Primary cutaneous anaplastic large cell lymphoma: A diagnostic challenge

Primary cutaneous anaplastic large cell lymphoma: A diagnostic challenge

P6645 P6801 Plasmablastic lymphoma of the oral cavity: A case report Janeth Cristina Cardona-Alzate, MD, Complejo Hospitalario de Toledo, Toledo, Sp...

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P6645

P6801

Plasmablastic lymphoma of the oral cavity: A case report Janeth Cristina Cardona-Alzate, MD, Complejo Hospitalario de Toledo, Toledo, Spain; Blas Alexis Gomez-Dorado, MD, Complejo Hospitalario de Toledo, Toledo, Spain; Cristina Schoendorff-Ortega, MD, Complejo Hospitalario de Toledo, Toledo, Spain; Fabienne Robuschi-Lestouquet, MD, Complejo Hospitalario de Toledo, Toledo, Spain; Silvia Honorato-Guerra, MD, Complejo Hospitalario de Toledo, Toledo, Spain

Primary cutaneous B-cell lymphoma: A case report Asfa Akhtar, DO, Cleveland Clinic Florida, Weston, FL, United States; Roya Ghorsriz, DO, Nova Southeastern University Department of Dermatology, Davie, FL, United States The classification of primary cutaneous B-cell lymphoma (PCBCL) is controversial. PCBCL is divided into 3 major subtypes based on the World Health OrganizationEuropean Organization for Research and Treatment of Cancer (EORTC). These include primary cutaneous follicle center lymphoma (PCFCL), primary cutaneous marginal zone B-cell lymphoma (PCMZL), and primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL, LT). A combination of clinical, histologic, immunologic, and molecular criteria is required in the diagnosis of PCBCL. Primary cutaneous B-cell lymphomas are generally located on the head and neck, with the trunk and extremities affected to a lesser degree. PCFCLs present with plaques or nodules localized to the scalp, face, or trunk followed by the legs. The upper extremities tend to be spared. PCMZLs comprise cases with erythematous papules, nodules or plaques on the trunk or extremities. PCLBCL, LTs present as rapidly growing violaceous-red tumors on the lower extremities. Immunohistologically all 3 PCBCLs are positive for the B-cell markers CD20 and CD79a. There has also been an associated relationship with infectious triggers to include Borrelia burgdorferi, Helicobacter pylori, and EpteineBarr virus. Systemic lymphoma workup is warranted in all cases of cutaneous B-cell lymphoma. Treatment involves multiple modalities to include specific treatment aimed at concurrent or suspected infection. We present a case of a 56-year-old Israeli man who presented to the deparment of dermatology with an asymptomatic localized eruption on the right posterior shoulder of unknown duration. His medical history was significant for hyperlipidemia. Physical examination revealed a circumscribed erythematous patch on the right posterior shoulder. Biopsies revealed an atypical intradermal nodular and diffuse lymphocytic proliferation consistent with primary cutaneous follicular center lymphoma with diffuse large cell pattern. A comprhehensive evaluation by the department of oncology was negative for systemic involvement. The patient underwent radiation therapy. Six months later, he developed a similar erythematous patch on his left mid back. Physical examination of the left mid back demonstrated a poorly circumscribed erythematous patch. Biopsies revealed cutaneous B-cell lymphoma, follicle center cell type. The patient was referred back to the department of oncology, where he was treated with 3 cycles of rituximab. He is pending followup.

Background: Plasmablastic lymphoma is a rare form of non-Hodgkin lymphoma that most frequently affects the oral cavity of HIV patients. It is recognized by WHO’s classification of lymphoproliferative disorders as a subtype of diffuse large B-cell lymphoma, HIV related and AIDS defining illness. Case report: A 56-year-old man presented with a 1-month history of rapidly growing mass in his tongue. He denied fever, chills, night sweats or weight loss. His medical history was significant for VIH (stage B3). Physical examination revealed a 3 3 4 cm pink-withe, ulcerated, exophytic lesion involving the left half of the dorsal surface of the tongue. Biopsy sample of the lesion showed atypical neoplastic infiltrates of plasmocytoid cells with abundant cytoplasm and prominent nucleoli. Inmunostaining was negative for CD2O, CD3, HHV8, BCL2, BCL6 and positive for CD38, CD138, EBER, MUM1. These finding were consistent with a diagnosis of plasmablastic lymphoma. A bone marrow aspirate and biopsy were done, and these confirmed infiltration of bone marrow (stage IV-A). CT imaging of the brain, neck, chest, abdomen and pelvis didn’t show lymph node involvement. The patient was transferred to the oncology service and treated with EPOCH (etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin) in association with HAART (highly active antiretroviral therapy). The disease progress and the patient was switched to bortezomib with poor clinical response. Discussion: Plasmablastic lymphoma accounts for 2.6% of all HIV-related nonHodgkin lymphomas, it was first described by Delecluse et al as highly malignant Bcell lymphoma with predilection for the oral cavity in advanced immunosuppressed patients, nevertheless extraoral localizations and immunocompetent patients affected had been reported. It has been associated with EpsteineBarr virus in his pathogenesis, the association with HHV-8 is not clear. The histology is characterized by immunoblastic morphology and plasma cell phenotype (negativity for typical Bcell antigens CD20 and positivity for plasma cell markers such as CD38, CD138 and VS38c). The differential diagnosis includes primarily infectious and others malignant processes like squamous cell tumor, metastatic tumor and Kaposi sarcoma. The prognosis is very poor despite chemotherapy and HAART. Dermatologists should be aware of this oral pathology in the diagnostic evaluation of HIV patients with rapidly growing oral mass.

Commercial support: None identified.

Commercial support: None identified.

P6785 Primary cutaneous anaplastic large cell lymphoma: A diagnostic challenge Andreia Almeida, MD, Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeiro, Brazil; Flavia Bonini, MD, Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeiro, Brazil; Flavia Brito, MD, Instituto de Dermatologia professor Rubem David Azulay, Rio de Janeiro, Brazil; Mercedes Pockstaller, MD, Instituto de Dermatologia professor Rubem David Azulay, Rio de Janeiro, Brazil; Patricia Rezende, MD, Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeiro, Brazil; Tullia Cuzzi, MD, Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeiro, Brazil Primary cutaneous CD30 lymphoproliferative disorders (LPDs) are the second most common group of cutaneous T-cell lymphomas (CTCLs), accounting for approximately 30% of CTCLs. This group includes primary cutaneous anaplastic large cell lymphoma (C-ALCL), lymphomatoid papulosis (LyP), and borderline cases. It is now generally accepted that C-ALCL and LyP form a spectrum of disease, and that histologic criteria alone are often insufficient to differentiate between these two ends of this spectrum. The clinical appearance and course are used as decisive criteria for the definite diagnosis and choice of treatment, thus requiring a multidisciplinary approach and expertise to ensure them. A 53-year-old man presented with a history of painless erythematous nodule on the right foot, progressing to increase the number of lesions and anatomical areas involved, in 9 months, such as trunk, thighs, legs and feet. He denied systemic symptoms. On examination, he had erythematous infiltrated plaques and nodules with central ulceration in the right lumbar region, bilateral popliteal fossa and the medial aspect of left foot. The diagnostic hypothesis was skin cancer and incisional biopsy and subsequently immunohistochemistry were necessary for definitive diagnosis. They revealed cutaneous CD30+ T cells, with CD3 e granzima Immunohistochemical expression, and after clinicopathological correlation to differentiate the type C LyP and C-ALCL, we diagnose C-ALCL. In the next moment, we excluded the systemic manifestation of the disease with normal carried out laboratory tests and imaging. The patient was treated with cyclophosphamide, doxorubicin, vincristine and prednisone regimen (CHOP). Currently, after six sessions of chemotherapy the patient is in remission of the disease. The differential diagnosis between PC-ALCL and LyP is important, since patients with LyP have a 10% to 20% increased risk for developing lymphoid malignancy in comparison to PC-ALCL and because the therapeutic strategy among these conditions differs. In C-ALCL, individual lesions are usually [2 cm in diameter and partial or complete spontaneous regression occurs in approximately 25% of patients, different from LyP. The prognosis is usually favorable, but the progression to extracutaneous disease is reportedly and occurs more frequently in patients with multifocal skin involvement. For this reason, systemic chemotherapy has generally been recommended for that subset of patients. Commercial support: None identified.

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J AM ACAD DERMATOL

P6567 Primary cutaneous diffuse large B-cell lymphoma, leg type: Two Bcl-2 negative cases Nicole Yi Zhen Chiang, MBChB, Birmingham, United Kingdom; Aleem Uddin, MBBS, Birmingham, United Kingdom; Anthony Abdullah, MBChB, Birmingham, United Kingdom; Donna Thompson, MBBS, Birmingham, United Kingdom; Parveen Abdullah, Birmingham, United Kingdom; Shireen Velangi, MBChB, Birmingham, United Kingdom Primary cutaneous diffuse large B-cell lymphoma, leg lype (PCLBCL, LT) is a rare cutaneous B-cell lymphoma with a predominance or confluent sheets of centroblasts and immunoblasts. Bcl-2 is strongly expressed in most cases. We report 2 cases of PCLBCL, LT with negative Bcl-2. One of these cases was associated with the use of immunosuppressants. Patient 1, a 76-year-old man with multiple sclerosis and bullous pemphigoid, presented with a 6-week history of asymptomatic lesions on his left leg. He had been taking azathioprine (75 mg twice daily) and prednisolone (7.5 mg daily) for bullous pemphigoid for the last 18 months. Examination showed multiple hard, purplish nodules on his left leg. Skin biopsy showed heavy infiltration of lymphoid cells in the dermis with a mixture of mature lymphoid cells and blasts cells. The cells were stained positive for CD79a, but negative for CD3, CD10, CD23, and Bcl-2. Patient 2, an 85-year-old woman with limited mobility caused by severe osteoarthritis, presented with a small red patch on her left lower leg, which developed into multiple nodules covering the whole of her left lower leg over the course of 4 months. The lesions were occasionally pruritic. Examination showed multiple large, tumid, red-purple, coalescing nodules with some areas of ulceration and yellow crusting. Skin biopsy showed large neoplastic cells infiltration in the dermis constituting mainly of immunoblasts and centroblasts with prominent mitosis and tangible-body macrophages. The cells were stained positive for CD20, CD79a, Bcl-6, CD10 and MUM1, but negative for Bcl-2. Clinical and histologic findings in both cases were diagnostic of PCLBCL, LT. Both patients did not have any extracutaneous dissemination. Patient 1 died shortly after diagnosis with a bronchopneumonia. Patient 2 was commenced on low dose oral chemotherapy (Prednisolone and etoposide) with adjuvant radiotherapy as a palliative treatment. PCLBCL, LT is an aggressive lymphoma. Both our cases were Bcl-2 negative, an uncommon feature in PCLBCL, LT. Prognosis for PCLBCL, LT is generally poor with an overall 5-year survival rate of 41%. PCLBCL, LT has been associated with immunodeficiency before, and immunosuppression may have played a role in inducing PCLBCL, LT in patient 1. Commercial support: None identified.

APRIL 2013