Merkel cell carcinoma of the alveolar mucosa in a young adult: A rare case report

Merkel cell carcinoma (MCC) is an extremely rare and aggressive primary neuroendocrine neoplasm of the skin with a poor prognosis. It occurs mainly in the sixth and seventh decades of life and is usually associated with damage caused by over exposure to the sun. Its occurrence in intraoral mucosal sites is rare, and we know of only six reported cases. We report a rare case of MCC that arose in the alveolar mucosa of a young adult.     

Transmigration of mandibular canines: a report of six cases and a review of the literature

BACKGROUND: The transmigration of a mandibular canine is a rare phenomenon, the etiology of which is not clear. The literature on this rare condition is reviewed, and six cases of transmigrated mandibular canines are presented. REPORT: Panoramic radiographic examination of six patients revealed each patient had one impacted transmigrated mandibular canine. Of the six impacted teeth, the left mandibular canine was involved in four instances and the right in two. In one case the transmigrated canine was associated with a dentigerous cyst. SUMMARY: Transmigration of the mandibular canine is a rare event, and early radiographic examination of the patient is important for treatment. In addition, future studies may lead to a better understanding of this rare anomaly and improvement of the classification criteria.     

The role of prosthodontists in habilitation and rehabilitation in rare disorders: the ectodermal dysplasia experience

PURPOSE: The aim of this article is to highlight the strong worldwide trend of enhancing the opportunities for individuals with rare disorders to get adequate information and treatment. Hypohidrotic ectodermal dysplasia is used as an example. MATERIALS AND METHODS: The development in recent years of databases for rare disorders, the establishment of resource centers in dentistry, and the strong movement of user influence by the formation of patient organizations and exchange of information through the Internet are described. Reference is made to the recent Scandinavian consensus conference on ectodermal dysplasia. RESULTS: Motives for the centralization of treatment planning to resource centers for persons with rare disorders, the compilation of treatment outcomes in databases, and the advocation of a multidisciplinary team approach in dentistry are presented. CONCLUSION: The prosthodontic profession has a key position in the habilitation and rehabilitation of many different diagnoses of rare disorders.     

Pneumomediastinum secondary to isolated orbital floor fracture

Pneumomediastinum (PM) secondary to an isolated orbital blow-out fracture is a rare but potentially severe and life-threatening complication. Fortunately, airway obstruction, pneumothorax, pneumopericardium, and mediastinitis are rare complications of PM. Blowing the nose after a maxillofacial trauma increases pressure in the paranasal sinuses, which may lead to emphysema through a breach of the lining mucosa to the surrounding soft tissues in the head, neck, and, on occasions, to the mediastinum. We report here the rare case of a patient with PM after isolated orbital fracture. Only 3 similar cases have been previously reported in the literature.     

Colloid milium of the oral cavity: a rare presentation

Colloid milium (CM) is a rare condition characterized by the presence of multiple dome-shaped amber- or flesh-colored papules developing on sun-exposed skin. It is a degenerative condition linked to excessive sun exposure and possibly exposure to petroleum products and hydroquinone. The origin of the colloid deposition in the dermis is uncertain, although it is thought to be due to degeneration of elastic fibers. The condition is rare, with only 100 case reports documented in the world literature. The most commonly involved sites are the face, periorbital region, backs of the hands, back and sides of the neck, and ears. Involvement of the oral cavity is extremely rare. The purpose of this article is to report an extremely rare first case of CM of the oral cavity and to discuss the clinical, pathologic and therapeutic spectra of CM.     

Adaptation of the Panorex II for use with rare earth screen-film combinations

A series of radiographs of a tissue-equivalent phantom was taken in order to arrive at satisfactory radiographic techniques for the use of rare earth screens with the Panorex II. The radiation reduction needed for use of the rare earth screens was made with various filters. There was a 40% to 60% reduction in patient exposure for all of the rare earth exposures. Raters agreed that all films that were ranked were diagnostically acceptable, although differences were noted.     

Peripheral ameloblastic fibroma of the maxilla: report of a case and review of the literature

Peripheral odontogenic lesions are considered to be rare within the classification of odontogenic tumors. Also referred to as extraosseous or soft tissue odontogenic tumors, peripheral odontogenic tumors share the same histopathologic characteristics of their central or intraosseous counterparts. Ameloblastic fibroma is a rare odontogenic tumor that arises from both odontogenic epithelium and connective tissue. Only 2 cases of peripheral ameloblastic fibroma have been reported in the English-language literature, one of which did not show the classic features of an ameloblastic fibroma. In this report, we describe a rare case of a peripheral ameloblastic fibroma in the maxilla of a 3-year-old girl.     

下颌骨骨肉瘤伴对侧骨髓炎1例报告并文献复习

发生在下颌骨的骨肉瘤比较少见,同时伴对侧颌骨骨髓炎者更为罕见。本文报告下颌骨骨肉瘤伴对侧颌骨骨髓炎1例,并结合相关文献对其临床表现、诊断、治疗和预后进行分析。     

口腔罕见病与遗传病生物样本库的建立与完善

目的：：建立口腔罕见病与遗传病生物样本库,实现样本信息系统化管理。方法：以第四军医大学口腔医院为依托,与符合或疑似口腔罕见病与遗传病病症的患者或家属签订知情同意书,采用相关技术提取各类样本,完善样本的临床、遗传及分子生物学信息,录入生物样本库管理系统,通过系统产生的二维条码实施样本入库。定期对样本进行质量控制。结果：自2010年起筹建口腔罕见病与遗传病生物样本库,于2011年开始录入样本,目前已经实现了对7000余份口腔罕见病与遗传病样本的系统信息化管理,这些样本包括血液、组织、细胞、DNA、RNA、蛋白、引物等多种类型;在样本库建立和维护的过程中,从样本入库、保存、出库等多个环节实现了对样本的质量控制,同时还制定了近10种规章制度完善生物样本库的建设。结论：口腔罕见病与遗传病生物样本库的建立使疾病样本的储存系统化和规范化,为保留珍贵的疾病样本资源提供了保证,也为科研课题的实施和科研单位之间的合作提供了便利。     

Bilateral antrochoanal polyps: possible pathogenesis

Antrochoanal polyp is a rare, benign solitary mass that arises from maxillary sinus. It exits through sinus ostium, passes across the middle meatus, and reaches into the choana. In general, it represents up to 6% of all nasal polyps. Although it is usually unilateral, in extreme rare cases it could be bilateral.We herein report a case of an 11-year-old girl who presented with bilateral antrochoanal polyps and was managed successfully by endoscopic surgical approach. We also hypothesize a possible pathophysiologic explanation of this rare disease.     

A case of primary diffuse large B‐cell non‐Hodgkin's lymphoma misdiagnosed as chronic periapical periodontitis

Lymphoma is a malignant neoplasm of component cells of the lymphoid system which is very rare in the jaws. Here we report a case of primary diffuse large B‐cell lymphoma located in the periapical region of a mandibular molar which was misdiagnosed as chronic periapical periodontitis. The present case was diagnosed at an early stage and effectively managed by chemotherapy. Although lymphoma of the mandible is rare, it must be considered in the differential diagnosis of radiolucent lesions in this region. Lack of knowledge of this rare presentation may lead to delays in diagnosis and poor prognosis.     

Extraskeletal chondroma in the auricle

Extraskeletal chondroma is a rare benign tumor that develops in soft tissues, and histologically, it is a lobulated nodule surrounded by a fibrous capsule, and the inside consists of mature cartilage cells and diffuse hyalinized matrix. It occurs preferentially in the hand and foot area of adults, and the development in the head and neck area is very rare. We had a very rare case of extraskeletal chondroma that developed in the auricle and report the case together with a review of the literature.     

罕见病的牙颌面临床表现与治疗

罕见病是一类遗传性的、慢性的、不可治愈且发病率相对较低的疾病。由于其发病率低,相关医疗资源较为缺乏,对其诊断及治疗的研究也受到限制。近期国家五部委发布的《第一批罕见病目录》纳入了121种罕见病,第一次明确界定了我国罕见病的范围。临床资料显示一些罕见病常伴有牙颌面表现,为了实现对罕见病的早期诊断与治疗,本文对目录纳入的罕见病牙颌面临床表现及相关治疗手段进行总结,以期为口腔科医生提供临床诊断依据。     

Cystadenocarcinoma of salivary gland presenting as a cystic lesion in the mandible

Cystadenocarcinoma is a rare salivary neoplasm. It occurs in major and minor salivary glands and usually has a good prognosis. Mandibular involvement by salivary gland tumors at presentation is exceptionally rare. We present the first case, to our knowledge, of salivary gland cystadenocarcinoma appearing as a cystic lesion in the mandible.     

Rare earth screens for panoramic radiography

Six panoramic radiographs, each made with a different screen/film combination, were compared for "periapical visibility" by fourteen dentists. Two combinations (a par-speed and a high-speed) used calcium tungstate screens, and three used rare earth screen systems four times faster than par-speed. One other combination consisted of a single rare earth screen plus a single-coated detail film. The radiographs produced by the faster rare earth screen/film combinations were found to be similar in quality to those produced by calcium tungstate screens. Rare earth screens could be used for panoramic radiography while maintaining quality and reducing exposure.     

A rare case of an aggressive osteoblastoma of the squamous temporal bone: A unique presentation with literature review

Aggressive osteoblastoma is a rare osteoid tissue forming tumour commonly affecting the spine with predilection for the posterior elements. Calvarial involvement is extremely rare with only two reported cases in the literature. Due to its overlapping clinical, radiographic and histological features with ossifying fibroma, benign osteoblastoma and osteosarcoma, it is very difficult accurately to diagnose this lesion at an early stage. A rare case of an aggressive osteoblastoma of the squamous temporal bone in a young male is presented here which was misdiagnosed twice before reaching the final diagnosis by correlating clinical, radiographic and histopathological features.     

Acute myelofibrosis and multiple chloromas of the mandible and skin

Acute myelofibrosis is a rare clinical entity characterized by severe marrow fibrosis and peripheral blood pancytopenia. It may be very closely related to and has been considered synonymous with megakaryocytic leukemia. Chloroma is also a rare entity composed of a localized collection of immature myeloid cells and is associated with a known or covert leukemia. A patient presenting with a rare combination of acute myelofibrosis, megakaryocytic leukemia and multiple chloromas of the mandible and skin, are described. Although presenting a diagnostic challenge, their combination in one patient aids in the understanding of the myeloproliferative process. To our knowledge, this is the 3rd case of chloroma involving the oral osseous structures.     

Malformation in the primary and permanent dentitions following trauma prior to tooth eruption: a case report

Dento-facial injuries that occur prior to the eruption of teeth in the primary dentition are rare, but can result in damage to the primary dentition. We report a rare case where an injury to the anterior maxilla and mandible of an infant prior to primary tooth eruption resulted in hypoplasia, displacement and impaction of the primary dentition and damage to a developing permanent tooth.     

颌面部原发性皮肤间变大细胞淋巴瘤1例报告及文献复习

[提要]原发性CD30+间变性大细胞淋巴瘤是一种少见的非霍奇金淋巴瘤,该病主要好发于60岁左右,常发生于四肢和躯干,临床检查为质韧、边界清楚、结节状肿物.组织病理学检查显示皮下组织内有大量淋巴细胞,形态学结合免疫组织化学是诊断本病较为有效的方法.皮肤原发性病变临床少见,原发于颌面部者更为罕见.本文对临床上发现的1例原发...     

Rare oral cavity presentation of a B-cell lymphoblastic lymphoma. A case report and review of the literature

Lymphoblastic lymphoma is an uncommon malignancy, with most cases showing a T-cell phenotype and presenting as a mediastinal mass. By contrast, B-cell lymphoblastic lymphoma/leukemia is a rare high-grade malignancy that comprises approximately 10% of all lymphoblastic lymphomas. Lymphomas of the oral cavity are rare and typically present as intraosseous lesions that are most commonly diffuse large B-cell type. Here we present what we believe is the first B-cell lymphoblastic lymphoma initially presenting in the oral cavity. The case involves a 46-year-old white woman who presented with a mass in the right mandible. This report discusses this rare malignancy, including clinical presentation, histopathologic features, immunologic profile, treatment, and prognosis. This case emphasizes the importance of recognizing rare entities that may present in the oral cavity and the impact of the disease and its management.