GCT-14. SECOND-LOOK SURGERY FOR INTRACRANIAL GERM CELL TUMORS

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.

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Second-Look Surgery for Intracranial Germ Cell Tumors

Neurosurgery ◽

10.1227/neu.0000000000000697 ◽

2015 ◽

Vol 76 (6) ◽

pp. 658-662 ◽

Cited By ~ 11

Author(s):

Hideki Ogiwara ◽

Chikako Kiyotani ◽

Keita Terashima ◽

Nobuhito Morota

Keyword(s):

Germ Cell ◽

Tumor Markers ◽

Mature Teratoma ◽

Germ Cell Tumors ◽

Residual Tumor ◽

Complete Response ◽

Mixed Germ Cell Tumor ◽

Second Look ◽

Intracranial Germ Cell Tumors ◽

Atypical Cells

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.

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Immature Teratoma and Mature Cystic Teratoma

JOURNAL OBGIN EMAS ◽

10.25077/aoj.5.2.139-147.2021 ◽

2021 ◽

Vol 5 (2) ◽

pp. 139-147

Author(s):

Nova Fenita Sari ◽

RZ Nizar

Keyword(s):

Germ Cell ◽

Mature Teratoma ◽

Relevant Literature ◽

Primordial Germ Cells ◽

Germ Cell Tumors ◽

Cystic Teratoma ◽

Residual Tumor ◽

Scientific Paper ◽

Immature Teratoma ◽

Mature Cystic Teratoma

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

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Evaluation of neoadjuvant therapy in patients with nongerminomatous malignant germ cell tumors

Journal of Neurosurgery Pediatrics ◽

10.3171/2011.1.peds10433 ◽

2011 ◽

Vol 7 (4) ◽

pp. 431-438 ◽

Cited By ~ 15

Author(s):

Hideo Nakamura ◽

Keishi Makino ◽

Masato Kochi ◽

Yukitaka Ushio ◽

Jun-ichi Kuratsu

Keyword(s):

Germ Cell ◽

Neoadjuvant Therapy ◽

Tumor Markers ◽

Chorionic Gonadotropin ◽

Human Chorionic Gonadotropin ◽

Tumor Recurrence ◽

Germ Cell Tumors ◽

Residual Tumor ◽

The Other ◽

Malignant Germ Cell Tumors

Object The authors evaluated the effectiveness of a neoadjuvant therapy (NAT) consisting of combined chemoand radiotherapy followed by complete resection of the residual tumor in patients with nongerminomatous malignant germ cell tumors (NGMGCTs). Methods The authors treated 14 consecutive patients in whom NGMGCTs were diagnosed based on elevated levels of the tumor markers α-fetoprotein, human chorionic gonadotropin, and the β-subunit of human chorionic gonadotropin (β-HCG). Chemotherapy and radiotherapy were performed, and after the serum tumor markers level was in the normal or near-normal range, the residual tumors were completely resected. Results Residual tumors were confirmed in 11 of the 14 patients after NAT, and total removal was successful in 10 of the 11 patients. In the other patient the residual tumor could not be completely excised because it was attached to a deep vein. The follow-up duration ranged from 1.2 to 22.2 years. The 5-year event-free and total survival rates were 86% and 93%, respectively. Although 3 patients died, 2 of tumor recurrence and 1 of a radiation-induced secondary tumor (glioblastoma), the other 11 are alive and without evidence of tumor recurrence. Conclusions The authors consider their NAT protocol for NGMGCT to be highly effective in relation to survival for the patients with NGMGCT, but there are several quality of life issues that need to be resolved.

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NI-11 Clinical significance of intracystic diffusion hyperintensity lesions remaining after treatment of intracranial germ cell tumor

Neuro-Oncology Advances ◽

10.1093/noajnl/vdaa143.059 ◽

2020 ◽

Vol 2 (Supplement_3) ◽

pp. ii14-ii14

Author(s):

Motoki Takano ◽

Takeshi Takayasu ◽

Ushio Yonezawa ◽

Akira Taguchi ◽

Kazuhiko Sugiyama ◽

...

Keyword(s):

Germ Cell ◽

Clinical Significance ◽

Cyst Wall ◽

High Intensity ◽

Mature Teratoma ◽

Germ Cell Tumors ◽

Residual Tumor ◽

Cystic Tumor ◽

Intracranial Germ Cell Tumor ◽

Intensity Lesion

Abstract Background and purpose: About 30% of intracranial germ cell tumors are mixed germ cell tumors and teratomas are often found as those components. Intense chemoradiotherapy is performed according to the malignancy of the histopathology, but high-intensity lesion inside the cystic tumor on diffusion weighted imaging (DWI) sometimes remains after completion of the chemoradiotherapy. In this study, we examined the clinical significance of the DWI high-intensity lesion remaining in the cyst. METHODS: Five patients after initial chemoradiotherapy were resected residual tumor by craniotomy at our hospital from 2009 to 2019. Preoperative gadolinium-enhanced MRI defined the non-contrast-enhanced part of the tumor as intracystic, and DWI intensity was classified by its look as low-intensity, equal-intensity, and high-intensity compared to the cortex of the same slice. DWI signals in the solid area, cyst wall, and cyst were evaluated. Results: All cases were mature teratoma in histopathology, and no other tumor components were observed. On DWI, the cyst wall and solid part were visualized with low signal. High-intensity lesions and equal-intensity lesions in the cyst cavity were found in 3 and 1 cases, respectively. In these cases, pathological findings revealed a keratin-like substance in the cyst. Discussion: The intracystic high and equal intensity lesions on DWI removed after completion of chemoradiotherapy are considered to reflect the keratin-like component of mature teratoma. If DWI- high intensity and equal intensity lesions remain in the cyst of the tumor after the completion of chemoradiotherapy, tumor shrinkage cannot be expected even if the chemotherapy is strengthened. In such cases, we should consider to removing them by surgery. Conclusion: When DWI high and equal intensity lesions are found in the cysts of tumors remaining after chemoradiotherapy for intracranial germ tumors, it is possible that mature teratoma remains.

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Mixed germ cell tumor of the pituitary-hypothalamic region presenting as craniopharyngioma: case report and review of the literature

Arquivos Brasileiros de Endocrinologia & Metabologia ◽

10.1590/s0004-27302008000900015 ◽

2008 ◽

Vol 52 (9) ◽

pp. 1501-1504 ◽

Cited By ~ 6

Author(s):

Claudia Veiga Chang ◽

Vânia dos Santos Nunes ◽

Andre Carvalho Felicio ◽

Marco Antonio Zanini ◽

Malebranche B. C. Cunha-Neto ◽

...

Keyword(s):

Case Report ◽

Germ Cell ◽

Mature Teratoma ◽

Germ Cell Tumors ◽

Cartilaginous Tissue ◽

Review Of The Literature ◽

Radiological Findings ◽

Hypothalamic Region ◽

Radiological Features ◽

Mixed Germ Cell Tumor

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.

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Novel Diagnostic Methods and Posttreatment Clinical Phenotypes Among Intracranial Germ Cell Tumors

Neurosurgery ◽

10.1093/neuros/nyaa108 ◽

2020 ◽

Vol 87 (3) ◽

pp. 563-572 ◽

Cited By ~ 2

Author(s):

Hirokazu Takami ◽

Avital Perry ◽

Christopher S Graffeo ◽

Caterina Giannini ◽

David J Daniels

Keyword(s):

Cohort Study ◽

Germ Cell ◽

Statistical Significance ◽

Mature Teratoma ◽

Germ Cell Tumors ◽

Extent Of Resection ◽

Diagnostic Methods ◽

Cell Fraction ◽

Clinical Phenotypes ◽

Second Look

Abstract BACKGROUND Central nervous system (CNS) germ cell tumors (GCT) are rare and complex pediatric neoplasms, the optimal management of which remains an area of active investigation. OBJECTIVE To present an updated cohort study, with particular attention to novel diagnostic methods and posttreatment clinical phenotypes. METHODS A single-institution cohort study of 80 primary, neurosurgically managed, CNS GCTs was conducted at Mayo Clinic, 1988-2017. RESULTS Postchemotherapy resection (eg, second-look surgery) was frequently required (27.0%), especially after adjuvant therapies for nongerminomatous GCTs (NGGCTs; 14 of 28 cases, excluding mature teratoma) and significantly associated with pineal lesions, as compared to neurohypophyseal or bifocal lesions (43.6% vs 5.9% vs 6.7%, P = .004), a finding that retained statistical significance after adjusting for index extent of resection and histology (P = .04). Essentially every NGGCT case underwent at least 1 craniotomy, either on presentation, as second-look surgery, or following local recurrence. Mature teratomatous tissue was highly incident in second-look specimens (84.2%), even among lesions initially diagnosed as germinomas. Pretreatment cerebrospinal fluid (CSF) cell fraction analysis demonstrated an association between single lesions and neutrophil predominance, whereas nongerminomatous GCTs were associated with increased monocyte fractions. CONCLUSION CNS GCTs are clinically heterogeneous lesions, resulting in numerous opportunities for improved understanding and clinical management via novel diagnostic and therapeutic protocols. Samples from second-look surgeries for recurrent germinomas frequently demonstrate teratomatous tissue, suggesting possible underdiagnosis of mixed GCTs—particularly among pineal lesions. GCT subtypes demonstrate differential cell fraction distributions on CSF analysis, a novel and perhaps diagnostically helpful finding that requires validation in external cohorts.

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Etoposide and Cisplatin Chemotherapy for Metastatic Good-Risk Germ Cell Tumors

Journal of Clinical Oncology ◽

10.1200/jco.2005.03.6616 ◽

2005 ◽

Vol 23 (36) ◽

pp. 9290-9294 ◽

Cited By ~ 71

Author(s):

G. Varuni Kondagunta ◽

Jennifer Bacik ◽

Dean Bajorin ◽

Deborah Dobrzynski ◽

Joel Sheinfeld ◽

...

Keyword(s):

Germ Cell ◽

Residual Disease ◽

Mature Teratoma ◽

Germ Cell Tumors ◽

Complete Response ◽

Late Relapse ◽

Standard Regimen ◽

Consensus Classification ◽

Good Risk

Purpose To assess response, overall survival, and relapse-free survival of patients with good-risk metastatic germ cell tumor (GCT) by International Germ Cell Consensus Classification Group (IGCCCG) criteria treated with four cycles of etoposide and cisplatin (EP). Patients and Methods Two hundred eighty-nine patients with IGCCCG good-risk GCT were treated with four cycles of EP. EP consisted of four cycles of etoposide 100 mg/m2 and cisplatin 20 mg/m2 on days 1 to 5 every 21 days. Results Two hundred eighty-two of 289 patients (98%) achieved a complete response; 269 (93%) responded to chemotherapy alone and 13 (5%) responded to chemotherapy plus surgical resection of viable disease (GCT other than mature teratoma). Seventeen (6%) experienced relapse, and nine (3%) died as a result of disease at a median follow-up of 7.7 years (range, 0.4 to 21.1 years). Sixty-two of 204 patients (30%) with nonseminoma had findings of teratoma or viable GCT at postchemotherapy surgery. Conclusion Four cycles of EP is a highly effective therapy for patients with good-risk GCT, with a high cure rate, low relapse rate, and little evidence of late relapse. Postchemotherapy surgery resection of residual disease remains an important aspect of treatment for these patients. Four cycles of EP is acceptable as a standard regimen for the treatment of good-risk metastatic GCT, and serves as an alternative to three cycles of bleomycin and etoposide before cisplatin.

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GCT-70. INTRACRANIAL GROWING TERATOMA SYNDROME IN CHILDREN

Neuro-Oncology ◽

10.1093/neuonc/noaa222.286 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii342-iii342

Author(s):

Maria Carter Febres ◽

Carol S Bruggers ◽

Holly Zhou ◽

Arie Perry ◽

John Kestle ◽

...

Keyword(s):

Germ Cell ◽

Obstructive Hydrocephalus ◽

Mature Teratoma ◽

Germ Cell Tumors ◽

Residual Tumor ◽

Complete Resection ◽

Pineal Tumor ◽

Tumor Biopsy ◽

Growing Teratoma Syndrome ◽

Multiagent Chemotherapy

Abstract Germ cell tumors account for less than 5% of all intracranial malignancies in children. Intracranial growing teratoma syndrome (GTS) is a rare pathophysiologic process characterized by growth of mature teratoma elements of a non-germinomatous germ cell tumor (NGGCT) during or following treatment with chemotherapy, in addition to normalization of or declining AFP/βHCG of the cerebral spinal fluid (CSF)/serum. A 13-year-old male presented with headache, emesis, and diplopia. MRI of the brain/spine revealed a localized 3.1 x 3.1 x 3.2 cm pineal tumor. Biopsy confirmed NGGCT (germinoma, immature and mature teratoma). Serum AFP (227ng/ul) and βHCG (12 IU/L) and CSF AFP (21ng/ul) and βHCG (31 IU/L) were elevated. Prior to cycle two of chemotherapy, he developed unstable gait and moderate hearing loss. Repeat MRI brain demonstrated tumor enlargement (4.4 x 5.2 x 5.1 cm) and obstructive hydrocephalus, although serum AFP/βHCG had normalized. Gross total resection of tumor confirmed GTS, without residual immature/malignant elements. Following six cycles of multiagent chemotherapy (carboplatin, etoposide, ifosfamide) and proton beam craniospinal irradiation (36 Gy with 18 Gy boost), he remains free of disease at eleven months since diagnosis. The pathogenesis of GTS remains unclear. Care must be taken to avoid misdiagnosing GTS as progressive NGGCT, as treatment and prognosis differ significantly. Second-look surgery, with a goal of complete resection, should be considered in cases of NGGCT when residual tumor grows during or following therapy, as this may represent GTS. Although histologically benign, GTS can be fatal. In patients with GTS, complete resection is usually curative.

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Malignant ovarian germ cell tumors in children: A single centre experience

10.1055/s-0039-1685314 ◽

2016 ◽

Author(s):

Priyanka Soni ◽

Shalini Mishra ◽

Sandeep Jain ◽

Gauri Kapoor

Keyword(s):

Germ Cell ◽

Germ Cell Tumor ◽

Mature Teratoma ◽

Germ Cell Tumors ◽

Stage Iv ◽

Contralateral Side ◽

Cell Tumor ◽

Excellent Outcome ◽

Mixed Germ Cell Tumor ◽

Single Centre Experience

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.

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

Journal of Clinical Oncology ◽

10.1200/jco.19.00502 ◽

2019 ◽

Vol 37 (22) ◽

pp. 1856-1862 ◽

Cited By ~ 3

Author(s):

Christian K. Kollmannsberger ◽

Lucia Nappi ◽

Craig Nichols

Keyword(s):

Germ Cell ◽

Ct Scan ◽

Mature Teratoma ◽

Relevant Literature ◽

Germ Cell Tumors ◽

Distant Metastases ◽

Management Options ◽

Mixed Germ Cell Tumor ◽

Left Testicle ◽

Management Approaches

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.

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