Journal of Clinical Oncology, Vol 21, Issue 15
(August), 2003: 2993-2995
© 2003 American Society for Clinical Oncology
Uncommon Presentations of Malignancies
CASE 1. EXTRAOCULAR MUSCLE PALSIES IN SUBCUTANEOUS PANNICULITIS-LIKE T-CELL LYMPHOMA
Gregory D. Leonard,
Upendra Hegde,
John Butman,
Elaine S. Jaffe,
Wyndham H. Wilson
Medical Oncology Clinical Research Unit, National Cancer Institute, Bethesda, MD
A 27-year-old male reported the gradual onset of fever, malaise and diplopia. Physical examination revealed proptosis with bilateral abducens and superior and inferior oblique muscle palsies. A rash on the right calf was noted and biopsied. An atypical lymphocytic infiltration of the subcutaneous tissue was observed with karyohexis and histiocytic cells (H) showing active phagocytic activity (Fig 1 ). Immunophenotyping demonstrated the atypical lymphoid cells to have a cytotoxic T-cell phenotype (CD3+, CD2+, T-cell intracellular antigen 1+, and ßF1+) staining intensely positive for the CD8 lymphocyte marker, consistent with a subcutaneous panniculitis-like T-cell lymphoma (Fig 2). Short tau inversion recovery MRI documented hyperintensity and enlargement of the extraocular muscles and infiltration of the retro-orbital fatty tissues (Fig 3). These findings represent edema or tumor infiltration as indicated by comparison with the normal hypodense signal characteristics of these structures in a normal patient (Fig 4). Before therapy, the rash extended to involve all of his body (Fig 5). The hemophagocytic syndrome also developed; it consists of histiocytic proliferation and activation within the reticuloendothelial system associated with phagocytosis of blood elements, fever, hepatosplenomegaly, and coagulopathy. He was treated with two cycles of etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin and one cycle of ifosfamide, etoposide and cytarabine chemotherapy, but the responses were temporary. He then underwent a sibling-matched allogeneic transplantation using total-body irradiation as induction therapy, but he died on day 30 from multifactorial causes. Autopsy did not reveal any evidence of residual lymphoma.
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Fig 1. Subcutaneous tissue was observed with karyohexis and histiocytic cells showing active phagocytic activity.
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Fig 2. Immunophenotyping of atypical lymphoid cells with a cytotoxic T-cell phenotype (CD3+, CD2+, T-cell intracellular antigen 1+, and ßF1+) staining intensely positive for the CD8 lymphocyte marker, consistent with a subcutaneous panniculitis-like T-cell lymphoma.
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Fig 3. Short tan invariation recovery MRI documenting hyperintensity and enlargement of the extraocular muscles and infiltration of the retro-orbital fatty tissues.
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Fig 4. Normal hypodense signal characteristics of the extraocular muscles and retro-orbital fatty tissues in a normal patient.
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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare disease first described in 1991.1 Previously described conditions such as malignant histiocytosis and histiocytic cytophagic panniculitis are likely to have included many cases of SPTCL but were misdiagnosed before the routine use of immunohistochemistry.2 The differential diagnosis also includes anaplastic large-cell lymphomas and T-cell and natural-killer-cell lymphomas. It is defined as a separate entity by the WHO lymphoma classification3 and is characterized by rimming of fat cells by neoplastic T-lymphocytes as shown in Figure 1 . It rarely extends into the dermis. Nodal and visceral disease is also rare. It may present in an indolent manner but more commonly is aggressive, especially in the presence of the hemophagocytic syndrome.4 Complete responses, as with our case, can beobserved but are rarely sustained with the aggressive subtype.5 No optimal therapy has been defined for this disease. Autologous peripheral-blood stem-cell transplantation has been attempted in a number of patients,6 but this is the first recorded case of allogeneic transplantation.
REFERENCES
1. Gonzalez CL, Medeiros LJ, Braziel RM, et al: T-cell lymphoma involving subcutaneous tissue. Am J Surg Pathol 15:17–27, 1991[Medline]
2. Pernicario C, Zalla MJ, White JW, et al: Subcutaneous T-cell lymphoma: report of two additional cases and further observations. Arch Dermatol 129:1171–1176, 1993[Abstract/Free Full Text]
3. Harris NL, Jaffe ES, Diebold J, et al: The World Health Organization classification of neoplasms of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting—Airle House, Virginia, November, 1997. Hematol J 1:53–66, 2000[CrossRef][Medline]
4. Weenig RH, Ng CS, Perniciaro C: Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as a lipomembranous panniculitis and a review of 72 cases in the literature. Am J Dermatopathol 23:206–215, 2001[CrossRef][Medline]
5. Salhany KE, Macon WR, Choi JK, et al: Subcutaneous panniculitis-like T-cell lymphoma. Am J Surg Pathol 22:881–893, 1998[CrossRef][Medline]
6. Hashimoto H, Sawada K, Koizumi K, et al: Effective CD34+ selected autologous peripheral blood stem cell transplantation in a patient with subcutaneous panniculitic T cell lymphoma (SPTCL) transformed into leukemia. Bone Marrow Transplant 24:1369–1371, 1999[CrossRef][Medline]
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