Immature ovarian teratoma with gliomatosis peritonei, paraneoplastic hyponatremia and growing teratoma syndrome: a case report and literature review.

Introduction and relevance: Paraneoplastic hyponatremia is often secondary to syndrome of inappropriate antidiuretic hormone secretion (SIADH) by tumour cells. Immature ovarian teratomas (IOT) are uncommon and may present with SIADH. Case report: A 26-year-old female presented with a 3-month history of abdominal pain and constipation. Imaging identified a mixed solid-cystic right ovarian mass containing fat and peritoneal deposits. Biochemistry showed severe, refractory hyponatremia (117 mmol/l). She underwent diagnostic fertility-preserving right salpingo-oophorectomy and resection of peritoneal nodules with the aim to achieve symptom control and hyponatraemia resolution. Pathology revealed a FIGO Stage 2 Grade 2 IOT with extensive benign peritoneal gliomatosis. Initial management was conservative. After 6 months of active follow-up, a rise in AFP, and recurrent hyponatremia supported the decision to administer three cycles of Bleomycin-Etoposide-Cisplatin chemotherapy. One month later, given radiological disease progression despite satisfactory biomarker response, cytoreductive surgery with complete macroscopic resection was performed. Pathology consisted solely of peritoneal mature glial elements: a growing teratoma syndrome (GTS). The patient remains disease-free after 2 years of surveillance. Clinical discussion: Specimen histological assessment from the patient's initial surgery showed immature neuroectodermal tubules, which are thought to be the source of vasopressin secretion. The authors hypothesise that recurrent hyponatremia and rising AFP levels represented postoperative disease relapse. Biochemical response despite radiological disease progression was pathognomonic of a GTS. Conclusion: Paraneoplastic SIADH secondary to an IOT must be considered in female patients presenting with abdominal symptoms and hyponatremia. Management requires a multidisciplinary approach. Serum electrolytes are useful surveillance biomarkers supplementary to tumour markers.

Exploring the role of SOX 2 and OCT 4 in the pathogenesis of gliomatosis peritonei: the clinicopathological profile of eleven cases.

Objective: Gliomatosis peritonei (GP) is a rare entity characterized by multiple mature glial tissue implants in association with ovarian teratomas in the peritoneum and omentum. To date, only 100 cases have been published. Not much is known about the origin, clinicopathological profile or prognosis of GP. SOX2 and OCT4 are recently recognized markers of embryonic stem cell differentiation. Here, the role of SOX2 and OCT4 in the pathogenesis of 11 cases of GP are reported and clinicopathological factors are described. Material and Methods: This was a retrospective study of six years duration (2017-2022). All the cases of GP were retrieved from archives, the diagnosis was confirmed and clinicopathological factors were noted. Immunohistochemical (IHC) investigation for glial fibrillary acid protein (GFAP) and S100 was noted wherever available. IHC for SOX2 and OCT4 was performed using an avidin-biotin technique. Results: There were 11 cases of GP identified. The median age was 29 years and 1/11 cases had nodal gliomatosis as well. There were eight cases of immature teratoma and three cases of mature cystic teratoma. SOX2 was positive in all foci of GP, while OCT4 was negative. These foci were also positive for GFAP and S100. Conclusion: A possibility of GP should be considered as a differential, clinically and radiologically, in cases of omental nodularity. Adequate sampling at the time of surgery is essential to rule out metastasis or growing teratoma syndrome. SOX2, a stem cell marker inducing neural differentiation, may play a crucial role in the development of GP in association with other transcription factors.

A Report of a Rare Case of Rapidly Progressing Immature Teratoma With Associated Splenic Metastasis and Gliomatosis Peritonei.

Gliomatosis peritonei (GP) is a rare condition of mature glial tissue within the peritoneum often associated with immature teratomas. This was a case of rapid progression of immature teratoma with splenic lesions and associated GP. The patient was a 21-year-old female who presented with abdominal pain and CT imaging showing suspected malignant teratoma. The patient underwent exploratory laparotomy with fertility-sparing debulking surgery and was diagnosed with stage IIIC grade 3 immature teratoma. She then received adjuvant chemotherapy with bleomycin, etoposide, and cisplatin. Surveillance imaging demonstrated a non-avid splenic lesion. The tumor markers remained normal. She underwent robotic splenectomy and partial peritonectomy with intra-operative findings revealing numerous peritoneal nodules. Follow-up surveillance imaging showed no further lesions. The final histopathology examination demonstrated mature and mesenchymal neural tissue consistent with residual teratoma and no immature elements. The specimens were largely composed of nodules of mature glial tissue and focal areas of mature neuronal tissue. Immunohistochemistry demonstrated glial fibrillary acidic protein (GFAP) and S100 expression, confirming neural origin tissue. Octamer-binding transcription factor 4 (OCT-4) immunostain was negative which confirmed the absence of immature neural tissue. We report a rare case of rapid progression of immature teratoma with splenic metastasis and peritoneal nodules found ultimately to be mature teratoma and associated GP. Recognition of rapidly growing teratoma with new lesions as potential GP is imperative to prevent misdiagnosis as recurrence or progression of disease. This case was treated with secondary debulking surgery which should be a consideration of management if surgically feasible.

Ovarian Teratoma Presenting With Gliomatosis Peritonei and a Melange of Symptoms Mimicking Ovarian Cancer in a Paediatric Patient.

Gliomatosis peritonei (GP) is a rare condition characterised by mature glial nodules that implant in the peritoneum, lymph nodes, or omentum. GP is typically associated with mature or immature ovarian teratomas and usually affects adolescent females. Although neuroglia may be a standard feature of mature ovarian teratomas, widespread peritoneal glial nodules, ascites, and pleural effusion are rare, particularly in the paediatric population. We report a case of a giant left mature ovarian teratoma associated with GP and omental splenunculus in a 12-year-old female who presented with constipation, an adnexal mass, ascites, pleural effusion, and elevated CA-125 levels. The patient successfully underwent fertility-sparing surgery in the form of a left salpingo-oophorectomy, omentectomy, and resection of peritoneal glial deposits. In light of the current scarcity of data on this clinical entity in the literature, we hope to raise awareness of this rare presentation of mature ovarian teratoma, the challenges associated with preoperative diagnosis, and the impact of fertility-sparing surgery on potential oncological and reproductive outcomes in a paediatric patient.

Primary immature teratoma in the liver with growing teratoma syndrome and gliomatosis peritonei: a rare case report.

BACKGROUND: Primary liver immature teratoma is extremely rare and only 4 cases have been reported, let alone with growing teratoma syndrome (GTS) and/or gliomatosis peritonei (GP). CASE PRESENTATION: Here, we report a case of a 44-year-old female presenting with progressive abdominal distension and elevated serum alpha fetal protein (AFP) level. CT/MRI scans revealed a large cystic-solid mass in the right lobe of the liver, accompanied with implant or metastasis in the abdominal cavity. Pathologic examination at biopsy suggested immature teratoma. After 4 cycles of chemotherapy, an MRI showed a slight increase in tumor size. Therefore, surgical resection of the right lobe of the liver was performed. The final histological diagnosis was a mature teratoma (tumor size 28 cm × 14 cm × 13 cm), with no residual immature component, and the diagnosis of GTS was considered. The patient continued to receive 2 courses of postoperative chemotherapy. An abdominal CT scan revealed innumerable miliary nodules in bilateral adnexal areas 2 months after surgery. Histologically, large numbers of mature glia were observed, supporting the diagnosis of GP. CONCLUSIONS: We report for the first time a case of primary liver immature teratoma with GTS and GP in an adult. Longer follow-up is needed to assess definitive efficacy.

Aggressive Gliomatosis Peritonei Arising from Ovarian Mature Teratoma with NF1 Mutation: A Case Report and Literature Review.

Background: GP arising from ovarian mature teratoma is a rare disease, and no confirmed pathogenesis signature genes are reported. The progress of GP is seen as relatively slow. Rare aggressive GP cases with poor prognosis were reported and no guidelines to follow for treatment. Case Presentation: Herein, we report a 17-year-old girl with a 3-year-history of GP arising from ovarian mature teratoma. Surgeries and drug therapy were used to treat the aggressively growing tumour. Genetic profiling revealed the pathogenic mutation with potential therapeutic approaches. We firstly reported the NF1 mutations in GP secondary to teratomas and may cause bad prognosis. Conclusion: GP arising from ovarian mature teratoma is rare; we found NF1 mutation could be the trigger of GP. The study may provide new insights into a better understanding of this rare disease.

Growing Teratoma Syndrome with Synchronous Gliomatosis Peritonei during Chemotherapy in Ovarian Immature Teratoma: A Case Report and Literature Review.

Coexistent growing teratoma syndrome (GTS) and gliomatosis peritonei (GP) arising during chemotherapy of ovarian immature teratoma (IMT) is extremely rare and can be misdiagnosed as recurrent or progressive disease. We present a 33-year-old woman diagnosed with GTS with synchronous GP during chemotherapy of IMT. She underwent ovarian cystectomy due to ovarian immature teratoma and chemotherapy were administered. The α-fetoprotein (AFP) concentration decreased from 28.7 ng/mL to normal after the second cycle. Four days after the third cycle of chemotherapy, ultrasound and CT revealed an 8-cm mass with negative tumor markers in the pouch of Douglas. An exploratory laparotomy was conducted, and a smooth round cystic-solid 8-cm mass was noted in the pouch of Douglas. Extensive peritoneal seeding glial nodules were also observed on the surface of the uterus, peritoneum, and omentum. The patient underwent a partial omentectomy, intact resection of the tumor, and resection of most of the glial nodules. Postoperative pathology demonstrated a pure mature cystic teratoma component in the mass, as well as diffuse GP involving the uterine serosa, peritoneum, and omentum; this diagnosis of GTS with synchorous GP should be considered in IMT patients with mass newly identified during chemotherapy while tumor markers are normal after treatment.

Mature ovarian teratoma with gliomatosis peritonei: A rare case report.

Gliomatosis peritonei (GP) is rarely observed along with mature ovarian teratoma. However, it is important to recognize the benign nature of GP when associated with mature ovarian teratoma. Treatment for primary tumor and long-term follow-up is vital.

Extratesticular gliomatosis peritonei after mesenteric teratoma: a case report and literature review.

Mesenteric teratoma is a rare extragonadal teratoma. Gliomatosis peritonei (GP) is mature glial tissue implanted into the peritoneum's surface and is mainly accompanied by ovarian teratoma. Only a few cases of gliomatosis have occurred in the extraperitoneum. We present a rare case of a 3-year-old boy who presented with extratesticular GP after excision of an immature mesenteric teratoma at 2 months old. After the extratesticular mass was excised, we found ductile tissue on the surface of the terminal spermatic cord and epididymis. Some ductile tissue of the epididymis was removed and sent to a laboratory for a pathological examination. The mass and the ductile tissue of the epididymis had a hard consistency. The pathological diagnosis was extratesticular gliomatosis. Complete surgical resection of the teratoma and GP is helpful for identifying the presence of malignant lesions and for preventing malignant transformation. However, characteristics of GP lesions are extensive and they are difficult to completely remove. Moreover, GP is usually benign. Therefore, the residual GP tissue was not completely removed in our case. The child is still in good health, but requires lifelong follow-up. In conclusion, we report our experience of a rare case of extraperitoneal GP from an extragonadal teratoma.

Growing Teratoma Syndrome and Gliomatosis Peritonei in a 15-Year-Old Girl With Immature Ovarian Teratoma: Case Report and Review of the Literature.

Gliomatosis peritonei (GP) is a rare clinical condition characterized by presence of mature glial cells in the peritoneum. Growing teratoma syndrome (GTS) is described as an uncommon phenomenon that could be related to the incidence of non-seminomatous germ cell tumors. We report a case of a patient treated for immature ovarian teratoma, in whom both GP and GTS were observed, an association to date scarcely described in literature. A 15-year-old girl presented to the emergency department with severe pain in her lower abdomen and right lumbar region. Upon admission, concentration of α-fetoprotein (AFP) was 1500 ng/mL and ß-human chorionic gonadotropin (ß-hCG) less than 2 ng/mL. A computed tomography (CT) scan of the abdominal cavity and pelvis confirmed the presence of an anomalous mass in the abdominal cavity and pelvis. Initial surgery was performed. Histopathology revealed the presence of immature teratoma with epithelial elements. Normalization of AFP was achieved within 8 weeks. Five months after surgery, a progressive increase in AFP was noted. Magnetic resonance imaging (MRI) and CT scans of the pelvis minor showed local relapse. Evaluation of the remission after 2 blocks of preoperative chemotherapy revealed the presence of a large tumor mass in the pelvis minor, despite normalization of the AFP concentration. After opening the abdominal walls, numerous abnormal white nodules were observed in the peritoneum. Histopathology revealed the presence of mature glial tissue (gliomatosis peritonei). The remaining tumor mass was removed, and histopathology confirmed existence of mature teratoma (growing teratoma syndrome). Postoperative chemotherapy was continued. To date, the patient remains under clinical and laboratory remission. Concomitant incidence of GP and GTS, although rare, should always be taken into consideration in pediatric patients with diagnosis of either condition.

Huge ovarian mature cystic teratoma with gliomatosis peritonei and massive ascites in a postmenopausal woman.

Teratoma of the ovary is the most frequently encountered germ cell tumor. It usually occurs in young women. Gliomatosis peritonei (GP) is mature neural glial tissue implanted onto the peritoneal surface. We present a case of a mature teratoma accompanied by GP and massive ascites in postmenopausal women. A 54-year-old, G0P0, woman presented in the gynecology outpatient department with abdominal distension for 6 months. Computed tomography scan of the abdomen and pelvis displayed an ovarian mass about 20 cm × 18 cm with peritoneal seeding, ascites, and enlarged paraaortic lymph nodes. A total hysterectomy and bilateral adnexectomy were performed. The pathology showed the left ovary contained a dermoid cyst. The biopsy of the peritoneal nodule displayed glial tissue confirming the diagnosis of GP. The patient remained in good condition 6 months postoperatively. We suggest GP be considered in patients presenting with teratomas and massive ascites. The radiological diagnosis is challenging due to the rarity of GP. Continued follow-up of patients with teratomas and GP is mandatory due to the potential of malignant transformation.

Gliomatosis peritonei with 18F-fluorodeoxyglucose accumulation and contrast enhancement secondary to immature teratoma: A case report.

Gliomatosis peritonei (GP) is a rare condition characterized by mature glial tissue implants widespread in the peritoneum, which is occasionally followed by treatment for immature teratoma (IM). The present study reported a case of GP with fluorodeoxyglucose (FDG) accumulation and contrast enhancement followed by treatment for IM. A 30-year-old female, 2-gravida and 0-para, underwent laparotomy with hysterectomy, bilateral salpingo-oophorectomy, and partial omentectomy followed by four cycles of chemotherapy with bleomycin, etoposide, and cisplatin for IM (Grade 2) of stage IIIC. At the 6-month follow-up, computed tomography (CT) revealed a 1-cm mass with contrast enhancement on splenic flexure. Positron-emission tomography (PET)/CT revealed intense FDG accumulation at the same site. Although α-fetoprotein, which was elevated preoperatively, remained normal, she was diagnosed with IM recurrence. The patient underwent three cycles of chemotherapy with paclitaxel, ifosfamide, and cisplatin, but the size, the degree of contrast enhancement and FDG accumulation of the mass did not change after chemotherapy. A diagnostic laparoscopy was performed. which revealed multiple small peritoneal implants, including a 1-cm mass at the splenic flexure. The 1-cm mass was dissected at the splenic flexure and some other implants. Mature well-differentiated glial tissue with non-atypia was identified in all tissue, and a diagnosis of GP was made. The patient is currently undergoing regular follow-up. Few reports are available regarding FDG-PET/CT imaging of GP. GP should be considered as the differential diagnosis of FDG-avid mass followed by IM therapy. A laparoscopic diagnosis is useful to obtain an accurate diagnosis of GP.

Gliomatosis peritonei with bilateral ovarian teratomas: A report of two cases.

Gliomatosis peritonei (GP) is characterized by the presence of benign, mature neuroglial implants throughout the peritoneum and is typically accompanied by mature or immature ovarian teratomas. GP is a condition that has only been described relatively recently, with ~100 cases reported in the English literature. The majority of reported cases have focused on the pathology and clinical treatment of the disease; radiological findings are distinct, but the discussion of this is scarce in the literature. The current study presents two cases of GP with bilateral ovarian teratomas and provides a review of the relevant literature, with particular emphasis on the radiological differential diagnosis. The present study reinforces previously reported observations from imaging analysis and suggests that radiological investigation alone cannot sufficiently aid the differentiation of benign glial deposits from diffuse peritoneal malignant seeding. However, radiologists should be familiar with this rare condition in order to provide an accurate diagnosis, particularly in ovarian tumor staging, which may markedly impact the administered treatment. It is recommended that doctors undertake long-term follow-ups in patients presenting with GP due to the potential for malignant transformation.

Gliomatosis peritonei: a series of eight cases and review of the literature.

BACKGROUND: Gliomatosis peritonei (GP) is a rare condition characterized by mature glial tissue implants widespread in the peritoneum. The GP is often associated with ovarian teratoma. However, little is known about the characteristics and prognosis of GP. The purpose of this study was to describe the features, treatment, and prognosis of GP. Additionally, we review previously reported cases of GP, summarizing the presently known data. METHODS: From January 2000 to January 2016, cases of ovarian teratoma and GP treated at Peking Union Medical College Hospital were reviewed. We assessed the pathology, treatments, and outcomes along with prognostic information. Additionally, the literature regarding this clinical condition was also reviewed. RESULTS: Eight patients met the inclusion criteria. Patients had a median age of 20 (range, 15-25) years. GP was diagnosed as the primary tumor in 6 patients and at a secondary surgery in two patients. The primary ovarian tumor consisted of immature teratoma (n = 7) and mature teratoma (n = 1). Grades of immature ovarian teratoma were 2, grade 1; 3, grade 2; and 2, grade 3. Tumors mean had a size of 20.4 (range, 11-30) cm. The median follow-up time was 60.5 (range, 3-144) months. All cases had conservative surgery and seven of them had macroscopic residual disease postoperatively. During the study period, the eight patients remained alive and asymptomatic. Three patients in the study experienced spontaneous pregnancy. After reviewing the existing literature, a total of 14 patients with nodal gliomatosis were present and 10 of them were alive. According to the literature review, five articles reported more than five cases. Of a total of 67 patients, 60 of them remained alive. CONCLUSION: The prognosis of immature ovarian teratoma with GP is favorable. Complete resection of GP is often difficult. Residual peritoneal disease in GP can be asymptomatic and quiescent over a long period. A more conservative surgical approach may be carried out in patients with massive peritoneal spread after the presence of metastatic immature elements is excluded. Owing to the risk of recurrence and malignant transformation of GP, a long-term follow-up is necessary for patients with residual peritoneal disease.

Ovarian immature teratoma with gliomatosis peritonei and pleural glial implant: a case report.

Gliomatosis peritonei is a very rare phenomenon occurring almost exclusively in association with ovarian immature teratoma. It is characterized by numerous benign, mature glial nodules in the omentum and mesentery. The presence of glial tissue outside abdominal cavity is extremely rare in the setting of ovarian immature teratoma. We present a case of ovarian immature teratoma with both gliomatosis peritonei and pleural glial implant in a 4-year-old girl. Glial emboli were present in the pleural implant, suggesting lymphovascular dissemination might be the cause of extra-abdominal glial implantation.

Peritoneal and nodal gliomatosis with endometriosis, accompanied with ovarian immature teratoma: a case study and literature review.

Gliomatosis peritonei (GP) indicates the peritoneal implantation of mature neuroglial tissue and is usually accompanied by ovarian mature or immature teratoma. Here, we report a case of ovarian immature teratoma associated with gliomatosis involving the peritoneum, lymph nodes and Douglas' pouch, where gliomatosis coexisted with endometriosis. As far as we know, only seven cases of GP have been reported as coexisting with endometriosis. Eight cases with mature glial tissue in the lymph nodes, i.e., nodal gliomatosis, have been published either in association with GP or in its absence. Metaplasia of pluripotent coelomic stem cells has been suggested to be responsible for the pathogenesis of endometriosis and GP rather than implantation metastases of ovarian teratomatous tumor with varying maturation. This theory is also applied to GP independently of ovarian teratomatous tumors. To the best of our knowledge, nodal gliomatosis coexisting with GP and also involving endometriosis has not yet been reported.

Peritoneal and Nodal Gliomatosis with Endometriosis, Accompanied with Ovarian Immature Teratoma: A Case Study and Literature Review

Gliomatosis peritonei (GP) indicates the peritoneal implantation of mature neuroglial tissue and is usually accompanied by ovarian mature or immature teratoma. Here, we report a case of ovarian immature teratoma associated with gliomatosis involving the peritoneum, lymph nodes and Douglas' pouch, where gliomatosis coexisted with endometriosis. As far as we know, only seven cases of GP have been reported as coexisting with endometriosis. Eight cases with mature glial tissue in the lymph nodes, i.e., nodal gliomatosis, have been published either in association with GP or in its absence. Metaplasia of pluripotent coelomic stem cells has been suggested to be responsible for the pathogenesis of endometriosis and GP rather than implantation metastases of ovarian teratomatous tumor with varying maturation. This theory is also applied to GP independently of ovarian teratomatous tumors. To the best of our knowledge, nodal gliomatosis coexisting with GP and also involving endometriosis has not yet been reported.

A case of Gliomatosis Peritonei after 10 years following Operation of Immature Teratoma of the Ovary / 부인종양

Gliomatosis peritonei, the implantation of neuroglial tissue upon the peritoneal surface, is a rare event most often associated with solid or immature teratoma of the ovary in young girls. The majority of cases occur in association with teratomas containing immature element. However malignant transformation of the glial tissue has been reported. Here we experienced a case of mature glial implants presenting in an 27-year-old female, 10 years after initial diagnosis and removal of an ovarian immature teratoma, and report this case with brief review of literatures.

A Case of Immature Teratoma of the Ovary with Gliomatosis Peritonei / 대한산부인과학회잡지

Immature teratoma is composed of varying quantities of immature differentiating among anyone or all three germ layer. The pure immature teratoma accounts for fewer than 1% of all ovarian cancer, but it is the second most common germ cell malignancy. About 50% of pure immature teratomas of the ovary occur in women between the ages of 10 and 20 years, and they rarely occur in postmenopausal women. The most frequent site of dissemination is the peritoneum, and much less commonly, the retroperitoneal lymph node. Among the tumors with embryonal elements, those containing neural tissues demonstrate most clearly the importance of the ability to mature. Gliomatosis peritonei is the most dramatic demonstration of the significance of maturation, because most patients with these tumors have survived, even with this disseminated disease. The purpose of this paper is to report on a immature ovarian teratoma with predominantly mature glial tissues in peritoneum which we have experienced in this hospital recently with brief review of the literature.

A Case of Gliomatosis Peritonei / 대한산부인과학회잡지

Gliomatosis peritonei is the implantation of glial tissue with the piertoneal cavity associa-ted with ovarian teratoma. Although the mechanism of implantation is unknown, it probably occurs most commonly from extrusion of mature glial tissue through capsular defects in the tumor. Previous reports have emphasized improved outcomes when these implants are found to be mature, even if the ovarian component is immature. We experienced one case of ovarian immature teratoma(Grade I) with gliomatosis peritonei, which was treated with conservative surgery.