CASE REPORTS
Primary histiocytic sarcoma of the uterine cervix: an extremely rare entity
FARIBA BINESH1, MOJGAN KARIMI-ZARCHI2, MOHAMMAD REZA VAHIDFAR3, ZAHRA KARGAR HADGIABADI1
1
Department of Pathology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
Department of Obstetrics and Gynaecology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
3
Medical Oncologist, Yazd, Iran
2
Objective. Histiocytic sarcoma (HS) is an extraordinary rare tumor and it has an offensive
clinical course. HS of the uterine cervix is a far uncommon tumor with just a few cases described so
far. Here we presented a case of primary HS of the uterine cervix in a 62-year-old female initially
misdiagnosed as large cell non-keratinizing squamous cell carcinoma. To the best of our knowledge,
this is the first reported case of HS of the uterine cervix from Iran.
Case report. The patient presented with post-menopausal vaginal bleeding. Further evaluations
revealed the presence of a relatively large cervical mass that was sampled. Under the preliminary
diagnosis of large cell non-keratinizing squamous cell carcinoma, the patient underwent radical
surgery. Postoperative pathological examination confirmed the diagnosis of HS of the uterine cervix.
The patient received chemotherapy.
Conclusion. Clinical presentations of HS of the uterine cervix are very similar to more common
cervical tumors; as a result its diagnosis relies on immunohistochemical methods. A correct diagnose
could lead to the proper and timely treatment.
Keywords: Histiocytic sarcoma, Uterine cervix.
INTRODUCTION
CASE REPORT
Histiocytic sarcoma (HS), previously defined
as true histiocytic lymphoma is an uncommon
offensive tumor. It comprises less than 1% of all
non Hodgkin lymphomas [1]. Histiocytic sarcoma
is designated as a malignant tumor consisting of cells
with some characteristic features of normal histiocytes [2]. It is identified as one of six subgroups of
dendritic lineage tumors. Dendritic lineage tumors
comprise follicular dendritic cell sarcoma, interdigitating cell sarcoma, Langerhans cell sarcoma,
Langerhans cell histiocytosis, and dendritic cell
sarcoma NOS [3]. Nowadays the word “true histiocytic
lymphoma” is equivalent to histiocytic sarcoma.
Majority of the patients of HS are adult males. The
most common primary sites of involvement are
lymph nodes, skin and gastrointestinal tract [4].
Cervical HS is extremely rare. Therefore diagnosis
of HS is based on morphologic aspects and exclusion
of other similar neoplasms by immunohistochemistry
(IHC) methods. Due to miss of knowledge of such
an uncommon entity of ten these cases usually
undergo a wide surgical excision. Here we present
a case of primary HS of the uterine cervix in a
62-year-old female, initially misdiagnosed as nonkeratinizing squamous cell carcinoma.
A 62 year-old multiparous lady presented at
another hospital with complaint of post-menopausal
vaginal bleeding. Pelvic examination revealed a
relatively large uterine cervical mass which was
biopsied. The pathology report was a large cell nonkeratinizing squamous cell carcinoma. The patient
subsequently referred to Department of Obstetrics
and Gynaecology in our Hospital. Her pelvic
examination showed a smooth cervical mass of
about 9×5×3cm. Laboratory results were unremarkable. Then total abdominal hysterectomy and
bilateral salpingoophorectomy were performed. The
specimen was sent to the Department of Pathology
for histological evaluation. Macroscopically, the
specimen consisted of a cervix, uterus, and bilateral
tubo-ovaries. The uterus measured 5 cm×4.5 cm×3 cm.
The cervix contained an irregular pinkish-grey
mass with a diameter of 6 cm. The endometrium
was thin and unremarkable grossly and an intramural
1 cm leiomyoma was also present in the myometrium. Microscopically, the wall of the cervix
was infiltrated by malignant cells, but the squamous
epithelium and endocervical glands were intact.
The tumor cells were relatively large and had
pleomorphic nuclei with fine chromatin and bizarre
ROM. J. INTERN. MED., 2017, 55, 1, 53–56
DOI: 10.1515/rjim-2016-0051
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Fariba Binesh et al.
silhouettes with eminent nucleoli. The cytoplasm
was abundant and eosinophilic. There were foci of
tumor necrosis. Immunohistochemically, the neoplastic
cells expressed CD45, CD68 but were negative for
CD20, HMB45, S100 and CKAE1/AE3. The
histological and immunohistochemical results were
compatible with HS arising in the cervix. The
endometrium and bilateral tuboovaries were not
involved. Review of outside slides related to previous
cervical biopsy was done and confirmed the
diagnosis of HS.
She was referred for further management of
the disease to the hemato-oncologist. Chest radiography and abdominal ultrasonography were unremarkable. Contrast-enhanced CT of the neck, thorax
and abdomen were performed. No pathological
lymphadenopathy was noted. As a result the final
diagnosis was primary HS of the uterine cervix.
She received 6 cycles of chemotherapy (CHOP).
Radiotherapy was done in her hometown. The patient
status is stationary.
DISCUSSION
Mathe et al. explained histiocytic sarcoma for
the first time in 1970 [5]. HS is a rare tumor of
obscure cause [1]. HS most often occurs at a mean
age of 46years years [6]. This disease is usually
seen in males [1]. Our patient was a 62-year-old
lady. Clinical presentation varies from solitary
lesion to widely spread disease [7]. It frequently
involves the gastrointestinal tract, the skin, and lymph
nodes. In fact enlarged lymph node is the most
common exhibition. Salivary gland, lung, liver,
central nervous system, bone marrow and thyroid
are other sites of involvement [8]. Primary cervical
2
HS is an extremely uncommon diagnosis. Patients
usually present with abnormal vaginal bleeding,
abdominal mass or pelvic pain. The tumor presents
as a quickly growing lump involving the uterine
cervix. Microscopically, the tumor is composed by
proliferation of large round to ovoid cells which are
non-cohesive [9]. The correct diagnose is rests on
the confirmation of the histiocytic nature and the
exclusion of other entities such as diffuse large cell
lymphoma, anaplastic large cell lymphoma, large
cell carcinoma and melanoma [10]. Final differentiations can be made by immunohistochemistry
(IHC) methods. Immunohistochemically, histiocytic
sarcoma is identified by the expression of CD68,
lysozyme, CD11c, and CD14 in addition to the lack
of expression of B-cell, T cell, myeloid and
follicular dendritic cell markers. It is alleged that
CD163 and CD68 are the best markers for diagnosis
[7]. In our cases, the tumor cells showed the
histiocytic markers by IHC. Radiological imaging
especially MRI is useful for staging purposes.
Since most of these neoplasms have a solid yet
smooth surface, the diagnosis usually relies on
resected surgical specimens. The HS usually diagnosed
at an advanced clinical stage, so response to
therapy (either radiation or chemotherapy) is poor
[11]. However, localized, early stage disease has a
good prognosis [6]. Our case completely met these
criteria. Principal treatment of HS consists of
surgery with negative surgical margins, combined
with radiation therapy. Chemotherapy has been
added to previous therapies [12]. The clinical
course of patients with HS is variable. Some
correlation exists between stage and prognosis.
Some authors have suggested that advanced stages
correlate with poorer prognosis. Patients with
localized disease may have long survival [6, 11].
Figure 1. Section shows large non-cohesive highly pleomorphic tumor cells (H&E × 40).
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Histiocytic sarcoma of the uterine cervix
55
A-CD45
B-CD68
C-CD20
D-pancytokeratin
Figure 2 (A-CD45, B-CD68, C-CD20, D-pancytokeratin). Tumor cells were positive for CD45
and CD68 and were negative for CD20 and pancytokeratin (IHC stain).
In conclusion, cervical HS is a rare entity. Its
clinical presentations are very similar to more
common cervical tumors. As a result, clinical
suspicion with well planned diagnostic procedures
helps us diagnose this uncommon tumor. Diagnosis
of HS relies mainly on morphology and immunohistochemical methods. A correct diagnosis could
lead to the proper and timely treatment.
Acknowledgments. The authors are indebted to the staff of the
Pathology Unit at Shahid Sadoughi Hospital in Yazd, Iran,
especially Miss Fatemeh Pourhosseini and Mr Mohammad Ali
Didehban for their cooperation in this study.
Conflicts of interest. The authors confirm that there are no
known conflicts of interest associated with this publication and
there has been no significant financial support for this work
that could have influenced its outcome.
Obiectiv. Sarcomul histiocitar (HS) este o neoplazie extrem de rară.
HS localizat la nivelul cervicului uterin este o tumoare neobişnuită, fiind puţine
cazuri descrise în literatură până acum. Este prezentat cazul unei paciente de
62 de ani care a fost iniţial diagnosticată cu carcinom scuamos cu celulă mare
nekeratinizată. Este, de altfel, primul caz din Iran raportat cu asemenea patologie.
Studiu de caz. Pacienta s-a prezentat cu metroragie. La examenul fizic s-a
observat o masă tumorală. Sub diagnosticul preliminar de carcinom scuamos cu
celulă mare nekeratinizată pacienta a suferit tratament chirurgical radical.
Analiza piesei chirurgicale a demonstrat diagnosticul de HS a cervixului. Pacienta
a primit ulterior chimioterapie.
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Fariba Binesh et al.
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Concluzii. Prezentarea clinică a fost similară cu alte tumori cervicale
uterine ce sunt mult mai frecvente. Diagnosticul se poate realiza cu certitudine
prin metode imunohistochimice.
Correspondence to: Fariba Binesh, MD
Department of Pathology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran
E-mail: [email protected]
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Received July 21, 2016
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