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OJHAS Vol. 11, Issue 1:
(Jan-Mar 2012) |
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Non-Hodgkin’s Lymphoma of the Uterine Cervix |
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Vasudev DS, Saptahgiri
Institute of Medical Sciences and Research Centre,
Amrit Kaur Kaler,
MVJ Medical College & Research Hospital. |
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Address for Correspondence |
MVJ Medical College & Research Hospital,
India.
E-mail:
amrit_kaler@yahoo.co.in |
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Vasudev
DS, Kaler AK. Non-Hodgkin’s Lymphoma of the Uterine Cervix. Online J Health Allied Scs.
2012;11(1):13 |
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Submitted: Feb 22,
2012;
Accepted: Mar 20, 2012; Published: Apr 15, 2012 |
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| Abstract: |
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Non Hodgkin’s Lymphoma (NHL) causes many deaths worldwide
and its incidence is increasing. They occur commonly in middle aged
and elderly people and are disseminated at diagnosis. We present an
interesting case of NHL in a 52 years old female, who presented with
past a history of postmenopausal bleeding. A 3 x 5 cms endocervical
polyp was noticed in the cervix. Biopsy of the polyp revealed it to
be a CD20-positive diffuse large B-cell (DLBCL)-type NHL. She was diagnosed
as stage IE after staging work-up. She attained a complete response,
and has been in remission for 1 year 8 months.
Key Words:
Non-Hodgkin's lymphoma; Vaginal bleeding; Uterine cervix
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Primary Non Hodgkin’s lymphoma of the uterine cervix
is a rare event that demands particular attention because of the more
frequent appearance of an intense inflammatory reaction in this site,
such as chronic follicular cervicitis. The genital tract, in fact, is
more frequently the site of secondary extranodal lymphomas or leukemic
processes in the course of systemic metastasis. Primary NHL of uterine
cervix is rare with less than 60 cases reported.[1] It accounts for only 1% of extra abdominal lymphomas. Abnormal
vaginal bleeding is the common symptom of many gynecological disorders,
but an extremely rare presentation in Non-Hodgkin’s Lymphoma. As these
tumors arise from the cervical stroma, the epithelium is initially preserved
and therefore cytology is often normal.
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A 52 years old female presented with
post menopausal bleeding of 2 years. There was no history of fever,
night sweats, or weight loss. On general physical examination, there
was mild pallor. There was no lymphadenopathy or hepatosplenomegaly.
Other systemic examination was normal. Colposcopic examination revealed
a sessile polypoidal mass involving the posterior lip of cervix. The
lesion was soft and bled on contact. Other pelvic visceral structures
were normal. Her biochemical investigations and chest X-ray were normal.
CT scan of the abdomen and pelvis showed a large enhancing cervical
mass, 30x16x38 mm, connecting the endometrial cavity was noted. No gross
adenopathy identified. Bone marrow aspiration showed normal histology.
Pap smear examination did not reveal any dysplastic or malignant cells.
Patient underwent panhysterectomy procedure.
Fig. 1: Gross specimen showing a polypoidal mass
protruding from the cervix. |
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Pathological examination of the specimen showed a
pink, soft, sessile polypoid lesion 5 x3 centimeteres involving the
posterior lip of cervix (Fig 1). The uterus, ovaries, tubes and the
parametrial soft tissue were normal. Histological examination of the
cervical polyp showed circumscribed nodules of large lymphoid cells
with hyperchromatic oval to round nuclei with prominent nucleoli (Fig
2 & 3). Few mitotic figures were present. Periphery showed small
to medium sized lymphocytes. There was also chronic cervicitis with
squamous metaplasia of the endocervical mucosa. Depending upon the histopathological
findings, a diagnosis of Non-Hodgkin's Lymphoma, diffuse large B cell
type was made. It was staged as IA-E by Ann Arbor Staging. Immunostaining
revealed majority of the large cells were positive for B cell lineage
CD20 and CD45 (LCA) (Fig 4 & 5) for membranous and cytoplasmic positivity
and negative for CD3, cytokeratin.
Primary malignant lymphoma of the female
genital tract is rare.
Review of the literature suggests that NHL of cervix affects one in
753 cases of non Hodgkin’s lymphoma. One in 175 female
extranodal lymphomas is likely to originate in the vagina, uterus,
or cervix.[2] Crisp et al reviewed cases from three hospitals
for 1969-1979 and identified only six cases of primary pelvic NHL, including
one ovarian, one cervical and three retroperitoneal tumors.[3] Charleton et al, found thirteen cases of malignant lymphomas of the
female genital tract, other than the ovary among 9500 lymphomas in women,
a prevalence of 0.14%.[4] Although all are uncommon, malignant
lymphomas is more common in cervix than corpus of the uterus.
A lymphoid proliferation of the cervix is a common
finding often associated with chronic cervicitis. However, nodular or
diffuse inflammatory reactions containing large lymphoid cells within
an indolent clinical setting pose a problem of differential diagnosis
from lymphomatous processes. Careful high power examination in the lower
genital tract is necessary to avoid a diagnostic error. Clinical presence
of a mass lesion and deep invasion of monomorphic lymphoid-like cells
are the main morphological criteria distinguishing malignant lymphoma
from lymphoma-like lesions and immunohistochemical staining clearly
helps pathologists in their attempts at differential diagnosis.
About 33% of NHL arise in tissues other than lymph
nodes, spleen, Waldeyer’s ring and thymus and these are referred as
primary extra nodal lymphomas.[5] Cervical lymphoma presents
either as primary or systemic involvement. In our study only cervix
was involved. The important point is that the lymphomas are present
as initial presentation in the female genital tract or extra cervical
involvement, the diagnosis can never be achieved clinically. The majority
of the NHLs arising from the uterine cervix is represented by high grade
lymphomas. Diffuse large B cell lymphoma is the most common subtype
of NHL, but other subtypes have been described, including follicular
lymphomas. Immunohistochemical stains are invaluable in aiding the distinction
of many of these entities. In our case, the tumor was positive for CD
20 and common leucocyte antigen (LCA) and negative for CD 3 and cytokeratin.
The incidence of non-Hodgkin’s lymphoma, especially
extra nodal lymphoma, has increased in recent decades. The etiology
of NHL has been hypothesized to include infectious agents
such as the human immunodeficiency virus (HIV), immunosuppressive
therapies, environmental exposures to pesticides and pollutants,
and improved diagnostic techniques.[6] Our case was not associated
with HIV.
Lymphomas of the cervix generally
occur in the fifth decade with a wide age range of 20 to 80
years. Benign and malignant cervical processes often have a polypoidal
appearance, endocervical mucosa polyp represents the most common in
this site. Abnormal vaginal bleeding is the
second most common presenting symptom.[7] Other presenting
complaints include vaginal discharge, dyspareunia or pelvic
pain. B symptoms like fever, weight loss and peripheral lymphadenopathy
was not a feature in our case.
The cervical cytology
in these patients with lymphoma is negative, which is probably due to
the fact that most lymphomas in this location are subepithelial
unless there is ulceration.[8] In such cases, this type of
abnormality is commonly underdiagnosed.
Frequent misdiagnosis in cervical lymphomas include
sarcoma, poorly differentiated carcinoma, neuroendocrine tumors and
chronic inflammation.[4] Lymphomas involving the extranodal
lymphomas are staged by lymphoma staging classification i.e. Ann Arbor
classification. The patient fulfilled the criteria of stage 1 disease.
Cervical lymphoma generally has a good
prognosis as compared to nodal lymphomas, with an overall median survival
of 4 years. Extent of disease, size of primary tumour and the type of
lymphoma are significant prognostic features. Patients with advanced-stage
diffuse large B-cell lymphoma are primarily treated with chemotherapy.
Radiotherapy is considered, if there is bulky disease at presentation.[9]
Although primary non Hodgkin’s lymphoma
is rare, the disease may be encountered more frequently as the incidence
of extranodal non-Hodgkin’s Lymphoma is increasing. Cervical cytology
may be undiagnostic in such cases. Therefore, Clinicians and pathologists
should be aware of this diagnosis in patients presenting with abnormal
vaginal bleeding and negative papaniculaou test and to include cervical
lymphomas in the differential diagnosis of gynaecological cancers.
- John CK et al. Clinicopathologic features
of six cases of primary cervical lymphoma. American Journal of Obstetrics
and Gynecology. 2005;193(3 Pt 1):866-872.
- Grace A, O’Connell N, Byrne P, et al. Malignant
lymphoma of the cervix: An unusual presentation and a rare disease. Eur J Gynecol Oncol.
1999;20:26-28.
- Crisp WE, Surwit EA, Grogan TM, Freedman MF. Malignant
pelvic lymphoma. Am J Obstet Gynaecol. 1982;143:69-73.
- Charlton I, Norris J, King FM. Malignant reticuloendothelial
disease involving the ovary as a primary manifestation. Cancer. 1974;34:397-407.
- Moller MB, Pederson NT, Christenensen BE. Diffude
large B cell lymphoma: clinical implications of extranodal versus nodal
presentation- a population based study of 1575 cases. Br J Haematol 2004;124:151-159.
- Trenhaile TR, Killackey MA. Primary
pelvic Non-Hodgkin’s Lymphoma.
Obstetrics and Gynecology. 2001;97:717-720.
- Aozasa K, Saeki K, Ohasawa M et al. Malignant
lymphoma of the uterus: Report of seven cases with immunohistochemical
study. Cancer. 1993;72:1959-1964.
- King J, Elkhalifa M, Michael C. Malignant lymphoma
identified on cervical cytologic smear with immunophenotypic analysis.
Acta Cytologica 1996;40:1228.
- Stroh EL, Besa P, Cox D et al. Treatment
of patients with lymphomas of the uterus or cervix with combination
chemotherapy and radiation therapy. Cancer. 1995;75:2392-2399.
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