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Journal of Clinical Oncology, Vol 26, No 3 (January 20), 2008: pp. 501-503 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2007.14.2794
Skeletal Muscle Peripheral T-Cell LymphomaDivision of Hematology & Oncology, H. Lee Moffitt Cancer Center and Research Institute, University of South Florida, Tampa, FL
Department of Pathology and Laboratory Medicine, H. Lee Moffitt Cancer Center & Research Institute, University of South Florida, Tampa, FL
Department of Interdisciplinary Oncology, Division of Blood and Marrow Transplant, H. Lee Moffitt Cancer Center & Research Institute, University of South Florida, Tampa, FL
Department of Interdisciplinary Oncology, Division of Hematopathology & Laboratory Medicine, H. Lee Moffitt Cancer Center & Research Institute, University of South Florida, Tampa, FL
Department of Interdisciplinary Oncology, Division of Malignant Hematology, H. Lee Moffitt Cancer Center & Research Institute, University of South Florida, Tampa, FL A 56-year-old previously healthy male presented for further evaluation of a 7-month history of progressive right ankle swelling. An ultrasound of the affected limb demonstrated thrombophlebitis. Additionally, a mass involving the medial aspect of his right thigh was incidentally detected. Magnetic resonance imaging (MRI) revealed a 5.6-cm heterogeneous mass involving the right sartorius muscle, which was suspicious for a soft tissue sarcoma. Contrasted computed tomography (CT) scans of the thorax, abdomen, and pelvis revealed no evidence of mediastinal lymphadenopathy, lung infiltrates, retroperitoneal lymphadenopathy, or pelvic masses. There were no pathologic abnormalities involving his osseous structures. He underwent complete resection of the mass. The pathology demonstrated tumor infiltrating the muscle as an extensive, vaguely nodular proliferation of atypical lymphocytes ranging in size from small to large cells admixed with clusters of epithelioid histiocytes destroying muscle bundles ( Fig 1, arrows) and creating intervening bands of collagen sclerosis (arrow head). Immunohistochemical staining revealed that the atypical lymphocyte populations were positive for CD3, CD4, CD5, weakly CD57, and negative for CD8, CD20, CD30, and CD56. Cytoplasmic CD3e expression by immunohistochemistry highlights the T-cell lineage of this neoplasm ( Fig 2). The diagnosis was consistent with extranodal peripheral T-cell lymphoma, unspecified (PTCL, u). Bone marrow biopsy revealed a normocellular marrow with trilineage hematopoiesis and no cytologic or morphologic evidence of lymphoma. Flow cytometry revealed no evidence of a clonal lymphoproliferative disorder. Antibodies to human immunodeficiency virus I/II and human T-cell lymphotrophic virus type I/II were negative. Lactate dehydrogenase was normal. Positron emission tomography (PET) imaging with [18F]fluorodeoxyglucose (FDG) scan, however, demonstrated multiple sites of hypermetabolic activity within musculature. A selected PET scan coronal image ( Fig 3) demonstrates increased FDG uptake (arrows) within the abdominal wall musculature, left external oblique muscle just above the left iliac crest, bilateral pelvic musculature, left sartorius musculature extending down throughout the bilateral quadriceps, and hamstring musculature. Confirmatory MRI of the lower extremities detected multiple enhancing foci within the soft tissues of both thighs correlating with the abnormal FDG-PET scan findings. Selected axial T1-weighted MRI images ( Fig 4) demonstrate abnormal masses involving the sartorius muscle of the right leg and the vastus medialis muscle of the left leg (arrows). The patient underwent chemotherapy with six cycles of CHOP (cyclophosphamide, hydroxydaunomyocin, oncovin, prednisone). A complete radiological response was achieved after three cycles. He was further treated with high-dose chemotherapy, conditioned with carmustine, etoposide, cytarabine, and melphalan, followed by an autologous peripheral-blood hematopoietic cell transplantation as consolidation while in first remission. He is without evidence of disease 12 months following autologous stem-cell transplantation.
Primary skeletal muscle non-Hodgkin's lymphoma (PSM NHL) is a rare manifestation of NHL accounting for only 0.1% of all lymphomas.1 An increased frequency of PSM NHL (7%) was detected in patients with AIDS-associated lymphomas.2 A Medline search revealed only 62 well-documented cases of PSM NHL by 2005.3 This condition most commonly affects skeletal muscles of the lower extremities and pelvic region. Additionally, the majority of these lymphomas demonstrates a B-cell immunophenotype and usually behaves in an aggressive biologic manner.4 Mature post-thymic T-cell neoplasms constitute 10% to 12% of all NHLs.5 PTCL,u represents the most common subtype of mature aggressive T-cell lymphomas, manifesting most often as a nodal disease.6 Solely, extranodal PTCL,u is a rare presentation among these neoplasms. Additionally, few cases of primary aggressive mature T-cell lymphomas of skeletal muscle have been described in the literature.7-9 Although larger masses involving PSM NHL usually appear as isodense or hypodense foci with contrasted CT imaging, this imaging modality might fail to detect discrete lymphomatous infiltrates involving muscle.10,11 MRI imaging provides enhanced sensitivity for imaging lymphomas involving skeletal muscle. These neoplasms will appear homogeneous and isointense to muscle on T1-weighted MRI images, and homogeneous and hyperintense on T2-weighted MRI images with diffuse enhancement after administration of contrast.12,13 A recent study suggests that FDG-PET scans are sensitive tests for demonstrating involvement of T-cell and natural killer cell cell lymphomas in all anatomic regions except for skin.14 FDG-PET scans are useful in aiding the diagnosis and staging of lymphomas with almost 100% accuracy in aggressive subtypes, including PTCL,u.15,16 Anthracycline-containing regimens remain the backbone of primary therapy, though optimal therapy has still not been defined. The role for high-dose chemotherapy and stem-cell transplantation has not been established for patients in the first remission; however, it should be considered for treating patients with systemic extranodal subtypes and high international prognostic index scores.6 Our case demonstrates that the higher resolution and sensitivity of MRI and FDG-PET scans, respectively, compared with contrasted CT scans are useful in the detection of disseminated lymphoma in skeletal muscle. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
REFERENCES 1. Travis WD, Banks PM, Reiman HM: Primary extranodal soft tissue lymphoma of the extremities. Am J Surg Pathol 11:359-366, 1987[CrossRef][Medline] 2. Raphael M, Gentilhomme O, Tulliez M, et al: Histopathologic features of high-grade non-Hodgkin's lymphomas in acquired immunodeficiency syndrome: The french study group of pathology for human immunodeficiency virus-associated tumors. Arch Pathol Lab Med 115:15-20, 1991[Medline] 3. Ueno A, Kuribayashi K, Iyama S, et al: Two cases of primary skeletal muscle lymphoma, and a review of the literature. Rinsho Ketsueki 46:1141-1145, 2005[Medline] 4. Keung YK, Liang R: Report of a case of primary skeletal muscle lymphoma and review of the literature. Acta Haematol 96:184-186, 1996[Medline] 5. Ralfkiaer E, Muller-Hermelink HK, Jaffe ES: Mature T-cell and NK-cell Neoplasms, in Jaffe E, Harris N, Stein H, Vardiman J, (eds): Pathology and Genetics of Tumours of the Haematopoietic and Lymphoid Tissues: World Health Organization Classification of Tumours. Lyon, France: IARC Press, 2001, pp 227-229 6. Savage KJ, Chhanabhai M, Gascoyne RD, et al: Characterization of peripheral T-cell lymphomas in a single North American institution by the WHO classification. Ann Oncol 15:1467-1475, 2004 7. Chim CS, Choy C, Liang R: Primary anaplastic large cell lymphoma of skeletal muscle presenting with compartment syndrome. Leuk Lymphoma 33:601-605, 1999[Medline] 8. Chim CS, Loong F, Ooi GC, et al: Primary skeletal muscle lymphoma. Am J Med 112:79-80, 2002[CrossRef][Medline] 9. Lum GH, Cosgriff TM, Byrne R, et al: Primary T-cell lymphoma of muscle in a patient infected with human immunodeficiency virus. Am J Med 95:545-546, 1993[CrossRef][Medline] 10. Grem JL, Neville AJ, Smith SC, et al: Massive skeletal muscle invasion by lymphoma. Arch Intern Med 145:1818-1820, 1985 11. Panicek DM, Lautin JL, Schwartz LH, et al: Non-Hodgkin lymphoma in skeletal muscle manifesting as homogeneous masses with CT attenuation similar to muscle. Skeletal Radiol 26:633-635, 1997[CrossRef][Medline] 12. Eustace S, Winalski CS, McGowen A, et al: Skeletal muscle lymphoma: Observations at MR imaging. Skeletal Radiol 25:425-430, 1996[CrossRef][Medline] 13. Weatherall P: Imaging of muscle tumors. Semin Musculoskelet Radiol 4:435-458, 2000[CrossRef][Medline] 14. Kako S, Isutsu K, Ota Y, et al: FDG-PET in T-cell and NK-cell Neoplasms. Ann Oncol Advance Access 1-6, 2007 15. Gill SI, Gibbs SD, Hicks RJ, et al: Primary skeletal muscle marginal zone lymphoma with persistent tissue tropism and PET-avidity. Leuk Lymphoma 47:117-120, 2006[CrossRef][Medline] 16. Tsukamoto N, Kojima M, Hasegawa M, et al: The usefulness of (18)F-fluorodeoxyglucose positron emission tomography ((18)F-FDG-PET) and a comparison of (18)F-FDG-pet with (67)gallium scintigraphy in the evaluation of lymphoma: Relation to histologic subtypes based on the World Health Organization classification. Cancer 110:652-659, 2007[CrossRef][Medline]
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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