Intestinal diffuse ganglioneuromatosis is a rare, benign, neoplastic condition characterized by disseminated proliferation of neural elements. It has an established association with several systemic disorders including: multiple endocrine neoplasia IIB (MEN IIB) and von Recklinghausen’s disease. However, isolated GNs are very rare and sporadic. We report a case of a 6-year-old patient was referred to the emergency department due to a colon perforation during endoscopy. He then underwent sigmoidectomy and the pathologic studies revealed prominent signs of diffuse ganglioneuromatosis. Unfortunately, the patient died the day after because of the late diagnosis and treatment. Such presentation emphasizes the importance of considering diffuse ganglioneuromatosis, among other causes, in patients with intractable GI symptoms despite medical treatment.

Ameloblastic fibrosarcoma (AFS) is a rare, aggressive malignant odontogenic tumor. AFS is seen most frequently in second and third decades of life. We are reporting a case of a low grade AFS in a 21-year-old male complaining of a painless swelling in the right side of the maxilla. The patient was treated with surgical excision followed by radiotherapy, which is considered the most effective approach for most of soft tissue sarcomas. AFS has a high-reported recurrence rate (up to 37%); therefore, long-term surveillance for recurrence is crucial.