223Y. Hamada et al.: Ovarian Teratoma with Gliomatosis PeritoneiSurg TodayJpn J Surg (1998) 28:223–226

Abstract: Gliomatosis peritonei, a rare condition related toovarian teratomas, involves the peritoneal implantation ofnumerous nodules of predominantly mature glial tissues. Wereport herein the cases of two patients with immature ovarianteratoma associated with gliomatosis peritonei, in one ofwhom a rapid progression of teratomatous implants occurred14 weeks after her initial surgery. Gliomatosis peritonei isconsidered benign in most cases; however, some reports havedocumented the rapid recurrence of immature peritoneal im-plants, as implantation is associated with teratomas of allgrades. Thus, in the face of peritoneal implants suspected tobe of a teratomatous nature, thorough and extensive samplingis essential to exclude the presence of immature elementswhich may imply a poor prognosis and require aggressivetherapy.

Key Words: ovarian immature teratoma, gliomatosis peritonei

Introduction

Gliomatosis peritonei is associated with ovarianteratoma, and is characterized by the peritoneal implan-tation of miliary nodules composed mostly of matureglial tissues.1–3 Despite often widespread involvement ofthe peritoneal surfaces, gliomatosis peritonei is not re-ported to have a poor prognosis even when associatedwith immature teratomas.1 This condition is relativelyrare, with only about 50 cases having been reported inthe English literature.4,5 We report herein the cases oftwo patients, one of whom experienced the rapid pro-gression of implants of immature teratoma. Follow-

ing the presentation of these two cases, we examinethe clinicopathological characteristics of this entityand review some previous reports of this unusualphenomenon.

Case Reports

Case 1

A 12-year-old girl was admitted to our hospital with a 1-month history of abdominal distention and constipa-tion. Her serum α-fetoprotein (AFP) level was normal;however, abdominal plain X-ray, abdominal ultra-sound, and magnetic resonance image (MRI) (Fig. 1)revealed a solid and cystic mass with calcification andascites in the peritoneal cavity. As teratoma sometimesshows calcium deposits and teeth without root canals, adiagnosis of ovarian teratoma was preoperatively sus-pected. At laparotomy, the right ovary was found tohave been replaced by a large, smooth, gray-pink masswith a capsular tear at one pole. Multiple, firm, gray-white nodules, 0.1 to 0.3 cm in diameter, were noted inthe pelvic peritoneum, mesenterium, and omentum. Abiopsy of the omental implants revealed mature glialtissue. A right salpingo-oophorectomy and partialomentectomy were performed. The patient was thenadministered intraperitoneal cisplatinum, mitomycin C,and OK-432 according to the gynecologist’s advice. Shehad an uneventful postoperative course and has re-mained well without any signs of recurrence throughoutthe 20 months since her surgery.

Pathologic Findings. The right ovarian tumor weighed2990 g and measured 30 3 20 3 17cm. The mass wassolid and cystic, and comprised of an immatureteratoma (grade 1) with multiple serosal nodules includ-ing the omentum, which was composed exclusively ofmature glial tissue (grade 0).

Reprint requests to: Y. Hamada(Received for publication on June 3, 1996; accepted on Jan. 7,1997)

Ovarian Teratoma with Gliomatosis Peritonei: Report of Two Cases

Yoshinori Hamada1, Akihide Tanano1, Masahito Sato1, Masazumi Tsuji1, Noriko Sakaida2,Akiharu Okamura2, and Koshiro Hioki1

1 Second Department of Surgery and 2 Department of Clinical Pathology, Kansai Medical University, 10-15 Fumizono, Moriguchi City, Osaka570, Japan

224 Y. Hamada et al.: Ovarian Teratoma with Gliomatosis Peritonei

Fig. 1. Case 1. T1 weighted MRI of the ovarian tumor reveal-ing a solid and cystic mass with calcification and ascites in theperitoneal cavity. Arrows indicate the solid and cystic mass(M), calcification (C), and ascites (A)

Case 2

A 9-year-old girl presented with a 2-week history of anenlarging abdominal mass. Physical examination re-vealed a large, smooth, nontender mass extending fromthe pelvis to the upper abdomen. The serum AFP was235ng/ml. Abdominal X-rays, ultrasonography, MRI,and computed tomography (CT) scan showed a largecystic and solid mass with numerous calcifications, lead-ing to a preoperative diagnosis of ovarian teratoma withmalignant potential. At laparotomy, 1400ml of yellow-ish, clear ascites was drained and a large right ovariantumor with an almost smooth surface and a capsulartear was found. Numerous, firm, gray-white nodules, 0.1to 0.3cm in diameter, were noted over the surface of thetumor, posterior broad ligaments, pelvic peritoneum,and omentum. A yellowish mass (Fig. 2) measuring ap-proximately 2cm in diameter in the cul-de-sac wasbiopsized and partially removed. As intraoperativepathological reports on implants of both the omentumand cul-de-sac revealed mature teratoma, a right

Fig. 3. Case 2. Intraoperative view of the recurrent tumor

Fig. 2. Case 2. Intraoperative view of the yellowish mass inthe cul-de-sac

salpingo-oophorectomy and partial omentectomy wereperformed. No further therapy was given.

The patient was readmitted 14 weeks after the initiallaparotomy for removal of a recurrent pelvic mass. TheAFP level had re-elevated to 214ng/ml. At the secondlaparotomy, a small amount of ascites was present andthe recurrent tumor (Fig. 3) seemed to arise from theoriginal cul-de-sac mass. Numerous, gray-white nod-ules, 0.2 to 0.3cm in diameter, were noted across thesurface of the peritoneum. Pathological examination ofthe recurrent tumor and omental implants revealedimmature teratomas of grade 1 and grade 0, respec-tively. Removal of the recurrent tumor and partialomentectomy were performed. The patient was giventhree cycles of cisplatinum — vinblastin–bleomycin(PVB) therapy and has remained well with a normalAFP level for 6 months since the second laparotomy.

225Y. Hamada et al.: Ovarian Teratoma with Gliomatosis Peritonei

Pathologic Findings. The original right ovarian tumorweighed 2 450 g and measured 21 3 17 3 12cm. Themass was solid and cystic. Light microscopic sectionsshowed a grade 1 immature teratoma with partially im-mature neuroepithelium (Fig. 4). Numerous omentaland peritoneal nodules were composed of mature glialtissues. Immunohistochemically, glial fibrillary acidicprotein (GFAP) was positive in the mature glial tissue,but negative in the immature neuroepithelial elements.CA19-9, AFP, and carcinoembryonic antigen were posi-tive in the respiratory as well as alimentary epithelia.

The recurrent tumor weighed 320g, measured 12 310 3 6.5 cm, and contained both solid and mul-tiloculated cystic lesions. The tumor was grade 1 imma-ture teratoma with more predominant immatureneuroepithelial elements than in the original tumor(Fig. 5). The omental implants also consisted of matureglial tissues.

Discussion

Gliomatosis peritonei is a condition that appears grosslyas miliary, firm, gray-white nodules implanted on theperitoneal surface or in the omentum. These nodulesmay vary in size, but according to most reports they areusually around 0.3 cm in diameter.1–3 The most oftenhypothesized mode of dissemination of gliomatosisperitonei is intraperitoneal spread via a capsular defectin the primary tumor, either spontaneous or surgical.2

However, both our patients were found to have hadspontaneous rupture of the tumor with a large amountof yellowish ascites.

The surgical staging system most commonly em-ployed for ovarian tumors was developed by the Inter-

national Federation of Gynecology and Obstetrics(FIGO).6 According to this staging system, the tumorsfrom both our patients were classified as Stage Ic. How-ever, for ovarian immature teratomas, the primary tu-mor and implants should both be graded according to ahistologic grading system,1,4,7 that is based on the quan-tity of neuroepithelium. This system has become widelyaccepted as the preferred method of determining thedistinct therapeutic and prognostic implications.

The prognosis of immature ovarian teratoma isclosely associated with the tumor grade.4 Gallion et al.8

reviewed 150 cases of immature ovarian teratoma andfound 2-year survival rates of 81%, 51%, and 33% forgrades 1, 2, and 3, respectively. However, patients withimmature ovarian teratomas in association with matureglial implants appear to have a much better prognosis,1,9

although this is clearly dependent on two importantfactors: first, the peritoneal surfaces and omentum, in-cluding the diaphragmatic surfaces, must be thoroughlysampled histologically; and second, all of the implantsmust be mature, i.e., without immature elements. Ifthese two conditions are met, the prognosis of the dis-ease is excellent.10

Regarding treatment, while it has generally been es-tablished that patients with grade 1 primary tumors andgrade 0 glial implants do not need further chemo-therapy, determining which patients require treatmentwith chemotherapy is still under discussion.4 Moreover,the effects of chemotherapy on the course and outcomeof pregnancies following successful cancer treatment inchildhood are still unelucidated.11

According to our review of the literature, four casesshowed adverse outcomes.2,10,12,13 Albeits12 reported a 9-year-old girl who developed the rapid recurrence ofperitoneal implants and died 5 months after her initial

Fig. 4. Case 2. H&E staining of the primary ovarian tumor(3100). Neuroepithelial rosettes and tubules in the matureglial tissues and a glandular component resembling the primi-tive gut (grade 1)

Fig. 5. Case 2. H&E staining of the recurrent tumor (340).Note the more immature neuroepithelial components thanthose in the primary tumor (grade 1)

226 Y. Hamada et al.: Ovarian Teratoma with Gliomatosis Peritonei

surgery, while Khoo et al.13 reported a patient withgrade 2 teratoma and grade 2 immature glial implantswho died 12 months after presentation. These two casesfailed to meet the two previously cited criteria becausethe glial implants were not completely mature. Nielsenet al.2 also reported a case of recurrent immatureteratoma which developed rapidly due to inadequatesampling. Shefren et al.10 reported a unique case of a 16-year-old girl with grade 3 immature teratoma in whommalignant transformation of mature glial implantsdeveloped 5 years after her initial surgery.

We interpret our case 2 as immature teratomabecause of the rapid progression following incompleteresection. The universally gray-white and smooth-surfaced peritoneal implants, 0.1 to 0.3cm in size, asseen in our case 1, were mature implants. However,implants with any other appearance, such as a largersize or different color, such as yellow, and not smoothbut rough-surfaced, might suggest an immature nature.Furthermore, a re-elevated serum AFP level after theinitial surgery could indicate the recurrence of imma-ture elements of the tumor, since postoperative moni-toring of the AFP level has been reported necessary todetect the regrowth of immature tissue and to evaluatethe therapeutic effects.14

Our report of case 2 emphasizes the importance ofaccurate histologic grading of peritoneal implants.Thus, as a first step, we strongly urge that differentiationbetween benign and malignant teratomatous implantsshould be sought based in part on the gross appearanceof the peritoneal implants. As a second step, biopsysampling by the surgeon and thorough sectioning by thepathologist should be performed to reduce the chanceof misinterpreting peritoneal implants.

References

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