INTRODUCTION: Testicular tumours in childhood have diverse characteristics for different age ranges. This study aimed to describe the pattern, presentation and outcomes of primary testicular tumours in a paediatric population. METHODS: A retrospective study was conducted from January 2010 to December 2020 on children (≤18 years) with a diagnosis of primary testicular tumour. Baseline demographics, clinical characteristics, pathology, treatment and outcomes of these patients were analysed. The data were entered into IBM SPSS Statistics version 20.0. Chi-square test and Fisher's exact test were applied to find the statistical significance, which was set at P value ≤ 0.05. RESULTS: The study included 115 males, with 85 (73.9%) patients in the prepubertal age range with a mean age of 2.53 ± 2.06 years and 30 (26.1%) patients in the postpubertal group with a mean age of 15.73 ± 1.25 years. Yolk sac tumour was the most common (62.6%) histological subtype. Majority (46.1%) of patients had stage I disease on presentation, while 29.6% had stage IV disease. All patients underwent upfront high inguinal radical orchiectomy, which was followed by platinum-based adjuvant chemotherapy in 67% of the patients. The five-year event-free survival and overall survival for all patients were 75% and 91%, respectively. CONCLUSION Primary testicular tumours follow a bimodal age distribution pattern. Majority of patients can be cured with platinum-based chemotherapy despite having advanced disease at presentation.
Humans ; Male ; Testicular Neoplasms/mortality* ; Retrospective Studies ; Adolescent ; Child ; Child, Preschool ; Orchiectomy/methods* ; Chemotherapy, Adjuvant ; Treatment Outcome ; Neoplasm Staging ; Infant ; Endodermal Sinus Tumor/therapy* ; Neoplasms, Germ Cell and Embryonal

A case of primary yolk sac tumor of endometrium was retrospectively analyzed and relevant literature was systematically reviewed. We found that the primary yolk sac tumor of endometrium showed low incidence rate and was clinically characterized by abnormal vaginal bleeding, intrauterine lesions and significantly elevated alpha-fetoprotein (AFP) level. The final diagnosis should be confirmed by pathological examination. There is no guideline for the treatment of primary yolk sac tumor of endometrium due to its rareness. For now, surgery and chemotherapy are the major therapies and the curative effect is satisfactory in some cases.
Endodermal Sinus Tumor ; diagnosis ; epidemiology ; therapy ; Female ; Humans ; Incidence ; Retrospective Studies ; Uterus ; pathology ; alpha-Fetoproteins ; metabolism

Germ cell tumors the designation given to neoplasm arising from the cells of the germline, the cells that are destined to become either the egg or the sperm. These tumors have a number of unique features that includes bimodal and wide age distribution, remarkable phenotypic diversity, and varying biologic behavior. During infancy, sacrococcygeal locations predominate with either teratomas in neonates or endodermal sinus tumors in infants above three months. After puberty, non-germinomatous germ cell tumors predominate with gonadal, mediastinal or intracranial tumor. Specific subtypes of germ cell tumors secrete proteins as tumor markers. Surgical resection of the tumor is necessary to establish the diagnosis and for staging of the extent of tumor spread. Except for teratoma, germ cell tumors are highly sensitive to chemotherapy in particular cisplatin. The most commonly used chemotherapy regimen for malignant germ cell tumors is PEB (cisplatin, etoposide and bleomycin). Prognosis is good even in metastatic diseases. Patients with relapsed or recurrent disease may be candidates for high dose chemotherapy and autologous hematopoietic stem cell transplantation.
Adolescent ; Age Distribution ; Choriocarcinoma ; Cisplatin ; Diagnosis ; Drug Therapy ; Endodermal Sinus Tumor ; Etoposide ; Female ; Germinoma ; Gonads ; Hematopoietic Stem Cell Transplantation ; Humans ; Infant ; Infant, Newborn ; Neoplasms, Germ Cell and Embryonal ; Ovum ; Pediatrics ; Pregnancy ; Prognosis ; Puberty ; Spermatozoa ; Teratoma ; Biomarkers, Tumor

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

OBJECTIVETo study the treatment and outcome of childhood endodermal sinus tumor.

METHODSThe clinical data of twelve children with endodermal sinus tumor between April 2000 and July 2013 were reviewed. The basic demographics, stages of the lesion and the treatment outcome were analyzed. Of the twelve patients, seven were boys and five were girls. The age of the disease onset was between 1 and 3.3 years, except one in 11 years. Two patients were in Brodeur Stage I, four in Stage II, two in Stage III, and four in Stage IV. One patient underwent surgery alone, one underwent surgery plus a combination therapy with vincristine, actinomycin and cyclophosphamide (VAC), and the other ten were treated by surgery with the use of cisplatin, etoposide and bleomycin (PEB) before or after the operation.

RESULTSEleven patients were successfully followed up and ten were alive. The length of survival was 4.5 to 66 months in the 10 patients. In the 10 patients treated with PEB before or after surgery, 8 achieved complete remission, one achieved partial remission and one was not followed up. The major complications associated with the PEB regimen included myelosuppression and gastrointestinal upset symptoms and no late toxicity was observed.

CONCLUSIONSPreoperative or postoperative administration of PEB may be an effective and safe management modality for childhood endodermal sinus tumor. Nevertheless, further validation is warranted in prospective studies involving a larger sample size.

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Child, Preschool ; Combined Modality Therapy ; Endodermal Sinus Tumor ; mortality ; pathology ; therapy ; Female ; Humans ; Infant ; Male ; Neoplasm Staging ; alpha-Fetoproteins ; analysis

Endodermal sinus tumor is a type of germ cell tumor that is relatively common in children. An important problem concerns secondary neoplasms after treatment. We report a case of osteosarcoma that developed five years after treatment of intrapelvic endodermal sinus tumor. The patient was a seven-year-old girl who presented with right thigh and knee joint pain. The patient had been diagnosed with endodermal sinus tumor five years previously and treated with a regimen consisting of high dose cisplatin, etoposide, bleomycin and surgery but without radiotherapy. We detected a mass shadow on the right distal femur that proved to be osteoblastic osteosarcoma by incisional biopsy. The patient received surgical treatment after chemotherapy that included high dose methotrexate. The follow-up bone scan revealed no abnormal uptakes. There has been no evidence of recurrence eighteen months of follow-up after chemotherapy.
Biopsy ; Bleomycin ; Child ; Cisplatin ; Drug Therapy ; Endodermal Sinus Tumor ; Etoposide ; Female ; Femur ; Follow-Up Studies ; Humans ; Knee Joint ; Methotrexate ; Neoplasms, Germ Cell and Embryonal ; Neoplasms, Second Primary ; Osteoblasts ; Osteosarcoma ; Radiotherapy ; Recurrence ; Thigh

Malignant salivary gland tumors only represent 0.08% of all childhood tumors and mucoepidermoid carcinoma (MEC) is the most common histologic type. Although there are many reports describing second malignant neoplasm (SMN) in patients treated for childhood cancer, salivary gland tumors rarely appears. In Korea, there has been no report about MEC that developed in children as a SMN. We report a MEC in a 4 years and 8 months old female child that developed after completing treatment for yolk sac tumor of lower abdomen. The primary tumor presented with metastasis at the time of diagnosis, and therefore, the child underwent high-dose chemotherapy with autologous peripheral blood stem cell transplantation along with surgery and radiotherapy. Three years and five months after completing treatment, MEC developed in her submandibular gland. She was treated with surgery and radiotherapy and is in disease free state for 5 months at the time of this writing.
Abdomen ; Carcinoma, Mucoepidermoid ; Child ; Diagnosis ; Drug Therapy ; Endodermal Sinus Tumor ; Female ; Humans ; Korea ; Neoplasm Metastasis ; Peripheral Blood Stem Cell Transplantation ; Radiotherapy ; Salivary Gland Neoplasms ; Salivary Glands ; Submandibular Gland ; Writing

OBJECTIVETo clarify the clinical features, therapeutic method and outcomes of the primary endodermal sinus tumors (ESTs) in the posterior cranial fossa.

METHODSThe English literatures on EST in the posterior cranial fossa were retrieved from PubMed and reviewed. And a 4-year-old boy diagnosed with EST in our hospital was reported. The clinical manifestations, therapy, pathologic features, and prognosis of these cases were analyzed.

RESULTSOnly seven cases of the ESTs in the posterior cranial fossa were enrolled in this review, including six cases searched from the PubMed and one case from our hospital. Six patients were boy and one patient's gender was not available from the report. Ages ranged from 1 to 5 years (mean 3.14 years). The mean tumor size in our cohort was 4.4 cm. Six cases came from East Asia. Schiller-Duval bodies were found in all seven neoplasms. All tumors were positive for alpha-fetoprotein. The alpha-fetoprotein level in serum was increased to a very high level before therapy and depressed quickly after the effective chemotherapy. The mean follow-up time was 24.4 months (range 5-52 months). Six tumors were totally removed, and four of them recurred. Three cases died including one whose tumor was partially removed.

CONCLUSIONSThe serum alpha-fetoprotein level is well correlated with the severity of the tumor. A combination of operation and chemotherapy might be the effective management for EST in the posterior cranial fossa. The prognosis of extragonadal intracranial EST is poor.

Child, Preschool ; Cranial Fossa, Posterior ; Endodermal Sinus Tumor ; pathology ; therapy ; Female ; Humans ; Infant ; Male ; Skull Neoplasms ; pathology ; therapy ; alpha-Fetoproteins ; analysis

OBJECTIVE: The aim of this study was to evaluate the oncologic and reproductive outcomes of patients with ovarian yolk sac tumor after bleomycin, etoposide, cisplatin (BEP) chemotherapy following surgery. METHODS: Of 145 patients with histologically confirmed malignant ovarian germ cell tumor, 43 had yolk sac tumor and received BEP chemotherapy after surgery. A retrospective analysis of these patients was performed. RESULTS: The mean age of 43 patients was 24.8 years (range, 7 to 59 years). Thirty eight patients were nulliparous. Of 179 BEP chemotherapy cycles, grade 1~2 hematologic and non-hematologic adverse events occurred in 46 cycles in 21 patients. Thirty nine patients showed complete remission, 1 patient showed partial remission, and 3 patient had progressive disease during BEP chemotherapy. After median follow-up time of 57 months (range, 3 to 153 months), 5 patients had recurrent disease and three of them died of disease. The 5-year recurrence free survival rate and overall survival rate were 86% and 94%, respectively. After chemotherapy, all but one premenarchal patients had normal menstruation. Of them 5 patients tried to conceive and 3 of them succeeded in pregnancy. CONCLUSION: BEP chemotherapy was very safe and effective in patients with ovarian yolk sac tumor. Survival outcomes are excellent and reproductive outcomes are promising after BEP chemotherapy.
Bleomycin ; Cisplatin ; Drug Therapy, Combination ; Endodermal Sinus Tumor ; Etoposide ; Female ; Follow-Up Studies ; Humans ; Menstruation ; Neoplasms, Germ Cell and Embryonal ; Pregnancy ; Recurrence ; Retrospective Studies ; Survival Rate ; Yolk Sac

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