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Giuseppe Cinalli, Alessandra Marini, Carmela Russo, Pietro Spennato, Giuseppe Mirone, Claudio Ruggiero, Lucia Quaglietta, Maria Serena de’ Santi, and Eugenio Covelli

OBJECTIVE

The goals of this study were to evaluate the extent of resection (EOR) obtained with an occipital interhemispheric transtentorial approach (OITA) in a series of pediatric patients with pineal region tumors and to define preoperative radiological factors predictive of the EOR.

METHODS

This is a retrospective cohort study of a series of pediatric patients with pineal tumors who underwent surgery through a microsurgical OITA performed by the senior author during the period from January 2006 to January 2020. The tumor volume was measured preoperatively, and then on sagittal midline cuts the authors identified the most cranial point of the torcular Herophili (defined as the “Herophilus point”) and the lowest point of the inferior profile of the vein of Galen (defined as the “Galen point”). The line joining these two points (defined as the "Herophilus-Galen line" [H-G line]) was used to identify the "Herophilus-Galen plane" (H-G plane) perpendicular to the sagittal plane. Tumor volumes located below and above this plane were measured. EOR was evaluated by measuring residual tumor volume visible on T1 volumetric injected sequences of immediate postoperative MRI.

RESULTS

Thirty patients were selected for study inclusion. The preoperative mean tumor volume was 15.120 cm3 (range 0.129–104.3 cm3). The mean volumes were 2.717 cm3 (range 0–31 cm3) above the H-G plane and 12.40 cm3 (median 5.27 cm3, range 0.12–72.87 cm3) below the H-G plane. Three patients underwent only biopsy. Of the remaining 27 patients, gross-total resection (GTR; 100% tumor volume) was achieved in 20 patients (74%). In the remaining 7 patients, the mean residual tumor volume was 7.3 cm3 (range 0.26–17.88 cm3). In 3 of these patients, GTR was accomplished after further surgical procedures (1 in 2 patients, 3 in 1 patient) for an overall GTR rate of 85.18%. Larger tumor volume was significantly associated with incomplete resection (p < 0.001). A tumor volume ≤ 2 cm3 above the H-G plane (p = 0.003), linear extension ≤ 1 mm above the H-G line, and pineal histology were predictive of GTR at first OITA procedure (p = 0.001).

CONCLUSIONS

The H-G line is an intuitive, easy-to-use, and reliable indicator of the superior anatomical limit of visibility during the microsurgical OITA. This anatomical landmark may be useful as a predictor of EOR for pineal tumors performed through this approach. The main limitations of this study are the small number of patients and the exclusively pediatric age of the patient population.

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Giancarlo Nicosia, Pietro Spennato, Ferdinando Aliberti, Daniele Cascone, Lucia Quaglietta, Maria Elena Errico, Mario Muto, Franco Ionna, and Giuseppe Cinalli

Melanotic neuroectodermal tumor of infancy is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, growing rapidly and developing during the 1st year of life. It most commonly arises from the maxilla, cranial vault, and mandible. Occasionally, it exhibits malignant behavior with local lymph nodes involvement. Cases misdiagnosed and left untreated for a long time can present challenges due to the tumor mass and infiltration. In these cases, adjuvant chemotherapy can be extremely helpful before radical excision.

Authors of this report describe a 4-year-old boy from a developing country who was referred to their hospital with an ulcerated bulging lesion in the midline/right parietooccipital region, extending to the right laterocervical and parotid regions, resulting in significant craniofacial deformation. Magnetic resonance imaging of the brain revealed a highly enhancing tumor with intracranial and extracranial development extending mainly at the level of the right parietooccipital region, with lytic and hypertrophic alterations of the skull. The patient was managed with neoadjuvant and adjuvant chemotherapy and radically resective surgery on metastatic lymph nodes and the primary tumor of the skull. Scheduled radiotherapy was not performed, according to the parents' wishes. The patient returned to his native country where the lesion recurred, and he ultimately died approximately 10 months after the end of the treatment.

The literature indicates that tumor removal alone has been the treatment of choice in most isolated cases, but in cases of highly advanced tumor with involvement of the skull and cervical lymph nodes, it is preferable to proceed with preoperative chemotherapy with the aim of reducing the tumor volume, allowing better technical conditions for complete surgical removal, and decreasing the risk of local recurrence or metastasis.

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Rodolfo Maduri, Viviana Aureli, Vincent Dunet, Roy Thomas Daniel, and Mahmoud Messerer

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Giuseppe Cinalli, Daniel T. Aguirre, Giuseppe Mirone, Claudio Ruggiero, Daniele Cascone, Lucia Quaglietta, Ferdinando Aliberti, Serena de’ Santi, Maria Consiglio Buonocore, Anna Nastro, and Pietro Spennato

OBJECTIVE

In the past, the outcome of surgical treatment for thalamic tumor was poor. These lesions were often considered inoperable. However, contemporary microsurgical techniques, together with improvements in neuroimaging that enable accurate presurgical planning, allow resection to be accomplished in a safer way.

METHODS

The medical records, imaging studies, and operative and pathology reports obtained for pediatric patients who were treated for thalamic tumors at the authors’ department were reviewed. Neuronavigation and intraoperative monitoring of motor and somatosensory evoked potentials were used. Preoperative tractography, which helped to identify internal capsule fibers, was very important in selecting the surgical strategy. Postoperatively, an MRI study performed within 24 hours was used to assess the extent of tumor resection as partial (≤ 90%), subtotal (> 90%), or gross total (no residual tumor).

RESULTS

Since 2002, 27 children with thalamic tumors have been treated at the authors’ department. There were 9 patients with unilateral thalamic tumors, 16 with thalamopeduncular tumors, and 2 with a bilateral tumor. These last 2 patients underwent endoscopic biopsy and implantation of a ventriculoperitoneal shunt. Thirty-nine tumor debulking procedures were performed in the remaining 25 patients. Different surgical approaches were chosen according to tumor location and displacement of the posterior limb of the internal capsule (as studied on axial T2-weighted MRI) and corticospinal tract (as studied on diffusion tensor imaging with tractography, after it became available). In 12 cases, multiple procedures were performed; in 7 cases, these were done as part of a planned multistage resection. In the remaining 5 cases, the second procedure was necessary because of late recurrence or regrowth of residual tumor.

At the end of the surgical phase, of 25 patients, 15 (60%) achieved a gross-total resection, 4 (16%) achieved a subtotal resection, and 6 (24%) achieved a partial resection. Eighteen patients harbored low-grade tumors in our series. In this group, the mean follow-up was 45 months (range 4–132 months). At the end of follow-up, 1 patient was dead, 12 patients were alive with no evidence of disease, 4 patients were alive with stable disease, and 1 was lost to follow-up. All patients were independent in their daily lives. The outcome of high-grade tumors in 9 patients was very poor: 2 patients died immediately after surgery, 6 died of progressive disease, and 1 was alive with residual disease at the time of this report.

CONCLUSIONS

This institutional review seems to offer further evidence in favor of attempts at radical resection in pediatric patients harboring unilateral thalamic or thalamopeduncular tumors. In low-grade gliomas, radical resection in a single or staged procedure can be curative without complementary treatment. Recurrences or residual regrowth can be safely managed surgically. In high-grade tumors, the role of and opportunity for radical or partial resection remains a matter of debate.