Intraoral neural neoplasms though unusual may be clinically significant. The aim of this study was to categorize and evaluate oral neural tumors in a large oral pathology biopsy service. With IRB approval, a retrospective search of all neural neoplasms of the oral cavity in the archives of the University of Florida Oral Pathology Biopsy Service spanning from 1994 to 2015 was performed. Extraoral cases as well as cases with insufficient patient information were excluded. A total of 340 out of 164,578 submitted specimens in a 22 year period (0.2%) were included with a mean age of 43.3 years (range: 6-89), and 44% male and 56% female. The most commonly affected locations were: tongue (37.5%), palate (22%), lip (19%), and gingiva (14%). The microscopic diagnoses rendered, in descending order of frequency were: neurofibromas (NFs): 123 (36%), granular cell tumor (GCT): 108 (32%), schwannomas: 61 (17%), palisaded encapsulated neuromas: 39 (11%), benign neural lesion not otherwise specified: 8 (2%), and mucosal neuroma c/w multiple endocrine neoplasia type 2B (MEN 2B): 1 (< 0.5%). Six cases of NF reported a history of neurofibromatosis Type 1 (NF 1). Four cases showed multifocal lesions. Immunohistochemical staining was performed on equivocal cases (25% of the lesions) and all were confirmed by their S-100 positivity. Intraoral neural neoplasms, though uncommon should be in the differential diagnosis of oral soft tissue entities and specific consideration to syndromal linkage is paramount as this may impact patient management.

Objective:Rare disease Background:Schwannoma is an uncommon benign tumor that develops from Schwann cells in the peripheral nervous system; 25-50% of such tumors occur in the head and neck. A rare variety of schwannoma, known as an ancient schwannoma, exhibits calcification, cystic degeneration, hemorrhage, myxoid stroma, pleomorphism, and nuclear hyperchromatism. Such characteristics may contribute to this tumor being incorrectly diagnosed as malignant. Almost all reported intra-oral ancient schwannomas behave innocuously. Case Report:A 20-year-old man presented to the oral surgery clinic with a right facial swelling that began a few months before. CT and MRI were performed and showed a well-defined cystic lesion anterior to the right masseter muscle, located in the subcutaneous area and measuring 28×24×20 mm. No extension to the surrounding tissues nor any other similar lesions were seen. Microscopic examination revealed an encapsulated nodular mass with cystic cavities, hemorrhage, and variably distributed myxoid and hyalinized stroma. The tumor demonstrated 2 distinct cell distributions: hypercellular areas (Antoni A) and myxoid hypocellular areas (Antoni B). Focal areas in high-power magnification showed atypical cells with hyperchromatic and pleomorphic nuclei. The tumor cells showed diffuse positivity for S-100 in IHC. The diagnosis of ancient schwannoma was made. At 1-year follow-up, there was no evidence of nerve injury or recurrence. Conclusions:One more case of oral ancient schwannoma is added to the literature. This is the first reported in Saudi Arabia.It is prudent to be familiar with the degenerative and cytologic alterations of ancient schwannoma, as this may limit the likelihood of misdiagnosis and unnecessary treatment of this benign tumor, with an excellent prognosis.

Oral papillary squamous cell carcinoma (PSCC) is an unusual variant of squamous cell carcinoma with a better prognosis. The most common location of PSCC in the oral cavity is the gingiva and buccal mucosa, and it is exceedingly rare in the tongue. Herein, we present a case of PSCC in an 85-year-old male with a history of heart transplant and long-term use of immunosuppression medication. A verrucous pedunculated mass measuring 3.5 cm in the greatest dimension was present on the tip of tongue and a partial glossectomy was performed. Histological diagnosis was well differentiated PSCC with focal and minimal stalk invasion. No vascular nor perineural invasion was identified. Based on the current WHO and AJCC oral cancer staging system, the tumor stage was T2N0M0. The tumor cells were focally positive for p16, but in situ hybridization was negative for low-risk HPV (types 6 and 11) and high-risk HPV (types 16, 18, 31, 33 and 51). To the best of our knowledge, this is the first documented case of PSCC present on the tip of tongue in patients with long-term immune suppression. The pathogenesis, stage and prognosis of this entity are discussed. More case studies with long-term follow up are needed to achieve an accurate tumor stage and definite prognosis.

Challenging differential diagnosis Background:Carcinosarcoma of the sinonasal tract is an extremely rare malignant neoplasm; it is often designated as carcinoma with spindle cell or sarcomatoid features. We report a case of carcinosarcoma arising in a pre-existing inverted Schneiderian papilloma in the left maxillary antrum and nasal cavity of a 72-year old male patient. Case Report:The patient had a significant history of radiotherapy for squamous cell carcinoma in the sinonasal area, 3 decades ago. The patient presented with chief complaints of left nasal blockage, nasal discharge, anosmia, and occasional epistaxis. Computed tomography scan displayed a lobular soft tissue mass resulting in narrowing of the nasopharyngeal airway with massive destruction of palatal tissue. The lesion was resected via endoscopic surgery. Macroscopically, a white fleshy appearance with necrosis was noted in the submitted specimen. Microscopically, the tumor was composed of pleomorphic epithelial and spindle cells with numerous mitoses and remarkable tissue necrosis. Residual inverted papilloma (IP) with high-grade dysplasia, and minimal foci of moderately differentiated squamous cell carcinoma (SCC) component was present at the tumor margin.A distinct zone of transition of SCC to spindle cell carcinoma (SpSCC) was noted and confirmed by focal positivity of p63 in epithelial and sacromatoid components. The pleomorphic sarcomatoid tumor was positive for vimentin with Ki67 highlighting 70% of tumor cells. A final diagnosis of sinonasal spindle cell carcinoma associated with residual inverted papilloma was rendered. Conclusions:Due to the rarity of such cases, the prognosis and response to treatment is unclear. No effective directed treatment has been developed. Unfortunately, the patient refused any further treatment and died of persistent disease. To the best of our knowledge, only one case of sinonasal carcinosarcoma arising from dysplastic inverted papilloma has been reported. The distinct possibility of previous radiotherapy contributing to development of sarcomatoid features in this neoplasm should also be considered.

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