Gonadal gct is the most common tumor cell type and constitutes 90% of the gcts, followed by mediastinal gct which affects the area ahead of the other. Rescorla, in pediatric surgery seventh edition, 2012 mediastinal germ cell tumors. 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. Pdf mediastinal germ cell tumors in childhood researchgate. These tumors are typically diagnosed in young adults and. Germ cell tumors are one of the causes of anterior mediastinal mass, and any of the germ cell histologies may be identified. Before you want to biopsy an anterior mediastinal mass, do not forget thta some of these lesions can be vascular in origin. P rimary germ cell tumors gct of the mediastinum are relatively rare and represent approximately 1015% of mediastinal tumors. Mediastinal germ cell tumors are primarily located in the anterior mediastinum, near the thymus gland, and make up about 10% to 20% of all childhood mediastinal tumors. In adults, extragonadal germ cell tumors account for 15% of primary anterior mediastinal tumors 12. Tumor markers were elevated, and a biopsy revealed a nonsemino matous mediastinal germ cell tumor.
Testicular ultrasonography us showed multiple, bilateral punctate echoes that are characteristic of tm. Histiocytic sarcoma following combination chemotherapy for. We herein report on a case of histiocytic sarcoma following combination chemotherapy for a primary mediastinal germ cell tumor in a 26yearold asian man who visited the general medicine department of a hospital with complaints of cough and high fever. Feb 19, 2014 background primary malignant mediastinal germ cell tumor pmmgct is rare and sometimes the prognosis of the patients with pmmgct is not very satisfactory. 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. An enigma of surgical consideration, authorfirdaus hayati and nurayub mohd ali and levin kesu belani and n. These tumors typically have yolk sac histology, are locally extensive or metastatic at the time of diagnosis, respond well to chemotherapy. Thymoma appears onctasaroundorovalsofttissue massintheanterior mediastinum 5. Highdose chemotherapy followed by autologous peripheral blood stem cell transplantation for recurrent primary mediastinal malignant germ cell tumor. Mediastinal germ cell tumor an overview sciencedirect. Primary mediastinal germ cell tumors are rare neoplasms that usually occur in young men. The most common testicular germ cell tumors are seminomas. It is rare the presence of a synchronous gonadal tumor, however in our experience 4. Mediastinal germ cell tumors in childhood the journal of thoracic.
This is the seventh case of extragonadal germ cell tumor with tm. Protocol for the examination of biopsy specimens from. Nov 18, 2020 primary mediastinal germ cell tumor pmgct is a rare and heterogeneous entity. Outcomes following surgery for primary mediastinal. Most mediastinal germ cell tumors arise within the anterior mediastinum, particularly the anterosuperior portion. Clinical information required to definitively assign stage eg, gross residual disease or presence of metastatic disease may not be available to the pathologist. The purpose of this study is to compare the clinical characteristics, biomarkers, and imaging findings of seminomas and nsgcts and to determine whether these features could help distinguish. Metastatic mediastinal germcell tumor and concurrent covid. In addition to physical examination and routine laboratory studies, initial evaluation should include ct of the chest and abdomen, and determination of serum levels of hcg and alphafetoprotein. Cog staging is based on pretreatment tumor characteristics. The most common germ cell tumor, particularly in children, is benign teratoma.
Mediastinal germ cell tumor and acute lymphoblastic. In our series, however, we observed an unusually high percentage of female patients 43. Fdgpet introduction a malignant mediastinal germ cell tumor gcts is rare and represents only 1% to 4% of all mediastinal tumors. Jan 09, 2021 histiocytic sarcoma is considered an extremely rare condition. 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. Primary mediastinal germ cell tumors are clinically classified into mature benign teratomas and malignant germ cell tumors. Louis, for symptoms that subsequently were proven to be due to a primary mediastinal germcell tumor. Intensive chemotherapy and radical resections for primary. Cureus mediastinal germ cell tumor with testicular. Mediastinal germ cell tumor and acute lymphoblastic leukemia.
Hematopoietic cell transplantation in the treatment of. Pediatric mediastinal germ cell tumors kakkar mediastinum. Germ cell tumors gcts are a heterogeneous group of malignant and nonmalignant neoplasms putatively originating from the same precursor, the primordial germ cell. Malignant gct in the mediastinum, which account for 14% of. Clinical input obtained in 2010 found strong support for autologous hct as a. Anterior mediastinal germ cell tumors radiology reference. The evidence is insufficient to determine the effects of the technology on health outcomes. This is the seventh case of extragonadal germ cell tumor. Highpower photomicrograph o f the seminomatous tumor shows a diffuse pattern composed of uniform cells.
One 2007 case report has described successful treatment of a refractory mediastinal germ cell tumor with allogeneic hct. 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. Mediastinal germ cell tumor an overview sciencedirect topics. Computed tomographic ct scans in four patients demonstrated large anterior mediastinal masses obliterating fat planes and ex. It has been estimated that extragonadal germ cell tumors may account for approximately 115% of mediastinal tumors in adults and approximately 25% in children classification and stagingour patient population was overwhelmingly composed of men 320 men vs. Primary mediastinal germ cell tumors in children and adolescents. Mediastinal germ cell tumors mediastinal tumors are relatively common in childhood and adolescence and are more common in boys than girls.
In our experience, the mediastinal site represents 7. Management of the primary malignant mediastinal germ cell. Only 3% to 8% of mediastinal germ cell tumors arise within the posterior mediastinum. Germ cell tumors of the mediastinum, which include benign teratomas, nevertheless are rare and represent only 1% to 6% of the total number of mediastinal tumors. Knapp rh, hurt rd, payne ws 1985 malignant germ cell tumors. The four ts make up the mnemonic for anterior mediastinal masses. Primary mediastinal malignant germ cell tumors gcts are rare and have a worse prognosis than their gonadal counterparts. 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. Even the germ cell tumors arise from the pluripotent cells of the thymus. Mediastinal germ cell tumour during the covid19 pandemic. Transthoracic needle biopsy of the mediastinal tumor was consistent with a mixed germ cell tumor. Surgical treatment of malignant mediastinal nonseminomatous. Primary malignant germ cell tumors of the mediastinum are categorized histologically as seminomas or nonseminomatous germ cell tumors.
Germ cell tumors are second only to lymphoma as the cause of a thymicanterior mediastinal mass. Primary mediastinal germ cell tumors mgct are unusual tumors that bear similar histopathological features to those occurring in. Germ cell tumors commonly occur in young men, mean age of 31 years1. Pdf germ cell tumors gcts constitute a heterogeneous group of benign and malignant neoplasms typically arising in the midline which are. Ct of primary germcell tumors of the mediastinum ajr.
A rare case of hemophagocytic lymphohistiocytosis in a. Zakaria and mohd ramzisham abdul rahman, journalcase reports in. Malignant germ cell tumors of ovarian origin are classified as dysgerminomas or nondysgerminomas. Pdf germ cell tumors of the mediastinum cesar moran. Most mediastinal malignant tumors are large and cause symptoms by compressing or invading adjacent structures, including. Ct is the modality of choice for the diagnostic evaluation of these tumors. Mediastinal germ cell tumors in pediatric patients.
Histologically, primary mediastinal germ cell tumours mgcts. The term dermoid cyst is commonly applied due to the frequent expression of the ectodermal component of the teratoma. Mediastinal tumor an overview sciencedirect topics. Malignant germ cell tumors of the mediastinum the journal of.
Einhorn department of medicine, indiana university. Nonseminomatous tumor types include embryonal cell tumor, yolk sac tumor, and teratomas. More than half of mediastinal germ cell tumors are mature teratomas. When chemotherapy is not deferrable nappi and colleagues recently reported the viewpoints of 53 highly experienced practitioners regarding the management of patients with germ cell tumors gcts during the covid19 pandemic 1. Voss, in oncology of infancy and childhood, 2009 germ cell tumor. However, among individuals aged less than 30 years the proportion of gcts among all malignancies is 11%. A rare case of hemophagocytic lymphohistiocytosis in a young.
Germ cell tumors gcts are classified as extragonadal if there is no evidence of a primary tumor in the testes or ovaries and based upon histology, they are further subclassified as seminomas termed dysgerminomas in women, nonseminomatous gcts termed nondysgerminomas in women, mature teratomas, and immature teratomas 1. Mediastinal germ cell tumors gcts are rare and usually located in anterior mediastinum. Mature teratomas should be surgically excised after diagnosis, because unnecessary delay may result in the rupture of tumors or in malignant degeneration. Occasionally, these tumors are found to arise in extragonadal sites, usually along midline structures, such as the pineal gland, retroperitoneum, mediastinum, and sacral area. Protocol for the examination of resection specimens from. We evaluated the role of the surgical treatment and the effect of multimodality treatment strategy for patients with pmmgct. Dulmet em, machiarini p, suc b, verley jm 1993 germ cell tumors of the mediastinum. Pdf benign and malignant germ cell tumors of the mediastinum. Pdf germcell tumors of the mediastinum cesar moran.
Primary mediastinal germ cell tumorsthe university of. Unlike benign germ cell tumors of the mediastinum, malignant mediastinal tumors are usually symptomatic at the time of diagnosis. Procedure biopsy consider extragonadal germ cell biopsy protocol tumor type testicular germ cell tumors consider the testis protocol. Primary malignant mediastinal germ cell tumors pmmgcts including seminomas and nonseminomatous germ cell tumors nsgcts are rare, and sometimes the diagnosis is very difficult. Germ cell tumors compromise approximately 6% to 18% of mediastinal tumors, 77 and of these, 86% are benign. Malignant nonseminomatous germ cell tumors are heterogeneous tumors with irregular borders due to invasion of adjacent structures. Keino d, kondoh k, murata s, ohyama r, morimoto m, muto s, fukuda m, wakisaka m, kitagawa h, kinoshita a. Nov 16, 2020 some germ cell tumors gcts in men develop into hematologic malignancies. Clinical and radiographic characterization of primary. Primary mediastinal germ cell tumor pmgct is a rare and heterogeneous entity. Mediastinal germ cell tumors are frequent in young men and there are few publications in women 1,2,6. Hematopoietic cell transplantation in the treatment of germ. The initial symptoms are usually pain, cough, dyspnea, cervical lymphadenopathy or superior vena cava syndrome 1,2,5.
Mediastinal tumors are relatively common in childhood and adolescence and are more common in boys than girls. Establishment of a leukemia cell line with i12p from a patient with a mediastinal germ cell tumor and acute lymphoblastic leukemia peter a. Mediastinal germ cell tumors gcts are a rare and heterogeneous. Successful resection of a mediastinal nonseminomatous germ. In over 95% of cases, the standard posteroanterior. These tumors are typically diagnosed in young adults and carry a poor prognosis. Primary mediastinal germ cell tumors gcts are rare in children and still represent a challenge for both adult and pediatric oncologists because of their worse outcome compared to their gonadal counterpart. Some germ cell tumors gcts in men develop into hematologic malignancies.
Germ cell tumors account for approximately 1215% of all anterior mediastinal tumors and of less than 0. Metastatic mediastinal germcell tumor and concurrent. We conducted this study to evaluate the role of radiotherapy on treatment outcomes and prognostic factors in pmgct that may allow a more adapted treatment strategy to improve survival. Mediastinal germ cell tumor mahua roy,1 rajat bandyopadhyay,2 narayan pandit,3 soumita sengupta4 introduction t he most common cause of svcs in children is nonhodgkin lymphoma nhl while in adults, it is lung cancer. Germ cell tumors are neoplasms that typically occur in the gonads where they originate from multipotential primitive germ cells. According to the international germ cell cancer consensus group, this represented poorrisk disease due to the mediastinal origin. The diagnosis of a mediastinal germ cell tumor should be considered in all young males with a mediastinal mass. Twelve mediastinal germ cell tumors were classified as pure teratoma, five contained embryonal carcinoma admixed with other germ cell components, and four. Methods a total of 54 patients with pmmgct in a followup from 1990 to 2009. The most frequent site of extragonadal germ cell tumours is the anterior mediastinum 1. Ct shows the location and extent of the tumors as well as intrinsic elements including soft tissue, fat, fluid, and calcification.
Mediastinal germ cell, pineoblastoma, hairy cell lymphoma, medullogastoma, uterine pecoma, small lung, neuroendocrine, granulosa cell tumor, rhabdomyosarcoma, anaplastic oligodentroglima, small cell cervical, appenendiceal mucinous reoplism and theres more you probably never heard of. Metastatic mediastinal germ cell tumor and concurrent covid19. Management of primary malignant germ cell tumor of the. Jan 27, 2021 mediastinal gcts represent nearly 2%4% of all gcts and account for 1%4% of mediastinal tumors. Germ cell tumors includes pediatric and adult patients with germ cell tumors located in the mediastinum, sacrococcygeal area, retroperitoneum, and neck the following should not be reported using this protocol. The prognosis after surgical removal of tumor is good and there. Mediastinal nonseminomatous germ cell tumors the annals of.
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