Review of Experience with Primitive Neuroectodermal Tumors of Childhood

Abstract Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.

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PATH-08. THE IMPORTANCE OF RE-DIAGNOSIS OF TUMORS PREVIOUSLY CLASSIFIED AS CENTRAL NERVOUS SYSTEM PRIMITIVE NEUROECTODERMAL TUMORS

Neuro-Oncology ◽

10.1093/neuonc/noaa222.644 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii426-iii426

Author(s):

Naohide Fujita ◽

Osamu Akiyama ◽

Akihide Kondo

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Brain Tumors ◽

Easy Access ◽

Molecular Analyses ◽

Primitive Neuroectodermal Tumors ◽

Neuroectodermal Tumors ◽

Sanger Sequence ◽

Embryonal Brain ◽

Polymerase Chain

Abstract BACKGROUND The recent molecular analyses have revealed that central nervous system primitive neuroectodermal tumors (CNS PNETs) those having clusters of small round tumor cells are genetically different tumors. However, the concepts of CNS PNET are complicated, and it is difficult to diagnose them appropriately in clinical field. To overcome this difficulty, we reviewed previous studies associated with CNS PNETs, and carried out several approaches, those are relatively easy access to use in clinics, for our 8 samples of embryonal brain tumors diagnosed CNS PNETs in our institution, initially. METHODS We used in combination with immunohistochemistry (IHC), Sanger sequence, Pyrosequence, polymerase chain reaction (PCR), real time PCR and copy number analysis referring recent reports. RESULTS In terms of the diagnosis three out of 8 cases were changed based on the results in this study from previous diagnoses. CONCLUSION In this review, it seemed that either the histopathological evaluation or molecular analyses would be not enough to make accurate diagnosis of CNS embryonal brain tumors, and it is essential to combine both of them including recent comprehensive analysis methods.

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Cisplatin in the treatment of recurrent childhood primary brain tumors.

Journal of Clinical Oncology ◽

10.1200/jco.1988.6.1.62 ◽

1988 ◽

Vol 6 (1) ◽

pp. 62-66 ◽

Cited By ~ 89

Author(s):

R W Walker ◽

J C Allen

Keyword(s):

Brain Tumors ◽

Germ Cell ◽

Germ Cell Tumor ◽

Overall Response Rate ◽

Response Rate ◽

Cell Tumor ◽

Primitive Neuroectodermal Tumors ◽

Primary Brain Tumors ◽

Overall Response ◽

Neuroectodermal Tumors

Thirty-three patients were treated with intravenous (IV) cisplatin (CPDD) of whom 32 were considered evaluable. There were 14 medulloblastomas, five primitive neuroectodermal tumors (PNET), nine gliomas, three ependymomas, and one germ cell tumor. The overall response rate was 13 of 32 (41%). Eleven responses (five complete [CR], five partial [PR], one mixed [MR]) were noted in the patients with medulloblastoma. The response rate within this group was 79%. Toxicity was tolerable, although it precluded further therapy in five patients.

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Primitive neuroectodermal tumor of the esophagus with metastasis in the pineal gland

Endoscopy International Open ◽

10.1055/a-0977-2614 ◽

2019 ◽

Vol 07 (09) ◽

pp. E1163-E1165

Author(s):

Leonardo Blas Jhon ◽

Paloma Sánchez-Fayos ◽

Maria Jesus Martín Relloso ◽

Daniel Calero Barón ◽

Juan Carlos Porres Cubero

Keyword(s):

Case Report ◽

Pineal Gland ◽

Disease Progression ◽

Rare Case ◽

Primitive Neuroectodermal Tumor ◽

Malignant Neoplasms ◽

Primitive Neuroectodermal Tumors ◽

First Case ◽

Neuroectodermal Tumors ◽

Chemotherapeutic Treatment

AbstractPrimitive neuroectodermal tumors (PNET) are very rare tumors that belong to a family of malignant neoplasms of tiny round cells which are derived from the neural crest. This report discusses a rare case of an adult woman with esophageal PNET, confirmed by immunohistochemistry, that presented with metastasis to the pineal gland. To our knowledge, this is the first case report of a PNET with these features. Despite surgery and chemotherapeutic treatment, our case has shown disease progression.

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Somatic mutations ofWNT/wingless signaling pathway components in primitive neuroectodermal tumors

International Journal of Cancer ◽

10.1002/ijc.1342 ◽

2001 ◽

Vol 93 (3) ◽

pp. 445-449 ◽

Cited By ~ 117

Author(s):

Arend Koch ◽

Anke Waha ◽

J�rg C. Tonn ◽

Nils S�rensen ◽

Frank Berthold ◽

...

Keyword(s):

Signaling Pathway ◽

Somatic Mutations ◽

Primitive Neuroectodermal Tumors ◽

Neuroectodermal Tumors ◽

Wingless Signaling

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Multiple granular cell tumors and primitive neuroectodermal tumors (PNETs) - is there a syndromic relationship?

JDDG Journal der Deutschen Dermatologischen Gesellschaft ◽

10.1111/ddg.12683 ◽

2015 ◽

Vol 14 (1) ◽

pp. 59-61

Author(s):

Sandra Mauer ◽

Claudia Pföhler ◽

Yoo Jin Kim ◽

Thomas Krenn ◽

Sabine Heine ◽

...

Keyword(s):

Granular Cell ◽

Primitive Neuroectodermal Tumors ◽

Neuroectodermal Tumors

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Intracranial Peripheral Primitive Neuroectodermal Tumors of the Cavernous Sinus: A Diagnostic Peculiarity

Archives of Pathology & Laboratory Medicine ◽

10.5858/2005-129-e11-ippnto ◽

2005 ◽

Vol 129 (1) ◽

pp. e11-e15 ◽

Cited By ~ 1

Author(s):

Muhammad Idrees ◽

Chirag Gandhi ◽

Simone Betchen ◽

James Strauchen ◽

Wesley King ◽

...

Keyword(s):

Cavernous Sinus ◽

Ewing Sarcoma ◽

Moderate Amount ◽

Primitive Neuroectodermal Tumors ◽

Neuroectodermal Tumors ◽

Male Preponderance ◽

Poorly Differentiated ◽

Immunohistochemical Techniques ◽

The Right ◽

Peripheral Primitive Neuroectodermal Tumors

Abstract Peripheral primitive neuroectodermal tumors (pPNETs) are aggressive, poorly differentiated neoplasms that occur in children and young adults. These tumors are associated with a peak incidence in the second decade and a slight male preponderance. Recently, Ewing sarcoma and pPNET tumors have been proven to carry identical translocations, the most common being t(11;22)(q24;q12). Intracranial Ewing sarcoma/pPNETs have rarely been described in the literature. We studied a case of intracranial pPNET arising in the right cavernous sinus of a 46-year-old man. On imaging, the tumor had both sellar and suprasellar components and was centered within the right parasellar region. Histologically, the tumor was composed of intermediate to large cells with round to oval hyperchromatic nuclei with distinct nucleoli. The cells contained a moderate amount of slightly basophilic cytoplasm. The tumor was markedly fibrotic and had collagen bands surrounding both individual and groups of cells. A large immunohistochemical panel was positive only for CD99 and vimentin. Fluorescence in situ hybridization did not show translocations associated with Ewing sarcoma/pPNET. However, a small percentage of these tumors can be negative for this translocation. In these cases, histology and immunohistochemical techniques in the absence of an alternative diagnosis are the only tools available to establish the diagnosis.

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Primitive Neuroectodermal Tumor of the Lung

Archives of Pathology & Laboratory Medicine ◽

10.5858/2001-125-0397-pntotl ◽

2001 ◽

Vol 125 (3) ◽

pp. 397-399

Author(s):

Andrea G. Kahn ◽

Alejandra Avagnina ◽

Jorge Nazar ◽

Boris Elsner

Keyword(s):

Primitive Neuroectodermal Tumor ◽

Neuron Specific Enolase ◽

Middle Lobe ◽

Neurosecretory Granules ◽

Primitive Neuroectodermal Tumors ◽

Ultrastructural Studies ◽

Neuroectodermal Tumors ◽

Right Middle Lobe ◽

Cytoplasmic Processes

Abstract Primitive neuroectodermal tumors occur most frequently in bone and soft tissue but have been reported in other locations. Primary lung primitive neuroectodermal tumors without pleural or chest wall involvement are extremely rare. We present a case with immunohistochemical and ultrastructural studies and follow-up of the patient. An 18-year-old man presented with hemoptysis. Chest radiographs revealed a right middle lobe mass, and bronchoscopy showed an endobronchial tumor. The lesion was resected by middle lobectomy. After 2 years, a local recurrence was treated by pneumonectomy. The patient died after surgery. Histologically, the tumor was composed of uniform cells with round nuclei and scanty cytoplasm arranged in cohesive lobules with occasional rosette formation. Immunohistochemically, the tumor was positive for vimentin, CD99, neuron-specific enolase, and neurofilaments. Ultrastructural study revealed neurosecretory granules and cytoplasmic processes. Our case shows the value of immunohistochemistry and electron microscopy in the diagnosis of primitive neuroectodermal tumors in unusual locations.

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