颞骨朗格汉斯组织细胞增生症22例临床分析

目的 分析颞骨朗格汉斯组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床表现、诊断、治疗及预后.方法 回顾性分析我院15年来收治的22例颞骨LCH,研究其临床表现、诊断、治疗及预后,并随访1～15年,进行生存分析和无病生存率的Log-rank检验.结果 22例中1例为多系统高危组,3例为多系统低危组,18例为单系统组.临床表现以耳部为主,颞骨CT见大范围的溶骨性骨质破坏.22例患者的误诊率为72.7%(15/22).所有患者采用不同组合的综合治疗,随访1～15年,存活21例(95.5%),死亡1例(4.5%),后遗尿崩症、侏儒症、癫癎各1例,单耳听力严重下降2例,后遗症发生率22.7%(5/22),多系统组与单系统组无病生存曲线差异有统计学意义(x~2=5.87,P<0.05).结论 颞骨LCH多数累及单系统,以耳部临床表现为主,易误诊,治疗方案宜根据受累系统来选择,单系统组预后明显好于多系统组.     

Langerhans' cell histiocytosis of the temporal bone in children

OBJECTIVE: Langerhans' cell histiocytosis (LCH) is a rare pathology that implies an abnormal proliferation of these kinds of cells associated with a granular infiltration that affects different structures of the human body, including the temporal bone. The authors present their series of LCH of the temporal bone in children at the Sainte-Justine university hospital. The twofold objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare these results with previously reported series. METHODS: A retrospective study was conducted between 1984 and 2007 with patients diagnosed and treated for a LCH of the temporal bone at the Sainte-Justine university hospital, a paediatric tertiary care center. A chart review was performed to obtain demographic, clinical, paraclinical, and therapeutic data. They were analysed and compared to other published series. Through a MEDLINE query, we found that since 1966, 50 articles dealing with a LCH of the temporal bone have been published. RESULTS: Fifty-nine cases of LCH were diagnosed and among them, 10 children had temporal bone involvement. They were four females and six males with a mean age of 3.28 years. The two most frequent clinical manifestations were the presence of a mass in the temporal region (70%) and otitis (60%). Two of our patients had a unifocal lesion of the temporal bone implicated. Eight patients had a multisystem involvement among which, two showed evidence of organ dysfunction. In 80% of cases, the diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The common radiological finding on a skull CT scan is a lytic lesion in the temporal bone. Seven patients were treated by chemotherapy, two were treated by radiotherapy as a primary treatment, and one received radiotherapy for a recurrence on the pituitary gland. Finally, one patient was treated with local steroid injections. Two patients had a recurrence. All our patients were in total remission on a mean average of 1.6 years after the diagnosis. Our results concord with other studies in which the prognosis of unifocal bone disease is excellent and children with a multifocal disease have a survival rate of 65-100%. CONCLUSION: LCH is a rare disease. A high-index suspicion should be raised in the context of a temporal mass, chronic otitis, and otorrhea. A biopsy is recommended in the presence of a temporal bone lytic lesion. Chemotherapy is the preferred therapeutic modality.     

耳颞部朗格汉斯组织细胞增生症(附10例报告)

目的研究耳颞部朗格汉斯组织细胞增生症(LCH)的诊断和治疗。方法回顾分析10例经病理证实的耳颞部LCH病例。10例中男女各5例,年龄1~60岁,平均18.3岁。3例为韩-薛-柯病,7例为嗜酸性肉肿。临床主要表现为耳颞肿胀、颅骨缺损、耳漏、鼓膜穿孔、耳道肉芽、耳鸣、眩晕、耳聋、头痛、尿崩症等。结果8例接受手术加放疗后痊愈,2例接受手术后化疗治愈。遗留尿崩症、侏儒症各1例。结论LCH根据临床表现、影像学及组织病理学特征可做出诊断。手术、放疗和化疗是治疗LCH的有效疗法。尽管LCH病情危重,只要未累及重要生命器官、不误诊、及时合理治疗,一般预后良好,但其后遗尿崩症或侏儒症残疾则顽固难治。     

鼻及咽部树突细胞肿瘤4例临床分析并文献复习

目的：探讨树突细胞肿瘤的临床表现、病理特点、治疗和预后。方法：4例鼻及咽部树突细胞肿瘤患者中，滤泡状树突细胞肉瘤（FDCS）2例，朗格汉斯组织细胞增多症（LCH）1例，朗格汉斯细胞肉瘤（LCS）1例。2例FDCS中有1例行综合治疗（手术加放化疗），1例仅行放化疗；1例LCH及1例LCS患儿均行手术治疗。结果：2例FDCS患者，l例术后行4个疗程CHOP方案及同步放疗（50Gy）后达完全缓解，1例仅行放化疗者随访7个月仍带瘤生存；1例LCH患儿随访2年余，肿瘤无复发及转移；1例LCS患儿，术后未行放化疗，随访10个月后死亡。结论：树突细胞肿瘤罕见，临床易误诊，确诊需病理组织形态、免疫组织化学及电镜相结合，FDCS、LCH及LCS具有独特的病理表现、免疫表型、诊治及预后。     

Langerhans' cell histiocytosis: paediatric head and neck study

OBJECTIVE: This study presents the experience of the Montreal Children's Hospital (MCH) with Langerhans' cell histiocytosis (LCH) and reviews the new advances in diagnosis and therapy of this disorder. DESIGN: Retrospective study of 20 patients seen between July 1986 and July 1997 diagnosed with LCH. METHODS: All of the 20 charts were examined for variables including age, sex, area involved, treatment modalities, and complications. RESULT: Sixty-five percent of patients presented with localized lesions and 35% with multisystem involvement. The most common involved area was the skull, and 57% of skull lesions involved frontal bone. The temporal bone was involved in 25% of cases. The most common ear symptom was otorrhea. CONCLUSION: Langerhans' cell histiocytosis is a rare paediatric disorder. Head and neck involvement occurs frequently in both localized and multisystem disease. The prognosis is highly dependent on the age and number of systems involved.     

Multimodal treatment strategy for Langerhans cell histiocytosis at head and neck manifestations

Langerhans cell histiocytosis (LCH) is a rare disorder with different clinical features.An established standardized treatment does not exist.We present a case report of a patient with localized LCH of the temporal bone and discuss the interdisciplinary treatment strategies. We reviewed the international literature and summarized the current knowledge. Beside a wait and see policy in cases without symptomatic disease, surgery, radiotherapy or chemotherapy, and combinations of these options are used as treatment modalities.While surgery or radiotherapy are preferred in localized (symptomatic) lesions, stage-adapted chemotherapeutic regimens are the treatments of choice in disseminated disease. Treatment selection depends on the individual clinical features.     

Histiocytosis X

Histiocytosis X may be isolated or generalized in its clinical involvement. Spontaneous resolution may occur, although progression to diffuse systemic disease has also been found. The disease is believed to originate from the mononuclear phagocytic series of cells, although the reason for this activation remains unknown. A possible local reaction to an inciting agent in the nasopharynx or oral cavity has been suggested by the frequent head and neck manifestations in this disease. The prognosis and treatment of patients should be predicated on the staging or extent of disease. Local disease should be treated in a conservative manner. Low dose radiation therapy should be used for inaccessible lesions or lesions in which removal may produce serious morbidity. In the future, randomized treatment protocols need to be undertaken to clarify the status of chemotherapy and immunotherapy in patients with disseminated or aggressive disease.     

Bilateral cochlear implantation in bilateral Langerhans cell histiocytosis

Abstract

Background

Langerhans’ cell histiocytosis (LCH) is a rare proliferative disorder that can have otologic manifestations in up to 30% of patients. Treatment of local and systemic disease may include medical, surgical, and radiation therapies. Involvement of the temporal bone can lead to conductive and, rarely, sensorineural hearing loss. Post-labyrinthectomy cochlear implantation can be an effective treatment option for sensorineural hearing loss in the setting of persistent LCH.

Methods

A retrospective case review at a tertiary academic medical center. Hearing in Noise Test (HINT) performed before and after bilateral cochlear implantation was examined.

Results

Following bilateral partial labyrinthectomy, post-operative testing showed a HINT in quiet of 17%. Left-sided cochlear implant followed by immunosuppressive therapy for persistent disease showed marked improvement with post-operative HINT in a quiet room of 80% and 63% at 1 and 2 years. Fifty-five months after left implantation, and 10 months after right cochlear implantation, binaural post-operative HINT in quiet was 81%.

Conclusion

Surgical excision of LCH lesion remains a mainstay of treatment for temporal bone involvement. Bilateral cochlear implant with adjuvant immunosuppression in our patient demonstrated both immediate and delayed improvement in auditory function after staged cochlear implantation in the setting of persistent disease.     

Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis

Objective

Our review aims: (1) to highlight the suspicion of sensorineural hearing loss (SNHL) in temporal bone Langerhans’ cell histiocytosis (LCH); (2) to assess the evolution of SNHL in this pathology; (3) to identify radiologic findings of the otic capsule invasion by LCH; (4) to determine prognostic factors for SNHL in temporal bone LCH.

Methods

We performed a literature review through MEDLINE for SNHL in temporal bone LCH related articles that were published between 1954 and 2008. We identified 12 related studies of which 18 patients were noted. We also added our case to this series. The information from the reports was analyzed to characterize the clinical and demographic data and to focus on the sensorineural hearing aspect of the disease and on the damage of the semicircular canals (SCC) and the cochlea.

Results

Ten percent of patients with temporal bone LCH presented SNHL. The mean age of patients is 3.5 years among children and 35.5 years among adults. Male to female ratio is 1:1.14. There were 13 unilateral cases, 6 bilateral cases and 1 case of multisystemic histiocytosis. Cochlea and SSCs were involved in 4 and 20 temporal bones, respectively. The lateral SCC is the most frequently eroded canal. In 23 ears hearing level was reported: 15 ears had a SNHL and the remaining was a mixed or a conductive hearing loss type. 10 ears suffered from a profound hearing loss and none of them improved after treatment regardless otic capsule affected structures. In all cases of normal hearing, moderate hearing loss and severe hearing loss before treatment cochlea have not been affected. However a single or two semicircular canals invasions have been noted. Where it is reported (15 out of 25 temporal bone) there was a radiological healing of the otic capsule lesion after treatment. Remineralization occurs 6 months after treatment.

Conclusion

In LCH, hearing loss level before treatment can be considered as a prognostic indicator for hearing in response to treatment. Even though bone mineralization is essential for a hearing improvement, radiological healing is not a prognostic factor for better hearing level after treatment.     

Aural symptoms as primary presentation of Langerhan's cell histiocytosis

Langerhan's cell histiocytosis is an uncommon granulomatous disease, characterized by the idiopathic proliferation of Langerhan's cells or their marrow precursors. It encompasses the diseases previously associated with histiocytosis X-eosinophilic granuloma, Hand-Schuller-Christian syndrome and Letterer-Siwe syndrome. A series of 54 patients were diagnosed with this condition in Dublin over a 33-year-period (1959–1992). Twenty-seven patients had aural symptoms, of whom 15 had no other lesions at the time of presentation. Otorrhoea was the most frequent otological symptom, followed by lesions in the temporal bone. LCH may mimic common aural conditions such as otitis externa, otitis media or acute mastoiditis and a high index of suspicion is required to recognize it on clinical presentation. The clinical presentation of multi-focal disease may be so dramatic that the otologic findings may be initially overlooked. The mortality rate was 14.8%. Therapeutic regimes included no treatment, curettage, chemotherapy, radiotherapy or multi-modality treatment.     

外耳道腺样囊性癌24例临床分析

目的探讨外耳道腺样囊性癌的临床诊断和治疗策略。方法回顾性分析解放军总医院1996—2007年经治的24例外耳道腺样囊性癌患者的临床资料,并用Kaplan-Meier法计算生存率。结果全组24例患者中,行局限性外耳道肿物切除术3例,行部分骨性外耳道和软骨切除加乳突根治术11例,行颞骨次全切除术9例,颞骨全切除术1例。4例晚期患者腮腺因受侵而切除。23例患者术后加用辅助放疗。全组总的5年累计生存率为71-4％．10年累计生存率为38．1％;临床误诊的患者与未被误诊患者的10年生存率分别为21．9％和59-3％（P=0．012）。结论外耳道腺样囊性癌生长缓慢,但长期效果差,对肿瘤的扩大切除可能提高生存率、降低复发,手术加放疗是有效的综合治疗策略。     

Involvement of the maxillary sinus in Langerhans cell histiocytosis]

AIM: A clinical presentation of Langerhans cell histiocytosis (LCH) in the maxillary sinus of two patients is given. LCH in the maxillary sinus is a rare occurrence. Our aim was to compare the different treatment alternatives available and to suggest a classification as well as a therapeutic regime. PATIENTS AND METHOD: Records and clinical data of two patients treated between 1994 and 2001 were retrospectively evaluated. Both patients suffered from LCH in the maxillary sinus and the maxilla regions. Only surgical treatment was used. After resection, a large defect of the maxillary sinus, which did not allowing coverage, was seen in both cases. After reconstructive operations, closure was finally achieved. Both patients underwent follow-ups, whereby one suffered from a relapse after 15 months. RESULTS: Although one of the patients under investigation showed a recurrence of LCH, we are of the opinion that surgical treatment is very effective in the elimination of this condition. A proposal for a classification of LCH in the oral-maxillo-facial-region is made. CONCLUSIONS: The evaluation of our clinical study suggests that LCH is a disease that should be treated surgically. Only in very severe cases should the surgical treatment be complimented by either radiotherapy or chemotherapy. In disseminated cases, especially chemotherapy seems to improve the outcome. Surgery offers the possibility of eliminating systemic side effects.     

Cetuximab with radiotherapy as an alternative treatment for advanced squamous cell carcinoma of the temporal bone

The prognosis of advanced temporal bone cancer is poor, because complete surgical resection is difficult to achieve. Chemoradiotherapy is one of the available curative treatment options; however, its systemic effects on the patient restrict the use of this treatment. A 69-year-old female (who needed peritoneal dialysis) presented at our clinic with T4 left external auditory canal cancer and was treated with cetuximab plus radiotherapy (RT). The primary lesion showed complete response. The patient is currently alive with no evidence of disease two years after completion of the treatment and does not show any late toxicity. This is the first advanced temporal bone cancer patient treated with RT plus cetuximab. Cetuximab plus RT might be a treatment alternative for patients with advanced temporal bone cancer.     

Rhabdomyosarcoma of the ear and temporal bone

Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood, involves the temporal bone in approximately 7% of reported cases. Until recently, the outcome of this disease was always fatal. The recent Intergroup Rhabdomyosarcoma Study Group (IRS-I) reported on the efficacy of multimodality therapy consisting of multiagent chemotherapy, radiation, and surgical resection when indicated. Twelve patients with rhabdomyosarcoma involving the temporal bone were treated between 1966 and 1988. Three patients were treated according to the IRS-I protocols and the remaining nine patients received various combinations of treatment modalities. Ten patients succumbed to their disease, most with distant metastases or intracranial extension. Two patients are alive; one at 5 1/2 years and one at 19 years. It is apparent that, although survival for rhabdomyosarcoma in general has improved with the use of IRS-I protocols, prognosis remains poor for disease involving the temporal bone and other parameningeal sites.     

Langerhans' cell histiocytosis with bilateral temporal bone involvement

BACKGROUND: Langerhans' cell histiocytosis (LCH) is a rare disease with variable clinical appearance. The etiology of LCH remains unclear to date. It is currently believed that clonal accumulation and proliferation of CD1a-positive Langerhans' cells are causative. METHODS: A 2-year-old boy presented with hypacusis and disturbance of balance. Auditory brainstem-evoked responses (ABRs) revealed severe bilateral labyrinthine hearing loss. Magnetic resonance imaging (MRI) showed inflammatory changes with bone erosion in both temporal bones including labyrinthine systems and mastoid processes. RESULTS: During bilateral surgical exploration, fragile slightly yellowish tissue with necrotic areas was found that turned out to be LCH on histology. Chemotherapy with vinblastine and prednisone was subsequently initiated, leading to continuing complete remission. CONCLUSIONS: The initial presentation of LCH with bilateral temporal bone involvement is a very rare condition. The signs and symptoms of otologic histiocytosis can mimic those of acute and chronic infectious ear disease. Only a surgically obtained biopsy leads to definitive diagnosis and appropriate therapy.     

鼻咽癌放疗后颞骨放射性骨坏死的诊治

目的总结鼻咽癌放射性颞骨坏死的临床资料,为今后的诊断和治疗提供经验。方法根据骨坏死的范围是否局限,分为局限性和广泛性的放射性颞骨坏死,对1995～2002年期间收治的13例鼻咽癌放射性颞骨坏死的患者进行回顾性分析,9例局限性骨坏死采用手术清除死骨8例,对症治疗1例;4例广泛性骨坏死采用手术治疗3例,保守治疗1例。结果9例局限性骨坏死,经手术一次性治愈7例,1例再次手术治愈;1例保守治疗,经历4年后死骨脱落,无新死骨形成;4例广泛性骨坏死除了保守治疗1例死于鼻咽大出血外,另3例经手术治愈。结论手术治疗是有效的治疗手段,广泛性的骨坏死可引起危及生命的并发症。     

Eosinophilic granuloma of the temporal bone. Case report and literature review

BACKGROUND: Eosinophilic granuloma, Hand-Schüller-Christian disease an Letterer-Siwe disease are characterised by ideopathic proliferation of histiocytes producing focal or systemic manifestations. Definitive diagnosis of histiocytosis is made by histopathology including immunohistochemical detection of S-100 and CD1a antigens. In general these diseases are summarised under the term Langerhans-cell histiocytosis (LCH). The localised form of LCH, in which the disease is limited to bones, lymphatic nodes or the lung, is commonly referred to as eosinophilic granuloma. Surgical excision, radiotherapy and chemotherapy, either alone or in combination, are the main treatment options. CASE REPORT: We present the case of a nine-year old boy with an extended eosinophilic granuloma of left temporal bone. The patient was submitted to a chemotherapeutic protocol with glucocorticoids, vinblastine and etoposide. Until today a successful treatment and a complete remission for one year can be reported. CONCLUSIONS: Unifocal eosinophilic granuloma is usually treated by local excision and low-dose irradiation. However treatment with the chemotherapeutic protocol offered an excellent alternative avoiding extensive surgical destruction of the temporal bone in this case.     

The outcome of treatments for carcinoma of the external auditory canal

OBJECTIVE: A retrospective analysis of management and survival of patients treated for temporal bone carcinoma. PATIENTS AND METHODS: Thirty patients underwent treatment for carcinoma of the temporal bone. Twenty-five squamous cell carcinomas, 1 melanoma, 2 basocellular carcinomas and 2 adenoid cystic carcinomas were treated. Thirteen patients were treated before for the same disease. RESULTS: Staging revealed 12 T1 and T2, 6 T3 and 12 T4 tumours. The mean follow up was 5 years (2-276 months). The Kaplan Meier survival curves showed survival rates at 2 years of 82%, 67% and 32%, and at 5 years of 82%, 67% and 17%, respectively for the stages T1 or T2, T3 and T4. At the end of follow up at 9 years the survival rates were 66%, 66% and 17% for the stages T1 or T2, T3 and T4 respectively. Overall stages a complete remission was found in 65% and 23%, and deceased was 35% and 77%, respectively for the primary treatment group and the salvage surgery group. CONCLUSION: Long-term prognosis of the carcinoma of the external auditory canal mainly depends on the stage and primary treatment. Surgery (lateral temporal bone or subtotal temporal bone resection, both in combination with a neck dissection and a parotidectomy) and adjuvant radiotherapy is the treatment of choice for part of stage T1 and all T2 and T3 tumours. The improved survival (65%) of patients treated de novo compared with those treated with salvage surgery (23%) suggests that early referral and aggressive primary surgical treatment with postoperative radiotherapy offer the greatest chance of cure.     

Facial paralysis in temporal bone trauma]

OBJECTIVE: To study the cause and treatment of facial paralysis in temporal bone trauma. METHODS: A retrospective review of 28 patients with facial paralysis resulting from temporal bone trauma was conducted, House-Brackmann(H-B) system was used to evaluate the recovery of facial nerve function, U test was employed for statistic analysis. RESULTS: Twenty-six cases underwent following up facial nerve function of all patients was recovery partly after surgery, 46% cases reached H-B II, 84% cases reached H-B III, comparing facial nerve decompression within 4 months of injury to beyond 4 months of injury, recovery to H-B II or more of facial nerve function was 60% and 0%, respectively. The difference was significant(P < 0.05). CONCLUSION: Surgery decompression is effective on facial nerve palsy caused by temporal bone trauma. It is recommended that surgery decompression be carried out as soon as possible since a better prognosis is obtained if it is performed within four months after injury.     

Retrospective study of Langerhans cell histiocytosis in ear,nose and neck

ObjectiveLangerhans cell histiocytosis (LCH) is a rare clinical disorder. We retrospectively analysed the clinical manifestations, treatments and prognoses of LCH cases involving the ear, nose, and neck.Materials and methods28 cases with confirmed LCH in ear, nose or neck were reviewed. We recorded patient age, sex, chief complaints, accompanying symptoms, lesional sites, radiological data, treatments and pathologies. Whole-exome sequencing was performed on the patient diagnosed with LCH and Treacher-Collins syndrome (TCS).ResultsThe mean age was 14.86 years. Most LCH was in the ear (93%), usually in the mastoid. The most common symptoms were an ear mass and a purulent discharge. Imaging was not very useful. Treatments included surgery, chemotherapy, and radioactive particle implantation. Some cases exhibited multisystem involvement. Most patients enjoyed good prognoses. One patient was diagnosed with both temporal LCH and TCS. Whole-exome sequencing revealed a heterozygous c.261_272delAGGTACCCTTCC(p.87_91delRGTLPinsR) mutation in exon 2 of the POLR1D gene (NM_015972).ConclusionLCH mostly occurs in children. In head and neck it affects principally the mastoid part of the temporal bone. Treatments include surgery, chemotherapy, and irradiation. Most patients enjoy good prognoses. LCH accompanied by TCS is rare and increases the difficulty of diagnosis; molecular data aid in TCS identification.