Neuroendocrine tumours of the digestive tract report of two cases and literature review

Neuroendocrine tumors (NET) of the digestive system are rare. They comprise only 1% of all the tumors of the gastrointestinal tract. The aim of this study is to report two cases of exceptional localization of non secretary neuroendocrine tumors. Their difficulty in diagnosis, treatment and controversy in chemotherapy merit their study in depth. These are two patients that were treated in the department of general surgery (Beau Séjour) in Charles Nicolle Hospital for non secretary (NET) of the pancreas and stomach. The first was presented as a painless epigastria mass with frequent diarrhea. The second was presented as a painful mass in the left hypochondriac area without any other symptoms. Biochemical evaluation confirmed their non secretary properties. Surgery remains the first line of treatment. It was possible for the gastric, but not for the pancreatic tumor. Both were very advanced and had metastasis. Histopathological evaluation and immunohistochemical study using the method of peroxydase antiperoxydase (PAP) complexes for some special monoclonal antibodies confirmed the diagnosis. Chemotherapy of both cases was mal tolerated and was discussed in details.     

Kinsbourne syndrome: review of our cases

INTRODUCTION: The childhood opsoclonus-myoclonus or Kinsbourne syndrome, is a uncommon process, of acute or subacute beginning, which affects infant and children. It's course is characterized by opsoclonus, polimyoclonias and cerebellar ataxia. The disease is frequently associated to neuroblastoma (46%). MATERIAL AND METHODS: We present a retrospective study on 9 patients, emphasizing the clinical presentation and the evolution aspects. RESULTS AND CONCLUSIONS: We found changes in the EEG in three cases. Most surprising is the scarce incidence of neuroblastoma, which has been found only in a one out of nine patients. We found three cases with relapse during the treatment or on withdrawal and one of them relapsed twice again. The evolution has been variable, since 5/9 patient have presented some type of mild or moderate neuro-psychological sequelae. Out of three patient with relapses, two presented permanent neurological sequelae. A patient which suffered three relapses, is also the one which presents more serious sequelae.     

Megalourethra: a report of four cases and review of the literature

Megalourethra is a rare congenital anomaly characterized by severe dilatation of the penile urethra. Four cases of congenital megalourethra were seen at Sir Padampat Mother and Child Health Institute, Jaipur, during the last 10 years. Three cases of scaphoid megalourethra had no other associated congenital anomalies and were treat-ed successfully without any complications, while one patient with a fusiform megalourethra had severe associated congenital anomalies and died. These cases are reported with a review of the literature.     

Leri's melorheostosis: three pediatric cases and a review of the literature

Three pediatric cases of melorheostosis are described. Growth disorders of the limbs are often the first signs in children. The radiological appearance consists of long tracks of increased cortical radiodensity in the long bones and of small dense islets in the epiphyses.     

Ureterovaginal fistula: a report of 12 cases and review of the literature

Ureterovaginal fistula is an uncommon complication of pelvic operations, seen most often after Wertheim's hysterectomy. We report 12 cases of ureterovaginal fistulas seen during a 20-year period, all of which followed operations for benign gynecologic conditions. Most patients had no urinary symptoms until the sudden onset of incontinence 1 to 4 weeks postoperatively. Diagnosis was established readily by a combination of excretory urography, cystography, cystoscopy, retrograde pyeloureterography and dye studies. In our series only 1 patient was treated by primary nephrectomy, while 11 underwent ureteroneocystostomy: 2 with a Boari flap and 9 by a direct method. Reconstruction failed in 2 patients, 1 of whom required a secondary nephrectomy.     

Parameatal cyst: presentation of two cases and a review of the literature

OBJECTIVE: To describe two additional cases of parameatal urethral cyst. The literature is reviewed and the etiology and treatment of this disease are discussed. METHODS: Two patients aged 20 and 24 years with parameatal urethral cyst are described. The patients were seen at the Gea González Hospital (México) during the period 1987-1988. The first case was asymptomatic and the cyst had been present since birth and in the other case, the cyst appeared at age 24 and caused irregular stream. Laboratory tests, surgical excision and pathological analysis of the cyst were performed. RESULTS: The laboratory tests were normal, no problems were encountered during surgical excision, the symptoms disappeared and there were no postoperative complications or recurrence. Pathological analysis demonstrated columnar epithelium in both cases. CONCLUSIONS: Parameatal urethral cyst is a rare benign condition that is asymptomatic in most of the cases. It may be present since birth or appear later and is prevalent in young males. Its etiology remains unclear and treatment is by complete surgical excision to avoid complications and recurrence.     

Atypical ameloblastoma: report of 3 cases and a review of the literature

The aim of our study was to test the hypothesis that the better absorption of sorbitol when ingested with glucose could be related to a delayed gastric emptying. We tested the effect of the ingestion of glucose and lipids on the gastric emptying and intestinal absorption of sorbitol in six healthy volunteers, using gastric scintigraphy and hydrogen breath test. After an overnight fast, subjects ingested in random order, on 48-h test periods separated by at least one week, the following solutions: (a) 20 g sorbitol alone; (b) 20 g sorbitol and 20 g glucose; (c) 20 g sorbitol and 9 g lipids. Isotopic acquisitions were taken for 3 h following the ingestion of sorbitol labelled with 111Indium. Hydrogen concentration was measured in end-expiratory samples during 5 h, and the areas under the breath hydrogen curve, reflecting the amounts of sorbitol unabsorbed in the small bowel, were compared between periods. Mean area under the curve was 397 +/- 159 when sorbitol was ingested alone, and this was significantly lower when ingested with glucose or lipids (313 +/- 181 and 337 +/- 135, respectively; P < 0.05). The three curves of sorbitol gastric emptying differed significantly from each other, the gastric emptying being the slowest for sorbitol plus lipids, and the fastest for sorbitol taken alone. We found a positive correlation between the half-emptying time and the hydrogen areas under the curve (r = 0.46, P = 0.05). In conclusion, our study demonstrates that adding glucose or lipids to a solution of sorbitol slows the gastric emptying of sorbitol, resulting in a better intestinal absorption of sorbitol.     

Gangliogliomas: a clinicopathological study of 25 cases and review of the literature

The histopathological, clinical, and radiological findings in 25 patients (median age 20.5 years; range 1.7-64.2 years) with gangliogliomas were assessed to correlate degree of astrocytic anaplasia and proliferative potential with recurrence or survival. Most patients (64%) presented with seizures (median Karnofsky Performance Score 90%; range 70-100%). Computerized tomography and magnetic resonance imaging showed nonspecific abnormalities. Neoplastic ganglion cells were defined as heterotopic, irregularly grouped, or having more than one nucleus of bizarre shape or size. The astrocytic component was moderately anaplastic in 15 cases and highly anaplastic (HAA) in 10. Eight patients had gross total resection, 11 had subtotal resection, and six underwent biopsy. Ten patients (five gross total resection, three subtotal resection, two biopsy) had no further treatment, 15 underwent external irradiation, and five had adjuvant chemotherapy. Twenty-four patients are alive 15-394 weeks (median 203.5 weeks) postoperatively; one with ganglioglioma-HAA died at 65 weeks. No tumor recurred after gross total resection. Duration of preoperative symptoms < 1 year, greater anaplasia, and age > 30 years at diagnosis may have increased the risk of recurrence after subtotal resection or biopsy by four, three, and two times, respectively (not significant). Bromodeoxyuridine labeling index (BUdR LI) was < 1% in eight non-recurring tumors and 1.3% in another recurring twice (second recurrence LI = 1.6%). Most patients with ganglioglioma have a good prognosis. After gross total resection, only observation is required. After subtotal resection or biopsy, recurrence is possible. BUdR labeling may guide further therapy.     

Multiple hydatid cysts of the brain: a case report and review of the literature

Multiple hydatid cysts of the brain are uncommon and may be either primary or secondary. We report a 12-year-old child with multiple hydatid cysts of the brain occurring 1 year after surgical rupture of a primary large and infected cerebral hydatid cyst. Surgical removal of hydatid cysts was successfully performed. Albendazole (10 mg/kg twice daily for 12 weeks) was administered to the patient in the postoperative stage.     

Endobronchial lipoma: two cases and review of the literature

Endobronchial lipoma is a benign tumour of the large bronchi occurring in middle-aged men. To the 38 successfully treated cases in the English literature a further 2 are added. The symptoms are those of obstructive pneumonitis mimicking bronchogenic carcinoma, and the result of delayed therapy may be bronchiectasis. Treatment includes local resection through a bronchoscope or a bronchotomy incision, or removal, if necessary, of the obstructed lobe or lung at thoracotomy. Smoking may be important in the pathogenesis of this tumour.     

黄色肉芽肿性肾盂肾炎2例报告并文献复习

目的:探讨黄色肉芽肿性肾盂肾炎(Xanthogranuiomatous pyelonephritis,XGP)的分型及诊治方法,提高诊断率.方法:回顾性分析2例黄色肉芽肿性肾盂肾炎的临床资料.结果:2例均经CT检查及快速冰冻切片确诊,1例弥漫型XGP行肾切除术,1例局灶型XGP行部分肾切除术.结论:快速冰冻切片是诊断和决定手术范围的直接证据,术前CT检查有助于鉴别诊断.     

Distal digital keratoacanthoma: a report of 12 cases and a review of the literature

Twelve cases of distal digital keratoacanthoma (DKA) affecting the subungual area or the proximal nail fold are reported. The distal phalanx of the toe was affected in three cases. Spontaneous resolution occurred in one; one other recurred after surgery. We also discuss the link between DKA and incontinentia pigmenti subungual tumours; these entities are indistinguishable.     

Lingual osseous choristoma: a study of eight cases and review of the literature

A choristoma is a tumor-like mass of normal tissue in an "abnormal" location. Lingual osseous choristoma, previously known as osteoma of the tongue, is a rare entity: we found documentation of eight cases at our center during the 10-year period between 1987 and 1996. These cases were analyzed along with 50 others reported in the English language literature between 1913 and 1996. Lingual osseous choristoma frequently occurs during the third and fourth decades of life, and predominantly affects women (81%). Most of the osseous choristomas in our review were located in the posterior third of the tongue, primarily at or close to the foramen cecum or circumvallate papillae (87.9%). While 39.7% of the patients were asymptomatic, the remaining patients complained of symptoms including a lump in the throat (25.8%), dysphagia (6.9%), gagging (5.1%), nausea (3.4%) and irritation (3.4%). Treatment of lingual osseous choristoma consists of simple excision. The tumor's origin has been discussed elsewhere.     

Aneurysm of the internal auditory artery: our experience and review of the literature

The aneurysms of the internal auditory artery (IAA) situated distal from anterior inferior cerebellar artery (AICA)-IAA junction, are extremely rare lesions. A case of distal aneurysm if IAA is presented causing subarachnoid haemorrhage (SAH) and complete ipsilateral deafness. After the neurosurgical treatment the hearing of the patient definitely improved. The literature of distal aneurysms of AICA is reviewed focusing on the clinical features of these malformations, causing cerebello-pontine angel (CPA) symptoms with or without SAH.     

Multiple idiopathic arterial aneurysms in children: a case report and review of the literature

A 6-year-old boy from China presented initially at 2 years of age with a pulsatile mass in his right antecubital fossa. He was not fully evaluated until the age of six years, when pan-angiography and computed axial tomography scan revealed multiple aneurysms of his right brachial artery, right radial artery aneurysms, an infrarenal aortic aneurysm, and a right internal carotid artery aneurysm in the region of the cavernous sinus. The patient underwent uneventful repairs of both the abdominal aortic aneurysm and the multiple aneurysms of the right arm. Pathologic evaluation was significant for medial fibrosis of the arterial wall with decreased and disordered elastin fibers. Review of the previously reported cases in children indicate the upper extremity arteries are involved in 92% of patients, the aortoiliac region in 92% of patients, and the renal/mesenteric vessels in 77% of cases. Lower extremity and cerebrovascular arteries are involved to a lesser extent. Children with peripheral aneurysms should have pan-angiography performed before treatment is begun. Surgical repair in these cases has been excellent.     

Promoting adolescent mental health in primary care: a review of the literature

High levels of psychological disturbance amongst adolescents have been linked to behaviours which can damage physical health, and with mental health problems in adulthood. The aim of this review was to see if published literature supports the hypothesis that primary care is a suitable setting in which mental health problems in adolescents can be prevented by early detection and treatment. Medline, BIDS, SIGLE and Psychlit databases (January 1990-February 1997) were systematically searched for English language studies on adolescent health promotion and mental health in primary care; reference sections were checked for earlier work. When offered, adolescent health checks and clinics have been well received with attendance rates of 73% and 83% reported, respectively. Primary care offers a setting for the prevention and detection of mental health problems in adolescents. Further research is needed to determine cost effective ways of using these opportunities.     

Pharmacotherapy of primary obsessive-compulsive disorder: review of the literature

Obsessive-compulsive disorder (OCD) is a chronic illness that affects 2-3% of Americans during their lifetime. It is characterized by recurrent obtrusive thoughts (obsessions) that compel patients to perform repetitive behaviors that can be excessively time consuming and cause marked distress. More than 40 controlled trials have been published on the treatment of OCD. Of drugs available to treat the disorder, serotonin reuptake inhibitors (SRIs) are most studied. With SRIs, symptoms improve in 22-62% of patients, but complete remission is rare. An agent is often selected based on side effects and patient tolerance, since SRIs are all equally effective. If no response is seen with average dosages, dosages should be increased to the maximum within 4-8 weeks from starting treatment. In patients with partial response, the dosage should be increased to the maximum by 5-9 weeks. Before determining the effectiveness of therapy, a trial of 8-13 weeks is necessary.     

Splanchnic aneurysms: 10 treated cases and review of the literature

This study was designed to determine the effects of a membrane permeant phosphodiesterase-resistant analog of cGMP on lung liquid production and pulmonary blood flow at the time of birth. Experiments were performed on seven fetal sheep prepared for chronic measurements of lung liquid production (Jv), pulmonary blood flow (Qp) and pressure, as well as systemic pressure. Injection of either 8-bromo-cGMP or saline were made via a catheter inserted in the left pulmonary artery. Experiments consisted of 1 h of control, 1 h of infusion, and 2 h of recovery. Data were analyzed by ANOVA and Newman-Keuls test. After infusion of 8-bromo-cGMP, Jv was decreased by 70 and 44% from control in h 3 and 4, respectively. Qp was elevated by 100 mL/min in h 2 and 3 and continued to be elevated by 50 mL/min in h 4. Saline infused animals showed no significant changes in Qp and Jv. This study demonstrates that 8-bromo-cGMP decreases lung liquid production and increases pulmonary blood flow in near term fetal sheep. Although blood flow increased in h 2, lung liquid production did not decrease at this time, suggesting a time dissociation between changes in pulmonary blood flow and lung liquid production. Thus, it is possible that a common transduction pathway involving cGMP may be responsible for lung liquid reduction and elevation of pulmonary blood flow at birth. However, Qp and Jv may not be causally related.     

The high jugular bulb: report of five cases and a review of the literature

The jugular bulb varies widely in position and dimensions. A high jugular bulb is not an uncommon finding in temporal bones. Besides our five cases with different clinical manifestations, we review 52 cases diagnosed clinically and published previously in the English literature. The high jugular bulb occurs more often on the right temporal bone. Certain pathologic conditions, such as an abnormal bone formation, an aberrant sinusojugular system, or decreased pneumatization of the mastoid bone, may predispose an individual to its occurrence. Most people with this anatomical variation remain asymptomatic. However, various otologic problems and symptoms may be attributed to this condition. A high resolution computed tomography scan is the most convenient diagnostic tool at present. Law projection plain mastoid radiography may reveal a high jugular bulb in certain cases, providing a preoperative warning to an otologic surgeon. An exploratory tympanotomy is not suggested for those with a high jugular bulb with a conductive hearing loss, while jugular vein ligation has been reported to have good results in alleviating intractable pulsatile tinnitus. For most asymptomatic patients, regular long-term follow-up is recommended.