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Extragonadal germ cell tumors (EGGCTs) are uncommon neoplasms, which arise in anatomical locations other than gonads. The pathogenesis of these neoplasms is still poorly understood and it is a matter of debate if they really represent extragondal primary neoplasms or rather extragondal metastasis from occult gonadal neoplasms. The actual observations suggest that EGGCTs represent a unique entity, so their biology and behavior are substantially different from gonadal counterparts. The diagnosis of EGGCTs is often challenging, and differential diagnosis is particularly wide. Nevertheless, a correct diagnosis is essential for the correct management of the patient. We summarize the state of art about EGGCTs, with particular emphasis on diagnosis and prognosis. Extragonadal germ cell tumors (EGGCTs) are uncommon neoplasms with peculiar molecular and pathological features; the diagnosis of these neoplasms may be challenging, due to wide differential diagnosis depending to the variable anatomical location. The authors summarize the state of art about EGGCTs, with particular emphasis on diagnosis and prognosis.

Teratomas are germ cell tumors, commonly affecting ovaries. Teratomas rarely affect extragonadal tissues. Few cases of teratomas occurring in extragonadal tissues have been reported in the past. However, no studies have reported cases of primary teratomas occurring in the broad ligament. In this study, we report a case of a sexually inactive young woman with a 3-day history of lower abdominal pain. B-ultrasound examination revealed an abnormal strong echogenicity mass in the left adnexal area. An exploratory laparotomy was performed on her. The surgery revealed normal size and appearance of both ovaries and uterus. However, a cyst was observed in the left broad ligament, which was diagnosed as benign mature teratoma based on pathology. In this study, we report a rare case of broad ligament teratoma. The clinical data enrich our understanding of teratomas and provide a reference for further studies.

A rare extragonadal mixed germ cell tumor of the sacrococcygeal area presenting with mature thymic tissue in the teratomatous component, a rare finding and the first reported case of such an association.

PurposeTo present the current evidence and the development of studies in recent years on the management of extragonadal germ cell tumors (EGCT).MethodsA systematic literature search was conducted in Medline and the Cochrane Library. Studies within the search period (January 2010 to February 2021) that addressed the classification, diagnosis, prognosis, treatment, and follow-up of extragonadal tumors were included. Risk of bias was assessed and relevant data were extracted in evidence tables.ResultsThe systematic search identified nine studies. Germ cell tumors (GCT) arise predominantly from within the testis, but about 5% of the tumors are primarily located extragonadal. EGCT are localized primarily mediastinal or retroperitoneal in the midline of the body. EGCT patients are classified according to the IGCCCG classification. Consecutively, all mediastinal non-seminomatous EGCT patients belong to the “poor prognosis” group. In contrast mediastinal seminoma and both retroperitoneal seminoma and non-seminoma patients seem to have a similar prognosis as patients with gonadal GCTs and metastasis at theses respective sites. The standard chemotherapy regimen for patients with a EGCT consists of 3–4 cycles (good vs intermediate prognosis) of bleomycin, etoposid, cisplatin (BEP); however, due to their very poor prognosis patients with non-seminomatous mediastinal GCT should receive a dose-intensified or high-dose chemotherapy approach upfront on an individual basis and should thus be referred to expert centers Ifosfamide may be exchanged for bleomycin in cases of additional pulmonary metastasis due to subsequently planned resections. In general patients with non-seminomatous EGCT, residual tumor resection (RTR) should be performed after chemotherapy.ConclusionIn general, non-seminomatous EGCT have a poorer prognosis compared to testicular GCT, while seminomatous EGGCT seem to have a similar prognosis to patients with metastatic testicular seminoma. The current insights on EGCT are limited, since all data are mainly based on case series and studies with small patient numbers and non-comparative studies. In general, systemic treatment should be performed like in testicular metastatic GCTs but upfront dose intensification of chemotherapy should be considered for mediastinal non-seminoma patients. Thus, EGCT should be referred to interdisciplinary centers with utmost experience in the treatment of germ cell tumors.

Teratomas are common tumors in children. The most common sites are gonads, however, several extragonadal sites are well known such as sacrococcygeal region, mediastinum, head and neck, stomach, and vagina. Occasionally, teratomas occur in unusual sites which pose diagnostic difficulties. Here, we describe two cases of infantile teratomas with unusual presentation. The first case was a 6-month-old girl who presented with a mass in the right side of the abdomen. Computed tomography (CT) scan showed a solid-cystic mass in the right suprarenal region. The second was a 4-month-old boy who came with a huge abdominal mass. Contrast-enhanced CT showed a tumor arising from the jejunal mesentery. On microscopic examination, they were diagnosed as mature and immature teratoma, respectively. Although neuroblastoma is the most common infantile adrenal neoplasm, the possibility of teratoma should be considered for cystic adrenal lesions. Mesenteric teratoma can be difficult to distinguish radiologically from the more common lymphangioma; however, accurate diagnosis is important in view of long-term follow-up for teratomas because of the possibility of malignant transformation.

Background Mature cystic teratomas of the mediastinum are typically germ cell tumors with indolent growth, with documented annual growth rates often less than 5 centimeters. We report a case with an abnormal growth rate (Average diameter growth rate 7.14 mm/month) and significant increased 18 F fluorodeoxyglucose (18 F FDG) uptake, which challenges conventional diagnostic concepts. Case presentation A 34-year-old female presented with progressive chest tightness. Serial CT imaging revealed a right anterior superior mediastinal mass that grew from approximately 1.5 cm in long diameter to 6.5 cm over 7 months (Average diameter growth rate 7.14 mm/month). 18 F fluorodeoxyglucose Positron Emission Tomography-Computed Tomography(18 F-FDG PET/CT) revealed the lesion with high metabolic heterogeneity, and the maximum standardized uptake value (SUVmax 6.9). Pathological examination after thoracoscopic-assisted complete resection confirmed the diagnosis of mature cystic teratoma with mature glial components. Conclusion This case suggests that lesions with rapid growth and significantly increased FDG uptake do not necessarily indicate malignant transformation of teratoma. Multidisciplinary decision-making should be based on dynamic quantitative imaging and metabolic feature analysis.Categories: Pathology, Oncology, Pulmonology.

Extragonadal germ cell tumors (EGCTs) are relatively rare tumors, accounting for 1%-5% of all GCTs. In this review, we summarize the current research progress regarding the pathogenesis, diagnosis, and treatment of EGCTs from an immunology perspective. The histological origin of EGCTs is related to a gonadal origin, but they are located outside the gonad. They show great variation in morphology and can occur in the cranium, mediastinum, sacrococcygeal bone, and other areas. The pathogenesis of EGCTs is poorly understood, and their differential diagnosis is extensive and challenging. EGCT behavior varies greatly according to patient age, histological subtype, and clinical stage. This review provides ideas for the future application of immunology in the fight against such diseases, which is a hot topic currently.

Purpose To provide a comprehensive understanding and propose a strategy for the management of extragonadal malignant germ cell tumors (EMGCTs) arising from extracranial and nonvaginal sites. Methods We retrospectively reviewed the cases of 12 patients with EMGCTs arising from extracranial and nonvaginal sites treated in our center over the past 38 years. Data on clinicopathological characteristics, treatment modalities, and follow-up information were analyzed. Results Among 209 patients diagnosed with EMGCTs, 12 women (5.7%) with EMGCTs of extracranial and nonvaginal sites were identified. These patients had tumors in the sacrococcygeal region ( n  = 4), abdominal cavity ( n  = 3), groin region ( n  = 2), uterus ( n  = 2), and mediastinum ( n  = 1). The median age at diagnosis was 23 years. Symptoms included abnormal uterine bleeding ( n  = 3), abdominal discomfort ( n  = 3), compression symptoms ( n  = 3), palpable mass ( n  = 2), and asymptomatic ( n  = 1). Yolk sac tumors (YSTs) were the most common histologic type. The median level of serum alpha-fetoprotein (AFP), a sensitive tumor marker, was 8216 ng/ml (2.7–74,157 ng/ml). One patient started bleomycin/etoposide/cisplatin without a pathologic diagnosis based on clinical diagnosis (high AFP levels and imaging findings), and 11 patients started chemotherapy following tumor biopsy or surgical resection. During the follow-up, one patient suffered a recurrence, two patients were alive with disease, and nine patients were disease-free. Conclusions Extracranial and nonvaginal EMGCTs are a heterogeneous group of tumors due to their varied onset ages, location, and clinical presentation. An all-around clinical evaluation is crucial for selecting appropriate treatment. Most patients achieve a good prognosis after surgical resection and chemotherapy. Patients with these rare diseases may benefit from individualized treatment and timely referral to experienced medical centers.

We report the first case of bilateral testicular tumors occurring after treatment for EGCT. Careful long‐term surveillance is essential because metachronous tumors may develop even several years after initial therapy.

Abstract Hepatoduodenal ligament teratomas are very rare, and there are fewer than 20 cases reported in the literature. A 22-year-old woman with fever and acute abdominal pain due a mature cystic teratoma in the hepatoduodenal ligament. This occurrence when presented with acute cholangitis was the first case of this rare tumor. Previous cases seen in adults were asymptomatic and were diagnosed incidentally on investigations. The mass was successfully removed operation on this patient without affecting the portal triad structures. This case adds to our knowledge of how these rare tumors may present clinically. It also highlights the relevance of this diagnosis in young adults with acute febrile illness and appropriate imaging characteristics.