For children with stage II testicular malignant germ cell tumors (MGCT), the survival is good with surgery and adjuvant chemotherapy. However, there is limited data on surgical results for cases in which there was no imaging or pathologic evidence of residual tumor, but in which serum tumor markers either increased or failed to normalize after an appropriate period of half-life time post-surgery. To determine the use of chemotherapy for children with stage II germ cell tumors, we analyzed the outcomes (relapse rate and overall survival) of patients who were treated at the Sun Yat-sen University Cancer Center between January 1990 and May 2013. Twenty-four pediatric patients with a median age of 20 months (range, 4 months to 17 years) were enrolled in this study. In 20 cases (83.3%), the tumors had yolk sac histology. For definitive treatment, 21 patients underwent surgery alone, and 3 patients received surgery and adjuvant chemotherapy. No relapse was observed in the 3 patients who received adjuvant chemotherapy, whereas relapse occurred in 16 of the 21 patients (76.2%) treated with surgery alone. There were a total of 2 deaths. Treatment was stopped for 1 patient, who died 3 months later due to the tumor. The other patient achieved complete response after salvage treatment, but developed lung and pelvic metastases 7 months later and died of the tumor after stopping treatment. For children treated with surgery alone and surgery combined with adjuvant chemotherapy, the 3-year event-free survival rates were 23.8% and 100%, respectively (P = 0.042), and the 3-year overall survival rates were 90.5% and 100%, respectively (P = 0.588). These results suggest that adjuvant chemotherapy can help to reduce the recurrence rate and increase the survival rate for patients with stage II germ cell tumors.
Adolescent ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; Combined Modality Therapy ; Humans ; Infant ; Male ; Neoplasm Staging ; Neoplasms, Germ Cell and Embryonal ; mortality ; pathology ; therapy ; Survival Rate ; Testicular Neoplasms ; mortality ; pathology ; therapy

Adenocarcinoma ; pathology ; therapy ; Choriocarcinoma, Non-gestational ; pathology ; therapy ; Combined Modality Therapy ; Female ; Humans ; Lung Neoplasms ; secondary ; Middle Aged ; Neoplasms, Germ Cell and Embryonal ; pathology ; therapy ; Urinary Bladder Neoplasms ; pathology ; therapy ; Uterine Neoplasms ; pathology ; therapy

OBJECTIVETo report a rare case of ectopic mesonephric duct cyst with ectopic testicular malignancy and improve the diagnosis and treatment of the disease.

METHODSWe retrospectively analyzed the clinical data of a case of ectopic mesonephric duct cyst with ectopic testicular malignancy, reviewed relevant literature at home and abroad, and investigated the pathogenesis, diagnosis and treatment of the disease.

RESULTSA large cyst and the right ectopic malignant testis were removed via abdominal incision, and the left undescended testis was lowered into the scrotum. Pathological examination confirmed the lesion to be right ectopic mesonephric duct cyst with right ectopic testicular seminoma. No metastasis was found during a year of follow-up.

CONCLUSIONEctopic mesonephric duct cyst with ectopic testicular malignancy was a rare disease. Imaging examination contributes to its diagnosis, but it has to be confirmed by pathology. Surgical removal should be performed as early as possible and follow-up treatment depends on the pathologic type and stage of ectopic testicular malignancy.

Cryptorchidism ; therapy ; Cysts ; pathology ; Humans ; Male ; Neoplasms, Germ Cell and Embryonal ; surgery ; Seminoma ; surgery ; Testicular Neoplasms ; surgery ; Testis ; surgery ; Wolffian Ducts ; pathology

The undifferentiated (embryonal) sarcoma of the liver (USL) has previously been called malignant mesenchymoma, undifferentiated sarcoma and fibromyxosarcoma. USL was named as an entity by Stocker and Ishak in 1978 on the basis of an Armed Forces Institute of Pathology (AFIP) series of 31 cases. The USL is a rare primary neoplasm of a mesenchymal origin and it predominantly occurs in children. Stocker reported that it was fourth in frequency among the liver tumors of childhood, following hepatoblastoma, hemangioendothelioma and hepatocellular carcinoma. Although there has been controversy as to the most appropriate treatment, the studies have reported that long term survival is possible after complete surgical resection with or without perioperative chemotherapy. This tumor's frequency in the adult population is extremely low. We report here on a case of USL in an adult woman with the review of the relevant literature.
Adult* ; Arm ; Carcinoma, Hepatocellular ; Child ; Drug Therapy ; Female ; Hemangioendothelioma ; Hepatoblastoma ; Humans ; Liver Neoplasms ; Liver* ; Mesenchymoma ; Neoplasms, Germ Cell and Embryonal ; Pathology ; Sarcoma*

OBJECTIVETo investigate the clinicopathological characteristics, diagnosis and treatment of primary testicular yolk sac tumor (YST).

METHODSWe studied 8 cases of primary testicular YST by microscopy and immunohistochemistry.

RESULTSThe 8 cases of primary testicular YST, including 2 consultation cases, were confirmed from 1998 to 2013, accounting for 10.7% (8/75) of all the testicular germ cell tumors diagnosed in our hospital. The patients ranged in age from 7 to 43 years, 23.9 years on average. The main clinical manifestation of the patients was painless unilateral testis swelling. Microscopically, reticular tissues, schiller-duvaI (S-D) bodies, and eosin-stain transparent bodies were seen in the tumors. One of the cases was confirmed to be simple YST, while the other 7 mixed YST. AFP was a characteristic immunophenotype marker of the tumors.

CONCLUSIONPrimary testicular YST is a rare malignancyr with poor prognosis. Its diagnosis depends on preoperative AFP test and postoperative pathology. Comprehensive treatment, including orchiectomy, chemotherapy, and radiotherapy, can prolong the survival of the patients.

Adolescent ; Adult ; Child ; Endodermal Sinus Tumor ; metabolism ; pathology ; therapy ; Humans ; Immunohistochemistry ; Male ; Neoplasms, Germ Cell and Embryonal ; metabolism ; pathology ; therapy ; Orchiectomy ; Rare Diseases ; metabolism ; pathology ; therapy ; Testicular Neoplasms ; metabolism ; pathology ; therapy ; Young Adult

A retrospective analysis has been made of 40 patients with pineal region tumors and CNS germ cell tumors who were treated at Yonsei University Hospital, Department of Radiation Oncology between 1971 and 1985. A tissue diagnosis was obtained before radiotherapy in 19 patients and 21 patients were irradiated without histological diagnosis. Among 19 biopsy-proven cases, 14 were germinomas, 2 were teratomas, and the others were two pineocytomas and one pineoblastoma. In the earlier period, every attempt was made to obtain a tissue pathology by either stereotaxic biopsy or open craniotomy before irradiation. However, in recent years, with the advent of CT scan, a trial radiotherapy with a modest dose of 20 Gy in 2 weeks was attempted in cases of highly suspected germinomas by CT scan findings. Further management after trial radiation depended on the radiation response shown on the follow-up CT scan and tumor marker study. Radiation fields varied from a small local field to whole brain or entire neuroaxis irradiation. Most patients received 40-50 Gy to the primary tumor site and 20-30 Gy to the neuroaxis. Twenty-nine of the total 40 patients are alive without of disease 22-144 months after treatment and the overall 5-year recurrence-free survival rate was 74.4%, Univariate analysis of prognostic factors at presentation showed that tumor type was highly correlated with outcome. Two of fourteen biopsy-proven germinomas and none of nine presumed germinomas by trial radiation recurred. On the other hand, five of six patients who showed poor response to trial radiation died of uncontrolled disease and only one patient with elevated AFP in serum and CSF was salvaged by chemotherapy. On the basis of the results of this study, application of trial radiation therapy without tissue biopsy is well justified as a treatment modality in a suspected germinoma by CT scan finding. Aggressive combined modality approaches with surgery, radiotherapy and chemotherapy need to be investigated to improve results in radioresistant tumors.
Biopsy ; Brain ; Craniotomy ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Germ Cells* ; Germinoma ; Hand ; Humans ; Neoplasms, Germ Cell and Embryonal* ; Pathology ; Pinealoma ; Radiation Oncology ; Radiotherapy ; Retrospective Studies ; Survival Rate ; Teratoma ; Tomography, X-Ray Computed

OBJECTIVE: To explore the appropriate treatment of malignant germ cell tumor (MGCT) in the female genital system, and to analyze the factors influencing both therapeutic response and survival outcome. METHODS: A cohort of 230-Chinese women diagnosed with MGCT of the genital system was retrospectively reviewed and prospectively followed. The demographic and pathological features, extent of disease and surgery, treatment efficiency, recurrence and survival were analyzed. RESULTS: MGCTs from different genital origins shared a similar therapeutic strategy and response, except that all eight vaginal cases were infantile yolk sac tumors. The patients' cure rate following the initial treatment, 5-year overall survival and disease-free survival (DFS) were 85.02%, 95.00%, and 86.00%, respectively. Although more extensive excision could enhance the remission rate; it did not improve the patients' survival. Instead, the level of the medical institution, extent of surgery and disease were independent prognostic factors for relapse (p<0.05). Approximately 20% of patients had recurrent or refractory disease, more than half of whom were in remission following secondary cytoreductive surgery with salvage chemotherapy. CONCLUSION: Fertility-sparing surgery with or without standardized PEB/PVB (cisplatin, etoposide/vincristine, and bleomycin) chemotherapy is applicable for female MGCTs of different origins. Comprehensive staging is not required; nor is excessive debulking suggested. Appropriate cytoreduction by surgery and antineoplastic medicine at an experienced medical institution can bring about an excellent prognosis for these patients.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Combined Modality Therapy ; Cytoreduction Surgical Procedures ; Female ; Genital Neoplasms, Female/diagnosis/mortality/pathology/*therapy ; Humans ; Infant ; Middle Aged ; Neoplasms, Germ Cell and Embryonal/diagnosis/mortality/pathology/*therapy ; Prognosis ; Recurrence ; Survival Analysis ; Young Adult

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Neoplasms, Germ Cell and Embryonal ; diagnosis ; drug therapy ; pathology ; Ovarian Neoplasms ; diagnosis ; drug therapy ; Testicular Neoplasms ; diagnosis ; drug therapy

Laparoscopic retroperitoneal lymph node dissection, especially when performed with the da Vinci Surgical System (Intuitive Surgical), has shown excellent cosmetic results with similar oncologic outcomes to those of open surgery. In this study, we present a case of robot-assisted retroperitoneal lymph node dissection performed in an 18-year-old man who was diagnosed with a stage IIIb mixed germ cell tumor and who was initially treated with radical orchiectomy, followed by chemotherapy. This case shows that robot-assisted retroperitoneal lymph node dissection is technically feasible, safe, and cosmetically favorable, even when performed on patients with high-stage disease or after chemotherapy.
Adolescent ; Chemotherapy, Adjuvant ; Humans ; Laparoscopy/methods ; Lymph Node Excision/*methods ; Lymphatic Metastasis ; Male ; Neoplasm Staging ; Neoplasms, Germ Cell and Embryonal/pathology/radiography/*secondary/therapy ; Orchiectomy ; Robotic Surgical Procedures/*methods ; Testicular Neoplasms/pathology/radiography/*secondary/therapy ; Tomography, X-Ray Computed

Primary intracranial germ cell tumors(GCTs) are relatively rare brain tumors that show a diverse range of histologic features from benign to highly malignant conditions. To determine their clinical findings, pathology, treatment and outcome, we analyzed the medical records of 45 patients with primary intracranial GCTs treated at our hospital between June 1989 and December 1996. Thirty-two were males and 13 were females, and their ages ranged from three to 43 years. Fifteen cases were located in the pineal region and 13 in the suprasellar. The remaining locations were the basal ganglia in eight cases, both the pineal and suprasellar region in five, and others in four. In the pineal region, there was a male predominance(13:2), but in the suprasellar region, more cases(ten of 13) involved females. Of the 15 patients with tumors of the pineal region, increased intracranial pressure(IICP) was evident in 12 and six had Parinaud's syndrome. Of the 13 patients with tumors of suprasellar region, nine had diabetes insipidus; seven, visual deficit; and six, hypopituitarism. Germinoma was the most common histologic type. Other types of histology were two teratomas, three embryonal carcinomas, one endodermal sinus tumor, one choriocarcinoma, and five mixed GCTs. All patients except those with a teratoma underwent whole craniospinal irradiation. We performed gross total or subtotal removal in cases of non-germinomatous GCTs(NGGCTs) and mixed tumors, but biopsy or partial removal was preferred for the germinomas. Thirteen of 45 patients received adjuvant chemotherapy. All malignant NGGCT and mixed tumor patients were treated with adjuvant chemotherapy, as well as three of 33 germinoma patients. Three of five malignant NGGCT patients and two of five mixed tumor patients died of tumor progression. Two of 33 germinoma patients died not of disease progression but of other causes. Actuarial survival records showed that overall two-year and five-year survival rates were 89.9% and 71.9%, respectively. There were no statistically significant differences with regard to patient's age, sex, or tumor location. With regard to their histology and surgical extent, malignant NGGCTs and mixed tumors showed statistically significant differences. Five-year surival rates of germinoma and malignant NGGCT patients were 83.1% and 53.3%, respectively. We suppose that the appropriate combination of chemotherapy and surgery, with or without radiation therapy, remains to be defined, and that to determine the appropriate management protocol for malignant NGGCTs and mixed tumors, larger series of patients must be analyzed.
Basal Ganglia ; Biopsy ; Brain Neoplasms ; Carcinoma, Embryonal ; Chemotherapy, Adjuvant ; Choriocarcinoma ; Craniospinal Irradiation ; Diabetes Insipidus ; Disease Progression ; Drug Therapy ; Endodermal Sinus Tumor ; Female ; Germ Cells* ; Germinoma ; Humans ; Hypopituitarism ; Male ; Medical Records ; Neoplasms, Germ Cell and Embryonal* ; Ocular Motility Disorders ; Pathology ; Pregnancy ; Survival Rate ; Teratoma