Growing teratoma syndrome of the ovary: review of literature and first report of a carcinoid tumor arising in a growing teratoma of the ovary

We report the first case of a secondary tumor arising from a peritoneal nodule of mature teratoma in a patient with growing teratoma syndrome (GTS) of the ovary. The patient originally presented 19 years ago with an immature teratoma of the ovary and positive retroperitoneal lymph nodes. After surgery and chemotherapy, mature teratomas recurred as abdominal and pelvic masses after 1, 6, and 19 years. Upon the last recurrence, a trabecular carcinoid tumor developed in a mature teratoma associated with the liver. This case illustrates the importance of long-term follow-up for patients with GTS of the ovary, where the recurrent masses can appear many years after the primary tumor, compress the abdominal and pelvic structures and give rise to secondary neoplasms. In addition, we present a literature review of GTS of the ovary and some novel observations about this entity. On the basis of our review of ovarian GTS cases in the literature, we have found that ovarian GTS nodules tend to appear for the first time within 2 years of the initial primary. They remain confined almost exclusively to the pelvis, abdomen, and the retroperitoneum and do not venture to distant systemic sites. This new information may help identify and screen women with germ cell tumors of the ovary at risk for GTS.     

Teratoma following cisplatin-based combination chemotherapy for nonseminomatous germ cell tumors: a clinicopathological correlation

From April 1975 through May 1981, 51 patients had teratoma resected from residual disease following cisplatin-based combination chemotherapy. All patients had normal serum markers before resection of abdominal (25), lung (12), mediastinal (5), thoracoabdominal (8) or other (1) disease. Teratoma was classified as mature in 29 cases, immature in 15 or immature with nongerm cell elements in 7. Of the 51 patients 31 (61 per cent) remain free of recurrent disease, while 20 either had recurrent carcinoma (10) or teratoma (10) requiring further therapy. Nine patients died, including 1 in whom angiosarcoma developed, which was thought to be secondary to previous radiation therapy. In 4 patients the initial relapse of carcinoma developed beyond 2 years after resection. Univariate factors predicting for relapse include tumor burden, immature teratoma with nongerm cell elements and site (mediastinum), while only immature teratoma with nongerm cell elements and site predicted for survival. Patients with immature teratoma had a comparable relapse-free and over-all survival as those with mature teratoma. Using a multivariate analysis, primary tumor site at the mediastinum was the most significant adverse factor predictive for relapse and survival. This study appears to support the various pre-clinical models that demonstrate multipotential capabilities of teratoma. Complete surgical excision of teratoma remains the most effective treatment with continued close followup recommended for high risk patients (immature teratoma with nongerm cell elements, large tumor burden and primary mediastinal tumors) with resected teratoma.     

Immature teratoma of the ovary with gliomatosis peritonei. A case report

Gliomatosis peritonei is a rare complication of immature teratoma of the ovary and should not be confused with metastatic ovarian carcinoma. Treatment depends on the histological grading of the gliomatous lesions. All grades, except grade 0, qualify for adjuvant chemotherapy. Repeated laparotomies for cytological sampling and the removal of tumour are essential. A 16-year-old Ovambo nulligravida presenting with gliomatosis peritonei was apparently cured after 5 laparotomies for removal of tumour and 13 courses of combination chemotherapy.     

Resection of thoracic and abdominal teratoma in patients after cisplatin-based chemotherapy for germ cell tumor. Late results

Fifty-one patients with primary testicular (N = 46) or mediastinal germ cell cancer (N = 5) were treated from April, 1975, through May, 1981, and had teratoma resected from residual disease after cisplatin-based combination chemotherapy. All patients had normal serum markers before resection of pulmonary (N = 12), mediastinal (N = 5), thoracoabdominal (N = 8), supraclavicular (N = 1) or abdominal disease (N = 25). Teratoma was classified as mature teratoma (N = 29), immature teratoma (N = 15), or immature teratoma with non-germ cell elements (N = 7). Thirty of 51 (60%) patients remain free of recurrent disease, whereas 20 patients have either recurrent carcinoma (N = 10) or teratoma (N = 10). One patient has a presumed second malignancy. After additional chemotherapy, four patients with recurrent carcinoma are alive and disease free and six have died. After an additional operation, eight of 10 patients with recurrent teratoma are long-term survivors. In four patients the initial relapse of carcinoma developed more than 2 years after therapy; in an additional patient carcinoma recurred after a 32 month disease-free survival period. Univariate factors predicting for relapse include tumor burden, immature teratoma with non-germ cell elements, and site (mediastinum), whereas only immature teratoma with non-germ cell elements and site predicted for survival. Immature teratoma and mature teratoma had similar relapse-free intervals and overall survival intervals. According to a multivariate analysis, primary tumor site at the mediastinum is the most significant adverse factor predictive for both relapse and survival (two of five patients survived). This study appears to support the various preclinical models that demonstrate multipotential capabilities of teratoma. Complete surgical excision of teratoma remains the most effective treatment with continued close follow-up recommended for high-risk patients (immature teratoma with non-germ cell elements, large tumor burden, or primary mediastinal tumors.     

Primary immature teratoma of the prostate with angiosarcoma component: Its unusual response to chemotherapy

Here, we present a case of immature teratoma of prostatic origin, which included an angiosarcoma component. This mass proved unresponsive against preoperative chemotherapy for immature teratoma. Angiosarcoma, although quite rare, should be included in the differential diagnosis as a component of prostatic teratoma, especially in cases in which the tumor proves unresponsive to well-known chemotherapeutic protocols. This is, to our knowledge, the first reported case of primary prostatic immature teratoma containing an angiosarcoma component.     

41例卵巢成熟性囊性畸胎瘤腹腔镜手术治疗

目的　探讨卵巢成熟性囊性畸胎瘤腹腔镜手术的技巧和安全性。　方法　对 4 1例卵巢成熟性囊性畸胎瘤患者在腹腔镜下行肿物剔出术或附件切除术 ,采用自制标本袋盛装卵巢肿物 ,避免囊肿内容物污染盆腔。　结果　 4 1例中 4 0例在腹腔镜下完成手术 ,1例因手术困难而中转开腹手术。术后随访 4月～ 6年 ,均未见复发。　结论　腹腔镜手术治疗卵巢成熟性囊性畸胎瘤是一种安全、有效的方法 ,具有出血少、损伤小、恢复快、无远期并发症等优点。     

Rhabdomyosarcoma in a patient treated for metastatic germ cell tumour of the testis containing teratoma--a case report

A patient who developed a rhabdomyosarcoma following apparently successful chemotherapy for metastatic germ cell testicular carcinoma is presented. This newly recognized association may be seen particularly in patients whose initial germ cell malignancy contains immature teratoma. Possible reasons for this are discussed. The findings in this patient suggest that re-biopsy of recurrent disease be undertaken wherever possible, particularly where immature teratoma was a feature of the initial histopathology. A proportion of relapsing patients as described may not in fact have recurrent germ cell malignancy, but may have developed high grade, and often chemoresistant sarcomas. These second tumours appear to have an extremely poor prognosis, unless amenable to complete surgical resection.     

Suprasellar malignant teratoma with 11-year survival--case report.

Long-term survival of malignant teratoma is rare. A 16-year-old female with a suprasellar malignant teratoma survived for 11 years before succumbing to embryonal carcinoma. A suprasellar immature teratoma with increased serum alpha-fetoprotein level was verified histologically. Radiation therapy and combined chemotherapy were given after partial removal. Eleven years later, the primary lesion recurred as embryonal carcinoma.     

RHABDOMYOSARCOMA IN A PATIENT TREATED FOR METASTATIC GERM CELL TUMOUR OF THE TESTIS CONTAINING TERATOMA—A CASE REPORT

A patient who developed a rhabdomyosarcoma following apparently successful chemotherapy for metastatic germ cell testicular carcinoma is presented. This newly recognized association may be seen particularly in patients whose initial germ cell malignancy contains immature teratoma. Possible reasons for this are discussed. The findings in this patient suggest that re-biopsy of recurrent disease be undertaken wherever possible, particularly where immature teratoma was a feature of the initial histopathology. A proportion of relapsing patients as described may not in fact have recurrent germ cell malignancy, but may have developed high grade, and often chemoresistant sarcomas. These second tumours appear to have an extremely poor prognosis, unless amenable to complete surgical resection.     

Sacrococcygeal teratoma of newborn infants; a report of two cases]

Two cases of sacrococcygeal teratoma in female infants are reported. Case 1. A newborn baby with a hemispheric mass on her hip underwent surgery 3 days after birth and the lesion proved to be an immature teratoma. The serum AFP level was very high but became normal one month after the operation. The child also had agenesis of the corpus callosum and arachnoid cysts in right middle fossa. She died after developing shunt infection. Case 2. A newborn baby with a mature teratoma was operated on the day following birth. The tumor was subtotally removed, and there has been no recurrence after 6 months. Sacrococcygeal teratoma in female infants is often associated with agenesis of the corpus callosum and arachnoid cysts. They tend to develop almost at the same time as the teratoma. It is often difficult to determine whether the infant should be treated by chemotherapy or not if the teratoma is immature, especially when it has been totally removed, the serum AFP is normal, and the tumor is pathologically of a low grade.     

Chemotherapy of disseminated germ cell testicular tumors with cis-diamminedichloroplatinum and other drugs

The effect of single CDDP therapy and PVB therapy was examined in 7 cases of stage III germ cell testicular tumors with measurable metastases. The mean age of the patients was 30.6 years old, and their histological types of primary sites were seminoma in 3 cases, embryonal carcinoma in 2, immature teratoma in 1 case and embryonal carcinoma + teratoma in 1 case. In 1 case of seminoma, 375 mg of CDDP was administered. In 1 case of embryonal carcinoma + teratoma, 100 mg of CDDP and then 2 courses of PVB therapy were performed, and 3 courses of PVB therapy were given in all other cases. Three cases showed complete response, 2 cases partial response and 2 cases no change. Pulmonary metastatic nodules were extirpated after the PVB therapy in 1 of the cases showing no change, and the histological examination of these nodules was found to be mature teratoma. As a result, the effectiveness of the chemotherapy alone was 71.4%, and that of chemotherapy + surgical operation was 85.7%. Significance of intensive chemotherapy and necessity of extirpation of residual metastatic nodules after intensive chemotherapy in the management of advanced germ cell testicular tumors are stressed.     

Sacrococcygeal teratoma: A 33-year experience

During the years 1941 through 1973, 48 patients, 16 males and 32 females, with sacrococcygeal teratoma were seen at the Childrens Hospital of Los Angeles. Forty-four patients have been followed, three are lost to follow-up, and one patient died 2 wk after excision of teratoma.Of the 44 patients with follow-up, 26 had teratoma with mature tissues only, all these patients are living. Six patients had tumor containing mature and embryonic tissues. Of these, five are living and one died with metastases of malignant teratoma 1 yr after excision of the primary tumor. Of the remaining 12 patients, 11 have died during the first 4 yr of life due to malignant teratoma and only one is living without recurrence 15 yr after excision of teratoma containing frankly neoplastic tissues. Recurrence and/or metastasis of malignant sacrococcygeal teratoma was lethal in all instances.     

Treatment of mediastinal immature teratoma in a child with precocious puberty and Klinefelter's syndrome

Teratoma is the most common germ cell tumor, which can be divided into the mature and the immature histologically. Concurrent Klinefelter's syndrome may be overlooked in a patient with a germ cell tumor. This is because the tumor that secrets alpha-fetoprotein and beta human chorionic gonadotropin can mimic puberty in a patient with Klinefelter's syndrome, masking the usual clinical signs. In reviewing the literature on the subject, the role of neoadjuvant and adjuvant chemotherapy remains ill-defined for the immature teratoma. Age-dependent prognosis seems to demonstrate that children with immature teratomas have a better outcome. We share the experience of treating a child with immature teratoma with surgical excision alone, and it ended in a local recurrence.     

Nephroblastoma arising in a germ cell tumor of testicular origin

We report a nephroblastoma arising in a germ cell tumor of testicular origin occurring in a 22-year-old man. Orchiectomy demonstrated a malignant mixed germ cell tumor composed of mature and immature teratoma with nephroblastoma and rhabdomyosarcoma. Following chemotherapy, the patient developed supraclavicular and retroperitoneal lymphadenopathy. Excision demonstrated metastatic teratoma at both sites. No recurrence was noted with 21 months of additional follow-up. Using tissue microdissection and loss of heterozygosity analysis, we investigated the clonality of the mature teratoma, immature teratoma, nephroblastoma, and rhabdomyosarcoma components of the primary tumor and of the metastatic mature teratoma at the two separate distant sites. Nine microsatellite polymorphic makers were used to examine the pattern of allelic loss in both primary and metastatic tumors. Loss of heterozygosity was found in 4 DNA loci, and the same pattern of allelic loss was demonstrated at all 4 loci in all of the different components of the primary tumor and the metastatic mature teratomas, supporting the germ cell tumor origin of the nephroblastoma component. Loss of heterozygosity on chromosome 17p13 (TP53) was detected in metastatic mature teratoma, but not in the primary tumor. Loss of heterozygosity was observed at 11p13, the locus of WT1 inactivation in patients genetically predisposed to nephroblastoma, and this loss may be an important genetic mechanism in nephroblastomatous differentiation of germ cell tumors. These data support a common clonal origin for nephroblastoma and the other germ cell tumor components.     

Recurrence of intracranial germinoma initially treated with chemotherapy only

Irradiation for intracranial germinoma may be associated with significant neuroendocrinological sequelae, so the establishment of lower effective doses of radiation is desirable. Eight consecutive patients with intracranial germinoma underwent combination chemotherapy with BEP (bleomycin, etoposide, and cisplatin) or PE (cisplatin and etoposide) without irradiation between 1996 and 1997. The diagnosis was based on endoscopic or stereotactic biopsy, or transsphenoidal surgery in 7 cases. These 7 patients obtained complete response (CR) after treatment with chemotherapy only. The diagnosis of pure germinoma was based on neuroradiographic appearances and the normal levels of tumor markers in patients with suprasellar and pineal-region germ cell tumors. Although all patients obtained CR, 5 patients relapsed at a mean period of 19 months after the initial therapy. The mean follow-up period was 53 months. Two of these 5 patients had recurrence around the ventricle wall. Additional chemotherapy and total ventricle irradiation (24 Gy) achieved CR, but delayed seeding in the optic nerves outside the irradiated field was detected. One patient suffered peritoneal dissemination via a ventriculoperitoneal shunt with intracranial dissemination. Another patient obtained CR after PE therapy, but there was not histological verification. However, 35 months after the initial therapy, surgical extirpation of the recurrent tumor revealed immature teratoma. One patient refused treatment for recurrence at the initial tumor site and died 42 months after the initial treatment. Adequate initial therapy is absolutely essential for the treatment of intracranial germinoma. The chemotherapy regimens in use today cannot be recommended as initial therapy without irradiation because of the high recurrence rates.     

En Bloc Aortic Resection for Bulky Metastatic Germ Cell Tumors

Between 1989 and 1993, 97 patients with stages B3 and/or C nonseminomatous germ cell tumors of the testes underwent induction chemotherapy followed by retroperitoneal lymph node dissection. Of these patients 6 (ages 22 to 41 years) had gross extension of tumor into the aortic adventitia at operation, which necessitated en bloc aortic and, on 3 occasions, iliac artery resection for complete tumor removal. Aortic continuity was restored by a woven Dacron tube or bifurcated graft. All grafts were covered with omentum. There were no postoperative vascular complications. Pathological study of the residual retroperitoneal disease demonstrated that 2 patients had mature teratoma only, 2 had mature teratoma with occasional nests of immature teratoma and 2 had residual yolk sac, embryonal or choriocarcinoma elements. The latter 3 patients underwent postoperative salvage chemotherapy with varying combinations of bleomycin, etoposide, ifosfamide and cisplatin. At 4 to 55 months 4 patients were disease-free, while 2 died of metastatic disease. No problems related to the aortic reconstruction have occurred. This small experience demonstrates that, if necessary, complete surgical en bloc extirpation of bulky metastatic germ cell tumors and the aorta/iliac artery can be performed safely with a satisfactory long-term outcome.     

Growing mediastinal metastatic tumour in a patient with burned out testicular cancer

A 27-year-old man was referred to us for resection of a post-chemotherapy growing mass in the mediastinum. He had been treated with 4 courses of combination chemotherapy for his testicular cancer. The primary lesion was burned out and the anterior mediastinal metastatic tumour contained immature teratoma. After normalization of tumour marker levels, the mediastinal mass was resected completely and the resected specimen showed teratoma with rhabdomyosarcoma. Two rare clinical conditions have been seen in this case.     

The role of thoracotomy in managing postchemotherapy residual thoracic masses in patients with nonseminomatous germ cell tumours

OBJECTIVE: To evaluate the clinical outcome and identify prognostic variables in patients with nonseminomatous germ cell tumours undergoing postchemotherapy thoracotomy for residual masses, as the role of this procedure is controversial. PATIENTS AND METHODS: Of 385 patients who underwent postchemotherapy retroperitoneal lymph node dissections between 1988 and 1998, 105 also had 130 thoracotomies. The clinical presentation, chemotherapy regimens, marker status, primary tumour histology, pathology of all resected masses, and clinical outcome of these 105 patients were analysed. RESULTS: The overall discordance rate for synchronous thoracic and retroperitoneal masses was 28%; that for asynchronous thoracic and retroperitoneal masses was 57%. Independent prognostic factors for residual thoracic teratoma or cancer were teratoma (mature or immature) in the primary tumour or retroperitoneal teratoma or cancer. Although three of 12 patients with residual thoracic cancer remained with no evidence of disease, residual thoracic cancer is an independent prognostic factor (P < 0.001) against disease-free survival. CONCLUSION: Postchemotherapy thoracotomy yields important prognostic information, and is therapeutic for most patients with teratoma and a subset with residual viable cancer. The prognostic criteria predictive of fibrosis are not sufficiently accurate to omit resection of residual thoracic masses.     

The management of metastatic germ cell tumours and the clinical utility of lactate dehydrogenase estimations

Forty-five patients with metastatic germ cell tumour were treated with chemotherapy. Complete remission was achieved in 63% of all cases and in 65% of patients whose primary tumour arose in the testis or ovary. Surgical resection of abdominal masses persisting after chemotherapy was performed in seven patients, two of whom were found to have persistent tumours. Twenty-seven of the 33 patients with teratoma originating in the gonads remain in complete remission. Total serum LDH activity was elevated in 28 of the patients with measurable disease. The increased LDH was not accompanied by significant alteration in other hepatic enzymes nor were hepatic metastases demonstrable in these patients. Fractionation of the LDH demonstrated that the increased LDH in these patients was located in either iso-enzymes 1 or fractions 1 + 2. Alteration of the serum LDH activity correlated with the response to therapy and warrants further study.