid cells which are CD20 and Bcl-2 positive but CD10 and Bcl-6 negative.[11] Hence, cases with large round cell morphology and Bcl-2 negativity will comprise the other type as the present case.

Recommended treatment of cases of other type is similar to PCBCLLT with multiagent chemotherapy of R-CHOP regimen giving a favorable response.[13] The 5-year survival reported in these cases is 50%.

   Conclusion Top

The new definitions of the groups of PCLBCLLT, PCLBCLO, and PCFCLDT allow a more reliable distinction between indolent and aggressive types of cutaneous large Bcls and facilitate the decision whether chemotherapy or radiation will be used as the treatment of choice.[9] FNAC can help in initial quick diagnosis as in the present case. However, confirmation by biopsy and immunochemistry is necessary for definite diagnosis.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Bourdeanu L, Menon R, Somlo G. Diffuse large B-cell lymphoma with calf muscle localization. Case Rep Hematol 2011;2011:292494.  Back to cited text no. 1
Kodama K, Massone C, Chott A, Metze D, Kerl H, Cerroni L. Primary cutaneous large B-cell lymphomas: Clinicopathological features, classification, and prognostic factors in a large series of patients. Blood 2005;106:2491-7.  Back to cited text no. 2
Pandolfino TL, Siegel RS, Kuzel TM, Rosen ST, Guitart J. Primary cutaneous B-cell lymphoma: Review and current concepts. J Clin Oncol 2000;18:2152-68.  Back to cited text no. 3
Santucci M, Pimpinelli N. Primary cutaneous B-cell lymphomas. Current concepts. I. Haematologica 2004;89:1360-71.  Back to cited text no. 4
Senff NJ, Noordijk EM, Kim YH, Bagot M, Berti E, Cerroni L, 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;112:1600-9.  Back to cited text no. 5
Slater DN. MALT and SALT: The clue to cutaneous B-cell lymphoproliferative disease. Br J Dermatol 1994;131:557-61.  Back to cited text no. 6
Jelic S, Filipovic-Ljeskovic I. Positive serology for Lyme disease borrelias in primary cutaneous B-cell lymphoma: A study in 22 patients; is it a fortuitous finding? Hematol Oncol 1999;17:107-16.  Back to cited text no. 7
Gellrich S, Rutz S, Golembowski S, Jacobs C, von Zimmermann M, Lorenz P, et al. Primary cutaneous follicle center cell lymphomas and large B cell lymphomas of the leg descend from germinal center cells. A single cell polymerase chain reaction analysis. J Invest Dermatol 2001;117:1512-20.  Back to cited text no. 8
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 9
Kim BK, Surti U, Pandya AG, Swerdlow SH. Primary and secondary cutaneous diffuse large B-cell lymphomas: A multiparameter analysis of 25 cases including fluorescence in situ hybridization for t(14;18) translocation. Am J Surg Pathol 2003;27:356-64.  Back to cited text no. 10
Kim MJ, Hong ME, Maeng CH, Jung HA, Hong JY, Choi MK, et al. Clinical features and treatment outcomes of primary cutaneous B-cell lymphoma: A single-center analysis in South Korea. Int J Hematol 2015;101:273-8.  Back to cited text no. 11
Plaza JA, Kacerovska D, Stockman DL, Buonaccorsi JN, Baillargeon P, Suster S, et al. The histomorphologic spectrum of primary cutaneous diffuse large B-cell lymphoma: A study of 79 cases. Am J Dermatopathol 2011;33:649-55.  Back to cited text no. 12
Li X, Liu Z, Cao J, Hong X, Wang J, Chen F, et al. Rituximab in combination with CHOP chemotherapy for the treatment of diffuse large B cell lymphoma in China: A 10-year retrospective follow-up analysis of 437 cases from Shanghai Lymphoma Research Group. Ann Hematol 2012;91:837-45.  Back to cited text no. 13

Correspondence Address:
Richa D Patel
Department of Pathology, R. N. Cooper Hospital, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.208414

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