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Pathological Case of the Month FREE

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Section Editor: Enid Gilbert-barness, MD

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Arch Pediatr Adolesc Med. 1998;152(3):302. doi:.
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Published online

DIAGNOSIS AND DISCUSSION: IMMATURE OVARIAN TERATOMA WITH GLIOMATOSIS PERITONEI

Figure 1. A large, partially calcified intraperitoneal mass.

Figure 2. Grade 1 immature ovarian teratoma with neuroepithelial rosette formation (hematoxylin-eosin, original magnification ×132).

Figure 3. Grade 0 glial implants in omentum (hematoxylin-eosin, original magnification ×66).

Ovarian teratomas are graded by criteria of Robboy and Scully,1 which have been modified by Thurlbeck and Scully2 and Dehner.3 A teratoma with no immature tissue is grade 0; with fewer than 1 low-power field of immature tissue per slide is grade 1; with more than 1 but fewer than 4 low-power fields per slide of immature neuroepithelial foci is grade 2; and a teratoma with consecutive microscopic fields of immature tissue, either immature neuroepithelial or somatic, is grade 3. All grades 1 and 2 ovarian teratomas in children act in a benign fashion. All grade 3 teratomas that exhibit malignant behavior tend to have foci of yolk sac tumor.

Light microscopy showed a grade 1 immature teratoma of the ovary. Most areas of the tumor were composed of abundant mature glial tissue, choroid plexus, skin and adnexal structures, gut mucosa, bone, cartilage, and teeth. Only scanty amounts of immature mesenchymal elements, cartilage, and primitive neuroepithelium were observed (Figure 2). The omental implants were composed of nodular mature astroglial tissue (Figure 3) and were positive for glial fibrillary acidic protein and S100 protein.

Gliomatosis peritonei (GP) is characterized by miliary implants of mature glial tissue on the peritoneum or omentum.4 Gliomatosis peritonei is usually associated with immature, or rarely with mature, ovarian teratoma (OT). Recognition of this relationship is important because the gross appearance of GP looks like peritoneal carcinomatosis5; hence, many cases of low-grade OT associated with GP were treated initially by excision and followed improperly by chemotherapy or radiotherapy.1,4,610 Despite widespread involvement of peritoneal surfaces, GP does not adversely affect the prognosis of primary OT.

Before making the diagnosis of GP, 2 important prerequisites must be strictly met: (1) peritoneal surface, omentum, and diaphragmatic surfaces must be extensively sampled during the exploratory operation and (2) each of the sampled implants should be composed exclusively, or almost exclusively, of grade 0 glial tissue according to the criteria proposed by Thurlbeck and Scully.2

A review of the English-language literature showed only about 65 isolated cases of glial implants on the peritoneum associated with OT reported before 1994. The largest series of cases are from Robboy and Scully,1 5 cases; Norris et al,6 7; Nogales et al,7 4; Nielsen et al,11 4; and Harms and Janig,8 13. Others were isolated cases.2,4,5,9,10,12 Of the reported cases, some immature glial implants (grades 1, 2, or 3) or other teratomatous implants, or both, were found, either at the time of the first operation or within a short period thereafter. However, according to the 2 previously mentioned diagnostic criteria, such cases, instead of being diagnosed as GP, should have been diagnosed as metastatic teratoma that would require further aggressive therapy.12 With these considerations, only 45 of the cases are acceptable as GP. Of these 45 cases, the average age at the time of diagnosis was 15 years (range, 2-50 years); the average time of follow-up was 46 months (range, 6-120 months).

The study by Norris and associates6 showed that when only OT was present, the 10-year survival rate for patients with grade 3 OT was 30%; grade 2 OT, 62%; and grade 1 OT, 82%. However, we found that only 1 of the 45 instances of OT with GP underwent malignant transformation 5 years after the initial operation, resulting in the eventual death of the patient. These results suggest that GP does not adversely affect the prognosis of OT and may paradoxically improve the prognosis of primary OT. For the remaining 44 cases, the benign peritoneal glial implants persisted without change, fibrosis, or disappearance. Because most of the patients with GP associated with OT survived, a conservative surgical approach without adjuvant radiotherapy or chemotherapy is recommended for GP.

Extensive sampling of all peritoneal implants is important in the diagnosis of GP. If no other teratomatous elements or malignant glial tissue can be found in the implants, the mature glial implants can be ignored and the methods of therapy should be judged only by the stage and grade of the primary OT. 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 OT.

Accepted for publication February 1, 1997.

Corresponding author: Jiann-Shang Chou, Department of Pathology, Taipei Municipal Chung-Hsiao Hospital, Taipei, Taiwan 10598, Republic of China.

Robboy  SJScully  RE Ovarian teratoma with glial implants on the peritoneum: an analysis of 12 cases. Hum Pathol. 1970;1643- 644
Link to Article
Thurlbeck  WMScully  RE Solid teratoma of the ovary: a clinicopathological analysis of 9 cases. Cancer. 1960;13804- 810
Link to Article
Dehner  LP Germ cell tumor of the mediastinum. Semin Diagn Pathol. 1990;7266- 284
Proskauer  GG Solid teratoma of the ovary with neurological metastases. Obstet Gynecol. 1946;52845- 849
Roscher  AAWeinstein  ECPowsner  L Giant teratomas with benign glial abdominal seeding, mimicking diffuse abdominal carcinomatosis. Int Surg. 1975;60461- 465
Norris  HJZirkin  HJBenson  WL Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases. Cancer. 1976;372359- 2372
Link to Article
Nogales  FF  JrFavara  BEMajor  FJSilverberg  SG Immature teratoma of the ovary with a neural component ("solid" teratoma). Hum Pathol. 1976;7625- 641
Link to Article
Harms  DJanig  U Gliomatosis peritonei in childhood and adolescence: clinicopathological study of 13 cases including immunohistochemical findings. Pathol Res Pract. 1989;184422- 430
Link to Article
Fortt  RWMathie  IK Gliomatosis peritonei caused by ovarian teratoma. J Clin Pathol. 1969;22348- 353
Link to Article
Chuang  JHChen  L Ovarian teratoma with gliomatosis peritonei. J Pediatr Surg. 1992;27662- 664
Link to Article
Nielsen  SNJScheithauer  BWGaffey  TA Gliomatosis peritonei. Cancer. 1985;562499- 2503
Link to Article
Du Toit  JPDeale  CJCDavey  PMJ Immature teratoma of the ovary gliomatosis peritonei: a case report. South African Med J. 1985;671017- 1020

Tables

References

Robboy  SJScully  RE Ovarian teratoma with glial implants on the peritoneum: an analysis of 12 cases. Hum Pathol. 1970;1643- 644
Link to Article
Thurlbeck  WMScully  RE Solid teratoma of the ovary: a clinicopathological analysis of 9 cases. Cancer. 1960;13804- 810
Link to Article
Dehner  LP Germ cell tumor of the mediastinum. Semin Diagn Pathol. 1990;7266- 284
Proskauer  GG Solid teratoma of the ovary with neurological metastases. Obstet Gynecol. 1946;52845- 849
Roscher  AAWeinstein  ECPowsner  L Giant teratomas with benign glial abdominal seeding, mimicking diffuse abdominal carcinomatosis. Int Surg. 1975;60461- 465
Norris  HJZirkin  HJBenson  WL Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases. Cancer. 1976;372359- 2372
Link to Article
Nogales  FF  JrFavara  BEMajor  FJSilverberg  SG Immature teratoma of the ovary with a neural component ("solid" teratoma). Hum Pathol. 1976;7625- 641
Link to Article
Harms  DJanig  U Gliomatosis peritonei in childhood and adolescence: clinicopathological study of 13 cases including immunohistochemical findings. Pathol Res Pract. 1989;184422- 430
Link to Article
Fortt  RWMathie  IK Gliomatosis peritonei caused by ovarian teratoma. J Clin Pathol. 1969;22348- 353
Link to Article
Chuang  JHChen  L Ovarian teratoma with gliomatosis peritonei. J Pediatr Surg. 1992;27662- 664
Link to Article
Nielsen  SNJScheithauer  BWGaffey  TA Gliomatosis peritonei. Cancer. 1985;562499- 2503
Link to Article
Du Toit  JPDeale  CJCDavey  PMJ Immature teratoma of the ovary gliomatosis peritonei: a case report. South African Med J. 1985;671017- 1020

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