ABSTRACT
True malignant mixed tumors, also known as salivary gland carcinosarcoma (SCS), are uncommon yet highly aggressive lesions associated with a poor prognosis. These tumors exhibit a distinctive biphasic structure characterized by both epithelial and mesenchymal components. Recent research has shown that the majority of SCS cases stem from pre-existing pleomorphic adenomas (PAs), suggesting a stepwise developmental pattern. In this report, we present a case of a 73-year-old female with SCS and describe the clinical, radiographic, and pathologic observations. Notably, the SCS was associated with a residual PA. The SCS displayed a CTNNB1::PLAG1 gene rearrangement, providing a molecular basis for its origin from the PA. Further DNA genomic analysis exposed mutations in BAP1, PER1, and LRPB1. Our findings provide support to the theory that SCS emerges from a pre-existing PA while highlighting the multiple genetic changes that could contribute to malignant transformation.
Subject(s)
Adenoma, Pleomorphic , Carcinosarcoma , Humans , Female , Aged , Carcinosarcoma/genetics , Carcinosarcoma/pathology , Adenoma, Pleomorphic/genetics , Adenoma, Pleomorphic/pathology , Adenoma, Pleomorphic/diagnostic imaging , Salivary Gland Neoplasms/genetics , Salivary Gland Neoplasms/pathology , Salivary Gland Neoplasms/diagnostic imagingABSTRACT
A healthy but slightly pale 24-year-old female patient with a history of "turning blue" following dental procedures performed under local anesthesia claimed allergies to sulfa drugs, aspirin, Benadryl, and "all caines." The patient also acknowledged mild cyanosis after extreme exertion, Native American ancestry, and a 1996 diagnosis of methemoglobinemia following administration of a sulfa drug. Previous medical and dental records were reviewed. Restoration of several teeth and extraction of 2 third-molar teeth were completed under general anesthesia. Anesthesia was induced with propofol, nasotracheal intubation was accomplished with succinylcholine, and anesthesia was maintained with desflurane in oxygen supplemented by meperidine without local anesthesia. Vital signs, including pulse oximetry, remained stable, and the patient was dismissed after a 2-hour recovery/observation period. The patient experienced no postoperative complications. This case report provides a review of literature and clinical guidelines for management of methemoglobinemia-susceptible patients.
