Primary Non-Hodgkin’s Lymphoma of the Uterine Cervix: Case Report of Long-Term Survival Patient

Introduction

Primary malignant lymphomas involving the cervix uterine are extremely rare [1]. This report describes one patient with non-Hodgkin’s lymphoma of the uterine cervix treated successfully with a combination of chemotherapy and radiation therapy at the national institute of oncology over the 16 years.

Case Report

A 49-year-old patient was seen in 1997, complaining of menorrhagia and vaginal bleeding after intercourse for 8 months. On gynecological examination, there was an exophytic tumor arising from the cervix and involving the anterior and left walls of the upper part of the vagina, with the spread of the both parametrium–cervical stroma, and FIGO stage was IIb. Histology of a cervical biopsy showed a diffuse B cell non-Hodgkin’s lymphoma. Further immunohistochemical studies were conducted to specify the immune phenotype of the tumor. Tumor cells showed intensive CD-20 and CD-45 positivity (Fig. 1). Pan computerized tomography (CT) showed a bulky uterine cervix tumor with probable infiltration of the parametria (Fig. 2). There was no evidence of other sites of disease. Bone marrow biopsy and spinal fluid analysis were negative for lymphoma cells. The tumor was classified as stage IEA according to the Ann Arbor classification. After six courses of cytotoxic chemotherapy according to the CHOP protocol (cyclophosphamide, adriamycin, vincristine, and prednisone), she was treated with external radiotherapy to the pelvis (46 Gy in 23 fractions). A complete response was obtained. This patient has been followed up for 16 years with no evidence of recurrent disease.

Fig. 1.

IHC: CD-20 and CD-45 positivity

Fig. 2.

Axial CT: bulky uterine cervix tumor

Discussion

Primary malignant lymphomas of the genital tract are a class of rare disease [1–3]. They account for 1.5 % of extranodal non-Hodgkin’s lymphomas [4]. Only 0.12 % of all non-Hodgkin’s lymphomas originate from the uterine cervix [5].

Cervical lymphoma may present in a wide age range (20–80 years); however, 80 % of patients are premenopausal, and cervical lymphoma tends to occur in younger patients compared to other lymphomas [2, 6, 7]. The common presenting symptoms are abnormal vaginal bleeding (70 %), perineal discomfort (40 %), persistent vaginal discharge (20 %) and, less frequently, abdominal masses, dyspareunia, and urinary retention [2, 3, 6].

This situation makes cervical punch biopsy and immunohistochemical studies indispensable for diagnosis [4, 8]. The most frequent histology is the diffuse large B-cell non-Hodgkin’s lymphoma of the WHO classification [8–11]. Besides, a complete physical examination searching for enlarged peripheral lymph nodes, further investigation should always include chest, abdominal, and pelvic CT scans along with bone marrow biopsy [8].

According to case reports and short series, there is no widely accepted consensus on its management; the mainstay of therapy consists of irradiation alone or irradiation with either surgery or chemotherapy [1, 4, 5, 12]. Others used, only combination chemotherapy [5]. Our patient was treated with a combination of chemotherapy and radiotherapy.

The use of chemotherapy and radiation was reported by Stroh et al. [13–15] who reported 16 cases of lymphomas of the cervix, of which 12 cases were treated with a combination of radiation and chemotherapy. In this series, 90 % of patients with low risk factors were disease free at 5 years. They concluded that combined modality treatment with CHOP-based chemotherapy and moderate doses of radiation was the best treatment for non-Hodgkin’s lymphoma of the cervix.

Recently, Heredia et al. [8] reported two cases of non-Hodgkin’s lymphoma of the uterine cervix treated with three courses of CHOP and radiotherapy. One patient had 61 months disease-free survival after the initial diagnosis and another had 15 months.

Thus, combination chemotherapy has been proven effective, resulting in excellent response rate and long-term cures [6].

In general, the most common stage at time of diagnoses of malignant lymphomas are IE tumors according to the Ann Arbor Staging classification for lymphomas, for which pelvic radiotherapy or neoadjuvant chemotherapy has been successfully applied as first lines of treatment [3].

An overall 5-year survival rate of 77 % was reported for 18 cases of cervical lymphomas. Prognosis is still considered to be good [8].

Conclusion

Primary lymphoma of the uterine cervix is a rare malignancy. Most patients present with stage IE disease. The good results obtained by some authors in patients treated with combination chemotherapy in conjunction with irradiation have suggested that this management can be routinely used in clinical practice. Prognosis generally tends to be favorable.