However, there is an increased incidence of mediastinal germ cell tumors m. Primary germ cell tumors in the mediastinum wiley online library. Mediastinal germ cell tumors have long been the subject of fascination for pathologists and developmental biologists as well as a subject of controversy for clinicians. Gct are thought to be derived from a common embryologic origin, with potential differences in the maturation stage of the cells transformed. Nonseminomatous germ cell tumors are cancer and are found in the brain. Primary mediastinal germ cell tumors are rare neoplasms that usually occur in young men. Pure seminoma is sensitive to radiotherapy and the prognosis is good. Extragonadal germ cell tumors comprise a rare subgroup of germ cell neoplasms, most of which arise in the anterior mediastinum. Mediastinal germ cell tumor and acute lymphoblastic.
Extragonadal germ cell tumors egcts are among the most perplexing. Ginsberg t he role of the surgeon in the management of mediastinal germ cell tumors has rapidly evolved over the past decade. Gct in ks, whereas the discussion of a generally higher tumor risk in this condition is still controversial. Primary germ cell tumors in the mediastinum takeda. Pdf ct of primary germcell tumors of the mediastinum. Primary mediastinal malignant gct pmmgct is a rare cancer contributing mediastinal tumors. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. Background primary mediastinal germ cell tumors gcts. On rare occasions, the tumor arises from extragonadal sites, including the sacrococcygeal region, uterus, vagina, prostate, retroperitoneum, liver, mediastinum commonly in the anterior, pineal gland, and third ventricle. Pediatric mediastinal germ cell tumors kakkar mediastinum.
View enhanced pdf access article on wiley online library html view. The term dermoid cyst is commonly applied due to the frequent expression of the ectodermal component of the teratoma. Primary mediastinal germ cell tumor the mediastinum is the most common site of extragonadal germ cell tumors 11, constituting 5070% of all extragonadal germ cell tumors. Incidentally discovered primary mediastinal germ cell tumor. A total of 54 patients with pmmgct in a followup from 1990 to 2009. Germ cell tumors are found widely throughout the body, and encompass a wide range of individual tumors this article does not deal with any specific body locations. Germ cell tumors are one of the causes of anterior mediastinal mass, and any of the germ cell. Primary mediastinal germ cell tumors are rare and account for 1 to 2 percent of germ cell tumors and 1 to 3. Mediastinal germ cell tumors in pediatric patients. Gonadal germ cell tumor is generally the most common germ cell tumor type and constitutes cases90% of germ cell tumors, whereas the mediastinum is the second most frequently affected area timodalityahead of other extragonadal areas, which include retroperitoneum, mentthe sacrococcygeal area, and the central nervous system 1. Only 3% to 8% of mediastinal germ cell tumors arise within the posterior mediastinum.
This case report describes a 24yearold man with an unusual presentation for an extragonadal germ cell tumor. Germ cell tumors in the mediastinum are rare and often occur in young patients but may occur in older patients. For detailed discussion please refer the articles listed at the end of this page. Although all pmnsgct arise in the anterior mediastinal compartment, the exact location, size, and degree of adjacent. Surgical management of primary mediastinal germ cell tumors. A study of patients with primary mediastinal germ cell. The most frequent site of extragonadal germ cell tumours is the anterior mediastinum 1. Because the thymus is a technically easily resectable structure with little consequence to the patient after removal, in the majority.
All patients received treatment with cisplatincontaining or carboplatincontaining therapy as their. Abstract background the histogenesis of extragonadal germ cell tumors remains an enigma. The most common histologic type of mediastinal germ cell tumor is mature teratoma, which is typically asymptomatic and incidentally discovered. Yolk sac tumors, also called endodermal sinus tumors are usually cancer.
Primary posterior mediastinal germ cell tumor in a child. Primary malignant mediastinal germ cell tumors are the most common extragonadal germ cell tumors in young adults pmmgct. Radiographically, these tumors appear as rounded, often lobulated masses. Technique of mediastinal germ cell tumor resection kenneth a. Histologically, primary mediastinal germ cell tumours mgcts. Fiftytwo patients underwent surgical resections, while. Confirmation that a gct of the mediastinum gctm is indeed a primary. The mediastinum is the most common anatomic site for extragonadal germ cell tumours in adults. Mediastinal germ cell tumors are uncommon tumors that occur predominantly within the anterior mediastinum and frequently present as a very large mass with local compression. This series was collected by a retrospective questionnaire completed on all the patients with mediastinal germ cell tumors seen and treated at the participating centers during the period of.
Potential complications of these masses due to localized invasion include hemoptysis, postobstructive pneumonia, and superior vena cava syndrome. Seminoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma and teratoma have distinct morphologic features with high grade nuclei. Childhood gcts are rare in children younger than 15 years, accounting for approximately 3% of cancers in this age group. The other 5 patients had mixed nonseminomatous germ cell tumors, 3 of which contained elements of yolk sac tumor. Primary mediastinal germ cell tumors pmgcts are rare, which often makes them difficult to treat. Malignant gct in the mediastinum, which account for 14% of. More than half of mediastinal germ cell tumors are mature teratomas.
Mediastinal tumors are relatively common in childhood and adolescence and are more common in boys than girls. Most mediastinal germ cell tumors arise within the anterior mediastinum, particularly the anterosuperior portion. Teratomas are germ cell tumors that consist of two to three layers of total three germinal layers 1. Primary malignant mediastinal germ cell tumor pmmgct is rare and sometimes the prognosis of the patients with pmmgct is not very satisfactory. While the event free survival was significantly higher in patients who received. Furthermore, primary malignant mediastinal germ cell tumors pmmgct are rather rare and represent only 1% to 4% of all mediastinal tumors. Pdf benign and malignant germ cell tumors of the mediastinum. Primary mediastinal germ cell tumor with intratubular germ cell. Multimodality treatment of germ cell tumors of the mediastinum. Primary malignant mediastinal germ cell tumors pmmgct are the most common extragonadal germ cell tumors in young adults 1,2.
Computed tomographic ct scans in four patients demonstrated large anterior mediastinal masses. Fine needle aspiration of mediastinal germ cell tumors. Cancers free fulltext targeting epcam by a bispecific. A rare subgroup of ks patients consists of prepubertal children with precocious puberty due to human chorionic gonadotropin hcg. Primary germ cell tumors of the mediastinum moran 1997. Malignant gct in the mediastinum, which account for 14% of mediastinal tumors, have different clinical characteristics from testicular gct despite sharing similar histologic and serologic features. Therefore, resection of the entire thymus gland affords the best chance of complete surgical resection. Primary germ cell tumors gct of the mediastinum share similar. Now many think that germ cell tumors contain fat and if a lesion does not contain fat, it cannot be a germ cell tumor.
Hematologic disorders associated with primary mediastinal nonseminomatous germ cell tumors article pdf available in jnci journal of the national cancer institute 921. Herein, we examined patients with pmgcts who underwent multimodal treatment. Anterior mediastinal germ cell tumors dr mostafa elfeky and assoc prof frank gaillard et al. We evaluated the role of the surgical treatment and the effect of multimodality treatment strategy for patients with pmmgct. An international germ cell tumor prognostic classification has been developed based on a retrospective analysis of 5,202 patients with metastatic nonseminomatous germ cell tumors and 660 patients with metastatic seminomatous germ cell tumors.
Although the histomorphological features of mediastinal gcts are similar to those of gonadal gcts, the biological behavior, clinical. Childhood extracranial germ cell tumors treatment pdq. However, discrepancies between the genetic alterations and immunoprofiles of mediastinal and testicular seminomas have been reported, raising the question of whether techniques that are useful in the diagnosis of gonadal seminoma are applicable to its mediastinal counterpart. Germ cell tumors compromise approximately 6% to 18% of mediastinal tumors, 77 and of these, 86% are benign. The most frequent site of extragonadal germ cell tumours is the anterior mediastinum. Cureus nonseminomatous extragonadal germ cell tumor. Mediastinal germ cell tumor an overview sciencedirect topics.
Extragonadal germ cell tumors gcts are mainly midline in location, with mediastinum being the most common site. Refer to the pdq summary on childhood central nervous system germ cell tumors treatment for information about the treatment of intracranial gcts. Pdf germ cell tumors gcts constitute a heterogeneous group of benign and. Management of the primary malignant mediastinal germ cell.
Before this, heroic attempts were made to totally excise these highly malignant tumors and, even with the rare total. Speculations regarding etiology have ranged from the opinion that these tumors are really of gonadal origin and represent spread from an occult or burned out primary tumor to the view that these tumors are of true. Technique of mediastinal germ cell tumor resection. Multiple different types of mediastinal masses may be encountered on imaging techniques in symptomatic and asymptomatic patients. They are a diagnostic challenge and have a worse prognosis than their gonadal counterparts. Histologically, primary mediastinal germ cell tumours mgcts classification includes seminomatous and nonseminomatous type. The most common extragonadal site of primary germ cell tumors is the anterior mediastinum.
Establishment of a leukemia cell line with i12p from a patient with a mediastinal germ cell tumor and acute lymphoblastic leukemia peter a. Prognostic factors in mediastinal germ cell tumors include. This finding is very specific for a germ cell tumor. Most germ cell tumors arise from the testicles and often are selfdiagnosed. Primary mediastinal germ cell tumors pmgcts account for less than 5% of germ cell malignancies and are more common in men but also occur in women. In our experience, the mediastinal site represents 7. Pdf primary mediastinal germ cell tumors in children and.
Mediastinal germ cell tumors gcts are a heterogeneous group of benign. Mediastinal germ cell tumor an overview sciencedirect. Four patients had teratocarcinoma or malignant teratoma of the mediastinum, and 3 had pure yolksac tumor. The majority of primary mediastinal germ cell tumors arise in the thymic tissue of the anterior mediastinum. The most common germ cell tumor, particularly in children, is benign teratoma. Yolk sac tumor yst, embryonal carcinoma ec, choriocarcinoma cc, and combined germ cell tumors cgcts of the mediastinum are uncommon neoplasms. Extragonadal germ cell tumors are rare and vary greatly in their clinical presentations.
Klinefelter syndrome and mediastinal germ cell tumors. While the eventfree survival was significantly higher in patients who received. Survival outcomes for men with mediastinal germcell. Primary germ cell tumors gct of the mediastinum are relatively rare and represent approximately 1015% of mediastinal tumors. P rimary germ cell tumors gct of the mediastinum are relatively rare and represent approximately 1015% of mediastinal tumors. Mediastinal germ cell tumors mgcts account for 19%25% of all mediastinal tumors and 3%6% of all gcts in pediatric age 1,2, 3, 19,20. Only sporadic cases have been documented in the literature. Germ cell tumors arise from embryonic tissue that failed to migrate during organogenesis 2. Yolk sac tumor is the most common malignant neoplasm of germ cell origin and usually occurs in infant testes or ovaries. The most frequent mediastinal masses in adults are thymoma, lymphoma, thyroid masses, and germ cell tumors. You have to remember, that only about 60 % of germ cell tumors contain fat, so you must realize that the absence of fat does not exclude a germ cell tumor from the.
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