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We report clinical, imaging and pathology findings of a patient with leprosy of the great auricular nerve who presented with palpable nodules and numbness of the left ear and scalp. Ultrasound and contrast-enhanced CT imaging of the neck demonstrated diffuse enlargement and enhancement of the great auricular nerve. Biopsy of the enlarged left great auricular nerve revealed granulomatous inflammation with necrosis involving the nerve with rare Fite-positive organisms compatible with mycobacterium. Positive PCR for Mycobacterium leprae of the surgical specimen confirmed diagnosis of Hansen's disease.

We report a case of a 77-year-old female with purulent chondritis of the thyroid cartilage who was initially referred for laryngeal neoplasm. Purulent chondritis of the laryngeal cartilage is a rare entity with three reports in the literature. The unique CT imaging features of expansile laryngeal cartilage with peripheral rim enhancement and central fluid-attenuation correlate to the abscess formation between the inner and outer perichondria. The correct imaging assessment prompts surgical management and avoid misdiagnosis.

Node positive head and neck squamous cell carcinomas (HNSCCs) patients exhibit worse outcomes in terms of regional neck control, risk for distant metastases and overall survival. Smaller non-palpable lymph nodes may be inflammatory or may harbor clinically occult metastases, a characterization that can be challenging to make using routine imaging modalities. Ferumoxytol has been previously investigated as an intra-tumoral contrast agent for magnetic resonance imaging (MRI) for intracranial malignancies and lymph node agent in prostate cancer. Hence, our group was motivated to carry out a prospective feasibility study to assess the feasibility of ferumoxytol dynamic contrast enhanced (DCE)-weighted MRI relative to that of gadolinium-based DCE-MRI for nodal and primary tumor imaging in patients with biopsy-proven node-positive HNSCC or melanoma. Although this institutional review board (IRB)-approved study was prematurely terminated because of an FDA black box warning, the investigators sought to curate and publish this unique dataset of matched clinical, and anatomical and DCE MRI data for the enrolled five patients to be available for scientists interested in molecular imaging., Measurement(s)imaging assay • head and neck squamous cell carcinomaTechnology Type(s)magnetic resonance imagingFactor Type(s)contrast agentSample Characteristic - OrganismHomo sapiens Machine-accessible metadata file describing the reported data: 10.6084/m9.figshare.11409516

Key Clinical Message This study highlights gamma knife stereotactic radiosurgery (GK‐SRS) as boost therapy in a patient with adenoid cystic carcinoma of the parotid involving the skull base and invasion of the facial nerve. Using GK‐SRS, dose to the brainstem and temporal lobe were reduced when compared to less conformal radiotherapy techniques.

We report the imaging and clinical findings in 3 patients with osteoradionecrosis of the styloid process, an uncommon consequence of radiation therapy for head and neck malignancy.

Nasopharyngeal carcinoma (NPC) is a head and neck cancer arising out of the epithelium of the nasopharynx. There is an annual incidence of 0.5–2.0 cases per 100,000 in the US and Europe, although numbers as high as 25 per 100,000 may be seen in endemic countries such as China 1. Males are at an increased risk and while there has been evidence of a link to dietary practices in endemic areas, the vast majority of data suggest Epstein–Barr virus (EBV) as the predominant pathogenic factor in NPC 2, 3. Given the association with EBV, the histology and natural history of NPC differ from most other cancers of the head and neck. Treatment of early and locally advanced disease is typically nonsurgical because of the proximity of nearby vital structures. Thus, there is a strong role for noninvasive therapy in the form of radiation ± chemotherapy, which is considered standard of care for this disease 4. Detection of nodal metastasis in the neck or spread to adjacent structures including the skull base, nasal cavity, sphenoid bones, pterygoids, prevertebral muscles, and oropharynx with occult or frank primary disease is relatively common in NPC 5, 6. In contrast, intracranial extension of NPC is not as common especially in the absence of an obvious primary lesion 7. In this case report, we discuss a patient with a diagnosis of NPC that presented with extensive skull base disease and metastasis to the right temporal lobe with subtle findings in the nasopharynx.

OBJECTIVE The aim of this study was to present the imaging findings of pleural metastases from head and neck cancer and correlate them with prognosis for overall survival, which to our knowledge have not received much attention in radiology literature. METHODS We reviewed 23 cases of head and neck cancer with pleural metastases that were seen on head and neck imaging. Computed tomography (CT), magnetic resonance imaging, and positron emission tomography/CT imaging findings were reviewed. The location and cell type of the primary cancer were studied. The effect of the pleural metastases on patient survival was analyzed. RESULTS Twelve (57%) of the 21 cases of pleural metastasis were missed initially on the neck CT but were visible in retrospect. Nine of 11 patients whom we had followed up for 1.5 years or longer have died, often within a few of months of presenting with pleural metastases. CONCLUSIONS The presence of pleural metastasis in head and neck cancer is an extremely poor prognostic indicator for survival. They are potentially missed on the neck CT. Given its significant clinical impact, head and neck radiologists should specifically look for pleural metastasis when reviewing imaging studies of patients with head and neck cancer.

Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in Western countries with an incidence of 3-5 cases per 100,000 persons. Most patients follow an indolent clinical course with eventual progression and need for therapy. In contrast, T-prolymphocytic leukemia (T-PLL) is a rare type of T-cell leukemia with most patients having an aggressive clinical course and a dismal prognosis. Therapies are limited for T-PLL patients and there is a high relapse rate. Morphologically, the cells of CLL and T-PLL can show overlapping features. Here, we report the case of a 61-year-old man who presented with a clinically indolent CLL and T-PLL, initially diagnosed solely and followed as CLL, despite the presence of an associated but unrecognized aberrant T-cell population in blood. After 2 years, the T-PLL component became more apparent with cutaneous and hematologic manifestations and the diagnosis was confirmed by immunophenotypic and cytogenetic analysis. Fluorescencein situhybridization demonstrated anATMdeletion in both CLL and T-PLL components. Retrospective testing demonstrated that composite CLL and T-PLL were both present in skin and lymph nodes as well as in blood and bone marrow since initial presentation. This case is also unique because it highlights that a subset of T-PLL patients can present with clinically indolent disease. The concomitant detection ofATMmutation in CLL and T-PLL components raises the possibility of a common pathogenic mechanism.

Background The survival advantage of induction chemotherapy (IC) followed by locoregional treatment is controversial in locally advanced head and neck squamous cell carcinoma (LAHNSCC). We previously showed feasibility and safety of cetuximab-based IC (paclitaxel/carboplatin/cetuximab—PCC, and docetaxel/cisplatin/5-fluorouracil/cetuximab—C-TPF) followed by local therapy in LAHNSCC. The primary end point of this phase II clinical trial with randomization to PCC and C-TPF followed by combined local therapy in patients with LAHNSCC stratified by human papillomavirus (HPV) status and T-stage was 2-year progression-free survival (PFS) compared with historical control. Patients and methods Eligible patients were ≥18 years with squamous cell carcinoma of the oropharynx, oral cavity, nasopharynx, hypopharynx, or larynx with measurable stage IV (T0–4N2b–2c/3M0) and known HPV by p16 status. Stratification was by HPV and T-stage into one of the two risk groups: (i) low-risk: HPV-positive and T0–3 or HPV-negative and T0–2; (ii) intermediate/high-risk: HPV-positive and T4 or HPV-negative and T3–4. Patient reported outcomes were carried out. Results A total of 136 patients were randomized in the study, 68 to each arm. With a median follow up of 3.2 years, the 2-year PFS in the PCC arm was 89% in the overall, 96% in the low-risk and 67% in the intermediate/high-risk groups; in the C-TPF arm 2-year PFS was 88% in the overall, 88% in the low-risk and 89% in the intermediate/high-risk groups. Conclusion The observed 2-year PFS of PCC in the low-risk group and of C-TPF in the intermediate/high-risk group showed a 20% improvement compared with the historical control derived from RTOG-0129, therefore reaching the primary end point of the trial.

Background The purpose of this study was to evaluate the long-term outcomes after initial definitive or adjuvant radiotherapy (RT) for T3 laryngeal cancers. Methods We reviewed 412 patients treated for T3 laryngeal squamous cell cancer from 1985 to 2011. Results The 10-year overall survival (OS) was 35%; disease-specific-survival (DSS) was 61%; locoregional control was 76%; and freedom from distant metastasis was 83%. Chemotherapy, age, performance status

The purpose of this study was to present the imaging features of primary and secondary malignant lacrimal sac and nasolacrimal duct tumors and their pattern of tumor spread in 18 patients. The most common tumor histology in our series was squamous cell carcinoma. In 15/18 patients, tumor involved both the lacrimal sac and duct at the time of diagnosis. In 11/16 patients on CT, the nasolacrimal bony canal was smoothly expanded without erosive changes. The medial canthus region (16/18) was a frequent site of direct tumor spread. Two patients had intraconal orbital spread of tumor. Tumor spread to the sinus or nasal cavity was observed in 5/13 primary tumors. Only 1 patient presented with nodal metastasis. There was no intracranial tumor extension, perineural tumor spread along the infraorbital nerve, distant metastasis, or dacryocystocele formation in any of the patients at the time of diagnosis.

SMARCB1 (INI1)-deficient sinonasal carcinomas were first described in 2014, and this series of 17 cases represents the first imaging description. This tumor is part of a larger group of SMARCB1-deficient neoplasms, characterized by aggressive behavior and a rhabdoid cytopathologic appearance, that affect multiple anatomic sites. Clinical and imaging features overlap considerably with other aggressive sinonasal malignancies such as sinonasal undifferentiated carcinoma, which represents a common initial pathologic diagnosis in this entity. SMARCB1 (INI1)-deficient sinonasal tumors occurred most frequently in the nasoethmoidal region with invasion of the adjacent orbit and anterior cranial fossa. Avid contrast enhancement, intermediate to low T2 signal, and FDG avidity were frequent imaging features. Approximately half of the lesions demonstrated calcification, some with an unusual "hair on end" appearance, suggesting aggressive periosteal reaction.

In their article comparing MR imaging and dual-energy CT for the evaluation of cartilage invasion by laryngeal carcinoma,[1][1] the authors correctly stated in their introduction that the diagnosis of T4a disease—that is, tumor invading not just into but beyond the laryngeal cartilage and into the

Background Enhanced phosphoinositide 3-kinase (PI3K)/Akt/mammalian target of rapamycin (mTOR) pathway is one of the key adaptive changes accounting for epidermal growth factor receptor (EGFR) inhibitor-resistant growth in head and neck squamous cell carcinoma (HNSCC). We designed a phase II clinical trial of EGFR tyrosine kinase inhibitor (TKI), erlotinib, in association with the mTOR inhibitor, everolimus, based on the hypothesis that the downstream effects of Akt through inhibition of mTOR may enhance the effectiveness of the EGFR-TKI in patients with recurrent/metastatic HNSCC. Patients and methods Patients with histologically or cytologically confirmed platinum-resistant HNSCC received everolimus 5 mg and erlotinib 150 mg daily orally until disease progression, intolerable toxicity, investigator or patient decision. Cytokines and angiogenic factors profile, limited mutation analysis and p16 immunohistochemistry status were included in the biomarker analysis. Results Of the 35 assessable patients, 3 (8%) achieved partial response at 4 weeks, 1 confirmed at 12 weeks; overall response rate at 12 weeks was 2.8%. Twenty-seven (77%) patients achieved disease stabilization at 4 weeks, 11 (31%) confirmed at 12 weeks. Twelve-week progression-free survival (PFS) was 49%, median PFS 11.9 weeks and median overall survival (OS) 10.25 months. High neutrophil gelatinase lipocalin (P = 0.01) and vascular endothelial growth factor (VEGF) (P = 0.04) plasma levels were significantly associated with worse OS. Conclusions The combination of erlotinib and everolimus did not show significant benefit in unselected patients with platinum-resistant metastatic HNSCC despite a manageable toxicity profile. Markers of tumor invasion and hypoxia identify a group of patients with particularly poor prognosis. Clinical trial number NCT00942734.

Background Primary extradural meningiomas (PEMs) are rare, particularly those involving the paraspinal cervical area, so little is known about them. We identified a new case and compared it with 10 previous cases, addressed the diagnostic challenges, and highlighted the clinical and pathologic characteristics. Methods We conducted a case report and literature review of PEM cases reported since 1976. Results A 59-year-old man presented with right neck stiffness and discomfort that was present over the last several months, which is consistent with other cases. CT scan and MRI revealed a paraspinal cervical mass. Fine-needle aspiration (FNA) and core biopsy revealed spindle cells with whorling consistent with meningioma. The patient underwent complete en bloc resection of the tumor. The PEM was histologically benign. Conclusion Cervical PEMs should be considered in the differential diagnosis of a deep neck mass. These tumors are typically benign and are surgically resected. © 2014 Wiley Periodicals, Inc. Head Neck 37: E92–E95, 2015

BACKGROUND To evaluate long-term disease control, survival, and functional outcomes after surgical and nonsurgical initial treatment for T4 larynx cancer.

Background Preclinical data demonstrate a key role for the epidermal growth factor receptor (EGFR) in the carcinogenesis of cutaneous squamous cell carcinomas (CSCCs). There are, however, limited data on the efficacy of EGFR inhibitors in incurable, recurrent, and/or metastatic CSCC. Objective To determine the response rate to gefitinib in patients with CSCC not amenable to curative therapy including surgery or radiation. Methods This was a single-arm phase II study. A total of 40 patients were treated with gefitinib, 250 mg orally daily, until disease progression or intolerable toxicities. The prespecified target response rate of interest was 20%. Results The overall response rate was 16% (95% confidence interval, 0.06-0.32; 6 partial responses in 37 evaluable patients). An additional 13 patients (35%) had stable disease at 8 weeks. The median durations of response and progression-free survival were 31.4 months (95% confidence interval, 3.91-not applicable) and 3.8 months (95% confidence interval, 2.2-5.7), respectively. The side effect profile was consistent with the previous experience with gefitinib in other tumor types. Limitations This was a single-institution, single-arm study. The prespecified target response rate was not met. Conclusion Gefitinib demonstrated modest activity in incurable CSCC, with a favorable adverse event profile.

Adenocarcinoma of the middle ear is a rare entity that must be distinguished from other adenomatous tumors of the temporal bone. We present the first patient of an intestinal-type adenocarcinoma originating from the middle ear.A 58-year-old woman presented with an 8-year history of left otorrhea. Her middle ear effusions were quite thick and gummy. She underwent eight sets of pressure-equalization tubes within 3 years. In 2011, her physical examination identified a middle ear mass, and she underwent mastoidectomy. A middle ear adenoma was resected. She underwent an additional three mastoidectomies for recurrence, with pathology from the 4th mastoidectomy revealing a diagnosis of adenocarcinoma. Imaging, at that time, showed an extensive temporal bone and Eustachian tube tumor.She underwent a left subtotal temporal bone resection, parotidectomy, infratemporal fossa resection, dural resection, and microvascular free flap reconstruction followed by postoperative proton beam radiotherapy.The final pathology report revealed intestinal-type adenocarcinoma.Treatment was successful and the patient remains disease free 4 years later.This is the first report of an intestinal-type adenocarcinoma of the middle ear and temporal bone. This patient also illustrates the difficulty of accurate histologic diagnosis of adenomatous tumors of the middle ear.

Resumen Presentamos las metastasis durales como forma inusual de diseminacion de tumores nasosinusales malignos tratados; se revisan 20 casos diagnosticados durante el seguimiento imagenologico a un grupo tratado con reseccion craneofacial anterior. Evaluamos metastasis durales en 12 carcinomas nasosinusales indiferenciados, 7 neuroblastomas olfatorios y un carcinoma adenoquistico. En neuroblastomas olfatorios aparecieron metastasis durales en promedio 7,3 anos postratamiento. La distancia maxima del tumor a la metastasis fue de 14 cm para neuroblastoma olfatorio y de 4,3 cm para carcinoma nasosinusal indiferenciado. Observamos metastasis durales en los agujeros de trepanacion en el 50% de los carcinomas nasosinusales indiferenciados y en el 29% de los neuroblastomas olfatorios. Las metastasis durales presentaron patron nodular (60%), multinodular (10%), quistico (15%) y en placa (15%). Proponemos un mecanismo venoso local de diseminacion relacionado a disrupcion tumoral o quirurgica de la fosa craneal anterior. El seguimiento a largo plazo con inclusion craneal estaria indicado por la posible presentacion tardia y distante de metastasis durales.

6001 Background: We designed a randomized phase II trial to determine the efficacy of two induction chemotherapy (ICT) cetuximab (C) containing regimens with demonstrated efficacy and acceptable to...