Multifocal Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIA/DIG): An Institutional Series Report and a Clinical Summary of This Rare Tumor

Abstract

AimMultifocal desmoplastic infantile ganglioglioma/astrocytoma (DIA/DIG) has rarely been reported. Here, two cases have been presented, reviewing the literature and proposed treatment algorithms for this rare tumor.Patients and MethodsWe report two patients diagnosed with multifocal DIA/DIGs in West China Hospital. In addition, a literature review was performed, in October 2019, on case reports of DIA/DIGs with multifocal lesions. The clinical and radiological features, treatment, and outcome of this rare disease were discussed.ResultsDIA/DIGs with multifocal locations were rare, and only thirteen cases (including ours) had been reported. This series included 8 males and 5 females with a mean age of 31.4 ± 45.7 months (range, 3-144 months). The supratentorial hemisphere, suprasellar region, posterior cranial fossa, and spinal cord were frequently involved. Ten patients (76.9%) received surgical resection for the symptomatic lesions and three patients (23.1%) underwent biopsy. Seven patients received chemotherapy postoperatively. Six individuals had tumor recurrences during the follow-up period, while three patients had tumors that spontaneously regressed. Finally, two patients died of tumor progression and one patient died of respiratory insufficiency and hypothalamic dysfunction.ConclusionsMultifocal DIA/DIGs have more aggressive clinical behavior and poor outcome despite benign histology. DIA/DIGs should be included in the differential diagnosis of multifocal brain tumors in children. The mainstay of treatment is surgical resection; adjuvant treatment with chemotherapeutic drugs is unknown and requires additional research.

Malignant transformation of desmoplastic infantile tumors in a non-infant: a case report

“…DITs often occur in infants and rarely in adults ( 2 , 9 ), which is divided into the infant group (<5 years old) and the non-infant group (>5 years old) ( 10 ). These low-grade tumors usually have a good prognosis, but some have malignant features, such as multiple intracranial lesions ( 3 , 4 ), postoperative recurrence ( 2 , 4 , 5 ), meningeal diffusion, and metastasis ( 6 ).…”

“…If surgical resection is complete, the patient has a good prognosis without radiotherapy and chemotherapy. However, the benign nature of this tumor has been questioned with successive reports of atypical, aggressive, and multifocal DITs (2)(3)(4)(5)(6). Here, we report a rare case of DITs in a non-infantile who underwent a malignant transformation after surgical resection 12 years later, followed by spread and metastasis.…”