Intraparotid Kikuchi-Fujimoto disease masquerading as a parotid gland tumor

The Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a self-limiting lesion of unknown cause first described in 1972 independently by Kikuchi and Fujimoto et al. This self-limiting disorder usually occurs in Asian women in their late 20s or early 30s. Typically, it runs a benign course and appears to resolve spontaneously 1 to 6 months after definite diagnosis. The Kikuchi-Fujimoto disease usually manifests as a localized cervical lymphadenopathy; therefore, most patients with this disease are seen in ears, nose, and throat practice. Nodal involvement other than in the neck area or extranodal involvement is rare. In this situation, however, the Kikuchi-Fujimoto disease is easily confused with other less-benign conditions. We describe a case of Kikuchi-Fujimoto disease in a 30-year-old man that presented as a parotid gland tumor. This is the third study to document intraparotid Kikuchi-Fujimoto disease in the English literature. Our report illustrates the clinical features of this unusual condition and emphasizes potential confusion with other diagnoses.     

Necrotizing lymphadenitis (Kikuchi-Fujimoto disease) as a rare cause of cervical lymphadenopathies. Diagnosis and differential diagnosis

BACKGROUND: The ENT specialist is often confronted with the inquiry as to the cause of lymph node enlargements. Common causes include inflammations, neoplastic, or autoimmune diseases. We report on three cases of Kikuchi-Fujimoto disease as a benign self-limiting lymphadenopathy of unknown etiology, which is usually found in young women between 20 and 30 years of age. Main symptoms are indolent or light tender, enlarged lymph nodes in the neck area. The correct diagnosis requires the histologic examination of the lymph node. Even for the experienced pathologist the differential diagnosis of malignant systemic disorders can be difficult. PATIENTS: Three female patients suffering from Kikuchi-Fujimoto disease were treated in our department between September 1997 and March 1998. RESULTS: All patients showed indolent and enlarged lymph nodes in the neck. In one case we were able to diagnose an acute cytomegalovirus infection; in another case an acute Epstein-Barr virus infection. After 3-5 months the symptoms disappeared spontaneously in all patients. In two of the three cases, histologic lymph node examination revealed T-cell lymphoma and Hodgkin's disease and in one case led to initial chemotherapeutic treatment. As a result of a second histologic examination, both diagnoses were reviewed and Kikuchi-Fujimoto disease was diagnosed. Chemotherapy was discontinued. CONCLUSIONS: Kikuchi-Fujimoto disease is a benign, self-limiting lymphadenopathy which is usually diagnosed in young women. Distinguishing Kikuchi-Fujimoto disease from malignant systemic disorders may even be difficult for pathologists using histological examination techniques and requires close cooperation between the ENT specialist and the pathologist. In order to minimize the risk of misdiagnosis and incorrect treatment, the ENT specialist should inform the pathologist about the differential diagnosis of Kikuchi-Fujimoto disease in such cases.     

Cervical lymphadenopathy secondary to Kikuchi-Fujimoto disease in a child: case report

While most cases of cervical lymphadenopathy resolve with conservative management, persistent adenopathy can lead to costly investigations and invasive treatments. One cause of persistent adenopathy, as well as a variety of other associated systemic symptoms, is Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis). This rare entity can be diagnosed only by excisional biopsy on the basis of its characteristic histologic appearance. We describe a case of persistent cervical lymphadenopathy secondary to Kikuchi-Fujimoto disease in an 11-year-old girl.     

Endolymph magnetic resonance imaging: Contribution of saccule and utricle analysis in the management of patients with sensorineural ear disorders

Endolymphatic hydrops features excess endolymph in the membranous labyrinth, and is a marker of Menière's disease. Between the early 1980s and late 2000s, MRI in Menière's disease aimed purely to rule out tumor or malformation as differential diagnoses for the pressure disorder. Progress in high-resolution MRI, however, now enables excess endolymph to be visualized in the membranous labyrinth, differentiating saccule and utricle in Menière's disease and in other clinical presentations such as cochleovestibular schwannoma. More recently, non-visibility of the saccule was demonstrated in a subgroup of Menière's disease patients, and utricle atelectasis in case of uni- or bilateral vestibular areflexia. Endolymph quantification remains highly controversial in terms of grading approach, but a simple semiology based on excess or deficient visualization of endolymph according to the compartment sheds light on the pathophysiological mechanisms of cochleovestibular disorder and may in future allow effective monitoring of medical and surgical treatment.     

Kikuchi-Fujimoto disease mimicking thyroid metastasis

INTRODUCTION: Kikuchi-Fujimoto lymphadenopathy is a subacute necrotizing lymphadenopathy of unknown origin. It usually affects cervical lymph nodes. Lymph node biopsy is needed for establishing the diagnosis. This disease must be considered as a possible cause of lymphadenopathy of unknown origin, certainly if classical clinical features lead to diagnostic confusion. OBSERVATION: A 41-year-old female presented a thyroid cancer treated by thyroidectomy associated with administration of I131. She also presented a right cervical metastasis treated by surgery. She had a cervical right cervical lymphadenopathy, hypermetabolic on the FDG scan, treated by a modified neck dissection. Microscopy of the surgical specimen showed necroziting lymphadenitis. DISCUSSION: Kikuchi-Fujimoto lymphadenopathy is a subacute necroziting lymphadenopathy of unknown origin, involved more commonly cervical lymph nodes unilaterally. It is more common in Asia, predominantly in young women. This lesion has been reported in patients of virtually any age or gender. No specific biological findings have been described. Lymph node biopsy is needed for establishing the diagnosis. The spontaneous course is usually favorable but one fatal case has been reported.     

Kikuchi-Fujimoto disease: Report of 4 cases and review of the literature

IntroductionKikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign disease of unknown origin predominantly affecting young women and presenting in the form of cervical lymphadenopathy and/or prolonged fever.Material and methodsThe authors report 4 cases of Kikuchi-Fujimoto disease diagnosed in the Internal Medicine Department of Ibn Sina university hospital in Rabat between 2009 and 2010.ResultsThese 4 women with a mean age of 27 ± 8.6 years [16–37] were admitted with febrile syndrome and cervical lymphadenopathy. The diagnosis was based on histological examination of a lymph node biopsy. The disease was associated with systemic lupus erythematosus in one case and actinomycosis in another case. A favourable course was observed in response to corticosteroid therapy in two patients, antibiotic therapy in one patient and antipyretic treatment alone in the fourth patient.ConclusionIn the light of these four cases, the authors discuss the diagnostic difficulties, the modalities of treatment of Kikuchi-Fujimoto disease and its clinical course.     

Kikuchi's disease: a case report and review of the literature

Kikuchi's disease is a necrotizing lymphadenitis that is prevalent in Asia and is being increasingly recognized in other areas of the world. It usually occurs in women in their late 20s or early 30s and manifests as a posterior cervical adenopathy. It resolves spontaneously, usually over a period of several weeks to 6 months. Its initial clinical appearance is commonly similar to that of a lymphoma, and it can be pathologically misdiagnosed as such. Kikuchi's disease might be associated with systemic lupus erythematosus. We report a case of Kikuchi's disease that occurred in a 36-year-old Asian woman. We discuss the clinical features, differential diagnosis, radiographic evaluation, and pathology of this case, and we review the literature in an effort to assist otolaryngologists in diagnosing this benign and uncommon entity.     

Late recovery with cyclosporine-A of an auto-immune sudden sensorineural hearing loss

Sensorineural hearing loss of immune-mediated origin may be present as a symptom in systemic autoimmune diseases or may occur as a primary disorder without other organ involvement (auto-immune inner ear disease). The diagnosis of auto-immune inner ear disease is still predicated on clinical features and to date specific diagnostic tests are not available. We report a case of right-sided sudden hearing loss in a female patient in which the clinical manifestations, in addition to ANA positivity and hypocomplementaemia allowed us to hypothesize an auto-immune inner ear disease. The immunosuppressive treatment with cyclosporine-A was capable of a recovery of the hearing that, however, occurred progressively with normalization of the hearing function after 1 year of treatment. cyclosporine-A could be proposed as a therapeutic option in case of auto-immune inner ear disease allowing the suspension of corticosteroids that, at high dose, expose patients to potentially serious adverse events.     

Kikuchi-Fujimoto disease: report of two cases

Kikuchi-Fujimoto disease, described for the first time in 1972, is an unusual diagnosis that is characterized by lymph node enlargement and fever. Cervical lymph nodes are affected so often that ENT specialists should be aware of this entity during differential diagnosis. The diagnosis was done by histological features, necrotizing lymphadenitis. The disease is self-limited and it has good prognosis. We have reviewed two cases in our hospital. We report an aseptic meningoencephalitis as a rare complication.     

Alleinige Kondylusresektion versus alloplastische Rekonstruktion

Castleman’s disease, also called angiofollicular lymph node hyperplasia or benign giant lymphoma, is a rare lymphoproliferative disorder of unknown etiology. Three histologic subtypes are described – hyaline vascular (80–90%), plasma cell (10–20%), and mixed (rare). In the clinical setting, localized and multicentric entities are distinguished. Due to the lack of tumor-specific clinical, biochemical, and radiological features, final diagnosis of Castleman’s disease depends on histopathology. Surgical tumor resection is the treatment of choice for localized disease. Prognosis is good, and adjuvant therapy is not required. Therefore, early invasive removal and histopathological differentiation from neoplasia is mandatory. In contrast, the prognosis for multicentric Castleman’s disease remains poor even if multimodal treatment regimens are employed. Major clinical symptoms, histology, and therapy are summarized, and the disease characteristics are highlighted presenting the case of an 11-year-old girl. On admission, the patient complained of a painless pharyngeal tumor mass and ipsilateral lymph node swelling. Magnetic resonance imaging revealed a parapharyngeal contrast-enhancing lesion extending from the hypopharynx to the skull base without signs of infiltration and accompanied by ipsilateral lymph node hyperplasia of the neck. Laboratory test results were within normal limits. After tumor resection, histopathological examination, and clinical staging, localized Castleman’s disease was diagnosed (hyaline vascular subtype).     

Bilateral acoustic neurofibromatosis (neurofibromatosis 2): a disorder distinct from von Recklinghausen's neurofibromatosis (neurofibromatosis 1)

A case report of bilateral acoustic neurofibromatosis (NF-2) is presented with temporal bone histopathology. The distinct clinical and genetic features of NF-2 are reviewed in the context of recent data that show it to be a disorder distinct from classic von Recklinghausen's disease (NF-1).     

Castleman disease presenting in the neck: Report of a case and review of the literature

Castleman disease is a rare lymphoproliferative disorder with two primary subtypes that vary in presentation and course. Unicentric Castleman disease (UCD) presents as a solitary mass, most commonly in the mediastinum, and rarely in the head and neck. In contrast to multicentric Castleman disease (MCD), which features peripheral lymphadenopathy and numerous systemic symptoms, UCD is not typically associated with generalized symptoms. Here, we present an unusual case of UCD presenting as a slowly expanding, isolated neck mass in a 29-year-old woman. This case demonstrates the distinguishing clinical, radiologic, and histologic findings unique to UCD, which is often misdiagnosed as lymphoma or other malignant process. These findings stand in contrast to those observed in MCD patients, and hence, offer insight into the practical aspects of diagnosis and management of Castleman disease in the head and neck.     

Linear IgA disease presenting as desquamative gingivitis: a pattern poorly recognized in medicine

Linear IgA disease is a subepidermal blistering disorder of unknown cause. Along with dermatologic blistering, other manifestations include oral ulceration and desquamative gingivitis. We present a case of linear IgA disease that manifested simply as oropharyngeal ulceration and desquamative gingivitis, resulting in considerable delay in diagnosis and treatment. This article (1) highlights the difficulties of diagnosing linear IgA disease when the predominant presenting features are mucosal and (2) reviews the current literature regarding linear IgA disease.     

Kikuchi-Fujimoto disease: histiocytic necrotising lymphadenitis

BACKGROUND: Kikuchi-Fujimoto disease or necrotizing histiocyte lymphadenitis is a disease of unknown cause whose main clinical feature is adenopathy, generally in a cervical location. The disease was described for the first time in Japan in 1972 and the first case in France was reported in 1986. Clinicians should be aware of this uncommon disease in order to differentiate it from infectious adenitis, lymphoma and collagenosis.CASE REPORT: A 21-year-old woman consulted the emergency physician in January 2002 for a right cervical mass which had developed rapidly with no associated symptom. Other smaller masses developed within the following days. Laboratory results demonstrated discrete inflammation. All serologies tested were normal. Diagnostic cervicotomy was performed. The histological diagnosis was necrotizing adenitis.DISCUSSION: Necrotizing histiocyte lymphadenitis is generally observed in young women in their twenties. There has been no predominant ethnic background. Laboratory tests are normal excepting for discrete signs of inflammation. Diagnosis is provided by pathology and immunohistochemistry. The clinical course is generally spontaneously favorable in six months. Relapse is uncommon.     

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.     

Temporal Bone Myeloid Sarcoma

Myeloid sarcoma is a rare condition that''s caused by the aggregation of immature myeloid cells in leukemic patients. Myeloid sarcoma occurring in the temporal bone more frequently involves the mastoid bone than is the case for metastatic lesions arising from non-systemic malignancies. The disease is difficult to diagnose when it presents with symptoms that mimic otomastoiditis. However, an early diagnosis is important in order to achieve complete remission of the disease. Magnetic resonance imaging of the temporal bone is useful for making the diagnosis of myeloid sarcoma, and especially to evaluate the extent of disease. High-dose radio- or chemotherapies are the first-line approaches and possibly the only approaches to achieve complete remission and to cure the disease. With the aim of improving our understanding of myeloid sarcoma in the temporal bone, the present report describes our experience with 5 such cases and we compare the clinical features of these 5 patients with those clinical features of patients who have metastatic lesions.     

Orbital cellulitis or lymphoma? A diagnostic challenge

We present a case report of an aggressive natural killer T cell lymphoma in a police officer who presented with combined features of orbital cellulitis and mid-facial destruction. However, his initial diagnosis was confused with other disease conditions that had overlapping features. This emphasizes the significance of clinical alertness and adequate tissue sampling; this can have a great impact on early diagnosis and treatment.     

Velopharyngeal insufficiency as the initial manifestation of a Myotonic Dystrophy type 1: A case report

Velopharyngeal insufficiency (VPI) is a disorder affecting nasal resonance during speech. The most common cause of VPI is a cleft palate. However, VPI can occur as a consequence of a neuromuscular or neurologic disorder without a palatal malformation. Myotonic Dystrophy type 1 is a hereditary muscular disorder. In these cases, the abnormality is located on the 19q13.3 region, including a myotonic dystrophy protein kinase CTG repeat. This disease has several clinical manifestation features depending of the number of CTG repeats. The myotonic dystrophy can affect several muscle groups. VPI associated with the involvement of the velopharyngeal muscles, has been reported as the main clinical feature in some cases. The purpose of this paper is to present a case of VPI as the main clinical manifestation in a patient with a Myotonic Dystrophy type 1.     

Clinical and morphological findings in Tangier disease (hypo-alpha-lipoporteinaemia) and its ENT-significance (author's transl)]

The report deals with the first three cases of Tangier disease (Hypo-alpha-Lipoproteinaemia) observed in Germany. This rare metabolic disorder is distinguished by a diminution of HDL-Lipoproteins in serum and a lipid storage in histiocytes. The prominent symptom is the orange yellow discolouration of hyperplastic tonsills and adenoids; further symptoms of the disease generally are various, the development is little typical. The morphological substrat of the deranged fat metabolism is cholesteryl ester storage in the reticuloendothelial system, smooth muscle cells, pericytes and Schwann cells of peripheral nerves. M. Tangier can be differentiated from other lysosomal defects by ultrastructural and chemical parameters, it seems to have a autosomal recessive inheritance. It is the ENT-specialist, who can establish an early diagnosis of this disease, provided he is aware of its clinical features.     

内耳Mondini畸形临床分析

目的探讨内耳Mondini畸形的病因、临床特征、诊断和治疗。方法分析2例Mondini畸形患者的临床资料并复习相关文献。结果 Mondini畸形属于常染色体显性遗传病;主要表现为听力下降,形成脑脊液漏后可伴发反复发作的脑膜炎,单侧畸形者,因对侧听力正常,常就诊于其他科室易漏诊;其诊断需结合病史、听力学及影像学检查;手术修补对脑脊液漏效果好,本文2例术后随访1年余,均未见复发。结论听力下降并反复发作不明原因的脑脊液漏伴脑膜炎者应疑为内耳Mondini畸形,CT与MRI检查是确诊该病的主要依据,对伴发脑脊液耳漏的患者,鼓室探查修补术是目前唯一有效的方法。