Sarcoma Primitive Neuroectodermal Tumor Research Articles

Conservative Treatment of Ewing's Sarcoma of the Uterus in Young Women.

Ewing sarcoma-primitive neuroectodermal tumors (ES/PNETs) constitute a family of neoplasms characterized by a continuum of neuroectodermal differentiations. ES/PNET of the uterus is rare. There are 48 cases of ES/PNET of the uterus published in the literature as far as we know. We describe a case of Ewing sarcoma of the uterus occurring in a 17-year-old woman presenting with a two-month history of pelvic pain. After surgical excision and microscopic, immunohistochemical, and electron microscopy examination, the diagnosis of Ewing sarcoma of the uterus was suggested. This report will discuss the diagnosis and surgical and clinical management of Ewing uterine sarcoma in young women, according to the available literature. In spite of the rarity of ES/PNETs, they should be taken into account in the differential diagnosis of uterine neoplasms in young women.

Macrophage Infiltration Predicts a Poor Prognosis for Human Ewing Sarcoma

Ewing sarcoma-primitive neuroectodermal tumor (EWS) is associated with the most unfavorable prognosis of all primary musculoskeletal tumors. The objective of the present study was to investigate whether tumor-associated macrophages (TAMs) affect the development of EWS. TAMs were isolated from mouse xenografts using CD11b magnetic beads and examined for their cytokine expression and osteoclastic differentiation. To evaluate the role of TAMs in xenograft formation, liposome-encapsulated clodronate was used to deplete TAMs in mice. Macrophage infiltration and tumor microvascular density were histologically evaluated in 41 patients with EWS, and association with prognosis was examined using Kaplan-Meier survival analysis. In mouse EWS xenografts, TAMs expressed higher concentrations of cytokines including interleukin-6, keratinocyte-derived chemokine, and monocyte chemotactic protein-1. TAMs were more capable than normal monocytes of differentiating into tartrate-resistant acid phosphatase-positive giant cells. Depleting macrophages using liposome-encapsulated clodronate significantly inhibited development of EWS xenografts. In human EWS samples, higher levels of CD68-positive macrophages were associated with poorer overall survival. In addition, enhanced vascularity, increase in the amount of C-reactive protein, and higher white blood cell counts were also associated with poor prognosis and macrophage infiltration. TAMs seem to enhance the progression of EWS by stimulating both angiogenesis and osteoclastogenesis. Further investigation of the behavior of TAMs may lead to development of biologically targeted therapies for EWS.

Ewing sarcoma-primitive neuroectodermal tumor of the uterus: a clinicopathologic, immunohistochemical and ultrastructural study of one case

Ewing sarcoma-primitive neuroectodermal tumors (ES/PNET) constitute a family of neoplasms characterized by a continuum of neuroectodermal differentiation. ES/PNET of the uterus is rare. There are 43 cases published in the English literature as far as we know. We describe an additional case. A 56-year-old woman presented with a 2-month history of irregular menopausal vaginal bleeding. After surgical excision, microscopic, immunohistochemical and electron microscopic examination suggested the diagnosis of ES/PNET. The patient underwent combined chemotherapy consisting of ifosfamide, etoposide, and cisplatin. She was alive with no evidence of recurrence or metastasis after 41 months of the initial operation. In spite of the rarity of ES/PNET, we should consider it in the differential diagnosis of small cell neoplasms of the uterus.

Calcifying Nested Stromal-epithelial Tumors of the Liver

There is a rare primary liver tumor that has been reported as "ossifying stromal-epithelial tumor" (3 cases), "desmoplastic nested spindle cell tumor" (4 cases), and "nested stromal-epithelial tumor" (6 cases). Herein we report 9 cases of this tumor, including 3 previously reported, from the files of the Armed Forces Institute of Pathology. All tumors were discovered incidentally in patients between 2 and 33 years of age. Four had a history of calcified hepatic nodules since childhood (ages 4 to 10 y). One had Cushing syndrome that abated after excision. Eight patients had a partial hepatectomy and 1 underwent liver transplantation. The tumors ranged from 5.5 to 20 cm and had a characteristic histologic appearance with irregular, sharply circumscribed nests and islands of bland-appearing spindled to focally epithelioid cells, surrounded by a cellular desmoplastic stroma. The tumor nests had focal psammoma-like calcifications with or without ossification. Immunohistochemistry demonstrated at least focal positivity for keratin cocktail AE1/AE3/LP34 in all 9 cases, and Wilms tumor suppressor gene (7/7) with variable staining for other epithelial (except keratins 7 and 20), neural, and mesenchymal markers. None of the tumors was positive for Ewing sarcoma-primitive neuroectodermal tumors, desmoplastic small round cell tumor, and SYT-SSX fusion transcript. Follow-up revealed that 1 patient had 2 local recurrences successfully treated by radiofrequency ablation. The patient who underwent liver transplantation died of postoperative complications. Six patients were alive and well up to 22 years after surgery. We propose the name "calcifying nested stromal and epithelial tumor" for this rare but distinctive clinicopathologic entity of uncertain histogenesis. On the basis of currently available information, this tumor is best considered a low-grade malignancy.

Carboplatin and paclitaxel adjuvant chemotherapy in primitive neuroectodermal tumor of the uterine corpus

Primitive neuroectodermal tumor of the uterine corpus (PNET) is rare and appears to have an aggressive clinical course. We report on a postmenopausal woman with optimal surgically cytoreduced advanced-stage PNET in which adjuvant combination chemotherapy with platinum and taxane agents was unsuccessful in extending her disease-free survival.

Primary Ewing Sarcoma of Lumbar Spine With Massive Intraspinal Extension

Primary vertebral Ewing sarcoma-primitive neuroectodermal tumor is uncommon. Although epidural extension has been seen in such tumors, cases with massive intraspinal involvement are decidedly rare. Here we present the case of a 4-year-old girl with back pain and difficulty walking. Magnetic resonance imaging showed a mass filling the spinal canal from T(11) to the L(3)/L(4) levels. Vertebral involvement with extension into the paraspinal soft tissue through neural foramina was seen. Histologically, a small-blue-cell tumor with strong membranous CD99 reactivity was noted. Molecular analysis revealed translocation t(11;22)(q24;q12), thus confirming the diagnosis of Ewing sarcoma-primitive neuroectodermal tumor. Our case emphasizes that vertebral Ewing sarcoma-primitive neuroectodermal tumor may present with massive intraspinal extension and should be included in the differential diagnosis of intraspinal lesions.

Primary Ewing's sarcoma-primitive neuroectodermal tumor of the uterus: A case report and literature review

Background Primary Ewing's sarcoma-primitive neuroectodermal tumor (ES-PNET) of the uterus is an extremely rare malignancy. Case A 30-year-old Korean woman presented with abnormal uterine bleeding with uterine enlargement. A computed tomography (CT) scan and magnetic resonance imaging (MRI) of the abdomen and pelvis showed a huge uterine mass measuring 18 × 20 × 21 cm, metastasis to both pelvic and para-aortic lymph nodes, and omental infiltration. The pathology report of the uterine mass described a uniformly hypercellular tumor, which was arranged in diffuse solid sheets of uniform, small, rounded, and sometimes spindle-shaped cells, with scanty cytoplasm. Immunohistochemically, the mass tested positive for vimentin, CD99, and chromogranin. The patient received several courses of combination chemotherapy and radiotherapy but died from tumor progression 16 months after the initial diagnosis. Conclusion(s) This is a rare case of primary uterine ES-PNET in a woman of reproductive age. A review of the literature indicates that primary uterine ES-PNET requires early diagnosis and multimodality treatment including surgery, chemotherapy, and radiotherapy. The behavior of this tumor is potentially aggressive.

Primary Ewing's sarcoma–primitive neuroectodermal tumor of the vagina

Background. Only three cases of Ewing's sarcoma–primitive neuroectodermal tumor (ES–PNET) occurring in the vagina have been previously reported. Case. A 30-year-old Chinese woman presented with a vaginal mass measuring 5 cm in greatest dimension. The tumor was composed of solid sheets of undifferentiated small round cells with numerous Homer–Wright rosettes. Immunohistochemically, it was positive for CD99, FlI-1 protein, synaptophysin, neuron-specific enolase, vimentin, and S-100 protein. The patient underwent wide local excision of the tumor and subsequent total abdominal hysterectomy with postoperative chemotherapy and radiotherapy. She is disease-free 36 months after the initial surgery. Conclusion. This is a rare case of ES–PNET occurring in the vagina and the first showing many distinctive Homer–Wright rosettes. A review of literature indicates that vaginal or vulvar ES–PNET affects younger women, and it may have a relatively favorable outcome.

Visceral primitive peripheral neuroectodermal tumors: A clinicopathologic and molecular study

Ewing sarcoma–primitive neuroectodermal tumor (EWS/PNET) belongs to the group of pediatric small round blue cell tumors; although EWS/PNET is classically a tumor of the soft tissue or bone in children and young adults, individual cases have been described in patients of all ages. A group of chromosomal translocations involving the EWS gene and a member of the Ets transcription factor family of genes has been detected in EWS/PNET, and heterogeneity in the precise breakpoint of the translocation has been shown to generate a group of related fusion transcripts that may have prognostic significance. Within the last decade, the clinicopathologic spectrum of EWS/PNET has been markedly expanded by recognition that the tumor may also have a visceral origin. To determine whether visceral EWS/PNET has the same pattern of genetic alterations and range of fusion transcripts as EWS/PNET of bone and soft tissue, we performed reverse-transcription polymerase chain reaction–based testing of formalin-fixed, paraffin-embedded tissue from a series of visceral tumors for which the diagnosis of EWS/PNET was well established. Together with additional cases compiled from the literature, EWS-Fli1 (or a related fusion transcript) was present in 18 of 19 visceral EWS/PNET, with a distribution of transcript types not statistically different from EWS/PNET of soft tissue and bone (P >.05, X2 test). These results firmly establish the genetic relationship between EWS/PNET of visceral sites, soft tissue, and bone. HUM PATHOL 32:1109-1115. Copyright © 2001 by W.B. Saunders Company