Skeletal Radiol DOI 10.1007/s00256-014-1879-5

CASE REPORT

Subcutaneous panniculitis-like T-cell lymphoma: MRI features and literature review Benjamin D. Levine & Leanne L. Seeger & Aaron W. James & Kambiz Motamedi

Received: 16 September 2013 / Revised: 18 March 2014 / Accepted: 20 March 2014 # ISS 2014

Abstract Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) represents a rare subclassification of peripheral Tcell lymphoma (PTCL). We present a case of a 21-year-old female who presented with a 1-month history of pain in the left buttock and hip, tender left inguinal lymph nodes, fevers, and night sweats. Percutaneous core needle biopsy was diagnostic for SPTCL with CD8+ cells positive for cytotoxic granules. Magnetic resonance imaging (MRI) features of SPTCL with a review of the literature are discussed. Keywords T-cell lymphoma . Panniculitis . Subcutaneous panniculitis-like T-cell lymphoma . MRI . Imaging

Introduction According to the World Health Organization (WHO) classification, lymphoid malignancies are divided into those related to T-cells, B-cells, and Hodgkin disease [1]. T-cell neoplasms are further divided into precursor T-cell neoplasms and peripheral (mature) T-cell neoplasms. Although 20–30 % of all non-Hodgkin’s lymphoma is extranodal, primary involvement of the musculoskeletal system is relatively rare [2]. With regard to the skin and subcutaneous tissues, T-cell neoplasms are 3.5 times more common than B-cell neoplasms [3]. Peripheral T-cell lymphoma (PTCL) is a relatively uncommon type of lymphoma, particularly in Western countries where it has an even lower prevalence. It affects both males B. D. Levine (*) : L. L. Seeger : K. Motamedi Department of Radiological Sciences, UCLA-Santa Monica Medical Center and Orthopedic Hospital, 1250 16th Street, Suite 2340, Santa Monica, CA 90404, USA e-mail: [email protected] A. W. James Department of Pathology and Laboratory Medicine, UCLA-Santa Monica Medical Center and Orthopedic Hospital, Santa Monica, CA, USA

and females, but is uncommon in children [4]. PTCLs account for approximately 25 % of all non-Hodgkin lymphomas in Korea, and 10–15 % in Europe [5]. PTCLs are overall associated with a 5-year survival of 25 % and demonstrate a poorer response to treatment than other lymphomas. Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) represents a rare subclassification of peripheral T-cell lymphoma. SPTCL can be differentiated from cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome) mainly due to its location, the former being subcutaneous, and the latter being cutaneous. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. Mycosis fungoides is indolent, often presenting first with eczema or dermatitis, with later progression to cutaneous tumors. In Sézary syndrome, malignant T-cells are found in the peripheral blood, skin, lymph nodes, or viscera. Sézary syndrome is associated with an average survival of 32 months [6]. In contrast to the cutaneous T-cell lymphomas, SPTCL does not typically infiltrate the dermis or epidermis. The most important role of imaging in both SPTCL and the cutaneous lymphomas is accurate differentiation of the process from several mimics of the disease. In addition, imaging is critical for evaluation of local extent of disease, disease progression, and stage. We present here a case of a 21-year-old female with SPTCL. MRI features and a review of the literature will be discussed.

Case report The patient is a 21-year-old female who presented with a 1-month history of pain and tenderness in the left buttock and hip area, tender left inguinal lymph nodes, daily fevers to 100.6 °F (often rising to 103 °F), and night sweats. Initial physical examination was significant for a tender right posterior cervical lymph node and tender left inguinal lymph nodes. In addition, there were two large palpable areas in the soft

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Fig. 3 Hematoxylin and eosin (H&E)-stained section at 100× magnification. The atypical lymphoid infiltrate involves the fat lobules with neoplastic lymphocytes (red arrowheads) rimming the individual fat cells. The neoplastic cells have round to oval-shaped hyperchromatic nuclei, irregular nuclear contours, inconspicuous nucleoli, and variable amounts of pale cytoplasm. Histiocytes, apoptotic debris, and scattered plasma cells are admixed. h histiocytes, p plasma cells Fig. 1 Coronal short tau inversion recovery (STIR) image (TR/TE 3,300/ 36) demonstrates two mass-like areas of increased signal infiltrating the subcutaneous tissues with evidence of lymphatic congestion (arrows)

tissue over the left lateral thigh and buttock that were firm, indurated, and tender with overlying ecchymosis.

Fig. 2 Axial T2 fat-suppressed image (TR/TE 4,516/70) shows enlarged left inguinal lymph nodes (arrows). The mass-like area of abnormal T2 signal is again seen infiltrating the subcutaneous tissues with evidence of lymphatic congestion

Initial evaluation for infection was negative. This included negative studies for Bartonella henselae, Coccidioides, mononucleosis, Lyme disease, gonococcus, acid-fast bacilli (AFB), chlamydia, and human papillomavirus (HPV). She had

Fig. 4 CD8 stain at 40× magnification. Immunoperoxidase stain for CD8 at 40× magnification. The diffuse, strong positive staining indicates the neoplastic cells are CD8-positive cytotoxic T-cells

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Fig. 5 PET scan demonstrating avid FDG uptake in the subcutaneous tissues along the lateral aspect of the hip/thigh (green arrow) corresponding to the more proximal infiltrative lesion on MRI. There is FDG uptake in the left inguinal lymph nodes (yellow arrow). Note physiologic FDG uptake in the bladder

negative serologies for hepatitis B and C, human immunodeficiency virus (HIV), rapid plasma regain (RPR), antinuclear antibody (ANA), and double-stranded DNA antibodies (dsDNA). C-reactive protein (CRP) was normal.

Initial magnetic resonance imaging (MRI) demonstrated two infiltrative mass-like lesions in the subcutaneous tissues along the lateral aspect of the thigh, in the lateral hip/gluteal region, as well as left inguinal lymphadenopathy (Figs. 1 and 2). Prebiopsy differential considerations based on the MRI, included an infectious process (cellulitis), connective tissue disease, or less likely lymphoma. Computed tomography (CT)-guided core needle biopsy in the area of induration and subcutaneous edema over the lateral aspect of the left thigh was performed. Histology was diagnostic for subcutaneous panniculitis-like T-cell lymphoma with CD8+ cells positive for cytotoxic granules (Figs. 3 and 4). The patient underwent whole-body Positron emission tomography/computed tomography (PET/CT) that revealed bilateral cervical, axillary, and inguinal lymph node uptake, the largest and most significant in the left inguinal area. The indurated areas in the left lateral thigh and left inguinal lymph nodes showed increased fluorodeoxyglucose (FDG) uptake (Fig. 5). The patient was initially treated with prednisone 100 mg per day with initial favorable response to treatment (Fig. 6). After 2 years, the patient relapsed, manifested by FDG uptake in new subcutaneous nodules and masses, intraperitoneal nodules, and axillary and inguinal lymphadenopathy. She was again further treated with prednisone, and at 3 years after presentation of disease, the patient had resolution of all previously seen areas of FDG uptake on PET/CT.

Discussion SPTCL is a rare subclassification of peripheral T-cell lymphoma. According to a case series by Ghobrial et al. [7] of 21 patients with SPTCL, the median age at diagnosis was

Fig. 6 Coronal short tau inversion recovery (STIR) image (TR/TE 6,000/35), 3 months after prednisone treatment, demonstrating interval decrease in size of the subcutaneous infiltrative lesions and lymphatic congestion (arrows)

Fig. 7 Axial T2 fat-suppressed image (TR/TE 4,516/70) demonstrating a central area of intermediate T2 signal within the infiltrative lesion (arrow)

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42 years, with presentations that included constitutional symptoms and elevated liver enzymes, as in our case. Similarly, in a group study of 83 cases of SPTCL by Willemze et al. [8], median age was 36 years with 19 % being 20 years or younger. In this study, 67 % of the patients were female and 33 % were male. As in our case, these studies suggest that SPTCL occurs more commonly in younger females. SPTCL is associated with cytopenias, elevated liver enzymes, and elevated LDH, all of which were present in our case. Initial diagnosis of SPTCL can be elusive and delayed, due to its indolent nature and the fact that it can mimic an inflammatory or infectious process both clinically and on imaging. It is often misdiagnosed initially as a benign and/or non-specific process. The clinical presentation of SPTCL is that of multiple subcutaneous nodules or plaques. These are similar in presentation clinically as those seen in infectious or inflammatory skin processes, such as connective tissue diseases or some inflammatory arthropathies (rheumatoid arthritis). The subcutaneous lesions are most commonly located on the trunk or lower extremities, and less commonly on the upper extremities or face [9]. Eventually these plaques or nodules can ulcerate and become necrotic. Two clinical courses of SPTCL are thought to characterize the disease process: a relapsing panniculitis or a rapid progression and clinical deterioration related to hemophagocytic syndrome (anemia with ingestion of erythrocytes by monocytes and macrophages) [5]. Such a syndrome often leads to a fatal outcome. Hemophagocytosis may present in the bone marrow, lymph nodes, spleen, or liver without direct involvement of these organs by the malignant lymphocytes [8]. Hemophagocytosis is characterized by anemia, leukopenia, thrombocytopenia, and hepatosplenomegaly. Although the clinical course of SPTCL is variable, the overall 5-year survival is approximately 80 % [6]. More recent studies have suggested SPTCL be divided into two distinct clinicopathologic entities based on different phenotypes (SPTCL-AB and SPTCL-GD) [7]. It is the SPTCL-AB without hemophagocytic syndrome that has an excellent prognosis where treatment with prednisone only rather than chemotherapy should be considered [7], as in our case. However, in a case series by Ghobrial et al. [8], high-dose chemotherapy followed by bone marrow transplant was associated with a survival benefit, although not statistically significant. Because systemic disease is common at initial presentation of SPTCL [3], whole-body MRI has been proposed as a useful initial and follow-up imaging modality to assess SPTCL [4]. The histological features of SPTCL are that of lymphohistiocytic cells that surround subcutaneous fat lobules. The lymphoid cells are said to “rim” the individual adipocytes (Fig. 3). Imaging features include multiple subcutaneous, enhancing nodules or plaques with an infiltrative pattern on CT or

MRI [4]. On MRI, nodular or mass-like enhancing areas infiltrate the subcutaneous tissues with lymphatic congestion (Fig. 1). At the center of the lesions there may be an area of intermediate T2-weighted signal (relative to skeletal muscle) that is helpful in differentiating a peripheral T-cell lymphoma from an inflammatory or infectious process (Fig. 7) [10]. Such processes that should be included in the differential diagnosis of SPTCL on MRI include rheumatoid nodules, connective tissue diseases such as lupus, infectious etiologies, or metastatic disease (melanoma or breast primary). Thus, the differential diagnosis of an infiltrative subcutaneous/soft tissue mass that demonstrates a surrounding inflammatory response on MRI should include these diseases in addition to a peripheral T cell lymphoma such as SPTCL.

Conclusions We present a case of SPTCL in a 21-year-old female with a clinical presentation similar to other reports in the literature. The MRI features of SPTCL include nodular or mass-like enhancing areas that infiltrate the subcutaneous tissues with lymphatic congestion. They may demonstrate central areas of intermediate T2-weighted signal (relative to skeletal muscle), a finding that can be seen in peripheral T-cell lymphoma, including SPTCL. This finding may be useful in differentiating a peripheral T cell lymphoma, such as SPTCL, from other inflammatory, infectious, or metastatic processes that can have similar MRI appearances. Although such infectious processes may show more edema than SPTCL, and metastatic processes are likely to be more nodular, it is the clinical findings in combination with imaging findings that narrow the differential. Ultimately, such cases may require tissue diagnosis.

Conflict of interest All the authors declare that they have no conflicts of interest.

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Skeletal Radiol 5. Lee HJ, Im JG, Goo JM, Kim KW, Choi BI, Chang KH, et al. Peripheral T-cell lymphoma: spectrum of imaging findings with clinical and pathologic features. Radiographics. 2003;23:7–28. 6. Otero HJ, Jagannathan JP, Prevedello LM, Johnston CJ, Ramaiya NH, Van den Abbeele AD, et al. CT and PET/CT findings of T-cell lymphoma. AJR. 2009;193:349–58. 7. Ghobrial IM, Weenig RH, Pittlekow MR, Guangzhi QU, Kurtin PJ, Ristow K, et al. Clinical outcome of patients with subcutaneous panniculitis-like T-cell lymphoma. Leuk Lymphoma. 2005;46: 703–8.

8. Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. Subcutaneous panniculitis like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC cutaneous lymphoma group study of 83 cases. Blood. 2008;111:838–45. 9. Weenig RH, Su WP. Subcutaneous panniculitis-like T-cell lymphoma presenting as venous stasis ulceration. Int J Dermatol. 2006;45:1083–5. 10. Kim EY, Kim SS, Ryoo JW, Na DG, Roh HG, Byun HS, et al. Primary peripheral T-cell lymphoma of the face other than mycosis fungoides: computed tomography and magnetic resonance imaging findings. J Comput Assist Tomogr. 2004;28:670–5.

Subcutaneous panniculitis-like T-cell lymphoma: MRI features and literature review.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) represents a rare subclassification of peripheral T-cell lymphoma (PTCL). We present a case of ...
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