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罕见的颌内丛状神经鞘瘤。

Unusual intramaxillary plexiform schwannoma.

作者信息

Lambade Pravin N, Lambade Dipti, Saha Tapan K, Dolas R S, Dhobley Akshay

机构信息

Department of Oral and Maxillofacial Surgery, Swargiya Dadasaheb Kalmegh Smruti Dental College and Hospital, Nagpur, Maharashtra, India.

出版信息

Oral Maxillofac Surg. 2013 Jun;17(2):137-40. doi: 10.1007/s10006-012-0345-6. Epub 2012 Jul 31.

Abstract

BACKGROUND

Neoplasms of peripheral nerve in the head and neck region are of common occurrence, but origin in the oral and para-oral tissues is uncommon and they rarely occur centrally within the jaws. Schwannoma is a benign neoplasm originating from the neural sheath of peripheral soft tissues, but its occurrence within the jaw bones is most unusual. Plexiform schwannoma is a unique variant of Schwann cell tumours having plexiform pattern. Literature revealed only one case of plexiform schwannoma of the jaw bones, i.e. involving the mandible.

CASE REPORT

In this report, we present the first documented case of intraosseous plexiform schwannoma of the maxilla, an extremely rare benign neurogenic tumour treated surgically.

DISCUSSION

Schwannoma is a benign neoplasm originating from the neural sheath of peripheral soft tissues, but to occur within the jaw bones is exceptional. Plexiform schwannoma is a rare variant of Schwann cell tumour having plexiform pattern of intraneural growth with multinodularity. Plexiform schwannoma is a benign neoplasm with no malignant potential, but recurrences are evident if excised incompletely. Plexiform schwannoma has similar clinical and histopathological features as that of plexiform neurofibroma which has high malignant potential; hence, it is imperative to correctly diagnose and differentiate this lesion as treatment modality of these two lesions differs.

摘要

背景

头颈部周围神经肿瘤较为常见,但起源于口腔及口周组织的并不常见,且很少发生于颌骨中央。施万细胞瘤是一种起源于周围软组织神经鞘的良性肿瘤,但其发生于颌骨内极为罕见。丛状施万细胞瘤是施万细胞肿瘤的一种独特变体,具有丛状结构。文献报道仅1例颌骨丛状施万细胞瘤,即累及下颌骨。

病例报告

在本报告中,我们呈现了首例上颌骨骨内丛状施万细胞瘤的记录病例,这是一种经手术治疗的极为罕见的良性神经源性肿瘤。

讨论

施万细胞瘤是起源于周围软组织神经鞘的良性肿瘤,但发生于颌骨内实属罕见。丛状施万细胞瘤是施万细胞肿瘤的一种罕见变体,具有神经内生长的丛状结构和多结节性。丛状施万细胞瘤是一种无恶性潜能的良性肿瘤,但如果切除不完全则容易复发。丛状施万细胞瘤具有与具有高恶性潜能的丛状神经纤维瘤相似的临床和组织病理学特征;因此,正确诊断和鉴别该病变至关重要,因为这两种病变的治疗方式不同。

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