Chronic sclerosing sialadenitis of the bilateral submandibular glands in childhood - a diagnostic dilemma.

Chronic sclerosing sialadenitis (CSS), currently included in the group of immunoglobulin G4 (IgG4)-related diseases, is an under-recognized inflammatory lesion that afflicts mostly the submandibular gland of 40-60 years adults. To our knowledge, only one case of CSS located in the submandibular gland has been reported in childhood to date. We present a case of CSS in a 5-year-old male child. He presented with bilateral submandibular swellings that clinically resembled discrete lumps, suspected to be tumors. The completely resected tumors composed predominantly of dense lymphoplasmacytic inflammatory infiltrate rich in IgG4-positive cells [77-90 IgG(+) cells per high-power field; IgG4(+)∕IgG(+) cells ratio of 42.77%]. We discuss the peculiarities of this case, and we also review the literature on CSS.

An Extremely Rare Case of Sebaceous Adenoma Involving the Parotid Gland.

Sebaceous adenoma is an extremely rare tumor located in the parotid gland. In the English literature, less than 10 cases have been reported. Sebaceous adenoma represents 0.5% of all monomorphic adenomas. The authors are presenting a case of sebaceous adenoma of the parotid gland in a 65-year-old female who presented a mass on the left parotid area that had been gradually enlarging for one year without symptoms of pain. On imaging (ultrasound), a well-defined mass lesion in the left parotid area was seen. Histopathological findings were consistent with sebaceous adenoma. Surgical excision is curative. The prognosis is excellent, with a low recurrence rate. The present case report will increase the awareness and possibility of this rare tumor occurring at an unusual site, thereby avoiding any chance of misdiagnosis.

MLH1, BRAF and p53 - searching for significant markers to predict evolution towards adenocarcinoma in colonic sessile serrated lesions.

BACKGROUND AND AIM: Colonic serrated lesions are premalignant lesions, using an alternative malignization pathway, including multiple genetic and epigenetic alterations, as: mismatch repair deficiency due to MutL homolog 1 (MLH1) promoter methylation, tumor protein p53 (TP53) mutations, activating mutations of v-Raf murine sarcoma viral oncogene homolog B (BRAF) and Kirsten rat sarcoma viral oncogene homolog (KRAS). Our study aims to evaluate MLH1, BRAF and p53 immunohistochemical (IHC) status in sessile serrated lesions (SSLs), with and without dysplasia. MATERIALS AND METHODS: This is a retrospective case-control study including 20 SSLs with dysplasia and 20 SSLs without dysplasia (matching sex and age). IHC expression of MLH1, BRAF and p53 was evaluated as the percent of nuclear loss of MLH1, cytoplasmic positivity of BRAF and nuclear positivity of p53. Data concerning age, sex, localization of the lesion, dysplasia and IHC results were statistically processed using Microsoft Excel. RESULTS: We had very polymorphous patterns of IHC expression for BRAF, MLH1 and p53, especially in the dysplastic group. Thus, two patients were BRAF+∕MLH1-∕p53+, three were BRAF+∕MLH1-∕p53-, one was BRAF+∕MLH1+∕p53- and six were BRAF+∕MLH1+∕p53+. Dysplastic lesions without BRAF mutation exhibited the following phenotype: one case BRAF-∕MLH1-∕p53+, four BRAF-∕MLH1-∕p53- and three BRAF-∕MLH1+∕p53+. In the control group (SSLs without dysplasia), there was a more homogenous distribution of cases: eight cases BRAF+∕MLH1+∕p53-, seven BRAF-∕MLH1+∕p53-, one BRAF-∕MLH1-∕p53+, two BRAF-∕MLH1-∕p53- and two BRAF-∕MLH1+∕p53+. CONCLUSIONS: There are more routes on the serrated pathway, with different mutations and time of acquisition of each genetic or epigenetic lesion with the same morphological result. These lesions should be stratified according to their risk to poor outcome and their need to further surveillance.

Eosinophilic sialodochitis: a case report of a rare disease.

Eosinophilic sialodochitis (ES) is a rare clinical entity which presents as recurrent major salivary gland swelling and the presence of eosinophil-rich mucus plugs or histopathological evidence of eosinophilic infiltration around the larger salivary gland ducts. We present a case of ES not related with underwent a left submaxillectomy because recurrent episodes of submandibular gland swelling associated for the last three years. The laboratory workup demonstrated eosinophils slightly elevated and high levels of immunoglobulin E (IgE) in peripheral blood. Pathology slides confirmed the final diagnosis of ES, showing a dense periductal eosinophil-rich inflammatory infiltrate and periductal fibrosis. Although the entity is well described in the literature, diagnosis is often difficult due to its clinical presentation being similar to other conditions. Lesions that should be taken into consideration in differential diagnosis are mentioned. This will be the first ES case report in Eastern European literature.