CASE 14692 Published on 27.06.2017

Epithelioid sarcoma of the uterine cervix

Section

Genital (female) imaging

Case Type

Clinical Cases

Authors

Tonolini Massimo, MD.

"Luigi Sacco" University Hospital,
Radiology Department;
Via G.B. Grassi 74
20157 Milan, Italy;
Email:mtonolini@sirm.org
Patient

52 years, female

Categories
Area of Interest Genital / Reproductive system female ; Imaging Technique MR
Clinical History
G1P1 postmenopausal female patient with history of thyroidectomy for well-differentiated carcinoma, on hormonal replacement therapy, suffering from vaginal bloody discharge for a week. Gynaecological visit revealed enlarged uterine cervix with hard consistency, lobulated mass oozing blood and causing obliteration of vaginal fornices. Remaining physical examination and laboratory tests within normal limits.
Imaging Findings
Initially, MRI (Fig. 1) showed that the lower third of the uterine cervix was affected by a heterogeneously solid mass with T1-intermediate, moderately high T2-weighted signal intensity which measured 52x36x30 mm in size. The lesion showed a predominantly exophytic growth, obliterated the vaginal fornices, and disrupted the outer stromal ring suggesting parametrial infiltration.
High b-value diffusion-weighted images depicted the true extent and well-demarcated, lobulated contours of the cervical mass, characterised by restricted diffusion with corresponding low apparent diffusion coefficient values. Peritoneal cul-de-sac effusion, pelvic lymphadenopathies, abnormalities of uterine body and adnexa were absent.
Biopsy results unexpectedly disclosed an unusual, necrotic and ulcerated malignant mesenchymal proliferation consistent with high-grade epithelioid sarcoma, histologically formed by atypical cells with clear cytoplasm, marked nuclear atypias (mitotic index 18/10) staining positive for vimentin, CD10, cytokeratines and S100.
Following radiotherapy, three months later MRI follow-up showed stable size and thickness of cervical mass compared to initial MRI, without appearance of lymphadenopathies and peritoneal effusion.
Discussion
Compared to the usual squamous histology, uterine cervix sarcomas (UCS) are distinctly rare and account for less than 1% of all cervical malignancies. UCS encompass a heterogeneous spectrum of rare mesenchymal tumours with variable histotypes including muscle, adipose, fibroblastic, histiocytic, nerve sheath and neuroectodermal components along with undifferentiated or uncertain tumours. Whereas rhabdomyosarcoma is typically encountered in young age (median age 18 years), other forms of UCS usually occur after reproductive age. Most UCS manifest with vaginal bleeding which may be profuse and can cause anaemia, and the diagnosis is obtained through biopsy [1-3].
Currently established as the best modality for pre- and post-treatment imaging of gynaecologic tumours, MRI consistently evaluates lesion volume, pattern of spread and nodal metastatic involvement of cervical tumours: these are best depicted in multiplanar high-resolution T2-weighted images as intermediate-to-high T2-weighted signal intensity tissue which is easily distinguishable from the hypointense cervical stroma [4-6].
Owing to their rarity, radiological literature lacks information on MRI appearances of UCS, which—as in the hereby presented patient—tend to present as bulky masses, sometimes with predominantly exophytic growth, which are indistinguishable from locally advanced squamous carcinomas [1-3].
Diffusion-weighted imaging (DWI) may offer additional information: albeit apparent diffusion coefficient (ADC) values considerably vary among different scanners and sequences, cervical tumours show visually restricted diffusion and low ADC (median 1.09±0.20x10-3 mm2/sec) compared to normal cervical tissue (1.79±0.24x10-3 mm2/sec). Therefore DWI may prove helpful in the detection of neoplastic tissue in the uterine cervix, and for staging purposes as it better evaluates tumour size, contour and extrauterine spread, while avoiding the possible size overestimation from oedema or inflammation on conventional MRI images. Albeit ADC values often overlap, DWI may yield some information about histologic features: as in this case, lower ADC values may help to distinguish aggressive, undifferentiated tumours from well- or moderately differentiated ones reflecting the higher tumour cellular density [4-7].
Due to the small number of patients, histological heterogeneity and variable biological behaviour, optimal treatment of UCS is uncertain: most patients are treated with either primary radiotherapy or radical hysterectomy and bilateral salpingo-oophorectomy, with or without adjuvant radio-chemotherapy. Albeit in the past UCS were generally considered aggressive neoplasms associated with dismal prognosis, current multimodal treatment may sometimes result in improved survival [1-3].
Differential Diagnosis List
Epithelioid sarcoma of the uterine cervix
Squamous cervical carcinoma
Cervical adenocarcinoma
Final Diagnosis
Epithelioid sarcoma of the uterine cervix
Case information
URL: https://www.eurorad.org/case/14692
DOI: 10.1594/EURORAD/CASE.14692
ISSN: 1563-4086
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