Embryonal tumors in the WHO CNS5 classification: A Review

Embryonal tumors are a heterogenous group of neoplasms mostly defined by recurrent genetic driver events. They have been, previously, broadly classified as either medulloblastoma or supratentorial primitive neuroectodermal tumors (PNETs). However, the application of DNA methylation/gene expression profiling in large series of neoplasms histologically defined as PNET, revealed tumors, which showed genetic events associated with glial tumors. These findings led to the definitive removal of the term “PNET” in the 2016 World Health Organization (WHO) classification of CNS tumors. Moreover, further studies on a large scale of methylation profiling have allowed the identification of new molecular-defined entities and have largely influenced the 5th edition of the WHO classification of CNS tumors (WHO CNS5) for both medulloblastomas and other CNS embryonal tumors. The importance of molecular characteristics in CNS embryonal tumors is well represented by the identification of different molecular groups and subgroups in medulloblastoma. So, in the CNS5, the emerged group 3 and group 4 belong to the classification, and the four molecular and morphologic types are now combined into a unique section. Among other embryonal tumors, two new recognized entities are introduced in CNS5: CNS neuroblastoma, FOXR2-activated, and CNS tumor with BCOR internal tandem duplication (ITD). Embryonal tumor with multilayered rosettes (ETMR), already present in the previous classification now has a revised nomenclature as a result of the new DICER1 alteration, additional to the formerly known C19MC. Regarding atypical teratoid/rhabdoid tumor (AT/RT), three molecular subgroups are recognized in CNS5. The combination of histopathological and molecular features reflects the complexity of all these tumors and gives critical information in terms of prognosis and therapy. This encourages the use of a layered diagnostic report with the integrated diagnosis at the top, succeeded by layers including the histological, molecular, and other essential details.

Clinicopatholoical Spectrum Of Embryonal Brain Tumors And Their Prognostic Significance – A Three Years Institutional Study At A Tertiary Care Hospital In South India

BACKGROUND:Central nervous system tumors constitute second most common paediatric cancers.Emyonal tumors are neoplasms of immature cells resembling primitive neuroepithelium.All are similarly aggressive and have a tendency to disseminate throughout CNS.Therefore identification of specific subtype helps in prognosis evaluation,to avoid unnecessary treatment related neurotoxicity and further treatment implications. METHODS: This is a retrospective study conducted at The Department of neuropathology, Institute of neurosurgery MMC/RGGGH from January 2015 to December 2017.A total of 34 cases of Emyonal tumors were reviewed during this period.Among which Medulloblastoma was reclassified histopathologically based upon the World Health Organisation 2016 classification of CNS tumors.Data on clinical presentation and radiological features of all cases were collected from patients records.In all cases gross features were recorded during grossing of the resected tumors.The tissue sections were processed and stained as per standard protocols. IHC markers were done in deserving cases .Age predilection,Sex Predilection,Tumor location,Comparison with squash and Histological grade in relation to age of emyonal tumors studied. RESULTS:Out of 1422 cases evaluated in adults emyonal tumors comprised 0.21%(3 cases).Out of 150 cases evaluated in chidren aged 16 years emyonal tumors comprised 20.6%(31 cases).Sex ratio(males to females) 2:1 males outnumbering females.94.11%(32 cases) of emyonal tumors presented as posterior fossa tumors,2.94%(1 case) presented as hypothalamic SOL,2.94%(1 case) presented with multiple lesions in spine and cranium–This was a rare case of Atypical Teratoid /Rhabdoid tumor presented with drop metastasis.5.88%(2 cases) presented as recurrent tumors.82.3%(28 cases) presented histologically as classic medulloblastomas,2.94%(1 case) presented as Anaplastic/Large cell type, 2.94%(1 case) presented as Desmoplastic type,11.76%(4 cases) presented as Atypical Teratoid/Rhabdoid tumor.Most commonly affected age group is 6–10 years comprising 50%(17 cases),20.5%(7 cases) affecting 0–5 years, 20.5%(7 cases) affecting 11–16 years. CONCLUSION:Emyonal tumors are highly malignant tumors affecting children from early infancy to adolescence .Because of efforts to avoid craniospinal irradiation in an attempt to lessen treatment related neurotoxicity,diagnosis and management is very important.

Atypical Teratoid/Rhabdoid Tumor Presenting as a Cutaneous Lesion: A Case Report / 대한피부과학회지

An atypical teratoid/rhabdoid tumor (AT/RT) is a rare malignancy occurring in the first few years of life. This tumor shows rapid growth, a poor response to treatment, and poor prognosis. Cutaneous metastases presents as hamartomatous lesions mimicking skin tags. Immunohistochemical examination shows varied patterns of expression based on the sites of the body affected. Integrase interactor-1 (INI-1) gene sequencing and loss of expression of INI-1 observed with immunostaining can confirm AT/RT. In our patient, the skin lesion was identified at birth. Histopathological examination of the skin lesion could not establish an accurate diagnosis. Two months later, the patient presented with a brain tumor. Immunohistochemical examination of the brain lesion revealed complete loss of INI-1 expression in tumor cells, and the lesion was diagnosed as AT/RT. After that, we can detect the loss of INI-1 expression in the skin on the back. We report a rare case of AT/RT affecting the brain with cutaneous metastasis diagnosed with immunohistochemical staining.

Malignant Brain Tumours in Children : Present and Future Perspectives

In contrast to many of the malignant tumors that occur in the central nervous system in adults, the management, responses to therapy, and future perspectives of children with malignant lesions of the brain hold considerable promise. Within the past 5 years, remarkable progress has been made with our understanding of the basic biology of the molecular genetics of several pediatric malignant brain tumors including medulloblastoma, ependymoma, atypical teratoid rhabdoid tumour, and high grade glioma/diffuse intrinsic pontine glioma. The recent literature in pediatric neuro-oncology was reviewed, and a summary of the major findings are presented. Meaningful sub-classifications of these tumors have arisen, placing children into discrete categories of disease with requirements for targeted therapy. While the mainstay of therapy these past 30 years has been a combination of central nervous system irradiation and conventional chemotherapy, now with the advent of high resolution genetic mapping, targeted therapies have emerged, and less emphasis is being placed on craniospinal irradiation. In this article, the present and future perspective of pediatric brain malignancy are reviewed in detail. The progress that has been made offers significant hope for the future for patients with these tumours.

Malignant Brain Tumours in Children : Present and Future Perspectives

In contrast to many of the malignant tumors that occur in the central nervous system in adults, the management, responses to therapy, and future perspectives of children with malignant lesions of the brain hold considerable promise. Within the past 5 years, remarkable progress has been made with our understanding of the basic biology of the molecular genetics of several pediatric malignant brain tumors including medulloblastoma, ependymoma, atypical teratoid rhabdoid tumour, and high grade glioma/diffuse intrinsic pontine glioma. The recent literature in pediatric neuro-oncology was reviewed, and a summary of the major findings are presented. Meaningful sub-classifications of these tumors have arisen, placing children into discrete categories of disease with requirements for targeted therapy. While the mainstay of therapy these past 30 years has been a combination of central nervous system irradiation and conventional chemotherapy, now with the advent of high resolution genetic mapping, targeted therapies have emerged, and less emphasis is being placed on craniospinal irradiation. In this article, the present and future perspective of pediatric brain malignancy are reviewed in detail. The progress that has been made offers significant hope for the future for patients with these tumours.

Imaging findings of atypical teratoid/rhabdoid tumor of central nervous system with clinical correlation / 中华放射学杂志

Objective To evaluate the imaging characteristics of atypical teratoid/rhab doid tumor (AT/RT) of central nervous system(CNS), and to improve the diagnostic ability of the disease. Methods The clinical and imaging findings of 9 patients were retrospectively analyzed. There were 5 male and 4 female, ages 7 months to 5 years,median age was 1.4 years. MR enhancement studies were obtained in all the cases. One case had CT enhancement examination. Results The lesions were seen in brain in 8 cases and in lumbosacral spinal cord in one case. The tumors size varied from 4.8—7.8 cm, Necrosis was seen in nine cases, cystic change in eight cases and hemorrhage in five cases. The tumors had high signal on DWI, and low signal on ADC map. Dura matter invasion(2 cases), cerebrospinal fluid spread(2 cases)and intracerebral metastasis were seen. Conclusion There are some relatively specific imaging findings of primary CNS AT/RT that could assist their diagnosis.

Tumor rabdoide teratoide en paciente pediátrico: reporte de caso / Teratoid rhabdoid tumor in pediatric patient: case report

El tumor Rabdoide/Teratoide atípico (AT/RT) representa un subtipo tumoral primario de sistema nervioso central bastante raro, con un alto grado de malignidad y de pobre pronóstico (grado IV según OMS) encontrándose principalmente en niños menores de 3 años sin antecedentes de importancia y con cuadros clínicos bastante insidiosos que representa un reto en cuanto al diagnóstico y tratamiento teniendo en cuenta la limitada cantidad de casos reportados a nivel mundial, así como poca literatura disponible. Presentamos el caso de una paciente femenina de 23 meses de edad quien ingresa al servicio de urgencias del Hospital Militar Central (Bogotá, Colombia) en coma con midriasis izquierda, deshidratación grado 2, con escanografía cerebral simple de ingreso donde se observa lesión ligeramente isodensa fronto parieto occipital izquierda con sangrado intralesional que ejerce importante efecto de masa desplazando la línea media 22 mm y produce herniación del uncus, paraclínicos que reportan anemia, trombocitopenia y tiempos de coagulación prolongados, por lo cual se decide ingresar a salas de cirugía como urgencia vital realizando resección de lesión macroscópica de aspecto grisáceo, muy vascularizada, similar al tejido cerebral con sangrado fácil y muy difícil de controlar. 72 horas después requiere nueva intervención quirúrgica por inestabilidad hemodinámica durante el postoperatorio encontrando lesión de similares características, se envían muestras a patología reportando diagnóstico patológico definitivo: tumor Teratoide Rabdoide atípico grado IV OMS.

Atypical teratoid rhabdoid tumor (AT/RT) represents a subtype of malignant CNS neoplasms quite rare, with a high degree of malignancy and poor prognosis (WHO grade IV) found mainly in children younger than 3 years old without medical history of diseases and with Clinical characteristics quite insidious that represents a challenge in the diagnosis and treatment taking into account the limited number of cases reported, as well as little literature available. We report a 23 months of age female who is admitted to the emergency room of the Central Military Hospital (Bogotá, Colombia) in coma with mydriasis, dehydration, In the cranial scan showed injury slightly isodensa fronto-parieto-occipital left with intralesional bleeding that exerts important mass effect by moving the Midline of the brain 22 mm and produces herniation of the uncus, paraclinical reported anemia, thrombocytopenia and prolonged coagulation times, by which it decides to enter rooms for surgery making resection of macroscopic lesion of grayish look, highly vascularized, similar to the brain tissue with easy bleeding and very difficult to control. 72 hours after requires new surgical intervention for hemodynamic instability during the postoperative period finding similar lesion characteristics, samples are sent to Pathology reporting final pathological diagnosis: tumor atypical Teratoid Rhabdoid WHO grade IV by OMS.

Atypical Teratoid Rhabdoid Tumor in a Child: Case Report / Tumor teratóide rabdóide atípico emuma criança: relato de caso

Atypical teratoid/rhabdoid tumor (ATRT) is a rare and aggressive type of embryonal tumor of the central nervous system (CNS) occurring in childhood. The present study aims to describe the case of a 16-year-old girl who presented with an occipital mass lesion that was diagnosed as ATRT.We present a brief review of the current knowledge of the treatment of this rare neoplasm. A previously healthy 16-year-old girl was referred after two episodes of partial complex seizure 2 weeks before admission. MRI showed a right parieto-occipital lesion with homogeneous contrast-enhancing and significant surrounding brain edema. The patient underwent uneventful surgical resection of the lesion and was discharged home on the fifth postoperative day. Pathologic examination revealed it to be ATRT. The patient was referred to chemotherapy and radiotherapy. After 6 months of follow-up, the patient remains free of seizure and disease progression. ATRT is a rare and aggressive disease. Therefore, early diagnosis and treatment may improve the patient's prognosis and quality of life.

O tumor teratoide rabdoide atípico (TTRA) é um tipo de neoplasia rara, com comportamento maligno, que atinge o sistema nervoso central (SNC) de crianças. O objetivo do presente estudo é relatar o caso de uma paciente de 16 anos de idade admitida no nosso serviço com uma lesão occipital que revelou tratar-se de TTRA em estudo anatomopatológico. Uma paciente previamente hígida foi admitida no nosso serviço referindo ocorrência de dois episódios de crises convulsivas nas últimas 2 semanas. A ressonância magnética de crânio mostrou a presença de lesão occipital direita com captação homogênea do meio de contraste. A paciente foi submetida a cirurgia de ressecção da lesão expansiva sem intercorrências. A análise histopatológica e imuno-histoquímica revelou tratar-se de TTRA. A paciente foi encaminhada para a realização de quimioterapia e radioterapia complementares ao tratamento cirúrgico. Após 6 meses de seguimento ambulatorial, a paciente encontra-se assintomática, sem recorrência das crises convulsivas e/ou progressão radiológica da doença. TTRA é uma doença rara e agressiva. Com isso, diagnóstico e tratamento antecipados podem aprimorar prognóstico e qualidade de vida dos pacientes.

Adult-Onset Sellar and Suprasellar Atypical Teratoid Rhabdoid Tumor Treated with a Multimodal Approach: A Case Report

We report a very rare case of sellar and suprasellar atypical teratoid rhabdoid tumor (ATRT) in a 42-year-old female patient. The tumor was removed subtotally with a transsphenoidal approach. Histopathologic study showed rhabdoid cells with prominent nucleoli and abundant cytoplasm. Immunohistochemistry for INI1 was completely negative in the tumor cells, consistent with ATRT. After surgery, she received radiotherapy including spinal irradiation with proton beam therapy and subsequent chemotherapy, with no evidence of recurrence for more than 2 years. Up to date, this is the 8th case of an adult-onset ATRT in the sellar or suprasellar region. Despite its rarity, ATRTs should be considered in the differential diagnosis of an unclear malignant sellar or suprasellar lesion in adult patients and the treatment strategies for adult ATRT patients could be differentiated from those of pediatric ATRT patients.

Imaging and pathological features of atypical teratoid/rhabdoid tumors in the central nervous system / 实用放射学杂志

Objective To investigate the imaging and pathological features of atypical teratoid/rhab doid tumor (AT/RT)occur-ring in the central nervous system (CNS).Methods The CT and MRI findings of 1 6 patients with CNS AT/RT were retrospectively analyzed,and their pathological and immunohistochemical results were studied.Results There were 12 males and 4 females. Tumors located is at supratentorial in 10 and infratentorial in 6.Cystic changes in tumors were noted in 12 cases.The solid portion of tumors was isointense on T2-weighted images relative to normal brain grey matter in 9 patients.Extensive peritumoral edema was observed in 1 1 lesions.The tumors showed bandlike rim of significant enhancement in 10 cases.The incidence of hemorrhage and calcification in tumors were 43.8% and 41.7% respectively.Histopathologically,AT/RT was characterized by the presence of rhab-doid cells associated with variable components of epithelial,primitive neuroectodermal and mesenchymal differentiation.Conclusion Atypical teratoid/rhabdoid tumor is several imaging findings which are relatively specific on CT and MR images.CT and MRI may provide valuable information for pre-operation diagnosis and prognostic evaluation in patients with CNS AT/RT.

Intracranial Atypical Teratoid/Rhabdoid Tumor: Retrospective Analysis in a Single Institution / 대한소아혈액종양학회지

PURPOSE: Intracranial atypical teratoid/rhabdoid tumor (ATRT) is an extremely rare and aggressive tumor of early childhood. In this study, we evaluated the clinical characteristics, therapeutic approaches and outcomes of children who were treated at Seoul National University Children's Hospital (SNUCH). METHODS: We reviewed the clinical records of seven patients who were diagnosed as ATRT at SNUCH between January 2000 and July 2005. RESULTS: Of the seven patients, three patients were male and four were female. Median age at diagnosis was 13 months ranging from 3 months to 67 months. The tumors occurred in the infratentorial area in six and at multiple sites in one patient. Metastatic disease at diagnosis was present in two patients. One showed cerebrospinal fluid (CSF) dissemination and the other showed bony metastasis. Tumor excision was performed in all patients, and with the exception of two cases that refused further treatment, five patients received postoperative chemotherpay. One patient with CSF dissemination received radiotherapy and intrathecal chemotherapy as well. Of all the patients who received chemotherapy, two patients died during treatment because of tumor progression. The chemotherapy regimen was changed in three patients during treatment because the tumor showed poor response to chemotherapy. The median length of follow-up for five patients receiving chemotherapy was 6 months and the overall survival (OS) and event free survival (EFS) were 33.3% and 0%, respectively. CONCLUSION: Though various therapeutic approaches have led to improved survival in ATRT, the prognosis of ATRT is dismal compared with other brain tumors. A precise pathologic diagnosis is crucial and intensified treatment modalities should be considered according to the extent of tumor. To establish optimal treatment guidelines, a cooperative prospective study is needed and the efficacy of individual regimens should be analyzed.

Atypical Teratoid Rhabdoid Tumors in Adult Patient with Multiple Lesions

Primary atypical teratoid/rhabdoid tumor(AT/RT) of the central nervous system is a recently described, highly malignant neoplasm that usually occur in the posterior fossa of children. Although AT/RT usually occurs in young children, AT/RT is being recognized in adults with increasing frequency. The authors report 49-year-old man with multiple AT/RT lesions (right lateral ventricle, right temporal lobe and right cerebellum). Histopathologic findings showed typical rhabdoid cells with eccentric nuclei and prominent nucleoli. Eventhough the tumor was removed, a patient was dead in one month after surgery due to recurrence and rapid regrowth of the tumor.

A Case of Atypical Teratoid/Rhabdoid Tumor Arising from the Supratentorial Area / 소아과

Atypical teratoid/rhabdoid tumor may arise at any central nervous system location, but it is most commonly located in the cerebellum(60 percent). The incidence of this tumor remains unclear but it occurs most commonly in children less than 2 years of age. This highly malignant tumor shows a rapid progression and nonspecific radiologic findings. We report a case of primary intracranial atypical teratoid/rhabdoid tumor arising from the supratentorial area in early infancy. The diagnosis was made based on distinctive light microscopy and immunohistochemical findings. Despite aggressive surgical treatment with adjuvant chemotherapy, he died six months after his second operation.

A Case of an Atypical Teratoid/Rhabdoid Tumor of the Cerebellum / 대한소아신경학회지

Primary central nervous system atypical teratoid/rhabdoid tumors are rare and extremely aggressive malignancies of early childhood. These tumors are most common in infants less than 2 years of age. Diagnosis is based on distinctive light microscopic and immunohistochemical findings, coupled with a molecular genetic analysis. A histologic features of these tumors are epithelial and/or mesenchymal components in addition to rhabdoid, with or without neuroepithelial fields. The expression of the epithelial membrane antigen, vimentin, and the smooth muscle actin are characteristic of these tumors. Treatment includes surgery, chemotherapy, and radiotherapy. Prognosis is poor despite of an aggressive therapy. We report one case of an atypical teratoid/rhabdoid tumor in a young child. She is an 18 month-old-girl who presented with central type facial palsy. After extensive surgery she improved transiently but relapsed immediately. Her condition was not permitted to receive radiotherapy or chemotherapy. Thereafter, with phrenic nerve palsy, she suffered from recurrent episodes of pneumonia and respiratory difficulties. Finaly, she was expired three months after the diagnosis and treatment.

Clinicopathologic Findings of Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System

OBJECTIVE: Atypical teratoid/rhabdoid tumor(AT/RT) is a new entity among malignant pediatric brain tumors, and shows variable histopathologic features. The authors investigate the clinicopathologic and cytogenetic features of the tumor. METHODS: Five cases were included in this study ; three of them were primarily diagnosed, and two cases were reclassified from primitive neuroectodermal tumor/medulloblastoma to AT/RT. Mean age of patients at diagnosis was 5.6 years. The tumors were located in infratentorial or supratentorial areas. Maximum survival period was 13 months. RESULTS: Histopathologically, the tumors were mainly composed of modified rhabdoid cells and undifferentiated small cells, and mixed with epithelial, mesenchymal components, and other features mimicking glioma and chordoma. The histopathologic features were supported by polyphenotypic immunoreactivity, including epithelial membrane antigen, cytokeratin, vimentin, smooth muscle actin, and glial fibrillary acidic protein. Cytogenetic studies for karyotype analysis and fluorescent in situ hybridization revealed monosomy of chromosome 22 in two cases out of three cases of the tumor. CONCLUSION: Atypical teratoid/rhabdoid tumor may be an unique clinicopathologic entity, and histopathologic diagnosis should be made carefully by differentiating other polymorphous tumors of the brain.

Intracranial atypical teratoid/rhabdoid tumor:Pathological,clinical and imaging features / 医学研究生学报

Atypical teratoid/rhabdoid tumor of the central nervous system has aggressive growth with high potential CSF dissemination and frequently affects very young children.The tumor contains rhabdoid cells and primitive neuroectodermal,malignant mesenchymal and epithelial elements.Immunohistochemistry is helpful in differentiating atypical teratoid/rhabdoid tumor from primitive neuroectodermal tumor/medulloblastoma.Atypical teratoid/rhabdoid tumor is cytogenetically characterized by monosomy 22 or deletion of chromosome band 22q11.2.Intensified therapy combined with surgical resection has been suggested for its treatment.Due to the secondary cystic/necrotic changes and hemorrhage,the imaging features of the tumor are variable.

Atypical Teratoid/Rhabdoid Tumor in Central Nervous System: Report of 2 Cases

We report two cases of atypical teratoid/rhabdoid tumor(AT/RT) in the central nervous system. Primary central nervous system AT/RT is an extremely rare malignant tumor affecting infants and young children. These tumors have been diagnosed previously as primitive neuroectodermal tumors(PNETs) because AT/RT contains fields indistinguishable from classic PNETs. Separation of these two tumor types is crucial because the prognosis for AT/RT is poor even when treatment includes surgery with or without radiation therapy and/or chemotherapy. Clinical, radiological and histopathological features of AT/RT are presented.

Primary Intracranial Atypical Teratoid/Rhabdoid Tumor in a Child: A Case Report

Rhabdoid tumors of the central nervous system are rare malignancies. Primary central nervous system atypical teratoid/rhabdoid tumors (ATT/RhTs) mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumor-medulloblastoma (PNET-MB) but have different biological behaviors. We report a case of primary intracranial ATT/RhT in the posterior cranial fossa of a child. Preoperative radiological diagnosis was PNET-MB, but pathological diagnosis is ATT/ RhT. The case involved a 16-month-old baby boy who presented with severe headache, vomiting, and gait disturbance. He was treated by surgical resection, chemotherapy, and radiotherapy. Despite aggressive therapy, he died 19 months after diagnosis. Clinical, radiological, and histopathological features of primary intracranial ATT/RhT are discussed with a special emphasis on the differential diagnosis from PNET-MB.

Classical malignant rhabdoid tumor of central nervous system in 9-year-old Korean

A Malignant rhabdoid tumor (MRT) arising in the right temporoparietal lobe of a 9-year-old boy is described along with the results of an immunohistochemical study. The patient initially sought medical attention for a ptosis and right sided headache. The child underwent a subtotal resection of the tumor, followed by radiotherapy and systemic chemotherapy, but died three years after surgery. A MRT, a primary neoplasm of the central nervous system (CNS), is an entity of unknown histogenesis with a dismal prognosis, which only occurs in early childhood. Histologically similar tumors with more varied morphological features have been designated as an atypical teratoid/rhabdoid tumor. However, a classical MRT is extremely rare in the CNS and our case represents a classical CNS MRT.

A Case of Central Nervous System Atypical Teratoid/Rhabdoid Tumor of The 4th Ventricle : A Highly Malignant Tumor in Infancy and Childhood Frequently Mistaken for Medulloblastoma

Central nervous system atypical teratoid/rhabdoid tumor(CNS rhabdoid tumor) is a rare malignancy of uncertain origin. It typically occurs in infants and young children and comprises only a small fraction of pediatrics CNS malignancies. The tumor contains a large spindled cell component as classical rhabdoid morphology and focal areas resembling primitive neuroectodermal tumor. The tumor is defined histopathologically by the presence of rhabdoid cells, but contains considerable heterogeneity of the cell type, including the frequent presence of primitive neuroectodermal tumor. The prognosis for children with CNS rhabdoid tumor is dismal. We experienced a case of a three-year-old female who had been transferred to our hospital for seizure and vomiting. She was diagnosed as CNS atypical teratoid/rhabdoid tumor by biopsy. In spite of surgery and intensive postoperative multi-agents chemotherapy, she developed a local recurrence around the operation site at six months after surgery. We present this case with a brief review of related literatures.