|
|
||||||||
Case Report |
ur Toprak1
ref Pa
ao
lu1 All authors: Department of Radiology, Ankara Numune Education and Research Hospital, Sihhiye, Ankara TR-06100, Turkey.
Received April 16, 2003;
accepted after revision October 21, 2003.
Address correspondence to U. Toprak.
Introduction
|
|
|---|
|
|
|---|
Transabdominal sonography revealed a left cornual, myometrial complex cystic mass with smooth borders that contained nodular solid areas and cystic spaces with thick septations (Fig. 1A). Additionally, multiple small myometrial nodules with hypoechoic peripheral rims were shown (Fig. 1B), some of which had centrally located cystic areas. Several myometrial lesions were compressing the endometrium, but they had no clear association with the endometrium. A left adnexal and extraovarian cystic lesion with fine septations was displacing the left ovary. A simple ovarian cyst was also present in the left ovary.
|
|
Contrast-enhanced CT displayed heterogeneous enhancement of the solid components and septations of the cornual complex cystic lesion and similar enhancements in the small nodules (Fig. 1C).
|
After unenhanced T1-weighted MRI and T2-weighted MRI, contrast-enhanced T1-weighted MRI was performed. Solid parts, septations of left cornual cystic lesion, and small myometrial nodules were isointense with the myometrium on unenhanced T1-weighted images and hyperintense on T2-weighted images. Heterogeneous enhancement was also present after gadolinium administration (Fig. 1D). The cystic component of the cornual lesion had higher signal intensity than urine on T1- and T2-weighted images. Some of the small myometrial nodules had hypointense centers on both sequences. MRI revealed no clear association with endometrium (Figs. 1D and 1E). A diffuse permeation from adjacent myometrium leading to diffuse thickening at the right fallopian wall was present, which was sustained throughout the broad and round ligaments and extended to the peritoneum. The content of the left adnexal cystic lesion displacing the left ovary was slightly more intense than the cystic lesion in the left ovary (Fig. 1F). Radiologic examination findings were suggestive of the adnexal cystic lesion's being a peritoneal inclusion cyst.
|
|
|
An endometrial curettage specimen showed no disease. Thus, total abdominal hysterectomy was performed, and study of the pathologic specimen yielded the diagnosis of a low-grade endometrial stromal sarcoma. No endometrial component was detected. However, a lymphangitic invasion of the myometrial tissue throughout the uterus, including the cervical myometrium, was present. The neoplastic process penetrated the serosa and reached the peritoneum. The left adnexal cyst was confirmed intraoperatively to be a peritoneal inclusion cyst.
|
|
|---|
Endometrial stromal sarcoma leads to the same symptoms as those of any other uterine sarcoma or endometrial carcinoma. More than half the cases occur in premenopausal women, particularly those with low-grade endometrial stromal sarcoma [1]. Endometrial curettage may not yield a significant result for low-grade endometrial stromal sarcoma because of its similarity to normal endometrial tissue [4]. Endometrial biopsy results were unremarkable in our patient, and in the radiologic studies lesions were described as completely myometrial.
Although uterine sarcomas are described as aggressive neoplasms, endometrial stromal sarcoma has a low potential for spreading. Although it is more common in patients between ages 42 and 53, low-grade endometrial stromal sarcoma tends to occur in a younger age group (mean, 39 years) [1].
Endometrial stromal sarcoma can spread to the vagina, fallopian tubes, uterine ligaments, ovaries, bladder, and ureters [4]. In our patient, diffuse myometrial permeation of the right fallopian tube extended to the broad and round ligaments and invaded the peritoneum without any interruption. Radiologic studies revealed no endometrial component, and endometrial curettage was unremarkable.
Definitive diagnosis of any uterine sarcoma based on sonography alone is not possible [5] because these kinds of tumors may have nonspecific findings such as polypoid endometrial masses, endomyometrial thickening, and adnexal masses. Accordingly, no diagnostic sonographic finding was detected in our patient.
Uterine sarcomas have no specific tomographic feature to aid in the differential diagnosis, and CT is mostly used to detect distant metastases and stage the disease. Low-density mass with necrosis is the most common pattern of uterine sarcomas [6]. In our case, heterogeneously enhanced left cornual complex mass and multiple contrast-enhanced small myometrial nodules with cystic centers were low-grade endometrial stromal sarcoma lesions.
Tumoral lesion of the endometrial stromal sarcoma is isointense relative to the myometrium on T1-weighted MRI and hyperintense on T2-weighted MRI. Furthermore, it shows heterogeneous but prominent enhancement in contrast-enhanced images because of its rich vascularity. In addition to the lesions in the form of large masses with irregular margins, multiple nodular mass formations and intramyometrial wormlike nodular extensions are frequently seen. Marginal nodular lesion is a common finding and is representative of its invasive character [7].
Leiomyomas can have variable appearances on MRI depending on cellularity and the presence of cystic degeneration, necrosis, hemorrhage, and calcification, and they are usually well demarcated [7]. Thus, radiologic differentiation could be possible before surgery. In our patient, tumoral infiltration up to the peritoneum was not compatible with leiomyoma.
It is difficult to differentiate endometrial stromal sarcoma from leiomyosarcoma even with MRI. Leiomyosarcomas have variable signal intensities on T2-weighted images, and their masses display irregular margins [2].
Endometrial cancer presents with endometrial masses that have intermediate to low signal intensity on T1-weighted images and low signal intensity on T2-weighted images, whereas signal intensity of endometrial stromal sarcoma is higher on T2-weighted images. Unlike endometrial stromal sarcoma masses, endometrial carcinoma masses are not well enhanced [4].
Adenomyosis may affect the myometrium diffusely. Unless a hemorrhagic complication supervenes, the lesion of adenomyosis is hypointense relative to the myometrium on both T1- and T2-weighted MRI [4].
Conventional treatment of endometrial stromal sarcoma is hysterectomy [4]; thus, it was performed on our patient. Chemotherapy and radiotherapy should be considered as other treatment options.
Peritoneal inclusion cyst is diagnosed when an intact ovary has a cystic appearance and often forms after the surgical procedures in the pelvis, but it is also caused by endometriosis or pelvic inflammatory disease [8]. Interestingly, endometriosis can lead to endometrial stromal sarcoma as well as to peritoneal inclusion cysts [3]. Nevertheless, neither the study of the pathologic specimen nor the history of the patient showed endometriosis.
In conclusion, endometrial stromal sarcoma should be kept in mind in the differential diagnosis of heterogeneously enhanced, complex cystic, and invasive myometrial masses, particularly among the patient group of premenopausal women.
|
|
|---|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |