Indian Journal of Cancer, Vol. 42, No. 3, July-September, 2005, pp. 165-167
Mature ovarian teratoma with gliomatosis peritonei - A case report
Das CJ, Sharma R, Thulkar S, Mukhopadhyay S, Deka D, Mannan R
Departments of Radiology All India Institute of Medical Sciences, Ansari Nagar, New Delhi
Code Number: cn05030
AbstractGliomatosis peritonei (GP), a rare condition related to ovarian teratomas, is characterized by miliary implants of mature glial tissues on the peritoneum or omentum. We report herein a case of mature teratoma of the ovary with GP with imaging features and pathological correlation
Keywords: CT scan, Gliomatosis peritonei, Ovarian mature teratoma
IntroductionGliomatosis peritonei (GP) is the implantation of mature neural glial tissue on surfaces of visceral or parietal peritoneum. This condition is usually seen in patient with immature ovarian teratoma and rarely found with mature teratomas. We present an unique case of mature teratoma of the ovary associated with GP.
A 20-year-old woman was admitted to gynecology out-patient department with 1-month history of vague abdominal pain and lump in the lower abdomen. Her serum a-fetoprotein (AFP) level was normal. Plain X-ray abdomen was unremarkable. Ultrasonography revealed a poorly defined heterogeneously hyperechoic mass with foci of distal shadowing. Contrast enhanced CT scan [Figure - 1] showed complex solid cystic mass in left lower abdomen and pelvis. Fat containing areas and multiple foci of calcification were noted within the mass. The adjacent bowel loops were infiltrated but the uterus and urinary bladder were free from the mass. At laparotomy, a right ovarian tumor was found, which measured 8 cm in diameter with solid and cystic component. Delicate adhesions were noted between the tumor and the lateral pelvic wall. In addition, multiple firm, gray-white, 0.3-1.2 cm nodules were present on the anterior peritoneum, uterine serosa, and omentum. The left ovary was unremarkable. Right salpingo-oophorectomy, partial omentectomy, and biopsy of the nodules were performed. The postoperative period was uneventful. No additional therapy was given. The patient is alive and well at follow up 26 months postoperatively.
Histopathologic examination revealed features of a mature teratoma comprising skin and adnexal structures, gut epithelium, respiratory epithelium, foci of mature cartilage, and mature glial tissue [Figure - 2]. Twenty sections were examined from the ovarian tumor and none of them revealed any immature element, including primitive neuroectodermal tissue.
The peritoneal implants and the omental deposits all revealed Grade 0 mature astroglial tissue. The glial fibrillary acidic protein (GFAP) immunostain confirmed the glial nature of the tissue.
Gliomatosis peritonei, the miliary implants of mature glial tissue on the peritoneum, is an infrequently reported complication of ovarian teratoma. It has been found to occur almost exclusively in females with ovarian teratomas, though there are stray reports of its association with pregnancy, ventriculoperitoneal shunts performed for hydrocephalus., The mechanism of implantation is unknown and two theories to explain the origin of GP have been proposed. In one, glial implants arise from the teratoma, whereas in the other, pluripotent stem cells in the peritoneum or subjacent mesenchyme undergo glial metaplasia.
All grades of ovarian teratomas have been described, with immature teratomas being more commonly associated with this condition. The condition is relatively rare and only about 88 cases of glial implants on the peritoneum associated with ovarian teratomas have been reported in the literature.- The first case from India was reported by Joshi et al. in 1981 after which three more cases had been reported. ,,, Despite the fact that neural tissue may be found in > 30% of mature teratomas, it is rare to have GP associated with mature teratomas.
The prognosis of ovarian teratoma is closely associated with tumor grade as proposed by Thurlback and Scully, which was modified by Norris et al. In the series by Norris et al. of 58 patients, 5-year survival rate for patients with Grades 1-3 were 82, 63, and 30%, respectively. Paradoxically, patients who have immature ovarian teratomas in association with mature glial implants appear to have a much improved prognosis., This statement holds true only if stringent criteria for diagnosis of GP is adhered to, as proposed by Thurlback and Scully:(a) peritoneal surface, omentum, and diaphragmatic surfaces must be extensively sampled histologically and (b) each of the sampled implants should be composed exclusively, or almost exclusively, of Grade 0 glial tissue. If these two conditions are met, the prognosis of the disease is excellent.
There are 11 cases reported so far, that had an adverse outcome. Shefren et al. reported a 16-year-old girl with Grade 3 teratoma, who developed malignant glial peritoneal implants and died 5.5 years after her initial surgery. Dadmanesh et al. reported a patient with Grade 1 teratoma and Grade 1 immature glial implant diagnosed 10 months after initial operation, who died 8 years after oophorectomy. These two cases are of interest in this context as both the cases developed malignancy after a long symptom-free interval.
Regarding treatment, therapy should be directed by the grade of the primary tumor and not by the glial implants, if they are extensively sampled and all are mature. However, extensive sampling of all peritoneal implants is important. If no other teratomatous elements or malignant glial tissue is found in the implants, the mature glial implants can be ignored and the method of therapy should be judged only by the stage and grade of the primary ovarian teratoma. However, if immature glial tissue or other teratomatous components or both are present in the peritoneum or omentum, the treatment should be the same as for metastatic ovarian teratoma. Despite often wide spread involvement of peritoneal surfaces, GP is reported to impart an improved prognosis even in high-grade ovarian teratomas.,
In summary, GP is a rare condition that generally imparts a favorable prognosis to patients with ovarian teratomas. If patients undergo extensive staging, peritoneal implants are well sampled, and histologic description shows the implants to be completely mature, a benign clinical course is to be expected. However, long-term follow up, even in the face of mature peritoneal glial implants is highly recommended because of established cases of malignant transformation of the glial components long after initial surgery.
Copyright 2005 - Indian Journal of Cancer
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