Case reports

1992, The British Journal of Radiology, 65, 170-172

Case reports Magnetic resonance imaging of peritoneal leiomyosarcomatosis By Robert W. Kerslake, MRCP, FRCR and Brian S. Worthington, BSc, FRCR Sub-Department of Academic Radiology, University Hospital, Nottingham, UK

{Received 2 May 1991, accepted 13 June 1991)

Keywords: Leiomyosarcoma, Magnetic resonance imaging, Peritoneal tumour

Diffuse peritoneal and omental metastatic disease is a well recognized phenomenon and usually results from primary gastrointestinal tract or epithelial ovarian tumours. Leiomyosarcoma, an uncommon malignant neoplasm of smooth muscle origin that tends to arise in the genitourinary tract, retroperitoneum, alimentary tract or soft tissues, only infrequently gives rise to peritoneal or omental metastases. We report a patient with a primary high-grade leiomyosarcoma of the uterus, who subsequently developed diffuse peritoneal metastatic disease, demonstrated by magnetic resonance imaging (MRI) and confirmed surgically. These unusual MRI appearances do not appear to have been reported previously. Case report A previously well 43-year-old woman presented with polymenorrhagia and was found clinically to have a uterus enlarged to 12-14 weeks' gestational size. She underwent a routine hysterectomy, and at laparotomy an enlarged fibroid uterus (15 cm x 9 cm x 7 cm) was removed and a right haemosalpinx resected. The ovaries were normal and were conserved. HistoJogical examination of the resected uterus showed a highgrade (mitotic count of more than 10/10 high-power field) leiomyosarcoma in a partially necrotic pedunculated fibroid of 5 cm diameter situated within the upper uterine cavity. There were several foci of myometrial invasion, the deepest extending to 0.3 cm from the serosal surface. Some regions of hyalinization and necrosis were present. Islands of tumour abutted but were not invading thin-walled blood vessels. The histology of the right fallopian tube showed chronic salpingitis only. No additional treatment was given at that time. At follow-up 4 months later she was found to have a large abdominopelvic mass, which was considered to represent either recurrent leiomyosarcoma or possibly a primary ovarian neoplasm. MRI was carried out on a 0.15 T resistive system using sagittal SE 2000/80, sagittal SE 1840/60, axial SE 500/40 and coronal IR 1660/400/40 sequences. This showed the pelvis and lower abdominal cavity to be filled with multiple well defined spherical masses that, in some regions, formed conglomerate masses of up to 10 cm diameter (Fig. 1). There was loss of tissue planes against adjacent structures though no frank invasion was identified. The masses were predominantly of intermediate to low

Address correspondence to Dr R. W. Kerslake, MRI Unit, University Hospital, Nottingham NG7 2UH, UK.

170

signal intensity on all pulse sequences, similar, though not identical, to that of striated muscle. Some lesions displayed centrally increased signal intensity on r2-weighted sequences and reduced signal intensity on the IR sequence. There was a small amount of ascitic fluid. No lymph node enlargement was identified. A further laparotomy was undertaken, which confirmed the abdomen and pelvis to be full of rounded rubbery tumour masses of up to 10 cm diameter. There were numerous large vessels supplying these areas and the omentum was adherent to much of the tumour. A small quantity of ascitic fluid was present. Resection was impossible, but several tumour nodules were excised and histological examination confirmed the presence of leiomyosarcoma of similar morphology to the primary tumour. There was no evidence of ovarian malignancy. Palliative chemotherapy using the CYVADIC regime (cyclophosphamide, vincristine, adriamycin, and DTIC) was commenced. Discussion

Uterine leiomyosarcoma is a rare malignancy and the diagnosis often remains unsuspected up to the time of histological examination, as exemplified by this case. In uteri excised because of the presence of symptomatic leiomyomas, the incidence of leiomyosarcoma is of the order of 0.7% (Lawler et al, 1990). The prognosis is generally poor (Bartisch et al, 1968) but appears better when there is no macroscopic tumour spread beyond the pseudocapsule of gross vascular invasion (Gudgeon, 1968). Both of these apparently better prognostic features were present in the case discussed. MRI is an excellent technique for imaging uterine leiomyomas and is capable of accurately demonstrating their number, size, location and the presence and extent of degeneration (Hricak et al, 1986; Kerslake & Worthington, 1990). The prospective diagnosis of sarcomatous degeneration within uterine leiomyomas has not, to our knowledge, been reported and in our experience has not been possible (Kerslake & Worthington, 1990). In a recent report, dissemination of leiomyosarcoma within the peritoneal cavity was observed in 14 out of 118 patients with tumours arising from a variety of primary sites. Of the 19 patients with a primary uterine leiomyosarcoma, only two had peritoneal metastases (McLeod et al, 1984). Peritoneal spread may occur The British Journal of Radiology, February 1992

Case reports

(a) Figure 1. (a) Parasagittal SE 2000/80 and (b) coronal IR 1660/400 images. Multiple discrete and conglomerate tumour masses (curved open arrow) are present within the pelvis and lower abdominal cavity. They are of intermediate to low signal intensity but some show central hypointensity on the IR sequence (fine arrow). A small volume of ascites is present (bold arrow).

directly through the mesentery, by embolic metastases or by intraperitoneal seeding via ascitic fluid. Computed tomography (CT) has been found useful in the detection of peritoneal and serosal deposits of leiomyosarcoma (McLeod et al, 1984; Choi et al, 1990). The gross morphological appearances on CT of central lowattenuation changes within large soft-tissue masses correlate well with the central necrosis or cyst formation seen on pathological examination of high-grade tumours (Choi et al, 1990). Ascites has been reported to occur in 50% of patients with peritoneal leiomyosarcomatosis in one series (McLeod et al, 1984) but in none of another (Choi et al, 1990). The presence of multiple peritoneal tumour masses in the absence of ascites is relatively unusual with advanced ovarian or gastrointestinal malignancies and is considered by some authors to favour a diagnosis of either peritoneal leiomyosarcomatosis (Choi et al, 1990) or the apparently benign condition of leiomyomatosis peritonealis disseminata (LPD) (Renigers et al, 1985). There is some debate as to the benign nature of LPD as patients with multiple peritoneal deposits of low-grade leiomyosarcoma may present very similar gross appearances. The differential diagnosis of multiple peritoneal masses includes metastatic ovarian or gastrointestinal neoplasm, lymphoma, leiomyosarcoma, other sarcomas and LPD. In this particular case, the question of an ovarian malignancy had been raised clinically. Although the ovaries were not positively identified by MRI, the very well defined spherical masses of predominantly low Vol. 65, No. 770

signal intensity would be atypical for either primary or metastic ovarian neoplasm. Primary ovarian carcinoma of predominantly low signal intensity on r2-weighted images has not been reported (Weinreb et al, 1990). Recurrent or residual ovarian carcinoma is typically of very high signal intensity when heavily r2-weighted or STIR sequences are employed. Using these sequences, even small-volume recurrences may be detected owing to their conspicuity against a background of suppressed signal from bowel contents and pelvic fat (Kerslake et al, 1990). The enhanced vascular supply encountered at surgery was not apparent from the MR images, even in retrospect. In all other respects, however, MRI gave an accurate depiction of the gross morphological features that were encountered at operation. The presence of poorly defined central regions of hyperintensity on r2-weighted images correlated with regions of necrosis and cyst formation shown pathologically, in keeping with a high-grade leiomyosarcoma. In this case the antecedent history of a primary uterine leiomyosarcoma enabled a specific diagnosis to be suggested following MRI. However, peritoneal leiomyosarcomatosis may occur in the absence of a known primary tumour. Thus, when MRI demonstrates multiple, spherical, well defined peritoneal and mesenteric masses with central heterogeneity and with or without small volumes of ascites, peritoneal leiomyosarcomatosis should be considered amongst the differential diagnosis, even in the absence of a known primary tumour. 171

Case reports

1992, The British Journal of Radiology, 65, 172-175 detection of recurrent Radiology, 177, 242.

ovarian

carcinoma

(Abstract).

Acknowledgments Dr R. W. Kerslake is supported by the Department of Health and the Medical Research Council.

KERSLAKE, R. W. & WORTHINGTON,

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manifestations of peritoneal leiomyosarcomatosis. American Journal of Roentgenology, 155, 799-801. GUDGEON, D. H., 1968. Leiomyosarcoma of the uterus. Obstetrics & Gynecology, 32, 96-100. HRICAK, H., TSCHOLAKOFF, D., HEINRICHS, L., FISHER, M. R., DOOMS, G. C , REINHOLD, C. & JAFFE, R. B., 1986. Uterine

leiomyomas: correlation of MR, histopathological findings and symptoms. Radiology, 158, 385-391. KERSLAKE, R. W., PERKINS, A. C , SYMONDS, I., POWELL, M. C , SCOTT, I. V., WASTIE, M. L., SYMONDS, E. M. &

WORTHINGTON, B. S., 1990. Prospective study of MR imaging and immunoscintigraphy using OC-125 in the

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leiomyomas: the role of MRI in informing management decisions (Abstract). Clinical Radiology, 42, 379. B., 1990. Leiomyosarcoma in a series of hysterectomies performed for presumed leiomyomas. American Journal of Obstetrics and Gynecology, 162, 968-974. MCLEOD,

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The use of 123 l-Hippuran renography in the management of synchronous bilateral upper urinary tract transitional cell carcinomata By D. P. S. Sandhu, FRCS, *P. Sambrook, FRCR and N. J . R. George, M D , FRCS Departments of Urology and *Radiology, University Hospital of South Manchester, West Didsbury, Manchester, UK (Received 15 April 1991 and in revised form 8 July 1991, accepted 20 September 1991)

Keywords: Ureteric tumours,

123

I-Hippuran renogram, Ureteroscopy

distal ureter (Fig. Ib). Serum creatinine was normal. A Bilateral simuitaneous/y occurring ma/ignant tumours (he 123 I-Hippuran renogram demonstrated the obstructed left of the upper tracts are very rare, and present the difficult problem of preservation of renal function and kidney to contribute 36% of global function. At initial cystoscopic assessment the urethra, bladder and adequate tumour clearance. We report such a case and ureteric orifices were normal. Ureteroscopy was not attempted discuss the difficulties and value of the modern methods on the left as the developing urographic appearances were 123 of management, including ureteroscopy and I- considered to be pathognomonic of ureteric tumour (Fig. lb). Hippuran gamma camera renography with creatinine Right ureteroscopy (9.5 FG) was successful in reaching the clearance to assess total and split renal function and pelvis and tumour-like fronds were seen in the upper pole, but adequate biopsy material could not be obtained. Right retrorecovery. Case report A 62-year-old housewife was referred with right loin and back ache associated with haematuria. Two years earlier she had been investigated for right loin pain without haematuria and had normal excretion urography at that time. A repeat urogram revealed a radiolucent filling defect in the upper pole calyx of the right kidney (Fig. la), with hydronephrosis and hydroureter on the left side resulting from probable tumour in Address correspondence to D. P. S. Sandhu, FRCS, Department of Urology, City Hospital, Nottingham NG5 1PB, UK.

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grade ureteropyelogram appeared to confirm a right pole tumour, although pelvic urine draining from the catheter surprisingly did not subsequently provide positive cytological proof of malignancy. Following external-beam radiation to the left hemipelvis, the distal left ureter was excised (Fig. 2b), and reimplanted into the bladder using the psoas hitch procedure. Intraoperative frozen section confirmed that the distal end of the remaining ureter was tumour free. A subsequent histological section confirmed a Grade 2 WHO papillary transitional cell carcinoma without evidence of invasion into the ureteric wall. Following surgery the total glomerular filtration rate (GFR) as measured by creatinine clearance improved to 95 ml/min after 7 days, with split renal function tests showing that the

The British Journal of Radiology, February 1992

Magnetic resonance imaging of peritoneal leiomyosarcomatosis.

Case reports 1992, The British Journal of Radiology, 65, 170-172 Case reports Magnetic resonance imaging of peritoneal leiomyosarcomatosis By Robert...
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