Oral lesions of systemic lupus erythematosus: A collaborative Latin American study.

BACKGROUND: Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease that may affect the oral mucosa. The variable spectrum of oral lesions observed in SLE can pose challenges in diagnosis, particularly when the lesions occur in isolation. The aim of this study was to describe the oral lesions occurring in patients with SLE from Latin America. METHODS: This collaborative record-based study involving 11 oral and maxillofacial pathology and medicine services across Venezuela, Argentina, Chile, Brazil, and Mexico describes the clinicopathological profile of SLE-related oral lesions. RESULTS: Seventy patients with SLE and oral lesions were included in the study. The majority were females (75.7%; female/male ratio: 3.1:1) and white (62.1%), with a mean age of 38.4 years (range: 11-77 years). The most common site of oral lesions was the hard/soft palate (32.0%). Clinically, oral lesions predominantly presented as ulcers (26.6%), erosions (26.6%), and white lesions (23.4%). Isolated oral lesions occurred in 65.2% of individuals, while cutaneous manifestations occurred in 80.3%. The main clinical diagnostic hypothesis in 71.4% of cases was an immune-mediated disease. Oral biopsies followed by histopathological analysis were performed in 50 cases. CONCLUSION: Oral lesions of SLE exhibit a variety of clinical and histopathological features. A key point in diagnosis is that unusual oral changes without an obvious local cause may indicate a possible systemic condition presenting with oral lesions. A multidisciplinary approach, which includes regular oral examination, is warranted to identify oral lesions and provide treatment.

Oral solitary fibrous tumors: A collaborative clinicopathologic study of 19 cases.

OBJECTIVE: To report the clinicopathologic features of 19 oral solitary fibrous tumors (SFTs). METHODS: Clinical data were collected from the records of seven pathology services. All cases were re-evaluated by HE staining and confirmed by immunohistochemistry. RESULTS: The series comprised 11 females (57.9%) and 8 males (42.1%), with a mean age of 47.3 ± 14.7 years (range: 22-71 years) and a 1.3:1 female-to-male ratio. Most tumors affected the buccal mucosa (n = 7; 36.8%) and presented clinically as an asymptomatic solitary submucosal well-circumscribed nodule with coloration similar to the oral mucosa. Morphologically, most SFTs (n = 10; 52.6%) exhibited a classic hybrid pattern characterized by a well-circumscribed proliferation of densely cellular areas alternating with hypocellular areas in a variably collagenous vascular stroma. Remnants of accessory salivary glands were observed in two cases (n = 2; 10.5%). All tumors were positive for STAT6 and CD34 (n = 19; 100%). Outcome information was available from 6 patients (31.6%), with clinical follow-up ranging from 6 to 24 months (mean ± SD, 9.5 ± 6.8 months), and none developed local recurrence. CONCLUSIONS: Oral SFTs are rare and often clinically misdiagnosed. Pathologists should consider SFT in the differential diagnosis of oral spindle cell tumors. Accurate diagnosis requires careful morphological evaluation supported by immunohistochemical analysis.

Acquired oral syphilis: A multicenter study of 339 patients from South America.

OBJECTIVE: To report the clinicopathologic features of acquired oral syphilis cases in South American countries. MATERIALS AND METHODS: Clinical data were retrospectively collected from the records of 18 oral diagnostic services in Argentina, Brazil, Chile, Colombia, Venezuela, Uruguay, and Peru. Serologies of nontreponemal and treponemal tests were used for diagnosis. RESULTS: The series comprised 339 cases of acquired oral syphilis. Secondary syphilis ranked as the most common stage (86.7%). Lesions were more frequent among males (58.0%) and young adults with a mean age of 33.3 years. Individuals aged 20-29 years were most affected (35.3%). The most commonly involved sites were the tongue (31.6%), lip/labial commissure (25.1%), and hard/soft palate (20.4%). Clinically, acquired oral syphilis usually presented as mucous patches (28.4%), papules (25.7%), and ulcers (18.1%). Skin manifestations occurred in 67.7% of individuals, while lymphadenopathy and fever were observed in 61.3% and 11.6% of all subjects, respectively. Most patients were treated with the benzathine penicillin G antibiotic. CONCLUSION: This report validates the spread of acquired oral syphilis infection among young adults in South America. Our directives include accessible diagnostic tools for proper disease screening, surveillance, and counselling of affected individuals, especially in low- and middle-income countries.

Sialolipomas of minor salivary glands: A multi-institutional study and literature review.

BACKGROUND: Sialolipoma is a rare histological variant of lipoma commonly misdiagnosed and composed of a proliferation of mature adipocytes with secondary entrapment of normal salivary gland tissue. The purpose of the present study is to report the clinicopathologic and immunohistochemical features of 10 new cases of sialolipomas in conjunction with a review of the literature. METHODS: A retrospective descriptive cross-sectional study was performed. A total of 54,190 biopsy records of oral and maxillofacial lesions from four oral and maxillofacial pathology services in Brazil were analysed. All cases of lipomas were reviewed, and clinical, demographic and histopathological data were collected of all cases compatible with sialolipomas. In addition, immunohistochemistry stains (AE1/AE3, CK7, 34ßE12, S-100, HHF35, α-SMA and Ki-67) and a literature review based on a search of three electronic databases (PubMed, Web of Science and Scopus) were performed. RESULTS: Among all lipomas reviewed, there were 10 cases of sialolipomas. The series comprised of 7 females (70.0%) and 3 males (30.0%), with a mean age of 46.1 ± 21.5 years (range: 11-71 years) and a 2.3:1 female-to-male ratio. The lower lip (n = 3, 30.0%) and tongue (n = 2, 20.0%) were the most common locations, presenting clinically as a nodule of slow growth and normal colour. Conservative surgical excision was the treatment in all cases. No recurrence was observed. CONCLUSION: Sialolipomas are a rare histological variant of lipoma, affecting the salivary glands, mainly in the parotid gland and palate of female adults. Pathologists must recognise sialolipomas to avoid misdiagnoses with other lipomatous tumours that can affect salivary glands.

Mature T/NK-Cell lymphomas of the oral and maxillofacial region: A multi-institutional collaborative study.

BACKGROUND: The diagnosis of oral and maxillofacial mature T/NK-cell neoplasms is challenging because of their rarity, morphological heterogeneity and complex immunophenotype with scarce available data describing their clinical and microscopic aspects. Therefore, in this study, we investigated a series of mature T/NK-cell neoplasms affecting this anatomical region and provided an updated literature review. METHODS: Cases diagnosed as mature T/NK-cell lymphomas affecting the oral and maxillofacial region were retrospectively retrieved from six pathology files and their diagnoses were confirmed using haematoxylin and eosin-stained slides, immunohistochemical reactions and in situ hybridization for Epstein-Barr virus (EBV) detection. Patients' clinical data were collected from their pathology forms. RESULTS: A total of 22 cases were included in this study. Eleven (50%) consisted of extranodal NK/T-cell lymphomas, nasal type; eight (36.4%) were peripheral T-cell lymphomas, NOS; two (9.1%) were adult T-cell leukaemia/lymphomas, and one (4.5%) was an ALK-positive anaplastic large cell lymphoma. Overall, males predominated, with a mean age of 55.7 years. The palate was the most affected site (50%), and tumours usually presented as destructive and painful ulcers. EBV was present in all cases of extranodal NK/T-cell lymphoma nasal type but was absent in the other subtypes. CONCLUSION: Among mature T/NK-cell lymphomas of the oral and maxillofacial region, extranodal NK/T-cell lymphoma, nasal type and peripheral T-cell lymphoma, NOS predominated. Older men were the most affected patients, and this heterogeneous group of neoplasms has a very aggressive clinical behaviour.

An extensive intraoral blue nevus in an elder patient.

OBJECTIVE: To report a rare case of intraoral extensive blue nevus in an elder patient. BACKGROUND: Oral blue nevi is a well-recognised skin melanocytic neoplasm that rarely occurs in the oral cavity and may mimic melanoma in the early stages. METHODS: An incisional biopsy was performed, and the diagnosis was blue nevus. CONCLUSION: Both the clinician and pathologist must be aware of such a presentation to diagnose and treat appropriately.

Gingival squamous cell carcinoma mimicking a non-neoplastic proliferative lesion in an older patient.

OBJECTIVE: To report a case of gingival squamous cell carcinoma (SCC) in an elderly patient mimicking a non-neoplastic proliferative lesion. BACKGROUND: Oral SCC is a well-recognised malignancy of the oral cavity that may mimic benign reactive proliferative processes. METHODS: An excisional biopsy was performed, and the diagnosis was gingival SCC. CONCLUSION: It is essential that both the clinician and pathologist to be aware of such a presentation to allow for proper diagnosis and treatment.

Intratarsal Keratinous Cyst Clinically Misdiagnosed as a Chalazion.

The intratarsal keratinous cyst (IKC) is a recently described entity, often clinically misdiagnosed as a chalazion. We report a case of a 61-year-old male patient with a chief complaint of a small lesion on the upper eyelid that evolved over six months. On physical examination, an asymptomatic, firm nodule was identified on the left upper eyelid. The patient reported no history of trauma. A provisional diagnosis of chalazion was established, and an excisional biopsy was performed. Histopathologically, the lesion was lined with a stratified squamous epithelium, with a corrugated epithelial surface showing abrupt keratinization without keratohyalin granules, and compact keratinous-appearing material in the cystic lumen. The diagnosis was IKC. No signs of recurrence were observed after one year of follow-up. It is essential to accurately diagnose IKC and distinguish it from chalazion and epidermal inclusion cysts, because IKC requires complete surgical excision and can exhibit multiple recurrences if not properly removed.

Primary immune thrombocytopenic purpura with exuberant oral manifestations in a patient with Turner syndrome.

BACKGROUND: Primary Immune Thrombocytopenic Purpura (ITP) is an autoimmune hematological condition characterized by isolated thrombocytopenia and frequently presents with oral manifestations. However, reports of primary ITP in patients with Turner Syndrome (TS) are exceptionally rare, with few cases documented in the literature. Herein, we describe an unusual case of primary ITP with exuberant oral manifestations in a patient with TS. CASE REPORT: A 29-year-old woman was referred to an oral diagnostic service with complaints of "blood blisters and gum bleeding" lasting 8 h. On extraoral physical examination, multiple petechiae were observed in the upper and lower limbs, in addition to hemorrhagic extravasation in the right ocular sclera (hyposphagma). On intraoral examination, multiple vesicles and blisters filled with blood were identified on the lower lip, back of the tongue, and buccal mucosa, along with spontaneous gingival bleeding and hemorrhagic petechiae on the palate. Laboratory tests revealed thrombocytopenia (5000/mm3), whereas the blood count showed normality in the red and white series. After excluding other etiological factors or associated diseases, the patient was diagnosed with severe ITP and began treatment with systemic corticosteroids in the intensive care unit, resulting in a successful increase in platelets. After a 2-year follow-up, the patient remains free of ITP recurrences. CONCLUSION: Oral manifestations may be one of the first signs of ITP. Therefore, it is essential that dentists are familiar with the condition and, when faced with unusual oral bleeding, consider the possibility of a hematological disorder such as ITP, ensuring a correct and early diagnosis. Moreover, the presence of ITP can further exacerbate complications associated with TS. Therefore, rigorous follow-up of these patients is crucial, considering the high incidence of cardiovascular and autoimmune diseases and the reduced life expectancy of these patients.

Oral granular cell tumor: a collaborative clinicopathological study of 61 cases.

BACKGROUND: Granular Cell Tumor (GCT) is an uncommon benign lesion in the oral cavity whose pathogenesis remains poorly understood. Due to their infrequent occurrence and similarity to other oral lesions, they are often forgotten during the initial clinical diagnosis. Therefore, understanding its prevalence, clinical and pathological characteristics is crucial for an accurate diagnosis and adequate management. METHODS: All cases diagnosed as GCTs in six Brazilian and Argentinian oral diagnostic centers were re-evaluated by HE staining, and clinical, demographic, and histopathological data were collected and evaluated. RESULTS: The series comprised 45 female (73.8%) and 16 male (26.2%), with a 2.8:1 female-to-male ratio and a mean age of 35.3 ± 16.9 years (range: 7-77 years). Most cases occurred on the tongue (n = 49; 81.6%) and presented clinically as asymptomatic papules or nodules (n = 50; 89.3%) with a normochromic (n = 25; 45.5%) or yellowish (n = 11; 20.0%) coloration and sizes ranging from 0.2 to 3.0 cm (mean ± SD: 1.40 ± 0.75 cm). Morphologically, most tumors were characterized by a poorly delimited proliferation (n = 52; 88.1%) of cells typically rounded to polygonal containing abundant, eosinophilic, finely granular cytoplasm. Pustulo-ovoid bodies of Milian were identified in all lesions (n = 61; 100%). Entrapment of skeletal striated muscle (n = 44; 72.1%) and nerve fibers (n = 42; 68.9%) were common findings. Pseudoepitheliomatous hyperplasia (PEH) was observed in 23 cases (39.0%). In only 27.7% of cases (n = 13) there was agreement between the clinical and histopathological diagnosis. Outcome information was available from 16 patients (26.2%), with clinical follow-up ranging from 4 to 36 months (mean 13.3 months), and none developed local recurrence. CONCLUSIONS: The clinical and histopathological features of GCTs were consistent with those described in previous studies. In general, these lesions have a predilection for the lateral region of the tongue in adult women. It is essential to consider GCTs in the differential diagnosis of yellow or normochromic papules and nodules in the oral cavity. Histopathological evaluation is essential for the definitive diagnosis and the prognosis is excellent.

Clinicopathologic and imaging features of odontogenic myxomas: a multi-institutional study.

PURPOSE: This study aims to report clinicopathologic and imaging features of odontogenic myxomas (OM), highlighting uncommon findings. METHODS: Clinicopathologic and imaging data of OMs diagnosed in the five Brazilian diagnostic pathology centers were collected and analyzed. RESULTS: The series comprised 42 females (68.9%) and 19 males (31.1%), with a 2.2:1 female-to-male ratio and a mean age of 34.5±15.4 years (range: 4-80). Clinically, most OMs presented as painless intraoral swelling (n = 36; 70.6%) in the mandible (n=37; 59.7%). Multilocular lesions (n=30; 83.3%) were more common than unilocular lesions (n=6; 16.7%). There was no statistically significant difference between the average size of unilocular and multilocular OMs (p=0.2431). The borders of OMs were mainly well-defined (n=24; 66.7%) with different degrees of cortication. Only seven tumors caused tooth resorption (15.9%), while 24 (54.5%) caused tooth displacement. Cortical bone perforation was observed in 12 (38.7%) cases. Morphologically, OMs were characterized mainly by stellate or spindle-shaped cells in a myxoid background (n=53; 85.5%). Surgical resection was the most common treatment modality (n=15; 65.2%), followed by conservative surgery (n=8; 34.8%). Outcomes were available in 20 cases (32.3%). Seven of these patients had local recurrence (35%). Enucleation was the treatment with the highest recurrence rate (4/7; 57.1%). CONCLUSIONS: OM has a predilection for the posterior region of the jaws of female adults. Despite their bland morphological appearance, they displayed diverse imaging features. Clinicians must include the OM in the differential diagnosis of osteolytic lesions of the jaws. A long follow-up is needed to monitor possible recurrences.

Basal cell adenocarcinoma of the soft palate: A closer look for an exceedingly rare salivary gland tumor.

Basal cell adenocarcinoma (BCAd) is an extremely rare primary biphasic carcinoma of the salivary glands with few well-documented cases reported in the literature. Herein, we report a rare case of a 44-year-old male patient who presented an oral medicine service with an erythematous nodular lesion on the soft palate, measuring 1.5 cm in its largest diameter, with a 5-year duration. The clinical diagnosis was pleomorphic adenoma, and an excisional biopsy was performed. Histopathological analysis revealed a biphasic infiltrative tumor composed of a mixture of central ductal cells and abluminal basal cells with slight atypia arranged in solid, trabecular, tubular and cribriform growth patterns in a loose stroma. The peripheral cells show a palisading arrangement with round hyperchromatic nuclei and scanty cytoplasm. Occasional mitotic figures were seen. Few spindle-shaped cells suggestive of myoepithelial cells were present in the stroma surrounding the basaloid tumor nests. The diagnosis was BCAd. The patient was referred to a head and neck service and has been followed up for 8 months with no signs of recurrence. In conclusion, although the diagnosis of BCAd can be challenging due to its rarity and morphological overlap with other salivary gland lesions, a meticulous morphological assessment is key for accurate diagnosis, especially in cases originating from minor salivary glands. Surgical excision with a wide safety margin is the treatment of choice and long-term follow-up is recommended to monitor possible recurrences.

Squamous Odontogenic Tumor in the Posterior Region of Maxilla.

Squamous odontogenic tumor (SOT) is a rare benign but locally infiltrative tumor often misdiagnosed as other entities, such as ameloblastoma and squamous cell carcinoma, due to overlapping morphological findings. We document here the clinicopathological and imaging findings of an aggressive intraosseous SOT in the posterior left region of the maxilla in a 25-year-old male patient. On intraoral examination, the tumor extended from the region of the left lateral incisor to the upper left premolar and was covered by reddish mucosa, with discrete areas of ulceration. Imaging exams revealed an osteolytic lesion causing thinning, erosion, and buccal and lingual cortical plate perforation associated with an impacted canine. Microscopically, the tumor showed a proliferation of islands of well-differentiated squamous epithelium in a variably collagenized background. The peripheral cells of the islands were flat or slightly cuboidal and did not exhibit nuclei with peripheral palisade and reverse polarization. The diagnosis of SOT was rendered. The patient underwent surgical resection and has been under clinical follow-up for approximately 12 months with no signs of recurrence. A careful morphological evaluation is essential to avoid misdiagnosis and ensure a satisfactory treatment approach.

Lingual metastasis as the first presentation of clear cell renal cell carcinoma: Report of a rare case clinically mimicking a benign lesion.

Metastases in the oral cavity are rare and frequently associated with widespread disease and poor prognosis. Clear cell renal cell carcinoma (ccRCC) is the most common subtype of renal cell carcinoma (RCC), known for its propensity for metastatic spread. The diagnosis of oral metastases from ccRCC is challenging, especially when the patient has no history of primary neoplasm. Herein, we reported a rare metastatic ccRCC in a 58-year-old female presenting clinically as a painless nodule on the tongue mimicking a benign lesion. Microscopically, the tumor showed a proliferation of clear cells organized in nests with a predominantly lobular arrangement. The dense connective tissue stroma was highly vascularized, exhibiting some areas with a hyalinized aspect. Immunohistochemistry showed focal positivity for pan-cytokeratin AE1/AE3 and strong positivity for CK8, CK18, epithelial membrane antigen (EMA), vimentin, and CD10. S-100 protein, HMB-45, CK7, and CK20 were negative. The definitive diagnosis was metastatic ccRCC. The treatment was initiated with sunitinib. However, the disease progressed, and the patient was submitted to palliative care. Despite the low survival rate of patients with metastases, the clinician's experience in identifying an oral metastatic lesion may reveal undiagnosed primary tumors and provide better prognosis and survival rates.

Gingival rhabdomyosarcoma mimicking a non-neoplastic proliferative lesion: A diagnostic challenge.

Rhabdomyosarcoma (RMS) is a soft tissue sarcoma that develops from skeletal striated muscle cells. RMSs are exceedingly rare in the oral cavity, particularly in the gingiva. Herein, we reported the clinicopathological and immunohistochemical features of a rare case of RMS in a 30-year-old female presenting clinically as a painful polypoid nodule on the mandibular gingiva. Microscopically, the tumor showed atypical spindle cells with elongated nuclei and eosinophilic cytoplasm arranged in a fascicular pattern. In focal areas, the tumor cells exhibited rhabdomyoblastic differentiation. Immunohistochemistry showed strong positivity for desmin, myogenin (scattered cells), and MyoD1. The patient underwent surgical resection followed by postoperative complementary radio- and chemotherapy. However, the patient had a local recurrence seven months after the initial treatment. She was submitted to a total mandibulectomy associated with adjuvant radiotherapy. However, she died two months after reoperation due to complications secondary to radiation therapy. Because of the rarity in the oral cavity and non-specific signs and symptoms, the clinical diagnosis of RMS is difficult and often overlooked. Therefore, careful histopathological and immunohistochemistry analysis of these tumors is essential to correct diagnosis. Early surgical excision with tumor-free margins and prolonged follow-up are strongly recommended.

Salivary gland tumors: A 13-year clinicopathologic retrospective study in a Brazilian northeast population.

Background: The present study aimed to evaluate the clinicopathologic features of salivary gland tumors (SGTs) in a Brazillian northeast population. Material and Methods: A retrospective descriptive cross-sectional study was performed (1995-2009). All cases of SGTs diagnosed in a private surgical pathology service in Brazil were reviewed, and clinicopathological data were collected. Results: A total of 23.258 histopathological records of biopsies were analyzed, and 174 cases were diagnosed as SGTs (0.7%). Of these, 117 (67.2%) were classified as benign, and 57 (32.8%) were malignant. The series comprised 89 females (51.1%) and 85 males (48.9%), with a mean age of 50.2 years (range: 3-96 years) and a roughly equal female-to-male ratio (1:1). Most tumors occurred in the parotid gland (n = 82, 47.1%), followed by the palate (n = 45, 25.9%), submandibular gland (n = 15, 8.6%). Pleomorphic adenoma (n = 83; 70.9%) and mucoepidermoid carcinoma (n = 19, 33.3%) were the most frequent benign and malignant tumors, respectively. After reevaluation of morphology and immunohistochemical analysis, seven tumors (4.0%) were reclassified following the current WHO Classification of the Head and Neck Tumors. Conclusions: The general features of SGT from the studied Brazilian population were similar to previously published reports in other countries. However, SGTs do not show any sex predilection. Although careful morphological analysis is the key to the correct diagnosis of these tumors, immunohistochemical analysis is essential to establish an accurate diagnosis in the face of challenging cases. Key words:Salivary gland tumors, epidemiology, head and neck pathology.