Oral leiomyomatous hamartoma associated with upper lip midline malformation: Case report and review of the literature

A 3-month-old male patient presented an upper lip malformation associated to a polypoid nodule in the maxillary anterior alveolar ridge. Microscopically, the nodule was predominantly composed by a non-encapsulated smooth muscle proliferation intermingled by numerous blood vessels of varied sizes and scattered small nerve fibers. The smooth muscle tissue was positive for desmin and smooth muscle actin, while endothelial cells were highlighted by CD34 staining. The final diagnosis was of an oral leiomyomatous hamartoma associated with upper lip malformation. After surgical treatment, no recurrence has been observed after 2 years of follow-up.     

喉错构瘤一例

患者男,61岁,因咽部不适伴嗓音改变2月余于2010年5月19日入院.患者无呼吸困难、咳血、呛咳及吞咽困难等症状.查体:全身一般情况良好,生命体征平稳,心率80次/min,呼吸18次/min,心电图、胸部X线检查心肺无异常.纤维喉镜下见喉人口处被一类球形肿物遮蔽,肿物随呼吸上下移动,肿物直径约2.5 cm,边缘清楚,其蒂部与右侧杓会厌襞相连,肿物被覆黏膜光滑,色泽正常;双侧声带正常,活动良好,声门闭合完全.喉CT:右侧杓会厌襞处见一类圆形低密度肿物影,边缘光滑,内部密度均匀,CT值约36 HU(图1a,b).术前诊断为喉会厌囊肿.采用经喉气管插管静脉复合麻醉支撑喉镜下喉会厌肿物切除术.术中以喉钳触动肿物呈实性感,有一定活动度.镜下以喉黏膜刀沿肿物正中切开被覆黏膜,将肿瘤完整剥离、摘除.术中出血不多,无手术并发症.摘除的肿瘤质地较韧,剖检瘤体显示其为实性肿瘤.术后病理诊断为间质性喉错构瘤,平滑肌肌动蛋白(smooth muscle actin,SMA)、结蛋白(desmin)和CD34免疫组化染色均呈阳性(图2a～d).患者术后恢复佳,术后3 d痊愈出院.术后6个月随访及纤维喉镜检查,患者情况良好,局部无复发.     

咽鼓管错构瘤1例报道并文献复习

目的 探讨咽鼓管错构瘤的临床表现、诊断与治疗及预后.方法 回顾性分析萍乡市人民医院收治的1例经病理确诊的咽鼓管错构瘤患者,经耳显微镜在全麻下行咽鼓管肿瘤切除及鼓室成型和听骨链重建术,术中完整切除肿瘤.结果 术后病理学提示符合错构瘤.经过半年随访,肿瘤无复发,鼓膜完整,听力得到提高.结论 错构瘤是一种可发生于全身的良性肿...     

Giant hamartoma of the retro- and parapharyngeal region. Case report and review of literature

Hamartoma designates a tumor-like, non-neoplastic malformation, or in-born error of tissue development, often with an abnormal mixture of tissue indigenous to the region. In the head and neck, epithelial and mesenchymal hamartomas are uncommon. We present the case of a 9-year-old boy with a huge hamartoma of 800ml (cm(3)) volume of the right retro- and parapharyngeal region. It had fully occluded the nasopharynx, indented the base of the tongue and left a minimal airway passage transorally. Food uptake was severely hampered by the lesion. The patient underwent a combined transoral and external approach for complete resection of the tumor. This is the first reported case of a retro- and parapharyngeal hamartoma with metaplastic bone formation in the literature and further more, the largest hamartoma in head and neck ever reported in this age group.     

Posterior cleft larynx associated with hamartoma: a case report and literature review

Posterior cleft larynx associated with a local hamartoma is a rare malformation complex. Clinical diagnosis may be elusive. We report such a combination in a newborn infant dying soon after birth with upper airways obstruction.     

鼻腔呼吸道上皮腺瘤样错构瘤1例并文献复习

目的 探讨鼻腔呼吸上皮性腺瘤样错构瘤(REAH)的临床、影像、病理学特征及治疗。 方法 分析1例鼻腔REAH患者的临床资料并复习相关文献进行总结。 结果 鼻腔REAH临床表现常以鼻塞、嗅觉减退为主。影像学方面,嗅裂增宽>10 mm是本病在CT上的特征性表现。本病确诊主要依靠病理检查,其显微镜下特点是大量内衬呼吸道纤毛上皮的腺体显著增生。 结论 鼻腔REAH临床表现无特异性,因而影像学及组织学检查对本病的早期诊断尤其重要。内镜下切除肿瘤是本病的最佳治疗手段。     

Infantile congenital petrosal cholesteatoma: a case report and literature review

Congenital cholesteatoma of the middle ear is frequently seen in younger children including infant. By contrast, that of the petrous bone is rarely seen in younger children. We report an exceptional case occurring in 23-month-old infant who presented with unilateral facial nerve paralysis at about 18-month-old. Computed tomography and magnetic resonance imaging revealed congenital cholesteatoma measuring approximately 2 cm in the area of the right geniculate ganglion. The mass was completely eradicated via the middle fossa approach, which allowed for preservation of hearing. The facial nerve maintained intact during surgery and paralysis showed partial recovery after the operation. To our knowledge, the present case seems to be the youngest case of congenital petrosal cholesteatoma reported, and also demonstrates congenital petrosal cholesteatoma could exhibit facial nerve paralysis in early childhood.     

Bilateral congenital lacrimal fistulae: a case report and review of the literature

The lacrimal system is comprised of the lacrimal glands for tear production and the lacrimal drainage system for draining tears away from the eyes. Congenital lacrimal system anomalies other than nasolacrimal duct obstruction are uncommon. Congenital lacrimal fistulae are a rare developmental anomaly, and when they occur, they are usually unilateral. Fistulae and diverticulae can originate from the canaliculus, lacrimal sac, or nasolacrimal duct. They can be seen externally as small orifices or pits located inferior and/or medial to the medial canthi. These anomalies are often asymptomatic and may go undetected. However, they can also present with epiphora or discharge. We report the case of a 4-year-old male with bilateral congenital lacrimal fistulae. The patient presented to our clinic after his parents discovered bilateral pits located inferior and medial to the medial canthi. The pits had first been noticed only a few weeks prior to presentation. There was no history of drainage or infection. The diagnosis of bilateral congenital lacrimal fistulae was confirmed with computed tomographic imaging. No other systemic, nasal, or ocular anomalies were found. The patient was referred to pediatric ophthalmology and because the lacrimal fistulae have been asymptomatic to this point, it was decided to proceed with a course of observation. We will discuss the embryologic basis for congenital lacrimal fistulae, as well as the typical presentation and possible treatment modalities. The presence of lacrimal fistulae is an indicator to search for a variety of underlying systemic and ocular anomalies.     

Teratoma tongue: Case report and review of literature

Teratomas are true neoplasms composed of tissues from all three germinal layers and may exhibit variable levels of maturity. They have an unknown origin and eccentric microscopic appearance. Teratomas arising from the oral cavity are rare in the newborn; only 22 cases have been reported in the literature. We describe a female neonate with an oral teratoma originating from the tongue that was successfully treated with surgery.     

Laryngeal kaposiform hemangioendothelioma: Case report and literature review

The kaposiform hemangioendothelioma (KHE) is an uncommon vascular neoplasm of borderline or intermediate malignant potential in which involvement of the head and neck region is rare.     

Respiratory epitheloid adenomatoid hamartoma of the nasal cavity--case report and review of the literature

BACKGROUND: Hamartomas are tumour-like malformations, resulting from excessive proliferation of otherwise normal local tissue components. Hamartomas of the nasal cavity and paranasal sinuses are relatively rare, especially respiratory epithelial adenomatoid hamartomas. This benign lesion is characterized by a glandular proliferation originating from the surface of the respiratory epithelium. In the German speaking literature this entity has not been described before. PATIENT AND RESULTS: We report the case of a 51-year-old white female, presenting with a respiratory epithelial adenomatoid hamartoma of her left nasal cavity. The patient suffered from nasal obstruction. The CT scan showed a soft tissue like lesion obstructing the patient's left choana. After local resection of the tumour, which originated from the superior turbinate through functional endoscopic sinus surgery the patient's symptoms were gone. Follow-up after six months showed no evidence of recurrence. CONCLUSION: The histopathological differential diagnosis of respiratory epithelial adenomatoid hamartomas includes inverted papilloma and adenocarcinoma. Therefore the histological recognition of these hamartomas impacts on therapy. Misinterpretation as a true neoplasm may result in unnecessarily radical surgery. Local surgical resection is the treatment of choice for this lesion.     

婴儿鼻腔软骨间叶性错构瘤2例及文献复习

目的 探讨婴儿鼻腔软骨间叶性错构瘤的临床及病理特点、诊断、治疗及预后。 方法 回顾性分析2016年5月至2019年3月上海交通大学医学院附属新华医院收治的2例经病理证实为鼻腔软骨间叶性错构瘤患儿的临床及病理资料,并对该病的诊断、治疗及预后进行探讨。 结果 起源于鼻腔或颅底的鼻腔软骨间叶性错构瘤经鼻内镜术完整切除,长期随访无复发。组织学显示间叶细胞及胶原纤维围绕软骨结构,呈良性增生特性。 结论 婴儿鼻腔软骨间叶性错构瘤临床表现多样且无特异性,早期诊断困难。鼻内镜手术是治疗鼻腔软骨间叶性错构瘤的主要手段,并且具有良好的临床效果。     

Alveolar rhabdomyosarcoma of the larynx: Case report and literature review

We report the 11th well-documented case of laryngeal rhabdomyosarcoma and only the second of laryngeal alveolar rhabdomyosarcoma. The optimum treatment of head and neck rhabdomyosarcoma has not yet been defined, but it appears that the ideal should consist of an aggressive multimodality approach. This includes surgical extirpation if it can be done without major morbidity, postoperative radiotherapy with a margin around known and suspected disease, and multiagent chemotherapy. Unlike most sarcomas, rhabdomyosarcoma (especially alveolar rhabdomyosarcoma) spreads by way of the lymphatic system as well as the blood stream. Inclusion of the draining lymphatics in the radiation field must be given strong consideration.