Primary Primitive Neuroectodermal Tumor of the Conus Medullaris in an Elderly Patient: A Case Report and Review of the Literature

Shimosawa, H.; Matsumoto, Mitsuomi; Yabe, Hiromasa; Mukai, Minoru; Toyama, Yoshihiro; Morioka, Harumi

doi:10.1159/000323263

Cited by 7 publications

(8 citation statements)

References 22 publications

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“…5 Previous studies have shown that CD99 has a high sensitivity and specificity for primary spinal pPNETs, which might obviate the need for chromosomal studies, though chromosomal studies can be complementary to achieving a definite diagnosis. 5,21 Besides, NSE, Syn, S-100, vimentin, and CD56 are commonly expressed in pPNETs, while pNETs often have negative staining for GFAP, which is also useful for reaching a differential diagnosis. 22 Due to the rarity of the disease, there are no standard therapeutic strategies to treat primary spinal PNETs.…”

Section: Discussionmentioning

confidence: 99%

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Wang

Zhang

Song³

et al. 2021

J Neurol Surg A Cent Eur Neurosurg

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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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Section: Discussionmentioning

confidence: 99%

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Wang

Zhang

Song³

et al. 2021

J Neurol Surg A Cent Eur Neurosurg

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“…The majority of spinal PNETs are the result of the subarachnoidal spread of tumors in the neuraxis. 8 Before the diagnosis of primary spinal PNET can be confirmed, the more common "drop" metastases of intracranial origin must be excluded. 9 This is the main reason why the patient underwent cranial and whole spine MRI before starting craniospinal irradiation (CSI) and concurrent chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Spinal Primitive Neuroectodermal Tumor (PNET) in a 64-year-old Male Treated with Surgery, Radiotherapy, and Chemotherapy: A Case Report

Gantuangco

Manalo-Igot

2021

Acta Med Philipp

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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.

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“…The tumor usually presents with nonspecific symptoms such as paraparesis, paresthesias, gait disturbance, and low back pain as in our case. [ 10 11 ] MRI features of PNET are also usually nonspecific, with most of them being hyperintense on T2-WI and iso- to hypointense on T1-WI, with heterogeneous enhancement on postcontrast sequences. In our case, it was hypointense on T1-WI and T2-WI and had no obvious contrast enhancement.…”

Section: Discussionmentioning

confidence: 99%

Primary intraspinal extradural primitive neuroectodermal tumor: A rare case

et al. 2016

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Primitive neuroectodermal tumors (PNETs) are aggressive childhood malignancies and are difficult to treat. Primary intraspinal PNETs are rare. These patients have poor prognosis with short survival time even after surgery and chemoradiation. As there are no standard guidelines exist for the management of these tumors, a multidisciplinary approach has been employed with varying success. According to the review of literature, only few cases of primary intraspinal extradural PNETs have been reported. Herein, author has described a case of intraspinal, extradural PNET.

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Primary Primitive Neuroectodermal Tumor of the Conus Medullaris in an Elderly Patient: A Case Report and Review of the Literature

Cited by 7 publications

References 22 publications

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

Spinal Primitive Neuroectodermal Tumor (PNET) in a 64-year-old Male Treated with Surgery, Radiotherapy, and Chemotherapy: A Case Report

Primary intraspinal extradural primitive neuroectodermal tumor: A rare case

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