Kaohsiung J Med Sci March 2007 • Vol 23 • No 3 138
© 2007 Elsevier. All rights reserved.
Involvement of non-Hodgkin’s lymphoma (NHL) in the female genital tract is uncommon, with the ovary being the principal site. Primary NHL of the female genital tract is rarer, accounting for 1.5–2% of extra-nodal malignant lymphoma, with the majority of cases associated with the ovary, uterine cervix, and vagina, and fewer with the uterine corpus [1]. To our knowl-edge, fewer than 20 cases of primary lymphoma aris-ing in the uterine corpus have been reported in the literature [2,3]. Most of the reported cases have been diagnosed as diffuse large cell lymphoma by the working formulation and have shown B-cell pheno-types. We report an unusual case of uterine T-cell lymphoma, which was initially diagnosed as a possible necrotic leiomyosarcoma after negative endometrial curettage.
C
ASEP
RESENTATIONA 68-year-old woman, gravida 4, para 4, who was menopausal without hormone replacement therapy, presented with fever, vaginal bleeding, and lower abdomen pain of 20 days’ duration. Physical exam-ination revealed an anemic-looking condition with no peripheral lymphadenopathy or hepatospleno-megaly. Pelvic examination disclosed a necrotic vagina and a bulky uterus. Pelvic ultrasonography confirmed the enlarged uterus, which measured 10.6×7.9×6.2cm. She subsequently underwent vaginal and cervical biopsy, and endometrial curettage. Vaginal biopsy showed some necrotic debris and a degenerated specimen that was covered by squamous epithelium. Cervical biopsy and endometrial curettage demon-strated absence of malignant cells. Laboratory data, including complete blood count and differential count, showed only an abnormal hemoglobin level (8.7 g/dL). Chest roentgenogram was normal. Computed tomog-raphy (CT) scan of the abdomen showed a huge mass, measuring 8.3 cm, with homogeneous en-hancement at the uterus. No enlarged pelvic lymph Received: January 6, 2006 Accepted: August 24, 2006
Address correspondence and reprint requests to: Dr Shih-Cheng Hsu, Department of Obstetrics and Gynecology, Kaohsiung Medical University Hospital, 100 Tzyou 1stRoad, Kaohsiung 807, Taiwan.
E-mail: chenmed.tw@yahoo.com.tw
P
RIMARY
T-
CELL
L
YMPHOMA OF THE
U
TERINE
C
ORPUS
Ching-Ju Shen,1Eing-Mei Tsai,1,2Kun-Bow Tsai,3Ching-Hu Wu,1and Shih-Cheng Hsu1,2
1Department of Obstetrics and Gynecology, Kaohsiung Medical University Hospital, 2Department of
Obstetrics and Gynecology, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, and 3Department of Pathology, Kaohsiung Municipal Hsiao-Kang Hospital,
Kaohsiung, Taiwan.
Malignant lymphoma involving the genital tract is rare, and primary lymphoma of the uterus is even rarer. Immunohistochemically, most of the reported cases have been diffuse large cell lym-phomas with a B-cell nature. Herein, we report an unusual case of uterine T-cell lymphoma in a 68-year-old woman. She suffered from fever, vaginal bleeding, and a huge mass in the lower abdomen. The initial clinical and histologic studies failed to indicate a cell type. We provide the clinical details of the case and discuss the diagnostic pitfalls.
Key Words:T-cell lymphoma, uterus (Kaohsiung J Med Sci 2007;23:138–41)
Uterine T-cell lymphoma
Kaohsiung J Med Sci March 2007 • Vol 23 • No 3 139
node was found. Although the cytologic study was negative, a provisional diagnosis of necrotic leio-myosarcoma was made. Total abdominal hysterec-tomy, bilateral salpingo-oophorechysterec-tomy, and pelvic lymph node biopsy were carried out. Intraoperative findings revealed no small and large bowel, dia-phragm, and peritoneum implants. A bulky uterus with smooth surface was also noted and the adnexa were remarkable.
Microscopically, the sections of myometrium showed diffuse transmural infiltration of medium-sized lymphoid cells with hyperchromatic nuclei and pinkish cytoplasm. The sections of cervix showed diffuse infiltration of atypical lymphoid cells with necrotizing inflammation. Immunohistochemically, the tumor cells were positive for LCA, CD3 and CD45RO, and negative for CD20 and terminal deoxynucleotidyl
transferase (TdT), which suggested peripheral T-cell lymphoma (Figure). To exclude the possibility of leu-kemia, the patient received peripheral blood and bone marrow smear after operation, which were negative.
D
ISCUSSIONExtranodal involvement in NHL is not unusual, and the gastrointestinal tract and skin are the particular sites. Extranodal lymphomas are generally described as being either “primary” or “secondary”. The crite-ria for primary uterine lymphoma were first pro-posed by Fox and More: (1) clinically confined to the uterus; (2) no evidence of leukemia; (3) a fairly long interval between the primary uterine lymphoma and the secondary tumor [4]. In a large review of 1,467
Figure.(A) Diffuse infiltrates of small to medium-sized lymphoid cells exhibiting round nuclei with clumped chromatin and scattered mitotic figures (arrows) (hematoxylin & eosin, 100×). (B) CD3 and (C) CD45RO reveal positive diffuse immunostain (immunohisto-chemical stain, 40×). (D) The lymphoma cells are negative with terminal deoxynucleotidyl transferase stain (immunohistochemical stain, 20×).
A B
C D
H&E CD3
Kaohsiung J Med Sci March 2007 • Vol 23 • No 3 140
C.J. Shen, E.M. Tsai, K.B. Tsai, et al
cases done by the National Cancer Institute, primary NHL of the uterine corpus accounted for about 0.002% of extranodal malignant lymphoma [5]. In this case, clinical examination and CT revealed no tumor other than that in the uterus, and leukemia was absent after peripheral and bone marrow smear examinations. These findings indicate that the patient had primary uterine lymphoma.
Most primary lymphomas of the uterine corpus are NHLs, and the majority of these are diffuse large B-cell types. Immunohistochemical staining can be used to differentiate between B- or T-cell type. In this patient, the tumor had diffuse, medium-sized cell type histologically, and was classified as a peripheral T-cell lymphoma (LCA+, CD3+, CD45RO+, CD20−, TdT−) from immunohistochemistry. The origin of leukemia must be excluded if lymphoma is diagnosed. Hence, bone marrow biopsy was performed after surgery, and it was negative for leukemia. According to the Revised European-American classification of lymphoid neoplasms, peripheral T-cell lymphoma accounts for 6% of NHL cases. Its prognosis is poor and the 5-year survival rate is less than 30% [6]. Because of the rarity of primary NHL of the uterus, the optimal treatment protocol is unclear. Most clinicians favor simple hysterectomy and bilateral salpingo-oophorectomy, chemotherapy, irradiation, and their combinations.
It is difficult to diagnose primary lymphoma of the cervix and corpus by cytologic examination. Cervico-vaginal cytology plays only a limited role in the diag-nosis of primary or secondary lymphoma of the female genital tract, even in an accessible location such as the cervix, because the tumor cells are almost subepi-thelial and rarely exfoliate without surface ulcera-tion. Usually, the presence of a marked inflammatory exudate masks these tumor cells. On the other hand, endometrial cytology often fails to detect the tumor cells that may result from necrotic tissues in the uterine cavity. Of the 22 malignant lymphomas collected by
Whitaker [7], only five of the 13 cases cytologically examined were positive. In the series of Harris and Scully [8], only two of the 10 cases examined cytolog-ically were positive. In our case, endometrial curet-tage demonstrated no lymphoma cells. This led us to an incorrect initial diagnosis.
In conclusion, uterine lymphoma is a rare tumor where correct preoperative diagnosis is required. Un-fortunately, correct diagnosis may be delayed because the initial cytopathologic study often fails to show malignancy, with the resulting clinical diagnosis often being benign uterine mass. Malignant lymphoma should be kept in mind in patients suffering from uterine mass and unknown fever.
R
EFERENCES1. Scollo P, Licitra G, Galia A. Uterine localization of a non-Hodgkin’s lymphoma. A case report and review of literature. Eur J Gynaecol Oncol 1996;17:241–6. 2. Renno SI, Moreland WS, Pettenati MJ, et al. Primary
malignant lymphoma of uterine corpus: case report and review of the literature. Ann Hematol 2002;81:44–7. 3. Vang R, Medeiros LJ, Ha CS, et al. Non-Hodgkin’s
lymphomas involving the uterus: a clinicopathologic analysis of 26 cases. Mod Pathol 2000;13:19–28. 4. Fox H, More JR. Primary malignant lymphoma of the
uterus. J Clin Pathol 1965;18:723–8.
5. Latteri MA, Cipolla C, Gebbia V, et al. Primary extra-nodal non-Hodgkin lymphomas of the uterus and the breast: report of three cases. Eur J Surg Oncol 1995;21: 432–4.
6. Lopez-Guillermo A, Cid J, Salar A, et al. Peripheral T-cell lymphomas: initial features, natural history, and prognostic factors in a series of 174 patients diagnosed according to the R.E.A.L. Classification. Ann Oncol 1998;9:849–55.
7. Whitaker D. The role of cytology in the detection of malignant lymphoma of the uterine cervix. Acta Cytol 1976;20:510–3.
8. Harris NL, Scully RE. Malignant lymphoma and gran-ulocytic sarcoma of the uterus and vagina. Cancer 1984; 53:2530–45.
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