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1.
J Egypt Natl Canc Inst ; 36(1): 15, 2024 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-38736004

RESUMO

BACKGROUND: Giant sacrococcygeal teratomas (SCTs) are at risk of perinatal morbidity and mortality due to their high vascularity. Pre-operative embolization of the feeding arteries, prior to complete surgical resection, may assist in minimizing the intraoperative blood loss by occluding these feeding arteries. CASE PRESENTATION: We present a case of a highly vascular giant SCT in a neonate, which was successfully embolized through an endovascular approach prior to surgery. The femoral artery approach was chosen, with access established using a Micropuncture introducer as a sheath. Embolization was performed using a combination of microcoils, Gelfoam slurry, and polyvinyl alcohol particles. The patient developed femoral artery spasm post-procedure, which resolved with the application of a glyceryl trinitrate patch. CONCLUSIONS: Performing pre-operative endovascular embolization on a giant sacrococcygeal teratoma presents particular challenges, primarily due to the difficulty in assessing small vessels and the potential complications associated with this procedure. Nevertheless, this technique proves exceptionally valuable in helping the surgeon minimize blood loss during surgery, thereby reducing the risks of morbidity and mortality. Comprehensive planning for the embolization procedure is essential, encompassing the identification of potential vascular access points and alternatives, along with careful selection of the appropriate catheter.


Assuntos
Embolização Terapêutica , Região Sacrococcígea , Teratoma , Humanos , Teratoma/terapia , Teratoma/patologia , Embolização Terapêutica/métodos , Recém-Nascido , Feminino , Procedimentos Endovasculares/métodos , Cuidados Pré-Operatórios , Resultado do Tratamento , Artéria Femoral/cirurgia , Artéria Femoral/diagnóstico por imagem
2.
Curr Oncol ; 31(4): 1831-1838, 2024 Mar 29.
Artigo em Inglês | MEDLINE | ID: mdl-38668041

RESUMO

Molecular analysis of the growing teratoma syndrome has not been extensively studied. Here, we report a 14-year-old boy with a growing mass during treatment for a mixed germ cell tumor of the pineal region. Tumor markers were negative; thus, growing teratoma syndrome was suspected. A radical resection via the occipital transtentorial approach was performed, and histopathological examination revealed a teratoma with malignant features. Methylation classifier analysis confirmed the diagnosis of teratoma, and DMRT1 loss and 12p gain were identified by copy number variation analysis, potentially elucidating the cause of growth and malignant transformation of the teratoma. The patient remains in remission after intense chemoradiation treatment as a high-risk germ cell tumor.


Assuntos
Teratoma , Humanos , Masculino , Teratoma/terapia , Teratoma/patologia , Adolescente , Neoplasias Encefálicas/terapia , Terapia Combinada
3.
Eur J Cancer ; 202: 114042, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38564927

RESUMO

AIMS: To resolve the ongoing controversy surrounding the impact of teratoma (TER) in the primary among patients with metastatic testicular non-seminomatous germ-cell tumours (NSGCT). PATIENTS AND METHODS: Using the International Germ Cell Cancer Collaborative Group (IGCCCG) Update Consortium database, we compared the survival probabilities of patients with metastatic testicular GCT with TER (TER) or without TER (NTER) in their primaries corrected for known prognostic factors. Progression-free survival (5y-PFS) and overall survival at 5 years (5y-OS) were estimated by the Kaplan-Meier method. RESULTS: Among 6792 patients with metastatic testicular NSGCT, 3224 (47%) had TER in their primary, and 3568 (53%) did not. In the IGCCCG good prognosis group, the 5y-PFS was 87.8% in TER versus 92.0% in NTER patients (p = 0.0001), the respective 5y-OS were 94.5% versus 96.5% (p = 0.0032). The corresponding figures in the intermediate prognosis group were 5y-PFS 76.9% versus 81.6% (p = 0.0432) in TER and NTER and 5y-OS 90.4% versus 90.9% (p = 0.8514), respectively. In the poor prognosis group, there was no difference, neither in 5y-PFS [54.3% in TER patients versus 55.4% (p = 0.7472) in NTER], nor in 5y-OS [69.4% versus 67.7% (p = 0.3841)]. NSGCT patients with TER had more residual masses (65.3% versus 51.7%, p < 0.0001), and therefore received post-chemotherapy surgery more frequently than NTER patients (46.8% versus 32.0%, p < 0.0001). CONCLUSION: Teratoma in the primary tumour of patients with metastatic NSGCT negatively impacts on survival in the good and intermediate, but not in the poor IGCCCG prognostic groups.


Assuntos
Neoplasias Embrionárias de Células Germinativas , Seminoma , Teratoma , Neoplasias Testiculares , Masculino , Humanos , Neoplasias Testiculares/tratamento farmacológico , Neoplasias Embrionárias de Células Germinativas/terapia , Prognóstico , Teratoma/terapia , Fatores de Risco , Estudos Retrospectivos
4.
Clin Oncol (R Coll Radiol) ; 36(6): e137-e145, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38565457

RESUMO

AIMS: Sinonasal teratocarcinosarcomas (SNTCS) are rare sinonasal malignancies, the incidence of which is less than 1% of all tumors. There is limited data available on SNTCS's, often as case reports and small case series. The management of SNTCS is complicated because of its location, locally aggressive biology, difficulty in achieving complete resection, and limited data on chemotherapy in these malignancies. This audit was performed to understand the role of neoadjuvant chemotherapy (NACT) in SNTCS's, its ability to downstage the disease, achieve complete resection, and impact on long-term survival outcomes. METHODS: This was a retrospective analysis of a prospectively maintained database approved by the Institutional Ethics Committee (IEC). The baseline characteristics, the extent of tumor, Kadish stage, NACT regimen, and adverse events were extracted from the Electronic Medical Records and the patient's case file. Patients with baseline extensive/inoperable disease were referred for NACT from the multidisciplinary joint clinic followed by response assessment (RECIST v1.1). Patients underwent skull-base surgery if respectable post-completion of NACT, however, if deemed unresectable were treated with non-surgical modalities or palliative therapies. RESULTS: The data of 27 patients were evaluated from the year 2015-2022. The median age was 42 years (IQR:30-56) and 85.2% (n = 23) were males. The ECOG-PS was 0-1 in 88.8% (n = 24) patients. All 27 patients received NACT in view of extensive disease at presentation. 74.1% (n = 20) patients received Cisplatin-Etoposide and 25.9% (n = 7) received other chemotherapy regimens. The median number of chemotherapy cycles was 2(IQR:2-3). 96.3% patients (n = 26) completed the planned NACT cycles. 70.4% (n = 19) patients achieved a partial response in post-NACT imaging. 77.8% (n = 18) underwent surgery, 18.5% (n = 5) received CTRT, and 7.4% (n = 2) received definitive-RT alone. The median PFS and OS of the cohort was 19months (95%CI:12.0-25.6) and 23months (95%CI:5.94-40.06) respectively. CONCLUSION: NACT is safe, feasible, and effective with significant response rates, leading to effective downstaging, resectability and improved survival in patients with locally advanced SNTCS's.


Assuntos
Carcinossarcoma , Terapia Neoadjuvante , Neoplasias Nasais , Centros de Atenção Terciária , Humanos , Masculino , Feminino , Estudos Retrospectivos , Índia , Adulto , Pessoa de Meia-Idade , Terapia Neoadjuvante/métodos , Carcinossarcoma/tratamento farmacológico , Carcinossarcoma/terapia , Carcinossarcoma/patologia , Neoplasias dos Seios Paranasais/tratamento farmacológico , Neoplasias dos Seios Paranasais/terapia , Neoplasias dos Seios Paranasais/patologia , Teratoma/tratamento farmacológico , Teratoma/terapia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Idoso , Quimioterapia Adjuvante/métodos
5.
Pediatr Blood Cancer ; 71(5): e30910, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38342954

RESUMO

BACKGROUND: The contribution of tumor type, multimodal treatment, and other patient-related factors upon long-term cognitive sequelae in infant brain tumor survivors remains undefined. We add our retrospective analysis of neuropsychological and quality of survival (QoS) outcome data of survivors of atypical teratoid/rhabdoid tumors (ATRT) and extracranial malignant rhabdoid tumors of the soft tissues (eMRT) and kidneys (RTK) treated within the same framework. Neuropsychological data from children with ATRT were compared to data from children with non-irradiated low-grade glioma (LGG). PATIENTS AND METHODS: Following surgery, patients (0-36 months at diagnosis) had received radio-chemotherapy (up to 54 Gy; ATRT: n = 13; eMRT/RTK: n = 7), chemotherapy only (LGG: n = 4; eMRT/RTK: n = 1) or had been observed (LGG: n = 11). Neuropsychological evaluation employing comparable tests was performed at median 6.8 years (ATRT), 6.6 years (eMRT/RTK), and 5.2 years (LGG) post diagnosis. RESULTS: We detected sequelae in various domains for all tumor types. Group comparison showed impairments, specifically in fluid intelligence (p = .041; d = 1.11) and visual processing (p = .001; d = 2.09) in ATRT patients when compared to LGG patients. Results for psychomotor speed and attention abilities were significantly below the norm for both groups (p < .001-.019; d = 0.79-1.90). Diagnosis predicted impairments of cognitive outcome, while sex- and age-related variables did not. QoS outcome for all rhabdoid patients displayed impairments mainly in social (p = .008; d = 0.74) and school functioning (p = .048; d = 0.67), as well as lower overall scores in psychosocial functioning (p = .023; d = 0.78) and quality of life (p = .006; d = 0.79) compared to healthy controls. CONCLUSION: Survivors of infant ATRT experience various late effects in cognition and QoS following multimodal treatment, while infant LGG patients without radiotherapy demonstrated comparable impairments in psychomotor and attention abilities. Early onset and multimodal treatment of rhabdoid tumors require close monitoring of neuropsychological and QoS sequelae.


Assuntos
Neoplasias Encefálicas , Neoplasias do Sistema Nervoso Central , Glioma , Neoplasias Neuroepiteliomatosas , Tumor Rabdoide , Teratoma , Criança , Lactente , Humanos , Tumor Rabdoide/complicações , Tumor Rabdoide/terapia , Estudos Retrospectivos , Qualidade de Vida , Teratoma/complicações , Teratoma/terapia , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/terapia , Neoplasias do Sistema Nervoso Central/patologia , Progressão da Doença , Percepção Visual , Cognição , Sobreviventes
6.
Horm Mol Biol Clin Investig ; 45(1): 35-39, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38282269

RESUMO

OBJECTIVES: Cancerous transformation in mature cystic ovarian teratoma is rare. Herein, we reported a case of squamous cell carcinoma transformation in mature cystic ovarian teratoma and performed an in-depth literature review to highlight the risk factors, prognosis, and suggested treatment for these patients. CASE PRESENTATION: We report a 66-years old postmenopausal woman diagnosed with a 120×90 (mm) mass at the left adnexa compatible with mature cystic ovarian teratoma. Following resection, the histopathological investigations showed malignant transformation in her mature cystic ovarian teratoma, and the immunohistochemistry for cytokeratin (CK) 5/6 and tumor protein 63 (P63) indicated squamous cell carcinoma transformation. She has been observed for her stage IA tumor and has been cancer-free for 6 months. CONCLUSIONS: Although malignant transformation in mature cystic ovarian teratoma is rare, it should be suspected if certain risk factors, e.g., elderly and high tumor size, exist. Stage IA patients' prognosis is favorable, and chemotherapy is not recommended.


Assuntos
Carcinoma de Células Escamosas , Neoplasias Ovarianas , Teratoma , Humanos , Feminino , Idoso , Neoplasias Ovarianas/patologia , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/patologia , Teratoma/diagnóstico , Teratoma/terapia , Teratoma/patologia , Prognóstico , Transformação Celular Neoplásica/patologia
7.
Oncology ; 102(2): 183-194, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-37634491

RESUMO

INTRODUCTION: Atypical teratoid rhabdoid tumor (ATRT) is among the most aggressive central nervous system malignancies. Although rare, this tumor typically afflicts young children and results in mortality within months. Here, we aim to determine key clinical features and treatment options that impact the survival of patients with ATRT. METHODS: From the year 2000 to 2019, 363 patients with ATRT were identified from the Surveillance, Epidemiology, and End Results database. Univariate analysis was used to identify variables that had a significant impact on the primary endpoint of overall survival (OS). Multivariable analysis was then used to identify independent predictors of survival. RESULTS: The median OS of the entire cohort was 13 months. Univariate analysis identified ages between 1 and 3 years, ages between 4 and 17 years, years of diagnosis between 2010 and 2019, and the receipt of treatment to have a significant impact on survival. In multivariable analysis, ages between 1 and 3 years and receipt of treatment were the only significant independent predictors of survival. The median OS was significantly greater in patients who received surgical treatment, chemotherapy, or radiation when compared to those who did not receive any treatment. In general, the receipt of any combination of therapies improved the median OS significantly. The receipt of triple therapy had the greatest impact on survival. DISCUSSION: This study highlights the survival benefit of a multimodal approach in the treatment of ATRT. The use of triple therapy, including surgery, radiation, and chemotherapy, was found to have the greatest survival benefit for patients. Overall, these findings may guide future care for patients with ATRT.


Assuntos
Neoplasias do Sistema Nervoso Central , Tumor Rabdoide , Teratoma , Criança , Humanos , Pré-Escolar , Lactente , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Neoplasias do Sistema Nervoso Central/patologia , Teratoma/terapia , Teratoma/tratamento farmacológico , Tumor Rabdoide/patologia , Tumor Rabdoide/cirurgia , Terapia Combinada
8.
BMJ Case Rep ; 16(10)2023 Oct 29.
Artigo em Inglês | MEDLINE | ID: mdl-37899082

RESUMO

In young women with anti-N-methyl-D-aspartate receptor (anti-NMDAR) autoimmune encephalitis (AE), co-occurrence with ovarian teratoma is common. While the management of mature teratoma with AE is well documented, literature on managing immature teratoma (IT) in tandem with AE is relatively scarce. Here, we report a case of a female patient in her early adolescence who presented with abdominal pain and was diagnosed with grade 3 IT combined with anti-NMDAR AE after an ovarian tumour was discovered and resected. Postsurgery, the patient received immunotherapy, chemotherapy and antiepileptic therapy, and two follow-up evaluations showed no signs of recurrence or sequelae. This case highlights the importance of a high index of suspicion for concurrent AE in the presence of ovarian teratoma, particularly IT, and the crucial role of concurrent administration of immunotherapy and chemotherapy following tumour resection in impacting prognosis.


Assuntos
Encefalite Antirreceptor de N-Metil-D-Aspartato , Neoplasias Ovarianas , Teratoma , Adolescente , Feminino , Humanos , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/terapia , Neoplasias Ovarianas/diagnóstico , Encefalite Antirreceptor de N-Metil-D-Aspartato/complicações , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico , Encefalite Antirreceptor de N-Metil-D-Aspartato/terapia , Teratoma/complicações , Teratoma/terapia , Teratoma/diagnóstico , Receptores de N-Metil-D-Aspartato
9.
BMC Womens Health ; 23(1): 497, 2023 09 19.
Artigo em Inglês | MEDLINE | ID: mdl-37726744

RESUMO

BACKGROUND: Struma ovarii (SO) is a rare tumor and may transform into ovarian strumal carcinoid (OSC) and/or malignant struma ovarii (MSO), but the incidence, clinical characteristics, and survival outcomes have not been well defined. METHODS: We conducted a retrospective study of patients with ovarian strumal diseases treated in the our hospital between 1980 and 2022. Subgroup analyses of SO, OSC, and MSO were subsequently performed. RESULTS: A total of 275 cases (2.14%) were identified in a cohort of 12,864 patients with ovarian teratomas, where SO, OSC, and MSO accounted for 83.3%, 12.0%, and 4.7% of cases, respectively. There were no significant differences in age, tumor sizes, elevated tumor markers, and ascites among the three subgroups. At initial treatment, all patients with SO or OSC had FIGO stage I disease except one SO patient presenting metastatic disease, ten patients had MSO confined to the ovary, whereas other three patients had metastatic diseases. Two patients with SO respectively relapsed at peritoneum and anterior mesorectum, while none of the OSC patients presented tumor recurrence or death despite different surgical procedures employed. The 5-year recurrence-free survival rate was 88.9%, and only one death occurred at 9.5 years after diagnosis in patients with MSO. Radioiodine therapy showed satisfactory therapeutic efficacy, but these patients showed poor responses to the chemotherapy. CONCLUSION: 2.14% of ovarian teratoma could be classified as SO, of which 12.0% and 4.7% of SO may transform into OSC and MSO, repsectively. The survival outcomes were excellent even after SO transformed into OSC or MSO. SYNOPSIS: SO occupied 2.14% of ovarian teratoma, where 12.0% and 4.7% of SO may transform into OSC and MSO, respectively, and had excellent survival outcomes.


Assuntos
Tumor Carcinoide , Neoplasias Ovarianas , Estruma Ovariano , Teratoma , Feminino , Humanos , Neoplasias Ovarianas/epidemiologia , Neoplasias Ovarianas/terapia , Estruma Ovariano/epidemiologia , Estruma Ovariano/terapia , Estudos Retrospectivos , Incidência , Radioisótopos do Iodo , Recidiva Local de Neoplasia , Teratoma/epidemiologia , Teratoma/terapia
10.
Pediatr Hematol Oncol ; 40(7): 629-642, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37519026

RESUMO

Atypical teratoid/rhabdoid tumor (AT/RT) is a rare aggressive central nervous system tumor that typically affects children under three years old and has poor survival with a high risk for neurologic deficits. The primary purpose of this study was to successfully treat the disease and delay or avoid whole-brain radiotherapy for children with AT/RT. A retrospective analysis was performed for six children diagnosed with AT/RT and treated with multimodal treatment at a single institute between 2014 and 2020. Furthermore, germline SMARCB1 aberrations and MGMT methylation status of the tumors were analyzed. One patient who did not receive a modified IRS-III regimen replaced with ifosphamide, carboplatin, and etoposide (ICE) in induction chemotherapy was excluded from this analysis. Five patients who received ICE therapy were under three years old. After a surgical approach, they received intensive chemotherapy and high-dose chemotherapy with autologous peripheral blood stem cell transplantation (HDCT/autoPBSCT) followed by intrathecal topotecan maintenance therapy. Three patients underwent single HDCT/autoPBSCT, and the other two received sequential treatment. Two patients with germline SMARCB1 aberrations and metastases died of progressive AT/RT or therapy-related malignancy, while 3 with localized tumors without germline SMARCB1 aberrations remained alive. One survivor received local radiotherapy only, while the other two did not undergo radiotherapy. All three surviving patients were able to avoid whole-brain radiotherapy. Our results suggest that AT/RT patients with localized tumors without germline SMARCB1 aberrations can be rescued with multimodal therapy, including induction therapy containing ICE followed by HDCT/autoPBSCT and intrathecal topotecan maintenance therapy without radiotherapy. Further large-scale studies are necessary to confirm this hypothesis.


Assuntos
Neoplasias do Sistema Nervoso Central , Tumor Rabdoide , Teratoma , Criança , Humanos , Lactente , Pré-Escolar , Topotecan/uso terapêutico , Tumor Rabdoide/tratamento farmacológico , Tumor Rabdoide/genética , Estudos Retrospectivos , Neoplasias do Sistema Nervoso Central/terapia , Neoplasias do Sistema Nervoso Central/tratamento farmacológico , Terapia Combinada , Carboplatina , Etoposídeo/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ifosfamida/uso terapêutico , Encéfalo/patologia , Teratoma/genética , Teratoma/terapia
11.
Adv Exp Med Biol ; 1405: 421-455, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37452948

RESUMO

Primary central nervous system germ cell tumors (CNS GCTs) are part of the GCTs in children and adults. This tumor entity presents with geographic variation, age, and sex predilection. There are two age peaks of incidence distribution at the first few months of life and in adolescence. CNS GCTs are heterogeneous in histopathological subtypes, locations, and tumor marker (AFP, ß-hCG) secretions. In the WHO CNS tumor classification, GCTS are classified as germinoma and nongerminomatous GCT (NGGCT) with different subtypes (including teratoma). Excluding mature teratoma, the remaining NGGCTs are malignant (NGMGCT). In teratoma, growing teratoma syndrome and teratoma with somatic-type malignancy should be highlighted. The common intracranial locations are pineal region, neurohypophysis (NH), bifocal pineal-NH, basal ganglia, and cerebral ventricle. Above 50% of intracranial GCTs (IGCTs) present obstructive hydrocephalus. Spinal tumors are rare. Age, locations, hydrocephalus, and serum/CSF titer of ß-hCG correlate with clinical manifestations. Delayed diagnosis is common in tumors arising in neurohypophysis, bifocal, and basal ganglia resulting in the increasing of physical dysfunction and hormonal deficits. Staging work-up includes CSF cytology for tumor cells and contrast-enhanced MRI of brain and spine for macroscopic metastasis before treatment commences. The therapeutic approach of CNS GCTs integrates locations, histopathology, staging, tumor marker level, and therapeutic classification. Treatment strategies include surgical biopsy/excision, chemotherapy, radiotherapy (single or combination). Secreting tumors with consistent imaging may not require histopathological diagnosis. Primary germinomas are highly radiosensitive and the therapeutic aim is to maintain high survival rate using optimal radiotherapy regimen with/without chemotherapy combination. Primary NGNGCTs are less radiosensitive. The therapeutic aim is to increase survival utilizing more intensive chemotherapy and radiotherapy. The negative prognostic factors are residue disease at the end of treatment and serum or CSF AFP level >1000 ng/mL at diagnosis. In refractory or recurrent NMGGCTs, besides high-dose chemotherapy, new therapy is necessary. Molecular profiling and analysis help for translational research. Survivors of pediatric brain tumors frequently experience cancer-related cognitive dysfunction, physical disability, pituitary hormone deficiency, and other CNS complications after cranial radiotherapy. Continuous surveillance and assessment may lead to improvements in treatment protocols, transdisciplinary interventions, after-treatment rehabilitation, and quality of life.


Assuntos
Neoplasias Encefálicas , Germinoma , Neoplasias Embrionárias de Células Germinativas , Neoplasias da Medula Espinal , Neoplasias da Coluna Vertebral , Teratoma , Criança , Adulto , Adolescente , Humanos , alfa-Fetoproteínas/metabolismo , Qualidade de Vida , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias Embrionárias de Células Germinativas/terapia , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/patologia , Germinoma/diagnóstico , Germinoma/patologia , Germinoma/terapia , Teratoma/diagnóstico , Teratoma/terapia , Encéfalo/metabolismo , Estudos Retrospectivos
12.
Childs Nerv Syst ; 39(8): 2019-2026, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37160436

RESUMO

BACKGROUND: Spinal atypical teratoid rhabdoid tumor (AT/RT) is an extremely rare tumor and represents less than 2% of all AT/RTs. METHODS: Available medical literature on spinal AT/RT in English was retrieved from PubMed and comprehensively reviewed. Clinical presentation, diagnosis, management, prognosis, and outcome in patients with spinal AT/RT have been elucidated by citing a case of extradural AT/RT of the cervicodorsal spine. RESULTS: The age at presentation is usually less than 3 years. The most common site is the cervicodorsal spine. The most frequent tumor location is intradural extramedullary. A contrast-enhanced magnetic resonance imaging (MRI) of the entire neuraxis is the imaging modality of choice. The incidence of leptomeningeal dissemination is high (15-30%). Histopathological examination shows an admixture of primitive neuroectodermal, mesenchymal, and epithelial elements along with rhabdoid cells. Loss of SMARCB1/INI1 is considered pathognomonic of AT/RT. Maximal safe resection of tumor is the initial management of choice. Thereafter focal radiotherapy for localized tumor or craniospinal irradiation for leptomeningeal dissemination should be considered. Post-operative intensive polychemotherapy including intrathecal and high-dose chemotherapy (with autologous stem cell rescue) is usually considered to optimize survival. Typically, the time to recurrence and overall survival are less than 6 and 12 months, respectively. However, with judicious multimodality management long-term survivors are increasingly being recognized. The illustrative patient was a 18-month-old girl diagnosed with extradural AT/RT of the cervicodorsal spine (C3-D1), who was managed with maximal safe resection of tumor, multiagent chemotherapy (ICE-ifosfamide, carboplatin, etoposide) and focal RT to the tumor bed-50.4 Gy/28 fractions/5.5 weeks. At the last follow-up visit, 30 months after surgery, she had complete clinicoradiological response. CONCLUSION: Multimodal treatment comprising maximal safe resection of tumor, multiagent chemotherapy (ICE), and focal RT can lead to successful outcome in patients with localized spinal AT/RT, under the age of 3 years.


Assuntos
Neoplasias do Sistema Nervoso Central , Tumor Rabdoide , Teratoma , Feminino , Humanos , Pré-Escolar , Lactente , Teratoma/diagnóstico por imagem , Teratoma/terapia , Tumor Rabdoide/diagnóstico por imagem , Tumor Rabdoide/terapia , Coluna Vertebral
13.
J Surg Res ; 288: 362-371, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37062232

RESUMO

INTRODUCTION: The purpose of this study was to perform a population-based investigation to assess the disease characteristics and prognosis of children and adolescents with malignant mediastinal germ cell tumors (MMGCT). METHODS: Data on the demographics, treatment, and survival outcomes of children and adolescents with MMGCT from January 1, 2000 to December 31, 2018 were obtained. To compare survival curves, the log-rank test was employed. The generation of survival curves based on different parameters was done using Kaplan-Meier estimations. Cox proportional hazards regression was performed to determine the variables linked to disease-specific survival. RESULTS: The selection criteria were met by 152 MMGCT patients, 130 of whom were male. Fifty three cases of mixed germ cell tumors (GCTs), 41 cases of malignant teratomas, 26 cases of yolk sac tumors, 14 cases of seminoma, 13 cases of choriocarcinomas, and five cases of embryonal carcinoma were reported. Overall survival at 3 and 5 y for all patients was 63.1% and 61.2%, respectively. Malignant teratoma, yolk sac tumors, and mixed GCTs in children and adolescents had comparable survival rates, while those with choriocarcinoma and embryonal carcinoma showed the worst prognosis. Embryonal carcinoma, malignant teratoma, mixed GCTs, and choriocarcinoma were found as risk factors by multivariate Cox proportional hazards analysis. In contrast, surgery and younger age were protective factors. However, chemotherapy alone showed no survival benefits. CONCLUSIONS: Our population-based evidence showed that MMGCT had worse prognosis in older children and adolescents. Choriocarcinomas and embryonal carcinomas had the worst prognosis. Surgery can prolong survival time. Chemotherapy and radiotherapy were not associated with improved prognosis.


Assuntos
Carcinoma Embrionário , Coriocarcinoma , Tumor do Seio Endodérmico , Neoplasias do Mediastino , Neoplasias Embrionárias de Células Germinativas , Teratoma , Neoplasias Testiculares , Gravidez , Feminino , Humanos , Masculino , Criança , Adolescente , Carcinoma Embrionário/patologia , Tumor do Seio Endodérmico/patologia , Neoplasias Embrionárias de Células Germinativas/epidemiologia , Neoplasias Embrionárias de Células Germinativas/terapia , Teratoma/epidemiologia , Teratoma/terapia , Teratoma/patologia , Neoplasias do Mediastino/epidemiologia , Neoplasias do Mediastino/terapia , Neoplasias Testiculares/patologia
14.
Neoplasia ; 37: 100880, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36773516

RESUMO

Atypical teratoid rhabdoid tumors (AT/RT) are malignant central nervous system (CNS) tumors that occur mostly in young children and have historically carried a very poor prognosis. While recent clinical trial results show that this tumor is curable, outcomes are still poor compared to other central nervous system embryonal tumors. We here review prior AT/RT clinical trials and highlight promising pre-clinical results that may inform novel clinical approaches to this aggressive cancer.


Assuntos
Neoplasias do Sistema Nervoso Central , Tumor Rabdoide , Teratoma , Criança , Pré-Escolar , Humanos , Lactente , Tumor Rabdoide/patologia , Tumor Rabdoide/terapia , Proteína SMARCB1 , Teratoma/patologia , Teratoma/terapia
15.
JNMA J Nepal Med Assoc ; 61(265): 727-730, 2023 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-38289795

RESUMO

Immature teratoma is one of the rare malignant germ cell tumours presented in pregnancy. Here, we present 26-year-old pregnant women who had an incidental finding of left adnexal mass in an anomaly scan at 19 weeks of pregnancy. Laparotomy with peritoneal fluid cytology, left salpingo-oophorectomy and omental biopsy at 20 weeks of pregnancy revealed immature teratoma stage 1A, grade 2 in the histopathology report. However, she followed up with the metastatic mass in the pouch of Douglas at 30 weeks of pregnancy in magnetic resonance imaging despite being counselled for possible chemotherapy and surveillance. A baby with a good Apgar score and grade 3 immature teratoma in the metastatic mass was revealed following the exploratory laparotomy and cesarean section at 36 weeks of pregnancy. Fertility-sparing surgery with chemotherapy during pregnancy for high-grade tumours may result in a good prognosis. Keywords: case reports; chemotherapy; immature teratoma; pregnancy; surgery.


Assuntos
Neoplasias Ovarianas , Complicações Neoplásicas na Gravidez , Teratoma , Gravidez , Feminino , Humanos , Adulto , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/cirurgia , Cesárea , Teratoma/diagnóstico , Teratoma/terapia , Salpingo-Ooforectomia/métodos , Complicações Neoplásicas na Gravidez/diagnóstico , Complicações Neoplásicas na Gravidez/cirurgia
16.
Diagn Pathol ; 17(1): 86, 2022 Oct 29.
Artigo em Inglês | MEDLINE | ID: mdl-36309682

RESUMO

BACKGROUND: Primary liver immature teratoma is extremely rare and only 4 cases have been reported, let alone with growing teratoma syndrome (GTS) and/or gliomatosis peritonei (GP). CASE PRESENTATION: Here, we report a case of a 44-year-old female presenting with progressive abdominal distension and elevated serum alpha fetal protein (AFP) level. CT/MRI scans revealed a large cystic-solid mass in the right lobe of the liver, accompanied with implant or metastasis in the abdominal cavity. Pathologic examination at biopsy suggested immature teratoma. After 4 cycles of chemotherapy, an MRI showed a slight increase in tumor size. Therefore, surgical resection of the right lobe of the liver was performed. The final histological diagnosis was a mature teratoma (tumor size 28 cm × 14 cm × 13 cm), with no residual immature component, and the diagnosis of GTS was considered. The patient continued to receive 2 courses of postoperative chemotherapy. An abdominal CT scan revealed innumerable miliary nodules in bilateral adnexal areas 2 months after surgery. Histologically, large numbers of mature glia were observed, supporting the diagnosis of GP. CONCLUSIONS: We report for the first time a case of primary liver immature teratoma with GTS and GP in an adult. Longer follow-up is needed to assess definitive efficacy.


Assuntos
Neoplasias Ovarianas , Teratoma , Adulto , Feminino , Humanos , Neoplasias Ovarianas/patologia , Teratoma/diagnóstico , Teratoma/terapia , Teratoma/patologia , Síndrome , Progressão da Doença , Fígado/patologia
17.
J Obstet Gynaecol Res ; 48(12): 3068-3076, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36053141

RESUMO

Mature cystic teratoma is the most common ovarian germ cell neoplasm. Malignant transformation is a rare occurrence, accounting for 1.5%-2% of cases. Malignant changes can arise from any constituent tissue of a teratoma; however, squamous cell carcinoma is the most common histologic type seen, followed by adenocarcinoma and sarcoma respectively. Tumor marker concentration levels, age, and the tumor maximum diameter are predictive indicators for malignant transformation. Proper diagnosis includes recognizing the possibility of malignant transformation versus excluding other differential options, such as metastasis. Primary cytoreductive surgery, adjuvant chemotherapy, and radiotherapy are the current treatment methods. The aim of the review is to discuss the clinical and pathologic features of malignant transformation within mature cystic teratomas, while reviewing the reported malignant types, differential diagnoses, and treatment options. Data sources include review of pertinent peer-reviewed literature on malignant transformation of mature cystic teratoma and cases seen in authors' institutional practice. Mature cystic teratomas are a commonly encountered benign ovarian tumor. However, the possibility of malignant transformation should remain in consideration, especially with given clinical or pathologic features: increased patient age, tumor size, or tumor marker levels. Thorough sampling of solid tumor foci can help identify malignant components. Awareness and proper diagnosis, along with early detection and clinical management, shows improved patient outcomes.


Assuntos
Cisto Dermoide , Neoplasias Ovarianas , Teratoma , Feminino , Humanos , Teratoma/diagnóstico , Teratoma/terapia , Transformação Celular Neoplásica/patologia , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/terapia , Neoplasias Ovarianas/metabolismo , Biomarcadores Tumorais
18.
Eur J Cancer ; 173: 59-70, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35863107

RESUMO

Ovarian immature teratoma is a rare subtype of germ cell tumour that can be pure or associated with non-teratomatous germ cell tumour elements and is graded based on extent of the immature neuroectodermal component. Immature teratoma (IT) can also be associated with somatic differentiation in the form of sarcoma, carcinoma, or extensive immature neuroectodermal elements and may produce low levels of serum alpha-fetoprotein. Variable interpretation of these issues underlies diagnostic and management dilemmas, resulting in substantial practice differences between paediatric and adult women with IT. The Malignant Germ Cell International Consortium (MaGIC) convened oncologists, surgeons, and pathologists to address the following crucial clinicopathologic issues related to IT: (1) grading of IT, (2) definition and significance of 'microscopic' yolk sac tumour, (3) transformation to a somatic malignancy, and (4) interpretation of serum tumour biomarkers. This review highlights the discussion, conclusions, and suggested next steps from this clinicopathologic conference.


Assuntos
Tumor do Seio Endodérmico , Neoplasias Embrionárias de Células Germinativas , Neoplasias Ovarianas , Teratoma , Adulto , Criança , Conferências de Consenso como Assunto , Tumor do Seio Endodérmico/tratamento farmacológico , Tumor do Seio Endodérmico/terapia , Feminino , Humanos , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias Embrionárias de Células Germinativas/terapia , Neoplasias Ovarianas/tratamento farmacológico , Neoplasias Ovarianas/terapia , Teratoma/tratamento farmacológico , Teratoma/terapia
19.
Curr Oncol ; 29(6): 4148-4154, 2022 06 07.
Artigo em Inglês | MEDLINE | ID: mdl-35735440

RESUMO

Growing teratoma syndrome (GTS) is rare and can mimic disease recurrence in patients with a history of immature teratoma. Benign hypermetabolic lymphadenopathy found on staging and surveillance computed tomography (CT) and positron emission tomography (PET) may lead to the presumption of metastatic malignancy. We report a case of a 38 year old with mixed mature and immature teratomas who developed new peritoneal masses after adjuvant chemotherapy despite a normalization of tumor markers. In addition to low FDG uptake observed in these peritoneal masses, a PET scan showed hypermetabolic lymphadenopathy and pulmonary and spleen lesions suggesting widespread metastases. Subsequent surgical resection confirmed a mixed pathology with GTS and sarcoidosis. We reviewed the current literature evidence of GTS and sarcoidosis as a benign cause of lymphadenopathy in cancer patients. We emphasize the importance of a tissue diagnosis before instituting therapy for presumed cancer recurrence to avoid potentially fatal diagnostic traps and management errors. A multiple disciplinary team approach is imperative in managing patients with suspected recurrent immature teratomas.


Assuntos
Linfadenopatia , Sarcoidose , Teratoma , Adulto , Humanos , Recidiva Local de Neoplasia , Tomografia por Emissão de Pósitrons , Sarcoidose/complicações , Sarcoidose/diagnóstico , Síndrome , Teratoma/tratamento farmacológico , Teratoma/terapia
20.
J Med Case Rep ; 16(1): 261, 2022 Jun 23.
Artigo em Inglês | MEDLINE | ID: mdl-35739572

RESUMO

BACKGROUND: We report a rare case in medical literature of a patient with pineal gland teratoma and uncommon metastases. Usually, metastases of this kind of tumor are located in several organs such as lung and breast, but here we found metastases to the spinal cord and vertebrae. CASE PRESENTATION: A 35-year-old Asian white man presented with diplopia and acute neural symptoms in the lower limbs such as numbness, tingling, and paralysis. His medical history was notable for pineal teratoma, treated 1 year previously with surgery, radiotherapy, and chemotherapy. Physical examination of the lower limbs showed absent reflexes and sensation with muscle power scale score of 1 in both limbs. Magnetic resonance imaging of brain and spine revealed many lesions in various locations, most compatible with neural, spinal, and vertebral metastases. Unfortunately, the patient died suddenly before any intervention was carried out. CONCLUSION: It is extremely rare for pineal region teratoma to metastasize to the spinal cord and vertebrae, thus more vigilant observation and examination should be provided to patients with pineal teratoma to detect any new lesions and prevent them from becoming dangerous.


Assuntos
Neoplasias Encefálicas , Pinealoma , Teratoma , Adulto , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/terapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pinealoma/diagnóstico por imagem , Pinealoma/terapia , Teratoma/diagnóstico por imagem , Teratoma/patologia , Teratoma/terapia
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