OBJECTIVES: This study aimed to evaluate the diagnostic accuracy of Cyclin D1 as an adjunct immunomarker in CD99 positive small round cell neoplasms with primary consideration of PNET/EWS. MATERIALS AND METHODS: Tissue from 2017 to 2023 with a histopathologic diagnosis of CD99 positive small round blue cell tumors with primary consideration of Primitive Neuroectodermal Tumor (PNET)/Ewing Sarcoma were retrieved and Cyclin D1 immunohistochemical staining done. Diagnostic accuracy of Cyclin D1 immunostaining was determined by calculating the sensitivity, specificity, positive predictive value, and negative predictive value. RESULTS: Cyclin D1 immunohistochemical staining was performed in 19 specimens available, of which 13 yielded a positive result. Of these, 8 had a final histopathologic diagnosis of CD99 positive small round blue cell tumor with primary consideration of PNET/Ewing Sarcoma, resulting in sensitivity of 61.54%, specificity of 100%, positive predictive value of 100% and negative predictive value of 50.0%. The overall accuracy is 72.2%. CONCLUSION Cyclin D1 can be used as an adjunct immunomarker to aid in the diagnosis of CD99 positive round cell tumor with primary consideration of PNET/Ewing Sarcoma specifically in resource limited settings where molecular testing is not readily available. Given the high specificity of Cyclin D1 in such cases, it can be used to rule out other small round blue cell tumors that can also stain positive for CD99 such as Rhabdomyosarcoma. However, interpretation must be done in conjunction with the results of other immunohistochemical stains in order to increase its diagnostic accuracy.
Human ; Male,Female ; Cells ; Sarcoma, Ewing ; Sarcoma ; Neuroectodermal tumors, Primitive ; Cyclin D1

Ewing's sarcoma in the cervix is characterized by extremely rare occurrence,high degree of malignancy,and rapid progression.The diagnosis of this disease is based on pathology and immunohistochemistry. The main image of the case reported in this paper showed the cervical cyst with solid mass,large volume,and uneven density and signal,and the solid part can be strengthened in enhanced scanning.Because of the rapid growth,the lesion is prone to liquefaction necrosis and bleeding.Since the metastasis occurs early,timely diagnosis is essential.
Cervix Uteri/pathology* ; Female ; Humans ; Immunohistochemistry ; Neuroectodermal Tumors, Primitive, Peripheral/pathology* ; Sarcoma, Ewing/pathology* ; Uterine Cervical Neoplasms

Child ; Family ; Humans ; Neuroectodermal Tumors, Primitive/pathology* ; Neuroectodermal Tumors, Primitive, Peripheral/pathology* ; Oropharynx

Primitive neuroectodermal tumors (PNETs) are a heterogeneous group of malignant neoplasms found primarily in childhood and early adulthood. In this paper, we described the case of a 64-year-old male with primary spinal PNET, successfully treated with surgery, craniospinal radiotherapy, and concurrent chemotherapy. This is the case of a 64-year-old male who presented with a 2-month history of bilateral lower extremity weakness and numbness associated with urinary and bowel incontinence. Work-up was done, and the spine's plain magnetic resonance imaging (MRI) revealed a heterogeneously enhancing intradural lesion with an extradural component at the right T9/T10 level, causing mild to moderate cord compression. The patient underwent laminectomy and gross total resection of the said tumor. Histology and immunohistochemistry were consistent with a primitive neuroectodermal tumor of the spine. The tumor recurred three months after the surgery, and the patient was then referred for radiation therapy with concurrent chemotherapy. Repeat spinal MRIs with three- to six-month intervals after treatment showed no tumor recurrence as of August 2021. Primary spinal PNETs are rarely found in adults, especially in the elderly. These tumors currently have no recommendations or guidelines regarding their management. Thus, most cases are presently being managed based on studies on children and central nervous system (CNS) PNETs. This paper presented a case of a successfully treated primary spinal PNET in the elderly. The management was primarily based on studies done on that of the pediatric population and CNS PNETs.
Neuroectodermal Tumors, Primitive ; Neuroectodermal Tumors, Primitive, Peripheral

Pulmonary neuroendocrine tumors (PNETs) are a kind of epithelial tumors originating from pulmonary neuroendocrine cells, accounting for about 20% of primary lung tumors, including typical carcinoid, atypical carcinoid, small cell carcinoma, and large cell neuroendocrine carcinoma. The morphologic and clinical characteristics of these four types of PNETs are relatively highly heterogeneous. Immune checkpoint inhibitors (ICIs) have been shown robust antitumor activity in a variety of solid tumors. Treatment regimens of advanced PNETs have developed greatly in the past decade, but ICIs are still in their infancy in the field of PNETs. This review focuses on the landscape of current clinical trials and research as well as the situation of ICIs-related biomarkers in PNETs.
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Carcinoid Tumor ; Carcinoma, Neuroendocrine ; Humans ; Immune Checkpoint Inhibitors ; Lung Neoplasms/drug therapy* ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors/drug therapy*

OBJCTIVE: To study the clinical effect of surgery combined with chemotherapy and radiotherapy in children with central primitive neuroectodermal tumor (cPNET), as well as the risks factors for poor prognosis. METHODS: A retrospective analysis was performed for the clinical data of 42 children who were diagnosed with cPNET from June 2012 to September 2018. RESULTS: The 42 children had a median overall survival (OS) time of 2.0 years and a median event-free survival (EFS) time of 1.3 years; the 1-, 3-, and 5-year OS rates were 76.2%±6.6%, 41.4%±8.7%, 37.3%±8.8% respectively, and the 1-, 3-, and 5-year EFS rates were 64.3%±7.4%, 32.7%±8.0%, 28.0%±8.1% respectively. The univariate analysis showed that there were significant differences in the OS and EFS rates among the children with different patterns of surgical resection, chemotherapy cycles, and risk grades (P<0.05), and there was also a significant difference in the OS rate between the children receiving radiotherapy and those not receiving radiotherapy (P<0.05). The multivariate Cox regression analysis showed that chemotherapy cycles and risk grade were independent influencing factors for EFS and OS rates (P<0.05). The EFS and OS rates increased with the increase in chemotherapy cycles and the reduction in risk grade. CONCLUSIONS Multimodality therapy with surgery, chemotherapy, and radiotherapy is an effective method for the treatment of cPNET in children. Early diagnosis and treatment and adherence to chemotherapy for as long as possible may improve EFS and OS rates.
Antineoplastic Combined Chemotherapy Protocols ; Child ; Combined Modality Therapy ; Disease-Free Survival ; Humans ; Neuroectodermal Tumors, Primitive ; Prognosis ; Retrospective Studies

Multiple primary malignancies are defined as two or three malignant neoplasms arising in different organ systems. Several cases of multiple primary malignancies are emerging in recent years due to the advancement in medical therapy and diagnostics. Multiple primary malignancies are not uncommon occurring at 0.7-16% of cancer patients, however, reported cases of multiple primary sarcomas are sparse. Presented in this report is a pediatric patient diagnosed with primary metachronous cerebral rhabdomyosarcoma after being treated for primitive neuroectodermal tumor/Ewing’s sarcoma of the oral cavity. Despite limited cases addressing multiple primary sarcomas, this entity must not be overlooked as it is associated with a meager outcome compared to an index case of sarcoma alone.
Sarcoma, Ewing ; Neuroectodermal Tumors, Primitive ; Rhabdomyosarcoma ; Immunohistochemistry

Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms arising from the pancreatic islet of Langerhans and can be functioning or non-functioning based on the clinical symptoms caused by hormonal secretions. PNETs are the second most common tumor of the pancreas and represent 1–2% of all pancreatic neoplasms. The incidence of pNETs appears to be rising and the prognosis seems to be improving, likely due to the improved treatment options. Recent updates of the World Health Organization classification and grading separate pNETs into 2 broad categories according to the histopathologic criteria, including the Ki-67 proliferative index and mitotic counts: well-differentiated NET and poorly-differentiated neuroendocrine carcinoma (NEC). The classification also incorporates a new subcategory of well-differentiated high-grade NEC (grade 3) to the well-differentiated NET category. This new classification algorithm aims to improve the prediction of the clinical outcomes and survival and help clinicians select better therapeutic strategies for patient care and management. The treatment of advanced or metastatic pNETs may include surgical resection, liver-directed therapies, and/or systemic treatments. In unresectable patients, the goals of these therapies are to palliate the tumor-related symptoms and prolong the lifespan. Systemic therapy consists of the following broad modalities: somatostatin analogues, molecular targeted therapy, systemic chemotherapy, and peptide receptor radionuclide therapy. In conclusion, pNETs are diagnosed increasingly throughout the world, usually with metastatic disease and requiring systemic therapy. Each patient should be evaluated thoroughly and discussed individually by a multidisciplinary and dedicated NET-expert team, which might consider all treatment options, including ongoing clinical trials before selecting the appropriate treatment sequence.
Carcinoma, Neuroendocrine ; Classification ; Drug Therapy ; Humans ; Incidence ; Islets of Langerhans ; Molecular Targeted Therapy ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors ; Pancreas ; Pancreatic Neoplasms ; Patient Care ; Prognosis ; Receptors, Peptide ; Somatostatin ; World Health Organization

PURPOSE: The 8th edition of the American Joint Committee on Cancer (AJCC) staging system for pancreatic neuroendocrine tumor (PNET) included several significant changes. We aim to evaluate this staging system compared to the 7th edition AJCC staging system and European Neuroendocrine Tumors Society (ENETS) system. MATERIALS AND METHODS: We used Korean nationwide surgery database (2000-2014). Of 972 patients who had undergone surgery for PNET, excluding patients diagnosed with ENETS/World Health Organization 2010 grade 3 (G3), only 472 patients with accurate stage were included. RESULTS: Poor discrimination in overall survival rate (OSR) was noted between AJCC 8th stage III and IV (p=0.180). The disease-free survival (DFS) curves of 8th AJCC classification were well separated between all stages. Compared with stage I, the hazard ratio of II, III, and IV was 3.808, 13.928, and 30.618, respectively (p=0.007, p < 0.001, and p < 0.001). The curves of OSR and DFS of certain prognostic group in AJCC 7th and ENETS overlapped. In ENETS staging system, no significant difference in DFS between stage IIB versus IIIA (p=0.909) and IIIA versus IIIB (p=0.291). In multivariable analysis, lymphovascular invasion (p=0.002), perineural invasion (p=0.003), and grade (p < 0.001) were identified as independent prognostic factors for DFS. CONCLUSION: This is the first large-scale validation of the AJCC 8th edition staging system for PNET. The revised 8th system provides better discrimination compared to that of the 7th edition and ENETS TNM system. This supports the clinical use of the system.
Classification ; Discrimination (Psychology) ; Disease-Free Survival ; Humans ; Joints ; Neoplasm Staging ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors ; Pancreas ; Survival Rate

Ewing sarcoma is the second most frequently occurring malignant tumor of the bone and soft tissue in adolescents and young adults. Genetically, Ewing sarcoma is characterized by balanced chromosomal translocation in which a member of FET gene family is fused with an ETS transcription factor, with the most common fusion being EWSR1-FLI1 (85% of cases). Treatment of Ewing sarcoma is based on multidisciplinary approach (local surgery, radiotherapy and multiagent chemotherapy), which are associated with chronic late effects that may compromise quality of life of survivors. First line treatment includes combination of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin. The beneficial role of high dose chemotherapy has been suggested in high-risk localized Ewing sarcoma patients, and the studies are being performed to investigate the role in metastatic disease. The 5-year overall survival for localized Ewing sarcoma has improved to reach 65% to 75%. But patients with metastatic disease have a 5-year survival rate of <30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrent tumor have a dismal prognosis. Novel therapeutic strategies based on understanding of molecular mechanisms are needed to improve the outcome of Ewing sarcoma and to lessen the treatment-related late effects.
Adolescent ; Cyclophosphamide ; Dactinomycin ; Doxorubicin ; Drug Therapy ; Etoposide ; Humans ; Ifosfamide ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive, Peripheral ; Prognosis ; Quality of Life ; Radiotherapy ; Sarcoma, Ewing ; Survival Rate ; Survivors ; Transcription Factors ; Translocation, Genetic ; Vincristine ; Young Adult