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Microsecretory adenocarcinoma (MSA) is a recently described salivary gland tumor with a characteristic histologic and immunophenotypic profile and recurrent MEF2C-SS18 fusions. Because only six cases of MSA have been published, its complete clinicopathologic spectrum is unclear, and its biologic behavior has not been documented. Here, we present an updated and expanded experience of 24 MSA cases. All cases of MSA were obtained from the authors’ files. Immunohistochemistry for S100, SOX10, p63, p40, SMA, calponin, and mammaglobin was performed. Molecular analysis was performed by targeted RNA sequencing, SS18 break apart fluorescence in situ hybridization, and/or reverse transcriptase polymerase chain reaction for MEF2C-SS18 fusion. Clinical follow-up was obtained from medical records. A total of 24 MSA cases were collected, from 13 women and 11 men, ranging from 17 to 83 years (mean 49.5 years). The vast majority (23 of 24) arose in the oral cavity, with the palate (n = 14) and buccal mucosa (n = 6) as the most frequent subsites. Tumors showed consistent histologic features including: (1) microcystic tubules, (2) flattened intercalated duct-like cells, (3) monotonous oval hyperchromatic nuclei, (4) abundant basophilic luminal secretions, (5) fibromyxoid stroma, and (6) circumscribed borders with subtle infiltration. The tumors were very consistently positive for S100 (24 of 24), p63 (24 of 24), and SOX10 (14 of 14) and negative for p40 (0 of 21), calponin (0 of 12) and mammaglobin (0 of 16), while SMA (4 of 20) was variable. MEF2C-SS18 fusion was demonstrated in 21 of 24 cases; in the remaining 3 cases with insufficient RNA, SS18 break apart FISH was positive. Treatment information was available in 17 cases, all of which were managed with surgery only. In 14 cases with follow-up (1–216 months, mean 30), no cases recurred or metastasized. MSA is a distinct salivary gland neoplasm with remarkably consistent clinical, histologic, immunophenotypic, and genetic features that generally behaves in an indolent manner following surgery alone. These observations solidify MSA as a unique, low-grade salivary gland carcinoma that warrants inclusion in the next version of the WHO classification of head and neck tumors.

Introduction Median maxillary anterior alveolar cleft (MAAC) is a defect presenting in 1 % of the population. MAAC was first reported by Gier and Fast in 1967. A study of 66 human fetuses done by Stout and Collet in 1969 found evidence of two cystic lesions associated with MAAC. These cysts were named median alveolar cyst (MAC). To the best of our knowledge, we are reporting for the first time a bona fide example of MAAC - MAC in a human being. Case report A healthy 14-year-old Saudi female with an anterior maxillary diastema was referred to the orthodontics clinic for consultation. Clinical examination revealed a double frenum connecting the maxillary lip and alveolar vestibule. A panoramic film and a cone beam CT revealed a radiolucency between the maxillary central incisors extending from the alveolar crest to the incisive foramen area. The labial cortical plate was missing while the palatal was intact. The radiologist interpretation was “enlarged nasopalatine canal”. No other physical or dental abnormalities were evident. Upon surgical exploration, no labial maxillary osseous plate was found however, soft tissue was present and excised. Microscopic examination of the excised tissue revealed a cystic process lined by acanthotic nonkeratinizing stratified squamous epithelium with intracellular edema. In addition, sebaceous glands, islands of squamous epithelium with keratin pearl formation and lymphoid infiltrates were seen within the cystic wall. A retrospective review of the imaging studies coupled to the microscopic findings resulted in diagnosis of median alveolar cyst associated with a median maxillary anterior cleft. Conclusion We report a rare case of MAAC with MAC showing a sebaceous component. It is thought that MAC most likely originates from epithelial invaginations derived from the anterior intermaxillary suture. However, the mechanism involved in the formation of these two conditions remains to be elucidated.

Introduction First described by Wright in 1981, orthokeratinized odontogenic cyst (OOC) represents a developmental condition derived from epithelial dental lamina rests. Radiographically, OOC appears as a unilocular radiolucency associated with an impacted mandibular third molar. Occasional examples of bilateral/multicentric OOCs have been reported in the literature. Here, we present the clinical, radiographic and microscopic features of a patient with bilateral mandibular OOCs. Case report A healthy 19-year-old male presented with asymptomatic unilocular radiolucencies associated with left and right impacted mandibular 3rd molars. Both molars were extracted and the associated lesions enucleated. Microscopic examination of both specimens showed identical microscopic features consisting of cystic cavities lined by orthokeratinized stratified squamous epithelium with hypergranulosis. A diagnosis of bilateral OOCs was rendered. No recurrence was evident after 4-months. Discussion Six cases (including ours) of bilateral/multicentric OOCs have been documented in the English and Spanish literature. An analysis of the published demographics of this condition showed that most bilateral/multicentric OOCs have a striking predilection for young adult males (age range: 19-41 years; mean age: 27.3 years) with only one case presenting in a female. Bilateral OOCs are almost exclusively associated with impacted mandibular third molars. However, one report documented OOCs in all quadrants. The follow-up period for these cases ranged from 4 months-13 years with no recurrence stated. Conclusion Bilateral OOCs are uncommon and appear to have an excellent prognosis with no recurrence expected. However, more reports with long-term follow-up are needed to draw meaningful conclusions about their biological behavior.

An important inconsistency currently exists in the literature on oral cancer. Reviewing this literature, one finds that the term oral cancer is defined and described with great variation. In a search in PubMed, at least 17 different terms were found for titles of papers reporting data on oral cancer. The variability of the terms used for designating anatomic regions and type of malignant neoplasms for reporting oral cancer has hampered the ability of researchers to effectively retrieve information concerning oral cancer. Therefore, it is sometimes extremely difficult to provide meaningful comparisons among various studies of oral cancer. Recently, a new ontological strategy that is rooted in consensus-based controlled vocabularies has been proposed to improve the consistency of data in dental research (Smith et al. in J Am Dent Assoc 141:1173–1175, 2010). In this paper, we analyzed the terminology dilemma on oral cancer and explained the current situation. We proposed a possible solution to the dilemma using an ontology-based approach. The advantages for applying this strategy are also discussed.

A 45-year-old white woman presented with a smooth, sessile, pink, exophytic, polypoid mass on the ventral surface of her tongue. The lesion was asymptomatic and the patient had not been aware of it. The lesion measured approximately 4.0 1.0 cm in its largest dimensions (Fig. 1). The patient’s medical history was significant for type II diabetes, asthma, arthritis, kidney dysfunction, hyperlipidemia, and previous lumbar spine injury. She reported a 20-year smoking history but denied alcohol use.

Neurofibromatosis Type 1 (NF1) is one of the most common autosomal dominant diseases affecting multiple systems including the vascular, skeletal, and central nervous system. Noonan syndrome (NS) is an autosomal dominant genetic disorder, associated with musculoskeletal and skin manifestations. Coexistence of central giant cell lesions in patients with both NS and NF1 were reported in the literature. Development of multiple central giant cell lesions in a patient with a Noonan syndrome has been referred to as Noonan-like syndrome. A few cases with features of NF1 and NS have been termed as NF1-NS. Here, we present a case of so-called NF-NS associated with a central giant cell lesion.

Sialolipoma is rare benign neoplasm arise from salivary glands (majors and minors) characterized by neoplastic adipose tissue with scattered non-neoplastic salivary gland acinus. To date 60 cases (including 5 cases reported in the present paper) have been reported in scientific literature. This article presents 5 new cases of sialolipoma affecting minor salivary glands (MiSG) and additionally reviews and analyzes the previously published cases to assess possible demographical differences between sialolipoma from minor and from major salivary glands. Case reports: 5 cases (3 females; 2 males; age means 63.8 years), of sialolipoma from MiSG, are reported. 2 of them were located in buccal mucosa, 1 in upper lip mucosa, 1 in floor of the mouth and 1 in retromolar area. All tumors were composed by neoplastic adipocytes cells interlaced with normal salivary gland acinus cover it by a fibrous tissue capsule. Analyzes of literature showed that MiSG sialolipoma is most frequent in females over 60 years old, therefore and in conclusion this article assess different demographical profile of sialolipoma in respect to their topography.

Previously we have described the transmission of malaria by the oral route in a murine model. Due to the similarities between Plasmodium and Babesia, we tried to reproduce oral transmission in parasites of the latter genus by ingestion of infected blood and by cannibalism. In the first case, experimental mice were inoculated orally with 20, 50, or 100 microliters of Babesia microti-infected blood, and in the second, each fasted experimental mouse was offered the corpse of an infected mouse serving as the bait inoculum. B. microti infection was acquired by 3.7% of all experimental animals orally inoculated with infected blood and by 15.1% of all mice inoculated by cannibalism. The approximate period of prepatency ran from 2 to 4 weeks. No control mouse acquired the infection. This represents the first time that oral transmission of babesiosis has been described. This kind of transmission may be present in nature. Babesiosis may be acquired and maintained in nature in the absence of ticks.

We report two unusual cases of solitary fibrous tumor (SFT) of minor salivary glands that microscopically mimicked pleomorphic adenoma. One of these lesions presented in the retromolar region and the other in the buccal mucosa. The microscopic features of these two tumors and their intimate relationship with regional mucous minor salivary glands posed a diagnostic challenge. Awareness of the morphological diversity of SFT coupled to a judicious use of appropriate immunohistochemical probes should prove valuable to accurately segregate SFT from pleomorphic adenoma.

Treatment of salivary stones includes both surgical and non-surgical techniques. Surgical approaches range from excision of the sialolith, for those near the duct orifice, to removal of the affected salivary gland and its associated duct, for stones near the hilum of the gland. We present a case of two sialoliths triggering an acute infection in a residual Wharton's duct, 12 years after the removal of the associated submandibular gland. Excision of the sialoliths and treatment of the infected duct via sialodochoplasty was successfully performed in this patient. If the Wharton's duct is not removed with the associated submandibular gland, the potential for infection and continuous growth of dormant calcifications exists. We also address the aetiology, pathogenesis, and management of patients with sialolithiasis in the absence of a major salivary gland.

Cheilitis glandularis (CG) is a rare inflammatory salivary gland disease that usually affects the lips. Although the etiology of CG is still unknown, it is believed to be a hereditary disease with an autosomal dominant pattern of inheritance. Three clinical presentations of CG are described in the literature: simple, superficial suppurative, and deep suppurative. A case of deep suppurative CG that extended to the buccal mucosa has been previously reported as suppurative stomatitis glandularis (SSG). Here we report a case of SSG in a 64-year-old white female with a history of bilateral renal transplants for adult polycystic kidney disease, who presented with painful swollen lips and bilateral buccal mucosal lesions. The diagnosis and management of the case is discussed. To the best of our knowledge, this is the second report of SSG, a rare condition affecting the minor salivary glands in the oral cavity.

A 46-year-old male sought periodontal care for a swelling on his right mandibular gingiva. An excisional biopsy revealed a well-differentiated squamous cell carcinoma. Surgical treatment consisted of a right segmental mandibulectomy with ipsilateral right neck dissection and fibular free flap reconstruction. Two days after the surgical procedure, a weakened Doppler signal suggested vascular compromise of the graft. The patient was returned to the operating room where complete thrombosis of the internal jugular vein (recipient vessel) was observed. This event prompted a complete hematological evaluation that disclosed low serum levels of protein S. The patient was started on systemic heparin and local medicinal leeches. A week later, systemic warfarin sodium was added and successfully resolved the vascular compromise of the graft. Two years later, the patient is active and lives a full life with occasional adjustments of warfarin sodium. This case represents the first report on the treatment of gingival carcinoma that led to the serendipitous discovery of an unrelated and unusual systemic condition, protein S deficiency.

A total of 116 young male CD1 mice were orally inoculated with mouse blood; half of the animals received 0.2 ml of uninfected blood and the others were given 0.2 ml of Plasmodium berghei yoelii-infected blood in six experiments performed at different times. Almost 30% of the experimental mice acquired malaria as demonstrated by the observation of parasites in their blood. In no case were parasites found in the blood of control mice. Rodent malaria parasites may be transmitted to CD1 mice by the ingestion of mouse blood parasitized by P. b. yoelii. As far as we know, this study represents the first demonstration of oral transmission of murine malaria. Oral transmission studies in this mouse-Plasmodium model may produce very important information on the biology of the malaria parasites.