Management of Stage II Germ Cell Tumors: Be Sure, Be Patient, Be Safe

2019 ◽  
Vol 37 (22) ◽  
pp. 1856-1862 ◽  
Author(s):  
Christian K. Kollmannsberger ◽  
Lucia Nappi ◽  
Craig Nichols

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors’ suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A healthy 27-year-old man discovered a left testicular mass. Several months later he saw an urologist, who palpated a suspicious mass on the left testicle; an ultrasound confirmed a 2-cm solid mass. Serum tumor marker testing disclosed a slightly elevated alpha-fetoprotein (AFP) of 12.3 µg/L (upper limit of normal, 8.0 µg/L), and a normal β-human chorionic gonadotropin (HCG). Staging imaging with a contrast-enhanced computed tomography (CT) scan of the chest/abdomen/pelvis showed no evidence for retroperitoneal lymphadenopathy or distant metastases. He underwent a left radical orchiectomy, and pathology showed a 1.5-cm mixed germ cell tumor with 85% embryonal, 10% yolk sac tumor, and 5% mature teratoma histologies. Lymphovascular invasion was present. His AFP normalized after surgery. After discussion of management alternatives, he chose active surveillance, but 4 months later a scheduled surveillance CT scan identified a 1.4-cm left para-aortic lymph node just below the left renal hilum ( Fig 1 ). Serum tumor markers remained negative. He returns to discuss his results and potential management options.

2008 ◽  
Vol 52 (9) ◽  
pp. 1501-1504 ◽  
Author(s):  
Claudia Veiga Chang ◽  
Vânia dos Santos Nunes ◽  
Andre Carvalho Felicio ◽  
Marco Antonio Zanini ◽  
Malebranche B. C. Cunha-Neto ◽  
...  

Craniopharyngiomas and germ cell tumors (GCT) may affect the pituitary-hypothalamic region during childhood. Although different in origin, their clinical and radiological features may be similar. In this article we present a 5-year-old girl with clinical and radiological findings (computer tomography calcification) that were initially considered as craniopharyngioma. However clinical outcome, blood and cerebral spinal fluid tumoral markers, and results from anatomopathology and immunohistochemistry disclosed a mixed GCT. This case report highlights that some clinical features and radiological findings of pituitary-hypothalamic tumors may be misdiagnosed as craniopharyngioma mainly when there is a mature teratoma with cartilaginous tissue differentiation.


Neurosurgery ◽  
2015 ◽  
Vol 76 (6) ◽  
pp. 658-662 ◽  
Author(s):  
Hideki Ogiwara ◽  
Chikako Kiyotani ◽  
Keita Terashima ◽  
Nobuhito Morota

Abstract BACKGROUND: The role of second-look surgery in intracranial germ cell tumors (GCTs) needs to be reviewed. OBJECTIVE: To present our experience of second-look surgery in patients with intracranial GCTs who showed less than complete response despite normalizing or decreasing tumor markers after chemotherapy. METHODS: Retrospective review of 7 patients who underwent second-look surgery for an intracranial GCT was performed. RESULTS: Of 23 consecutive patients with newly diagnosed intracranial GCTs treated between August 2003 and August 2013, 7 patients (30%) underwent second-look surgery. The mean age was 9.4 years. The initial diagnoses were mixed germ cell tumor in 5 and immature teratoma in 2. Second-look surgery was performed after 1 to 3 courses of chemotherapy. Magnetic resonance imaging at the surgery demonstrated increasing residual tumor in 4 and stable residual tumor in 3. Tumor markers were normalized in 5 and nearly normalized in 2. Gross total resection was achieved in all patients. Histopathology at second-look surgery revealed mature teratoma in 5, fibrosis with atypical cells in 1, and fibrosis in 1. All patients subsequently underwent additional chemoradiation therapy according to the initial diagnosis. All patients are alive with no evidence of recurrence, with a mean follow-up of 48 months. CONCLUSION: Second-look surgery plays an important role in the treatment of intracranial GCTs. Surgery may be encouraged at a relatively early phase after chemotherapy when the residual tumor increases or does not change size despite normalized or nearly normalized tumor markers in order to achieve complete resection and improve outcome.


2016 ◽  
Author(s):  
Priyanka Soni ◽  
Shalini Mishra ◽  
Sandeep Jain ◽  
Gauri Kapoor

Background: Germ-cell tumors (GCT) are the commonest ovarian neoplasm in the first two decades of life. Aim: To study the profile of ovarian GCT in children and their outcome. Methods: Retrospective study of all cases of malignant ovarian GCT in the pediatric age (up to 18 years) was done from January 2002 to December 2015. The medical records of all admitted cases during this period were reviewed and the data was analysed with respect to age at diagnosis, clinical presentation, tumor markers, surgical stage, tumor histology, therapy, clinical course, and outcome. Results: Girls with malignant ovarian GCT were seen at our institute during the study period. Out of these 25 underwent treatment. Mean age at presentation was 11.7 years (range: 3-18 years). Abdominal pain was the commonest presentation. Twelve (47.3%) had right sided disease, 11 (42%) had left sided disease and 2 had bilateral disease. Twelve cases (57.8%) were diagnosed as stage I disease, 5 (10.5%) as stage II, 7 (26.3%) as stage III and 1 (5.2%) as stage IV. Elevated AFP >1000 was found in 9 (47.3%), elevated B-HCG (>50) in 7 (42%) and elevated LDH (>1000) in 7 (36.8%) patients at presentation. Twenty (73.6%) patients underwent surgery prior to chemotherapy out of which 4 (21%) patients presented after undergoing surgery at other centre. Fourteen (57.8%) patients received 4 cycles of BEP based chemotherapy, 6 (21%) received 3 cycles, 2 (10.5%) received 2 cycles and 1 patient did not receive any chemotherapy as it was mature teratoma. The most common histology was dysgerminoma in 8 (42%) patients followed by mixed germ cell tumor in 4 (21%), teratoma in 3 (15.7%), embryonal carcinoma in 2 (10.5%) and yolk sac tumor and mature teratoma in 1 patient each. Four (21%) patients had relapse on contralateral side which was salvaged. 1 patient presented with relapse who underwent only surgery outside, 1 patient had ovarian torsion. Median follow up is 27months. The event free survival rate was 78.9%. Conclusion: This study confirms an excellent outcome for girls with ovarian germ cell tumor. Patients with advanced surgical stage relapsed frequently. The mainstay of treatment is fertility preserving surgery and cisplatin-based chemotherapy.


2021 ◽  
Vol 5 (2) ◽  
pp. 139-147
Author(s):  
Nova Fenita Sari ◽  
RZ Nizar

Introduction : Germ cell tumors arise from primordial germ cells and account for about 30% of all ovarian tumors. More than 95% of this group are benign dermoid cysts (mature cystic teratoma) and the remaining 5% are malignant. Ovarian teratomas represent 15% to 20% of ovarian germ cell tumors. Teratomas are classified as mature or immature and often consist of several embryological layers. While the mature type is benign, the immature type is more aggressive.Objective : Based on the above, this article will review about immature teratoma and mature cystic teratoma of the ovary. Material and methods : The method of writing this scientific paper is a literature review. The data used are sourced from relevant literature and in accordance with the topics discussed.Result : Teratomas are a common form of germ cell tumors. Teratomas are histologically defined as tumors containing tissue derived from all germ cell layers: ectoderm, mesoderm, and endoderm. Teratomas are classified as immature teratoma, mature teratoma and monodermal teratoma. Conclusion : Teratomas are usually asymptomatic and if there are symptoms, they tend to be non-specific. In patients with no residual tumor after surgery, the survival rate is 90-100%.Keywords: Teratoma, Immature Teratoma, Mature Cystic Teratoma


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii330-iii330
Author(s):  
Hideki Ogiwara

Abstract OBJECTIVE The authors present their experiences of second-look surgery in patients with intracranial GCTs who showed less than complete response despite normalizing or decreasing tumor markers after chemotherapy. METHODS Retrospective review of 14 patients who underwent second-look surgery for an intracranial GCT was performed. RESULTS Of 40 consecutive patients with newly diagnosed intracranial GCTs treated between August 2003 and 2019, 14 patients (35%) underwent second-look surgery. The mean age was 9.2 years. The initial diagnoses were mixed germ cell tumor in 6, immature teratoma in 4, yolk sac tumor in 2, and germinoma 2. Second-look surgery was performed after 1–3 courses of chemotherapy. Magnetic resonance imaging (MRI) at the surgery demonstrated increasing residual tumor in 8 and stable residual tumor in 6. Tumor markers were normalized in 10 and nearly-normalized in 4. Gross total resection was achieved in 12 patients and near-total resection in 2. Histopatholgy at second-look surgery revealed mature teratoma in 6, immature teratoma in 3, fibrosis with atypical cells in 2, and fibrosis in 3. Eleven patients subsequently underwent additional chemo-radiation therapy according to the initial diagnosis. All patients are alive with no evidence of recurrence with a mean follow-up of 69 months. CONCLUSIONS Second-look surgery plays an important role in the treatment of intracranial GCTs. Surgery may be encouraged at a relatively early phase after chemotherapy when the residual tumor increases or does not change the size despite normalized or nearly-normalized tumor markers in order to achieve complete resection and improve the outcome.


Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110265
Author(s):  
Camille K Milton ◽  
Panayiotis E Pelargos ◽  
Nathaniel D Stetson ◽  
Manuel Maldonado-Vital ◽  
Kar-Ming A Fung ◽  
...  

The diagnosis and management of mixed intracranial germ cell tumors may be complicated by the diversity present within this tumor category. Mixed germ cell tumors demonstrate variable natural histories which may be altered by the inclusion of even the most minute immature histological components. We report the case of an 18-year-old male who presented with a 3-month history of progressive headache and nausea leading to lethargy. Imaging revealed a giant pineal region mass extending superiorly from the roof of the fourth ventricle into the lateral ventricle, with resultant obstructive hydrocephalus. No spinal lesions were noted. Following gross total resection, the patient experienced marked improvement. Pathologic analysis identified an uncommon tumor composition: mature teratoma (96%), immature teratoma (2%), and germinoma (2%). Guided by the immature component, chemotherapy and radiation were added post-operatively to provide this patient with the greatest chance of long-term survival. Intracranial pathology, including germ cell tumors, should be included in the differential for any young patient presenting with new and progressive headache and nausea. This case emphasizes the benefit of a multimodal approach to mixed germ cell tumors of the pineal region and the importance of careful pathologic review of all submitted material.


Author(s):  
Faraz S. Vali ◽  
Amit Kyal ◽  
Parul I. Chaudhary ◽  
Sujatha Das ◽  
Aprateem Mukherjee ◽  
...  

Germ cell tumors represent only 20% to 25% of all benign and malignant ovarian neoplasms. Mixed germ cell tumors are a rare variety of non–dysgerminomatous germ cell tumors. They contain two or more elements; the most frequent combination being a dysgerminoma and an EST (Endodermal Sinus Tumor). We present a case of malignant mixed germ cell tumor comprising of yolk sac tumor, embryonal carcinoma and choriocarcinoma. A 13-year-old girl presented with a huge 25 x 18 cm mass in abdomen with raised values of CA-125, hCG, AFP (alpha-feto protein) and LDH (lactate dehydrogenase). She underwent laparotomy followed by unilateral salpingoopherectomy and infracolic omentectomy. Histopathology report revealed malignant mixed germ cell tumor comprising predominantly of EST with elements of embryonal carcinoma and non-gestational choriocarcinoma. Following surgery, she was started on adjuvant chemotherapy (Bleomycin, Etoposide and Cisplatin regimen). Mixed germ cell tumor (YST/EST, non-gestational choriocarcinoma and embryonal carcinoma) is a very rare tumor. Careful initial surgery with adequate staging biopsies followed by combination chemotherapy can greatly improve the prognosis of these patients


2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii23-ii23
Author(s):  
Kaishi Satomi ◽  
Hirokazu Takami ◽  
Shintaro Fukushima ◽  
Yoichi Nakazato ◽  
Shota Tanaka ◽  
...  

Abstract BACKGROUND Gain of short arm of chromosome 12 (12p) is commonly observed in testicular germ cell tumors (tGCTs). 12p gain is also frequently seen in intracranial GCTs (iGCTs). However, little is known about the clinical significance of 12p gain in iGCTs. MATERIALS AND METHODS We have collected over 200 fresh frozen tissue samples of iGCTs through the Intracranial Germ Cell Tumor Genome Analysis Consortium in Japan. Firstly, we analyzed DNA methylation status in 83 iGCTs, 3 seminomas and 6 normal control samples using Infinium Human Methylation 450K BeadChip array (Illumina, CA). Idat files were processed using R (Version 3.5.3) and minfi package (1.30.0) to generate copy number variations. Compared with average genome-wide copy number level, 12p gain was determined. Then, 58 iGCTs with clinicopathological information were analyzed for progression-free survival (PFS) and overall survival (OS). Those tumors that consist of only either germinoma and/or mature teratoma components were classified as Favorable Histology (FH) and all the others that contains malignant histological components were classified as Unfavorable Histology (UFH). RESULT 12p gain was observed in 100% (3/3) of seminoma, 13.6% (3/22) of germinoma, 16.7% (1/6) of mature teratoma, 25% (1/4) of immature teratoma, 55% (11/20) of mixed germ cell tumor, 100% (4/4) of yolk sac tumor, 100% (1/1) of embryonal carcinoma, and 100% (1/1) of choriocarcinoma. In total, 44.6% (37/83) of iGCT showed 12p gain. Regarding histological classification, the 12p gain rate in UFH (72%, 18/25) was significantly higher than that in FH (12.1%, 4/33, P<0.01). Both PFS and OS were significantly worse in iGCTs with 12p gain (PFS: P=0.027, OS: P=0.0012). DISCUSSION 12p gain can be a molecular marker to predict prognosis and histological malignancy in iGCTs.


2018 ◽  
Vol 36 (19) ◽  
pp. 1898-1901 ◽  
Author(s):  
Ahsun Riaz ◽  
Robert Lewandowski ◽  
Riad Salem

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors’ suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A 68-year-old man with a remote history of alcohol abuse presented with vague abdominal pain. A review of systems suggested the patient had an Eastern Cooperative Oncology Group performance status 1 (restriction of strenuous physical activity). There were no physical examination findings of note. Laboratory studies disclosed Child-Pugh A liver status (no ascites; no encephalopathy; total bilirubin, 1 mg/dL; albumin, 3.5 g/dL; and international normalized rato, 1.2). The alpha-fetoprotein was mildly elevated (19.5 ng/mL). Magnetic resonance imaging with contrast disclosed an infiltrative mass with extensive malignant right and left portal vein thrombosis ( Fig 1A ) with cavernous transformation of the portal vein. The infiltrative mass ( Fig 2A ) was biopsied, revealing hepatocellular carcinoma. No distant metastases were found on a bone scintigraphy or computerized tomography scan. Given these features, this patient was classified as Barcelona Clinic for Liver Cancer stage C. The patient was referred for management of advanced hepatocellular carcinoma.


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