British Journal of Radiology (2004) 77, 701-703
© 2004 British Institute of Radiology
doi: 10.1259/bjr/31755088
Pre-operative arterial embolisation of a uterine rhabdomyosarcoma in a 14-year-old girl
P L Giacalone, MD
1
P H Deisseignet, MD
1
P Roger, MD
2
P Taourel, MD
3
H Vernet, MD
4 and
F Laffargue, MD
1
1 Oncology Unit, Department of Obstetrics and Gynaecology, Hôpital Arnaud de Villeneuve, 371 rue du Doyen Gaston Giraud, 34295 Montpellier, 2 Department of Pathology, Hôpital Lapeyronie, 371 rue du Doyen Gaston Giraud, 34295 Montpellier, 3 Department of Radiology, Hôpital Lapeyronie, 371 rue du Doyen Gaston Giraud, 34295 Montpellier and 4 Department of Vascular Radiology, Hôpital Arnaud de Villeneuve, 371 rue du Doyen Gaston Giraud, 34295 Montpellier, France
Correspondence: Pierre-Ludovic Giacalone
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Abstract
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We report a young patient suffering from a uterine rhabdomyosarcoma. Particular features of the present case are: accuracy of the tumour spread evaluation performed by MRI of the pelvis; and the use of pre-operative arterial embolisation. The present multimodal management highlights the usefulness of cooperation between surgeons and radiologists in lowering operative bleeding and finally permitting uterine conservation.
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Case report
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A 14-year-old girl presented with a 3-month history of vaginal bleeding, particularly after sexual intercourse; intermittent pelvic pain; abnormal vaginal discharge; and dyspareunia. Clinical examination revealed a necrotic mass arising in the uterine cervix and obstructing the whole upper vagina. Haemoglobin was 8.5 g dl–1. An ultrasound scan of the pelvis, performed by the abdominal route, showed a well defined mass of intermediate echogenicity in the region of the uterine cavity, having a maximal diameter of 9 cm.
MRI of the pelvis was performed in the sagittal and axial planes and confirmed the presence of a polypoid mass measuring 9 cm in length, which was pedunculated from the uterine fundus and projecting into the upper vagina through the uterine cervix. The pedicle was not easily distinguished from the normal myometrium. The mass showed uniform high signal on T1 weighted images (Figure 1
) and intermediate signal on T2 weighted images (Figure 2). A hypervascularity was suspected because of a uniformly high enhancement after gadolinium enhanced MRI. There was no evidence of infiltration of adjacent pelvic structures or regional lymphadenopathy.
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Figure 1. Sagittal T1 weighted spin echo image of the pelvis demonstrating the mass arising from the uterine fundus and projecting through the uterine cervix. There were no signs of bladder, vaginal or rectal involvement by the mass.
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Figure 2. Sagittal T2 weighted spin echo image of the pelvis demonstrating the mass. Total uterine and mass length was 10.4 cm.
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The initial biopsy of the lower pole of the mass showed features suggestive of uterine fibroma with a high necrotic component. As this pattern is rare in a young female, a second biopsy was performed and concluded in the same diagnosis.
The results of the various examinations suggested that an attempt may be made to remove the mass hysteroscopically. The rational for this decision was: first to perform a complete mass analysis to assess the histological features; second to conserve the uterus because of the patient's youth; and third, in line with using the most conservative procedure, to limit intraoperative blood loss. Therefore tumour arterial embolisation was performed the day before surgery using absorbable gelatin sponge as a temporary occlusive agent. Diagnostic angiography and embolisation were performed under local anaesthesia using the femoral approach. A pelvic angiogram identified the right and left uterine arteries and determined the extent of the tumour's vascularity by revealing the extensive network of intrinsic arteries (Figure 3 and 4). The progress of vessel occlusion during embolisation was monitored with repeated angiograms. Embolisation was stopped when the flow in the distal tumour vessels ceased (Figure 5).
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Figure 3. Pre-embolisation angiogram showing the right and left uterine arteries and the tumour intrinsic vascularity mainly arising from the left vascular pelvic system.
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The surgical tumour resection was performed by the vaginal route using a diathermy loop. The tumour did not bleed and resection was performed through the uterine cervix to the uterine fundus. As there was concern about maintaining myometrial integrity after the wide resection, a laparoscopy was performed, which demonstrated a uterine wall defect located in the right side of the fundus. A laparotomy was then performed to further analyse and then repair the defect. Uterine reconstruction was easily accomplished by laparotomy, using two-layer sutures, which resulted in complete peritonealisation of the myometrium.
Complete histological analysis showed large focies of tumour necrosis. A mixture of fibroblast-like cells with clusters of mature cells containing distinct cross-striation and a matrix containing collagen and mucoid material was demonstrated in a small part of the mass. A musculoskeletal differentiation type was evidenced by immunohistochemical investigation and confirmed an embryonic rhabdomyosarcoma.
Post-operative venous haemoglobin was 8.0 g dl–1 and the patient did well. Thoracic and abdominal tomodensitometry was normal. No bone metastases were evidenced in the bone marrow aspiration and biopsy. 2 months post-operatively, clinical examination showed a normal uterine cervix. Pelvic MRI did not show early recurrence (Figure 6), and the patient is currently undergoing adjuvant polychemotherapy.
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Figure 6. Post-operative sagittal spin echo image of the pelvis showing total recovery of the uterine cervix aspect. No sign of early recurrence were observed.
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Discussion
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We report a case of uterine rhabdomyosarcoma in a young patient whose uterus was conserved through multimodal treatment. Genital rhabdomyosarcoma is a rare tumour observed in young and middle-aged women [1, 2]. For the past 3 decades, some multicentre study groups [1, 2] have been investigating this rare entity and have been able to define prognostic factors (tumour size and histology, local spread, lymphatic or distant metastasis) [2–5] and indicate probable treatment outcomes [6–13]. It is now acknowledged that conservative uterine treatment may be attempted under certain conditions using neoadjuvant chemotherapy [6–8]. In our case, however, the initial biopsy samples used for pre-operative histology had eliminated the possibility of neoadjuvant chemotherapy to shrink tumour size before surgery.
Pelvic MRI was thus of paramount importance in determining the pre-operative patterns. The lack of adjacent tissue invasion was easily confirmed. The tumour pedicle could be clearly seen to arise from the uterine fundus, which is not the most frequent presentation as genital rhabdomyosarcomas most commonly develop from the vagina and occasionally from the cervix [1, 3]. Last, this technique revealed the high vascular component of the tumour. The ensemble of these MRI results was critical to our decision to avoid wide radical surgery. The pedicle location and size allowed us to attempt conservative uterine treatment by the vaginal route, even though laparotomy and uterine reconstruction were ultimately needed. As we had clear evidence of the vascular component, we were able to choose pre-operative embolisation to limit operative bleeding and facilitate uterine reconstruction.
We chose to use a temporary occlusive agent to allow rapid vascular revascularization, thereby reducing the risk of ischaemic injury to the uterus. Furthermore, the conservation of uterine vascularization may be crucial for adjuvant chemotherapy as it has been shown that chemotherapy requires normal tissue vascularization to be effective.
Accurate management using pre-operative MRI to evaluate local spread, and vascular embolisation strongly highlight the advantages of cooperation between surgeons and radiologists. This case report is an example of what our and other's experience [14] has consistently shown us: a multimodal approach to diagnosis and treatment best serves the patients.
Received for publication July 2, 2002.
Accepted for publication November 4, 2002.
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References
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- Copeland LJ, Gershenson DM, Saul PB, Sneige N, Stringer CA, Edwards CL. Sarcoma botryoides of the female genital tract. Obstet Gynecol 1985;66:262–6.[Medline]
- Raney RB Jr, Crist W, Hays D, Newton W, Ruyman F, Teff M, et al. Soft tissue sarcoma of the perineal region in childhood. A report from the Intergroup Rhabdomyosarcoma Study I and II, 1972 through 1984. Cancer 1990;65:2787–92.[CrossRef][Medline]
- Hays DM, Shimada H, Raney RB Jr, Tefft M, Newton W, Crist WM, et al. Clinical staging and treatment results in rhabdomyosarcoma of the female genital tract among children and adolescents. Cancer 1988;61:1893–903.[CrossRef][Medline]
- Raney RB, Anderson JR, Barr FG, Donaldson SS, Pappo AS, Qualman SJ, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 2001;23:215–20.[CrossRef][Medline]
- Crist WM, Gamsey L, Beltangady MS, Gehan E, Ruymann F, Webber B, et al. Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee. J Clin Oncol 1990;8:443–52.[Abstract]
- Martelli H, Oberlin O, Rey A, Godzinski J, Spicer RD, Bouvet N, et al. Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: A report from the Study Committee of the International Society of Pediatric Oncology. J Clin Oncol 1999;17:2117–22.[Abstract/Free Full Text]
- Gerbaulet AP, Esche BA, Haie CM, Castaigne D, Flamant F, Chassagne D. Conservative treatment for lower gynecological tract malignancies in children and adolescents: the Institut Gustave-Roussy experience. Int J Radiat Oncol Biol Phys 1989;17:655–8.[Medline]
- Corpron CA, Andrassy RJ, Hays DM, Raney RB, Wiener ES, Lawrence W, et al. Conservative management of uterine pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III and IV pilot. J Pediatr Surg 1995;30:942–4.[CrossRef][Medline]
- Andrassy RJ, Hays DM, Raney RB, Wiener ES, Lawrence W, Lobe TE, et al. Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III. J Pediatr Surg 1995;30:1034–6.[CrossRef][Medline]
- Breitfeld PP, Lyden E, Raney RB, Teot LA, Wharam M, Lobe T, et al. Ifosfamide and etoposide are superior to vincristine and melphalan for pediatric metastatic rhabdomyosarcoma when administered with irradiation and combination chemotherapy: a report from the Intergroup Rhabdomyosarcoma Study Group. J Pediatr Hematol Oncol 2001;23:225–33.[CrossRef][Medline]
- Zanetta G, Rota SM, Lissoni A, Chiari S, Bratina G, Mangioni C. Conservative treatment followed by chemotherapy with doxorubicin and ifosfamide for cervical sarcoma botryoides in young females. Br J Cancer 1999;80:403–6.[CrossRef][Medline]
- Wolden SL, Anderson JR, Crist WM, Breneman JC, Wharam MD Jr, Wiener ES, et al. Indications for radiotherapy and chemotherapy adter complete resection in rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Studies I to III. J Clin Oncol 1999;17:3468–75.[Abstract/Free Full Text]
- Arndt CA, Donaldson, Anderson JR, Andrassy RJ. What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract? Cancer 2001;91:2454–68.[CrossRef][Medline]
- Hahlin M, Jaworski RC, Wain GV, Hamett PR, Neesham D, Bull C. Integrated multimodality therapy for embryonal rhabdomyosarcoma of the lower genital tract in postpubertal females. Gynecol Oncol 1998;70:141–6.[CrossRef][Medline]
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Abstract
Case report
