Myoepithelial carcinoma is characterized by nearly exclusive myoepithelial differentiation and evidence of malignancy. It may arise de novo or in preexisting benign tumors including pleomorphic adenoma and benign myoepithelioma. A 39-year-old lady presented with painless progressive swelling on the right cheek and right side of palate. On surgery, there was a mass in right maxillary sinus which was surgically excised and diagnosed on histopathology as pleomorphic adenoma. Subsequently, there were two recurrences. The first recurrence was in the right maxilla after 2 years that was removed surgically and diagnosed as pleomorphic adenoma. One year later, she came with rapidly progressive swelling in bilateral cheeks and face. Intraoperatively, there was a large tumor in both maxillary sinuses with extensive local infiltration. Histologically, it was diagnosed as myoepithelial carcinoma. Carcinoma ex pleomorphic adenoma is usually a high grade malignancy. It occurs most commonly in parotid gland followed by submandibular glands, minor salivary glands and occasionally in sublingual gland. To the best of our knowledge, this is the first case of myoepithelial carcinoma arising in a recurrent pleomorphic adenoma in the maxillary sinus.

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Langerhan's cell histiocytosis is an uncontrolled proliferation of dendritic cells. The involvement of skull base is rare. Variable clinical presentation and multi organ involvement often warrant a multidisciplinary approach for a successful diagnosis. We are reporting a case of 16-year-old male with sphenoid sinus Langerhan’s cell histiocytosis which presented as a sudden and painless loss of vision. It is a rare entity in the diagnosis of blindness. Delayed diagnosis and treatment can result in serious complications. The radiological features and management options are discussed with a review of the pertinent literature.

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Gastrointestinal stromal tumor (GIST) is the most common primary mesenchymal neoplasm of the gastrointestinal tract, and they are potentially malignant tumors with varied biological behavior. The objective of this study was to investigate the clinicopathological characteristics and prognostic factors of GISTs in our institution. Medical records of nine cases of GISTs diagnosed during January 2018 to March 2021 were reviewed. Details of patient demographics, clinical presentation, treatment details, and gross and histopathological features were noted. GISTs were commonly seen in stomach in 50–60-year age group with slight male preponderance. Abdominal pain was the most common symptom. Other sites encountered were colon, mesentery, peritoneum, and omentum. Most of the gastric GISTs were in low-risk group. Mean size of the tumor was 9.3 cm. Spindle cell type was the most common histological pattern with diffuse hypercellularity subtype. All cases were positive for CD117 (c-kit). One case was inoperable due to dissemination in abdominal cavity, surgery was done in all other cases, and imatinib therapy was given for high-risk cases postoperatively. GIST is uncommon with diverse clinical presentation. CD117 is a very sensitive marker for making a diagnosis of GIST. Successful management of GISTs requires complete surgical resection and adjuvant imatinib therapy for intermediate- and high-risk patient.