A case of diffuse leptomeningeal glioneuronal tumor in a 10‐year‐old boy: First report from Iran

Abstract

A 10‐year‐old boy who was referred due to acute hydrocephalus symptoms was diagnosed as the first case of pediatric DLGNT in Iran. The results suggested that using shunting for hydrocephaly and anti‐seizure medicines, as well as chemotherapeutic agents, can be an effective treatment strategy for DLGNT. Although the patient was stable without a tumor recurrence for a limited follow‐up period of 22 months, further studies are expected.

Diffuse Leptomeningeal Glioneuronal Tumour: An Intriguing Case Report of an Adult Patient with a Previous Optic Astrocytoma

“…The imaging features indicating a case of DLGT are a prominent leptomeningeal enhancement with or without communicating hydrocephalus [15], which is due to the pronounced development of the tumour within the subarachnoid space [16]. The immunohistochemistry of DLGT cells shows that they are positive for OLIG 2 and S100 and negative for GFAP, cytokeratin and EMA [16]; our patient was negative for EMA and positive for S100, and the others were not tested. Utilising the molecular genetic testing and pathohistological analysis, we were able to deduce that ours was not a case of a malignant transformation, but rather a case of another primary CNS tumour.…”

“…At present, no standardised treatment protocol has been developed for patients with DLGT, the majority are treated with a combination of surgery, chemotherapy and radiotherapy [14], however, targeted therapy and immunotherapy are also an option [11]. The majority of patients, who are treated with chemotherapy (systemic or intrathecal), receive a combination of temozolomide, carboplatin and vincristine [16]; the patient in our case report was administered carboplatin and vincristine systemically. The main purpose of the current surgical treatment is to insert a ventriculoperitoneal shunt in cases of hydrocephalus or to insert an Ommaya sac under the scalp for intraventricular chemotherapy [11].…”

“…Biopsy, histological analysis, and molecular genetic testing are crucial for diagnosing DLGT, as imaging findings are not specific enough and cases of DLGT have been misdiagnosed as tuberculosis [14,15]. The imaging features indicating a case of DLGT are a prominent leptomeningeal enhancement with or without communicating hydrocephalus [15], which is due to the pronounced development of the tumour within the subarachnoid space [16]. The immunohistochemistry of DLGT cells shows that they are positive for OLIG 2 and S100 and negative for GFAP, cytokeratin and EMA [16]; our patient was negative for EMA and positive for S100, and the others were not tested.…”

Rapidly progressive diffuse leptomeningeal glioneuronal tumor in an adult female: illustrative case

“… 4 , 5 In addition, multiple groups found that carboplatin and vincristine were able to improve survival and halt disease progression. 8 , 24 , 26 Combined treatment regimens may be more promising as complete remission of the disease was achieved using both craniospinal irradiation and temozolomide in 1 case report of a teenager. 27 No consensus has been achieved on optimal treatment protocols because of limited reports of this disease in the literature.…”

“…Various treatment regimens have been proposed in the literature, including craniospinal irradiation and the use of chemotherapeutic or biological agents such as carboplatin, bevacizumab, and temozolomide. 8,[24][25][26] Craniospinal irradiation provides more targeted treatment and has been documented to improve clinical outcomes and slow disease progression. 4,5 In addition, multiple groups found that carboplatin and vincristine were able to improve survival and halt disease progression.…”