Radiation-induced Desmoid Tumor Development in the Radiotherapy Field in a Child With Pineoblastoma: A Case Report.

Although treatment-related secondary malignancies are rare, they are important problems after the treatment of childhood malignant diseases. Irradiation-induced sarcomas are the development of sarcoma different from the primary tumor after a latent period of ≥3 years or more in the radiotherapy field. Desmoid tumor is extremely rare as irradiation-induced tumor. A 7.5-year-old girl was referred to our hospital after a subtotal mass excision for a solid lesion with a cystic component located in the pineal gland. Pathologic examination revealed pineoblastoma. After surgery, craniospinal radiotherapy, and chemotherapy consisting of vincristine, cisplatin, and etoposide were performed. Painless swelling in the left parieto-occipital region ~75 months after the end of the treatment developed in the patient. A mass was detected in the intracranial but extra-axial region by radiologic imaging methods. Due to the total removal of the mass and the absence of a tumor in the surgical margins, she was followed up without additional treatment. The pathologic diagnosis was a desmoid tumor. She was followed up disease free for ~7 years after the primary tumor and ~7 months after the secondary tumor. Treatment-related desmoid tumor development after treatment for a central nervous system tumor in a child is extremely rare.

Cerebral developmental venous anomalies in children with mismatch repair deficiency.

BACKGROUND: Constitutional mismatch repair deficiency (CMMRD) is one of the rare cancer predisposition syndromes. The aim of this study was to evaluate the cerebral developmental venous anomalies in children with central nervous system tumors associated with CMMRD, an area in which there is extremely little experience. METHODS: Data from children diagnosed with medulloblastoma and high grade central nervous sytem tumor were retrospectively collected. According to the European CMMRD criteria, nine patients were diagnosed as CMMRD syndrome and the others consisted of the group without CMMRD. All radiological examinations of these children were retrospectively reviewed. Whole exome sequencing was performed to index cases` germline DNA. RESULTS: Nine children from four families, six females and three males, were studied. The median age at the first tumor diagnosis was 4.5 years (range, 9 months to 14 years). All CMMRD patients had café au lait spots, but none fulfilled the diagnostic criteria for neurofibromatosis. The patients developed high-grade glial tumor (n: 7) and medulloblastoma (n: 2). The affected genes in the three families were MSH6 [c.478C > T (p.Gln160Ter)], MSH6 [c.2871dupC (p.Phe958LeufsTer5)] and MLH1 [c.236G > A(p.Arg79Lys)], respectively. Seven patients had multiple developmental venous anomalies; six patients had leptomeningeal enhancement; and five patients had cavernomas. None of these findings were present in the group without CMMRD. CONCLUSIONS: Constitutional mismatch repair deficiency should be considered when multiple developmental venous anomalies, cavernomas, and leptomeningeal enhancement are detected, especially in patients with café au lait spots.

Familial clustering of nasopharyngeal carcinoma in the family of an adolescent with nasopharyngeal carcinoma.

BACKGROUND: Nasopharyngeal carcinoma (NPC) is one of the rare malignant diseases of childhood, of which only 1% occurs in children. In recent years, genetic factors have attracted attention in NPC. A very limited data have been reported about clustering within families. CASE: Herein, the familial clustering of nasopharyngeal carcinoma in the family of an adolescent with nasopharyngeal carcinoma is presented. CONCLUSIONS: There is familial clustering in nasopharyngeal carcinoma (NPC), but our knowledge on this subject is limited, especially in children or adolescent populations. Therefore, we should be more careful in NPC in childhood, especially in first-degree relatives.

A pediatric bithalamic high grade glioma with concomitant H3K27M and EGFR mutations.

BACKGROUND: Despite many treatment approaches, survival rates in high grade glial tumors are still not at the desired level. One of the cause of this failure might be that although having similar histologic features, they may display different biological behaviors depending on molecular heterogeneity. CASE: A 10-year-old girl presented with sudden onset left sided hemiparesis, headache, and ataxia. Physical examination was normal except for left sided hemiparesis and ataxia. A hyperintense mass lesion involving the bilateral thalamus was detected in the axial T2-weighted and coronal FLAIR sequences on brain MRI. There was no enhancement in axial T1-weighted contrast-enhanced sequences. Due to the size and location of the tumor, the patient was considered inoperable. Intensity modulated radiotherapy was intended for curative treatment to the patient because the radiological findings suggested a low-grade glial tumor. Tumor was unresponsive to radiotherapy but biopsy could be performed. The histopathological examination revealed a diffuse glial tumor with increased cellularity, mild nuclear atypia and rare mitosis. Due to the infiltrative pattern of the tumor, it was accepted as a high grade diffuse glial tumor. A chemotherapy protocol including cisplatin and etoposide in the first cycle, vincristine and cyclophosphamide in the second cycle, and carboplatin and vincristine in the third cycle were instituted to the patient. After the third cycle of chemotherapy, the tumor progressed radiologically. H3.1 K27M c.83A > T (HIST1H3C p.Lys28Met), ATRX c.2169_2170del (p.Glu723AspfsTer9), TP53 c.338T > C (p.Phe113Ser), and EGFR c.2300_2308dup (p.Ala767_va1769dup) were detected in the genetic assessment of tumor tissue. The patient`s treatment was changed to vincristine, temozolomide, and irinotecan. Unfortunately, MRI showed progression after three cycles of second-line chemotherapy. The patient`s family refused any further treatment, and the patient died with progressive disease in a short time. CONCLUSIONS: EGFR mutation along with H3.1 K27M mutation is extremely rare in children to our knowledge. It should be kept in mind that if there is a possibility of targeted therapy, there may be a treatment option in this malignant disease with a poor prognosis.

Impact on the survival of neutrophil-lymphocyte ratio, platelet-lymphocyte ratio, and monocyte-lymphocyte ratio on prognosis in children with Hodgkin lymphoma.

OBJECTIVES: To evaluate the clinical utility of the recently described absolute neutrophil counts to absolute lymphocyte counts ratio (NLR), absolute platelet counts to absolute lymphocyte counts ratio (PLR), and absolute monocyte counts to absolute lymphocyte counts ratio (MLR) on prognosis in children with Hodgkin lymphoma (HL). METHODS: We retrospectively evaluated the clinical characteristics, laboratory features (lymphocyte counts, NLR, PLR, and MLR), treatment, and results of 52 children with HL in the Department of Pediatric Hematology and Oncology, Selcuk University, konya, Turkey, from January 2006 until December 2021. RESULTS: The patients included 27 (51.9%) females and 25 (48.1%) males. The age of the patients ranged between 3-17.5 years old (median: 9 years). There were 22 patients in stage II, 24 in stage III, and 6 in stage IV. The most prevalent histopathological subgroup was the nodular sclerosing type (53.8%). The 5-year overall survival rate was 93.7%. The overall survival rate differed based on lymphocyte counts (p<0.0001), NLR (p=0.018), and PLR (p=0.009). However, none of the prognostic factors in the univariate analysis were not prognostic risk factors (p>0.05) in the multivariate analysis. CONCLUSION: Lymphocyte counts, NLR, and PLR may be useful markers for determining the outcomes in children with HL.

Clinical Utility of Total Oxidative Stress and Total Antioxidant Capacity in Childhood Febrile Neutropenia.

The aim is to determine the oxidative status of children with febrile neutropenia (FEN). Blood samples were collected to determine the total antioxidant capacity (TAC) and total oxidative status (TOS) of healthy children (once) and children with FEN after 0, 48, and 96 hours. Eighteen patients with FEN were evaluated. The baseline TAC level of patients was significantly higher than that of the controls (P<0.0001). The TAC levels of patients with FEN with and without antibiotic modification were higher than those of the controls (P=0.002 and 0.02, respectively). The TAC levels of the patients with FEN with antibiotic modification were lower than those of the patients without antibiotic modification (P=0.0224). The oxidative stress index (OSI), calculated TOS/TAS, value of the children with FEN was lower than that of the controls (P<0.0001). The OSI values of the patients with FEN with and without antibiotic modification were lower than those of the control group (P=0.001 and <0.0001, respectively). The TAC values of the patients with antibiotic modification were higher than those of the patients without antibiotic modification (P=0.02). In conclusion, the oxidative status of the children with FEN was affected, and it can give information about the follow-up of FEN.

Cancer among syrian refugees living in Konya Province, Turkey.

BACKGROUND: With more than 3.6 million Syrian refugees Turkey hosts the world's largest number of Syrians. Considering the morbidity, mortality, and healthcare spending, cancer is one of the leading health and economic burden for patients and healthcare systems. However, very limited information available in the scientific literature to understand the burden and characteristics of cancer in countries hosting Syrian refugees. The aim of the present study is to evaluate the demographic and clinical characteristics, treatment outcome of Syrian cancer patients living in Konya, Turkey. METHODS: We retrospectively reviewed medical records of Syrian cancer patients at three major institutions from 2005 to 2020. The information regarding demographic and clinical characteristics of patients were identified. The number of days between the first symptom and diagnosis was considered as the "diagnostic interval". Patients who failed to attend clinics within four weeks of appointment were assumed abandoned treatment. Survival curves were estimated using the Kaplan-Meier method. RESULTS: We identified 230 adult and 38 children refugee diagnosed with cancer during the study period. With regards to adult patients, there were 114 (49.6%) male and 116 (50.4%) female. The median age at diagnosis was 52.4, 47.3 years for male, female respectively. The five most common cancer by site among all were; breast (24.8%), colorectal (10.9%), lung (7.4%), central nervous system (CNS) (7.0%), and stomach (5.2%). 93 (40.4%) had metastatic disease at diagnosis. The overall survival probability was 37.5% at five years for the adult population. Data were extracted for 20 boys and 18 girls with childhood cancer. Their median age at diagnosis was 5.8 and 6.0 years respectively. The three most common childhood cancer were; leukemias (21.1%), lymphomas (21.1%), and CNS (13.2%). Excluding leukemia, 13 (43.3%) of childhood cancer cases had the advanced disease at diagnosis. Three year survival probality was 69.5%. The median diagnostic interval for adult and childhood cancer was 96.5 (IQR = 53-165) and 23 (IQR = 13.5-59) days respectively. Twenty-one adults and four children had treatment abandonment. CONCLUSION: This study contributes to understanding the burden of cancer among Syrian refugees living in Konya, growing health issue for refugees. Larger and prospective studies will help to measure the real burden and compare the difference in cancer risk factors, care, and outcomes among the refugee and host populations.

Clinical Features, Treatment and Outcome of Childhood Glial Tumors.

AIM: To evaluate the clinical features, treatment approaches, and outcomes of glial tumors in children. MATERIAL AND METHODS: Files (2006 to 2020) of children diagnosed with glial tumors and followed-up were reviewed retrospectively. Information regarding demographic and clinical characteristics, treatment approaches, and outcomes were retrieved from the patients? files. RESULTS: Of the total of 180 pediatric patients diagnosed with brain tumors, 73 (40.6%) had glial tumors. The children with astrocytoma were in the age range of 2?18 years (median age: 8.7 years), while the ages of children with ependymoma ranged from three months to 10 years (median age: 3 years). This difference was statistically significant (p < 0.0001). The male to female ratio was 1.6. The most common symptoms or signs were headaches (n=34, 46.6%), abnormal gait or coordination (n=22, 30.2%), vomiting (n=21, 28.8%), and cranial nerve palsies (n=20, 27.4%). The pathological diagnoses were astrocytomas (n=53, 72.6%), oligodendroglial tumors (n=2, 2.7%), ependymoma (n=15, 20.7%), and other glial tumors (n=3, 4.1%). The most common tumor location was supratentorial (n=42, 57.5%), while midline glioma was detected in seven patients. The 5-year overall survival (OS) rate of all glial tumors, astrocytoma, and ependymoma was 42%, 40%, and 55%, respectively. The 5-year OS rate of the tumor Grade I, II, III, and IV was 77.2%, 45%, 32%, and 0%, respectively (p < 0.0001). The 5-year OS rate of supratentorial, infratentorial, and spinal tumors was 25.6%, 63.6%, and 50%, respectively (p=0.021). In Cox regression analysis, it was found that the tumor resection and grade had an effect on the tumor prognosis. CONCLUSION: Treatment results are not satisfactory in high-grade astrocytomas. There is a need for new treatment approaches that would take cognizance of molecular features and adopt multidisciplinary approaches.

Correlation of myeloid-derived suppressor cells with C-reactive protein, ferritin and lactate dehydrogenase levels in patients with severe COVID-19.

The novel coronavirus disease 2019 (COVID-19) remains a global health emergency, and understanding the interactions between the virus and host immune responses is crucial to preventing its lethal effects. The expansion of myeloid-derived suppressor cells (MDSCs) in COVID-19, thereby suppressing immune responses, has been described as responsible for the severity of the disease, but the correlation between MDSC subsets and COVID-19 severity remains elusive. Therefore, we classified patients according to clinical and laboratory findings-aiming to investigate the relationship between MDSC subsets and laboratory findings such as high C-reactive protein, ferritin and lactate dehydrogenase levels, which indicate the severity of the disease. Forty-one patients with COVID-19 (26 mild and 15 severe; mean age of 49.7 ± 15 years) and 26 healthy controls were included in this study. MDSCs were grouped into two major subsets-polymorphonuclear MDSCs (PMN-MDSCs) and monocytic MDSCs-by flow cytometric immunophenotyping, and PMN-MDSCs were defined as mature and immature, according to CD16 expressions, for the first time in COVID-19. Total MDSCs, PMN-MDSCs, mature PMN-MDSCs and monocytic MDSCs were significantly higher in patients with COVID-19 compared with the healthy controls (P < .05). Only PMN-MDSCs and their immature PMN-MDSC subsets were higher in the severe subgroup than in the mild subgroup. In addition, a significant correlation was found between C-reactive protein, ferritin and lactate dehydrogenase levels and MDSCs in patients with COVID-19. These findings suggest that MDSCs play a role in the pathogenesis of COVID-19, while PMN-MDSCs, especially immature PMN-MDSCs, are associated with the severity of the disease.

Nasopharyngeal carcinoma in a child with Kartagener`s syndrome.

BACKGROUND: Kartagener`s syndrome, a subgroup of primary ciliary dyskinesia, is characterized by situs inversus totalis, chronic sinusitis and bronchiectasis. To date, the association of malignant diseases and Kartagener`s syndrome has been reported and all cases except angioimmunoblastic T cell lymphoma in a child have been seen in adulthood. CASE: A 10-year-old boy who was followed with the diagnosis of Katagener`s syndrome, presented with a progressive mass in the cervical region for 6 months. Physical examination revealed mental retardation, multiple lymphadenopathies, the largest in the left cervical region (4x4 cm), and pectus carinatum. Also, on cardiovascular examination, apex beat was felt on the right fifth intercostal space along midclavicular line. Magnetic resonance imaging of nasopharynx showed narrowing of the nasopharyngeal airway with an increase in wall thickness up to 2.5 cm on the posterior wall of the nasopharynx. Also, bilateral multiple cervical lymphadenopathies were noted. The pathological examination of the biopsy from cervical lymphadenopathy revealed a diagnosis of undifferentiated nasopharyngeal carcinoma. Chemotherapy was started for nasopharyngeal carcinoma chemotherapy regimen including cisplatin, docetaxel, and 5-fluorouracil. After four cycles of chemotherapy there was a significant regression in nasopharyngeal mass and lymphadenopathies. The patient underwent radiotherapy to the nasopharynx and bilaterally cervical regions. The patient has been in follow-up for 6 years well and tumor free. However, he is still under the supervision of the pediatric immunology and allergy departments due to recurrent respiratory infections and sinusitis. CONCLUSION: We present a case of nasopharyngeal carcinoma which developed in a child with Kartagener`s syndrome. To our knowledge, this is the first report of nasopharyngeal carcinoma in a child with Kartagener`s Syndrome.

Pseudoangiomatous Stromal Hyperplasia of Breast in an Adolescent: A Case Report and Review of the Literature.

BACKGROUND: Pseudoangiomatous stromal hyperplasia (PASH) is a rare benign breast lesion that is is extremely rare in children and adolescents. CASE: A 13-year-old girl was admitted to our clinic with a giant mass in the left breast for 3 months. Physical examination findings of the patient were normal except for the mass in the breast. The mass was removed totally, and its pathological diagnosis was pseudoangiomatous stromal hyperplasia. The patient has been followed up for 6 months without disease.

A Prolonged Activated Partial Thromboplastin Time in a Child With Hodgkin Lymphoma.

A 3-year-old girl presented with fever and left-sided neck mass that did not resolve despite antibiotic treatment. Physical examination was normal except lymphadenopathy in the left cervical region. Complete blood count was Hb: 8.01 g/dL, leukocyte count: 4034/mm3, platelet count: 286,000/mm3. On preoperative period, coagulation studies revealed a prolonged activated partial thromboplastin time (aPTT, 46.1 s [N: 21 to 36]), corrected mixing test for aPTT. The prothrombin time and international normalized ratio were in normal limits. Factor VIII levels, von Willebrand factor antigen, and ristocetin cofactor were normal. After the patient was given fresh frozen plasma, lymph node excision was performed. As a result of pathologic examination, Hodgkin lymphoma was diagnosed as classic type. The patient was instituted ABVD chemotherapy protocol for Hodgkin lymphoma. The aPTT at the sixth day of treatment was within normal limits. Coagulation test abnormalities are extremely rare in Hodgkin lymphoma. To our knowledge, this is the first report of prolonged aPTT in a child with Hodgkin lymphoma.

Delayed Radiation Myelopathy in a Child With Hodgkin Lymphoma and ARTEMIS Mutation.

The authors present a case of delayed radiation myelopathy in a 12-year-old girl with Hodgkin lymphoma and Artemis mutation. This is the first of such a case presented in the literature.

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in Children With Non-Hodgkin Lymphoma.

The aim of this study is to evaluate the clinical and laboratory findings of pediatric patients with non-Hodgkin lymphoma (NHL) who developed Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Between 2006 and 2018, the medical records of child patients with NHL who developed SJS and TEN were reviewed retrospectively. SJS/TEN developed in 7 of 70 patients with NHL (10%). The pathologic subgroups of the patients with SJS/TEN were ALK-negative anaplastic large cell lymphoma (n: 3), Burkitt lymphoma (n: 2), lymphoblastic lymphoma (n: 1), and primary mediastinal B-cell lymphoma (n: 1). Five patients had TEN, 1 patient had SJS/TEN, and 1 patient developed only SJS. In 5 patients, both steroids and intravenous immunoglobulin were administered for treatment, and clinical improvement was achieved in 3 of these patients. Only steroid treatment was used for 1 patient, whereas for the other patient, intravenous immunoglobin was preferred. In addition, N-acetylcysteine treatment was administered for these 2 patients. Four patients with acute renal failure died, and it was found that SJS/TEN is observed more frequently in patients with NHL in which intensive treatment protocols with high-dose methotrexate are used more than with other childhood malignant diseases. Early diagnosis and administration of appropriate and supportive treatment approaches may improve the prognosis.

Pediatric Lymphoma and Solid Tumors Associated With Cancer Susceptibility Syndromes.

The aim of this study was to determine cancer susceptibility syndromes (CSSs) in children with lymphoma and solid tumors and to evaluate their effects on overall survival rates. Between 2006 and 2019, the oncologic charts of 672 patients who were followed-up with a diagnosis of lymphoma and solid tumor in our clinic were retrospectively reviewed. CSSs were detected in 42 children (6.25%). The most common CSS was DNA damage repair defects/genetic instability (in 18 of 42 patients with CSSs, 42.8%). In the patients with CSSs, 48 different cancers developed. The most common types of cancer were lymphoma (n: 10, 21%) and high-grade glial tumor (n: 9, 19%). The lymphoma subgroups were very rare childhood lymphomas, such as gray zone lymphoma and marginal zone lymphoma. The overall survival rates for patients with DNA damage repair defects/genetic instability; with CSSs other than DNA damage repair defects/patients with genetic instability syndrome; and without any CSS, were 9.7%, 65.1%, and 68.7%, respectively. The overall survival rate for patients with DNA damage repair defects/patients with genetic instability syndrome was lower than both patients with CSSs other than DNA damage repair defects/genetic instability syndrome (P=0.002) and those without any CSS (P<0.0001). CSSs should be kept in mind in children with cafe au lait spots; syndromic features; a family history of cancer, especially in siblings; and rare childhood cancers.

Calcified fibrous pseudotumor with Castleman disease

Simultaneous calcified fibrous pseudotumor (CFT) and Castleman disease (CD) is an extremely rare association. CD is an uncommon lymphoproliferative disease that can arise in various sites of the body, while CFT is a rare type of benign fibrous lesion that frequently affects children and young adults, occurring as solitary or multiple lesions throughout the human body. Both entities are rare and exhibit typical and diverse histomorphological features. We report the case of a 15-year-old female patient, who, at the age of 13 had a biopsy performed at an external medical center; however, after 4 months the lesion had regrown. This lesion was removed with a surgical operation; however, it regrew 2 years later and was removed a third time. The results of the latter two biopsies were the same: CFT accompanying CD. The histologic examination of the excised lymph node and the surrounding tissue showed hyalinized fibrous tissue containing dystrophic and psammomatous calcification. In this case, the hyaline vascular type of CD was found to be intertwined with a CFT, which hampered the differentiation of whether both entities emerged within the lymph node or if the CFT developed from the soft tissue and then involved the lymph node. Future studies involving larger case series will provide a more precise insight, which should serve to resolve the current uncertainty.

Calcified fibrous pseudotumor with Castleman disease.

Simultaneous calcified fibrous pseudotumor (CFT) and Castleman disease (CD) is an extremely rare association. CD is an uncommon lymphoproliferative disease that can arise in various sites of the body, while CFT is a rare type of benign fibrous lesion that frequently affects children and young adults, occurring as solitary or multiple lesions throughout the human body. Both entities are rare and exhibit typical and diverse histomorphological features. We report the case of a 15-year-old female patient, who, at the age of 13 had a biopsy performed at an external medical center; however, after 4 months the lesion had regrown. This lesion was removed with a surgical operation; however, it regrew 2 years later and was removed a third time. The results of the latter two biopsies were the same: CFT accompanying CD. The histologic examination of the excised lymph node and the surrounding tissue showed hyalinized fibrous tissue containing dystrophic and psammomatous calcification. In this case, the hyaline vascular type of CD was found to be intertwined with a CFT, which hampered the differentiation of whether both entities emerged within the lymph node or if the CFT developed from the soft tissue and then involved the lymph node. Future studies involving larger case series will provide a more precise insight, which should serve to resolve the current uncertainty.

Comparison of the Radiologic and Clinical Findings of Adolescents With Breast Complaints.

INTRODUCTION: The American College of Radiology's Breast Imaging Reporting and Data System (BI-RADS) was developed to guide imaging-based surgical treatment in patients with breast cancer. Studies confirming the BI-RADS did not include adolescents. To evaluate the validity of this classification system in adolescents, we aim to investigate the relationship between the BI-RADS and pathology findings in adolescents. METHODS: The medical data of 67 female adolescent patients, aged 12 to 18, referred to our clinic for breast-related complaints between 2013 and 2016 were reviewed retrospectively for demographic data and the results of radiologic and surgical pathologies. RESULTS: The main underlying reasons for the visit at the clinic were fullness, tenderness, pain, and palpable masses. Of the 67 patients, 46 were enrolled in the study. After breast ultrasonography, patients whose complaints had diminished were subtracted from the follow-up. The mean age of the patients was 16 years (12 to 18 y). The mean mass diameter size was 3.69 cm (0.9 to 15 cm), and the mean clinical follow-up was 65.3 days (11 to 1095 d). All the patients who were surgically intervened had benign pathology. Of the 21 surgically intervened patients with BI-RADS levels of ≥3, most had fibroadenomas. CONCLUSIONS: The BI-RADS classification-based treatment algorithm may not be valid in adolescents. In the present study, all patients with lesions with BI-RADS ≥3 levels had revealed benign pathologies. The BI-RADS classification may show an increased risk. However, to determine the need for a biopsy in adolescents, there is a need for larger-scale pediatric and adolescent studies using the BI-RADS classification.

An aggressive parameningeal rhabdomyosarcoma with multiple spinal cord metastases: a case report and review of the literature.

PURPOSE: Spinal cord metastasis from rhabdomyosarcoma (RMS) is extremely rare, with three cases reported to date. Herein, we report an aggressive case of RMS of the infratemporal fossa who which developed spinal cord metastases during treatment. CASE PRESENTATION: A 6-year-old girl presented with an enlarging painless mass around her right ear for 3 months. An enhanced magnetic resonance imaging (MRI) revealed a 5 × x4 × x4.5 5 cm mass on her right infratemporal fossa. A tru-cut biopsy was performed, and histopathologic examination revealed the diagnosis of rhabdomyosarcoma. At the time of the diagnosis, cerebrospinal fluid cytology was negative for malignant cells. The patient underwent induction chemotherapy. There was minimal response to chemotherapy, and the patient underwent curative radiotherapy. However, by 12th fraction of RT, the patient developed a progressive weakness on her lower extremity. Spinal MRI revealed multiple gross masses in different parts of the spinal cord. The local radiotherapy was changed toas craniospinal radiotherapy. However, two 2 weeks after the completion of the RT, the patient developed sepsis and expired because of septic shock. CONCLUSION: Parameningeal RMS is a peculiar subgroup of RMS, which needs an aggressive approach. Despite aggressive approach, meningeal spread is the most important cause of the treatment failure. We should keept in mind that during the treatment, there can be meningeal spread towards to either the brain or spinal cord; therefore, we should follow -up the patients closely from this aspect.