Oral Manifestations of Syphilis: a Review of the Clinical and Histopathologic Characteristics of a Reemerging Entity with Report of 19 New Cases.

BACKGROUND: Syphilis is a sexually-transmitted infectious disease caused by Treponema pallidum. Cases of primary and secondary syphilis are on the rise in the United States, with a 14.4% increase in new cases noted from 2017 to 2018 and an escalation of 71% between the years 2014 and 2018. Fulfilling its nickname of "the great imitator," oral manifestations of syphilis may mimic a variety of infectious, neoplastic, or immune-mediated processes, both clinically and histopathologically. This large spectrum of appearances can create a diagnostic challenge to the clinician and/or pathologist, leading to delay in diagnosis or misdiagnosis. METHODS: A database of oral syphilis cases was created from archives at the University of Kentucky, University of Pittsburgh, LIJMC, Columbia University MC, and University of Tennessee. The age, sex, race, location, duration, and clinical description were recorded. Cases without positive reaction upon immunohistochemistry or serologic tests were excluded. RESULTS: We identified 19 new cases of oral syphilis (17 males, one female, and one case unknown sex) and described the clinical and histopathological features of this re-emerging and potentially fatal disease. All cases demonstrated dense lymphoplasmacytic inflammation, often with inflammatory exocytosis or ulceration at the surface, and perivascular inflammation. CONCLUSIONS: Early recognition of the histopathologic and clinical manifestations of oral syphilis is imperative for prompt diagnosis, improved patient outcomes, and disease prevention.

Extra Nodal Rosai-Dorfman Disease Originating in the Nasal and Paranasal Complex and Gnathic Bones: A Systematic Analysis of Seven Cases and Review of Literature.

Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. The classic form of the condition includes a painless cervical lymphaenopathy accompanied by fever, weight loss and an elevated ESR. Extra nodal RDD (ENRDD) is most frequent in the head and neck. Thirty-eight cases of ENRDD have been described. Seven cases of ENRDD were identified in our pathology biopsy services. The demographic and clinical information was tabulated logically on the basis of age, gender, location and presence or absence of symptoms, treatment and follow-up. Radiographic and histopathological features were also examined. The findings in these cases were correlated with those available from the previously reported cases. Six cases affected women and one case was diagnosed in a male. The age ranged from 22-55 years. Three cases presented as a nasal mass. One of these lesions extended into the paranasal sinuses. One case was located in the maxilla and extended to involve the maxillary sinus. Three cases were diagnosed in the mandible. The maxillary and one mandibular lesion (Case 2) resulted in significant painful irregular bone destruction with a non-healing socket and tooth mobility respectively. One mandibular lesion was asymptomatic (Case 6). The third case affecting the mandible presented as a rapidly expansile mass following a tooth extraction (Case 7). Nasal masses presented with symptoms of obstruction. Nasal masses were excised with no recurrence from up to 2-3 years of follow-up. The mandibular lesions were curetted aggressively. The oral mass in Case 7 was excised synchronously. No recurrence up to 2 years was recorded in Case 2. Follow-up information is not available for Cases 6 and 7. The maxillary lesion was not intervened surgically. The patient has persistent but stable disease for a follow-up period of 2 years. ENRDD is rarely considered in the differential diagnosis in the absence of lymph node involvement. Lesions of ENRDD resemble many other histiocytic and histiocyte-rich lesions of the head and neck. This makes the diagnosis of ENRDD challenging with the potential for under diagnosis or misdiagnosis and delay in treatment.

Angioleiomyoma (Vascular Leiomyoma) of the Oral Cavity.

A 70-year-old male presented with a slow growing, dome shaped and painless mass of the hard palate. The mass was excised. Histopathological examination confirmed the diagnosis of a angioleiomyoma (vascular leiomyoma). A leiomyoma is an uncommon benign tumor of smooth muscle differentiation. True leiomyomas of the oral cavity are rare and most oral tumors are derived from the smooth muscle of walls of blood vessels. Therefore, they are called vascular leiomyomas or angioleiomyomas. Clinically, they may resemble a myriad other conditions both benign and malignant. A definitive diagnosis depends upon histopathological examination of the biopsied tissue in correlation with the tumor cell immunohistochemistry. Tumors are excised and recurrence is rare. The histopathological findings and differential diagnosis of a case of a palatal angioleiomyoma are discussed.

Giant Pilomatrix Carcinoma of the Face.

This case report describes an unusually large facial pilomatrix carcinoma (PC) in a 60-year-old man. This PC had been growing slowly for 11 months and had recently ulcerated. It measured 9 cm × 7 cm × 5 cm. Initial punch biopsy findings were inconclusive. The tumor was excised in total with 5-mm margins in a supra-superficial musculoaponeurotic system plane. The defect was reconstructed with a cervicofacial bilobed rotational advancement flap. Histopathologic findings confirmed the diagnosis of PC. There was no lymph node spread or distant metastasis. The patient recovered with a good esthetic result. This case represents one of the largest facial PCs reported in the literature. Diagnostic challenges are discussed, and the literature is reviewed.

Intraosseous Hemangioma of the Frontal Bone. Report of a Case and Review of the Literature.

Intraosseous hemangiomas of the maxillofacial region are rare lesions that constitute less than 1% of all osseous tumors. A review of the literature on intraosseous hemangiomas of the skull and facial bones showed a limited number of publications, much of which were largely limited to case reports. This case report summarizes the workup and surgical treatment of a 39-year-old woman with an intraosseous hemangioma of the left frontal bone. The histology, treatment, and literature are reviewed.

Adenosquamous Carcinoma of the Tongue.

A 51-year-old white female presented with a painful ulcer of the left ventrolateral tongue. An incisional biopsy confirmed a diagnosis of adenosquamous carcinoma. The adenosquamous carcinoma is an uncommon malignant tumor with histopathological features of a squamous carcinoma and an adenocarcinoma. A definitive diagnosis requires histopathological examination of a deep biopsy involving the submucosal tissue. This malignant tumor shows aggressive behavior with early invasive growth and a poor prognosis. The histopathological findings and differential diagnosis of a case of adenosquamous carcinoma of the tongue are discussed.

Divergent Schwannoma-Like Phenotype in a Pleomorphic Adenoma.

The schwannoma-like pleomorphic adenoma is a rare histopathological variant of the pleomorphic adenoma. Five previous reports with seven cases exist in English language literature. These tumors present in the parotid gland most commonly. Intraparotid schwannomas of the facial nerve and schwannomas with glandular differentiation have also been reported. A 60-year-old male presented with an asymptomatic swelling over the left angle of the mandible. The swelling had been present for about 12 years with a recent increase in size. CT imaging showed a hyperdense circumscribed mass of the superficial lobe of the parotid. The working diagnosis was that of a benign tumor of salivary gland or soft tissue origin. The mass was excised with careful preservation of the facial nerve. The 3.5 cm mass was submitted for histopathological examination. The well-circumscribed, encapsulated mass showed a predominant sheet-like proliferation of Antoni type A-like tissue, Foci of glandular differentiation with duct-like structures were also seen. Cytological atypia or mitotic activity were not seen. Nuclei of lesional cells diffusely and strongly expressed reactivity to p63. The final diagnosis was a schwannoma-like pleomorphic adenoma. No recurrence has been reported in the 15 months since the removal. Facial nerve function is unimpaired with a House Brackmann facial nerve function score of one. The potential for misdiagnosis in fine needle aspirate and incisional biopsies is real in cases of schwannoma-like pleomorphic adenoma. The diagnostic pitfalls include the schwannoma and leiomyoma. Schwannomas with glandular differentiation have also been reported and therefore a misdiagnosis may potentially occur in excised specimens. Careful application of immunohistochemistry may help in the differentiation of these lesions.

Central Xanthoma of the Jaw Bones: A Benign Tumor.

Extragnathic xanthomas are seen in the bones or as soft tissue masses. They are often associated with hyperlipidemia and are considered as reactive or metabolic lesions. Only 19 cases of xanthomas of the jaws have been reported so far in the English literature. A total of ten cases of central xanthoma of the jaw bones were identified from the Oral and Maxillofacial Pathology biopsy services of the University of Washington and the Tufts University School of Dental Medicine, between the years 2000-2016. The demographic and clinical information on these cases was tabulated logically on the basis of age, gender, location and presence or absence of symptoms, extragnathic lesions and serum hyperlipidemia. Radiographic and histopathological features were also examined. The findings in these cases were correlated with those available from the previously reported cases. Majority of cases are seen in the second and third decades of life. There is no gender predilection. Jaw lesions presented as solitary radiolucencies with a predilection for the posterior mandible. Unlike maxillary lesions, pain and expansion are inconsistent findings in mandibular lesions. Jaw lesions are not associated with extragnathic bone or soft tissue involvement or a hyperlipidemia. The central xanthoma of the jaws is a unique benign tumor. Histopathologically, many other jaw lesions contain variable numbers of foamy histiocytes. Therefore, a diagnosis of a central xanthoma of the jaws must be made after excluding all other such histiocyte containing lesions. This requires correlation of histopathological findings with clinical and radiographic features.

Oral melanoma: Relevance to the dental team members.

BACKGROUND AND OVERVIEW: Oral melanomas vary in color and morphology and resemble myriad other reactive, benign, or malignant conditions. The authors describe a case report of a patient with a primary oral melanoma that presented as a nonspecific ulcer, which showed nodal metastasis during resection. CASE DESCRIPTION: A 64-year-old man who was examined by his periodontist to assess implant healing had a reddish-purple ulcer of the maxillary mucosa of 3 to 4 weeks duration. The implant was placed 19 weeks earlier in the mandible. The provisional diagnosis was that this ulcer was a traumatic or inflammatory lesion. The clinician biopsied the tissue at the 1-week follow-up appointment, which was identified as melanoma. The patient had a partial maxillectomy and ipsilateral neck dissection. Tissue examination showed nodal metastasis. Two months later, contralateral neck node metastasis was diagnosed and treated. CONCLUSIONS AND PRACTICAL IMPLICATIONS: In contrast to cutaneous melanoma, oral melanoma has a poor prognosis because of delayed diagnosis. Thorough oral examination at each dental visit may improve the outcome of this fatal condition.

Pseudomyogenic Hemangioendothelioma: A Vascular Tumor Previously Undescribed in the Oral Cavity.

The pseudomyogenic hemangioendothelioma (PMH) is a low-grade malignant vascular neoplasm of different tissue planes including skin and soft tissue. Primary tumors in the skeletal muscle and bone have also been diagnosed. The PMH was introduced into the WHO classification of tumors of soft tissue and bone in 2013. This is the first description of oral involvement. A 21-year-old female presented with a 2-month old swelling of her gingiva. The swelling appeared red in color and was soft in consistency. A clinical diagnosis of a pyogenic granuloma was made and an incisional biopsy was submitted for histopathological evaluation. The lesion consisted of a proliferation of spindle and epithelioid looking cells. Cells were arranged in loose fascicles and sheets. Rhabdomyoblast-like cells were also seen. No mitotic figures were present. Lesional cells were reactive to cytokeratin AE1/AE3 and CD31. Lesional cell reactivity to S100 protein, HMB 45, SMA, Desmin and CD34 was negative. Following the diagnosis, a wide excision for clear margins was performed. No recurrence has been reported 2 years since the removal. The PMH is a cutaneous tumor that behaves in an indolent fashion. This is the first report of oral involvement by this neoplasm. Recognition of its histopathological features and immunohistochemical reactivity will prevent misadventures in the diagnosis of oral lesions.

S-100 Negative Granular Cell Tumor (So-called Primitive Polypoid Non-neural Granular Cell Tumor) of the Oral Cavity.

Four cases of cutaneous S-100 negative granular cell tumor were described in 1991. Until now, only 3 cases of oral involvement have been documented in English literature. Two additional cases of oral S-100 negative granular cell tumor are described. Immunohistochemical markers were applied to exclude other lesions that may show the presence of granular cells. The clinical findings were correlated with the histopathological and immunohistochemical features to arrive at the appropriate diagnosis. S-100 negative granular cell tumors are erythematous polypoid masses commonly mistaken for granulation tissue or a pyogenic granuloma. Any part of the oral cavity may be affected. Histopathologically, the lesions consist of sheets, nests, and fascicles of granular cells that are S-100 negative. The granular cells are non-reactive to SMA, HMB45, Melan A, and CD163. The intracytoplasmic granules are diffusely and strongly positive to NKI/C3. The cell lineage of the S-100 negative granular cell tumor is obscure. Absence of staining with CD163 excludes a histiocytic lineage. Absence of staining with S-100 excludes a neural origin. Absence of staining with S-100 and key melanoma markers HMB45 and Melan A also excludes a melanocytic origin. In this context, positive reactivity with NKI/C3 is indicative of presence of intracytoplasmic lysosomal granules only. Greater awareness of this lesion in the oral cavity will result in better characterization of its biologic potential.

Oral atypical cellular blue nevus: an infiltrative melanocytic proliferation.

The atypical cellular blue nevus is an extremely rare nevomelanocytic lesion which lacks precise histologic characterization in the current literature. Given the potential for significant architectural and cytologic overlap with melanoma, further study, including molecular analysis, is needed. This is the first description of an atypical cellular blue nevus of the oral cavity.

Mandibular abnormalities in a patient with neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by the presence of cutaneous neurofibromas, multiple cafd-au-lait spots and pigmented nodules of the iris known as Lisch nodules. In some cases, the diagnosis can be made at birth while in others the diagnosis is made later in life based on the appearance of additional criteria. We describe radiographic abnormalities of the mandible in a young adult male with NF1.

Slow-growing gingival mass.

OBJECTIVES: A 33-year-old woman presented with a slow growing palatal gingival mass. The clinical differential diagnosis included benign tumors and tumor-like lesions, including the pyogenic granuloma, peripheral giant cell granuloma, peripheral ossifying fibroma, giant cell fibroma, peripheral odontogenic tumors, and oral focal mucinosis. STUDY DESIGN: The lesion was excised and histopathological examination followed by immunohistochemical staining was carried out. RESULTS: The microscopic findings and the immunohistochemical reactivity was diagnostic for a nerve sheath myxoma. CONCLUSIONS: The clinical features, microscopic findings, immunohistochemistry, and the differential diagnosis including the relationship to the neurothekeoma are discussed.

Intraoral phaeohyphomycosis.

Phaeohyphomycosis is an infection caused by pigment-producing saprophytic fungi. Systemic infections may occur in the immunocompromised patient. Infection in healthy individuals may result in subcutaneous abscess formation. Oral lesions appear to be rare. A case of intraoral phaeohyphomycosis presenting as a well-demarcated, painful nodule of the anterior hard palate in a 12-year-old healthy male is described. The mass was excised and the diagnosis was established following histopathologic examination of the tissue.

The epithelioid blue nevus: A rare intraoral nevomelanocytic tumor.

The epithelioid blue nevus (EBN) is considered a cutaneous marker of the Carney complex. Sporadic EBN has been reported in patients not exhibiting the Carney complex. The EBN does not exhibit unique clinical features that help to differentiate it from other lesions and is often provisionally diagnosed as an acquired melanocytic nevus, or a malignant melanoma. A 52-year-old African-American female had a 3-4 mm bluish macule of the left anterior hard palate. An excisional biopsy was performed to rule out an incipient melanoma. Formalin-fixed, hematoxylin and eosin-stained sections were examined microscopically. On the basis of histopathological features, a diagnosis of EBN was rendered. We document a case of the rare EBN affecting the oral mucosa. The patient did not exhibit any features associated with the Carney complex. Two years post-operatively, there is no evidence of a recurrent tumor.

Basal cell carcinoma of the buccal mucosa in a patient with nevoid basal cell carcinoma syndrome.

Basal cell carcinoma (BCC) of the oral cavity is a controversial lesion with clinical and histopathologic features that overlap with those of peripheral ameloblastoma (PA). Ber-EP4, a cell surface glycoprotein preferentially expressed in BCC of the skin, has been suggested as a useful marker to support the diagnosis of oral BCC.This study presents a case of intraoral BCC arising in the anterior buccal mucosa of a patient with nevoid basal cell carcinoma syndrome (NBCCS), which represents a previously unreported clinical finding, to our knowledge. Histopathologic and immunohistochemical features of the case were compared to examples of PA, conventional intraosseous ameloblastoma, sporadic cutaneous BCC and cutaneous BCC from NBCCS patients. Ber-EP4 expression by the oral tumor was distinct from both peripheral and intraosseous forms of ameloblastoma and was identical to cutaneous BCC in both sporadic and syndromic settings.