Level IV, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

IntroductionImmunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory condition. Forty-two cases with immunoglobulin G4-related sialadenitis (IgG4-RS) confirmed by histopathological and immunohistochemical assessment were studied to clarify the clinicopathologic characteristics of the salivary glands involved in IgG4-RS, especially the relationship between the histopathologic features and function of salivary glands or serum levels of IgG4.MethodsClinical, serologic, imaging and histopathological data of these cases were analyzed. CT volumes of submandibular, parotid, and lacrimal glands were calculated. The saliva flow rate was measured. Scintigraphy with 99mTc-pertechnetate was undertaken in 31 cases, and the concentration index (CI) and secretion index (SI) was calculated. Relationships between fibrosis severity and salivary gland function or serum IgG4 levels were analyzed.ResultsThe first symptom was swelling of bilateral submandibular or lacrimal glands. Physical examination showed multiple bilateral major salivary glands (including sublingual and accessory parotid glands) and lacrimal glands were enlarged in IgG4 RS. Multiple enlarged cervical lymph nodes were noted in 30 patients. Saliva flow at rest was lower than normal in 34 cases; stimulated saliva flow was lower than normal in 15 cases. Secretory function was reduced more severely in the submandibular glands than in the parotid glands. Serum levels of IgG4 were elevated in 95.2% of cases and 78.6% patients had increased IgE levels. Serum IgG4 level was higher and saliva secretion lower as glandular fibrosis increased.ConclusionsProminent changes in the morphology, histology, immunohistochemistry and secretion of the major salivary glands of IgG4-RS patients were accompanied by involvement of the lacrimal glands and cervical lymph nodes. Elevated IgE, allergic history, eosinophil infiltration suggest allergic reactions as a potential pathogenesis of IgG4-RS. Severity of glandular fibrosis correlated with salivary function and serum levels of IgG4.

Adenoid cystic carcinoma (ACC) is a relatively rare slow growing and often-aggressive epithelial-myoepithelial neoplasm that arises in multiple organs including the skin. The t(6;9) (q22–23;p23–24) translocation, resulting in a MYB-NFIB gene fusion has been found in ACCs from the salivary glands and other organs. Recently, MYB aberrations occurring in a subset (40%) of primary cutaneous ACC (PCACC) examples was described. Herein, we report 3 additional cases of PCACC harboring MYB aberrations. The tumors presented in 3 males aged 43, 81 and 55 years old and affected the extremities in the first 2 patients and the scalp in the third one. None of the patients had history of prior or concurrent ACC elsewhere. Lesions exhibited the classic ACC morphology of nests of basaloid cells arranged in cribriform and adenoid patterns. Sentinel lymph node biopsy was performed in two cases with one case showing lymph node positivity. Fluorescence in situ hybridization with break-apart probes for MYB and NFIB loci revealed that 2 cases showed MYB rearrangements while one case showed loss of one MYB signal. None of the cases showed NFIB rearrangements. We contribute with 3 additional cases of PCACC exhibiting MYB aberrations, the apparent driving genetic abnormality in these tumors.

Adamantinoma-like Ewing sarcoma (AES) is a rare variant of the Ewing family of tumors that resembles classic adamantinoma of bone. AES shows epithelial differentiation and a more complex immunohistochemical expression profile with keratin and basal marker immunoreactivity and can resemble a variety of carcinomas. We report an unusual case of an AES of the parotid gland that mimicked a basal cell adenocarcinoma. Like basal cell adenocarcinoma, this AES showed a nested basaloid proliferation with peripheral palisading in tumor nests with 'basaloid' epithelial differentiation as highlighted by cytokeratin AE1/3 and p40 positivity. However, unlike most basal cell adenocarcinomas, this tumor demonstrated high grade morphology, showed no true ductal or myoepithelial component, and also showed a tendency towards neuroectodermal phenotype with focal rosette formation, CD99 and weak synaptophysin immunoreactivity. EWSR1 and FLI1 fluorescence in situ hybridization confirmed the presence of a translocation supporting the diagnosis of AES. This is the first case of AES presenting as a primary parotid mass highlighting the potential to be mistaken for primary salivary gland carcinomas, which in addition to basal cell adenocarcinoma include other basaloid tumors such as adenoid cystic carcinoma.

ObjectiveTo observe the incidence of reversed halo sign in different pulmonary diseases and the pathological correspondence of reversed halo sign.MethodsRetrospectively studied the high resolution computer tomography scans of all the patients who were admitted in our department with abnormal pulmonary imaging, from 1st of January 2011 to 31st of December 2013, and all the cases with reversed halo sign on the high resolution computer tomography were collected. Clinical data such as pathological findings and confirmed diagnosis of the patients with reversed halo sign on the high resolution computer tomography scan were collected and summarized.ResultsOf 1546 abnormal High resolution computer tomography scans 108 had a reverse halo sign present, including 108 cases were observed with reversed halo sign in the high resolution computer tomography, including 40 cases of pulmonary tuberculosis, 43 cases of cryptogenic organizing pneumonia, 16 cases of lung cancer, 7 cases of sarcoidosis, and 1 case of pulmonary cryptococcosis, 1 case of granulomatosis with polyangiitis. Reversed halo sign had a higher incidence in granulomatous diseases (16.28%) compared with non-granulomatous diseases (9.97%).ConclusionsReversed halo sign is relatively non specific; it can be observed in different lung diseases, and different phases of diseases; reversed halo sign is more commonly found in granulomatous diseases compared with non-granulomatous diseases, and is most commonly observed in pulmonary tuberculosis among the granulomatous diseases, and in cryptogenic organizing pneumonia among the non-granulomatous diseases.

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Rationale:Plasmablastic lymphoma (PBL) is a rare subtype of human immunodeficiency virus (HIV)-related non-Hodgkin's lymphoma that predominantly manifests in the oral cavity.Patient concerns:Three cases of HIV-negative PBL were reported.Diagnoses:HIV-negative PBLInterventions:The patient had undergone chemotherapy.Outcomes:Clinical outcomes were very poor in Cases 1 and 3; Case 2, whose diagnosis suggested no bone marrow involvement, is still alive.Lessons subsections:These cases served to broaden the reported clinical spectrum of HIV-negative PBL. Clinicians and pathologists need to be familiar with lymphoma in the identified extra-oral PBL variation and there levant differential diagnosis procedures for this particular disease.

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