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

Female ; Humans ; Endodermal Sinus Tumor/pathology* ; Adenocarcinoma/pathology* ; Choriocarcinoma/pathology*

Turner syndrome is a congenital condition affecting 1 in every 2500 female live births. This condition is characterized by complete or partial loss of the X chromosome. They commonly present with normal female external and internal genitalia and may develop hypogonadism and streak ovaries later in life. We describe an unusual presentation of a case of Turner syndrome – a 31-year-old Filipino with male phenotype mosaic Turner syndrome, with 46,X,+mar[46]/45,X[4] chromosome, presenting with ambiguous genitalia and a pelvoabdominal mass. The patient underwent exploratory laparotomy, peritoneal fluid cytology, adhesiolysis, tumor debulking (gonadectomy) appendectomy, omentectomy, identification and inspection of bilateral ureters and bladder, gonioscopy and biopsy of the urogenital cavity (bladder vs. vagina). Histopathology revealed a mixed gonadal tumor, consisting of 70% yolk sac tumor, and 30% dysgerminoma. The patient eventually succumbed to postoperative complications. Postmortem fluorescence-in situ hybridization revealed a 46,X,+mar[46]/45,X,[4].ish der (Y) (DYZ3+), a marker of chromosome Y origin, consistent with a mosaic type Turner syndrome, associated with increased risk for gonadal malignancy.
Dysgerminoma ; mixed germ cell tumor ; mosaicism ; yolk sac tumor

Endodermal Sinus Tumor ; Female ; Humans ; Mixed Tumor, Malignant ; Neoplasms, Germ Cell and Embryonal ; Teratoma/pathology* ; Uterus/pathology*

Yolk sac tumor is the second most common subtype of ovarian germ cell tumors and is rare in postmenopausal women. The few cases in literature have found that in this age group, yolk sac tumors more commonly present as a mixed component, combined with epithelial tumors. We report a case of a 60-year-old female who presented with an enlarging abdominopelvic mass. Imaging pointed to an ovarian new growth. Total abdominal hysterectomy with bilateral salpingo-oophorectomy revealed a tumor with three populations composed of carcinomatous, sarcomatous, and germ cell components, which was ultimately diagnosed as an ovarian carcinosarcoma with concurrent yolk sac tumor based on histomorphology and immunohistochemical staining. This report also discusses the proposed pathogenesis, treatment, and prognosis of this uncommon entity.
Carcinosarcoma ; Endodermal Sinus Tumor ; Ovary

Yolk sac tumors are rare malignant germ cell neoplasms that usually arise from the gonads. Extragonadal yolk sac tumors (EGYSTs) frequently occur in the mediastinum in post-pubertal females. EGYSTs in the pelvis are extremely rare, and to date, only thirteen cases have been reported in the English literature. Among them, the primary EGYST of the pelvic peritoneum in post-pubertal females has only been reported in ten cases. The present case describes a 26-year-old female diagnosed with primary peritoneal yolk sac tumor located in the rectouterine pouch. We report clinical and tumor imaging features, including ultrasound, computed tomography (CT), magnetic resonance images (MRI), positron emission tomography-computed tomography (PET-CT), and present a review of the literature.
Adult ; Douglas' Pouch ; Electrons ; Endodermal Sinus Tumor ; Female ; Gonads ; Humans ; Magnetic Resonance Imaging ; Mediastinum ; Neoplasms, Germ Cell and Embryonal ; Pelvis ; Peritoneum ; Ultrasonography ; Yolk Sac

Endodermal Sinus Tumor/surgery* ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Orchiectomy/methods* ; Teratoma/surgery* ; Testicular Neoplasms/surgery* ; Testis/surgery* ; Treatment Outcome

OBJECTIVE: Primary mediastinal yolk sac tumor, which is also known as endodermal sinus tumor, is a rare but lethal neoplasm and its prognosis is very dismal. The current treatment for this tumor is controversial, and chemotherapy combined with resection of residual lesions is adopted sometimes. We summarized the experience of seven primary mediastinal yolk sac tumors treated with platinum-based chemotherapy and extended resection in Peking University First Hospital. METHODS: Clinicopathological data of the patients with primary mediastinal yolk sac tumor who received operation in Peking University First Hospital between August 2014 and August 2018 were collected and analyzed retrospectively. RESULTS: We experienced seven primary mediastinal yolk sac tumors during this period. Computed tomography scan revealed an anterior mediastinal tumor in all the patients and all of them had markedly raised alphafetoprotein (AFP) and normal β-human chorion gonadotropin (β-HCG). Five patients underwent needle core biopsy before treatment, which showed a mediastinal yolk sac tumor. All of these patients received preoperative platinum-based chemotherapy and they all presented partial response according to computed tomography. Two other patients did not receive preoperative biopsy, so they directly underwent extended resection. All of the seven patients underwent operation successfully and two of them experienced postoperative complications, including one with pneumonia and the other with atelectasis. R0 resection was achieved in six patients and R1 resection was achieved in the other patient. According to postoperative pathology, there were one microcyst subtype, one adenoid subtye, one giant capsule subtype and two hybrid subtypes. Surprisingly, there were no yolk sac tumor tissue in the other two patients after preoperative chemotherapy. All the patients received postoperative chemotherapy, excluded one patient who was unable to tolerate chemotherapy after operation. Three patients experienced postoperative pulmonary metastases within one year and two of them died soon. The other patient received chemotherapy and immunotherapy after recurrence and he was alive at the time of writing. Four other patients were alive without recurrence and metastasis. CONCLUSION Primary mediastinal yolk sac tumor is rare and its prognosis is poor. A multimodality approach including adjuvant chemotherapy and resection of residual lesions is the optimal treatment and it may lead to long-term survival.
Endodermal Sinus Tumor ; Humans ; Male ; Mediastinal Neoplasms ; Mediastinum ; Neoplasm Recurrence, Local ; Retrospective Studies

Ovarian cancer is the second most common gynecologic cancer worldwide and the six most common cancer among females. Germ cell tumorbs are the most common ovarian neoplasm in the first two decades of life constituting approximately two-thirds of all ovarian tumors. Malignant germ cell tumors constitute one-third of germ cell origin tutors and two-thirds of all ovarian malignancy in this age-group. This paper presents a case of a 19 year-old nulligravid who presented at the emergency room with abdominal pain, and was intraoperatively diagnosed with yolk sac tutor of the right ovary, stage 1A mature cystic teratoma of the left ovary. She subsequently underwent unilateral salpingooophorectomy and contralateral oophorocystectomy, left. Patient is advised chemotherapy postoperatively, with Bleomycin, Etoposide and Paclitaxel. This paper discusses the incidence, risk factors, prognosis and management of yolk of sac tutor in a young nulligravid.

Thymic adenocarcinoma is extremely rare. Although its histologic features have been occasionally reported, a lack of description of the cytologic features has hampered the prompt and accurate diagnosis of this condition. Herein, we describe the cytologic findings and histology of four aspiration cytology specimens of thymic adenocarcinoma. The specimens were obtained from primary tumors, metastatic lymph nodes, and pericardial effusions. All four specimens showed three-dimensional glandular clusters with a loss of polarity and nuclear overlapping. One specimen had extensive extracellular mucinous material. Three specimens contained tumor cells with intracytoplasmic vacuoles. While the specimen with extracellular mucin showed relatively mild cytologic atypia, other specimens exhibited more atypical cytologic changes: irregular nuclear membranes, a coarse chromatin pattern, and prominent nucleoli. The cytologic features were correlated with the histologic features in each case of enteric type thymic adenocarcinoma. The differential diagnosis included other thymic carcinomas, yolk sac tumors, and metastatic adenocarcinoma from the lung or colorectum.
Adenocarcinoma* ; Biopsy, Fine-Needle* ; Chromatin ; Diagnosis ; Diagnosis, Differential ; Endodermal Sinus Tumor ; Lung ; Lymph Nodes ; Mediastinum ; Mucins ; Nuclear Envelope ; Pericardial Effusion ; Thymoma ; Thymus Gland ; Vacuoles