Background: Pleomorphic adenoma (PA) is a benign tumor most common in major salivary glands. PA of pterygomaxillary space (PMS) is rare. Only one case of PA arising from the pterygopalatine fossa (PPF) was reported. We are presenting a second case, its presentation and surgical management, which is different and less morbid from the recently described reports. Case description: A 40-year-old female presented with ill-defined right cheek swelling for the last 5 years. On evaluation, there was a firm mass in the PMS, which was diagnosed as PA. Surgical excision was planned with sublabial incision combined with lip split, which could entirely reach, delineate, and remove the tumor in toto. The patient has recovered well and was discharged on the 3rd day. Conclusions: Pleomorphic adenomas of PMS present late due to their anatomical location and indolent course. Contrast-enhanced computed tomography scan is an important diagnostic tool. Fine-needle aspiration cytology from the buccal aspect reveals the probable histopathology. Sublabial incision with lip split is more useful in excising the moderate-sized tumors.

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Case PresentationA 53 year old white male presented with complaints of left-sided headaches radiating to the posterior left neck. The patient described the pain as intermittent and dull in nature and also mentioned some worsening of disequilibrium but denied vertigo. CT and MRI head revealed a retropharyngeal mass with invasion of virtually the entire skull base, including the clivus, medial temporal bones, as well as the medial sphenoid bones and a mass within the sphenoid sinus. HEENT examination was unremarkable. There were no other focal neurologic deficits.Diagnostic ProceduresFlexible laryngoscopy: fullness in the area of adenoids. Image-guided biopsy with transsphenoidal approach.PathologyA densely cellular neoplasm. Sheets of cells have hyperchromatic nuclei, occasional nucleoli, and rims of cytoplasm. There are scattered mitoses.ImmunohistochemistryCD138 positive, lamda positive, kappa negative.Final Pathologic Diagnosis1. Sphenoid sinus mass; plasmacytoma.Treatment CourseBMA, plasma cells 5-10%. Bone survey; no lytic lesions. S. proteins 7.6 g/dL, Alb. 4.4 g/dL. B2mic. 2.3 mg/L. SPEP IgG 1.04 g/dL, UPEP negative. At present the patient is receiving radiation therapy. We will obtain post radiation CT head to assess the response to therapy.DiscussionThe diagnosis of an extramedullary plasmacytoma depends upon the demonstration of a monoclonal plasma cell tumor at an extramedulary site with no evidence of MM based upon bone marrow examination and roentgenographic studies, absence of an M-protein in serum and/or urine, and no related organ or tissue impairment. The treatment of choice for extramedullary plasmacytoma is tumoricidal radiation in a dosage of 40 Gy over a four-week period. Adjuvant chemotherapy does not lower the relapse rate or increase disease-free survival in this group of patients.

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Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour that can occur in a wide variety of anatomical sites including the liver, lungs, skin, bone and is rarely reported in the oral cavity. The condition is classified as a tumour of intermediate malignancy as, in contrast to other types of haemangioendothelioma, EHE can result in distant metastasis and the death of the patient. Due to the minimal number of cases reported of individuals diagnosed with EHE, no definitive data on different treatment options exist and complete excision with follow up and consideration of adjuvant radiotherapy is the universal treatment option. This case report highlights the investigation, diagnosis and treatment of a 25-year-old man who was diagnosed with EHE arising within the mandible. The article also presents the published literature of documented cases of mandibular and maxillary EHE.