IMAGES
IN THE
MEDICAL SCIENCES
Bilateral Lacrimal Gland Involvement With Mantle Cell Lymphoma Umberto Falcone, MD,* Rita R. Parascandola, BD, Maria G. Ferrara, MD, Antonello Sica, MD, Anna Di Martino, MD and Salvatore Guastafierro, MD *Division of Hematology, Second University of Naples, Naples, Italy (E-mail: [email protected]) The authors have no financial or other conflicts of interest to disclose.
FIGURE 1
A
73-year-old woman presented with a rapidly growing bilateral orbital swelling, causing diplopia and epiphora. During physical examination, there were exophthalmos and orbital edema, both more prominent in the right eye (Figures 1A and 1B). The conjunctiva of both eyes was swollen. The lesions extended to the right iris and partially covered the left one. Computed tomography revealed, in both eyes, a mass (right eye: 2.7 cm 3 2.14 cm; left eye: 1.88 cm 3 1 cm) involving the lacrimal gland and adjacent to the superior rectus and lateral rectus muscles. Histological and immunohistochemical analyses showed a population of small atypical lymphocytes, with slightly irregular indented nuclei and moderately dispersed chromatin. Malignant cells were CD19+, CD20+, CD5+, CD232, CD102, and hyperexpression of cyclin D1 was detected. Cytogenetic analysis demonstrated the presence of a complex karyotype: t(11; 14)(q13; q32), t(7; 10)(q21; p12), dup (11)(q25, q13), and 217. The final diagnosis was mantle cell lymphoma (MCL), stage IV-A. The patient underwent 6 cycles of R-GIFOX (Rituximab-Gemcitabine, Ifosfamide, Oxaliplatin) achieving a complete response. Unfortunately, 18 months later, her disease relapsed (although at a different site), and she died 29 months after the initial diagnosis. Ocular adnexal lymphoma (OAL) accounts for approximately 1% to 2% of all non–Hodkgin’s lymphomas (NHL).1
268
OAL comprises both primary extranodal NHL and secondary involvement in systemic lymphomas. Although mucosa-associated lymphoid tissue represents the most common histology, MCL is observed in only very small percentages. MCL represents between 2% to 10% of all NHLs,2 and it has often an aggressive clinical behavior. The chromosomal translocation t(11; 14)(q13, q32), that juxtaposes the cyclin D1 and the immunoglobulin heavy chain genes, is detected in almost all cases, and it represents the molecular hallmark of MCL. The aim of treatment for younger patients still remains a long-lasting complete remission. In elderly patients treatment still represents a challenge. For fit elderly patients, long progression-free survival and molecular remissions are possible, whereas for the frail patients, the treatment should aim at reducing symptoms and maintaining quality of life (3). REFERENCES 1. Meunier J, Lumbroso-Le Rouic L, Vincent-Salomon A, et al. Ophthalmologic and intraocular non-Hodgkin’s lymphoma: a large single centre study of initial characteristics, natural history, and prognostic factors. Hematol Oncol 2004;22:143–158. 2. Cortelazzo S, Ponzoni M, Ferreri AJ, et al. Mantle cell lymphoma. Crit Rev Oncol Hematol 2012;82:78–101.
The American Journal of the Medical Sciences
Volume 349, Number 3, March 2015
IN THE
MEDICAL SCIENCES
Bilateral Lacrimal Gland Involvement With Mantle Cell Lymphoma Umberto Falcone, MD,* Rita R. Parascandola, BD, Maria G. Ferrara, MD, Antonello Sica, MD, Anna Di Martino, MD and Salvatore Guastafierro, MD *Division of Hematology, Second University of Naples, Naples, Italy (E-mail: [email protected]) The authors have no financial or other conflicts of interest to disclose.
FIGURE 1
A
73-year-old woman presented with a rapidly growing bilateral orbital swelling, causing diplopia and epiphora. During physical examination, there were exophthalmos and orbital edema, both more prominent in the right eye (Figures 1A and 1B). The conjunctiva of both eyes was swollen. The lesions extended to the right iris and partially covered the left one. Computed tomography revealed, in both eyes, a mass (right eye: 2.7 cm 3 2.14 cm; left eye: 1.88 cm 3 1 cm) involving the lacrimal gland and adjacent to the superior rectus and lateral rectus muscles. Histological and immunohistochemical analyses showed a population of small atypical lymphocytes, with slightly irregular indented nuclei and moderately dispersed chromatin. Malignant cells were CD19+, CD20+, CD5+, CD232, CD102, and hyperexpression of cyclin D1 was detected. Cytogenetic analysis demonstrated the presence of a complex karyotype: t(11; 14)(q13; q32), t(7; 10)(q21; p12), dup (11)(q25, q13), and 217. The final diagnosis was mantle cell lymphoma (MCL), stage IV-A. The patient underwent 6 cycles of R-GIFOX (Rituximab-Gemcitabine, Ifosfamide, Oxaliplatin) achieving a complete response. Unfortunately, 18 months later, her disease relapsed (although at a different site), and she died 29 months after the initial diagnosis. Ocular adnexal lymphoma (OAL) accounts for approximately 1% to 2% of all non–Hodkgin’s lymphomas (NHL).1
268
OAL comprises both primary extranodal NHL and secondary involvement in systemic lymphomas. Although mucosa-associated lymphoid tissue represents the most common histology, MCL is observed in only very small percentages. MCL represents between 2% to 10% of all NHLs,2 and it has often an aggressive clinical behavior. The chromosomal translocation t(11; 14)(q13, q32), that juxtaposes the cyclin D1 and the immunoglobulin heavy chain genes, is detected in almost all cases, and it represents the molecular hallmark of MCL. The aim of treatment for younger patients still remains a long-lasting complete remission. In elderly patients treatment still represents a challenge. For fit elderly patients, long progression-free survival and molecular remissions are possible, whereas for the frail patients, the treatment should aim at reducing symptoms and maintaining quality of life (3). REFERENCES 1. Meunier J, Lumbroso-Le Rouic L, Vincent-Salomon A, et al. Ophthalmologic and intraocular non-Hodgkin’s lymphoma: a large single centre study of initial characteristics, natural history, and prognostic factors. Hematol Oncol 2004;22:143–158. 2. Cortelazzo S, Ponzoni M, Ferreri AJ, et al. Mantle cell lymphoma. Crit Rev Oncol Hematol 2012;82:78–101.
The American Journal of the Medical Sciences
Volume 349, Number 3, March 2015
