European Journal of Radiology Extra 72 (2009) e57–e60
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Primary sciatic nerve lymphoma: A case report of a tumor in a rare location with MR findings Zhi-qun Wang, Yan-qing Dong, Kun-cheng Li ∗ Department of Radiology, Xuanwu Hospital of Capital Medical University, Beijing 100053, PR China
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Article history: Received 16 May 2009 Received in revised form 21 July 2009 Accepted 21 July 2009 Keywords: Lymphoma MR
a b s t r a c t The peripheral nerves are a very rare site of origin for primary lymphoma. A 52-year-old man presented with progressive paresthesias and numbness of the right lower extremity, associated with radiating pain from the posterior thigh to the foot. Electromyogram indicated nerve origin impairment the right lower extremity. Emission computed tomography; chest computed tomography; abdominal ultrasound and bone marrow biopsy were performed to exclude a systemic lymphoma. Magnetic resonance imaging studies revealed a solitary fusiform mass along the right sciatic nerve, at last pathological examination confirmed the primary large B-cell non-Hodgkin’s lymphoma of the sciatic nerve. © 2009 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Primary extranodal lymphomas can arise from central nervous system, gastrointestinal system and so on. However, lymphoma arising from the peripheral nerve is exceedingly rare. At here, we report the clinical, radiological and pathological findings in a case of primary sciatic nerve lymphoma. 2. Case report A 59-year-old man had complained of right sciatica for about one month. One month ago, the patient presented paresthesias and numbness of the right lower extremity. This particularly involved the lower buttock, posterior thigh and the posterior calf and knee. He had increasing symptoms, with pain radiating from the posterior thigh to the foot. In the following days, he developed rapidly progressive right lower extremity weakness. He was then admitted to our hospital. Physical examination revealed marked swelling in his right posterior thigh. A lump can be touched without activity. On neurological examination, mental status and cranial nerves were normal. Upper extremity and left lower extremity strength was normal. Right lower extremity strength was markedly reduced. The patient has no any immunodeficiency disease. Electromyogram (EMG) was performed. The right thigh muscles examined showed a small quantity motor unit, ventricular fibrillation and positive sharp wave. Nerve conduction studies revealed reduction in amplitude of motor nerve action potential of the right
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common peroneal and sciatic nerve, slowed conduction velocity of the above two nerves and absent right tibial motor nerve. Reduced amplitude of right sensory nerve action potential and slowed conduction velocity were found existed in peroneal nerve. The right tibial sensory nerve was absent. All these findings indicated that the right lower extremity existed nerve origin impairment. Emission computed tomography (ECT) was conducted in the following day. After intravenous injection 99mTc-methylene diphosphonate (99mTC-MDP), the whole body bone scanning showed increased radioactive uptake of the right lower extremity soft tissue. However, there were no abnormal radioactive uptakes such as collection, sparseness or defect in the other regions of the whole body. Chest computed tomography (CT) revealed the olden calcification in the left upper lobe of the lung. There were no other positive findings in the lung field and the mediastinum. Abdominal ultrasound revealed no abnormality in the liver, gall, pancreas, spleen and kidney. Bone marrow biopsy was also negative finding. Magnetic resonance images (MRI) were obtained with a 1.5 T MR scanner (Sonata, Siemens, Erlangen, Germany). A solitary fusiform lesion along the right sciatic nerve was revealed, from the inferior margion of the ectogluteus to popliteal area. The lesion showed hyperintense on T2-weighted images (Fig. 1) and isointense to muscles on T1-weighted images (Fig. 2). On the axial images, the lesion located among the biceps femoris, semimembranous and semitendinous. In the central of the lesion, sciatic nerve can be found. In addition, there were hyperintense in the muscles adjacent to the lesion on T2-weighted fat-suppressed images which indicated infiltration (Fig. 3). Enhanced T1-weighted images revealed a welldefined, strongly enhanced lesion with a small portion of necrosis (Figs. 4 and 5). The preoperative diagnosis was neurogenic tumor based on the MRI findings.
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Fig. 1. Axial T2-weighted (TR/TE = 4300/100) MR imaging shows a solitary lesion of hyperintensity signal among the biceps femoris, semimembranous and semitendinous. Sciatic nerve can be found in the central of the tumor.
Fig. 2. Axial T1-weighted (TR/TE = 400/18) MR imaging reveals a solitary lesion of isointensity signal among the biceps femoris, semimembranous and semitendinous.
Fig. 3. Axial T2-weighted (TR/TE = 2900/50), fat-suppressed MR imaging shows a solitary lesion of hyperintensity signal among the biceps femoris, semimembranous and semitendinous. In addition, there were hyperintensity signal in the above adjacent muscles.
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Fig. 6. Large part of tumor is composed of small and round cells (Hematoxylin & Eosin staining, 200×).
Fig. 4. On gadolinium-enhanced, fat-suppressed, coronal T1-weighted (TR/TE = 520/20) MR imaging, markedly enhancement of the lesion is noted along sciatic nerve with a little non-enhancing portions.
Intraoperative gross examination found that the tumor diffusely expanded the sciatic nerve, and extending surrounding muscles. The tumor originated from the sciatic nerve and presented fish appearance. Nerve fibrous degeneration was found. The final histological examination showed small, round-cell tumor cells which extensively infiltrated in the endoneurium or perineurium of the nerve fibers (Figs. 6 and 7). In the immunohistochemical study, the neoplastic cells showed clear positivity with reagents raised to leukocyte common antigen, and the cells were positive with CD20, CD79a and CD10. The tumor cells showed increased Ki67 expression (Ki67 > 60%). Thus, the tumor was diagnosed as a diffuse large B-cell non-Hodgkin’s lymphoma. At last, the tumor was resected with the sciatic nerve and complementary chemotherapy with cyclophosphamide and vincristine was
Fig. 7. The tumor cell is composed of cells with medium-sized nuclei containing dense chromatin and several nucleoli (Hematoxylin & Eosin staining, 400×).
Fig. 5. On gadolinium-enhanced, fat-suppressed, axial T1-weighted (TR/TE = 520/20) MR imaging, markedly enhancement of the lesion is noted, which makes the boundary very clear.
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then carried out. No radiation therapy was performed on the patient. 3. Discussion We reported one case of primary malignant lymphoma of peripheral nerve. The patient presented with pain and decreased sensory function in the distribution of the sciatic nerve. EMG indicated nerve origin impairment. There was no evidence of systemic tumor based on the ECT, chest CT, abdominal ultrasound and bone marrow biopsy. MRI revealed a solitary fusiform mass along the right sciatic nerve. At last the tumor was resected and the pathology confirmed the primary large B-cell non-Hodgkin’s lymphomas of the sciatic nerve. To our knowledge, only eleven cases have been reported of a primary lymphoma in peripheral nerve in the English literature [1–8]. Eight of the cases involved the sciatic nerve [1–3,5–8], one in the ulnar nerve [4], one in the radial nerve, and one in the thoracic sympathetic chain and spinal nerve [1]. All patients presented with sensory and motor impairment in the distribution of the affected nerve. Most cases were B-cell in origin. This patient’s clinical course gives us several good suggestions. Sciatica is not always suggestive of an intraspinal lesion; it may occur with a tumor along the sciatic nerve. Magnetic resonance imaging can be useful for identifying a clinically peripheral nerve tumor. There are several characteristics of the tumor in MRI: (1) the tumor mainly infiltrates along the peripheral nerve such as sciatic nerve, sometimes extending to surrounding muscles; (2) the tumor often presents fusiform, which is isointense on T1-weighted MR images and hyperintense on T2-weighted MR images; (3) In the central of the tumor, the peripheral nerve such as sciatic nerve can be found; (4) after administration of Gd-DTPA, the tumor shows markedly enhancement. Large part of the tumor is homogeneous enhanced. Sometimes, a small non-enhancing portion of the tumor which suggesting necrosis can be found. The primary sciatic nerve lymphoma should be differentiated from schwannoma and neurofibroma. These tumors also manifest as fusiform shape along the nerve distribution. However, in schwannoma the nerve is usually at the periphery of the mass and
a capsule is identified in 70% of cases. These tumors are more likely to manifest cystic and calcification. All these characters can be used to differentiate from sciatic nerve lymphoma. In neurofibromas, the nerve is often seen at the central position. However, neurofibromas most frequently affect patients who are 20–30 years old and most of them are superficial which arise in small nerves [9]. Pathological study revealed a small, round-cell tumor composed of cells with medium-sized nuclei containing dense chromatin and several nucleoli. Immunohistochemical study can differentiate the type of tumor cells. The B-cells were always positive with CD20 and CD79a. However, the T-cells were always positive with CD3 and CD45RO. This case was characterized by extensive neoplastic proliferation, which finally proved to be B-cell lymphoma. Conflict of interest None declared. References [1] Misdraji J, Ino Y, Louis DN, Rosenberg AE, Chiocca EA, Harris NL. Primary lymphoma of peripheral nerve: report of four cases. Am J Surg Pathol 2000;24(9):1257–65. [2] Descamps MJ, Barrett L, Groves M, et al. Primary sciatic nerve lymphoma: a case report and review of the literature. J Neurol Neurosurg Psychiatry 2006;77(9):1087–9. [3] Kanamori M, Matsui H, Yudoh K. Solitary T-cell lymphoma of the sciatic nerve: case report. Neurosurgery 1995;36(6):1203–5. [4] Teissier J. Primary malignant lymphoma localized in the trunk of the ulnar nerve at the elbow. A case report. Ann Chir Main Membr Super 1992;11(2):170–2. [5] Eusebi V, Bondi A, Cancellieri A, Canedi L, Frizzera G. Primary malignant lymphoma of sciatic nerve. Report of a case. Am J Surg Pathol 1990;14(9):881–5. [6] Pillay PK, Hardy Jr RW, Wilbourn AJ, Tubbs RR, Lederman RJ. Solitary primary lymphoma of the sciatic nerve: case report. Neurosurgery 1988;23(3):370–1. [7] Roncaroli F, Poppi M, Riccioni L, Frank F. Primary non-Hodgkin’s lymphoma of the sciatic nerve followed by localization in the central nervous system: case report and review of the literature. Neurosurgery 1997;40(3):618–22. [8] Quinones-Hinojosa A, Friedlander RM, Boyer PJ, Batchelor TT, Chiocca EA. Solitary sciatic nerve lymphoma as an initial manifestation of diffuse neurolymphomatosis. Case report and review of the literature. J Neurosurg 2000;92(1):165–9. [9] Pilavaki M, Chourmouzi D, Kiziridou A, Skordalaki A, Zarampoukas T, Drevelengas A. Imaging of peripheral nerve sheath tumors with pathologic correlation: pictorial review. Eur J Radiol 2004;52(3):229–39.