Orbital nerve sheath myxoma: a case report.

A 72-year-old man was referred to the Service of Ophthalmology due to a 2-year history of ptosis and a painless mass in the lateral orbital margin of the right eye. Orbital MRI revealed a well-demarcated lesion in the superotemporal quadrant of the orbit. After surgical excision, histopathological examination led to the diagnosis of nerve sheath myxoma, a tumor composed of myxoid nodules separated by fibrous septa with spindle-shaped and stellate cells. Many of these cells were immunostained with antibodies to S-100 protein. This is the first case reported in the literature of such a tumor located in the orbit, and, though extremely rare, it should be considered in the differential diagnosis of orbital tumors.

Spontaneous hearing improvement in a patient with an inflammatory myofibroblastic tumor of the temporal bone.

Inflammatory myofibroblastic tumor (IMT) is a rare lesion of unknown etiology and difficult diagnosis. The treatment of IMT is controversial. We report a case of IMT of the temporal bone in a young man presenting with a progressive hearing loss. Three years after diagnosis, partial hearing improvement has been documented.

Cyclin D1 expression and histopathologic features in vestibular schwannomas.

OBJECTIVE: To evaluate cyclin D1 expression in vestibular schwannoma and its relationship with histologic, clinical, and radiologic features. PATIENTS: Twenty-one patients with histologically confirmed vestibular schwannoma. INTERVENTION: Immunohistochemistry analysis was performed with anticyclin D1. Histopathologic features studied included Antoni pattern and nuclear and stromal degenerative changes. Clinical charts, audiometric data, and magnetic resonance imaging characteristics were reviewed. MAIN OUTCOME MEASURES: Cyclin D1 expression and its association with histologic, clinical, and radiologic findings. RESULTS: Cyclin D1 expression was found in 52% of cases. Cyclin D1 expression was more frequent in right-sided tumors (p = 0.02) and in tumors with nuclear degenerative changes (p < 0.0001). Patients with negative cyclin D1 expression had longer duration of deafness (p = 0.02) and higher 2,000-Hz hearing thresholds (p = 0.04) than cyclin D1+ patients. CONCLUSION: Cyclin D1 expression, present in nearly half of the cases, may play a role in the development of these tumors. Further studies are needed to fully understand the contributions of histopathologic and immunohistochemical factors to vestibular schwannoma biological activity.

Placental anomalies in children with infantile hemangioma.

Evaluation of the placenta provides some important insights into pathophysiologic changes that take place during the prenatal and intrapartum process. We investigated the pathogenic significance of placental features and their relationship to the development of infantile hemangioma in order to obtain a better understanding of its cause. Placental specimens were reviewed from 26 singleton pregnancies of women whose offspring weighed less than 1500 g. A group of 13 neonates who developed infantile hemangioma in the immediate neonate period were compared with 13 healthy preterm infants of comparable postconception age who had no infantile hemangioma. Pathologic placental changes were analyzed in both groups. Gross lesions with disturbance of the utero-placental circulation were found in all placentas from children who developed infantile hemangioma, including massive retroplacental hematoma in two infants, extensive ischemic infarction in seven, and large dilatated vascular communications, severe vasculitis, chorioamnionitis and funiculitis in four. Placental features included percentages greater than 25% of avascular villi, platelet and fibrin aggregates, and multifocal disease involving more than one histologic section. Examination of 13 placentas of low-birth-weight infants without infantile hemangioma only showed abnormal placentation in one and isolated villous dismaturity in two. The higher ratio of placental pathologic findings in patients with infantile hemangioma suggests that reduced placental oxygen diffusive conductance contributes to fetal hypoxic stress and that hypoxic/ischemic changes in the placenta could be related to infantile hemangioma development via vascular endothelial growth factor and placental growth factor expression, among others, within the villious vessels and throphoblasts.

Large cell neuroendocrine carcinoma of the parotid gland: case report and literature review.

A 74-year-old male presented with a large polinodular mass in the neck. Fine needle aspiration cytology (FNAC) showed an undifferentiated large cell carcinoma. Computed tomography (CT) showed a large parotid mass with multiple satelite nodules. The remaining radiological studies were normal. Radical parotidectomy was performed. The tumor was a large cell carcinoma with neuroendocrine features and positive immunostain for neuroendocrine markers. The patient received postoperative radiotherapy and was free of tumor eight months later. Only four cases of large cell neuroendocrine carcinoma (LCNEC) of the salivary gland have been communicated. All of them have involved the parotid gland. This tumor presents in elderly patients as a large infiltrating parotid mass. Fine needle aspiration cytology serves to recognize the carcinoma, but it fails in recognizing the neuroendocrine features of the tumor. The histopathological features of this tumor are the same as in other organs. Chromogranin and synaptophysin are useful immunohistochemical markers. A primary location of the tumor in another organ, specially the lung, should be ruled out. Surgery is the main treatment modality and can be complemented with postoperative radiotherapy. The prognosis seems to be poor. More studies are needed to better define the therapeutical alternatives and prognostic factors of these rare tumors.

Functional neck dissection for the clinically negative neck: effectiveness and controversies.

This study was performed to evaluate the effectiveness of functional neck dissection in controlling metastasis to the clinically negative (cN0) neck, focusing on recurrences in the pathologically negative (pN0) neck and the role of extracapsular spread in the cN0 neck. A series of 172 patients (253 dissected fields) treated for cN0 laryngeal or hypopharyngeal cancer with a 5-year minimum follow-up is presented. Occult metastasis was observed in 30% of the patients. Extracapsular spread was present in 39% of the positive nodes. The neck recurrence rate was 5.2%. Surgical specimens from cases of neck recurrence in pN0 necks were reevaluated for micrometastasis by immunostaining with antibody for cytokeratins. The immunohistochemical findings were positive in 1 of 4 cases. Functional neck dissection provides good neck control and survival rates for the cN0 neck. The accurate prognostic significance of extracapsular spread in cN0 necks is still unknown. Micrometastasis alone may be insufficient to explain recurrences in pN0 necks.

Facial nerve schwannoma of the cerebellopontine angle: a diagnostic challenge.

Facial nerve schwannomas are rare lesions that may involve any segment of the facial nerve. Because of their rarity and the lack of a consistent clinical and radiological pattern, facial nerve schwannomas located at the cerebellopontine angle (CPA) and internal auditory canal (IAC) represent a diagnostic and therapeutic challenge for clinicians. In this report, a case of a CPA/IAC facial nerve schwannoma is presented. Contemporary diagnosis and management of this rare lesion are analyzed.

Avoiding misdiagnosis in ceruminous gland tumours.

Ceruminous gland tumours are infrequent lesions of the external auditory canal (EAC). Controversy still exists about nomenclature, classification, tissue of origin, and accurate diagnosis of these tumours. We present three cases of ceruminous gland tumours, including benign eccrine cylindroma, ceruminous adenoma, and adenoid cystic carcinoma. Superficial biopsy led to an initial erroneous diagnosis of adenocarcinoma in the latter. All cases were positive for cytokeratin and S-100 protein, supporting a ceruminous gland origin. The benign eccrine cylindroma should be included in the ceruminous gland tumour classification. A wide excisional biopsy should be performed in every EAC lesion. The terms 'ceruminoma' and 'cylindroma' should be avoided.

Cyclin D1 expression and facial function outcome after vestibular schwannoma surgery.

HYPOTHESIS: The proto-oncogen cyclin D1 has been implicated in the development and behavior of vestibular schwannoma. This study evaluates the association between cyclin D1 expression and other known prognostic factors in facial function outcome 1 year after vestibular schwannoma surgery. METHODS: Sixty-four patients undergoing surgery for vestibular schwannoma were studied. Immunohistochemistry analysis was performed with anticyclin D1 in all cases. Cyclin D1 expression, as well as other demographic, clinical, radiologic, and intraoperative data, was correlated with 1-year postoperative facial function. RESULTS: Good 1-year facial function (Grades 1-2) was achieved in 73% of cases. Cyclin D1 expression was found in 67% of the tumors. Positive cyclin D1 staining was more frequent in patients with Grades 1 to 2 (75%) than in those with Grades 3 to 6 (25%). Other significant variables were tumor volume and facial nerve stimulation after tumor resection. The area under the receiver operating characteristics curve increased when adding cyclin D1 expression to the multivariate model. CONCLUSION: Cyclin D1 expression is associated to facial outcome after vestibular schwannoma surgery. The prognostic value of cyclin D1 expression is independent of tumor size and facial nerve stimulation at the end of surgery.

Psammomatoid ossifying fibromas: immunohistochemical analysis and differential diagnosis with psammomatous meningiomas of craniofacial bones.

OBJECTIVE: To clarify the role of immunohistochemistry in the diagnosis of psammomatoid ossifying fibroma (PSOF), conventional cemento-ossifying fibroma (COF), and psammomatous meningioma (PM) of the craniofacial skeleton. STUDY DESIGN: The histology and immunohistochemistry of 4 PSOFs, 6 COFs, and 7 PMs was studied. Antibodies included EMA, cytokeratins, smooth muscle actin (SMA), desmin, vimentin, CD34, CD10, S-100 protein, and glial fibrillary acidic protein (GFAP). RESULTS: All PSOFs showed multiple round ossicles homogeneously distributed within a fibroblastic stroma. Psammomatous meningiomas had meningothelial features. All tumors, except 1 COF, were positive for EMA. All of them expressed vimentin, and none showed cytokeratins. Staining for SMA and S-100 protein was variable. CD10 was positive in all cases except 2 meningiomas. CD34 and GFAP stained only 1 case of meningioma each. CONCLUSIONS: The diagnosis of PSOF should rest on histologic features. An incorrect diagnosis of meningioma based on the expression of EMA should be avoided.

Smooth muscle choristoma of the internal auditory meatus.

Choristomas of the internal auditory meatus are very uncommon tumors. Because of their rarity and the lack of a consistent clinical and radiological pattern, they are usually misdiagnosed as vestibular schwannomas. Surgical exploration usually shows involvement of the vestibular, cochlear or facial nerve by the tumor, representing a diagnostic and therapeutic challenge to the surgeon. In this report, a case of a smooth muscle choristoma of the internal auditory meatus is presented. The lesion involved the vestibular and cochlear nerves and was extremely adherent to the facial nerve. Microscopic examination of the tumor revealed nerve fibers, smooth muscle and a small amount of adipose tissue. No striated muscle was found. The contemporary terminology, diagnosis and management of this infrequent lesion are analyzed.

The role of GLUT1 immunostaining in the diagnosis and classification of liver vascular tumors in children.

BACKGROUND/AIM: GLUT1 is an erythrocyte-type glucose transporter protein typically expressed in cutaneous proliferating hemangioma. Immunostaining for GLUT1 is becoming valuable for predicting the outcome of vascular skin tumors. Liver vascular tumors (LVTs) are a serious challenge for pediatric surgeons because of their often severe and sometimes unpredictable clinical course. To improve therapeutic strategies, we designed this study in which we tested in pathology specimens of LVT the hypothesis that GLUT1 expression could be useful to understand and classify LVT. MATERIAL AND METHODS: In the last 10 years, we treated 20 children with LVT. Pathology specimens from biopsy, excision, or autopsy were available in 11 of them. The paraffin sections were immunostained for GLUT1 and also for Ki-67 to assess the proportion of proliferating cells. Patients were divided into 2 groups: GLUT1-positive (n = 4) and GLUT1-negative (n = 7) that were compared for age at diagnosis, survival, and proportion of proliferating cells. Nonparametric and chi 2 tests were used for statistical analysis as appropriate. RESULTS: Mean age at diagnosis was higher in GLUT1-positive group, although not statistically significant in comparison with GLUT1-negative (308 +/- 515 vs 70 +/- 51 days, respectively). Three of 4 children in GLUT1-positive group died versus 1 of 7 in the GLUT1-negative group (not significant). All GLUT1-positive tumors were multicentric hemangiomata without central necrosis and only 1 with large vessels. Among GLUT1-negative tumors, 5 were solitary masses and 2 were multicentric (the value of the last 2 specimens was uncertain), 2 had central necrosis, and 2 had large vessels. Proliferation index was 18% +/- 1.42% and 1.42% +/- 0.97%, respectively, in each group ( P < .05). CONCLUSIONS: GLUT1-positive tumors have significantly higher proliferation rates than negative ones. Mortality tended to be higher in children with GLUT1-positive tumors. Positive GLUT1 immunostaining is likely specific for proliferating hemangioma, and it predicts the typical course of proliferation followed by involution.

Kaposiform hemangioendothelioma of the external auditory canal in an adult.

BACKGROUND: Kaposiform hemangioendothelioma is an uncommon vascular tumor initially reported to occur exclusively in children. METHODS: The presentation, pathologic evaluation, and management of an unusual case of kaposiform hemangioendothelioma is presented and discussed. RESULTS: A 27-year-old HIV-negative man was initially seen with a reddish nodule located in the outer third of the external auditory canal. Histologically, the tumor was composed of spindle-shaped cells arranged in short fascicles associated with small endothelial-like vascular spaces, similar in appearance to Kaposi's sarcoma. The lesion was locally excised but recurred 1 month later; then radiation therapy was performed. The patient remains well at 5-year follow-up. CONCLUSIONS: Recognition of kaposiform hemangioendothelioma is important to avoid possible confusion with a variety of vascular neoplasms with different biologic potential. This case presented some diagnostic difficulty because of the age of the patient and the unusual location of the lesion and had to be mainly distinguished from Kaposi's sarcoma.