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Case report |
Royal Marsden NHS Foundation Trust, Downs Road, Sutton, Surrey SM2 5PT, UK
Correspondence: Current address for R A England, Cumberland Infirmary, North Cumbria Acute Hospitals NHS Trust, Newtown Road, Carlisle, Cumbria CA2 7HY, UK. E-mail: rachel.england{at}ncumbria-acute.nhs.uk
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Abstract |
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Case report |
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At surgery a solid right ovarian tumour was removed. There was rupture of the tumour capsule but no ascites. There was evidence of additional disease within the peritoneum in the right pouch of Douglas and further evidence of disease spread to a right internal iliac lymph node. All visible disease was excised and peritoneal biopsies and washings taken. Post-operative AFP measured 18, returning to normal within 2 weeks of commencing chemotherapy. She underwent four cycles of bleomycin, etoposide and cisplatin combination chemotherapy. Histology diagnosed a malignant mixed germ cell tumour of the ovary composed of grade 2–3 immature solid teratoma admixed with microscopic foci of yolk sac tumour. There was a metastatic deposit of immature teratoma within the right pouch of Douglas and a deposit of mature glial tissue within the pelvic lymph node.
A 1 month post-operative CT scan of thorax, abdomen and pelvis was normal. A further CT scan performed 4 months post-operatively revealed a 2.7 cm predominantly cystic appearing lesion arising from the right adnexa. The patient had refused transvaginal ultrasound due to previous poor tolerance and an MRI was performed (1.5 Tesla Philips scanner; Philips, Best, The Netherlands) using coronal T1 weighted (spin echo (SE) 600/20 ms [repetition time (TR)/echo time (TE)]) and short tau inversion recovery (STIR) (IR 2500/30/130 ms [TR/TE/inversion time (TI)]), sagittal and axial T2 weighted (fast spin echo (FSE) 4000/90 ms [TR/effective TE]) with an 18–30 cm field of view, 256x512 matrix and a 4–5 mm slice thickness. Hyoscine butyl bromide 20 mg was given intramuscularly prior to image acquisition. The complex right adnexal mass was clearly demonstrated (Figure 1a,b
). Additional masses of smaller volume were seen within the right adnexa (Figure 1b), and adjacent to the uterus within the left pouch of Douglas (Figure 1c). On the basis of the histology from peritoneal biopsies and lymph node taken at the original surgical procedure it was felt that these deposits were most likely to represent glial elements of mature differentiated teratoma and it was decided to observe and repeat the MRI scan in 2 months' time.
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) although the larger right adnexal mass remained relatively unchanged (Figure 2a,b). As a consequence she underwent a second surgical procedure with extensive laparoscopic peritoneal review and removal of all abnormal tissue deposits. A total of six deposits were seen and removed from the right adnexa, left pouch of Douglas, left ovary and right abdominal wall. The nodular specimens were processed in their entirety and contained mature teratomatous elements, largely mature glial tissue, and compatible with origin from malignant immature teratoma of the ovary. Tumour markers have all remained normal following the initial course of chemotherapy. She remains well on continued review at 12 months.
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Discussion |
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Immature teratomas are largely solid tumours and occur most frequently in the second and first decades. When associated with mature glial implants within the peritoneum the prognosis is usually much better irrespective of the original tumour grade [2]. However, there have been descriptions of cases of mature gliomatosis peritonei which have seemingly evolved into malignant tumours [4–8].
The American Pediatric Oncology Group in collaboration with the Children's Cancer Group [4] has recommended that in patients with immature teratoma and no evidence of disease recurrence, surgery alone is effective treatment irrespective of the grade of primary tumour. Chemotherapy is recognized to significantly improve outcome in disease recurrence [9] but is recognized to carry significant side effects. They therefore recommend salvage chemotherapy for the subset of patients in whom recurrence is detected. As such, MRI may also have a role to play in the follow up of patients with total clearance of primary tumour and no evidence of metastases at original surgery. Patients in their study were followed by a mixture of chest radiograph and CT or MRI pelvis for 2 years (although they do not specify how frequently these studies were undertaken) with serum AFP levels and clinical examination for the duration of the study. Follow up ranged from 3 months to 7 years with a median of 4.2 years. 1 of 44 patients had a malignant recurrence which was detected at 18 weeks. Like our case she had immature ovarian teratoma with microscopic foci of yolk sac tumour, pre-operative raised AFP and gliomatosis peritonei within the peritoneum and sampled lymph node.
Shefren et al [5] report a case of malignant abdominal neoplasm 5.5 years after extensive original surgery for grade 3 teratoma. In intervening laparotomies only mature peritoneal glial tissue had been present. Dadmanesh et al [6] describe the case of a 13-year-old girl who developed a tumour resembling glioblastoma 7 years after original surgery for grade 1 teratoma and low grade gliomatosis peritonei. Both of these cases had been extensively sampled at original and subsequent operations and both had received chemotherapy. Trabelsi et al [7] report the case of a 37-year-old woman who presented 7 years after removal of an immature ovarian teratoma with a malignant tumour resembling glioblastoma. Given the time to malignant evolution in these examples, the follow up period in the Cushing et al study [4] may be insufficient to detect all malignant recurrences and therefore 2 years appears be an insufficient period of image-guided follow up.
Müller et al [8] have identified a total of 11 cases by literature review of gliomatosis peritonei with fatal outcome. Two are described above [5, 6], the other nine of these occurred within 12 months of original surgery and they postulate a lack of extensive histological sampling at the time of original surgery as a factor in confusing malignant tumour dissemination with subsequent malignant evolution of mature glial tissue. Hamada et al [10] also recognize the importance of extensive histological sampling at initial surgery, acknowledging incomplete resection of primary immature teratoma as the cause of recurrence 14 weeks post laparotomy in one of their cases.
As this condition is rare there is no widely accepted guidance as to how long and by which means these patients should be followed up. Peritoneal deposits are usually readily detected by CT, but long-term follow up is at the expense of a cumulative dose of medical exposure radiation and the subsequent increased risk of additional fatal cancers which is particularly relevant in this predominantly young patient group. This seems a high price to pay when the rate of malignant transformation of these glial deposits is thought to be low. Transvaginal ultrasound is an alternative radiation-free imaging technique for review of the pelvic structures. Most deposits of glial tissue are smaller than 3 mm, often described as miliary, and may be difficult to detect by ultrasound which is a largely operator-dependent technique. They may appear on any peritoneal surface and be obscured by bowel gas or lie within the abdomen and be out of the field of view of the transvaginal probe and therefore transvaginal ultrasound used alone has its limitations. A transvaginal route is also unacceptable in a paediatric population.
The repeated measurements of tumour markers, particularly AFP, is usually undertaken throughout follow up, but may provide false reassurance. Levels frequently stay within normal limits in cases of gliomatosis peritonei even when previously elevated in association with the original teratoma, and likewise may also remain normal in tumour recurrences containing immature endodermal elements [11].
MRI is free from radiation dose, usually well tolerated and as we have shown, demonstrates macronodules of glial tissue readily. Deposits of glial tissue are of moderately high signal on T2 weighted images, the sequence which provides the best contrast resolution. Contrast enhancement with gadolinium is not usually indicated. Lesions tend to be homogeneous although may be multilobulated as seen in Figure 1a
. Image quality may be improved by the administration of an antispasmodic drug which we use routinely for pelvic MR examinations. An initial staging CT to assess the upper abdomen and chest is still recommended in cases of immature teratoma.
Many more cases of mature gliomatosis peritonei need to be followed for several years before we can ascertain the true natural history of the condition and an accurate rate of malignant evolution. As cases of malignant evolution have been described up to 7 years from original surgical clearance of teratoma, we recommend that imaging follow up should occur annually for at least 5–7 years. As such we propose that MRI is a safe, reproducible and accurate technique for the imaging of patients with a history of immature ovarian teratoma and mature gliomatosis peritonei.
Received for publication April 26, 2005. Accepted for publication August 30, 2005.
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