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Primary lymphoma of the temporal bone presenting as XIIth cranial nerve weakness.


Only 1% of all cases of primary bone lymphoma involve the skull. (1) Histologically, 80% of all cases of primary bone lymphoma are of the diffuse large-cell lymphoma type. (1) Patients with a neoplasm of the temporal bone usually present with deficits in hearing, balance, and facial function. We report a new case of primary lymphoma of the temporal bone that manifested as XIIth cranial nerve palsy, and we review the recent literature on this entity.

Case report

A 23-year-old woman presented with a 2-day history of left mastoid headache that radiated toward the occiput. She also reported associated phonophobia, and her tongue movements were abnormal and interfered with her ability to swallow.

Findings on the head and neck examination were normal except for tongue deviation to the left with hemilingual atrophy (figure 1). Results of audiometry and tympanography were normal. Computed tomography (CT) of the temporal bone demonstrated a bony lesion in the left temporal and occipital bones (figure 2). Magnetic resonance imaging (MRI) with MR angiography showed an enhancing soft-tissue mass in the area of the left jugular foramen, jugular bulb, and left hypoglossal canal.

Frozen and fresh biopsy specimens obtained through a limited mastoidectomy showed large lymphoma cells consistent with diffuse large B-cell malignant lyrnphoma. The patient's disease was staged as IE (Ann Arbor classification: extranodal cancer limited to a single region). (2) CT of the chest, abdomen, and pelvis was negative for metastasis. Cerebrospinal fluid samples and bone marrow biopsies were negative for neoplastic cells.

The patient underwent chemoradiation therapy and was in remission for 1 year. When she relapsed, she was found to have acute lymphoblastic leukemia, and she died secondary to Aspergillus pneumonia and polymicrobial septicemia.


To the best of our knowledge, no case of primary bone lymphoma presenting as XIIth cranial nerve palsy has been previously reported in the literature. In fact, only 18 cases of primary temporal bone lymphoma have been reported in the literature (3) since the initial case report by Tucci et al in 1992. (4)

The development of any tongue asymmetry and atrophy warrants imaging of the head to look for either inflammatory or neoplastic lesions. Lesions should be biopsied whenever possible. Laboratory values specific to certain organs, such as liver function tests and creatinine measurements, may aid in detecting an unknown primary. Temporal bone lesions may be metastatic or primary. Metastatic lesions in the temporal bone typically spread from primaries in the breast, lung, kidney, and stomach, so these areas require special attention. (5-7)


The existence of two different diagnostic criteria for primary bone lymphoma makes comparisons among different case reports and studies difficult. Criteria proposed by Coley et al include (1) a primary focus in a single bone on admission, (2) unequivocal histologic proof from a bone lesion (not from a metastasis), and (3) metastasis present on admission only if regional or if the onset of the primary tumor preceded the appearance of the metastasis by at least 6 months. (8) The World Health Organization's criteria specify (1) a single skeletal site with or without regional lymph node involvement and (2) involvement of multiple bones with no visceral or lymph node involvement. (9)

The most common symptom of primarylymphoma of the temporal bone is hearing loss. Typically, the loss is conductive, as the otic capsule is more resistant to tumor invasion. (6) When sensorineural hearing loss occurs, it is typically the result of involvement of cranial nerve VIII. Meanwhile, the facial nerve sheath is relatively resistant to invasion, and thus paralysis is not seen unless nerve fibers are infiltrated by tumor cells or unless tumor cells destroy the facial canal. (10) Tumors usually infiltrate the facial nerve at the geniculate ganglion. (4) Vertigo can develop when there is bony erosion into the semicircular canals. Meanwhile, systemic findings such as fever or night sweats are rare. (11)

Most primary bone 1ymphomas are osteolytic on imaging studies. A periosteal reaction is possible, although mixed sclerotic-lytic lesions with soft-tissue involvement may also be seen. (12) Blastic lesions are more suggestive of metastatic disease than primary disease. (12) Other pathologies with a similar radiologic appearance include Ewing sarcoma, Hodgkin disease, solitary myeloma, neuroblastoma, eosinophilic granuloma, and a metastatic lesion from a nonosseous primary. (13) Therefore, any abnormal radiologic findings that are suspicious for a neoplasm should be biopsied. The cells of primary bone lymphoma may be of mixed appearance such that osteomyelitis is suspected. (14) Central nervous system involvement can be shown with Tl-weighted MRI. (13) CT is less sensitive and may not distinguish the difference between lesions that invade the parenchyma and extracerebral masses. (15)


Several chemoradiation regimens are available for use, including, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone). (16) Reported overall 5-year survival rates range from 60 to 83%. (16,17) The value of an extensive staging workup is questionable. In a retrospective study, Heyning et al observed that there were no statistically significant differences in survival among patients with stage I, II, and IV disease, although there was a slight but not significant trend toward worse outcomes in those with stage IV disease. (17) Similarly, Lewis et al found no statistically significant difference in long-term survival between patients with stage IE primary bone lymphoma and those with stage II or IV disease. (18) These two findings (17,18) put to question the value of the Ann Arbor staging classification system for primary bone lymphoma. Favorable prognostic factors seem to include age younger than 40 years, the use of combined-modality therapy (chemoradiation), an absence of B symptoms (fever, night sweats, and weight loss), normal lactate dehydrogenase levels, and female sex. (1) These prognostic factors may be more predictive of survival than the Ann Arbor stage.

In summary, any unexplained cranial nerve palsy can be a sign of malignancy. Imaging of the head and neck should be obtained to look for any suspicious lesions. If one is found, biopsy is indicated.


(1.) Beal K, Allen L, Yahalom J. Primary bone lymphoma: Treatment results and prognostic factors with long-term follow-up of 82 patients. Cancer 2006;106(12):2652-6.

(2.) Rademaker J. Hodgkin's and non-Hodgkin's lymphomas. Radiol Clin North Am 2007;45(1):69-83.

(3.) Ogawa S, Tawara I, Ueno S, et al. De novo CD5-positive diffuse large B-cell lymphoma of the temporal bone presenting with an external auditory canal tumor. Intern Med 2006;45(11):733-7.

(4.) Tucci DL, Lambert PR, Innes DJ Jr. Primarylymphoma of the temporal bone. Arch Otolaryngol Head Neck Surg 1992;118(1):83-5.

(5.) Hill BA, Kohut RI. Metastatic adenocarcinoma of the temporal bone. Arch Otolaryngol 1976;102(9):568-71.

(6.) Schuknecht HF, Allam AF, Murakami Y. Pathology of secondary malignant tumors of the temporal bone. Ann Otol Rhinol Laryngol 1968;77(1):5-22.

(7.) Maddox HE III. Metastatic tumors of the temporal bone. Ann Otol Rhinol Laryngol 1967;76(1): 149-65.

(8.) Coley BL, Higinbotham NL, Groesbeck HE Primary reticulum-cell sarcoma of bone; summary of 37 cases. Radiology 1950;55(5):641-58.

(9.) Unni KK, Hogendoorn PC. Malignant lymphoma. In: Fletcher CD, Unni KK, Mertens F, eds. Pathology and Genetics of Tumours of Soft Tissue and Bone. Kleihues P, Sobin LH, series eds. World Health Organization Classification of Tumours. Lyon, France: IARC Press; 2002:306-8.

(10.) Saito H, Chinzei K, Furuta M. Pathological features of peripheral facial paralysis caused by malignant tumour. Acta Otolaryngol Suppl 1988;446:165-71.

(11.) Dubey P, Ha CS, Besa PC, et al. Localized primary malignant lymphoma of bone. Int J Radiat Oncol Biol Phys 1997;37(5):1087-93.

(12.) Krishnan A, Shirkhoda A, Tehranzadeh J, et al. Primary bone lymphoma: Radiographic-MR imaging correlation. Radiographics 2003;23(6):1371-83; discussion 1384-7.

(13.) Paige ML, Bernstein JR. Transcalvarial primarylymphoma of bone: A report of two cases. Neuroradiology 1995;37(6):456-8.

(14.) Ostrowski ML, Unni KK, Banks PM, et al. Malignant lymphoma of bone. Cancer 1986;58(12):2646-55.

(15.) Zimmerman RA. Central nervous system lymphoma. Radiol Clin North Am 1990;28(4):697-721.

(16.) Tondini C, Zanini M, Lombardi F, et al. Combined modality treatment with primary CHOP chemotherapy followed by locoregional irradiation in stage I or II histologically aggressive non-Hodgkin's lymphomas. J Clin Oncol 1993;11(4):720-5.

(17.) Heyning FH, Hogendoorn PC, Kramer MH, et al. Primary non-Hodgkin's lymphoma of bone: A clinicopathological investigation of 60 cases. Leukemia 1999;13(12):2094-8.

(18.) Lewis VO, Primus G, Anastasi J, et al. Oncologic outcomes of primary lymphoma of bone in adults. Clin Orthop Relat Res 2003;(415):90-7.

Zi Yang Jiang, MD; Miriam I. Saadia-Redleaf, MD

From the Department of Otolaryngology, School of Medicine, University of Illinois at Chicago.

Corresponding author: Miriam I. Saadia-Redleaf, MD, Department of Otolaryngology, University of Illinois at Chicago, 1885 W. Taylor St., Chicago, IL 60612. E-mail:
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Author:Jiang, Zi Yang; Saadia-Redleaf, Miriam I.
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:1USA
Date:Mar 1, 2011
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