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.     

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.     

Simultaneous metastasized primary unknown signet ring cell carcinoma of the cervical lymph node and mucoepidermoid carcinoma of the parotid gland as double cancers

Lymph node metastasis from signet ring cellcarcinoma (SRCC) primary unknown is extremely rare. We here report a case of primary-unknown SRCC that metastasized to the cervical lymph nodes, co-existing with mucoepidermoid carcinoma (MEC) of the parotid gland as a simultaneous double cancer. A 68-year-old female patient with right swollen cervical lymph nodes consulted our medical center. A diagnosis of bilateral cervical lymph node metastasis and a right parotid tumor was made. After bilateral neck dissection and right parotidectomy, the pathological diagnosis was SRCC of primary unknown with metastasis to the cervical lymph node and MEC of the parotid gland. Examination of the CRTC1/3-MAML2 fusion gene showed no relation between SRCC of primary unknown with metastasis to the cervical lymph node and MEC of the parotid gland. Ten months after the first treatment, there was recurrence in the left neck lymph node, and left neck dissection was performed. Fourteen months after the first treatment, the patient is alive and cancer-free. This case is the fourth report of SRCC with lymph node metastasis, and highlights the value of fusion gene detection to determine relatedness between simultaneous cancers. Moreover, such cases should be closely monitored for the subsequent appearance of distant metastases.     

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.     

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.     

Clinical study of 28 cases of cervical lymph node metastasis from an unknown primary carcinoma

From 1989 to 2005, 28 patients--20 men and 8 women--with cervical lymph node metastasis from an unknown primary carcinoma were treated and studied retrospectively. In histological diagnosis, open biopsy was conducted in 11 patients and non-open biopsy (FNA or frozen section diagnosis during surgery) in 17. Blind biopsy under general anesthesia was conducted in 10 patients, showing one primary tumor in the nasopharynx. Tonsillectomy for diagnosis was not done. In region of maximum-size lymph node metastasis, the upper cervical region accounted for 22 cases (79%). The N stage of cervical lymph nodes was as follows: N2a in 4, N2b in 14, N2c in 3, and N3 in 7. The histopathological diagnosis of cervical lymph node was as follows: squamous cell carcinoma in 21, adenocarcinoma in 3, mucoepidermoid carcinoma in 2, and others in 2. Therapy was as follows: only neck dissection in 7, neck dissection with postoperative radiation therapy in 13, and irradiation and chemotherapy in 8. All patients treated with irradiation and chemotherapy had been judged to be inoperable. Seven patients were found to have a subsequent primary tumor. Primary tumor sites were as follows: tonsils in 3 and upper gingiva, base of tongue, lung, and nasopharynx in 1 each. FDG-PET was conducted in 7 patients but revealed no primary tumor. Overall 5-year survival in this study was 46%. We should pay particular attention to the tonsils for detecting primary tumors in patients with cervical metastasis from an unknown primary carcinoma.     

A case of follicular carcinoma of thyroid gland with concurrent tuberculous lymphadenitises

We report a case of follicular carcinoma of the thyroid gland with concurrent tuberculous lymphadenitises as neck lymph node metastases of thyroid carcinoma. A 71-year-old woman presented with multiple painless masses in the thyroid gland and painless lymphadenopathies in the right neck. She and her family had no previous history of tuberculosis. A diagnosis of thyroid cancer with lymph node metastases was made, and the patient underwent total thyroidectomy with neck dissection. Lymph nodes were hard and severely adhered to the internal jugular vein. The histopathological diagnosis was follicular carcinoma and multiple nodes of adenomatous goiter of the thyroid gland, and tuberculous lymphadenitises of lymph nodes in the right neck. There was no findings of coexisting pulmonary tuberculosis. The possibility of coexisting tuberculous lymphadenitis must thus be ruled out when we find painless lymph node swelling in aged patients with head and neck cancer including thyroid cancer.     

Sinus histiocytosis with massive lymphadenopathy (rosai-dorfman disease): Report of a case with respiratory tract involvement

The term “sinus histiocytosis with massive lymphadenopathy,” introduced in 1969 by Rosai and Dorfman, refers to a newly recognized disease entity characterized by painless cervical lymphadenopathy, fever, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. The typical course is one of insidious onset, protracted duration of the active disease state, and eventual spontaneous remission, occasionally with subsequent recurrences. Lymph nodes other than those in the cervical area may be involved, and extranodal involvement can occur (such as in the orbit, skin, or respiratory tract). Clinically, this entity may closely simulate malignant lymphoma or nonneoplastic conditions with lymphadenopathy. We present a case in which this disease process extensively involved the respiratory tract and produced obstructive symptoms. The otorhinolaryngologist should be aware of this disease entity and should include it in the differential diagnosis for patients with cervical lymph node enlargement or bronchial obstruction.     

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.     

Cervical lymphadenopathy in children--incidence and diagnostic management

OBJECTIVE: Palpable lymph nodes are common due to the reactive hyperplasia of lymphatic tissue mainly connected with local inflammatory process. Differential diagnosis of persistent nodular change on the neck is different in children, due to higher incidence of congenital abnormalities and infectious diseases and relative rarity of malignancies in that age group. The aim of our study was to analyse the most common causes of childhood cervical lymphadenopathy and determine of management guidelines on the basis of clinical examination and ultrasonographic evaluation. MATERIAL AND METHODS: The research covered 87 children with cervical lymphadenopathy. Age, gender and accompanying diseases of the patients were assessed. All the patients were diagnosed radiologically on the basis of ultrasonographic evaluation. RESULTS: Reactive inflammatory changes of bacterial origin were observed in 50 children (57.5%). Fever was the most common general symptom accompanying lymphadenopathy and was observed in 21 cases (24.1%). The ultrasonographic evaluation revealed oval-shaped lymph nodes with the domination of long axis in 78 patients (89.66%). The proper width of hilus and their proper vascularization were observed in 75 children (86.2%). Some additional clinical and laboratory tests were needed in the patients with abnormal sonographic image. CONCLUSIONS: Ultrasonographic imaging is extremely helpful in diagnostics, differentiation and following the treatment of childhood lymphadenopathy. Failure of regression after 4-6 weeks might be an indication for a diagnostic biopsy.     

Rosai Dorfman syndrome — A rare clinical entity

Sinus histiocytosis with massive lymphadenopathy (also known as Rosai Dorfman syndrome) is a unique disease of unknown etiology with a childhood predilection. It is an uncommon benign condition, often confused with lymphoma. Patients usually present with massive, painless, bilateral cervical lymphadenopathy and clinical course is variable. This condition has been sporadically reported in Indian literature. Here we report a 26-year-old women presenting with a painless bilateral cervical lymphadenopathy. A FNAC from cervical lymph node was compatible with Rosai Dorfman syndrome and confirmed histopathologically.     

Rosai-Dorfman syndrome. On the differential diagnosis of lymph node swelling]

The Rosai-Dorfman syndrome is a benign disease, one of its commonest affected sites being the cervical lymph nodes. This disease should be considered in the differential diagnosis of a cervical lymphadenopathy. The syndrome often begins with a chronic rhinitis, and is accompanied by sinusitis, tonsillitis or pharyngitis; blood tests indicate chronic infection. The pathogenesis of the disease is still unknown but may include infection or an abnormal autoimmune reaction. The diagnosis is established by the findings of characteristic histiocytes with haemophagocytosis. We report a female patient with persistent cervical lymph node swelling resistant to treatment. The cause was proved by histology to be a Rosai-Dorfman syndrome.     

Histiocytic necrotizing lymphadenitis (Kikuchi's disease)

Histiocytic necrotizing lymphandenitis (HNL), or Kikuchi's disease, is a newly recognized disease of unknown origin that causes cervical lymphadenitis, usually in young women. Initially described in Japan, cases of HNL are being reported in the United States and other western countries with increasing frequency. Symptoms of HNL include tender cervical adenopathy, fever, weight loss, and night sweats. Laboratory studies reveal leukopenia with relative granulocytopenia and lymphocytosis. Lymph node biopsy reveals areas with frank cellular necrosis, karyorrhexis, and absence of plasma cells. The histologic features of HNL are distinctive but can be confused with those of lymphoma. We describe two cases of HNL and present recommendations for diagnosis and treatment.     

Silicone lymphadenopathy: an unexpected cause of neck lumps

Objective: To report a rare case of silicone lymphadenopathy solely affecting the left supraclavicular lymph nodes. Case report: Our patient presented with a painless swelling in the left supraclavicular region. Notably, she had previously undergone cosmetic breast augmentation using silicone-containing implants. Radiological imaging and subsequent excisional biopsy of the swelling produced findings consistent with a silicone foreign body reaction secondary to bilateral breast implant rupture. Conclusion: Silicone lymphadenopathy following breast augmentation primarily affects the axillary nodes. Supraclavicular lymph node involvement is unusual. To our knowledge, this is the first report in the English language literature of silicone lymphadenopathy manifesting solely in the supraclavicular lymph nodes. Although the need to exclude malignancy in such cases is of the utmost importance, silicone lymphadenopathy should also be considered in the differential diagnosis. Fine needle aspiration cytology is a useful initial investigation, which may be followed up by excisional biopsy and histological analysis for further confirmatory diagnostic information.     

Thyroid tuberculosis diagnosed as papillary thyroid carcinoma with fever of unknown origin

Thyroid tuberculosis is a rare disease, very few cases have been reported. It is difficult to diagnose because of no typical characteristics. We report on a patient who underwent surgery for suspected thyroid carcinoma, but who was then diagnosed with thyroid tuberculosis. The patient was a woman in her 70s. She had been diagnosed with chronic renal failure and had been on peritoneal dialysis. She complained of fever and a painful left anterior neck swelling. Computed tomography showed thyroid tumor with cervical lymph node swelling, ultrasound-guided fine needle aspiration cytology was suspected for papillary thyroid carcinoma. We performed surgery to confirm the diagnosis and determine treatment. Procedures for thyroid carcinoma were followed, including left lobectomy of the thyroid gland, central lymph node dissection and right cervical lymph node resection. Pathological examination found no malignant findings in the thyroid tissue but did find a granulation layer even in the right cervical lymph node. Tuberculosis-specific IFN-γ assay was positive, we diagnosed thyroid and cervical lymph node tuberculosis. Postoperatively, the neck pain and fever improved, she was treated as an outpatient with antituberculosis drugs therapy. Thyroid tuberculosis must be considered in patients with immunocompromised, such as this patient, who was on peritoneal dialysis.     

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.     

组织细胞坏死性淋巴结炎11例临床分析

目的：探讨组织细胞坏死性淋巴结炎的临床特点。方法：回顾性分析11例组织细胞坏死性淋巴结炎的临床表现、淋巴结活检病理学特点及其诊治。结果：所有患者均有颈部淋巴结肿大和持续性发热,大多数患者白细胞减少、血沉增快。11例患者均由颈部淋巴结活检确诊。淋巴结活检病理学特点为不同程度的凝固性坏死伴多种形态的组织细胞、淋巴细胞浸润,无中性粒细胞浸润。应用疗程为2～4个月的肾上腺皮质激素治疗,疗效显著,未复发。结论：组织细胞坏死性淋巴结炎的临床表现无特异性,较易误诊,确诊主要依靠淋巴结活检。     

Secondary syphilis of the tonsil

Summary An 18-year-old man with an ulceration of his right tonsil and cervical lymphadenopathy was examined. The patient also had a papular eruption in his face, chest, abdomen, and upper extremities. There were no signs of genital involvement. Routine serological tests for syphilis were reactive and histological findings of a cervical lymph node biopsy were characteristic for syphilitic lymphadenitis. Spirochetes were also identified in the tissue sections. A diagnosis of secondary syphilis of the tonsil with cervical syphilitic lymphadenitis was made. In recent years, the number of new cases of syphilis has increased in Japan. Since the oral cavity is the most common extragenital site of syphilis, clinicians should bear in mind that oral lesions from an unknown cause might possibly be syphilitic.     

甲状腺乳头状癌颈部淋巴结转移规律的临床分析

目的 探讨甲状腺乳头状癌颈淋巴结转移规律,为选择最佳手术方式提供参考。方法 回顾分析2001年1月至2013年8月济南军区总医院甲状腺乳腺外科616例诊断为甲状腺乳头状癌患者的临床和病理资料。结果 616例有完整统计资料的甲状腺乳头状癌患者中,病理诊断颈部淋巴结总转移率为58.77%(362/616),中央区(Ⅵ区)淋巴结转移率为48.70%(300/616),颈侧区(Ⅱ、Ⅲ、Ⅳ、Ⅴ区)淋巴结转移率为38.80%(239/616),原发肿瘤的部位、最大直径、是否累及包膜、是否为多发病灶及患者年龄对颈部淋巴结转移率有显著影响,差异有统计学意义(P＜0.05),且双因素方差分析显示,肿瘤直径越大,转移到颈侧区的可能性越大。结论 甲状腺乳头状癌最常见的转移部位是Ⅵ区,术中应常规清除,其次依次为Ⅲ、Ⅳ、Ⅱ、Ⅴ区,对于患者原发肿瘤具有累及包膜、直径＞1cm、多发病灶及位于双侧等特点应清扫颈侧区,术中快速病理检查颈侧区淋巴结病理状态,确定颈侧区淋巴结清扫范围。     

甲状腺乳头状癌合并颈部局灶型Castleman病1例

摘要：目的探讨甲状腺乳头状癌(PTC)合并颈部局灶型Castleman病 (UCD)的临床诊治经验。方法回顾性分析1 例PTC合并颈部UCD的临床病例资料,并检索国内外文献。该患者术前行甲状腺结节穿刺细胞学检查(FNA)倾向PTC,合并右颈侧区淋巴结肿大,行甲状腺右侧腺叶合并峡部切除及右颈II、III、IV、VI区淋巴结清扫术。结果术后切口愈合良好,无声音嘶哑及低钙症状,术后 4 d治愈出院。术后随访6个月无复发。术后病理证实颈侧区肿大淋巴结为UCD。结论PTC合并UCD极为罕见,且UCD的术前诊断较为困难,但手术切除多可治愈;PTC合并颈部淋巴结肿大者,临床医生不仅需考虑甲状腺癌转移淋巴结可能,还需警惕UCD的存在。