Malignancies in Patients with IgG4-Related Diseases in Head and Neck Regions.

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized disease, characterized by high serum IgG4 concentrations and IgG4-producing plasma cell expansion with fibrotic or sclerotic changes in affected organs. Recent work has focused on the relationship between IgG4-RD and malignancies, but there is no report of malignancies associated with IgG4-RD in head and neck regions. The aim of this study was to analyze the clinicopathological characteristics of malignancies in patients with IgG4-RD in head and neck regions. We retrospectively analyzed 26 patients with IgG4-RD (12 men and 14 women aged 60.6 ± 11.6 years). The mean follow-up period was 26.6 months (from 12 to 96 months). These patients were divided into single-lesion group (n = 12) with IgG4-RD only in head and neck regions and multiple-lesion group (n = 14) with IgG4-RD in other regions. There was no significant difference in serum IgG4 concentrations between the single-lesion group (459.4 ± 336.4 mg/dL) and the multiple-lesion group (908.0 ± 739.2 mg/dL) (P = 0.07), whereas the IgG4/IgG ratio was significantly lower in the single-lesion group (22.8 ± 11.0%; n = 11) compared with the multiple-lesion group (31.7 ± 15.0%; n = 11, P = 0.02). Among the 26 patients, two patients (7.7%), both in the multiple-lesion group, developed life-threatening malignancies (salivary duct carcinoma in the submandibular gland and lymphoma in the orbital tissue). All physicians need to keep in mind the possible coexistence of malignancies in patients with IgG4-RD with high IgG4/IgG ratio and multiple lesions at the time of diagnosis.

MUC expression in adenosquamous carcinoma of the head and neck regions of Japanese patients: immunohistochemical analysis.

Adenosquamous carcinoma (ASC) is a rare malignant neoplasm of the head and neck regions. We elucidated the relationship between ASC and MUC molecule expression. We selected 14 cases of ASC in the head and neck, and examined them immunohistohcmically. Seven cases of tongue, 3 cases of larynx and 4 cases of hypopharynx carcinoma were selected. Nine (64.3%) of 14 cases showed lymph node metastasis at the diagnosis. Laryngeal and hypopharyngeal cases showed a higher stage. Six cases (66.7%), all of which showed lymph node metastases, died of disease. Immunohistochemical examinations showed that ASC was positive for both markers of squamous cell carcinoma and adenocarcinoma. ASC showed positivity for MUC1 (13 cases: 92.3%), MUC1core (12 cases: 85.2%) and MUC4 (12 cases: 85.7%). In rare ASC of the head and neck region, it was revealed that MUC1 and MUC4 expression might be associated with its nodal status.

An Extremely Rare Epithelioid Sarcoma Arising from the Infratemporal Fossa: A Case Report and Literature Review.

INTRODUCTION: Epithelioid sarcoma (ES) is a rare histopathological type of soft tissue sarcoma. They are subcutaneous soft tissue masses that tend to arise in extremity sites (the classic type, formerly referred to as distal type) or proximal midline region of the body (the proximal type), such as the perineum, genital tract, and pelvis. The head and neck regions are rarely affected by ES, but the infratemporal fossa (ITF) is extremely rare. ES involving the ITF has not been reported before in literature. In this paper, the imaging features of ES were reviewed in detail, and the anatomical structure and epidemiology of ITF were briefly introduced. SOURCES: We performed a systematic search from 3 databases, CNKI(China National Knowledge Internet), FMRS(Foreign Medical Literature Retrieval Service, Shenzhen METSTR Technology CO., Led. China), and PubMed, to obtain literature from January 1970 to July 2020. Epithelioid sarcoma, head and neck regions, infratemporal fossa, diagnostic imaging, anatomy, MRI, and CT were used as keywords for advanced retrieval. A study had to be found eligible for inclusion to be closely related to ES and/or ITF. A total of 129 related pieces of literature were retrieved successfully, of which 37 were closely related to this study. The case report in this article is from the Affiliated Hospital of North Sichuan Medical College. The present study was approved by the institutional review board of the Affiliated Hospital of North Sichuan Medical College and a written informed consent for the publication of the case was obtained from the patient. CASE PRESENTATION: A 33-year-old man accidentally found a slowly growing, painless mass in the left cheek more than a month ago. On plain computed tomography (CT) scan of the outpatient department, an oval slightly low-density mass with well-defined and uniform boundary in the infratemporal fossa was revealed, and on the contrast-enhanced scan, it was homogeneous and moderately enhanced. Initially, the CT appearance favored benign lesions. However, the possibility of a malignant tumor can not be excluded completely. Finally, the patient was referred to the inpatient department a tumor arose from IF and underwent a selective operation. The tumor was completely removed. Histologic findings were compatible with epithelioid sarcoma. Post-operatively, the patient received 2 cycles of radiotherapy, and there was no evidence of recurrence after 6 months follow- up. CONCLUSION: The imaging manifestations of ES are various. The anatomic structure of ITF is complex and the pathological types are various. It should be very careful in the qualitative diagnosis of tumors from the ITF, and advanced imaging techniques will be useful in imaging diagnosis.