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Tumors of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Some primary brain tumor types may benefit from neuraxis radiation because of their tendency to produce leptomeningeal spread or distal drop metastases. These include the following tumor types: Medulloblastoma.PNET.Germ cell tumors.High-grade ependymoma (WHO grade III).
Germ-Cell Cancer of the Testis and Related Neoplasms
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
A number of patients with germ-cell tumors present with symptoms from extra-gonadal disease. The most common symptoms in this situation are lumbar backache secondary to metastatic para-aortic lymphadenopathy. Where this disease is bulky there may be obstruction of the ureters(s) leading to hydronephrosis, bowel obstruction, or inferior vena cava occlusion (secondary to either tumor or vascular thrombus). Patients may also present with the signs and symptoms of deep vein thrombosis or pulmonary emboli. Patients with metastatic germ cell tumors (GCT) may present with lung metastases, manifested by chest pain, dyspnea, cough, or hemoptysis. Metastatic disease in the lung can be rapidly progressive, particularly in the choriocarcinoma syndrome,20 in which widespread vascular dissemination is common. Rarely, patients present with an inguinal lymph-node mass—usually a result of previous inguinal/scrotal surgery with resulting anomalous lymphatic drainage—or with a cervical nodal mass. Symptoms can arise due to involvement of the brain (e.g. headaches and fits) and bone (pain) though these represent a small proportion of presentations.
Hereditary Breast and Ovarian Cancer
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Affecting the germ cells, germ cell tumors include dysgerminoma (30%–50%), teratoma (20%), embryonal carcinoma (rare), and choriocarcinoma (very rare). Involving stromal cells (theca cells, fibroblasts, and Leydig cells) and gonadal primitive sex cords (granulosa cells and Sertoli cells), which are engaged in steroid hormone production (androgens, estrogens, and corticoids), sex cord−stromal tumors consist of pure stromal, pure sex cord, and mixed sex cord−stromal tumors. Of these, granulosa cell tumor (GCT) within the pure sex cord tumor category is most common, accounting for 5% of all malignant ovarian tumors [5].
Primary Mediastinal Germ Cell Tumors (PMGCT): A Real-world Analysis From A Tertiary Cancer Care Center in India
Published in Cancer Investigation, 2023
Aparna Sharma, Rohit Reddy, Raja Pramanik, Ranjit Kumar Sahoo, Seema Kaushal, K.P. Haresh, Sunil Kumar, Lalit Kumar, Atul Sharma, Atul Batra
Patients with primary extra gonadal germ cell tumors (EGGCT), a less commonly encountered subset of germ cell tumors, present with a mediastinal tumor in the absence of a gonadal primary (1). EGGCT is an uncommon entity and constitutes only around 1–5% of all germ cell tumors (2). Patients with EGGCT present as midline tumors extending variably from the pineal gland to the coccyx; the most common primary site being anterior mediastinum (50–70%) followed by retroperitoneum (30–40%) (3) and intracranial locations. Moreover, extragonadal GCTs are frequent in young children (sacrococcyx location). Furthermore, primary mediastinal germ cell tumors (PMGCT) constitute 12–16% of all mediastinal malignancies (4). They are broadly classified into two subtypes: seminomatous germ cell tumor, and nonseminomatous germ cell tumor (NSGCT) subtype that includes embryonal carcinoma (EC), teratomas (mature or immature), yolk sac carcinoma (YST) and choriocarcinoma and mixed histologies (2). Though EGGCT appear morphologically similar to the gonadal counterparts, they are clinically, molecularly and embryological distinct (5).
Balancing efficacy with long-term side-effects: can we safely de-escalate therapy for germ cell tumors?
Published in Expert Review of Anticancer Therapy, 2023
The long-term effects of chemotherapy and/or radiotherapy in managing germ cell tumors are significant. Minimizing the number of cycles given and avoiding unnecessary irradiation are important in minimizing long-term effects. Changes in practice which would reduce the risk of long-term effects are possible. The rarity of germ cell tumors and the concerns that any change in treatment may compromise the current excellent disease control are hampering the introduction of newer treatments in situations where definitive trials are unlikely. The introduction of miRNA technology in monitoring risk of relapse is likely to lead to more personalized treatment based on an individual’s response to therapy. The use of RPLND as a chemotherapy-sparing approach warrants further investigation. Extended follow-up is needed if any new approaches are necessary in case unanticipated late effects occur.
Pediatric Primary Yolk Sac Tumour of the Kidney: Recommendations for Pretreatment Diagnosis
Published in Fetal and Pediatric Pathology, 2023
Shilpi Thakur, Aanchal Kakkar, Manisha Jana, Prasenjit Das, Sandeep P. Agarwala, Venkateswaran K. Iyer
Germ cell tumors (GCTs) arise from differentiation of primordial germ cells. They most frequently occur in the gonads, but can arise at extra-gonadal sites, either in midline or non-midline locations. Extra-gonadal GCTs account for 1–2.5% of all GCTs, most commonly occur in the mediastinum and retroperitoneum, and have been reported at other unusual locations such as nasal sinuses, prostate, vagina and stomach [6]. Yolk sac tumor (YST) is a malignant GCT resembling extraembryonic structures including yolk sac, allantois, and extraembryonic mesenchyme [7, 8]. GCTs arising in unusual extragonadal locations are often unsuspected clinically, and due to their wide morphological spectrum, may resemble more common primary malignancies occurring in that organ. In core biopsies, all morphological features may not be evident, further enhancing the difficulty and resulting in erroneous diagnosis. We present an intrarenal YST initially misdiagnosed as Wilms tumor on core biopsy, which on resection was a YST, to emphasize that not all pediatric renal tumors are nephroblastoma. A pre-operative alpha-fetoprotein may have helped avoid this misdiagnosis.