Remission of a Primary, Recurrent Thoracic Ewing Sarcoma in a 74-year-old woman.

Ewing sarcoma is a primitive neuroectodermal tumor which seldom presents with primary disease in people over age 40 and outside of the appendicular or axial skeleton. We examine a case of primary thoracic Ewing Sarcoma diagnosed initially by CT-guided biopsy in a woman at the age of 74 years. The disease progressed after initial combined modality therapy consisting of neoadjuvant chemotherapy, surgical resection, and adjuvant radiation therapy and two additional courses of multiagent chemotherapy. After relapse of her disease, subsequent second- and third-line systemic agents which included chemotherapy and targeted agents were given with disease stabilization achieved now over 30 months from initial diagnosis. To our knowledge, this is the first report of a primary pulmonary Ewing sarcoma diagnosed in a patient greater than 70 years of age in whom multiple remissions have been achieved with tolerable toxicities.

Ewing's Sarcoma of the Breast in a Young Woman: A Case Report and Review of the Literature.

Ewing's Sarcoma Family Tumors (ESFT) include classic Ewing's sarcoma of bone, extra-skeletal Ewing's sarcoma (EES), malignant small cell tumor of the chest wall (Askin tumor), and soft tissue-based Peripheral Primitive Neuroectodermal tumors (pPNET). The t(11;22)(q24;q12) translocation is associated with 85% of tumors and leads to EWS-FLI-1 (Ewing's Sarcoma-Friend Leukemia Integration-1) formation. This is a potent transforming gene that encodes a chimeric protein that plays a role in the genesis of Ewing's Sarcoma and Primitive Neuroectodermal Tumors. The breast location of ESFT remains exceptional. The prognosis is among the poorest of all subtypes of breast cancer and even poorer than other extraosseous Ewing's sarcomas. We describe the case report of a 23-year-old patient with a growing breast lump, who required an accurate and challenging diagnostic estimation and who ultimately resulted in a peripheral primary neuroectodermal tumor (pPNET). Through this case description and a brief narrative review of the literature, we aim to highlight the rarity of ESFT located in the breast. Histopathological confirmation is mandatory for all growing masses of the breast to reach a conclusive diagnosis and plan the correct treatment. Patients with rare diagnoses should always be centralized in breast units, conducting multidisciplinary meetings and, when necessary, the diagnosis should be shared through wider national or international registries.

Primary intradural/extradural Ewing's sarcoma of the sacral spine: A case report and literature review.

BACKGROUND: Ewing's sarcoma (ES) is a malignant mesenchymal tumor, most often found in the long bones, and usually affecting children and adolescents in the second decade of life. ES of the spine is a clinical rarity. CASE DESCRIPTION: A 45-year-old male presented with a 3-month history of lower back pain which acutely worsened in conjunction with urinary retention. The magnetic resonance imaging revealed a mass extending from L5 to S2 with additional extension through the left S2-3 neural foramen. The metastatic workup was negative. At surgery, the lesion was both intradural and extradural. Following complete surgical resection, the patient was later treated with radiation and chemotherapy. CONCLUSION: Here, we report an adult male who acutely presented with low back pain attributable to primary intradural/extradural sacral ES.

Ovarian primary primitive neuroectodermal tumor: a review of cases at PUMCH and in the published literature.

BACKGROUND: The pathological characteristics, treatment strategies and prognosis of ovarian primary primitive neuroectodermal tumor (PNET) were unclear due to the rarity of PNET. All cases treated at Peking Union Medical College Hospital (PUMCH) between 1975 and 2016 and published in the English literature between 1980 to 2017 were reviewed. RESULTS: Finally four cases from PUMCH and 15 cases in the literature were included. The median age was 25 years (range 13-79), and the median diameter of the tumors was 13.4 cm (range 5.0-30.0). The most common initial symptoms were abdominal pain, bloating and a pelvic mass. Diagnosis primarily depended on immunohistochemical and fluorescence in situ hybridization data. Treatment consisted of surgery, various chemotherapy regimens and/or radiotherapy. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 15 and 52%, respectively. For patients with OS and PFS > 12 months, the median ages were 21 years (range 13-35) and 17 years (range 13-35), respectively, while for patients with OS < 12 months and PFS < 12 months, the median ages were 48 years (range 14-79) and 25 years (range 18-79), respectively. CONCLUSIONS: No standard therapy for ovarian primary PNET exists, and an individualized strategy is recommended. Young patients seem to have better prognoses.

[Six-year disease-free survival after chemotherapy treatment for metastatic renal peripheral neuroectodermal tumor]. / Six ans de survie sans progression après chimiothérapie pour une tumeur neuro-ectodermique périphérique rénale métastatique.

Peripheral neuroectodermal tumors (PNET) of the kidney are extremely rare. They are often diagnosed at a late stage due to their nonspecific clinical presentation. Treatment of patients with metastases is based on palliative chemotherapy. We here report a case of PNET of the kidney with sudden onset of metastases to the lymph nodes and to the skin. The patient showed good clinical and radiological response and experienced progression-free survival at 6 years after polychemotherapy with vincristine, doxorubicin and cyclophosphamide.

Tumor neuroectodérmico recidivante. A propósito de un caso y revisión bibliográfica / Recurrent neuroectodermal tumor. A case report and literatura review

Resumen ANTECEDENTES: La relación entre cáncer y embarazo supone 0.07% de las complicaciones gestacionales. Cuando estas situaciones coinciden el tratamiento del tumor se dificulta. El tumor neuroectodérmico primitivo es una neoplasia relacionada con el sarcoma de Ewing y su incidencia es excepcional durante el embarazo. CASO CLÍNICO: Paciente de 34 años, con 36.3 semanas de embarazo, que ingresó a la unidad hospitalaria por dolor abdominal irradiado al miembro inferior derecho. A la exploración física se palpó una tumoración de gran dimensión en la fosa iliaca derecha. La ecografía abdominal objetivó una imagen compatible con un mioma. La resonancia magnética reportó una masa de 16 x 13 x 17 cm, retroperitoneal, paravertebral, coincidente con tumor neuroectodérmico, sarcoma y tumor neurogénico. La paciente tuvo parto eutócico, sin administración de analgesia epidural, del que nació una niña de 2950 g, con Apgar 8-9. Se efectuó una biopsia por aspiración con aguja gruesa, que reportó un tumor neuroectodérmico primitivo. El tratamiento consistió en quimioterapia con protocolo VAC (vincristina, dactinomicina y ciclofosfamida [14 ciclos]) y adriamicina (6 a 8 ciclos de inducción). Actualmente padece dolor neuropático en la pierna derecha y permanece en rehabilitación, con tratamiento médico. CONCLUSIONES: Los tumores neuroectodérmicos primitivos son neoplasias excepcionales durante el embarazo. Se requieren estudios complementarios para conocer la relación exacta entre este tipo de tumores y el embarazo, y de esta forma establecer el protocolo de tratamiento adecuado.

Abstract BACKGROUND: The relationship between cancer and pregnancy accounts for 0.07% of gestational complications. This aspect makes treatment difficult and has a negative impact on pregnant patients. The primitive neuroectodermal tumor is a neoplasm related to Ewing's sarcoma and its incidence is exceptional during pregnancy. CLINICAL CASE: A 34-year-old patient, 36.3 weeks pregnant, who was admitted to the hospital unit due to abdominal pain radiating to the right lower limb. Physical examination revealed a large tumor in the right iliac fossa. The abdominal ultrasound showed an image compatible with a myoma. Magnetic resonance imaging revealed a mass of 16 x 13 x 17 cm, retroperitoneal, paravertebral, coinciding with neuroectodermal tumor, sarcoma and neurogenic tumor. The patient had eutocic delivery, without administration of epidural analgesia, from which a girl of 2950 g was born, and Apgar 8/9. An aspiration biopsy was performed with a thick needle, which reported a primitive neuroectodermal tumor. The treatment consisted of chemotherapy with VAC protocol (vincristine, dactinomycin and cyclophosphamide [14 cycles]) and adriamycin (6 to 8 induction cycles). He currently suffers from neuropathic pain in the right leg and remains in rehabilitation, with medical treatment. CONCLUSIONS: Primitive neuroectodermal tumors are exceptional neoplasms during pregnancy. Complementary studies are required to know the exact relationship between this type of tumors and pregnancy, and in this way establish the appropriate treatment strategy.

A very rare adult case of cervical neuroblastoma.

Neuroblastoma is the most common malignancy in children, but it rarely occurs in adults, especially in the female genital system. Here, we report a case of a 28-year-old woman who was diagnosed with uterine cervical neuroblastoma. A microscopic examination revealed that tumor cells in the solid component were interspersed within an abundant fibrillary background and separated by thin fibrous septa that were composed of mats of neuropil. Immunohistochemistry showed that the cells were positive for vimentin and neuroendocrine markers, such as CD56, NSE, and synaptophysin. Clinical management of neuroblastoma must be tailored to the pathological diagnosis. Cases of neuroblastoma that originate in the cervix of an adult patient are rare, and few treatment strategies and chemotherapeutic protocols are therefore available. The case reported here provides information regarding our experience with a pathological diagnosis of such a cancer and the treatment protocol used to manage it.

Early dural metastasis from a case of glioblastoma with primitive neuroectodermal differentiation: A case report and literature review.

Glioblastoma with a primitive neuroectodermal (PNET) variant is a rare primary parenchymal tumor. Only a few cases of extraparenchymal metastasis are reported in world literature. Although the overall survival duration of glioblastoma multiforme (GBM) with primitive neuroectodermal tumor (PNET) variety may be prolonged in comparison to classical glioblastoma, the metastatic trend is completely different, and the prognosis is worse. We report an early dural metastasis of pure PNET component appearing in a case of primary glioblastoma with PNET variant. The lesson learned from this case is to look for early craniospinal metastasis in GBM patient with PNET component, even after completion of adjuvant radiochemotherapy.

Cranial and spinal leptomeningeal dissemination in esthesioneuroblastoma: Two reports of distant central nervous system metastasis and rationale for treatment.

BACKGROUND: Esthesioneuroblastoma is a locally aggressive cancer of the nasal cavity. While systemic metastasis can occur in 10-30% of patients, there are only six reported cases of distal metastasis from leptomeningeal dissemination. CASE DESCRIPTION: The authors report two cases of esthesioneuroblastoma treated previously with multimodal therapy in which distal metastatic recurrence was found and describe their treatment protocol, which has resulted in long-term success. CONCLUSION: Understanding the drivers of leptomeningeal dissemination in more prevalent primary neuroectodermal tumors may hold the key to developing successful treatment algorithms for this disease.

Pathology of primary neuroectodermal tumor and its prognostic factors:A report of 35 cases / 第三军医大学学报

Objective To study the pathology of primary neuroectodermal tumor (PNET),and its diagnostic standards and prognostic factors.Methods Expression of CD99,FLI-1,Syn,NSE,S-100,NF,and Vim was detected in PNET tissues stained with HE and immmunohistochemistry (En Vision method).Survival rate of 33 PNET patients with complete clinical information was analyzed with COX regression analysis method.Results The positive expression rate of CD99,FLI-1,Vim,Syn,NSE and s-100 was 88.57%,51.43%,91.42%,48.57%,45.71%,and 22.86%,respectively.The sensitivity of combined CD99 and FLI-1 was 100%.However,NF was not expressed in all PNET tissues.When other factors were unchanged,no difference was found in the effect of age on the survival rate of patients.However,a significant difference was observed in the effect of PNET site and its treatment modalities on the survival rate of patients (P