Five-year remissions of metastatic solid teratoma of the ovary

To date, only three 5-year survivors with metastatic solid malignant teratoma of the ovary have been reported. The authors report two additional cases surviving 5 years or greater. In both cases, the embryonal teratomatous elements matured to Grade 0 peritoneal metastases after intensive chemotherapy and surgery with or without radiotherapy. Since only one of the two patients had an objective response to therapy, we speculated that the maturation of the tumor in both patients might be, but is not proven to be, a consequence of chemotherapy, surgery, and/or radiotherapy. Because the prognosis, even in Stage IA solid ovarian teratoma, is so poor, we suggest that all such patients should have 2 years of adjuvant chemotherapy consisting of vincristine, dactinomycin, and cyclophosphamide. Patients with demonstrable metastases should receive the same chemotherapy until either all tumor has regressed or all immature elements have reverted to mature tissue.     

Sacrococcygeal germ-cell tumours--the Red Cross War Memorial Children's Hospital experience, 1980-1996

OBJECTIVE: To document the experience of Red Cross War Memorial Children's Hospital in the treatment of sacrococcygeal germ-cell tumours. PATIENTS: Twenty-seven patients with sacrococcygeal germ-cell tumours were treated in our hospital from 1980 to 1996. DESIGN: A retrospective review of these patients' records was undertaken. RESULTS: There were 19 female and 8 male patients. Seventeen (63%) presented in the neonatal period, 13 on the first day of life. Complete surgical resection of the tumour was achieved in all patients with mature or immature teratomas (20 patients) and in 2 neonates with malignant tumours. The first of these 2 neonates, with a malignant teratoma, was not given chemotherapy and remains well 10 years later. The second, with a yolk-sac tumour, also received no initial chemotherapy. He relapsed at the age of 9 months and was successfully treated with repeat excision and chemotherapy. All 5 patients first diagnosed after the age of 1 year had malignant tumours. These patients had incomplete surgical resection (3) or biopsy only (2), and 3 were successfully treated with chemotherapy. One patient relapsed with yolksac tumour after initial complete resection of a mature teratoma. She was successfully treated with repeat surgery and chemotherapy.     

Covert bilaterality of mature ovarian teratomas

Over a 21-year period, 213 women with mature teratoma of the ovary were operated on at Parkland Memorial Hospital. Twenty-seven had bilateral tumors. In 22 of these 27 patients, bilateral involvement was evident on clinical examination, and in 5, biopsy of an abnormal but nonteratoid appearing ovary was required to make the diagnosis. Ninety patients with a visually normal opposite ovary had no identifiable tumor in that ovary by investigative incision or incidental excision. Neither incision nor excision of the normal contralateral ovary was performed in 58 cases, and subsequently, one of these women is known to have developed a teratoma in that ovary. We conclude that synchronous covert bilaterality of mature teratomas is not common, and a visually normal contralateral ovary should not routinely be bivalved or wedge biopsied.     

Immature teratoma of the ovary with a neural component ("solid" teratoma). A clinicopathologic study of 20 cases

Twenty cases of immature teratoma of the ovary with a neural component are analyzed. A plea is made for use of the nomenclature adopted from the new World Health Organization classification of ovarian tumors, the past confusion over terminology and histogenesis of this rare tumor is discussed. All the primary tumors in the present series contained at least some immature tissues (predominantly of neural origin) and were thus graded from 1 to 3 according to the criteria of Thurlbeck and Scully. No grade 0 tumors ("benign solid teratomas") were identified. We believe that thorough sectioning almost always insures the identification of immature elements. The prognosis was closely related to the histologic grade, but correlated poorly withthe clinical stage, the latter being influenced by the common finding (25 per cent of the cases in this series) of peritoneal implants composed exclusively of mature glial tissue, which is associated with a benign clinical evolution. This phenomenon of maturation or differentiation appears to be the rule rather than the exception in this tumor, since implants are usually of better or equal differentiation when compared with their primary tumors and older patients tend to have lower grade tumors than younger patients. Since the majority of patients with this tumor are young, primary surgical therapy should be conservative, unilateral salpingooophorectomy often being sufficient. Spontaneous or operative rupture of the tumor capsule carries an increased risk of subsequent dissemination. We have noted impressive clinical responses in patients with disseminated tumors of a high histologic grade after treatment with triple chemotherapy (vincristine, actinomycin D, and cyclophosphamide) but do not recommend adjuvant therapy in patients with only grade 0 implants.     

Neuroblastoma arising in testicular teratoma

One case of neuroblastoma arising in an adult immature testicular teratoma is described, with multiple systemic metastases, a partial response to intensive chemotherapy and a swift recurrence leading to death. Such instances of prevailing neuroblastoma with systemic metastases, have only seldomly been reported hitherto. Because of the teratoma and the focal presence of intratubular germ cell neoplasia of unclassified type, we think this tumor must be indeed of germ cell derivation.     

Botulinum toxin in the therapy of gustatory sweating

A case of mucin-producing adenocarcinoma arising from a mature cystic teratoma of the right ovary is reported in a 67-year-old multiparous Japanese woman. The patient underwent total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and adjuvant chemotherapy containing cisplatin, Adriamycin, and cyclophosphamide with the diagnosis of ovarian cancer, probable stage Ic. Treatment was successful even though the tumor extended through the cyst wall.     

The intermittent ductus revisited: echocardiographic evidence and successful coil occlusion: a case report and review of literature

We report the case of a 41-year-old woman with ovarian mixed germ cell tumor which was composed of polyembryoma and immature teratoma and who had high serum levels of alpha-fetoprotein (AFP) and human chorionic gonadotropin (hCG). By immunohistochemical methods, AFP was found in yolk sac cells of the embryoid bodies and immature hepatoid tissues, and hCG was found in giant syncytiotrophoblastic cells. She was treated with surgery followed by cisplatin-based combination chemotherapy. She is well, and her serum levels of AFP and hCG have not been elevated for more than 4 years after the treatment.     

Postchemotherapy residual masses in germ cell tumor patients: content, clinical features, and prognosis. Medical Research Council Testicular Tumour Working Party

BACKGROUND: In a retrospective study that included a detailed histopathologic review, the clinicopathologic features of patients with germ cell tumors (GCT) and resectable residual masses after chemotherapy were assessed. METHODS: Histologic material from 153 patients was available for review. Recorded details included primary histologic diagnosis, location, size and number of metastases, marker levels before and after chemotherapy, and completeness of surgical excision. A median of seven histologic sections per resection were reviewed by two pathologists independently (and together when disagreement occurred). In each case, details were recorded regarding fibrosis, necrosis, hemorrhage, embryonal carcinoma (undifferentiated teratoma), yolk sac tumor, choriocarcinoma (trophoblastic tumor), differentiated teratoma (mature and immature), dysplasia in somatic tissues, and non- germ cell tumor (GCT) malignancies. The percentage of the sample that each of these components comprised was also estimated. RESULTS: The median postchemotherapy follow-up time was 7 years, and 38 of 153 patients (25%) experienced disease progression. In a multivariate analysis, incomplete resection of all residual masses (in 38 patients) and the presence of malignant elements (in 23 patients) were independent risk factors for progression. In the subset of patients in whom all masses were completely resected, the presence of embryonal carcinoma (undifferentiated teratoma) was the single most significant risk factor for progression. Seven percent of patients had this factor, which was associated with a 2-year progression free survival rate of 12.5%, compared with 88.0% where this component was absent. CONCLUSIONS: Progression free survival can be predicted well by the completeness of excision of residual masses and the presence of malignant germ cell elements. The latter confers a relatively poor prognosis even if all of these elements are completely resected.     

Mediastinal teratomas: review of 15 pediatric cases

One hundred fifty-three children with a teratoma presented to one hospital between 1970 and March 1992. The clinical and pathological features of 15 patients with mediastinal teratomas are reviewed; six were newborn and nine aged from infancy to 13 years. Thirteen patients including the six newborns presented with respiratory distress and all 15 patients had a mass on chest radiograph. A definite diagnosis of teratoma was not made preoperatively in any of these patients. At operation, a median sternotomy was used to approach seven anterior tumors and a lateral thoracotomy performed in the other eight patients. Histologically two were mature, 10 had immature elements, and three were malignant teratomas. The patients with malignant tumors were all over 12 years of age and died within 6 months of treatment. All six neonates had immature teratomas. Raised serum alpha-fetoprotein levels provided useful markers in two patients with recurrent tumors. Three conclusions can be drawn: (1) mediastinal teratomas are rare in children and frequently are not diagnosed before operation; (2) in newborns these tumors may be immature and present with respiratory distress; and (3) a median sternotomy gives excellent exposure for anterior mediastinal tumors.     

Mature teratoma identified after postchemotherapy surgery in patients with disseminated nonseminomatous testicular germ cell tumors: a plea for an aggressive surgical approach

BACKGROUND: Mature teratoma is often found in resected retroperitoneal residual tumor masses (RRTM) after chemotherapy for disseminated nonseminomatous testicular germ cell tumors (NSTGCT). The aim of this report is to describe the clinical course of patients after resection of residual teratoma, with particular emphasis on relapse with either growing mature teratoma or secondary non-germ cell malignancy. METHODS: During the period 1979-1995, 113 patients underwent a laparotomy for resection of RRTM after chemotherapy for NSTGCT. Only patients with mature teratoma in the RRTM were included in the current study, and data on the patients who experienced relapse were studied in detail. RESULTS: Mature teratoma was found in 51 patients (45.1%) with RRTM resected after chemotherapy. Nine of these 51 patients (17.6%) relapsed; the relapses resulted from growing mature teratoma in 5 patients (9.8%), secondary non-germ cell malignancy in 3 patients (5.9%), and recurrent germ cell malignancy in 1 patient (2.0%). The primary treatment for all relapsing patients was surgical excision. All five patients with growing mature teratoma are alive without evidence of disease, as is the patient with recurrent germ cell malignancy. One of the three patients with non-germ cell malignancy died of disease, and the remaining two are alive with disease. CONCLUSIONS: Long term follow-up after resection of postchemotherapy residual teratoma is indicated because a proportion of patients develop growing mature teratoma or a secondary non-germ cell malignancy. The treatment for these recurrences should be complete surgical excision.     

Pure immature teratoma of the ovary: analysis of 22 cases

Between 1970 and 1991, 22 patients with pure immature teratoma were treated at the Center of Oncology in Krakow. Sixteen (72.7%) patients had stage I, four (18.2%) stage II, and two (9.1%) stage III of disease, nine (40.9%) patients had grade 1, 11 (50%) grade 2, and two (9.1%) grade 3 tumors. Eight stage Ia, grade 1 patients were treated with surgery only, the remaining 14 (63.6%) received postoperative chemotherapy. Five-year NED (no evidence of disease) survival was achieved in 81.8% of patients. Out of 16 stage I patients, 15 (93.8%) survived 5-year NED, out of six stage II and III, three (50%) patients only survived this period. We cured all grade 1 patients, and 81.8% (9/11) grade 2; two grade 3 patients died because of tumors. We also cured all six stage Ia patients, treated with unilateral salpingo-oophorectomy (with or without chemotherapy), and all eight stage Ia grade 1 patients treated with surgery only.     

Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases

The authors analyzed 153 cases of histologically verified intracranial germ cell tumors. The histological diagnosis was germinoma in 63 patients (41.2%), teratoma in 30 (19.6%), and other types of tumors in 60 patients (39.2%). The patients were treated by a consistent policy of surgical removal with histological verification followed by radiation therapy with or without chemotherapy. The 10- and 20-year survival rates of patients with pure germinoma were 92.7% and 80.6%, respectively. The 10-year survival rates of patients with mature teratoma and malignant teratoma were 92.9% and 70.7%, respectively. Patients with pure malignant germ cell tumors (embryonal carcinoma, yolk sac tumor, or choriocarcinoma) had a 3-year survival rate of 27.3%. The mixed tumors were divided into three subgroups: 1) mixed germinoma and teratoma; 2) mixed tumors whose predominant characteristics were germinoma or teratoma combined with some elements of pure malignant tumors; and 3) mixed tumors with predominantly pure malignant elements. The 3-year survival rates were 94.1% for the first group, 70% for the second group, and 9.3% for the third group, and the differences were statistically significant. Twenty-six patients with malignant tumors received chemotherapy that consisted of cisplatin and carboplatin combinations with or without radiation therapy. However, chemotherapy was not significantly more effective than radiation therapy alone. From these treatment results, the authors classified tumors into three groups with different prognoses and proposed a treatment guideline appropriate for the subgroups.     

Characteristics of rare ovarian tumors--possibilities of organ preservation

Whereas 65-70% of ovarian malignancies are of the epithelial type, the occurrence rate of stromal tumors is of approx. 7% and that of germ cell tumors of approx. 15%. Stromal tumors are mostly of the granulosa cell type, whereas germ cell tumors occur mainly as dysgerminoma (occurrence 0.9-2%), endodermal sinus tumors, or teratoma. Organ preservation is discussed in relation to the characteristics of these special tumor types. Granulosa cell tumors, representing 70% of the tumors of the gonadal stroma, occur unilaterally in approx. 97% of cases. 10-year-survival in stage I is over 90%. In stages II and III a complete remission after chemotherapy (acc. to the PVB, VAC, or BEP protocol) may be achieved in approx. 60% of cases. Due to these characteristics organ preservation seems feasible. Since dysgerminoma represent the most common malignant germ cell tumor in children, adolescents and pregnant women (up to 17% of all dysgerminoma are diagnosed during pregnancy), the wish for organ preservation is the more understandable. However, bilaterality, occurring in 20% of cases, has to be considered. Especially in large tumors lymphatic metastases also have to be taken into account. In cases of endodermal sinus tumors and teratoma, overall survival, mainly in patients with advanced disease, depends on the response to an aggressive chemotherapy. Preconditions for organ preservation are the patients' urgent childbearing desire, their information concerning the 5-7% risk of recurrence, an adequate oncologic postoperative care and optimally, after delivery, removal of the contralateral ovary and re-staging. The operative procedure requires removal of the corresponding adnexa, wedge dissection of the contralateral ovary, omentectomy, and depending on the histological tumor type a pelvic, possibly paraaortal lymph node dissection. No generally accepted standards are available for organ preserving surgery of stromal tumors, especially of the granulosa cell type. Prognosis is essentially influenced by a possible rupture being present, tumor size, cellular atypia, and the mitotic index. If one takes into consideration the possibility of lymph node metastases, at least a pelvic lymph node dissection should be recommended. In cases of metastases additionally a chemotherapy (VAC protocol) is indicated. Among germ cell tumors, dysgerminoma and non-dysgerminoma are treated differently. Non-dysgerminoma are endodermal sinus tumors, teratoma, embryonal carcinoma and mixed forms. For both groups operative management may aim at tumor reduction and in principle organ preservation. Whereas for dysgerminoma an adjuvant radiation therapy is feasible, in cases of non-dysgerminoma the response to a chemotherapy is the only factor influencing prognosis. Chemotherapy as adjuvant treatment is not indicated for pure dysgerminoma stage Ia, and pure solid teratoma stage Ia Gl. For all other dysgerminoma adjuvant chemotherapy, VAC protocol, and chemotherapy according the the BEP protocol for non-dysgerminoma is recommended. In cases of metastatic spread, in both groups an aggressive chemotherapy (BEP protocol) is most commonly performed.     

In vitro MR microscopy of the hippocampus in Alzheimer''s disease

In prepubertal children, teratoma is a benign tumor, whereas in adolescents and adults, it behaves as a malignant neoplasm. Adult patients without evidence of metastases may be candidates for surveillance after orchiectomy, and those with low-volume or borderline retroperitoneal metastases thought to contain teratoma might best be managed by surgery, as teratomatous deposits do not respond to chemotherapy. Patients with larger retroperitoneal metastases may be given chemotherapy before surgery to reduce or eliminate other germ-cell elements. Teratomatous masses persisting after chemotherapy are excised by most clinicians, in part to obtain a pathologic diagnosis. In childhood tumors, inguinal orchiectomy or enucleation is sufficient if one is certain the lesion contains only teratoma.     

A new antibiotic, U-43,120 (NSC-163500)

During the thirty-three years from 1941 through 1973, forty-two children with ovarian teratomas were seen. The most common complaint was that of abdominal pain and the most common physical finding was palpable lower abdominal mass. Thirty-seven patients had ovarian teratomas with nature tissues only. Of these, two patients have been lost to follow-up and the remainder are alive and well. One patient had teratoma containing mature and immature tissues (embryonic); this patient has remained well since operation. Four patients had malignant teratoma. Of these, two patients are dead due to the tumor and the two are living and well for six and nine years, respectively.     

Aggressive surgical management of testicular carcinoma metastatic to lungs and mediastinum

During the past six years, more than 200 patients were treated with chemotherapy for disseminated testicular cancer with a 70% complete remission rate. In 22 patients who were 17 to 46 years old, there was persistent thoracic disease, which was treated surgically. Six required a median sternotomy for bilateral pulmonary involvement or mediastinal metastasis. In 8 patients, chemotherapy had altered the histological appearance of the metastases from that of an undifferentiated primary tumor to a mature cystic teratoma. Five patients had nodules in the lungs, which were necrotic and fibrosed with no evidence of tumor. Nine showed embryonal cell carcinoma metastases in the lungs. All who had cystic teratoma are alive and free from disease. Three of the 5 with nodules and 1 of the 9 with metastases are currently free from disease. Agressive surgical intervention is important in this unique group of patients in order to determine the precise pathological category of the lesions, to remove intrathoracic malignancy, and to assess the need for additional chemotherapy. An operative mortality of zero and a low morbidity justify this approach.     

Germ-cell tumors of the brain--their course and management

Germ-cell tumors (GCT) of the CNS constitute a small group with an incidence varying in the range 0.1 to 3.4 per cent of all primary brain neoplasms. In the WHO classification of brain tumors they are differentiated in a separate group, and further subdivided into: germinoma, teratoma (mature, immature, malignant), embryonal carcinoma, choriocarcinoma, endodermal sinus tumor and mixed germinal tumors. Of them germinoma is the most frequently encountered (41-65 per cent of all GCT), whereas the incidence rate of mixed GCT may reach 32 per cent Mainly median structures are being involved--III ventricle (pineal region and hypothalamo-hypophyseal structures), basal ganglia, with isolated cases being described in corpus callosum, cerebellum, medulla oblongata and spinal cord. Eleven patients with a total of 13 verified GCT are presented (in one instance germinomas involving the pineal body and suprasellar region are simultaneously established). The clinical course data, results of the diagnostic examinations done (CT, brain angiography, tumor markers), treatment carried out--surgical, radio- and chemotherapy, as well as the outcome of the long-term follow-up study of patients are analyzed. Three patients have mixed GCT. It is of interest to note a case where ten years after the removal of a benign teratoma in the pineal region, germinoma in the same location is diagnosed and removed.     

Urinary leakage of tubular enzymes after shock wave lithotripsy

Germ cell tumours (GCTs) of the central nervous system (CNS) encompass various histological subtypes, and their optimal management has been the subject of debate. To indicate a better management strategy for each subtype, we analysed the records of 111 patients (median age 14 years), who underwent treatment since 1970. With a median follow-up duration of 86 months, the probability of surviving 5 years was: 96% for pure germinoma patients, 100% for mature teratoma, 67% for immature teratoma and 69% immature teratoma mixed with germinoma. The probability of cause-specific progression of germinomas producing human chorionic gonadotropin (HCG) was higher than that of non-producing germinomas (P < 0.01). GCTs that included a highly malignant component, such as embryonal carcinoma or yolk sac tumour, exhibited a poor prognosis with 38% chance of 5-year survival. Late adverse effects of therapy included stroke, secondary malignancy and cognitive, endocrinological, auditory and visual dysfunctions. Of 85 survivors with a median follow-up period of 99 months, 58 patients needed hormone replacement therapy, 26 patients showed poor performance status and, to date, only 1 patient has fathered children. Because the outcomes varied widely for each subtype, the traditional categories, that is, germinoma and non-germinomatous GCT as an extrapolation from the gonadal GCTs, are not suitable for appropriately selecting therapeutic regimen for CNS GCTs.     

Mitochondrial neurogastrointestinal encephalomyopathy presenting with protein-losing gastroenteropathy and serum copper deficiency: a case report

OBJECTIVE: To evaluate ultrasonic and magnetic resonance imaging (MRI) and Doppler examination in fetal sacrococcygeal teratoma (SCT), in respect to the postnatal findings and histological type of the tumor. STUDY DESIGN: Nine pregnancies complicated by histologically mature fetal sacrococcygeal teratoma in four cases and by immature/malignant teratoma in five cases. Transabdominal ultrasonic imaging and Doppler velocity waveforms were recorded during the second or last trimester in all cases, first trimester ultrasound examination was carried out in six cases and last trimester MRI in five cases. These findings were compared with postnatal and operative findings of the children. RESULTS: Ultrasound examination did not reveal intrapelvic parts of SCT, but this was possible by MRI. Velocity waveforms of the tumor arteries were similar in all histological types and the resistance index varied from 60 to 70. The mean gestational age at antepartal diagnosis was 25.2 weeks. Large tumor size with relatively large proportion of solid components was often recognized in cases with malignant/immature histology. CONCLUSIONS: Antepartal MRI is useful for examination of fetal SCT, but reliable differentiation of mature and immature SCT is not possible antepartally.