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.     

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.     

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.     

Cervical lymphadenitis and cat scratch disease (CSD): an overlooked disease?

Cat Scratch Disease (CSD) is a benign disease characterized by regional lymphadenopathy affecting most frequently the head and neck region in children and young adults. In the present paper, the authors describe four cases of CSD focusing on clinical history, diagnostic management and therapy. The main germ responsible for this lymph node disease is Bartonella henselae. Diagnosis is based on history, serology and histological findings. Clinical evolution is generally favorable despite the fact that complications occur in about 5% to 13% of patients including encephalitis, hepatitis and Parinaud's oculoglandular syndrome. Antibiotic treatment is only considered for highly symptomatic patients. Surgical excision of the lymphadenopathy is useful to establish the diagnosis when serology is not available and/or when the adenopathy become fluctuating. The authors emphasize the increasing incidence of patients with CSD in the ENT population and the algorithm for CSD disease affecting the cervical lymph nodes.     

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.     

Pediatric Kikuchi-Fujimoto disease masquerading as a submandibular gland tumor

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a rare disorder that typically affects the cervical lymph nodes. The disease usually occurs in women in their late 20s or early 30s. Reports in the pediatric literature are sparse. Most authors consider Kikuchi-Fujimoto disease as a self-limiting disorder that requires no specific management but long-term follow-up. The clinical features of Kikuchi-Fujimoto disease are easily confused with other less-benign conditions. Thus, an early biopsy is instrumental in making definite diagnosis and preventing unnecessary investigations. We describe a case of Kikuchi-Fujimoto disease in an 8-year-old boy which presenting as a submandibular gland tumor. The case illustrates the clinical features of this unusual condition and emphasizes the potential confusion with other diagnoses.     

Kikuchi–Fujimoto and Kimura diseases: the selected, rare causes of neck lymphadenopathy

Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a benign and self-limiting disease typically characterized by the enlargement of regional lymph nodes and accompanied by fever. KFD affects predominantly young adult females of Asian origin and is rarely seen in European countries, where it may cause diagnostic difficulties. Kimura disease is a rare and benign chronic inflammatory soft tissue disorder of unknown origin, characterized by a triad of painless subcutaneous masses in the head or neck region accompanied by regional lymphadenopathy, blood and tissue eosinophilia, and markedly elevated serum immunoglobulin E levels. Although most cases of Kimura disease have originated in China, Japan or Southeast Asia, there have been sporadic case reports from Europe and America. Herein, we review in detail the clinical presentations, complications and current concepts in the pathogenesis, diagnosis and treatment of these diseases.     

Kikuchi-Fujimoto disease: a report of 3 cases

Cervical lymphadenopathy has many underlying etiologies. One of its rare causes is Kikuchi-Fujimoto disease (Kikuchi's disease, histiocytic necrotizing lymphadenitis). We discovered such a cause in a 37-year-old woman who had presented with malaise, night sweats, and weight loss in addition to cervical lymphadenopathy. We based our diagnosis on excisional lymph node biopsy. We also review 2 other cases of Kikuchi's disease that were diagnosed by others at our institution. Clinically and histologically, Kikuchi's disease is very similar to lymphoma, and distinguishing the two is difficult. However, despite the fact that Kikuchi's disease is benign, an accurate diagnosis is important because misdiagnosis might lead to unnecessary surgery and/or chemotherapy.     

A case of cervical progressive transformation of germinal center

Progressive transformation of the germinal center is an unexplained borderline disease that presents as lymphadenopathy. The histological feature is an abnormally enlarged germinal center. Reports of this disease in otolaryngology are rare. A case of progressive transformation of the germinal center occurring in the bilateral multiple submandibular lymph nodes with swelling is presented. A 41-year-old man complained of bilateral painless cervical lymphadenopathy. Because fine needle cytology of the lymph nodes did not reveal any atypical cells, medical observation was performed. However, as the patient suffered from hoarseness, he consulted our hospital again. An otolaryngeal examination revealed mild enlarged bilateral lymphadenopathy. Removal of these lymph nodes to obtain a definitive diagnosis was performed. The pathological diagnosis was progressive transformation of the germinal center (PTGC). Immunostaining of the lymph nodes showed increased IgG4+cells (IgG4/IgG ratio=50%). To date, no significant changes in the cervical lymph nodes have been noted on follow-up. Because PTGC is apt to be misdiagnosed as Hodgkin's lymphoma, otolaryngologists should have full knowledge of PTGC.     

Clinical study on papillary thyroid carcinoma presenting with lymph node metastasi

Papillary thyroid carcinoma (PTC) may metastasize to cervical lymph nodes. It is, however, uncommon for a palpable neck node alone to lead to the diagnosis of this disease when it is not apparent at presentation. Standard treatment for such cases has not yet been established. We retrospectively analyzed clinical courses in 8 patients with thyroid papillary carcinoma presenting with palpable lymph node metastasis at Hokkaido University Hospital between 1990 and 2003. Three had high thyrogloblin in cervical cystic lesions, leading to the diagnosis of PTC with lymph node metastasis. In 4, PTC was diagnosed by pathological examination of cervical lymph nodes initially diagnosed as lateral cervical cysts. Preoperative examination did not indicate PTC within the gland in any case. All 8 were alive at the last visit after follow-up from 23 to 150 months (mean: 78 months). Total thyroidectomy was done on 4 and thyroid lobectomy on 3. Pathological examination of resected thyroid glands confirmed multifocal papillary carcinoma from 4 mm to 15 mm in diameter. Six underwent unilateral neck dissection and 1 chose bilateral dissection. The other patient received no additional surgery on either the thyroid or neck after the single enlarged lymph node initially diagnosed as a lateral cervical cyst was resected. Postoperative radioiodine treatment was done in 2 undergoing total thyroidectomy. Recurrence in the cervical area were observed in 1 whose neck dissection was insufficient. Based on these observations, we concluded that patients who undergo thyroid lobectomy and adequate neck dissection may enjoy longer survival than those treated with total thyroidectomy without sacrificing thyroid and parathyroid function. We therefore propose a prospective study on the effectiveness of thyroid lobectomy with neck dissection including positive nodes in patients with occult PTC presenting with lymph node metastasis.     

Malignant ameloblastoma: classification, diagnostic, and therapeutic challenges.

Ameloblastoma is a benign odontogenic neoplasm of the mandible and maxilla that rarely exhibits malignant behavior. We report the case of an aggressive malignant ameloblastoma of the mandible that presented with an unusual multiphasic, histologic pattern. Initial fine needle aspiration and radiographic findings showed features consistent with a benign, fibro-osseous lesion. However, aggressive growth and the association of enlarged submandibular lymph nodes suggested a more malignant potential. Treatment consisted of an angle-to-angle composite mandibular resection, right modified neck dissection, left functional supraomohyoid neck dissection, and anterior chin skin resection with iliac crest osteocutaneous free flap reconstruction. Microscopic evaluation showed primarily malignant ameloblastoma without cellular atypia and extensive fields of fibro-osseous tissue with smaller fields of clear cell odontogenic tumor. This multiphasic, histologic arrangement may explain the perplexing preoperative microscopic diagnosis, suggesting a benign fibro-osseous lesion. Of the lymph nodes analyzed, one from the right submandibular triangle exhibited metastatic, benign-appearing ameloblastoma without fibro-osseous or clear cell features. The absence of cellular features of malignancy in the tumor mass and lymph node metastasis suggest that the lesion should be classified as malignant ameloblastoma rather than ameloblastic carcinoma or odontogenic carcinoma. A malignant ameloblastoma with all 3 of the aforementioned microscopic features has not been previously reported. We review the classification of epithelial odontogenic malignancies. Lesions showing multiphasic patterns can create diagnostic dilemmas and may require extensive surgical sampling and/or removal to establish an accurate diagnosis.     

Quantification of lymph nodes in selective neck dissection

OBJECTIVES: Provide reference for surgeon and pathologist regarding expected yield from selective neck dissections. Quantify lymph nodes obtained from cadaver dissection based on current nodal classification and compare with clinical series. STUDY DESIGN: 1. Quantification of lymph nodes at levels I-V harvested from human cadavers and correlation with nodal grouping for supraomohyoid (I-III) and lateral (II-IV) neck dissections. 2. Retrospective review of operative specimens from clinical neck dissections for lymph node quantity. METHODS: 1. Twenty radical neck dissection specimens, harvested from 10 fresh human cadavers without evidence of head and neck cancer, were separated by nodal level for gross and microscopic examination by a pathologist. The quantity of nodes obtained per level for each specimen was tabulated. 2. Charts of patients treated with neck dissection for squamous cell carcinoma were reviewed and tabulated for type of dissection and number of lymph nodes reported. RESULTS: In the 20 cadaver neck dissections, the average number of lymph nodes removed for levels I-V was 24, with 13 for levels I-III and 19 for levels II-IV. In the clinical review, 98 total neck dissections were included. In the six supraomohyoid dissections, an average of 20 lymph nodes (range, 14-26) were found, with an average of 30 (range, 15-43) in the 11 lateral compartment specimens. In 81 radical or modified radical dissections, an average of 31 nodes (range, 19-63) was reported. CONCLUSIONS: The number of lymph nodes removed in selective neck dissection should be comparable to that of the corresponding levels in radical neck dissection, provided that strict adherence to surgical boundaries is maintained. Dissection of normal cadavers provides a reference for the surgeon and the pathologist but may under-represent lymph node quantity in the diseased state.     

Ultrasound evaluation of characteristics of cervical lymph nodes with special reference to color Doppler ultrasound. A contribution to differentiating reactive from metastatic lymph node involvement in the neck

BACKGROUND: Cervical mass due to lymphadenopathy is a common cause for consultation of an ENT specialist by patients. In many cases exact differentiation without biopsy between reactive and metastatic lymphnodes is difficult but crucial and necessary for each patient. Ultrasound is the imaging system with the highest sensitivity for the evaluation of pathological lymph nodes. However, differentiating benign and malignant lesions remains a problem. PATIENTS: In a prospective study, 138 cervical lymph nodes of 62 patients were evaluated according to conventional ultrasound criteria such as size, shape, brightness, demarcation, etc., and according to parameters of color doppler sonography such as intensity and localization of perfusion. The so called Pourcelot or resistance index, an objective parameter, was measured in order to examine a possible improvement of specificity in differential diagnosis of both entities. All lymph nodes were surgically removed and histologically examined after ultrasonography. RESULTS: 133 lymph nodes were evaluated in the study. Lymphadenitis was demonstrated in 72 cases, whereas 61 of the lumps showed metastases of squamous cell carcinoma of the head and neck region. Three patients with primary malignant lymphoma were excluded from the study. The conventional ultrasound parameters such as size, homogenity, shape and brightness did not reveal any substantial difference between the two groups. However, lymph node metastases significantly showed higher Doppler signals than the reactive ones. Most of the metastases were perfused in the periphery or had a diffuse spread of blood flow. The most valuable parameter from the prognostical point of view proved to be the Pourcelot Index with a threshold value less than 0.6 for metastases, which increased the specificity to 92% with a probability of p = 0.001. CONCLUSIONS: The results of this study demonstrate an increase of the ultrasound specificity in differentiation of pathological cervical lymph nodes using color flow imaging. Unfortunately, this method does not enable the physician to correctly diagnose the findings in all patients. Therefore histological evaluation is mandatory in all doubtful cases.     

Akute Toxoplasmose bei koexistentem Non-Hodgkin-Lymphom

There are many reasons for cervical lymph node enlargement. In particular, the large group of infectious diseases must be considered along with malignant diseases. The coexistence of an uncommon infectious disease with malignant disease is a rare event. We report the case of an otherwise healthy 69-year-old man with marked enlargement of his cervical lymph nodes. A diagnosis of a recent toxoplasmosis infection was made based on positive IgG and IgM toxoplasma titers and the results of fine-needle aspiration from a lymph node. Since the enlarged lymph nodes persisted for more than weeks, the lymph node was excised. Histological examination revealed a non-Hodgkin's lymphoma. IgM titers in toxoplasmosis can persist up to 1 year. In cases with rare infectious diseases like toxoplasmosis in immunocompromised patients, swollen lymph nodes that persist or grow should lead to the suspicion of additional disease. A diagnosis can be confirmed by removing a lymph node for histology.     

Incidence and significance of clinically unsuspected thyroid tissue in lymph nodes found during neck dissection in head and neck carcinoma patients

OBJECTIVES/HYPOTHESIS: The objectives were to quantify the incidence of clinically unsuspected thyroid tissue in cervical lymph nodes encountered during neck dissection in patients with head and neck carcinoma, to describe the location and histological aspect of these inclusions, and to assess their clinical significance. STUDY DESIGN: Retrospective study. METHODS: The histological records of 1123 neck dissections in 752 patients with head and neck carcinoma were reviewed. In cases with thyroid inclusions, the pathological diagnosis was reviewed and an immunohistochemical study against thyroglobulin and calcitonin was carried out. RESULTS: Clinically unsuspected thyroid tissue was found in lymph nodes in 11 of the 752 patients with head and neck carcinoma treated with neck dissection. In five cases, the thyroid inclusion was compatible with a metastases of an occult papillary thyroid carcinoma. In the other six cases, a collection of thyroid follicles without malignant characteristics was found beneath the lymph node capsule. These latter cases were considered benign thyroid inclusions. A thyroidectomy was performed in three of the patients with lymph node metastases of the papillary carcinoma. An occult papillary carcinoma was found in only one case. The other two patients had been treated previously with radiotherapy for an early-stage glottic carcinoma. Immunohistochemical study did not find calcitonin-positive cells within the benign thyroid inclusions. After a follow-up period ranging from 1.2 to 8.2 years, no patient had any kind of local, regional, or distant relapse related to the thyroid disease. CONCLUSION: The incidence of unsuspected thyroid tissue in lymph nodes of patients with head and neck carcinoma treated with neck dissection was 1.5%. Both lymph node metastases of a papillary carcinoma and benign thyroid inclusions were found. The study results suggest that the incidental finding of thyroid tissue in the lymph nodes during a neck dissection in patients with head and neck carcinoma does not necessarily indicate the need for aggressive therapy.     

Tularemia in the differential diagnosis of cervical lymph node enlargement. An outbreak of tularemia in Castilla-León, Spain

The presence of enlarged cervical lymph nodes is a diagnostic challenge in disease of the head and neck. Lymph node enlargement may be secondary to local or general infectious disease, to non-infectious systemic disease, or to lymphatic metastasis of tumoral processes. Among the many infectious processes that originate cervical lymph nodes is tularemia. This disease is uncommon in Spain, but was unusually frequent in Castilla-León in the last months of 1997, with 136 cases diagnosed in our hospital, 13 accompanied by lymph node enlargement of the head and neck. This article shows that tularemia should be considered in the differential diagnosis of cervical lymph node enlargement in our region.     

Acquired immune deficiency syndrome--related lymphadenopathies presenting in the salivary gland lymph nodes

Homosexual males at risk for acquired immune deficiency syndrome (AIDS) frequently present with peripheral lymphadenopathies that precede the opportunistic infections and neoplasias of this disease. Two homosexual males had enlarged salivary glands due to lymphadenopathies initially affecting intraparotid and perisubmaxillary gland lymph nodes. Both the hyperplastic and the atrophic histologic patterns of AIDS-related lymphadenopathies were observed in the lymph nodes. Subsequently, the two patients developed lymphoma and opportunistic infections, respectively. The AIDS-related lymphadenopathy developing in salivary gland lymph nodes is an entity that should be considered in the differential diagnosis of salivary gland tumors.     

A 5-year study of patients with neck lymphadenopathy

The purpose of this study was to investigate neck lymphadenopathy patients in our hospital, and to investigate items requiring attention on the occasion of examination of these patients. In this study, 134 patients with neck lymphadenopathy in the five years from April 2005 to March 2010 were included. The kind of diseases, the period of suffering (the period from onset to consultation), relationship with pain, radiological examination, fine needle aspiration cytology and lymph node biopsy findings were examined. Of 134 patients, the disease was inflammatory in 109 patients (81.3%) and malignant in 25 patients (18.7%). The suffering period was longer in the malignant group than in the inflammatory group. Furthermore, the inflammatory group had more patients with neck lymph node pain than the malignant group, and the group with the short suffering period had more patients with neck lymph node pain than that with the long suffering period. Fine needle aspiration cytology was performed in 36 patients (26.9%), and finally, all of the seven patients with class III were diagnosed as having malignant disease. A neck lymph node biopsy was performed in 38 patients (28.4%), and four of 38 patients were diagnosed as having metastatic carcinoma. Two patients in the inflammatory group and two patients in the malignant group took more than 90 days to reach a definite diagnosis. Many kind of diseases cause neck lymphadenopathy, and, therefore, it is important to perform a neck lymph node biopsy immediately, if it is difficult to establish a diagnosis.     

Angiolymphoide Hyperplasie mit Eosinophilie (Morbus Kimura)

Summary This disease is discussed in the past mostly in the dermatological literature, the ENT specialist is little aware of its existence. The condition is characterized by subcutaneous, painless nodules occurring most frequently in the region of the ear, parotid gland, cheek and lateral neck. The lesion must be differentiated from diseases of the lymph nodes, especially tumor metastases. The pathologic diagnosis is based upon a hyperplasia of angiolymphatic tissue and marked tissue eosinophilia. The prognosis appears to be favourable. A case is presented together with the clinical picture and histological results including electronmicroscopic finding. The differential diagnosis and treatment are discussed.     

Screening for lymph nodes in the neck with ultrasound

In a study of the value of ultrasound in staging patients with head and neck malignancies, we performed ultrasound of the neck. The results of this investigation were compared with palpation. A fine needle aspiration biopsy and/or histologic examination was carried out on lymph nodes which were found. One hundred and six patients were included in this study. In 44 of the patients no lymph nodes could be detected, either on palpation or by ultrasound examination. In the other 62 patients all palpable lymph nodes were also demonstrated by ultrasound. However, in 20 patients with negative palpatory findings, ultrasound revealed lymph nodes: 11 metastases and 9 benign nodes. In 40 patients an ultrasound guided fine needle aspiration biopsy (UGFNAB) was performed. In 85% of these patients a cytological diagnosis could be made. From these results we conclude that ultrasound and UGFNAB are of considerable value in staging head and neck malignancies.