Melanotic neuroectodermal tumor of infancy arising from the skull: report of an unusual case, review of the literature, and a diagnostic approach

Mengide, Juan Pablo; Jaimovich, Sebastián Gastón; Lubieniecki, Fabiana; Rugilo, Carlos; Lamas, Gabriela; Mantese, Beatriz

doi:10.1007/s00381-019-04476-7

Cited by 5 publications

(6 citation statements)

References 22 publications

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“…It is essentially a rapid-growing painless tumor causing facial malformation, while the mucosa surface is intact, pigmented and non-ulcerated [4,6]. To the best of our knowledge, there have been few studies focusing on the imaging findings of MNTI to date, and most of the studies were MNTIs from the skull [7][8][9][10][11][12]. During the past five years, four cases of MNTI occurring in maxillary bone have been confirmed by histopathology in our hospital.…”

Section: Introductionmentioning

confidence: 80%

“…In our patients, involved maxillae all presented osteolytic bone destruction, expansive bone destruction and fragmentary cortical bone. Case 2 and case 3 also showed spiculated/sunburst periosteal reaction, suggesting a rapid-growing or even malignant process [ 9 ]. Displaced or “free-floating” tooth germs can be seen in all the four patients.…”

Section: Discussionmentioning

confidence: 99%

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Clinical and imaging features of melanotic neuro-ectodermal tumor of infancy of the maxillary bone: report of four cases and review of the literature

et al. 2022

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Melanotic neuro-ectodermal tumor of infancy (MNTI) is an extremely rare tumor. The purpose of this study was to describe the imaging features of maxillary bone MNTIs and introduce the key points for clinical diagnosis of MNTI. We retrospectively reviewed four patients with histology-proven MNTIs arising from the maxillary bone. All patients underwent ultrasonic inspections, CT and/or MR scanning. Combined with previously literature, the imaging features were comprehensively evaluated and analyzed. All MNTIs showed a firm, non-ulcerated rapidly-growing soft-tissue swelling with pigmented (blue-colored or black-colored or gray-colored) mucosa. The onset ages were younger than 6 month-old. CT images showed osteolytic or expansive bone destruction of the involved maxillae, fragmentary cortical bone, “free-floating” tooth germs, with or without spiculated/sunburst periosteal reaction. The tumor appeared lightly hyper-intense on T2-weighted sequences, while isointense or lightly hypo-intense or lightly hyper-intense signal on T1-weighted sequences. Enhanced images all displayed heterogeneous enhancement. No metastasis features of lymph nodes or abdominal organs were demonstrated by cervical and abdominal ultrasonic inspections. As a conclusion, accurate recognition of the imaging features of MNTI combined with history and clinical manifestations (early infancy, painless, firm, pigmented mucosa, non-ulcerating lesion) can provide clues for diagnosis of this rare entity.

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Section: Introductionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Clinical and imaging features of melanotic neuro-ectodermal tumor of infancy of the maxillary bone: report of four cases and review of the literature

et al. 2022

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“…Although histological evaluation is important for the final diagnosis of a pathological lesion, in cases of MNTI it is a poor indicator of clinical behavior ( 11 ). However, immunohistochemical analysis provides some information about the aggressiveness of these tumors ( 17 ).…”

Section: Discussionmentioning

confidence: 99%

Melanotic Neuroectodermal Tumor of Infancy: the Use of Immunohistochemical Analysis

Sousa¹,

Almeida²,

Mendes³

et al. 2022

Acta Stomatol Croat

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The Melanotic Neuroectodermal Tumor of Infancy (MNTI) is an asymptomatic, pigmented neoplasm characterized by a fast and locally aggressive growth along with a rare tissue formation. In the diagnostic process, the use of imaging exams can suggest a local destruction suggestive of malignancy, a sign of bone remodeling and expansion. Therefore, as any early diagnosis minimizes risks and improves the prognosis of treatment for the patient, the aim of this study was, based on a clinical case report, to corroborate the use of histopathological analysis associated with immunohistochemistry. Thus, we conclude that the immunohistochemical exam is of great importance for a better complementation of the MNTI diagnosis process. In addition, it can reveal signs of possible aggressive growth.

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“…In the literature, there are only 3 publications mentioning tumor embolization in MNTI [6, 9, 10]. All 3 cases’ tumors were fed by the external carotid system, and one patient’s tumor was also fed by branches of anterior cerebral arteries which made them first diagnose wrongly as meningioma [10].…”

Section: Discussionmentioning

confidence: 99%

“…However, only 75 patients were reported in the latest systematic review by Ren et al [4]. After Ren et al's [4] study, an additional 3 more cases of skull MNTI were published [5][6][7]. Therefore, to date, <80 cases of skull MNTI are reported in the literature.…”

Section: Introductionmentioning

confidence: 99%

Melanotic Neuroectodermal Tumor of Infancy at Skull: Rare and Rapid-Growing Tumor but Histologically Benign

Yindeedej¹,

Kittisangvara²

2021

Pediatr Neurosurg

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Introduction: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and rapid-growing tumor. However, a neurosurgeon should not overlook this entity when differential diagnosing rapid-growing skull tumor because its histology nature is just benign, and the prognosis is much better than other malignant tumors. Case Presentation: We reported the case of a 5-month old male presenting with progressive rapid-growing skull tumor which became 10 cm in diameter in only 5 months compared to the normal head circumference at birth. At first, we thought of malignant skull tumor and performed only biopsy to establish diagnosis. But, when the pathology revealed benign MNTI, we performed preoperative tumor embolization and then radical surgery. Good result was observed. Discussion: Skull MNTI is the second most common location after the maxilla. Even advanced imaging nowadays cannot distinguish MNTI from other malignant tumors definitely. Urgent biopsy is recommended to establish diagnosis of this benign tumor first. Preoperative angiography with tumor embolization is recommended when feasible, followed by craniotomy with radical resection.

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Melanotic neuroectodermal tumor of infancy arising from the skull: report of an unusual case, review of the literature, and a diagnostic approach

Cited by 5 publications

References 22 publications

Clinical and imaging features of melanotic neuro-ectodermal tumor of infancy of the maxillary bone: report of four cases and review of the literature

Clinical and imaging features of melanotic neuro-ectodermal tumor of infancy of the maxillary bone: report of four cases and review of the literature

Melanotic Neuroectodermal Tumor of Infancy: the Use of Immunohistochemical Analysis

Melanotic Neuroectodermal Tumor of Infancy at Skull: Rare and Rapid-Growing Tumor but Histologically Benign

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