BACKGROUND: To investigate the clinicopathological characteristics of urinary bladder tumors, a rare malignancy, in patients 20 years or younger. METHODS: Using a retrospective chart review among patients who received bladder surgery at 2 institutions between July 1996 and January 2013, we analyzed the clinicopathological characteristics of urinary bladder tumors in 21 pediatric patients (male:female = 4.25:1.00; mean age, 12.1 years). RESULTS: Pathology revealed 9 urothelial tumors, 6 rhabdomyosarcomas, 1 low-grade leiomyosarcoma, 1 large cell neuroendocrine carcinoma, 1 inflammatory myofibroblastic tumor, and 3 cases of chronic inflammation without tumors (including 1 xanthogranulomatous inflammation). Urothelial tumors (mean patient age, 16.0 years) were benign or low-grade; and only transurethral resection of the bladder tumor was necessary for treatment. Patients with rhabdomyosarcomas (mean age, 5 years) underwent radiotherapy (if unresectable) or transurethral resection of the bladder tumor (if resectable), after chemotherapy. Of these patients, 2 underwent radical cystectomy, with the remaining patients not receiving a cystectomy. With the exception of one patient, all patients are currently alive and recurrence-free. CONCLUSION: Urothelial tumors were the most commonly found pediatric bladder tumor, with embryonal rhabdomyosarcoma being the second most common. Urothelial tumors are common in relatively older age. Since urothelial tumors in children typically have a good prognosis and rarely recur, transurethral resection of the bladder tumor is the treatment of choice. Rhabdomyosarcomas are common in younger patients. Since rhabdomyosarcoma is generally chemosensitive, chemotherapy and radiotherapy are the treatment of choice for bladder preservation in these patients.
Carcinoma, Neuroendocrine ; Child ; Cystectomy ; Drug Therapy ; Humans ; Inflammation ; Leiomyosarcoma ; Myofibroblasts ; Pathology ; Prognosis ; Radiotherapy ; Retrospective Studies ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Embryonal ; Urinary Bladder Neoplasms ; Urinary Bladder*

Primary mediastinal choriocarcinoma is an extremely rare extragonadal germ cell malignancy. A 58-year-old male presented with a lung mass, which was incidentally discovered during a periodic medical checkup. Percutaneous needle biopsy showed poorly differentiated carcinoma with large pleomorphic morphology. After the patient underwent right upper lobectomy and lymphadenectomy, the final diagnosis was choriocarcinoma. The patient received four sequential cycles of BEP chemotherapy (bleomycin, etoposide, cisplatin). After completion of BEP chemotherapy, follow-up positron emission tomography (PET) showed a complete metabolic response. Although the mediastinum is one of the most common primary sites of extragonadal germ cell tumors, primary mediastinal choriocarcinoma is liable to be misdiagnosed as lung cancer or Hodgkin lymphoma. Notably, large cell carcinoma of the lung can be confused with choriocarcinoma even after percutaneous needle biopsy. We report a case of primary mediastinal choriocarcinoma mimicking large cell carcinoma of the lung in a male patient in his 50s.
Biopsy, Needle ; Carcinoma, Large Cell* ; Choriocarcinoma* ; Diagnosis ; Drug Therapy ; Etoposide ; Female ; Follow-Up Studies ; Germ Cells ; Hodgkin Disease ; Humans ; Lung Neoplasms ; Lung* ; Lymph Node Excision ; Male ; Mediastinum ; Middle Aged ; Neoplasms, Germ Cell and Embryonal ; Positron-Emission Tomography ; Pregnancy

No abstract available.
Antineoplastic Agents/*pharmacology ; Carcinoma, Embryonal/*drug therapy ; Cell Differentiation/*drug effects ; Cell Proliferation/*drug effects ; Flavonoids/*pharmacology ; Humans ; Phenols/*pharmacology ; Thyroid Neoplasms/*drug therapy

Thyroid carcinogenesis is accompanied by loss of thyroid-specific functions and refractory to radioiodine and thyroid stimulating hormone (TSH) suppression therapy. Redifferentiating agents have been shown to inhibit tumor growth and improve the response to conventional therapy. Polyphenol phytochemicals (PPs) in fruits and vegetables have been reported to inhibit cancer initiation, promotion, progression and induce redifferentiation in selected types. In this study we examined PPs induce redifferentiation in thyroid cancer cell lines. We investigated the effects of genistein, resveratrol, quercetin, kaempferol, and resorcinol on the F9 embryonal carcinoma cell differentiation model. The thyroid cancer cell lines, TPC-1, FTC-133, NPA, FRO, and ARO, displayed growth inhibition in response to genistein, resveratrol, quercetin. We further demonstrated that genistein decreased the dedifferention marker CD97 in NPA cells and resveratrol decreased CD97 in FTC-133, NPA, FRO cells and quercetin decreased CD97 in all cell lines. We observed increased expression of differentiation marker NIS in FTC-133 cells in response to genistein, and resveratrol but no change in NPA, FRO, ARO cells. Quercetin increased or induced NIS in FTC-133, NPA, FRO cells. These findings suggest that PPs may provide a useful therapeutic intervention in thyroid cancer redifferentiation therapy.
Antigens, CD/metabolism ; Antineoplastic Agents/*pharmacology/therapeutic use ; Carcinoma, Embryonal/*drug therapy/metabolism ; Cell Differentiation/*drug effects ; Cell Line, Tumor ; Cell Proliferation/*drug effects ; Flavonoids/*pharmacology/therapeutic use ; Gene Expression Regulation, Neoplastic ; Genistein/pharmacology/therapeutic use ; Humans ; Kaempferols/pharmacology/therapeutic use ; Models, Biological ; Phenols/*pharmacology/therapeutic use ; Quercetin/pharmacology/therapeutic use ; Resorcinols/pharmacology/therapeutic use ; Stilbenes/pharmacology/therapeutic use ; Symporters/metabolism ; Thyroid Neoplasms/*drug therapy/metabolism

Adult ; Carcinoma, Embryonal ; drug therapy ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Endodermal Sinus Tumor ; drug therapy ; metabolism ; pathology ; surgery ; Humans ; Keratin-19 ; metabolism ; Ki-1 Antigen ; metabolism ; Male ; Orchiectomy ; methods ; Testicular Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; alpha-Fetoproteins ; metabolism

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*

OBJECTIVE: to evaluate the clinicopathologic characteristics of patients with ovarian malignant germ cell tumor. METHODS: We reviewed records of women who had malignant germ cell tumors of the ovary from 1991-2000. RESULTS: Thirty-seven women had surgical resections of malignant ovarian germ cell tumors and most received adjuvant therapy. Twenty-five women (68%) presented with stage I disease, and 12 (32%) had more advanced disease. Histology subtypes were: immature teratoma (n=16), dysgerminoma (n=9), yolk sac tumor (n=8), choriocarcinoma (n=2), embryonal carcinoma (n=1), and mixed germ cell tumor (n=1). The frequency of positive tumor markers were aFP, CA-125, beta-hCG, CA 19-9 and LDH in decreasing order. The mean age of the patients at presentation was 23.9 years (4-58). Surgical management of the 32 patients consisted of unilateral oophorectomy, or salpingo-oophorectomy, 1 woman with stage III disease underwent bilateral salpingo-oophorectomy and 4 women underwent total abdominal hysterctomy, bilateral or unilateral adnexectomy. Thirty-two women were treated with adjuvant combination chemotherapy (BEP, VAC, VBP) according to indications. The 5-year survival rate was 100%, and 5-year disease-free rate was 91.8%. Among twelve patients who attempted pregnancy, 8 succeeded in it and delivered normal full term babies. CONCLUSION: Current therapeutic strategies can allow most women with ovarian malignant germ cell tumors to have conservative surgery without compromising survival and to preserve their reproductive potential.
Carcinoma, Embryonal ; Choriocarcinoma ; Drug Therapy ; Drug Therapy, Combination ; Dysgerminoma ; Endodermal Sinus Tumor ; Female ; Germ Cells* ; Humans ; Neoplasms, Germ Cell and Embryonal* ; Ovariectomy ; Ovary* ; Pregnancy ; Survival Rate ; Teratoma ; Biomarkers, Tumor

Primary germ cell tumors of the mediastinum are rare, accounting 1-5% among all germ cell tumors and 10% of all neoplasms in this area. Approximately 85% of these tumors occur in men with a mean age 29 years. These tumors are mainly found in the anterior mediastinum and appear grossly as large lobulated masses. They are frequently invasive at the time of diagnosis and almost 90% of patients are symptomatic. Primary nonseminomatous germ cell tumor arising in the posterior mediastinum is very rare. We report a case of 37- year old male arising from the posterior mediastinum. Serum tumors markers including alpha-fetoprotein and beta-hCG which are usually elevated in germ cell tumor were not elevated. He was found to have a primary mediastinal embryonal carcinoma with pulmonary metastasis at open exploration. He was treated with debulking surgery and cisplatin-based chemotherapy, died of sepsis after 15 months postoperatively.
alpha-Fetoproteins ; Carcinoma, Embryonal* ; Diagnosis ; Drug Therapy ; Humans ; Male ; Mediastinum* ; Neoplasm Metastasis ; Neoplasms, Germ Cell and Embryonal ; Sepsis

Malignant germ cell tumors of the ovary are uncommon neoplasms. Although 20-25% of all ovarian tumors are derived from germ cells, only about 3% of germ cell tumors are malignant. Mixed germ cell tumors contain at least two malignant germ cell elements. These lesions should be managed with combination chemotherapy, preferably BEP. Recently we experienced a case of mixed germ cell tumor with 6 components of germ cell and sarcomatous change in a 11 year old girl. Preoperative CA-125, B-hCG, aFP, LDH, a-1-antitrypsin were elevated and the final pathologic report was mixed germ cell tumor composed of endodermal sinus tumor, embryonal carcinoma, mature and immature teratoma, choriocarcinoma, dysgerminoma and sarcomatous change, Postoperative chemotherapy with 6 courses of BEP regimen was performed and all tumor markers became normal after 4 courses of chemotherapy. What we interested in this case was several components of germ cells and sarcomatous change and the sarcomatous change might be derived from the mature cystic teratoma component, so we present this case with a brief review of the literatures here.
Carcinoma, Embryonal ; Child ; Choriocarcinoma ; Drug Therapy ; Drug Therapy, Combination ; Dysgerminoma ; Endodermal Sinus Tumor ; Female ; Germ Cells* ; Humans ; Neoplasms, Germ Cell and Embryonal* ; Ovary ; Pregnancy ; Teratoma ; Biomarkers, Tumor

Among 60 children with teratoma, forty-three (71.7 percent) were girls and 17 (28.3 percent) boys. Primary sites were sacrococcygeal in 30 patients (50 percent), retroperitoneal in 12 (20 percent), ovarian in 11 (18.3 percent), testicular in 3 (5 percent), and one in each of nasopharyngeal, gastric, hepatic and pancreatic (1.6 percent, respectively). Fifty-five (91.7 percent) teratomas were benign and 5 (8.3 percent) malignant. Malignant teratomas were detected only at sacrococcygeal region (16.7 percent). Older than 2 months of age at diagnosis, presence of urinary and colonic obstructive symptoms, multiple masses and elevated serum alpha-fetoprotein were indicators of malignancy in sacrococcygeal region. Tumor size, presence of calcification, and gross appearance (cystic or solid) did not correlate with malignant nature. Thirteen (21.7 percent) cases were associated with other anomalies. For the immature teratoma, the operative resection without adjuvant chemotherapy was enough. Three malignant cases were survived, one with chemotheapy for 3 years and the others without chemotherapy for 5 and 10 years.
alpha-Fetoproteins ; Carcinoma, Embryonal ; Chemotherapy, Adjuvant ; Child* ; Colon ; Diagnosis ; Drug Therapy ; Endodermal Sinus Tumor ; Female ; Humans ; Sacrococcygeal Region ; Teratoma*