Human T-Lymphotropic Virus Type-1 Related Adult T-cell Leukemia/Lymphoma Presenting as a Parotid Mass Diagnosed By Fine-Needle Aspiration Biopsy Guo-Xia Tong, M.D., Ph.D., Osvaldo Hernandez, M.D., Herman T. Yee, M.D., Ph.D., Shyun-Hua Zheng, M.D., Ph.D., and Grace C.H. Yang, M.D., F.I.A.C. * A 48-yr-old black woman with a history of blood transfusions for menorrhagia secondary to uterine fibroids but no known Carib- bean association presented with a 6-wk history of a rapidly en- larging right parotid mass. At the time of presentation, she could not close her right eye. An aspiration biopsy showed small, me- dium, and large lymphoma cells with angulated nuclei, red ma- cronucleoli, and basophilic cytoplasm with fine vacuoles. Flow cytometry indicated a (CD25 + /CD7 - ) T-cell lineage, suggesting an human T-lymphotropic virus (HTLV) 1-related T-cell leukemia/ lymphoma, which was confirmed by polymerase chain reaction (PCR)-based amplification on DNA extracted from fresh tissue with specific oligonucleotide primers for HTLV-1 DNA sequence. Histology showed interstitial infiltration and destruction of the parotid parenchyma by lymphoma cells without involvement of adjacent lymph nodes. Total body CT scan and magnetic reso- nance imaging (MRI) studies were negative for lymphadenopathy but showed liver metastasis. To our knowledge, this is the first reported case of HTLV-1-related primary parotid lymphoma as the initial presentation of adult T-cell leukemia/lymphoma. Diagn. Cytopathol. 2004;31:333–337. © 2004 Wiley-Liss, Inc. Key Words: primary parotid lymphoma; T-cell lymphoma; HTLV-1; adult T-cell lymphoma/leukemia; salivary gland neo- plasms Fine-needle aspiration (FNA) biopsy is used in the preop- erative evaluation of parotid masses to triage benign from malignant epithelial neoplasms. 1,2 Primary lymphoma of the salivary glands accounts for only 1–2% of neoplasms and 10% of malignancies of salivary glands and 5% of extran- odal lymphomas. 3 The majority of primary lymphomas of salivary glands are of B-cell lineage and they usually arise in the background of lymphoproliferation associated with Sjo ¨gren’s syndrome. The most common subtype is low- grade B-cell lymphoma of mucosa-associated lymphoid tis- sue (MALT) type. 3,4 In a previous study from our Cytopa- thology Laboratory, 5 all 18 cases of salivary gland lymphomas were B-cell lymphomas. Primary T-cell lym- phoma of the salivary gland is extraordinarily rare. Only 15 cases have been reported in the English literature, including 6 cases in the parotid gland, 5 cases in the submandibular gland, 2 cases in the sublingual gland, and 2 cases in which the salivary gland was unspecified. 6 Of the 15 cases, 9 cases were from Chinese patients from mainland China or Hong Kong, 7 4 cases were from Western population, and in the remainder, the patients’ nationalities were not specified. Many of these reported cases were positive for Epstein-Barr virus (EBV) or human immunodeficiency virus (HIV) in- fection. 7,8 We report here a primary T-cell lymphoma of the parotid gland in a black woman who was negative for both EBV and HIV infection but positive for human T-lympho- tropic virus type-1 (HTLV-1). To the best of our knowl- edge, it is the first reported case of HTLV-1-related primary parotid T-cell lymphoma and the fifth case of primary T-cell lymphoma of the salivary gland in the Western population. Clinical History A 48-yr-old previously healthy black woman noticed a small painless nodule in her right cheek 6 wk before her presentation at Bellevue Hospital Center. The nodule grew rapidly, and 4 wk later, she experienced excruciating pain radiating to her right forehead, eye, and retroauricular area. Department of Pathology, New York University School of Medicine, New York, New York *Correspondence to: Grace C.H. Yang, M.D., Cytopathology Labora- tory, NB 4S-16, New York University Medical Center, 462 First Ave., New York, NY 10016. E-mail: grace.yang@nyu.edu Received 23 October 2003; Accepted 27 April 2004 DOI 10.1002/dc.20121 Published online in Wiley InterScience (www.interscience.wiley.com). © 2004 WILEY-LISS, INC. Diagnostic Cytopathology, Vol 31, No 5 333