A case of osseous giant cell tumor of the temporal bone and tympanum

The giant-cell tumor (osteoclastoma) is very rare in the head and neck. Its histogenesis, diagnosis and classification are difficult. Although it is considered to be benign, it should be regarded by the clinician as malignant, because of its high recurrence rate, potential metastases and the difficulty of total removal. We performed an extended radical mastoidectomy in a 42-year-old woman with such a tumor of the petrous bone, primarily to prevent such complications.     

颈动脉体瘤和颈静脉球体瘤的手术治疗

目的：探讨颈静脉球体瘤和颈动脉体瘤的临床特性，手术要点。方法：单纯颈动脉体瘤者采用胸锁乳突肌前缘切口。颈静脉球体瘤及同侧同时患有颈动脉体瘤者，一律采用大“S”形切口，显露颈静脉孔和岩尖的颞下进路。结果：5例颈动脉体瘤患者，瘤体均彻底切除且完好保留了颈内动脉。5例颈静脉球体瘤患者3例肿瘤切除彻底，2例有意姑息切除瘤体，经1～3年随访，未见残余肿瘤增大。结论：化学感受器瘤具有一定的家族性。手术切除肿瘤是最主要的治疗方法。     

Osteoclastoma of the petrous temporal bone.

This paper has described an unusual case of osteoclastoma of the petrous temporal bone which presented with multiple cranial neuropathies. A radical course of radiotherapy reversed the neuropathies but it is too early to comment on possible cure.     

Laryngeal solitary fibrous tumor treated with CO<Subscript>2</Subscript> laser excision: case report

The solitary fibrous tumor is an uncommon unilateral benign lesion that usually arises from the pleura. Recently, these tumors have been described in many other locations, such as the mediastinum, head and neck, orbit and urogenital system. To date, only two cases of solitary fibrous tumor arising from the larynx have been reported in the scientific literature. We describe a new case of laryngeal solitary fibrous tumor localized at the right false vocal fold. A 29-year-old man presented with a 6-month history of progressive hoarseness and foreign body sensation in the throat. A mass lesion was recognized in the right false vocal fold, and CT scan demonstrated a large, well-defined tumor without signs of infiltration. The tumor was removed by transoral CO₂ laser surgery. The definitive diagnosis was obtained by immunohistochemical analysis that showed vimentin and CD34-positive cells. The patient remains free of tumor after 15 months of follow-up.     

Undifferentiated sarcoma of the sphenoid sinus

Paranasal sinuses sarcomas are rare and no treatments have been established. We report a young-adult case of sphenoid sinus sarcoma treated by carbon-ion radiotherapy. The patient presented with progressive left-sided visual impairment. A tumor was then identified and partial resection by transnasal approach was performed. The resected mass showed typical morphology of mesenchymal tumor, and morphological and molecular analyses ruled out a predominant-differentiation phenotype. The pathological diagnosis was undifferentiated sarcoma. The residual lesion was treated with carbon-ion radiotherapy, and tumor progression was absent for one year. The patient died of the tumor regrowth 20 months after initial diagnosis. Although this case had a poorer outcome compared with cases of the more-common sarcoma types, our experience suggested that carbon-ion radiotherapy is potentially beneficial in unresectable undifferentiated sarcomas cases of sphenoid sinus.     

Osteoclastoma of temporal bone

A 20 year male presented with a gradually increasing painless swelling at right temporal region with progressive hearing loss conductive in type. On plain X-ray-a wide radio translucent area of bone destruction was noted. The mass was excised as much as possible via post-auricular incision extending antiriorly toward the zygomatic root. Histopathological examination showed the features suggestive of osteoclastoma. Post-operative radiation was administered to deal with the remaining mass there was no recurrence of growth even     

颈静脉球瘤10例诊疗分析

目的分析探讨颈静脉球瘤临床特点，总结手术疗效，提高该病诊治经验。方法回顾性分析10例经数字减影血管造影和/或术后病理证实的颈静脉球瘤患者的临床资料，总结该病的临床特征、影像学特点、围手术期处理、手术方式及随访效果。结果所有患者均为单耳发病，其中男2例，女8例。表现为单侧搏动性耳鸣6例，听力下降8例，耳痛及眩晕3例，耳流脓性或脓血性分泌物4例，因体检发现耳道肿物就诊1例。6例因肿物突入外耳道，鼓膜不能窥及，4例可透过鼓膜看见鼓室内红色搏动，鼓膜稍向外膨隆。纯音测听示传导性聋4例，混合性聋5例，感音神经性聋1例。所有患者均行颞骨CT及MRI检查，鼓室球瘤可见鼓室、外耳道内充填不规则软组织密度影；颈静脉球体瘤可见颈静脉孔区软组织密度影，周围呈“蚕蚀样”骨质破坏，肿物突向鼓室及外耳道，增强扫描可见明显强化。3例颈静脉球体瘤行DSA检查可见颈静脉孔区浓密肿瘤染色，2例由咽升动脉供血，1例由咽升动脉和枕动脉供血。6例鼓室球瘤患者均行耳道乳突径路鼓室球瘤切除术，3例颈静脉球体瘤患者行颞下窝A型径路颈静脉球体瘤切除术，1例颈静脉球体瘤患者行伽玛刀治疗。所有病例随访10个月至5.5年，均无复发，未出现影响生活的严重并发症。结论颈静脉球瘤有特征性的临床表现，以单侧搏动性耳鸣和不同程度的听力下降最为多见，结合仔细的专科查体和完善的影响学检查不难诊断。鼓室球瘤患者通过中耳显微手术能达到良好疗效，颈静脉球体瘤患者因解剖位置特殊，对术者及围手术期的规范化处理要求较高，并且要求诊疗团队具有侧颅底外科手术的知识及经验，否则容易引起严重并发症。     

儿童颈段原发性气管恶性肿瘤的临床表现和治疗

目的：探讨儿童颈段原发性气管恶性肿瘤的临床表现和治疗方法。方法：对1998年以来经纤维喉镜、CT扫描、组织病理学诊断的5例原发性气管恶性肿瘤患儿的诊治进行回顾性分析。4例患儿于肿瘤下方行紧急气管切开,1例气管插管后气管切开,用气管镜和鼻内镜联合摘除气管内肿瘤;术后4例接受放疗,1例激光加放疗。结果：5例患儿随访3～8年无复发。结论：儿童气管恶性肿瘤,影像检查是有效的诊断手段,就诊时病情危重,宜行低位气管切开,逆行鼻内镜及硬管气管镜联合下彻底摘除肿瘤,术后辅以放疗可获得良好效果。儿童患者一般不宜行有创的气管壁切除术。     

Middle ear adenoma with neuroendocrine differentiation (MEA-ND) in the pediatric population

Middle ear adenoma with neuroendocrine differentiation is an uncommon tumor of the tympanic cavity. The terminology of this entity has suffered due to a poor understanding of the differentiation of these neoplasms, and has included both “adenoma” as well as “carcinoid tumor.” Immunohistochemical techniques have helped clarify that these tumors all share elements of neuroendocrine differentiation. Occurrences of this tumor in the pediatric population are extremely rare. In the world literature, only three pediatric cases of middle ear adenoma with neuroendocrine differentiation have been described. We report the youngest case to date of middle ear adenoma with neuroendocrine differentiation, in a 13-year-old boy. We discuss the differential diagnosis, surgical management, and pathology of this entity.     

Primary tumor volume of nasopharyngeal carcinoma: significance for survival

OBJECTIVES: To elucidate the effect of primary tumor volume (PTV) on treatment outcomes in patients with nasopharyngeal carcinoma (NPC) who were treated with radiotherapy or concurrent chemoradiotherapy. The TNM staging system developed by American Joint Committee of Cancer (AJCC) is universally used and accepted but its prediction of prognosis in NPC receives a lot of challenge. Primary tumor volume had been reported to have close relationship with prognosis of head and neck cancer. We may predict prognosis of NPC with PTV. METHODS: From 1999 to 2006, 91 patients with newly diagnosed NPC who were treated with radiotherapy or CCRT were enrolled in the study. Computed tomography-derived or magnetic resonance-derived primary tumor volume was calculated. The correlation between AJCC disease stage, primary tumor volume and disease-specific survival were analyzed. Multivariate analyses using the Cox proportional hazard model was performed. RESULTS: The median primary tumor volume for the whole series was 11.39 ml (range 1.25-166.58 ml). The median primary tumor volume was 2.69 ml in T1 disease, 10.14 ml in T2 disease, 15.41 ml in T3 disease, and 26.69 ml in T4 disease. Hazard ratio increased with tumor volume, ranging from 5.91 (95% confidence interval (95% CI) 1.13-26.43) for tumor volumes between 20 ml and 40 ml, and 15.72 (95% CI, 3.82-61.05) for tumor volumes >40 ml. With both tumor volume and T classification in the same Cox regression model, only tumor volume remained statistically significant in the prognosis of NPC. CONCLUSION: Primary tumor volume has closer relationship with survival rates of patients with NPC. Calculation of primary tumor volume may be further considered to improve the current staging system.     

Osteoclast-type giant cell neoplasms of the parotid gland

The parotid gland is added to the list of parenchymal organs, notably the pancreas, in which osteoclast-like cells appear as constituent cells in their neoplasms. The cells' role in the neoplasms is a reactive one or, more rarely, as an integral element in an osteoclast-type giant cell neoplasm or so-called osteoclastoma. Distinctive in histological appearance, the osteoclast-type giant cell neoplasm is a malignant lesion that, to date, has been described only in the pancreas and parotid glands. This report presents examples of each type of giant cell lesion in the parotid gland.     

Two cases of myxoma of the external auditory canal

Myxomas were found in the outer ear canal of a 66-year-old female and a 51-year-old male. Both tumors were soft and sessile and located at the antero-superior part of the external auditory canal near the eardrum. Profuse, clear mucus was seen when the tumor ruptured or was incised. Histology showed spindle-shaped and stellate cells in myxoid extracellular matrix. CT images revealed a small bone defect adjacent to the tumor in the meatal bone. This bony defect was confirmed during surgery. The bony defects seen in both cases may have something to do with the genesis of the tumor. We found only 5 sporadic cases, including ours, reported in the English literature. As myxoma of the outer ear canal is also reported in 18 patients as a symptom of Carney's complex, we must check for accompanying symptoms of the complex such as spotty skin pigmentation, endocrine tumors, or schwannoma. Total removal of the tumor is necessary to prevent recurrence.     

Multiple epuli in a newborn: A case report

Congenital epulis is a rare granular cell tumor that arises from the gingival mucosa. The cause of this lesion, first described by Neumann in 1871, remains unknown. Since that time, only case reports of the benign tumor have been published. The incidence is estimated at 0.0006%, of which 10% are multiple epuli. We describe a female infant with multiple large epuli arising from both alveolar ridges who was successfully treated surgically within the first 48 h of life. We discuss considerations for airway management and describe her postoperative course.     

Parapharyngeal and skull base yolk sac tumor: A case report with lessons in diagnosis and management

Yolk sac tumors are rare in the head and neck. A previously healthy 2-year-old female presented with a large parapharyngeal mass. Pathology was pathognomonic for yolk sac tumor, with glandular differentiation and focal mucin production, which has not been reported in a yolk sac tumor. She was treated aggressively with chemotherapy followed by endoscopic exploration with planned resection, but no viable tumor was encountered. Yolk sac tumors can be difficult to diagnose in the head and neck, but complete clinical response can be achieved. New endoscopic approaches to skull base tumors are applicable to the pediatric population with some technical modifications.     

p53 mutation in histologically normal mucosa of the aero-digestive tract is not a marker of increased risk for second primary carcinoma in head and neck cancer patients

Head and neck cancer patients are at high risk for developing second primary tumors. This is known as field cancerization of the aero-digestive tract. In a previous study, we showed that patients with multiple primary tumors were more likely to have p53 mutations in histologically normal mucosae than patients presenting with an isolated tumor. Based on this observation, we postulated that p53 mutations in normal tissue samples of patients bearing a single primary tumor could have a clinical value as a biomarker for the risk of developing second primary tumors. Thirty-five patients presenting with a single primary tumor were followed-up for a median of 51 months (range 1 month to 10.9 years) after biopsies of histologically normal squamous cell mucosa had been analyzed for p53 mutations with a yeast functional assay at the time of the primary tumor. During this follow-up, recurrences and non-sterilization of the primary tumor, occurrence of lymph node metastases, and of second primary tumors were evaluated. Sixteen (45.7%) patients were found to have p53 mutations in their normal squamous cell mucosa, and 19 (54.3%) patients showed no mutation. No relationship was found between p53 mutations and the occurrence of evaluated events during follow-up. Notably, the rate of second primary tumors was not associated with p53 mutations in the normal squamous mucosa. The correlation between p53 mutations in histologically normal mucosae and the incidence of second primary tumors is generally low. The benefit of analyzing p53 mutations in samples of normal squamous cell mucosa in every patient with a primary tumor of the head and neck is doubtful.     

Ethmoid tumor and oncogenic osteomalacia: Case report and review of the literature

IntroductionOncogenic osteomalacia is a very rare disease usually caused by a phosphaturic mesenchymal tumor, particularly the “mixed connective tissue type”, secreting FGF-23 hormone.ObjectiveThe authors report a case of ethmoid tumor associated with oncogenic osteomalacia and discuss management based on a review of the literature.Case summaryA 41-year-old woman with multiple fractures causing major disability was diagnosed with early-onset osteoporosis. CT scan followed by MRI, performed due to the concomitant presence of nasal obstruction, showed a right ethmoid tumor in contact with the dura mater and periorbital tissues, but with no signs of invasion. Endoscopic resection was performed with reconstruction of the defect of the cribriform plate by a nasoseptal flap. Nasal and bone symptoms subsequently resolved. Histological examination revealed a phosphaturic mesenchymal tumor.DiscussionTwelve cases of mesenchymal tumor of the ethmoid sinus associated with oncogenic osteomalacia have been reported to date. FGF-23 assay and whole-body MRI with STIR sequence are useful for the diagnosis. A very favorable outcome is observed after surgical treatment in the majority of cases.     

Giant cell tumor of hard palate

The giant cell tumors usually present in epiphysis of long bones. These are uncommon tumors in head and neck region. These tumors have benign historical features but can be aggressive locally and even can metastasise to lungs. This rare tumor in hard palate is being reported here which was excised transpalatly.     

中耳耵聍腺肿瘤3例并文献复习

目的 探讨中耳耵聍腺肿瘤的临床病理特点、诊治方法及预后情况.方法 回顾分析确诊为不同类型耵聍腺肿瘤3例患者的临床资料,加以总结.结果 随访至2014年12月,其中1例中耳耵聍腺瘤患者术后6年,未见复发及转移;1例中耳耵聍腺癌患者经手术及放疗后(20 Gy)1年复发,再次手术及放疗,2年后出现面瘫怀疑肿瘤复发,因患者放弃治疗,未能明确,但随访6年仍存活;1例腺样囊腺癌患者行2次手术及P53治疗1年后发生颌下转移,再次手术及放疗,随访9个月后,影像学提示肿瘤复发并转移至颅内,未再治疗,至随访结束时仍存活.结论 中耳耵聍腺肿瘤的早期诊断困难,易复发,其确诊有赖于临床特点、影像学和组织病理学检查,其治疗方式及预后与肿瘤类型相关,耵聍腺瘤以手术治疗为主,耵聍腺癌及腺样囊性癌应采用以手术治疗为主的综合治疗.     

Typical carcinoid tumor arising in the nose and paranasal sinuses—Case report

Carcinoid tumors arise from neuroendocrine cells, many of which are present in the digestive tract and lungs. There have been few reports of carcinoid tumors occurring in the nose and paranasal sinus area, and they are very rare. We encountered a patient with a carcinoid tumor that arose in the nose and paranasal sinuses, and we report the case with a review of the literature. The patient was a 75-year-old woman who began to experience right-sided nasal obstruction, and when her nose began to bleed on the right-side she was examined in our department. A tumor lesion that easily bled and had filled the right nasal cavity was observed. CT revealed a mass lesion with a marked contrast enhancement in the right nasal cavity, ethmoid sinus, and sphenoid sinus, and MRI showed numerous flow voids in the interior that seemed to be tumor blood vessels. The tumor was excised through a lateral rhinotomy. The histopathological diagnosis was a carcinoid tumor. Tumor recurrence was subsequently detected in the vicinity of the opening of the sphenoid sinus, and because the tumor was tending to grow larger, the tumor was resected. The patient has been followed up in the outpatient clinic, recurrence-free.     

Peripheral neck nerve tumor: A 73-case study and literature review

ObjectivesPeripheral neck nerve tumors are rare and mostly benign neoplasms. The exceptional malignant forms are very aggressive, and diagnosis is difficult. The objective of this study was to evaluate diagnostic and therapeutic management and identify possible predictive factors.Material and methodsA retrospective study was conducted of 73 patients treated for peripheral neck nerve tumor between 1995 and 2015.ResultsMean age was 44 years. The main presenting symptom was a cervical mass, isolated or associated with signs related to the affected nerve structure. Diagnosis was suspected by slow progression of a firm mass, featuring T1 hyposignal and T2 hypersignal on magnetic resonance imaging. Surgery was performed in 99% of cases, completed by adjuvant chemotherapy in case of malignant neuroblastic tumor. Type 1 neurofibromatosis and sudden increase in mass with or without associated pain suggested malignant transformation. Age below 10 years suggested neuroblastic tumor.ConclusionNeck nerve tumors are very often benign with low degenerative potential. Surgery is the treatment of choice after risk/benefit analysis. However, there is no clearly defined consensus regarding the timing of surgery for these lesions.