Characteristics of 18F-FDG PET/CT in patients with Kimura's disease from China.

BACKGROUND: 'Kimura's disease (KD) is a rare chronic inflammatory disorder of unknown etiology and is difficult to diagnose due to poor clinical presentation and imaging features. Few studies on characteristics of 18F-FDG PET/CT of KD have been reported. This study aimed to observe the reliable characteristics and usefulness of 18F-FDG PET/CT for the evaluation of consecutive patients with KD. METHODS: The clinical data and 18F-FDG PET/CT imaging findings of 8 patients with pathologically confirmed KD were reviewed retrospectively.18F-FDG PET/CT images were evaluated visually and semiquantitatively by measuring the maximum standardized uptake value (SUVmax). The correlations between clinical data and 18F-FDG PET/CT features were analyzed by simple linear regression. RESULTS: This study included 7 males and one female ranging in age from 17 to 79 years. The longest diameter of lesions ranged from 0.8 cm to 4.8 cm, and regional or generalized lymphadenopathy was found in all 8 patients with eosinophilia, while subcutaneous masses and salivary gland involvement concurrently were found in 4 patients. 18F-FDG PET/CT revealed that these involved lesions had high 18F-FDG uptake with SUVmax > 2.5 (2.6 to 6.3). Moreover, the margin of the lesions was well defined in 6 cases and ill defined in 2 cases, and homogeneous density and 18F-FDG uptake were both found in all these lesions. There was negative correlation between eosinophils and SUVmax (R2 = 0.538). CONCLUSIONS: Kimura's disease should be considered when 18F-FDG PET/CT is characterized by homogeneous lesions of regional or generalized lymphadenopathy, accompanied with subcutaneous masses and salivary gland involvement concurrently, especially in patients with eosinophilia.

Kimura disease of the breast: Case report and literature review of current management.

We report a rare case of Kimura disease in a 50-year old female patient who attended our tertiary level Breast Surgery Clinic.

Kimura's Disease.

Kimura's disease (KD) is a benign, rare and chronic inflammatory disorder of unknown etiology and it affects subcutaneous tissues, lymph nodes, and salivary glands. The clinical manifestation of cases reported is mainly small neoplasms or enlarged lymph nodes, while cases for large masses are rarely reported. This case reveals a female patient of KD with large masses in the head and neck region. The scope of the tumor is huge, which seriously affects the facial shape and psychology of the patient, thereby affecting the daily life of the patient. Although the patient had surgically removed the same site tumor 8 years ago, it was not possible to determine whether it was a recurrent case because the postoperative pathological diagnosis of first treatment was not confirmed. Under the general anesthesia, the right head and neck masses were removed. During the operation, the facial nerve branches were wrapped by the masses. After consulting the family members, it was agreed that the facial nerve should not be preserved for maximum extent removal of the tumor. Although the patient has facial paralysis symptoms after surgery, the facial shape and patient's psychology are greatly improved, and the patient is satisfied with the treatment effect. Surgical treatment is one of the effective ways to treat huge tumors in the head and neck of KD. This study has certain guiding significance for clinicians to treat huge tumors of head and neck in KD.

Kimura's disease associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease: a case report and review of the literature.

A 33-year-old Chinese man with 9-year history of Kimura's disease (KD) was admitted with a 1-month history of recurrent claudication. He did not have any clinical discomfort and had not taken any preventive medication in the past. He accepted percutaneous transluminal angioplasty and the pathologic diagnosis was reportedly consistent with necrotizing eosinophilic vasculitis. This is the rare reported case of KD associated necrotizing eosinophilic vasculitis presenting with recurrent peripheral arterial occlusive disease and the difficulties encountered in establishing an accurate diagnosis with unusual presentations. This case also highlights the possibility of recurrent complications without aggressive medical treatment in such unusual eosinophilic disorders.

Distinct, bilateral epithelioid hemangioma of the orbit.

UNLABELLED: ABSTRACT Introduction: Epithelioid hemangioma (EH) is a benign abnormal proliferation of endothelial cells usually arising in the head and neck region and presenting clinically as raised cutaneous lesions. EH rarely involves the orbit and has not been reported to occur bilaterally. CASE: A healthy 38 year old female developed sequential proptosis of each orbit over the course of several months. The patient underwent excisional biopsy confirming the diagnosis of EH in each orbit. She was treated with a course of high dose steroids and has been stable at 13 months following the most recent excision. CONCLUSION: This is the first reported case of bilateral EH.

Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features.

Kimura disease is a rare chronic inflammatory disorder mostly reported in males in second to third decades from south-east Asia. Head and neck is the most commonly involved region. The usual presentation is painless facial swelling with salivary gland involvement and regional lymphadenopathy. The diagnosis can be suspected on imaging but needs to be proven on histopathology. We describe three histopathology proven cases of Kimura disease and emphasize the specific clinic-radiological findings which can help in early identification of the condition and its differentiation from other diseases with similar presentation. Gray scale Ultrasound, contrast-enhanced ultrasound, and contrast-enhanced Magnetic Resonance Imaging (CE-MRI) were done in all patients and Computed Tomography (CT) was done in one. The salient imaging features were ill-defined solid mass in the subcutaneous location of the facial region which was heteroechoic on Gray scale Ultrasound, heterogeneously hyperintense on T2W MRI and without much diffusion restriction. There was involvement of salivary glands with ipsilateral cervical adenopathy. The lesion showed avid contrast enhancement on contrast-enhanced ultrasound and CE-MRI. We also present a systematic literature review of clinico-radiological features in Kimura disease in the head and neck. The articles with histopathologically proven cases and satisfactory description of imaging findings were included and we have compared the findings in our cases with published literature. In addition, we have included examples of radiological mimics of this disease. Certain imaging findings are highly suggestive and can aid in early diagnosis of Kimura disease which helps in early initiation of treatment with better patient prognosis.

Kimura Disease: A Rare Cause of Head and Neck Tumour in Children- A Case Report.

BACKGROUND: Kimura disease is a rare inflammatory condition classically manifested as painless subcutaneous nodules in the head and neck region and associated with regional cervical lymphadenopathy and salivary gland involvement. OBJECTIVE: The purpose of this report is to illustrate the diagnostic difficulty due to its rarity and non-specific clinical presentation. CASE PRESENTATION: We present a case of Kimura disease with bilateral eyelid swelling, parotid involvement, and cervical lymphadenopathy in a young boy. Computed Tomography (CT) showed ill- defined enhancing masses in both upper eyelids with lacrimal gland infiltration, multiple nodular lesions in both parotid glands, and bilateral enlargement of cervical nodes. After several multidisciplinary discussions and trials of medications, Kimura's disease was finally confirmed by histology examination. CONCLUSION: In conclusion, a rare disease such as Kimura must be considered earlier if the patient is not responding to the treatment.

Comparison between Kimura's disease and angiolymphoid hyperplasia with eosinophilia: case reports and literature review.

Kimura's disease (KD) is a rare chronic inflammatory or allergic disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm. Their relationship has always been debated. This article reports two rare cases, one of each disease. One patient was a 48-year-old female that presented with a mass on her right mandible. She also had oedema erythema and wheals on her lower limbs. She was diagnosed with Kimura's disease complicated with chronic urticaria. The second patient was a 23-year-old female that presented with multiple nodules of unequal size on the scalp. She was diagnosed with angiolymphoid hyperplasia with eosinophilia. The first patient recovered after being treated with surgical resection, glucocorticosteroids, cyclophosphamide and radiotherapy. The second patient underwent the first stage of surgical excision and is currently being followed-up. Comparison of the clinical and histopathological features of these two cases supports the theory that KD and ALHE are two separate disease entities.

Intracranial hemorrhage in Kimura's disease: coincidence or consequence?

OBJECTIVE: To report an unusual case of Kimura's disease associated with intracranial hemorrhage, the first such report in the literature. CASE REPORT: A 38-year-old man presented with chronic neurological disorders and a 10-year history of left cervical soft tissue swellings. Magnetic resonance imaging (MRI) of the cranium showed a subacute/chronic hematoma in the left occipitotemporal lobe and a chronic infarct in the right parietal lobe. Cervical computed tomography (CT) and MRI showed multiple masses on the left side of the neck and parotid gland. Histopathologic examination revealed lymphocyte and eosinophil infiltration, proliferation of hyalinated blood vessels, and interstitial fibrosis. Steroid therapy (2 mg/kg per day) was started and the lesions regressed partially. The masses and some enlarged regional lymph nodes were resected. CONCLUSIONS: Intracranial hemorrhage can either be a coincidental finding or complication of Kimura's disease. MRI and CT are effective modalities in radiologic diagnosis of this condition.

Bilateral asymmetric superficial soft tissue masses with extensive involvement of both upper extremities: demonstration of Kimura's disease by US and MRI (2008: 12b).

Kimura's disease is known as a rare chronic inflammatory disease occurring mainly in the head and neck regions of Asian male patients. We present a case of Kimura's disease with extensive involvement of the bilateral upper extremities in a 20-year-old Korean man. Ultrasonography (US) and magnetic resonance imaging (MRI) demonstrated the presence of partially ill-defined, conglomerated soft tissue masses centered in the subcutaneous layer of both upper arms, with cranial extension along the cutaneous nerve in the right lesion. Medial epitrochlear, axillary and lower cervical lymphadenopathy accompanied the masses.

Kimura's disease: imaging patterns on computed tomography.

AIM: To define the role of computed tomography (CT) in identifying and classifying the imaging patterns of diagnostic value in Kimura's disease of the head and neck. METHODS: A retrospective study was undertaken comprising 13 patients with histopathological evidence of Kimura's disease. The patients' clinical and pathological records were reviewed against a detailed analysis of their CT images performed from the base of the skull to the arch of the aorta. RESULTS: Both well-defined, nodular masses, as well as ill-defined plaque-like infiltrative masses were seen in the subcutaneous tissue of the head and neck region. All patients had lesions adjacent to the major salivary glands. The parotid gland was affected in 10 of the 13 cases and the submandibular gland was affected in the rest. Contrast enhancement was variable. More than half of the cases had associated lymphadenopathy. Some of them showed atrophy of the skin and subcutaneous fat overlying the subcutaneous masses. Blood eosinophilia was a consistent feature in all the cases. CONCLUSION: The patterns of distribution, morphology, and enhancement of the lesions in Kimura's disease that can be demonstrated at CT, enables a confident, non-invasive diagnosis of this condition, in an appropriate clinical context.

An unexpected asymptomatic epiglottal site of Kimura disease.

INTRODUCTION: Kimura disease (KD) is a chronic lymphoproliferative disorder of unknown etiology that affects the skin and lymph nodes, mostly observed in males of Asian descent. The natural history of asymptomatic epiglottal KD remains unknown. This rare site of KD is often only diagnosed when tumor growth starts to obstruct the upper airways. OBSERVATION: A 34-year-old North African male presented with fatigue and multiple, slowly progressive, fluctuating skin nodules in the right mandibular and retroauricular regions. Computed tomography of the head and neck revealed a large soft tissue tumor close to the right mandibular body and unexpected thickening of the epiglottis. Transnasal laryngoscopy confirmed the CT findings and showed thickening of the epiglottis. The diagnosis of KD was based on histological examination of biopsy specimens taken from the right mandibular tumor, a cervical lymph node, and the epiglottis. DISCUSSION: Most cases of KD with epiglottal involvement present with dysphonia and dysphagia. No consensus guidelines are available concerning the complementary investigations that should be performed. This case report raises the question of whether patients with suspected KD should be systematically screened for lesions in unusual and potentially dangerous anatomic sites.

Rare lesion, unusual location, uncommon presentation: a case of angiolymphoid hyperplasia with eosinophilia.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular proliferation of unknown pathogenesis that may be related to trauma. Although it affects mainly the head and neck, the zygomatic area is rarely involved. We report a case that affected the zygomatic region of a 46-year-old black man. The lesion had been present for about a year and the patient reported that it appeared after a facial injury during a soccer match. Clinical and tomographic investigations suggested a benign tumour, and the lesion was excised through an intraoral approach. Histopathological examination showed the unexpected diagnosis of ALHE. This tumour was interesting because of its rarity, and also because of its unusual site within the head and neck region. The diagnosis of ALHE is hardly ever considered in the differential diagnosis of zygomatic nodules.

Hypervascular Pseudonodular Plaque-Like Ultrasound Morphology in Angiolymphoid Hyperplasia. / Morfología ecográfica en placa pseudonodular hipervascular en hiperplasia angiolinfoide.

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular proliferation characterized by solitary or multiple angiomatous lesions. It is most common in young or middle-aged women, and the lesions typically affect the head and neck, showing a particular predilection for the periauricular region. The differential diagnosis in patients with ALHE is broad and includes both benign and malignant conditions. We report on a series of cases of periauricular ALHE in which ultrasound imaging revealed an hypervascular, pseudonodular and plaque-like morphology with clinical and histologic correlations. It also evidenced vascular communication between lesions that appeared to be separate on clinical examination. Familiarity with such ultrasound presentations could help to improve diagnostic accuracy and facilitate disease monitoring in patients with ALHE.

Kimura disease with nephrotic syndrome in a child- A rare association.

Lymphadenopathy along with various systemic manifestations is commonly encountered in pediatric patients, tuberculosis being the commonest etiology. Occasional patients may present a diagnostic conundrum. Here, the authors report an unusual manifestation of Kimura disease (KD) presenting as nephrotic syndrome associated with mesenteric lymphadenitis in an 11-year-old male child. KD is a chronic inflammatory disorder of unknown etiology. It typically affects young adult males in the age range of 27-40 years and usually presents as painless itchy nodular masses in the head and neck region. The involvement of mesenteric lymph nodes along with a very young age of presentation makes it a rare case, posing a diagnostic challenge for the unsuspecting physician.

Sonographic appearance of angiolymphoid hyperplasia with eosinophilia in the upper arm.

Angiolymphoid hyperplasia with eosinophilia is an uncommon benign condition characterized by cutaneous nodules involving primarily the head and neck regions of young adults. We report thecase of a 49-year-old woman with such a lesion in the arm. Sonographically, the lesion exhibited a hypoechoic rim and an echogenic central portion. On color Doppler imaging, the central portion was markedly vascular.