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Prognostic factors, staging and treatments in advanced stage mycosis fungoides and Sézary syndrome
848
606
Pityriasis lichenoideselike mycosis fungoides: Clinicopathologic, immunophenotypic, and molecular features Min Soo Jang, PhD, Kosin University College of Medicine, Busan, South Korea; Joon Hee Kim, MD, Kosin University College of Medicine, Busan, South Korea; Kang Hoon Lee, MD, Kosin University College of Medicine, Busan, South Korea; Sang Hwa Han, MD, Kosin University College of Medicine, Busan, South Korea; Jong Bin Park, MD, Kosin University College of Medicine, Busan, South Korea; Sang Tae Kim, PhD, Kosin University College of Medicine, Busan, South Korea; Kee Suck Suh, PhD, Kosin University College of Medicine, Busan, South Korea
Prognostic factors, staging and treatments in advanced stage mycosis fungoides and S ezary syndrome Rakhshandra Talpur, MD, UT MD Anderson Cancer Center, Houston, TX, United States; Silvia Alberti-Violetti, MD, UT MD Anderson Cancer Center, Houston TX, United States; Megan Schlichte, UT MD Anderson Cancer Center, Houston, TX, United States; Dawen Sui, UT MD Anderson Cancer Center, Houston, TX, United States; Madeleine Duvic, MD, UT MD Anderson Cancer Center, Houston, TX, United States Introduction: Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of non-Hodgkin lymphomas characterized by skin infiltration of neoplastic T lymphocytes. Mycosis fungoides (MF), and its leukemic variant Sezary syndrome (SS). Objectives: To evaluate prognostic factors including risk of disease progression from initial presentation and treatment in a single center cohort of advanced MF/SS patients evaluated from 2007 to the present. Methods: The prognostic variables effecting overall survival (OS) were examined in a prospectively collected cohort of 168 advanced stage MF and SS patients evaluated at MD Anderson Cancer Center. Results: Advanced patients included 97 MF (58%) and 71 SS (42%) with 106 (63%) males and 62 (37%) females. The median survival was 2.47 years (95% CI: 1.85 to 3.30). The range of the OS time was 7.21 years. The 1, 2, 5 and 7 year survival rate was 76.9%, 57.2%, 30.9%, and 13.3%. There were 79 (47%) deaths from any cause and 89 (53%) were alive at the end of analysis. Median OS for MF was 2.47 yrs and for SS was 2.19 yrs. The median OS for patients with large cell transformation (LCT) was 2.19 yrs, similar to OS of SS patients. Median overall survival of patients [65 years was 1.73 years compared to 4.36 years in patients \65 yrs of age (P ¼ .0005). In patients with nodal disease, the median OS for N3 was 1.09 yrs, N2; 0.79 yrs compared to 2.90 yrs in Nx patients (P # .0001). DSS for MF was 2.85 yrs; SS was 3.15 yrs. Most patients died from disease: stage IVB 47%, IIB 38% vs stage III only 9%. In patients with various levels of blood involvement, median OS with B2 ([1000 SS) was 2.19 yrs and B1 (\1000 and [250) was 2.56 yrs. In the median OS and DSS for patients with a positive clone in the blood was 1.09 and 1.55 yrs respectively. In patients without a clone in blood, median OS and DSS elevated LDH levels were clinically significant P ¼.04 with median OS 2.47 yrs. Expression of CD30 or CD25 was not significant for any of the two survival outcomes. Of 168 patients, 115 were treated with systemic therapies: including extracorporeal photopheresis (ECP), retinoids, interferons, targeted monoclonal antibodies, chemotherapy, total body skin electron beam, and stem cell transplant. The overall response rate (ORR) for all systemic therapies was 46% (n ¼ 52/115) with a complete response (CR) of 9% (n ¼ 10), partial response (PR) was 37% (n ¼ 42) and 38% (n ¼ 44/115) had progressive disease (PD). Conclusions: By univariate analysis, risk factors associated with disease progression or death included advanced age, lymph node involvement, and elevated LDH.
Background: Pityriasis lichenoideselike mycosis fungoides (PL-like MF) is a rare variant of mycosis fungoides, characterized clinically as having an PL-like skin eruption, and histologically as having mycosis fungoides. It is relatively unknown compared to other variants, and only few cases have been reported. Objectives: To investigate clinicopathologic, immunophenotypic, and molecular features of PL-like MF. Methods: This study was conducted on 13 patients with a PL-like lesion (4.6%) among 280 patients diagnosed with MF in the authors’ hospital. The patients underwent clinical, histopathologic, and molecular examinations to determine the characteristics of PL-like MF. Results: The patients’ ages ranged widely from 4 to 59 years. The mean age was 18 years including 10 patients aged 20 years or lower. They all had early-stage MF, of which the IB and IA types were observed in 11 patients and 2 patients, respectively. From a histologic perspective, 12 of 13 patients had a CD4/CD8 ratio \1. PCR detection of TCR gamma gene rearrangement was conducted in 10 patients and showed monoclonality in 7 patients. Eleven of 13 patients were treated with either PUVA (3 patients) or NBUVB (8 patients), and all of them showed complete responses. Conclusion: PL-like MF is a rare variant of MF, and seems to have favorable prognoses. In the case of a PL-like skin eruption, MF could be suspected. Thus, a biopsy is required to confirm PL-like MF. Commercial support: None identified.
Commercial support: None identified.
1958
Discussion: Primary cutaneous lymphomas (PCL) are defined as clonal proliferations of lymphocytes in the skin and can be classified according to the predominate tumor cell population; namely cutaneous T-cell lymphomas, cutaneous B-cell lymphomas and cutaneous lymphomas from natural killer or plasmacytoid dendritic cells. Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a type of nonHodgkin T-cell lymphoma that has no clinical sign of extracutaneous involvement at the time of diagnosis. This condition predominately affects adults with a male to female ratio of 2:1. Typically C-ALCL presents as a solitary or localized nodule(s) or tumor(s), often with a central ulceration. Some case reports state that the tumors may spontaneously heal; however, most lesions do not regress and often exhibit a high rate of cutaneous recurrences. Roughly 10% of patients will have extracutaneous involvement at the time of diagnosis, usually due to involvement of regional lymph nodes. Prognosis of C-ALCL for patients with limited disease is favorable with an estimated 5-year survival of approximately 95%.
Progression of primary cutaneous anaplastic CD30 positive lymphoma into systemic anaplastic lymphoma Hari Reddy, MBBS, University Hospital of North Durham, Durham, United Kingdom; Maneesha Vatve, MBBS, MD, University Hospital of North Durham, Durham, United Kingdom We present a case of primary cutaneous anaplastic large-cell lymphoma with extracutaneous dissemination manifesting as lymphadenopathy on either sides of diaphragm and also progression to systemic anaplastic lymphoma. A 68-year-old gentleman presented in Sep 2013 with a 6 months history of a lesion on the left medial malleolus which has been increasing in size and bleeding for the last 2 wks. The patient has a past history of raised cholesterol. Medication included omeprazole, aspirin, simvastatin and ibuprofen. Physical examination showed 5 3 4 cm plaque with ulceration, crusting and nodular element at superior end. Some pigment network at the inferior medial end. Routine hematology and biochemistry were normal. Blood film eleft shifted neutrophils, myelocytes. Polychromatic RBC.LDH -488. CT scan of the chest, abdomen and pelvis was unremarkable. Histopathologic examination of an incisional biopsy showed epidermis with mild psoriasiform hyperplasia and compact parakeratosis, small numbers of small lymphocytes without basal alignment, clustering or significant atypia. Within the dermis, extending from the papillary dermis to superficial subcutis, there was a prominent infiltrate of medium sized and large atypical lymphoid cells with prominent nucleoli, including small numbers of pleomorphic multinucleate forms. Immunohistochemical staining showed the neoplastic cells to be T-cells expressing CD2, CD4, MUM1 and CD30.The Ki-67 proliferation fraction of the neoplastic cells was high (approximately 70%). A diagnosis of primary cutaneous anaplastic CD30 positive lymphoma was made. Patient was given 5 cycles of CHOP chemotherapy but he developed new cutaneous lesions on left leg. PET CT shows evidence of new cutaneous lesions left leg as well as progression to systemic anaplastic lymphoma with stage IV disease. Commenced treatment with brentuximab vedotin and is responding well. Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma characterized by solitary or localized nodules or plaques, with or without superficial ulceration. Extra cutaneous dissemination is observed in approximately 10 percent of cases and usually manifests as regional lymphadenopathy. Approximately 25% of patients show partial or complete spontaneous regression. Primary cutaneous ALCL has a favorable prognosis, with a five-year disease specific survival of 95 percent.
Commercial support: None identified.
Commercial support: None identified.
1087 Primary cutaneous anaplastic large-cell lymphoma: A case report Kristen Whitney, DO, Saint Joseph Mercy Hospital, Ypsilanti, MI, United States; David Altman, MD, Midwest Center for Dermatology, Warren, MI, United States History: A 70-year-old Caucasian male with an unremarkable medical history presented with a nine-month history of a rapidly growing mass on the left maxilla. Physical examination of the left maxilla revealed a well-demarcated erythematous 4 3 4 cm tumor with a pearly border and central ulceration, extending from the superior border of the upper lip to the zygomatic arch. Histopathology: Three punch biopsies at the peripheral margin of the tumor demonstrated a poorly differentiated malignant neoplasm with large pleomorphic cells, enlarged hyperchromatic nuclei and prominent nucleoli. Abundant mitoses and regions of necrosis were seen. Immunohistochemical stains were strongly positive for CD30 and negative for ALK.
AB162
J AM ACAD DERMATOL
MAY 2015
606
Pityriasis lichenoideselike mycosis fungoides: Clinicopathologic, immunophenotypic, and molecular features Min Soo Jang, PhD, Kosin University College of Medicine, Busan, South Korea; Joon Hee Kim, MD, Kosin University College of Medicine, Busan, South Korea; Kang Hoon Lee, MD, Kosin University College of Medicine, Busan, South Korea; Sang Hwa Han, MD, Kosin University College of Medicine, Busan, South Korea; Jong Bin Park, MD, Kosin University College of Medicine, Busan, South Korea; Sang Tae Kim, PhD, Kosin University College of Medicine, Busan, South Korea; Kee Suck Suh, PhD, Kosin University College of Medicine, Busan, South Korea
Prognostic factors, staging and treatments in advanced stage mycosis fungoides and S ezary syndrome Rakhshandra Talpur, MD, UT MD Anderson Cancer Center, Houston, TX, United States; Silvia Alberti-Violetti, MD, UT MD Anderson Cancer Center, Houston TX, United States; Megan Schlichte, UT MD Anderson Cancer Center, Houston, TX, United States; Dawen Sui, UT MD Anderson Cancer Center, Houston, TX, United States; Madeleine Duvic, MD, UT MD Anderson Cancer Center, Houston, TX, United States Introduction: Cutaneous T-cell lymphomas (CTCL) are a heterogeneous group of non-Hodgkin lymphomas characterized by skin infiltration of neoplastic T lymphocytes. Mycosis fungoides (MF), and its leukemic variant Sezary syndrome (SS). Objectives: To evaluate prognostic factors including risk of disease progression from initial presentation and treatment in a single center cohort of advanced MF/SS patients evaluated from 2007 to the present. Methods: The prognostic variables effecting overall survival (OS) were examined in a prospectively collected cohort of 168 advanced stage MF and SS patients evaluated at MD Anderson Cancer Center. Results: Advanced patients included 97 MF (58%) and 71 SS (42%) with 106 (63%) males and 62 (37%) females. The median survival was 2.47 years (95% CI: 1.85 to 3.30). The range of the OS time was 7.21 years. The 1, 2, 5 and 7 year survival rate was 76.9%, 57.2%, 30.9%, and 13.3%. There were 79 (47%) deaths from any cause and 89 (53%) were alive at the end of analysis. Median OS for MF was 2.47 yrs and for SS was 2.19 yrs. The median OS for patients with large cell transformation (LCT) was 2.19 yrs, similar to OS of SS patients. Median overall survival of patients [65 years was 1.73 years compared to 4.36 years in patients \65 yrs of age (P ¼ .0005). In patients with nodal disease, the median OS for N3 was 1.09 yrs, N2; 0.79 yrs compared to 2.90 yrs in Nx patients (P # .0001). DSS for MF was 2.85 yrs; SS was 3.15 yrs. Most patients died from disease: stage IVB 47%, IIB 38% vs stage III only 9%. In patients with various levels of blood involvement, median OS with B2 ([1000 SS) was 2.19 yrs and B1 (\1000 and [250) was 2.56 yrs. In the median OS and DSS for patients with a positive clone in the blood was 1.09 and 1.55 yrs respectively. In patients without a clone in blood, median OS and DSS elevated LDH levels were clinically significant P ¼.04 with median OS 2.47 yrs. Expression of CD30 or CD25 was not significant for any of the two survival outcomes. Of 168 patients, 115 were treated with systemic therapies: including extracorporeal photopheresis (ECP), retinoids, interferons, targeted monoclonal antibodies, chemotherapy, total body skin electron beam, and stem cell transplant. The overall response rate (ORR) for all systemic therapies was 46% (n ¼ 52/115) with a complete response (CR) of 9% (n ¼ 10), partial response (PR) was 37% (n ¼ 42) and 38% (n ¼ 44/115) had progressive disease (PD). Conclusions: By univariate analysis, risk factors associated with disease progression or death included advanced age, lymph node involvement, and elevated LDH.
Background: Pityriasis lichenoideselike mycosis fungoides (PL-like MF) is a rare variant of mycosis fungoides, characterized clinically as having an PL-like skin eruption, and histologically as having mycosis fungoides. It is relatively unknown compared to other variants, and only few cases have been reported. Objectives: To investigate clinicopathologic, immunophenotypic, and molecular features of PL-like MF. Methods: This study was conducted on 13 patients with a PL-like lesion (4.6%) among 280 patients diagnosed with MF in the authors’ hospital. The patients underwent clinical, histopathologic, and molecular examinations to determine the characteristics of PL-like MF. Results: The patients’ ages ranged widely from 4 to 59 years. The mean age was 18 years including 10 patients aged 20 years or lower. They all had early-stage MF, of which the IB and IA types were observed in 11 patients and 2 patients, respectively. From a histologic perspective, 12 of 13 patients had a CD4/CD8 ratio \1. PCR detection of TCR gamma gene rearrangement was conducted in 10 patients and showed monoclonality in 7 patients. Eleven of 13 patients were treated with either PUVA (3 patients) or NBUVB (8 patients), and all of them showed complete responses. Conclusion: PL-like MF is a rare variant of MF, and seems to have favorable prognoses. In the case of a PL-like skin eruption, MF could be suspected. Thus, a biopsy is required to confirm PL-like MF. Commercial support: None identified.
Commercial support: None identified.
1958
Discussion: Primary cutaneous lymphomas (PCL) are defined as clonal proliferations of lymphocytes in the skin and can be classified according to the predominate tumor cell population; namely cutaneous T-cell lymphomas, cutaneous B-cell lymphomas and cutaneous lymphomas from natural killer or plasmacytoid dendritic cells. Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a type of nonHodgkin T-cell lymphoma that has no clinical sign of extracutaneous involvement at the time of diagnosis. This condition predominately affects adults with a male to female ratio of 2:1. Typically C-ALCL presents as a solitary or localized nodule(s) or tumor(s), often with a central ulceration. Some case reports state that the tumors may spontaneously heal; however, most lesions do not regress and often exhibit a high rate of cutaneous recurrences. Roughly 10% of patients will have extracutaneous involvement at the time of diagnosis, usually due to involvement of regional lymph nodes. Prognosis of C-ALCL for patients with limited disease is favorable with an estimated 5-year survival of approximately 95%.
Progression of primary cutaneous anaplastic CD30 positive lymphoma into systemic anaplastic lymphoma Hari Reddy, MBBS, University Hospital of North Durham, Durham, United Kingdom; Maneesha Vatve, MBBS, MD, University Hospital of North Durham, Durham, United Kingdom We present a case of primary cutaneous anaplastic large-cell lymphoma with extracutaneous dissemination manifesting as lymphadenopathy on either sides of diaphragm and also progression to systemic anaplastic lymphoma. A 68-year-old gentleman presented in Sep 2013 with a 6 months history of a lesion on the left medial malleolus which has been increasing in size and bleeding for the last 2 wks. The patient has a past history of raised cholesterol. Medication included omeprazole, aspirin, simvastatin and ibuprofen. Physical examination showed 5 3 4 cm plaque with ulceration, crusting and nodular element at superior end. Some pigment network at the inferior medial end. Routine hematology and biochemistry were normal. Blood film eleft shifted neutrophils, myelocytes. Polychromatic RBC.LDH -488. CT scan of the chest, abdomen and pelvis was unremarkable. Histopathologic examination of an incisional biopsy showed epidermis with mild psoriasiform hyperplasia and compact parakeratosis, small numbers of small lymphocytes without basal alignment, clustering or significant atypia. Within the dermis, extending from the papillary dermis to superficial subcutis, there was a prominent infiltrate of medium sized and large atypical lymphoid cells with prominent nucleoli, including small numbers of pleomorphic multinucleate forms. Immunohistochemical staining showed the neoplastic cells to be T-cells expressing CD2, CD4, MUM1 and CD30.The Ki-67 proliferation fraction of the neoplastic cells was high (approximately 70%). A diagnosis of primary cutaneous anaplastic CD30 positive lymphoma was made. Patient was given 5 cycles of CHOP chemotherapy but he developed new cutaneous lesions on left leg. PET CT shows evidence of new cutaneous lesions left leg as well as progression to systemic anaplastic lymphoma with stage IV disease. Commenced treatment with brentuximab vedotin and is responding well. Primary cutaneous anaplastic large-cell lymphoma (ALCL) is a form of cutaneous T-cell lymphoma characterized by solitary or localized nodules or plaques, with or without superficial ulceration. Extra cutaneous dissemination is observed in approximately 10 percent of cases and usually manifests as regional lymphadenopathy. Approximately 25% of patients show partial or complete spontaneous regression. Primary cutaneous ALCL has a favorable prognosis, with a five-year disease specific survival of 95 percent.
Commercial support: None identified.
Commercial support: None identified.
1087 Primary cutaneous anaplastic large-cell lymphoma: A case report Kristen Whitney, DO, Saint Joseph Mercy Hospital, Ypsilanti, MI, United States; David Altman, MD, Midwest Center for Dermatology, Warren, MI, United States History: A 70-year-old Caucasian male with an unremarkable medical history presented with a nine-month history of a rapidly growing mass on the left maxilla. Physical examination of the left maxilla revealed a well-demarcated erythematous 4 3 4 cm tumor with a pearly border and central ulceration, extending from the superior border of the upper lip to the zygomatic arch. Histopathology: Three punch biopsies at the peripheral margin of the tumor demonstrated a poorly differentiated malignant neoplasm with large pleomorphic cells, enlarged hyperchromatic nuclei and prominent nucleoli. Abundant mitoses and regions of necrosis were seen. Immunohistochemical stains were strongly positive for CD30 and negative for ALK.
AB162
J AM ACAD DERMATOL
MAY 2015