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 Table of Contents  
Year : 2021  |  Volume : 7  |  Issue : 2  |  Page : 132-135

Gliomatosis peritonei with ovarian teratoma: A report of three cases and literature review

1 Department of Gynecological Oncology, SCI, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
2 Department of Hematology (Pathology), Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
3 Department of Pathology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India

Date of Submission26-Aug-2021
Date of Decision01-Oct-2021
Date of Acceptance11-Oct-2021
Date of Web Publication17-Aug-2021

Correspondence Address:
Sangeeta Pankaj
Department of Gynecological Oncology, SCI, Indira Gandhi Institute of Medical Sciences, Patna - 800 014, Bihar
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jigims.jigims_40_21

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Gliomatosis Peritonei (GP) is a rare condition characterized by the presence of neuroglial implants throughout the peritoneum. It is a benign lesion with a potential for malignant transformation. Its association with ovarian teratomas has been sparsely reported in the literature. As GP is a rarity, it lacks forth set and accepted guidelines for management. Here, we report three intriguing cases of histopathologically confirmed GP in association with ovarian teratoma. All three patients were young females between 18 and 22 years of age and presented with abdominal swelling and ascites. On evaluation, they had raised tumor markers (CA-125, Agence France-Presse, and lactate dehydrogenase) and radiological findings were in favor of ovarian malignancy. Two of them underwent primary debulking surgery, whereas one received neoadjuvant chemotherapy in view of recurrent ovarian tumor of nonseminal germ cell nature followed by interval debulking surgery. Intraoperative findings in all three cases were almost similar. Unexpectedly, the histopathological findings were reported as immature teratoma in two cases and mature teratoma in one along with GP.

Keywords: Gliomatosis peritonei, neuroglial implants, teratoma

How to cite this article:
Abhilashi K, Kumari P, Kumari S, Rani J, Pankaj S, Choudhary V, Singh R. Gliomatosis peritonei with ovarian teratoma: A report of three cases and literature review. J Indira Gandhi Inst Med Sci 2021;7:132-5

How to cite this URL:
Abhilashi K, Kumari P, Kumari S, Rani J, Pankaj S, Choudhary V, Singh R. Gliomatosis peritonei with ovarian teratoma: A report of three cases and literature review. J Indira Gandhi Inst Med Sci [serial online] 2021 [cited 2022 Nov 28];7:132-5. Available from: http://www.jigims.co.in/text.asp?2021/7/2/132/331752

  Introduction Top

Teratoma is the most common germ cell tumor usually afflicting children and adolescents. It consists of adult and embryonal tissues derived from all three germ layers.[1] Gliomatosis peritonei (GP) is a rare condition associated with immature teratoma of the ovary and rarely with mature teratoma, pregnancy, and ventriculoperitoneal shunts. It was first described by Neuhauser in 1906, since then only about 100 cases have been described in the literature. GP is characterized by miliary to nodular implantation of glial tissues on the surface of the visceral and parietal peritoneum.

The index study presents three cases of GP, two of them associated with immature and one with mature teratoma.

  Case Reports Top

Case 1

An 18-year-old female P1 L1 came to the gynecologic oncology OPD, IGIMS, Patna, with the complaints of abdominal distension and pain of 6 months duration. During the physical examination of abdomen and pelvis, around 25 cm × 20 cm size lump of firm consistency with restricted mobility was felt along with ascites. The left-sided pleural effusion was present. Contrast-enhanced computed tomography (CECT) revealed 22 cm × 24 cm × 15 cm lesion arising from pelvis and extending up to epigastrium which was cystic with thick septa and multiple fat components and calcification with mild ascites. X-ray chest PA view was suggestive of the left side pleural effusion. Serum tumor markers analysis demonstrated increase in CA125 (333.7 U/ml), carbohydrate antigen (CA) 19-9 (1430 ng/ml), Agence France-Presse (AFP) (97.7U/ml), and lactate dehydrogenase (LDH) (357U/L).

Intraoperatively, moderate ascites along with the right ovarian mass of 25 cm × 30 cm size having solid and cystic areas were noticed and the left ovary was bulky. There were extensive gray military deposits present over peritoneum and intraabdominal organs. The intraoperative picture was suggestive of advanced-stage ovarian malignancy. Intraoperative frozen section was performed which was suggestive of immature teratoma. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, appendicectomy, and partial peritonectomy were performed. On histopathological examination, the right ovarian mass had immature teratoma Grade 1 while the left ovary had a mature teratoma. Omental and peritoneal implants showed mature neuroglial elements making the diagnosis of GP [Figure 1]. At present, patient is asymptomatic and on follow-up for the past 28 months.
Figure 1: Omental and peritoneal implants showed mature neuroglial elements

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Case 2

A 22-year-old unmarried female was referred to the OPD with complaint of progressively increasing abdominal swelling for 1 year. She had history of the right ovarian cystectomy 18 months back and the histopathological report was immature teratoma. Postoperatively, she had received 4 cycles of platinum-based adjuvant chemotherapy in view of immature teratoma with raised AFP (170.3U/ml). Within 6 months of chemotherapy, the patient again complained of abdominal swelling. On clinical examination, there was ascites along with 15 cm × 12 cm size firm abdominopelvic lump. Her serum tumor markers reported increase in CA125 (232.2 U/ml) while CA19-9 (21.5 ng/ml), AFP (4.7 U/ml), and LDH (225.7 U/L) were within normal range. CECT showed 10 cm × 12 cm × 15 cm solid cystic lesion in the peritoneal cavity with multiple calcifications and fat density and massive ascites.

Intraoperatively, there was moderate ascites, solid cystic right ovarian mass of 15 cm × 12 cm, and multiple deposits of 1–2 cm present all over peritoneum, and intraabdominal organs including the uterine surface [Figure 2]. The left ovary had cyst of 10 cm × 8 cm. Total omentectomy and peritonectomy were done along with bilateral salpingo-oophorectomy. The histopathological report of the tumor suggested mature teratoma and the deposits over omentum were confirmed as mature glial tissue implants. Immunohistochemical staining for glial fibrillary acidic protein (GFAP) and S-100 was positive. Her postoperative stay in hospital was uneventful and in the past 18 months of follow-up, she is asymptomatic without any recurrence.
Figure 2: Intraoperative picture of case 2 showing the tumor and deposits over gut

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Case 3

A 20-year-old female P2 L2 reported to the gynecologic oncology OPD with abdominal pain and distension for 4 months. Physical examination of pelvis and abdomen revealed an approximate 30 cm × 35 cm abdominopelvic lump of variegate consistency and ascites. The serum tumor marker analysis revealed increase in CA-125 (234.2 U/ml). CA19-9 (9.8 ng/ml), AFP (1.6 U/ml), and LDH (310.9U/L) were within the normal limits. CECT abdomen and pelvis revealed 30 cm × 28 cm × 30 cm solid cystic lesion arising from pelvis with the presence of fat and calcification. Intraopertively, the right ovarian solid cystic mass of 30 cm × 35 cm along with moderate ascites and miliary to nodular deposits were seen all over abdominal viscera, and peritoneal surface and enlarged paraaortic lymph node. Intraoperative frozen section of the tumor was sent and the report was in favor of immature teratoma. Thus hysterectomy, bilateral salpingo-oophorectomy, omentectomy, peritonectomy, and paraaortic lymphadenectomy were performed. Histopathological examination confirmed it to be immature teratoma Grade 3. The peritoneal implants consisted of mature neuroglial tissues. Immunohistochemical staining for GFAP and S-100 both was positive. The patient developed ascites in postoperative period and started on chemotherapy with bleomycin, etoposide, and cisplatin. She received only two cycles of chemotherapy and did not come back for further cycles of chemotherapy and after 6 months of surgery, she succumbed to death.

  Discussion Top

Immature teratoma is a preferred term for malignant ovarian teratoma and is usually seen in the first and second decade of life.[2] Our patients were also young belonging to the second and third decade. Abdominal pain and lump over a period are the common presentation. They may develop Pseudo-Meig's syndrome associated with ascites and pleural effusion due to teratoma. As the tumor occurs in young age females, conservative surgery with preservation of fertility should be performed. Case 1 and 3 were P1 L1 and P2 L2, respectively, not willing to retain fertility in future and frozen section also suggested malignant germ cell tumor (immature teratoma) henceforth complete surgical staging was performed.

Case 2 was unmarried girl and had recurrence of ovarian tumor along with tumor marker positivity following the course of adjuvant chemotherapy after initial ovarian cystectomy. She underwent fertility-sparing surgery. Histological grading of immature teratoma is an important prognostic factor and Grade 3 tumors are associated with a hazard ratio for mortality of 8.07 compared to well-differentiated tumors.[3] Although GP is often diagnosed on H and E stained tissue slides, IHC helps to differentiate it from low-grade epithelial ovarian tumors and also confirms its tissue of origin. (GFAP) staining is used to confirm the glial nature of the tissue and more strong the expression of GFAP, the more well differentiated the tumor is. Studies have shown that tissue staining positive for SOX-2 and negative for OCT4 and NANOG are suggestive of stem cell origin of GP. In our case, GP was diagnosed on H and E staining which were characterized by mature glial tissue in the peritoneum and immunohistochemical staining for GFAP and S-100 both were positive in two cases. A review of the literature reported by Chou et al. had found 65 cases of GP, which have favorable prognosis after surgical treatment.[4] Chemotherapy with the surgery has been reported to affect the prognosis positively. In patients with extraovarian spread, the microscopic feature of the metastasis is of prognostic importance. The implants contain mature elements composed of mature glial tissues and do not adversely affect the prognosis. Complete surgical resection is recommended for GP with the objective of confirming the diagnosis and excluding malignancy as well preventing the malignant transformation of residual lesions.[5] However, as the lesions in GP are extensive complete resection is usually difficult and in our cases also optimal resection of all the glial implants could not be achieved. Literatures have reported that GP is associated with frequent recurrences, especially in patients with immature teratoma. Rarely in patients with immature teratoma having mature glial implants, malignant transformation maybe observed several years after surgery. Due to the rarity of its occurrence, there are no clear guidelines for follow-up of these patients. In a study done by England et al., Magnetic resonance imaging and tumor markers were recommended for the monitoring of patients with immature ovarian teratoma and mature glial tissue implants.[6] Computed tomography (CT) and ultrasound have also been proposed for monitoring of the disease in other studies. In our case, we followed up the patients every 3 months with clinical examination, CT scan, and tumor markers. Two cases demonstrated positive prognosis within 18 months of follow-up period after surgery and one died after 6 months of surgery.

  Conclusion Top

GP is a rare condition associated with ovarian teratomas. As GP presents with extensive peritoneal implantation, optimal resection is often difficult to perform and intraoperatively may be misleading and can even lead to a wrong diagnosis of as an advanced-stage ovarian carcinomas or even peritoneal tuberculosis. Therefore, long-term follow-up of these patients is recommended due to the potential for malignant transformation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Rosai J. Female reproductive system-Ovary. In: Rosai and Ackerman Surgical Pathology. 10th ed. Vol. 2. Edinburg: Elsevier Mosby; 2011. p. 1553-635.  Back to cited text no. 1
Nur J, Akter S, Khanom R. Immature ovarian teratoma with gliomatosisperitonei – An unusual finding. J Dhaka Med Coll 2017;26:79-82.  Back to cited text no. 2
Jorge S, Jones NL, Chen L, Hou JY, Tergas AI, Burke WM, et al. Characteristics, treatment and outcomes of women with immature ovarian teratoma, 1998-2012. Gynecol Oncol 2016;142:261-6.  Back to cited text no. 3
Chou JS, Wu HP, Yu FT, Hu WM. Pathological case of the month. Immature ovarian teratoma with gliomatosis peritonei. Arch Pediatr Adolesc Med 1998;152:301-2.  Back to cited text no. 4
Mrabti H, El Ghissassi I, Sbitti Y, Amrani M, Hachi H, Errihani H. Growing teratoma syndrome and peritoneal gliomatosis. Case Rep Med 2011;2011:123527.  Back to cited text no. 5
England RA, DeSouza NM, Kaye SB. Gliomatosis peritonei: MRI appearances and its potential role in follow up. Br J Radiol 2014;80:953.  Back to cited text no. 6


  [Figure 1], [Figure 2]


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