Background Ameloblastomas are low-grade neoplasms of odontogenic epithelium that account for about 1% of all oral tumours and about 10% of odontogenic tumours. Rarely, these tumours have a potential of distance metastasis, and once they do metastasize, they are termed as metastatic ameloblastoma. Case presentation A 24-year-old African man presented with a massive swelling on the right side of the face for 2 years in 2018. He was previously operated for a swelling in the right side of the maxilla in 2012, which was histologically diagnosed as ameloblastoma. Clinical and radiological evaluation revealed a massive maxillary tumour that had a local extension to the brain. X-ray and CT scan of chest pointed out to metastatic mass in the lungs. Histology of the recurrent tumour mass revealed it to be follicular ameloblastoma and CT-guided fine-needle aspiration cytology of the lung mass showed microscopic features of ameloblastoma similar to primary jaw tumour. Conclusion A Close and long-term follow-up is mandatory in patients diagnosed with ameloblastoma even years after primary resection.

Background Epithelioid sarcoma, a rare soft tissue malignancy, is clinically aggressive and commonly affects distal extremities. Proximal, fibroma-like, and angiomatoid variants are lightly outlined. The proximal variant is the most aggressive, composed of larger cells and rhabdoid features, characteristically captivating one’s attention to rhabdomyosarcoma. We present a rare epithelioid sarcoma that histologically mimicked pleomorphic rhabdomyosarcoma of jaw in a 31-year-old male. Objectives To discuss histopathological features of epithelioid sarcoma of rhabdoid features, distinguishing it from other histological mimics like rhabdomyosarcoma. Methods A 31-year-old male was admitted at Muhimbili National Hospital in October 2017 with a 4-month history of slow-growing tender ulcer on the lower right jaw and right-side facial swelling. Clinical impression was squamous cell carcinoma with differential of adenocarcinoma. Various laboratory investigations, imaging, routine staining, and immunohistochemistry were performed. Results CT scan revealed a heterogenous mass in the right submandibular region. Initial examination from incision biopsy showed large oval pleomorphic tumor cells with enlarged vesicular nuclei and abnormal mitoses. Wide excision biopsy followed after conclusion of poorly differentiated carcinoma. Its examination revealed a diffuse, nodular tumor with large, polygonal, round, spindle cells displaying abundant deeply eosinophilic cytoplasm, sometimes showing eccentric vesicular nuclei. Rhabdoid features were observed. Immunohistochemistry revealed strong positivity for wide-spectrum cytokeratin, vimentin, and CD99 and was negative for EMA and myogenin. Conclusion Morphology by routine stains suggested both epithelial and mesenchymal malignancy. Cells with rhabdoid features suggested pleomorphic rhabdomyosarcoma with differential of epithelioid sarcoma. Cytokeratin, vimentin, and CD99 confirmed the diagnosis of epithelioid sarcoma, proximal variant. This case adds information on this rare entity emphasizing application of diagnostic immunohistochemistry procedures in the platform for confirmation of diagnosis to assist in proper patient management.

Introduction: Ameloblastoma is a benign odontogenic tumor that may exhibit aggressive biological behavior with local recurrence. It more commonly occurs in the mandible than the maxilla. Rarely, ameloblastoma may metastasize to local regional lymph nodes and/or distant organs, notably lungs, brain, and skin, hence the term metastasizing (malignant) ameloblastoma (MA), in keeping with the WHO definition of MA in its recent classification of odontogenic tumors (2017). Here, we report a rare case of maxillary ameloblastoma that metastasized to the lungs in a 22-year-old African male. Radiological evaluation revealed a massive maxillary tumor that had a local extension to the brain. Chest x-ray and CT scan pointed out metastatic mass to the lungs. Histology of the tumor mass revealed it to be follicular ameloblastoma with aggressive behavior shown by abnormal mitoses and further characterization by immunohistochemical reactivity to Ki-67. CT-guided fine-needle aspiration cytology of the lung mass showed microscopic features of ameloblastoma bearing resemblance to the primary jaw tumor. Socioeconomic constraints on the patient’s side and limited medical resources necessitated planning that served the purpose of proper diagnosis and treatment options. In this paper, we discuss the clinical behavior, differential diagnosis, and challenges faced by clinicians in managing metastatic ameloblastoma in a resource-limited setting. Conclusively, reporting this rare case and first of its kind in our locality raises awareness, hence reducing misdiagnosis of primary tumor in sites other than the jaw, while also sharing our experience of managing metastasizing ameloblastoma in a resource-limited setting to the larger medical community.