Primary Intraosseous Adenoid Cystic Carcinoma with Widespread Skeletal Metastases Showing Features of High-Grade Transfo
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Primary Intraosseous Adenoid Cystic Carcinoma with Widespread Skeletal Metastases Showing Features of High‑Grade Transformation Vindhya Savithri1 · Rakesh Suresh1 · Mahija Janardhanan1 · Thara Aravind1 · Mridula Mohan1 Received: 16 August 2020 / Accepted: 16 September 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Malignant salivary gland carcinomas arising primarily within the jaw bones are extremely rare. The most common salivary malignancy in these locations is mucoepidermoid carcinoma followed by adenoid cystic carcinoma and adenocarcinoma. The clinical picture and imaging studies of these malignancies may be confused with odontogenic lesions which are more common in this location. Adenoid cystic carcinomas have a prolonged clinical course, tendency for perineural invasion and distant metastasis and multiple recurrences. The diagnosis of these tumors requires thorough histopathologic examination. Immunohistochemical studies may be required in cases showing solid growth pattern. High-grade transformation, earlier termed as dedifferentiation, has been observed in several salivary gland carcinomas including adenoid cystic carcinoma. These transformed tumors are reported to have an extremely poor prognosis. Here, we report a case of primary intraosseous adenoid cystic carcinoma with extensive skeletal metastases which showed a negative staining with p63 and positive staining with CD117. The tumor had a predominant solid growth pattern with areas indicative of high-grade transformation. A negative p63 staining may indicate an incomplete or focal loss of abluminal layer and this is one of the criteria for high-grade transformation in adenoid cystic carcinoma. Keywords Adenoid cystic carcinoma · Salivary gland neoplasms · Head and neck neoplasms · Mandible · Humans · Female · Immunohistochemistry · Paraesthesia
Introduction Malignant salivary gland tumors are uncommon accounting for only 3% to 5% of all head and neck neoplasms [1]. Mucoepidermoid carcinoma and adenoid cystic carcinoma (ACC) are the commonly seen salivary gland malignancies. Salivary gland carcinomas located centrally within the mandible are rare, comprising less than 0.4% of all salivary gland carcinomas [2]. Primary intraosseous adenoid cystic carcinoma (PIACC) of the jaws is extremely uncommon. ACC typically presents with slow, indolent growth, but there is a high incidence of local recurrence and metastasis, which results in the low long-term survival rate [3]. The lung and bone are the most common sites of distant spread, but the extremely slow growth pattern of the tumor and the gradual * Vindhya Savithri [email protected] 1
Department of Oral Pathology & Microbiology, Amrita School of Dentistry, Amrita Vishwa Vidyapeetham, AIMS Campus, AIMS Ponekkara PO, Kochi 682041, Kerala, India
appearance of metastasis allow patients to lead a normal life for many years [3]. We report a case of a female patient who presented with jaw swelling accompanied by pain and paraesthesia. A diagnosis of PIACC
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