Trisal et al adjuvant abdominopelvic radiation with or without myeloablative chemotherapy with stem cell rescue have been reported to improve survival in DSRCT, although cure remains elusive in widespread disease.9 More effective therapeutic options including targeted therapies focusing on cellular regulatory mechanisms and pathways for this tumor need to be explored to improve outcome and reduce treatment related toxicity. In this case, contemporary curative treatment has been offered and in view of the localized disease a favorable outcome is expected. Sajid S. Qureshi Pediatric Surgical Oncology Services, Tata Memorial Centre, Bombay, India Mukta R. Ramadwar and Seethalakshmi Viswanathan Department of Pathology, Tata Memorial Centre, Bombay, India Ashish V. Bakshi and Brijesh Arora Department of Pediatric Oncology, Tata Memorial Centre, Bombay, India Tejpal Gupta and Siddharth Laskar Department of Radiation Oncology, Tata Memorial Centre, Bombay, India Seema S. Medhi Department of Radiodiagnosis, Tata Memorial Centre, Bombay, India Mary A. Muckaden Department of Radiation Oncology, Tata Memorial Centre, Bombay, India Shripad D. Banavali and Suresh K. Pai Department of Pediatric Oncology, Tata Memorial Centre, Bombay, India Sangeeta B. Desai Purna A. Kurkure Department of Pediatric Oncology, Tata Memorial Centre, Bombay, India © 2007 by American Society of Clinical Oncology AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest. REFERENCES 1. Gerald WL, Rosai J: Case 2: desmoplastic small round cell tumor with divergent differentiation. Pediatr Pathol 9:177-183, 1989 2. Gerald WL, Miller HK, Battifora H, et al: Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals. Am J Surg Pathol 15:499-513, 1991 3. Biswas G, Laskar S, Banavali SD, et al: Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment. Indian J Cancer 42:78-84, 2005 4. Chang F: Desmoplastic small round cell tumors: Cytologic, histologic, and immunohistochemical features. Arch Pathol Lab Med 130:728-732, 2006 5. Rodriguez E, Sreekantaiah C, Gerald W, et al: A recurring translocation, t(11;22)(p13;q11.2), characterizes intra-abdominal desmoplastic small round-cell tumors. Cancer Genet Cytogenet 69:17-21, 1993 6. Lee SB, Kolquist KA, Nichols K, et al: The EWS-WT1 translocation product induces PDGFA in desmoplastic small round-cell tumour. Nat Genet 17:309-313, 1997 7. Ito E, Honma R, Imai J, et al: A tetraspanin-family protein, T-cell acute lymphoblastic leukemia-associated antigen 1, is induced by the Ewing’s sarcomaWilms’ tumor 1 fusion protein of desmoplastic small roundcell tumor. Am J Pathol 163:2165-2172, 2003 8. Wong JC, Lee SB, Bell MD, et al: Induction of the interleukin-2/15 receptor beta-chain by the EWS-WT1 translocation product. Oncogene 21:2009-2019, 2002 9. Lal DR, Su WT, Wolden SL, et al: Results of multimodal treatment for desmoplastic round cell tumors. J Pediatr Surg 40:251-255, 2005 DOI: 10.1200/JCO.2007.11.9487 Department of Pathology, Tata Memorial Centre, Bombay, India ■ ■ ■ Thymoma-Associated Severe Aplastic Anemia Treated With Surgical Resection Followed by Allogeneic Stem-Cell Transplantation A 44-year-old man presented with chronic cough not resolving on antibiotic treatment, and was found to have a large anterior mediastinal mass on chest roentgenogram. Computed tomography demonstrated a 15 cm ⫻ 10 cm ⫻ 13 cm mediastinal mass beginning at the level of the innominate veins extending to the right anterolateral chest wall with displacement of the mediastinum and some compression of the superior vena cava (Fig 1). The spleen was slightly enlarged, and there was no lymphadenopathy. WBC count was 10,600/cc, hemoglobin 11.8 mg/dl, and platelet count 5,000 ⫻ 109/L. Differential count showed 15% polymorphonuclear leukocytes, 84% lymphocytes. Lactate dehydrogenase was within normal limits. Phenotype of peripheral blood demonstrated 96% lymphocytes and T cells, with a predominance of CD8-positive cells. Flow study demonstrated 98% CD2 positive, 96% CD3, 97% CD5, 98% CD7, 84% CD8, CD10 was negative as were CD19, CD20, CD23, FMC-7, and kappa lambda light chain. Both immunoglobulin G and immunoglobulin M antibodies against platelets were identified. Alpha-fetoprotein and carcinoembryonic agent were in normal range. Serum protein electrophoresis showed no evidence of monoclonal gammopathy. Coombs and HIV tests were negative. Bone marrow aspiration and biopsy demonstrated a hypocellular marrow (20%) with marked reduction in trilineage hematopoiesis. There was lym3374 phocytosis, predominantly of T lymphocytes. A diagnosis of idiopathic thrombocytopenic purpura was made, and the patient was placed on prednisone at 80 mg per day, and platelet count improved to 41,000 within 48 hours. After platelet count dropped again, the patient was treated with rituximab at 375 mg/m2. At this time, he was transferred to the City of Hope National Medical Center (Duarte, CA), where bone marrow aspiration and biopsy demonstrated severe hypocellular marrow (⬍ 5%) with marked reduction in hematopoiesis, consistent with severe aplastic anemia (severe aplastic anemia [SAA]; Fig 2). No evidence of lymphoma or leukemia was found. The patient was placed on cyclosporine. A thoracoscopic-assisted anterior mediastinotomy and biopsy of the mediastinal mass was consistent with a thymoma. At this time, it was thought that due to the autoimmune mediated pancytopenia the patient might have considerable difficulty tolerating chemotherapy. We elected to perform a thymectomy in an attempt to ablate the source of antibodies thereby improving the chances of recovery from bone marrow aplasia. On May 12, 2004, a right posterolateral thoracotomy was performed and the thymic mass resected en bloc along with part of the pericardium. The thymic tumor weighed 731 g and measured 15.0 cm in greatest dimension. The histology was consistent with a type A thymoma (WHO classification). Postoperatively the patient was continued on cyclosporine, granulocyte colony-stimulating factor, and platelet transfusions to maintain platelet count above 60,000/mm3. One month after surgery, a repeat bone marrow aspiration and biopsy showed persistent SAA. The patient underwent allogenic sibling JOURNAL OF CLINICAL ONCOLOGY Downloaded from jco.ascopubs.org on September 9, 2014. For personal use only. No other uses without permission. Copyright © 2007 American Society of Clinical Oncology. All rights reserved. Diagnosis in Oncology Fig 1. peripheral stem-cell transplantation for treatment of AA. He tolerated the transplantationwellwithoutanymajorcomplicationsandwasdischarged from the hospital 1 month later. A repeat bone marrow examination 1 year later demonstrated a normocellular marrow with active trilineage hematopoeisis. Thirty-two months after the completion of therapy, there was no evidence of recurrent thymoma. He had excellent graft function with mild graft-versus-host disease of the mucosa and liver. He has been maintained on immunosuppressive therapy with cyclosporine and mycophenolate mofetil. Myasthenia gravis is a common complication of thymoma caused by immune damage to the motor end plate. Pure red cell aplasia, often in combination with idiopathic thrombocytic purpura has been reported much less commonly in association with thymomas, and aplastic anemia has been reported after thymectomy.1,2 To the best of our knowledge, there are no published reports of patients with aplastic anemia resulting from thymoma who have undergone successful bone marrow transplantation after resection of the thymoma. The challenges of combining these treatments in this case arose from difficulties in treating the aplastic anemia with the thymoma in place versus the challenges of resecting the thymoma in the setting of severe pancytopenia. Cyclosporine has been shown to produce a good response in patients with red cell aplasia. While SAA can respond to cyclosporine alone, it is less effective than cyclosporine plus antithymocyte globulin.3,4 Approximately 60% of patients with SAA respond to combination of antithymocyte globulin plus cyclosporine; however, the response may not be durable. Allogeneic bone marrow transplantation remains the curative treatment for SAA.4 A recent report also showed that patients with agenerative anemia who have undergone thymectomy have not had a durable response.5 One of three such patients who underwent the operation has had complete remission for 2 years. Our patient did not respond to cyclosporine; however, thymectomy and allogeneic stem-cell transplantation resulted in normalization of his blood count and bone marrow function. Fifty percent of all patients with red cell aplasia will have a thymoma. The incidence of SAA is unknown. Twenty-five to 30% of those who undergo thymectomy will be cured of the RCA. It has been suggested that any patient with red cell aplasia should have thymectomy through a median sternotomy.6 We approached the thymoma via posterolateral thoracotomy to have a better visualization of the phrenic nerve and to improve hemostasis in the face of severely suppressed platelet counts. Vijay Trisal, Auayporn Nademanee, Sean K. Lau, and Frederic W. Grannis Jr From the Departments of Surgical Oncology, Hematology, Pathology, and Thoracic Surgery, City of Hope National Medical Center, Duarte, CA © 2007 by American Society of Clinical Oncology AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest. REFERENCES 1. Ritchie DS, Underhill C, Grigg AP: Aplastic anemia as a late complication of thymoma in remission. Eur J Hematol 68:389-391, 2002 2. Kobayashi H, Kitano K, Furuta S, et al: Aplastic anemia and idiopathic thrombocytopenic purpura with antibody to platelet glycoprotein IIb/IIIa following Resection of malignant thymoma. Acta hematol 90:42-45, 1993 3. Liozon E, Touati M, Bordessoule D, et al: Thymoma associated pancytopenia: Effectiveness of cyclosporine A. Ann Hematol 77:175-178, 1998 4. Neal S, Young S, Scheinberg P, et al: Current concepts in the pathophysiology and treatment of aplastic anemia. Blood 108:2509-2519, 2006 5. Marks P, Marks C: Thymoma and aregenerative anemia. South Med J 81:1182-1184, 1988 6. Zeok JV, Todd EP, Utley JR, et al: The role of thymectomy in red cell aplasia. Ann Thorac Surg 28:257-260 Fig 2. DOI: 10.1200/JCO.2007.11.6871 ■ ■ ■ 3375 www.jco.org Downloaded from jco.ascopubs.org on September 9, 2014. For personal use only. No other uses without permission. Copyright © 2007 American Society of Clinical Oncology. All rights reserved.
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