Rogério Oliveira Gondak

Oral pigmented lesions: Clinicopathologic features and review of the literature

Diagnosis of pigmented lesions of the oral cavity and perioral tissues is challenging. Even though epidemiology may be of some help in orientating the clinician and even though some lesions may confidently be diagnosed on clinical grounds alone, the definitive diagnosis usually requires histopathologic evaluation. Oral pigmentation can be physiological or pathological, and exogenous or endogenous. Color, location, distribution, and duration as well as drugs use, family history, and change in pattern are important for the differential diagnosis. Dark or black pigmented lesions can be focal, multifocal or diffuse macules, including entities such as racial pigmentation, melanotic macule, melanocytic nevus, blue nevus, smoker’s melanosis, oral melanoacanthoma, pigmentation by foreign bodies or induced by drugs, Peutz-Jeghers syndrome, Addison´s disease and oral melanoma. The aim of this review is to present the main oral black lesions contributing to better approach of the patients. Key words:Pigmentation, melanin, oral, diagnosis, management.

Carcinoma ex pleomorphic adenoma in a Brazilian population: clinico-pathological analysis of 38 cases

Carcinoma ex pleomorphic adenoma (CXPA) is a rare tumour, with different prevalence rates reported among studies. Epidemiological studies of large series of CXPAs in developing countries are scarce. The aim of the present study was to describe Brazilian patients with CXPA; this was a retrospective study of 38 patients. Demographic and clinico-pathological features were evaluated. No preferential gender was found, and the mean age at diagnosis was 57.6 years. The most commonly involved site was the parotid, followed by the submandibular and the minor salivary glands. A prevalence of clinical stages III and IV was observed at diagnosis. The most common histological subtypes were salivary duct carcinoma, adenocarcinoma not otherwise specified, myoepithelial carcinoma, and epithelial-myoepithelial carcinoma. Moreover, by invasive phase, most were frankly invasive carcinoma. Recurrence was observed in seven out of 24 patients with outcome information available, and all were invasive cases. All seven patients died of causes related to the disease. The distributions of cases according to age, gender, tumour location, and clinical stage were similar to those reported in the literature. Frankly invasive cases presented a worse prognosis. More information is needed to further our understanding of the clinico-pathological aspects of CXPA.

Increased number of Langerhans cells in oral lichen planus and oral lichenoid lesions.

OBJECTIVE The aim of this study was to quantify the presence of Langerhans cells (LC) in oral lichen planus (OLP) and oral lichenoid lesions (OLL), comparing them with normal epithelium. STUDY DESIGN Thirty-six patients with biopsy-proven OLP or OLL were selected for the study, as well as 23 control subjects free of inflammatory conditions. Immunohistochemical reactions were performed using the streptavidin-biotin peroxidase complex method with CD1a and CD83 primary antibodies. Densities were compared between groups and correlated with microscopic findings. RESULTS Patients with lichenoid conditions (OLP + OLL) presented higher densities of CD1a(+) cells than the control subjects (P = .03). Higher densities of CD1a were associated with a thinner layer of inflammatory cells (P = .02). CONCLUSIONS This study indicates that OLP and OLL are characterized by the recruitment of LC, which may play a significant role on its pathogenesis.

Depletion of Langerhans cells in the tongue from patients with advanced-stage acquired immune deficiency syndrome: relation to opportunistic infections

Gondak R O, Alves D B, Silva L F F, Mauad T & Vargas P A 
(2012) Histopathology 60, 497–503
Depletion of Langerhans cells in the tongue from patients with advanced‐stage acquired immune deficiency syndrome: relation to opportunistic infections

Primary synovial sarcoma involving the submandibular gland

Synovial sarcoma represents 5.6%-10% of all soft-tissue sarcomas. Adolescents and young adults are most frequently affected, mainly in the deep soft tissue of the extremities. Only 10% of synovial sarcomas affect the head and neck region; most of these are biphasic. We describe a case of an 18-year-old man who complained of a mass in the right submandibular region that had been present for approximately 12 months. On surgical removal, microscopic analysis showed a tumor formed by sheets of malignant spindle cells involving the submandibular gland. Immunohistochemistry displayed positivity for AE1/AE3, CK18/8, epithelial membrane antigen, CD99, CD56, and TLE-1. Based on these immunohistochemical and histopathologic features, a diagnosis of monophasic synovial sarcoma was rendered. The patient was treated with adjuvant radiotherapy and after 1 year was free of disease. To the best of our knowledge, this is the first reported case of synovial sarcoma involving the submandibular gland.

Maxillary desmoplastic fibroma with initial symptoms suggestive of sinusitis.

Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm characterized by the formation of abundant collagen fibers. It arises most commonly in the jaws and exhibits local aggressiveness and high recurrence rates after local resection. An uncommon case of expansive DF involving the right maxilla, maxillary sinus, and inferior orbital wall of a 49-year-old man whose initial symptoms were similar to acute sinusitis is presented, and the criteria for diagnosis and clinical management are discussed.

Carcinoma ex-pleomorphic adenoma of upper lip showing copy number loss of tumor suppressor genes.

BACKGROUND Carcinoma ex-pleomorphic adenoma (CXPA) is a malignant salivary gland tumor that arises rarely in the minor salivary glands. Although the etiology of CXPA remains unclear, the role of some tumor suppressor genes and oncogenes in CXPA is documented; however, other genes still need to be studied. STUDY DESIGN AND OBJECTIVE An uncommon case of CXPA involving the upper lip is presented, which was analyzed by a panel of tumor suppressor genes by multiplex ligation-dependent probe amplification. RESULTS The genes investigated in this study, a loss of copy number was detected for CASP8, CD44, CDH1, DAPK1, ESR1, RASSF1, and TP73. Immunohistochemical reactions for the validation of some of these results showed negativity for CD44, RASSF1, and p73. CONCLUSION A loss of copy number of the genes CD44, RASSF1, and TP73 may contribute to the carcinogenesis of CXPAs.

Reactive post-radiotherapy bone formation in the maxilla.

Objective High-dose radiation therapy affects bone metabolism, and therefore post-radiotherapy bone formation is an uncommon finding. This case describes an unusual ossification in the maxillary region identified after head and neck radiotherapy. Results A 45-year-old female patient was submitted to maxillary surgical resection and orbital exenteration due to squamous cell carcinoma. Radiotherapy post-surgery was performed because of compromised surgical margins. After 4 months of the conclusion of radiotherapy, the patient presented a bone exposition at surgical site. Surprisingly, the oral examination and computed tomography revealed a new formation of the right palatine vault and bone formation filling the nasal and orbital cavity. The incisional biopsy discarded residual disease or osteoradionecrosis and showed normal tissue. Conclusions To the best of the authors’ knowledge, this is the first case of wide bone formation as an early effect of head and neck radiotherapy.

Reduced number of CD1a+ and CD83+ interstitial dendritic cells in herpetic lesions (HSV‐1+) of the tongue in patients with advanced‐stage AIDS

tumours need to be differentiated from each other as they can have different outcomes: ASC is a very aggressive tumour, with a worse prognosis than highgrade MEC. Recently, we found that ASC of the head and neck region had a high rate of lymph node metastasis and a worse prognosis, especially in cases of MUC4 expression (K. Kusafuka et al., submitted). The most important histological features of ASC that differentiate it from MEC are: (i) keratin formation and intercellular bridges; (ii) desmoplasia; (iii) irregularlyshaped nests; (iv) prominent invasive growth near the tissues; and (v) the relative absence of goblet and intermediate cells. MECs, especially of low grade, frequently show CRTC1–MAML2 gene rearrangement, and even high-grade MECs infrequently show such a rearrangement. ASC often mimics high-grade MEC. To the best of our knowledge, this case may be the first well-documented case of ASC of a major salivary gland. It may be that some high-grade translocation-negative so-called MECs are in fact examples of ASC.

Bilateral osteoma of the maxillary sinus or anatomic variation

To the Editor: The nasal fossae and paranasal sinuses constitute an anatomical and functional unit. The paranasal sinuses communicate with the nasal cavities via narrow ducts that allow aeration and sinus drainage. Bone septa may occur due to zones of resorption, congenital condition, or acquired malformations. Congenital septa can develop in all maxillary sinus regions during the growth of the middle part of the face.3 On the other hand, atrophy-related resorption of the alveolar process generates in a vertical loss of bone volume, and progressive sinus pneumatization leads to an excavation of the alveolar process. The atrophy of the maxillary alveolar process proceeds irregularly in different regions and leaves bony ‘‘crests’’ on the maxillary sinus floor. Moreover, incomplete septa on the sinus floor can be considered to be the result of tooth loss and atrophy. Depending on the volume of the septum, a deficiency in irrigation and drainage sinus can cause episodes of inflammation. In addition to the atomic changes, the maxillary antrummay have benign osteogenic lesions such as osteomas that alter the anatomical conformation of the sinus cavity. Although the pathogenesis of osteomas remains controversial, there are 3 accepted theories of the etiology of paranasal sinus osteoma: traumatic, developmental, and infectious. Osteomas are characterized by the proliferation of compact or cancellous bone with sparse marrow tissue. The most common sites are mandible followed by the sinuses, particularly the frontal and ethmoidal. Its occurrence in the maxillary sinuses is less frequent. Differential diagnosis between central and peripheral types often confuses the clinician, and both lesions may simultaneously present in common radiological and clinical characteristics. Computed tomography (CT) is considered the most suitable imaging modality for the diagnosis of sinuses osteomas. CT allows high-resolution imaging of delicate bone structures and can be considered the method of choice for visualization of antral septa, and additional reconstructions allow an accurate visualization of specific anatomical variations. Patients with multiple osteomas associated with gastrointestinal disorders may be affected by Gardner syndrome. According to Sayan et al (2002), the triad of colorectal polyposis, skeletal abnormalities, and multiple impacted teeth is consistent with Gardner syndrome. A 65-year-old male patient was referred to Piracicaba Dental School, Oral Diagnosis Clinic, for evaluation of radiopacities in the maxillary sinuses. The patient was edentulous in the upper jaw and had slight discomfort in the posterior maxilla bilaterally for 2 months. The patient was a nonsmoker and had no medical history of sinusitis. In addition, the patient had no gastrointestinal disorders or skeletal changes. Extraoral examination revealed no alterations. On intraoral examination, the maxilla showed no swelling or changes in the recovering mucosa (Fig. 1A). Panoramic radiograph showed pneumatization of the maxillary sinuses and presence of bilateral radiopacities of approximately 1.0 0.5 cm each (Fig. 1B). In addition, a small radiopaque image was noted in the right posterior mandibular body suggestive of osseous sclerosis. CT revealed a hyperdense and well-delimited mass, which adhered to the lateral sinus wall. The remaining bony structures were within normal limits (Fig. 1C). The diagnostic hypotheses were anatomical variation of the maxillary sinus and atypical osteoma. Because of the symptoms, the patient underwent removal of the intrasinusal lesions under local anesthesia (Fig. 1D). A compact bone with sparse fibrovascular spaces with no sign of inflammation was noted on histological examination (Fig. 1E). The sample was dehydrated for scanning electron microscope (SEM; Jeol JSM-5600LV) and energy dispersive x-ray analysis (EDX) that showed a composition mostly of calcium (83.7%) and phosphorus (16.3%). Moreover, EDX was also performed on the normal bone tissue removed during surgery to access the maxillary sinus. A lower calcium concentration (71.2%) and higher phosphorus concentration (27.2%) were detected (Fig. 1F). In general, maxillary sinus osteomas are extremely rare and can be diagnosed by incidental panoramic radiologic examination. Depending on the location, osteomas may cause pain, swelling, sinusitis, nasal discharge, and, rarely, expand adjacent structures. Conversely, anatomic variation of the sinus maxillary similar to current has not yet been shown in the literature. The detection of abnormalities in this region is very important to rule out other diseases, and the SEM associated with EDX analysis can be useful to identify the composition and nature of structures of difficult diagnosis. Hence, the diagnosis of osteoma of the maxillary sinus was suggested due to clinical, tomographic, and EDX findings.