A Case Report of Glomus Tympanicum Complicated With Facial Nerve Palsy.

Introduction: Generally, glomus tumors are considered tumors of the autonomic system arising from chromaffin cells of the parasympathetic paraganglia of the skull base and neck. Glomus tympanicum is the most common primary tumor of the middle ear cavity and it arises from the paraganglia of the middle ear. Case Report: We present a case of glomus tympanicum presented in a 70-year-old woman, complicated with facial nerve palsy which at first sight was misdiagnosed as cholesteatoma. Patient presented in our clinic because of otorrhea, pulsatile tinnitus and hearing loss in the right ear. However, facial nerve function was good in the first examination (40 days before the surgery). Eventually, she treated successfully with a canal wall down mastoidectomy. Technique had been chosen because of the mass size and the involvement of external auditory canal, after a discussion with the patient. Conclusions: Although histologically benign, glomus tympanicum is slow growing and destructs adjacent tissues potentially. The two most common complaints are hearing loss (conductive) and pulsatile tinnitus. These neoplasms are more common in women and they can be diagnosed by CT or MRI scan. It is of high importance physicians suspect a glomus tumor when patient 's clinical findings are hearing loss and pulsatile tinnitus and use an intravascular agent in imaging so that the differential diagnosis will be supported.

Osteochondromas: review of the clinical, radiological and pathological features.

Osteochondroma is the most common benign bone tumor and usually occurs in the metaphyseal region of the long bones. This tumor takes the form of a cartilage-capped bony outgrowth on the surface of the bone. The vast majority (85%) of osteochondromas present as solitary, nonhereditary lesions. Approximately 15% of osteochondromas occur as multiple lesions in the context of hereditary multiple osteochondromas (HMOs), a disorder that is inherited in an autosomal dominant manner. Most lesions appear in children and adolescents as painless, slow-growing masses. However, depending on the location of the osteochondroma, significant symptoms may occur as a result of complications such as fracture, bony deformity, mechanical joint problems and vascular or neurologic compromise. Malignant transformation of osteochondromas can occur later in adulthood but rarely metastasize. The treatment of choice for osteochondroma is surgical unless the skeleton is still immature. Pathogenetic analysis showed that HMOs are caused by mutations in either of two genes: exostosis (multiple)-1 (EXT1), which is located on chromosome 8q24.11-q24.13 or exostosis (multiple)-2 (EXT2), which is located on chromosome 11p11-12. Recently, biallelic inactivation of the EXT1 locus was described in nonhereditary osteochondromas. The EXT1 and EXT2 proteins function in the biosynthesis of heparin sulfate proteoglycans (HSPGs) which are multifunctional proteins involved in several growth signaling pathways in the normal epiphyseal growth plate. Reduced EXT1 or EXT2 expression in osteochondromas is associated with disordered cellular distribution of HSPGs, resulting in defective endochondral ossification which is likely to be involved in the formation of osteochondromas. Here the clinical, radiological, pathological and pathogenetic features and the treatment modalities of osteochondroma are reviewed.

Immunohistochemical expression patterns of neural and neuroendocrine markers, the neural growth factor receptors and the beta-tubulin II and IV isotypes in human thymus.

Increasing evidence suggests that neuroimmune networks play key roles in the thymic histophysiology and pathology. Prompted by this, we analyzed by immunohistochemistry the distribution of human thymic cells expressing major neural and neuroendocrine markers and neural growth factor (NGF) receptors in combination with the expression patterns of various cytokeratins. Additionally, since some beta-tubulin isotypes are preferentially expressed in neuronal cells, the immunotopographical distribution of thymic cells expressing beta-tubulin II, III and IV was analyzed. Thymic epithelial cells (TECs) expressed protein gene product 9.5 (PGP 9.5), chromogranin A (CHRA), synaptophysin (SYN), neuron-specific enolase (NSE), tyrosine hydroxylase (TH), CD56, CD57, neurofilaments (NF) (140-160 kDa), NGF receptors (TrKA and p75), beta-tubulin II and IV isotypes and cytokeratin 7, 8, 10, 13, 14, 18 and 19. PGP 9.5 was preferentially expressed in cortical TEC whereas SYN, CHRA, NSE, TH and NF 140-160 kDa were preferentially expressed in medullary TECs and Hassal corpuscles. Variable levels of expression of beta-tubulin II and IV were observed in all TEC subtypes whereas beta-tubulin III was undetectable in TECs. Subcapsular and cortical TECs display higher expression of beta-tubulin IV and lower expression of beta-tubulin II in comparison to those observed in medullary TEC and Hassal corpuscles. The diversity of the immunotopographical distibution and the expression of neural and neuroendocrine markers, the NGF receptors TrKA and p75, and the beta-tubulin II and IV isotypes in the distinct subtypes of TEC may reflect the diversity of their biological functions and/or their different stages of differentiation. The present results provide further immunohistological evidence that numerous neural and neuroendocrine factors may be required for the development and function of the human thymic microenvironment.

Adamantinoma.

Adamantinoma is one of the rarest low-grade malignant bone tumours, representing less than 1% of them. Fisher in 1913 named this tumour adamantinoma because of its similarity to ameloblastoma of the jaw. It usually arises in the center of long bones, and 97% of all reported cases were in long tubular bones and mainly in the tibial mid shaft (80-85%). Other long bones not uncommonly affected are the humerus, ulna, femur, fibula and radius. Ribs, spine, metatarsal and carpal bones are very rarely affected. The symptoms are not specific but most frequently the patient complains about swelling, redness, pain and sensitivity of the bone where the tumour is located. Young males are more prone to develop adamantinoma than females. The tumour usually spreads to the lungs, the regional lymph nodes, or other bones. Wide tumour excision and limb salvage reconstruction surgery, or an amputation, are the current surgical treatment options. Radiotherapy and chemotherapy have not been shown to be effective modalities of treatment.

Primary lymphomas of bone.

Primary lymphomas of bone are uncommon malignancies. The vast majority of them are non-Hodgkin lymphoma (NHL), whereas primary Hodgkin lymphoma (HL) of bone is extremely rare. Patients with primary NHL of bone commonly present with local bone pain, soft tissue swelling, and a mass or a pathological fracture. There is a slight male preponderance, and most patients are over 45-50 years of age. Primary NHL of bone can arise in any part of the skeleton, but long bones (femurs, tibia) are the most common sites of presentation. Comprehensive immunohistochemical studies are required to establish an accurate histological diagnosis of primary NHL of bone. Most cases of primary NHL of bone are classified as diffuse large B-cell lymphomas (DLBCL) in the World Health Organisation (WHO) classification of hematological malignancies. On full staging evaluation, most patients have disease of stage IE or IIE according to the Ann Arbor system. Several studies indicate that patients with primary NHL of bone have a favorable outcome, especially when treated by combined modality therapy. A number of studies reported that clinical stage is the most important prognostic variable in predicting overall survival. Interestingly, the rare occurrence of primary lymphoma of bone is in contrast with the frequency of plasma cell tumors in bone. This could be due to the fact that, during normal B-cell differentiation, the bone marrow is the normal site of homing of plasma cells which are terminally-differentiated, immunoglobulin-secreting post-germinal center B-cells. In this respect, there is circumstancial evidence that primary NHL of bone may represent tumors of post-germinal center B-cells. The present review summarizes data on the histogenesis of primary NHL of bone in view of the recent histogenetic classification of DLBCL on the basis of the B-cell differentiation gene expression profiles (germinal center vs. post-germinal center B-cell differentiation).

Long-term outcome of pneumatic dilation in the treatment of achalasia.

OBJECTIVE: Achalasia is a well-defined esophageal motor disorder for which pneumatic dilation is an established therapeutic method. Even though it has been used for several years, there are limited data on the long-term outcomes of patients treated with this procedure. Hence, we aimed to evaluate the long-term efficacy of pneumatic dilation to control the symptoms of achalasia. METHODS: The medical records of all patients treated in our unit for achalasia with pneumatic dilation were reviewed. We identified the long-term result of the initial procedure, the date of the first dilation, and the time interval between dilation and retreatment. RESULTS: Of 260 patients who were treated with pneumatic dilation, 153 (67 men, 86 women) were followed up for more than 5 yr. The mean follow-up period was 11.09 +/- 3.91 yr, and the success rate of the dilation was 75.8%. Among these patients, 35 (19 men, 16 women) had follow-up periods of more than 15 yr. The mean follow-up time of those patients was 16.56 +/- 1.09 yr, and the success rate was 51.4%. Kaplan-Meier survival analysis showed that, overall, 50% of patients develop recurring symptoms after 10.92 yr. CONCLUSIONS: Although 51.4% of patients continued to be in clinical remission more than 15 yr after the initial pneumatic dilation, the long-term success rate of pneumatic dilation seems to drop progressively with time.