Metastasis of Primary Cutaneous Adenoid Cystic Carcinoma to the Nasal Septum after 17 years: A Case Report.

Primary cutaneous adenoid cystic carcinoma (PCACC) is extremely rare, and although distant metastasis has been reported, to date, there are no reports regarding metastasis to the nasal septum. We report a rare case of PCACC that metastasized to the nasal septum 17 years after the first surgery in a 59-year-old woman. She initially presented with a mass under the skin of her left mammary papilla. After a biopsy revealed the presence of an adenoid cystic carcinoma, the tumor was excised and definitively diagnosed as a PCACC. Five years after surgery, the patient presented with left lung metastasis and underwent a partial resection of the left lung. However, 8 years after this procedure, the patient had to undergo a partial resection of the right lung because of right lung metastasis. Four years thereafter, the patient presented with nasal septal metastasis. The tumor was excised successfully using a combined technique integrating intranasal and extranasal approaches. The patient is currently undergoing regular follow-up tests. Thus, in such cases, lifelong follow-up is necessary while checking for both distant metastasis and instances of local recurrence.

Sinonasal Inverted Papilloma Associated with Adenoid Cystic Carcinoma.

Inverted papilloma is the most common benign tumor of the paranasal sinuses with the possibility of malignant transformation. On the one hand, adenoid cystic carcinoma (ACC) is a rare malignant neoplasm that arises from the secretory glands. Sinonasal ACC accounts for 10%-25% of all head and neck ACC. This neoplasm is defined by its distinctive histologic appearance. Surgical resection, whenever possible, is the mainstay therapy. An association between inverted papilloma and malignancy is controversial. A synchronous carcinoma has been established at diagnosis in 3.3%-11% of cases, and the risk of metachronous carcinoma is <3%. We report a case of an 84-year-old female affected with sinonasal inverted papilloma associated with synchronous ACC. She had right nasal obstruction due to the rapid growth of the tumor. She was referred to our department for further workup. A frozen biopsy revealed part of the tumor as ACC. The tumor was consistent with preoperative imaging, and surgical findings also showed infiltration into the orbit. There was no consent for radical surgery with orbital exenteration and adjuvant chemoradiotherapy in consideration of her advanced age. This is the first case report of the association between sinonasal inverted papilloma and ACC within our retrieval capability.

Sebaceous carcinoma of the parotid gland: a case report.

BACKGROUND: Primary sebaceous carcinoma of the parotid gland is extremely rare, and because of its rarity, clinicopathological characteristics and histogenesis are not fully understood. METHODS: Here, we report a patient who presented with a left infra-auricular painless mass. We present the histological features and discuss possible optimal treatments based on previous literature. RESULTS: The mass was suspected to be a myoepithelial tumor or possibly a pleomorphic adenoma. Initially, the mass was resected with preservation of the facial nerve, but this caused facial palsy. Because the histological examination showed a sebaceous carcinoma and a part of the mass could be remaining on the facial nerve, additional surgery was performed, and the facial nerve was reconstructed with cervical nerve. Follow-up after 7 months showed no sign of recurrence of metastasis. CONCLUSION: We encountered a rare sebaceous carcinoma of the parotid gland. Additional surgery was performed because preoperative diagnosis was difficult.

Ototoxicity-induced loss of hearing and inner hair cells is attenuated by HSP70 gene transfer.

The most common reason for sensorineural deafness is death of hair cells (HCs). Heat shock proteins (HSPs) are molecular chaperones that participate in folding, targeting, and degrading proteins. HSP expression is increased in response to various environmental stresses to protect cells from damage. Here, we tested whether viral-mediated overexpression of HSP70 can protect HCs and hearing from severe ototoxicity (kanamycin and furosemide) in guinea pigs. Adenovirus-HSP70 mCherry (Ad.HSP70-mCherry) was injected to experimental animals and adenovirus-mCherry to controls, 4 days before the ototoxic insult. Hearing thresholds were measured by auditory brainstem response before the insult and again before sacrificing the animals, 14 days after the insult. Epi-fluorescence immunocytochemistry showed that injection of Ad.HSP70-mCherry resulted in mCherry fluorescence in nonsensory cells of the organ of Corti. The ototoxic insult eliminated both outer HCs and inner HCs throughout most of the cochlea of control (adenovirus-mCherry-injected) ears and contralateral (uninjected) ears. Ad.HSP70-mCherry-injected ears exhibited a significant preservation of inner HCs compared to control and contralateral ears, but outer HCs were not protected. Auditory brainstem response thresholds were significantly better in Ad.HSP70-mCherry-injected ears than in control and contralateral ears. Our data show that HSP70 augmentation may represent a potential therapy attenuating ototoxic inner HC loss.

Mice with conditional deletion of Cx26 exhibit no vestibular phenotype despite secondary loss of Cx30 in the vestibular end organs.

Connexins are components of gap junctions which facilitate transfer of small molecules between cells. One member of the connexin family, Connexin 26 (Cx26), is prevalent in gap junctions in sensory epithelia of the inner ear. Mutations of GJB2, the gene encoding Cx26, cause significant hearing loss in humans. The vestibular system, however, does not usually show significant functional deficits in humans with this mutation. Mouse models for loss of Cx26 function demonstrate hearing loss and cochlear pathology but the extent of vestibular dysfunction and organ pathology are less well characterized. To understand the vestibular effects of Cx26 mutations, we evaluated vestibular function and histology of the vestibular sensory epithelia in a conditional knockout (CKO) mouse with Cx26 loss of function. Transgenic C57BL/6 mice, in which cre-Sox10 drives excision of the Cx26 gene from non-sensory cells flanking the sensory epithelium of the inner ear (Gjb2-CKO), were compared to age-matched wild types. Animals were sacrificed at ages between 4 and 40 weeks and their cochlear and vestibular sensory organs harvested for histological examination. Cx26 immunoreactivity was prominent in the peripheral vestibular system and the cochlea of wild type mice, but absent in the Gjb2-CKO specimens. The hair cell population in the cochleae of the Gjb2-CKO mice was severely depleted but in the vestibular organs it was intact, despite absence of Cx26 expression. The vestibular organs appeared normal at the latest time point examined, 40 weeks. To determine whether compensation by another connexin explains survival of the normal vestibular sensory epithelium, we evaluated the presence of Cx30 in the Gjb2-CKO mouse. We found that Cx30 labeling was normal in the cochlea, but it was decreased or absent in the vestibular system. The vestibular phenotype of the mutants was not different from wild-types as determined by time on the rotarod, head stability tests and physiological responses to vestibular stimulation. Thus presence of Cx30 in the cochlea does not compensate for Cx26 loss, and the absence of both connexins from vestibular sensory epithelia is no more injurious than the absence of one of them. Further studies to uncover the physiological foundation for this difference between the cochlea and the vestibular organs may help in designing treatments for GJB2 mutations.

AICA syndrome with facial palsy following vertigo and acute sensorineural hearing loss.

We report a case of infarction of the anterior inferior cerebellar artery (AICA) with peripheral facial palsy following vertigo and acute sensorineural hearing loss. A 39-year-old female presented with vertigo and sudden hearing loss, tinnitus, and aural fullness of the right ear. An audiogram revealed a severe hearing loss at all tested frequencies in the right ear. Spontaneous nystagmus toward the left side was also observed. Otoneurological examinations showed sensorineural hearing loss of the right ear and horizontal and rotatory gaze nystagmus toward the left side, and a caloric reflex test demonstrated canal paresis. Initially, we diagnosed the patient for sudden deafness with vertigo. However, right peripheral facial palsy appeared 2 days later. An eye tracking test (ETT) and optokinetic pattern test (OKP) showed centralis abnormality. The patient's brain was examined by magnetic resonance imaging (MRI) and magnetic resonance angioglaphy (MRA) and showed an infarction localized in the pons and cerebellum. MRI and MRA revealed infarction of the right cerebellar hemisphere indicating occlusion of the AICA. Consequently, the patient was diagnosed with AICA syndrome but demonstrated regression following steroid and edaravone treatment. We suggest that performing MRI and MRA in the early stage of AICA syndrome is important for distinguishing cerebellar infarction resulting from vestibular disease.