Germ cell tumors (GCT) are a complex, heterogeneous collection of tumors that may present in either gonadal or extragonadal sites. They consist of a variety of benign and malignant histologies that can occur at several locations throughout the body. An important component of treatment is surgical resection, and while the key components of resection are site specific, the universal goals of GCT resection include the complete resection of tumor without violating the tumor capsule, while preserving function of surrounding organs, minimizing morbidity, and assessing for regional spread.

Extragonadal germ cell tumors (EGCTs) are rare, representing <5% of all germ cell tumors (GCTs). Whilst EGCTs share morphological and immunohistochemical features with their gonadal counterparts, they tend to be more aggressive and are frequently associated with secondary somatic malignancies. The aim of our study was to evaluate the clinical, morphological and immunohistochemical features, and to analyze tumors for chromosomal abnormalities of 12p, in addition to any novel genetic alterations, in a series of EGCTs. Seventy-seven EGCTs were included. Anterior mediastinum was the most common anatomic site, followed by central nervous system, retroperitoneum, sacroccygeal area, and neck. Whole genome SNP array identified isochromosome 12p in 26% of tumors. Additional cytogenetic abnormalities included the presence of gain of chr 21 in 37% of tumors. Somatic-type malignancies were identified in 8% of patients. Disease progression (metastasis and/or recurrence) was documented in 8 patients, most of whom died from their relapse. Three patients who died of disease had somatic-type malignancies. Mediastinal seminomas had a significantly better overall survival when compared to mediastinal non-seminomatous GCTs. Our study demonstrates that EGCTs share similar histologic features, but diverse clinical outcomes compared to their gonadal counterparts. Outcomes vary according to anatomic location and histologic subtypes. Our data corroborate that somatic-type malignancies are frequently encountered in mediastinal EGCTs and that their presence portends a poorer prognosis.

Malignant germ cell tumors (MGCTs) localized in the uterus are rare in prepubertal girls. They typically occur in postmenopausal women and are characterized by the presence of a pelvic mass and transvaginal bleeding. In this case, the authors describe the clinical features, radiologic findings, histopathologic description, and treatment received by an infant with a primary yolk sac tumor of the uterine wall. Currently, treatment for uterine GCTs is based on guidelines for GCTs. Surgery and bleomycin, etoposide, cisplatin (pBEP) chemotherapy are effective for uterine yolk sac tumors. After 46 months of clinical follow-up, which included abdominopelvic ultrasound and tumor marker assessments, our patient is free of disease, suggesting a favorable outcome.

Choriocarcinoma is a highly aggressive malignant tumor that occurs due to the formation of an abnormal trophoblast. Choriocarcinoma is classified into gestational (GC) and nongestational (NGC) subtypes. The majority of nongestational diseases are limited to ovaries. Extragonadal NGC is a sporadic occurrence and a diagnostic and therapeutic dilemma. Here, we present a young 24-year-old female who presented with a widespread metastatic disease to the brain, bilateral kidneys, lungs, liver, pancreas, and small bowel. She was diagnosed with extragonadal NGC, probably originating from her kidneys. She responded poorly to standard first-, second-, and third-line chemotherapies. Detailed literature analysis with various aspects of pathogenesis, diagnostic criteria, clinical presentation, and treatment options are discussed. There is an unmet need for further research and consensus on many aspects of this rare disease.

BACKGROUND: The occurrence of extragonadal germ cell tumors (EGGCTs), either as primary tumors or metastatic disease, is rare. Forms of cytologic sampling, including fluid analysis, fine-needle aspiration, and/or small-core needle biopsy, have been shown to be reliable methods for the diagnosis of germ cell tumors. This study aims to investigate the utility of cytopathologic techniques in the diagnosis of EGGCTs at the authors\' institution.
METHODS: The laboratory information system was queried over a period of 10 years (2012-2022) to identify all cytology cases diagnosed on fluid cytology, FNA, and/or small-core biopsy as germ cell tumors in extragonadal locations. Patient demographics, tumor location, serum tumor marker levels, cytopathologic diagnosis, and follow-up surgical resection data were reviewed and correlated.
RESULTS: A total of 35 cases from 32 patients (all males) were identified. Thirty specimens contained satisfactory material for diagnosis (86%) and five were less than optimal for evaluation (14%). Despite this, all cases had clinically useful cytopathologic diagnoses. A total of 19 cytology cases (16 patients) had follow-up resection specimens available. Of these, 11 patients underwent preoperative chemotherapy. Nine patients showed no evidence of residual tumor and two showed histologic concordance. Of the five patients who did not have preoperative chemotherapy, all showed concordant histologic diagnoses.
CONCLUSIONS: Cytology can provide a reliable, accurate method for diagnosing EGGCTs. The practice of preoperative (neoadjuvant) chemotherapy places an extreme importance on the initial cytopathologic diagnosis because the majority of patients with follow-up resection in this series showed no residual tumor.

Extragonadal germ cell tumors (GCTs) are a rare group of neoplasms that account for 1%-5% of all GCTs. These tumors can present with an unpredictable behavior and clinical manifestations depending on different factors such as histological subtype, anatomical site, and clinical stage. We report the case of a 43-year-old male patient with a primitive extragonadal seminoma located in the paravertebral dorsal region, an extremely rare site. He presented to our emergency department with a 3-month history of back pain and a 1-week history of fever of unknown origin. Imaging techniques revealed a solid tissue arising from the vertebral bodies of D9-D11 and extending in the paravertebral space. After a bone marrow biopsy and exclusion of testicular seminoma, he was diagnosed with primitive extragonadal seminoma. The patient underwent five cycles of chemotherapy, and the follow-up CT examinations showed a reduction of the mass initially till a complete remission with no evidence of recurrence.

BACKGROUND: Extragonadal germ cell tumours (EGGCTs) originated in Shoulder are extremely rare, with 1 case described in the literature. We report a case of a patient with a primary Right Shoulder mixed EGGCT.
METHODS: A 36-year-old male patient was hospitalized for 6 months due to progressive right shoulder swelling accompanied by pain. Subsequently, the right shoulder tumor was removed entirely. Gross pathological examination showed that the size of the tumor mass was about 14 × 10 × 6 cm.Mutations were observed in ENPEP (4q25), ZCCHC11, RREB1 (6p24.3), CKAP4 (12q23.3), and other genes were detected by whole exome sequencing. Histology revealed a mixed EGGCT of the Right Shoulder with immature teratoma and yolk sac tumour. The patient went through 6 cycles of chemotherapy. After 7 months of follow-up, the patient is recurrence.
CONCLUSIONS: The primary MEGCT of the shoulder is an extremely rare condition. However, the recurrence and metastasis rates are high. Therefore, further research is necessary to determine this rare disease\'s genetic and clinical characteristics to develop an effective treatment plan.

An 8-month-old, intact male, domestic shorthair cat was referred for a mass on the proximal ventral part of the tail which had been found since the animal was born, and due to the presence of a linear fissure with rows of ectopic teeth, the veterinarian suspected that the mass had recently ruptured. Tail amputation was elected and the entire mass was successfully surgically excised. From the gross examination, this mass had an open cyst-like structure with a prominent area composed of hair, teeth, and bone. Histopathology revealed two components of germinal layers including hair follicles, adnexal tissue, neural tissue, teeth, muscle, fat, bone, and lymphatic vessels. The histopathological diagnosis was consistent to mature teratoma. Although, complete excision could not be definitively confirmed histologically, this kitten is currently well and has not developed any recurrent mass at the surgical site after 2 years of post-operation.

UNASSIGNED: This study aimed to summarize the clinical features, treatment modalities, therapeutic effects, menstruation and fertility outcomes, and prognosis of extragonadal yolk sac tumors (YSTs) of the female genital tract.
UNASSIGNED: We reviewed 32 cases of extragonadal YSTs in the genital tract treated between 1983 and 2021. The medical records, including clinical characteristics, histopathology, treatments, chemo-reduced adverse events, and outcomes on long-term follow-up, were collected.
UNASSIGNED: Among the 32 cases, 30 were vaginal YSTs and two were uterine YSTs (endometrial and cervical). Thirty patients (30/32, 93.8%) were <4 years. Abnormal vaginal bleeding (n = 31) and elevated serum alpha-fetoprotein level (n = 32) were the most common presentations. Vaginohysteroscopy and/or pediatric rhinoscopy were used for diagnosis in 17 pediatric patients and evaluation of chemotherapeutic efficacy in 21 pediatric patients. All the patients received combination chemotherapy. Bleomycin/etoposide/cisplatin (BEP) was chosen with prior consideration in 28 cases; 21 patients were treated with BEP alone. Yellow or grayish-yellow tissue with irregular shape was found in 66.7% of the cases during repeat examinations. Five patients underwent surgeries during repeat examinations and follow-ups, and no evidence of malignancy was noted in them. Thirty-one patients achieved complete remission. During a median follow-up of 63 months (2.4-240.3 months), two patients experienced recurrence, three died, and 29 remained disease-free. One patient recovered menstruation and five had undergone menarche.
UNASSIGNED: BEP chemotherapy can serve as a preferred treatment modality for vaginal and uterine YSTs. Vaginohysteroscopy and pediatric rhinoscopy can be used for diagnosis and evaluation of chemotherapeutic efficacy in pediatric patients. YSTs possibly appear as yellow or grayish-yellow after chemotherapy.

Introduction Although nephroblastomas are frequently treated without prior biopsy, there are the occasional other pediatric renal tumors that require different management. In the literature, there are around 30 primary renal germ cell tumors (GCT), including four cases of Yolk sac tumor (YST). We present another primary renal YST.Case report: A five-year-old boy was diagnosed as Wilms tumor on radiology and needle biopsy. He received chemotherapy, with no response. The post-chemotherapy resection specimen revealed a YST.Conclusion: Renal YST may be indistinguishable from Wilms tumor clinically and radiologically. For pre-biopsy chemotherapy management protocols, serum tumor markers such as AFP may be recommended to identify the occasional GCT, including YST. Pre-chemotherapy needle biopsies may lead to misdiagnosis, and may require confirmation by an experienced pathologist or central review.