Year : 2006 | Volume
: 51 | Issue : 2 | Page : 125--127
Primary cutaneous diffuse large B-cell lymphoma of the upper limb
R Srinivasan1, Y Mutteswaraiah2, Sudha S Bhat1, Padmanabha R Bhat2, BM Vadhiraja3, KS Vengetesh2,
1 Department of Pathology, Kasutba Medical College, Manipal - 576104, Karnataka, India
2 Department of Surgery, Kasutba Medical College, Manipal - 576104, Karnataka, India
3 Department of Radiotherapy and Oncology, Kasutba Medical College, Manipal - 576104, Karnataka, India
Department of Pathology, Kasturba Medical College, Manipal - 576104, Karnataka
We report a case of a 70-year-old man who presented with a rapidly growing ulceroproliferative lesion on the right upper limb, clinically resembling a soft tissue sarcoma. The lesion turned out to be primary cutaneous CD 20 +, diffuse large B-cell lymphoma. There was no evidence of systemic involvement on computed tomography (CT) scans of the chest, abdomen and pelvis. Local radiotherapy induced complete remission of the B-cell lymphoma..
|How to cite this article:|
Srinivasan R, Mutteswaraiah Y, Bhat SS, Bhat PR, Vadhiraja B M, Vengetesh K S. Primary cutaneous diffuse large B-cell lymphoma of the upper limb.Indian J Dermatol 2006;51:125-127
|How to cite this URL:|
Srinivasan R, Mutteswaraiah Y, Bhat SS, Bhat PR, Vadhiraja B M, Vengetesh K S. Primary cutaneous diffuse large B-cell lymphoma of the upper limb. Indian J Dermatol [serial online] 2006 [cited 2020 Feb 19 ];51:125-127
Available from: http://www.e-ijd.org/text.asp?2006/51/2/125/26935
Cutaneous lymphomas are uncommon skin tumors, with skin being either the primary site for the origin of the tumor or more often the secondary site. Approximately 65% of the cutaneous lymphomas are T cell in origin and only 20-25% are thought to originate from the B cell, with majority of these being diffuse large B cell type., As defined by the Dutch and European Organization for research and treatment of cancer (EORTC) lymphoma working groups, the primary cutaneous lymphomas are those in which the skin involvement by the tumor is clinically evident with no extracutaneous disease at the time of diagnosis or for 6 months thereafter., Most of the primary cutaneous lymphomas have an indolent behaviour., According to the REAL/WHO classification those Non Hodgkin's lymphomas which have large cell morphology and diffuse growth pattern are designated as diffuse large B cell lymphoma (DLBCL). Although this classification does not identify primary cutaneous DLBCL as a specific type, it recommends reference to the site of origin when reporting cutaneous B cell lymphomas, since the site of presentation is a marker for the underlying biologic differences.,, Many studies have supported the contention of the EORTC cutaneous lymphoma working groups in implicating the location on the leg as the most significant factor in predicting adverse prognosis in case of primary cutaneous DLBCL, and hence has been classified as a separate entity., Those with similar morphology, but occurring at locations other than the leg are classified as Follicular center cell lymphomas., There is a growing acceptance that the primary cutaneous DLBCL has better prognosis than the morphologically identical lesion in lymph nodes and majority of these are de-novo lesions and diffusely involve the dermis.
In this report, we describe a 70-year-old man with primary cutaneous diffuse large B-cell lymphoma of the upper limb, who presented with an ulceroprolifeative lesion over the right elbow region prompting a clinical diagnosis of a soft tissue sarcoma.
A 70-year-old man presented with 3 months history of a rapidly growing ulceroproliferative lesion over the right elbow region. He was treated for iron deficiency anemia one year back.
At presentation the growth was present over the medial aspect of the right elbow region, measured 8 x 10 cm in size, with 6 x 6 cm ulcer over the swelling [Figure 1]. The floor of the ulcer was covered with slough, had raised edges and was not fixed to the underlying structures. No significant lymphadenopathy was detected clinically.
There was no evidence of lymphadenopathy on computed tomography (CT) scans of the chest, abdomen and pelvis. Peripheral blood smear examination showed a microcytic hypochromic blood picture. Bone marrow aspiration and biopsy showed erythroid hyperplasia with features of iron deficiency erythropoesis and no evidence of infiltration, indicating the absence of systemic involvement.
Biopsy from the mass showed a dense diffuse infiltrate of atypical lymphoid cells involving the entire dermis. There was no epidermotropism and no evidence of follicular architecture [Figure 2]. Majority of the tumor cells were large, non cleaved, centroblasts with a few immunoblasts [Figure 3].
On immunohistochemistry, the large atypical neoplastic cells were negative for EMA and S-100, thus ruling out the possibility of a carcinoma or a melanoma. the cells stained positive for CD 45, CD 20, and showed no staining with CD 45 RO confirming the lesion to be a lymphoma of B-cell phenotype. A final diagnosis of primary diffuse large B cell lymphoma of the upper limb based on the REAL/WHO classification was made.
Using a linear accelerator, local radiotherapy with a total dose of 40 Gy over 4 weeks duration was initiated. This led to a complete clinical remission of the tumor and the patient is disease free for the past 1 year.
Primary cutaneous diffuse large B-cell lymphoma (DLBCL) is an uncommon lymphoma and is sub classified based on the anatomic location. There is a growing consensus that the primary cutaneous DLBCL of leg is unique in its behaviour as opposed to the DLBCL occurring at the other sites.,,
Primary cutaneous DLBCL of leg differs from DLBCL occurring at the other sites in that these patients are of older age group, more frequently females, have a short duration of skin lesion before diagnosis, often develop extracutaneous disease, histologically are composed predominantly of round blasts cells as opposed to the cleaved cells and often express bcl-2 protein., They have reduced disease free survival and a worse prognosis.,
In the European multicentric study of 145 patients with primary cutaneous DLBCL, round-cell morphology, location on the leg, and multiple skin lesion at diagnosis were found to be independent adverse prognostic factors.
Primary cutaneous lymphomas are highly sensitive to radiotherapy, and hence, it is the treatment of choice for localized disease at presentation or relapse. Inclusion of margin of at least 2 cm of healthy skin into the irradiation field and an approximate total dose of 30 Gy results in complete remission.
Combination chemotherapy with CHOP is preferred in patiens with the tumor involving the leg, multiple skin lesions or with systemic involvement.,
Our case is interesting in that, unlike the usual primary cutaneous DLBCL occurring above the waist, the lesion was rapidly growing with short duration of 3 months presentation before diagnosis, and was ulceroproliferative in nature, as opposed to a plaque or a nodule, thus prompting a clinical diagnosis of a soft tissue sarcoma.
|1||Garbea A, Dippel E, Hildenbrand R, Bleyl U, Schadendorf D, Goerdt S. Cutaneous large B-cell lymphoma of the leg masquerading as a chronic venous ulcer. Br J Dermatol 2002;146:144-7. |
|2||Hembury TA, Lee B, Gascoyne RD, Macpherson N, Yang B, House N, et al . Primary cutaneous diffuse large B-cell lymphoma: A clinicopathologic study of 15 cases. Am J Clin Pathol 2002;117:574-80. |
|3||Willemze R, Kerl H, Sterry W, Berti E, Cerroni L, Chimenti S, et al . EORTC classification for Primary Cutaneous Lymphomas: A proposal from the Cutaneous lymphoma study group of European Organisation for Research and Treatment of Cancer. Blood 1997;90:354-71. |
|4||Gronbaek K, Moller PH, Nedergaard T, Thomsen K, Baadsgaard O, Hou-Jensen K, et al . Primary cutaneous B-cell lymphoma: A clinical, histological, phenotypic and genotypic study of 21 cases. Br J Dermatol 2000;142:913-23. |
|5||Goodlad JR, Krajewski AS, Batstone PJ, McKay P, White JM, Benton EC, et al . Primary cutaneous large B-cell lymphoma: Prognostic significance of Clinicopathological subtypes. Am J Surg Pathol 2003;27:1538-45. |
|6||Grange F, Bekkenk MW, Wechsler J, Meijer CJ, Cerroni L, Bernengo M, et al . Prognostic factors in primary cutaneous large B-cell lymphomas: A European multicentric study. J Clin Oncol 2001;19:3602-10.|