Spinal rosette-forming glioneuronal tumor

Abstract

Rationale: Rosette-forming glioneuronal tumor (RGNT) is a rare tumor which has been first reported as the fourth ventricle tumor by Komori et al and is classified as a distinct clinicopathological entity by the WHO Classification of Tumors of the Central Nervous System as in 2007. Although RGNTs were reported to occur in both supratentorial and inflatentorial sites, only 4 case reports of spinal RGNT have been demonstrated. Patient concerns: A 37-year-old female presenting with slowly progressing right-sided clumsiness. Cervical magnetic resonance imaging revealed a spinal intramedullary tumor between the C2 and C5 levels. Diagnoses: Pathological analysis showed unique biphasic cellular architecture consisting of perivascular pseudorosettes dominantly with few neurocytic rosettes and diffuse astrocytoma component. The tumor cells composed of perivascular pseudorosettes showed positivity for both synaptophysin and glial markers such as GFAP and Olig2. Therefore, the diagnosis of RGNT was made. Interventions: Gross total resection of the tumor was achieved. No adjuvant chemotherapy nor radiotherapy was conducted after operation. Outcomes: At 2 years after the operation, no recurrence was observed. Lessons: Although RGNT arising from the spinal cord is extremely rare, we need to consider the tumor as a differential diagnosis for intramedullary spinal cord tumors.

Spinal rosette-forming glioneuronal tumor: First case in a young child

“…Syringomyelia adjacent to the tumor has often been observed in the past spinal cases. Spinal RGNTs differ from intracranial RGNT in their lack of both satellite lesions on MRI and calcification on CT. Hemosiderin deposition (as is often the case with ependymoma) has been reported in only one spinal RGNT case [2 , 4 – 9] .…”

“…This newly recognized tumor entity is little known, and spinal manifestations of RGNT are especially rare. There have been only seven cases of spinal RGNT reported so far: two males, five females, ages 14‐44 years (average, thirty-two years) [4] , [5] , [6] , [7] , [8] – 9] . All but two of the spinal RGNT cases originated from the cervical region; the others involved the thoracic to lumbar regions.…”

“…The etiology of this tumor remains uncertain. Several research groups have suggested that RGNT might be derived from the subependymal plate that belongs to the second germinal layer [3 , 4 , 10] . Yang et al .…”

“…Surgical resection is reported to be the first-choice treatment for spinal RGNT, with no adjuvant therapy [4] . In previous spinal cases, patients underwent a total resection when feasible, without recurrence.…”

The rosette-forming glioneuronal tumor mimicked cerebral cysticercosis: a case report

“…On MRI, RGNT typical imaging findings are relatively well circumscribed, with both solid and cystic components with T1-hypo-intense and T2-hyper-intense located in or around the fourth ventricle. Gadolinium-based contrast enhancement could show variable or no enhancement, but with increasing reports of the disease, other positions have also been reported, including the pineal region, pons, thalamus, spinal cord, optic chiasm, cerebellar hemisphere, optic pathway, lateral ventricle, septum pellucidum, cerebellar vermis, and even temporal lobe [6][7][8][9].…”