Abstract
Abstract
Introduction
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Broad-ligament leiomyomas are often mistaken for other adnexal lesions that are commonly of ovarian origin. 6 Sometimes, imaging may not be able to clarify the situation—unless such a possibility is considered—with the result that patients mostly end up undergoing total abdominal hysterectomy and salpingo-oopherectomy. Ultrasonography (USG) is the commonest modality used, but it may be relatively nonspecific for differentiation of broad ligament or adnexal origins of the lesions, particularly if the ovaries are not imaged separately.
Computed tomography (CT) and magnetic resonance imaging (MRI) have been used in to evaluate pelvic pathologies—uterine, adnexal, and others—and also abdominopelvic extents of giant leiomyomas or ovarian lesions. CT has shown to be useful for showing associated lymphadenopathy, bowel displacement or involvement, ascites, and peritoneal and omental involvement. MRI is excellent for showing details of the endometrium, cervical canal, and the subserosal nature of lesions, and for evaluation of ovaries if they are visualized separately.
Leiomyomas undergo various secondary changes, such as degeneration, edema, hemorrhage, necrosis, and calcifications. Hyaline degeneration is the commonest (>50%), followed by cystic, myxoid, and red or carneous degeneration. Cystic degeneration is considered as an extreme sequela of edema in leiomyomas, and its incidence has been reported to be 4%. 6 Lipoleiomyomas are uncommon variants with macroscopic fat or microscopic foci of adipocytes. CT and MRI can identify macroscopic fat noninvasively. 7 This case report describes a 37-year-old female presenting with an abdominopelvic mass that was suspected initially of being an ovarian mass, that, however, turned out to be a broad-ligament plexiform lipoleiomyoma with cystic degeneration.
Case
A 37-year-old female presented with complaints of fullness in the lower abdomen since the past 2 years. Her menstrual cycles were regular. She had two full term normal deliveries before, with her last childbirth being 16 years ago and had also undergone tubal sterilization. This patient's family history revealed that her mother had had breast cancer.
The patient's last workup, in an outside private institution, had occurred >2 years ago. USG 2 years ago had shown a bulky uterus and a large, irregular, heterogeneously echogenic lesion with cystic areas measuring 16×9×8 cm in the right adnexal region. The left ovary was normal and right ovary was not visualized separately. MRI performed at the same time had shown a large, multilobulated, complex solid-cystic lesion in the right adnexa, suggesting either an exophytic uterine lesion (asubseroal myoma) or a right ovarian mass. Her CA-125 level was normal (30.8 U/mL).
Laparotomy had been performed 2 years prior to the current presentation, and, as the mass was very adherent to the uterus, only aspiration cytology and a biopsy of the right adnexal mass had been performed. Aspiration cytology results were negative for malignant cells and the biopsy reported leiomyomas with suspicious lymphatic elements. The patient had been counseled about the benign nature of the lesion and advised to undergo conservative management. Another MRI performed 3 months before her current admission (1.5 years since her first surgery) showed that her uterus was displaced to the left side by a large T1/T2 mixed signal intensity abdominopelvic mass, measuring 21×20×11 cm, with an incisional hernia at the umbilical region.
During the current admission, this patient had mild pallor with bilateral pitting pedal edema. On examination, her abdomen was noted to be distended with an infraumbilical longitudinal scar and a large incisional hernia. An irregular mass was felt; it was variegated in consistency, corresponding to 28–30 weeks' size with no obvious ascites. Speculum examination showed a healthy and high cervix. Her uterus was not felt separately on vaginal examination. A large mass was felt in the right fornix; the mass was firm in consistency.
USG showed a heterogeneous, mixed, solid-cystic mass in the right adnexa in continuity with the uterus, displacing the uterus to the left side. The mass was seen extending from the suprapubic and periumbilical regions up to the level of the kidneys superiorly; the mass had with poorly defined margins in the flanks laterally. Few coalescent cystic areas showed a “honeycomb” appearance (Fig. 1). The right ovary could not be visualized and the left ovary was noted to be normal.
Ultrasonographic images (longitudinal images in the right and left suprapubic regions) showing mixed solid-cystic lesion showing ‘honeycomb’ areas in continuity with displaced uterus.
Plain CT of the abdomen showed a large, well-demarcated abdominopelvic mass extending from the lower pole of the kidneys to the right adnexal level in continuity with the right body of the uterus, laterally reaching the flanks, displacing bowel loops in the area, and measuring 23×20×13 cm. The lesion was clearly separate from the bowel loops with clear cleavage of the fat plane. The lesion also showed mixed densities with peripheral hypodensities and internal, numerous, subtle hyperdense nodules corresponding to the “honeycomb” areas seen in the USG scan. No obvious fat density areas to suggest a lipoma were noted. No ureteric dilatation was noted, and there was no free fluid or para-aortic lymphadenopathy. The left ovary was seen lateral to the displaced uterus, but the right ovary could not be visualized separately in CT (Fig. 2).
Plain computed tomography axial sections
In view of these test results, either an ovarian lesion with mural nodules or a broad-ligament leiomyoma with cystic degeneration were considered as diagnostic possibilities.
To delineate the uterus and look for right ovary, MRI was performed, which showed a large T1 iso- to hypointense T2 mixed signal intensity mass showing similar extent and margins as in CT. The “honeycomb” areas showed T2 hypointensities suggesting proteinaceous or hemorrhagic contents. No T1 hyperintense fatty component was noted. The uterus was displaced to the left side and the mass appeared to be arising from the right side of the uterine body and cervix. The left ovary was noted and another ovarylike structure was also noted on the left side, antero-superior to the displaced uterine fundus, and was thought of as being the displaced right ovary (Fig. 3). The possibility of broad-ligament origin was strongly considered because of this extreme displacement of the right ovary to the left side.
Axial
The patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, and incisional hernia repair under combined spinal–epidural anesthesia. Her abdomen was opened by an infraumbilical midline longitudinal incision over the previous scar. There was a hernial sac of 6×7 cm with bowel adherent to it. Adhesiolysis was done.
This patient's anatomy was grossly distorted on the right side, with a large irregular solid-cystic mass in close relation to the body of the uterus, probably arising from the right broad ligament, measuring 22×20×15 cm with the minimal adherence of omentum anteriorly. Total excision of the mass on the right side was performed along with the ovary after opening the broad ligament, followed by hysterectomy and bilateral salpingo-oophorectomy. The surgical specimen was cystic and weighed 6 kg (Fig. 4). After excision and repair of the hernial sac, the patient's abdomen was closed in layers. Two units of blood were transfused intraoperatively. Her Postoperative period was uneventful and she was discharged on the eleventh postoperative day. She was doing well at a 6-week follow-up. Her lipid profile was within normal limits.
Intraoperative photograph
Results
The gross specimens were comprised of two large components: (1) the uterocervix, parts of bilateral tubes, the left ovary, and the broad ligament; and (2) a large solid-cystic mass with the attached right ovary, measuring 21×20×10 cm. A cut section of the large cystic mass close to the hilus of the right ovary showed multiple septations and fluid-filled loculi with partial solid and hemorrhagic areas. Cut sections of the uterocervix revealed an intramural cervical fibroid growth, measuring 6×9×5 cm, with a whorled appearance, along with tiny foci of yellowish areas. A smaller solid mass, measuring 4×4 cm, was seen in the right broad ligament close to the fundus of the uterus, and sections showed solid homogeneous tissue with a whorled appearance. The ovaries showed normal follicles.
Sections from the large cystic mass from the broad ligament showed longitudinal fascicles of spindle cells intersecting each other with admixed adipocytes, extensive areas of cystic change, and long cords and strands of cells embedded in a collagenous matrix (hyaline and plexiform changes; Fig. 5). On immunohistochemistry, spindle cells were noted to be strong and diffusely positive for smooth-muscle actin and desmin (Fig. 6). The histopathologic diagnosis was that of a plexiform lipoleiomyoma with cystic degeneration. There was also a cervical lesion with a similar tumor composed of longitudinal fascicles of spindle cells intersecting each other and admixed with lobules of adipocytes. A smaller mass adjacent to the fundus of the uterus revealed similar findings. Histopathology of the endometrium showed secretory-phase changes with normal myometrium.
Photomicrograph (H & E, 10×) showing fascicles of spindle cells intersecting each other and admixed with adipocytes in
Immunohistochemistry showing diffuse and strong positivity of smooth-muscle actin
As noted above, the patient was doing well as her 6-week follow-up and she had a normal lipid profile.
Discussion
Lipomatous or fatty tumors of the uterus have been first described by Brandfass and Everts-Suarez in 1955 8 and were classified pathologically into three types: (1) pure lipomas; (2) lipomas with other mesodermal components, such as lipoleiomyomas and angio- and fibromyolipomas; and (3) malignant liposarcomas.9,10 Lobstein as cited by Jacob et al., described an uncommon uterine lipoleiomyoma first in 1816; since then, these lesions have been reported commonly as arising from the uterus. 1 However, only 9 cases of broad ligament lipoleiomyoma have been reported in the English literature up this now, and all of them were in peri- and postmenopausal women.11–18
Adipocyte components in lipoleiomyomas may differ widely, and no criteria are defined regarding the distribution of adipocytes in the diagnosis of lipoleiomyoma. These tumors may contain microscopic foci of adipocytes resembling regular leiomyomas in gross appearance, or gross fatty changes may be seen resulting in yellow and lobulated cut surfaces. 14 The cervical tumor in the current case had a yellow cut surface grossly and high amounts of adipocytes noted on microscopy, whereas the broad-ligament tumor had only microscopic focal areas of adipocytes.
Various theories have been postulated for the presence of fat in leiomyomas, including lipomatous metaplasia of smooth-muscle cells, differentiation from embryonic adipocytes, and proliferation of nearby perivascular fat cells.8–10,19–22 Lipomatous metaplasia of smooth-muscle cells of leiomyomas is considered to be the most likely cause for development of lipoleiomyomas. 19 Cytogenetic analysis studies on uterine lipoleiomyomas have led researchers to speculate that the secondary chromosomal change involving chromosomal 5 may be responsible for the lipomatous change in leiomyomas. 20 Shintaku hypothesized the origin of lipoleiomyomas as being in two major groups according to histogenesis: (1) lipoleiomyomas caused by lipomatous metaplasia of leiomyomas; and (2) angiomyolipomas, which are choristomas similar to their renal counterparts. 21
However, in their description of 6 cases of uterine angiomyolipomas, Aung et al. showed that HMB-45, a marker for renal angiomyolipomas, was positive in only 3 cases and, in another 3 cases with angiomyolipoma-like vessels, the marker was negative. 22 These researchers also showed, using a Ki-67 labeling index, greater proliferation of fat portions of tumor were noted than the normal myometrium, suggesting that fat portions of the tumor are proliferating adipose tissue rather than fatty degeneration of muscle.
The histology of plexiform leiomyomas is distinct, with elongated cords, strands, and nests of cells embedded in a collagenous matrix. 2 A collagenous matrix is seen commonly in leiomyomas as part of hyaline degeneration. Although plexiform tumorlets of the uterus were initially considered as variants of epithelioid leiomyomas, 23 now, with molecular and cytogenetic analyses, these tumorlets have been shown to be distinct from the epithelioid leiomyomas. 24
Lipoleiomyomas have been reported mostly as arising from the uterus; the few reported cases of broad-ligament lipoleiomyoma have shown to be mimicking other adnexal or ovarian masses. Large broad-ligament leiomyomas with myxoid and cystic degeneration are confused with ovarian lesions.25–27 In USG, leiomyomas with cystic degeneration have been shown to produce a “honeycomb” appearance. 28 The present case also showed mixed solid-cystic areas with a “honeycomb” appearance. CT showed the well-demarcated margins better than USG, but could not show delineations of the right ovary. MRI showed the right ovarian displacement to the left side, which is possible only when there is broad-ligament lesion unlike other ovarian and pelvic lesions.
In the operative and gross findings, there were three lesions: (1) a large broad-ligament lesion; (2) a cervical myoma; and (3) and smaller myoma near the fundus of the uterus that were probably overshadowed by the larger lesion in imaging. This case illustrates the need to account for all lobulations or compartments in single large lesion distorting a patient's normal anatomy.
Conclusions
A large broad-ligament lesion can mimic ovarian masses and preoperative imaging and histopathologic confirmation may help in avoiding radical surgery.
Footnotes
Disclosure Statement
No competing financial conflicts of interest exist.