Primary pituitary neuroendocrine tumor: Case report and literature review.

BACKGROUND: Neuroendocrine tumors (NET) originate from the diffuse neuroendocrine system. These can arise in almost every organ of the body, although they are most commonly found in the gastrointestinal tract and respiratory system. The skull base and sellar region are extremely rare sites for neuroendocrine carcinoma. Consequently, in this case, both diagnosis and definition of surgical goals, as well as further treatment strategies were challenging. CASE DESCRIPTION: A 65-year-old woman was admitted to our Neurosurgery Department with a rapidly progressive visus reduction, drowsiness, polyuria, and polydipsia. Neuroimaging showed a sellar/suprasellar mass (diameter of 2 cm) with a heterogeneous signal compressing the optic chiasm and extending laterally toward the cavernous sinus. Differential diagnosis based on imaging included pituitary macroadenoma or metastasis. The patient underwent endoscopic endonasal transsphenoidal surgery. A total resection of the mass was impossible because of the infiltration of the optic chiasm and the intraoperative histological diagnosis of malignant epithelial neoplasm. Further histological evaluation revealed that the lesion was a NET with no other primary or metastatic sites detectable. Subsequently, the patient was successfully treated with fractioned stereotactic radiotherapy and polychemotherapy. Four years after the surgery, follow-up magnetic resonance imaging showed stability of the residual disease. Neurologic examination revealed a complete visual recovery. CONCLUSIONS: Primary pituitary NET, though rare, should be included in the differential diagnosis of sellar lesions. A multimodality treatment approach is needed. Finally, the present case highlights, that in the case of a pituitary lesion infiltrating the optic chiasm, including NET, the endoscopic endonasal transsphenoidal subtotal resection followed by fractioned stereotactic radiotherapy and chemotherapy may represent an effective and safe choice of treatment.

[Benign symmetrical lipomatosis: Madelung's disease]. / Lipomatosi simmetrica benigna: malattia di Madelung.

Multiple symmetrical lipomatosis, or Madelung's disease, is a rare disease of unknown etiology. It is characterized by the presence of loose adipose tissue deposits localized in the cervical region and in the upper body. The neoformations grow slowly and their initial consequence is purely esthetic. They can, however, lead to compression of the laryngeal-tracheal area and of the esophagus. This disease normally affects middle-aged males from the Mediterranean area with a history of alcohol abuse. Although most cases have been sporadic, a few authors have indicated that the disorder may be hereditary. It is thought that this pathology originates from an alteration in lipid metabolism. Surgical removal of the lipomatose mass is the treatment of choice even though there are frequently recurrences. A case is presented of a rare laryngeal localization of this disease and diagnosis and treatment are discussed.

Chronic tonsillitis in children: activation of polymorphonuclear cells from peripheral blood and tonsillar tissue. In vitro production of MPO, ECP and EPX.

Activation of polymorphonuclear cells (PMNs), neutrophils and eosinophils, occurs in response to infections. Myeloperoxidase (MPO) is an enzyme contained in neutrophils with toxic activity for many microorganisms including bacteria. Fosinophil cationic protein (ECP) and eosinophil X protein (EPX) are released by activated eosinophils in response to different inflammatory stimuli. Determination of serum levels of MPO, ECP and EPX permits assessment of the state of activation of these cells. The purpose of this study was to evaluate the state of activation of neutrophils and eosinophils present in tonsillar tissue and peripheral blood from 30 children (18 boys and 12 girls) undergoing tonsillectomy for chronic tonsillitis. For determination of serum levels of MPO, ECP and EPX, peripheral blood samples were obtained at the time of surgery: PMNs were separated by erythrosedimentation from peripheral blood and from tonsillar tissue after surgery and cultured for 7 days. The cells were plated (10(5) well) and the plates were incubated in 5% CO(2), 7% O(2), 80% N(2). After 2, 24, 48, 72, 96, 120, 144 h, supernatants were removed from the cultures of both tonsillar tissue and peripheral blood PMNs for determination of in vitro release of MPO, ECP and EPX. The assays were carried out using a radioimmunotechnique (RIA, Pharmacia) and the concentrations of the three proteins (mean/6 wells) for each time were expressed in mu g/l. The data suggest activation of PMNs in children with chronic tonsillitis.

The role of tissue colonization and bacterial resistance in recurrent tonsillitis.

Unsuccessful medical therapy for treatment of acute tonsillitis frequently results in onset of recurrent or chronic forms rendering surgical treatment necessary. We have studied some of the factors involved in the evolution of these chronic or recurrent forms and, in particular, the distribution of different bacterial strains in tonsillar tissue, their response to treatment with antibiotics and mechanisms of bacterial resistance. The distribution of bacterial flora (saprophytic and pathogenic) present on the surface of the tonsils, in the crypts and in the tonsillar tissue was studied in a control population of 30 children (20 boys and 10 girls) aged between 2-13 years, all affected with chronic tonsillitis and submitted to tonsillectomy. The same study was performed in a group of 80 sex and aged matched children, also presenting with chronic or recurrent tonsillitis and treated with amoxicillin or amoxicillin-clavulanic acid or cefaclor or clarithromycin 72 h before surgery. The 80 subjects were randomly divided into four groups so that each antibiotic was tested on 20 subjects. The distribution of the bacterial population on the surface of tonsillar tissue, in the crypts and in the deeper tonsillar tissue is of particular interest concerning the affinity of bacteria to the different tissue areas. In particular the interaction between crypt and tonsillar core, which could be a factor involved in the process of worsening of bacterial infection in the tonsils, is evidenced. The four antibodies tested showed different abilities to eradicate infection: Haemophilus influenzae was found to be the most resistant germ to antibiotic therapy and was thus the most frequent cause of recurrent infections. The characteristics and the mechanisms of adherence and resistance to beta lactam antibiotics were also analysed.

Correlations between the olfacto-respiratory reflex and nasal mucosa blood flow: comparative evaluation through rhinomanometry and laser-Doppler flowmeter testing.

The authors compared the reliability of the olfacto-respiratory test with the vasomotor response evoked by the olfactory stimulation of nasal mucosa, as detected by laser-Doppler flowmetry. The aim was to investigate the possible advantages offered by laser-Doppler flowmeter testing, since this machine has a great capacity for detecting even very small variations in blood flow. The authors, however, can affirm that laser-Doppler flowmetry is only able to detect reflex reactions evoked by stimuli affecting trigeminal innervation. No reaction could be detected following trial with purely olfactory odorants and, in any case, even the reaction to olfacto-trigeminal or gusto-olfacto-trigeminal stimuli could be detected only in response to stimulation that was quantitatively stronger than that necessary to trigger the corresponding respiratory reflex.