JEADV (2004) 18, 196–198
CASE REPOR T
Scalp tumour as a sign of systemic B-cell lymphoma
Blackwell Publishing Ltd.
S Magina,†* J Mesquita-Guimarães,† C Resende,† M Bello,‡ C Dias‡ Departments of †Dermatology and Venereology and ‡Internal Medicine, Faculty of Medicine, Hospital de S. João, Alameda Prof. Hernani Monteiro, 4200 Porto, Portugal. *Corresponding author, tel. +351225089088; E-mail: [email protected]
ABSTRAC T An 86-year-old man presented with a painful reddish tumour on the scalp with a 3-month history, mental confusion with recent onset and lymphadenopathies. Histological examination of the lymph node and cutaneous lesion revealed a dense infiltrate of atypical and large B cells. There was no evidence of bone marrow invasion. According to REAL (Revised European–American Classification of Lymphoid Neoplasms), this lymphoma was considered as a diffuse large B-cell lymphoma with concurrent cutaneous and nodal involvement. Cerebral computerized tomography (CT) scan showed bone and dura mater invasion in the right parieto-occipital region with collapse of lateral ventricle. The patient was submitted to systemic chemotherapy with cyclophosphamide, vincristine and prednisolone (CVP). There was a good response with regression of the cutaneous lesion, but the patient died after the third cycle. We point out the unusual clinical presentation and aggressive behaviour of this lymphoma. Key words: B-cell lymphoma, scalp, cutaneous involvement, classification Received: 20 June 2000, accepted 25 October 2002
Case report An 86-year-old, previously healthy, man presented with a painful reddish tumour on the scalp with a 3-month history of aggressive growing. This lesion was noticed after local trauma and interpreted as a haematoma. On the week before admission the patient had become confused and disorientated. Physical examination revealed a purple tumour on the right parieto-occipital region over an erythematous plaque that occupied the entire scalp (fig. 1). The surface of the lesion was friable and bled readily, with a clinical appearance resembling angiosarcoma. There were bilateral, cervical and supraclavicular lymphadenopathies without hepatosplenomegaly. An incisional biopsy from the cutaneous lesion revealed a dense non-epidermotropic neoplastic infiltrate throughout the entire dermis (fig. 2a). This infiltrate was composed mainly of large lymphoid cells containing nuclear atypias and frequent mitotic figures. In immunohistochemical studies, tumour cells expressed the B-cell-associated antigen marker CD20 (fig. 2b) and CD45. Examination of a lymph node biopsy demonstrated effacement of the node with large pleomorphic lymphoid cells, consistent with nodal lymphoma. The bone marrow biopsy showed no evidence of neoplastic cells. According to REAL 196
fig. 1 Tumour mass on the scalp.
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(Revised European–American Classification of Lymphoid Neoplasms), this lymphoma was considered as a diffuse large B-cell lymphoma with concurrent cutaneous and nodal involvement. Laboratory values revealed leucocytosis (10.4 × 109/L) with lymphopenia (1.22 × 109/L), elevation of seric β2-microglobulin (4660 µg/L) and lactate dehydrogenase (286 IU/L). Serum chemistry profile and immunoelectrophoresis were within normal values. Serological tests for human immunodeficiency virus (HIV), Epstein–Barr virus (EBV) and human T-cell lymphotropic virus (HTLV) were negative. Cerebral computerized tomography (CT) scan showed a volumous infiltration of epicranial tissues with bone and dura mater invasion in the right parieto-occipital region. This lesion was associated with tempero-occipital oedema and mass effect with collapse of trigone and posterior cornu of lateral ventricle. Thoracic and abdominal CT scans were normal. Radiotherapy was excluded because of lesion extension. Three weeks after admission, the patient was submitted to systemic chemotherapy with cyclophosphamide, vincristine and prednisolone (CVP). There was a good response with cutaneous lesion regression, but the patient died after the third cycle.
fig. 2 Cutaneous biopsy with (a) dense non-epidermotropic lymphoid infiltrate (H&E × 40). (b) Positivity of these large cells with B-cell-associated antigen CD20 (immunoperoxidase CD20; × 200).
Skin is the second most common site of extranodal lymphoma1 but non-Hodgkin’s lymphomas affecting the skin are mainly of T-cell origin. Cutaneous B-cell lymphomas represent a heterogeneous group of neoplasms with wide variation in histology, biology and prognosis.2–4 Dependent on the presence of cutaneous or extracutaneous involvement at the time of diagnosis, B-cell lymphomas can be subdivided into three groups: primary cutaneous lymphoma, concurrent cutaneous and nodal lymphoma and secondary cutaneous lymphoma.5 This distinction can, however, be difficult to make, especially before complete clinical evaluation and staging. In this patient there was concurrent cutaneous and nodal disease. The presence or absence of systemic spread is particularly important because primary cutaneous lymphomas are more indolent than nodal lymphomas and therefore they require different therapeutic approaches and have much a better prognosis.3 –5 There are many classification schemes for cutaneous lymphomas, with some confusion regarding the appropriate terminology. EORTC (European Organization for Research and Treatment of Cancer) classification was designed exclusively for the group of primary cutaneous lymphoma and excludes cases, like this one, of cutaneous lymphomas associated with systemic disease.6 Recently, the Revised European-American Classification of Lymphoid Neoplasms (REAL), based on the principle that each lymphoma should be viewed as a distinct disease, recognizes primary as well secondary cutaneous lymphomas.1,2 According to this classification, in B-cell lineage, the most common entities presenting in skin are follicular lymphomas,
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marginal zone lymphomas and, as in our report, diffuse large B-cell lymphomas.2 Although the incidence of secondary skin involvement in nodal B-cell lymphoma has not been determined, cutaneous lesions as a presenting sign are rare in immunocompetent patients.7,8 Bone involvement in non-Hodgkin lymphomas is also uncommon and with late onset.9 Although invasion of the skull is exceptional, lymphoma should be considered when there is a palpable scalp mass and neurological signs. Our case illustrates the unusual presentation of a B-cell lymphoma by a scalp tumour, skull involvement and mental confusion. Furthermore, lesions were trauma related. In a few other reports presentation of non-Hodgkin’s lymphoma was also trauma related but it is still uncertain whether this is merely coincidence.10,11 In our patient trauma may have favoured skull invasion. In systemic lymphomas, prognosis is impaired dramatically by skin involvement. In a series by Sterry et al., mean survival time was 6 months instead of 37 months when only the lymph nodes were involved.7 Extensive skin involvement, neurological lesion, the patient’s age and lymphoid cells morphology all contributed to the poor prognosis in this case.
Acknowledgements We thank Elsa Fonseca for the histological figures.
1 Sander CA, Flaig MJ et al. The Revised European–American Classification of Lymphoid Neoplasms (REAL): a preferred
approach for the classification of cutaneous lymphomas. Am J Dermatopathol 1999; 21(3): 274 –278. Sander CA, Flaig MJ. Morphologic spectrum of cutaneous B-cell lymphomas. Dermatol Clin 1999; 17(3): 593–599. Willemze R, Meijer CJLM et al. Primary cutaneous large cell lymphomas of follicular center cell origin. J Am Acad Dermatol 1987; 16: 518 –526. Santucci M, Pimpineli M, Arganini L. Primary cutaneous B-cell lymphoma: a unique type of low-grade lymphoma. Clinicopathologic and immunologic study of 83 cases. Cancer 1991; 67: 2311–2326. Garcia CF, Weiss LM, Warnke RA, Wood GS. Cutaneous follicular lymphoma. Am J Surg Pathol 1986; 10: 454 –463. Willemze R, Kerl H et al. EORTC classification for primary cutaneous lymphomas. A proposal from the Cutaneous Lymphoma Study Group of the European Organisation for Research and Treatment of Cancer (EORTC). Blood 1997; 90: 354 –371. Sterry W, Kruger GRF, Steigleder G. Skin involvement of malignant B-cell lymphomas. J Dermatol Surg Oncol 1984; 10(4): 276 –277. Helm KF, Su WP et al. Malignant lymphoma and leukemia with prominent ulceration: clinicopathologic correlation of 33 cases. J Am Acad Dermatol 1992; 27: 553 –559. Jimenez Moragas JM, Sanchez Rodriguez A et al. Non Hodgkin’s disease of the skull in a patient with AIDS. Ann Med Int 1999; 16(4): 186–188. Patel JK, Patel M et al. Trauma-related presentation of nonHodgkin’s lymphoma. New York State J Med 1992; 92(7): 332. Morioka T, Tashima T et al. Malignant lymphoma of the scalp at the site of a previous blunt trauma: report of two cases. Surg Neurol 1994; 42(2): 117–120.
© 2004 European Academy of Dermatology and Venereology JEADV (2004) 18, 196–198