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INTRODUCTION

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, up to one half of the MPNSTs occur in patients with neurofibromatosis type-1 (NF-1), while the rest are sporadic. Here, we present a 52-year-old woman with MPNST of the vulva without NF-1. We will discuss basics of the disease, treatment options and follow-up strategies.

PRESENTATION OF CASE

52-year-old female admitted to our hospital with complaint of abnormal uterine bleeding and rapidly growing vulvar mass. Excisional biopsy of the mass showed MPNST of the vulva. Afterwards, the patient underwent radical vulvectomy with inguinofemoral lymph node dissection. Short after the surgery, multiple lung metastasis were shown and responded to chemotherapy, but rapid local recurrence occurred short after the completion of the chemotherapy.

DISCUSSION

The primary treatment option in MPNSTs is surgical excision with or without adjuvant therapy. There is not enough data about the role of systemic chemotherapy in the management of MPNSTs and it still remains controversial.

CONCLUSION

In general, radiation therapy has not been demonstrated to improve overall survival. Complete surgical resection of the primary tumor is the mainstay of the treatment.  相似文献   
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PCR-RFLP (restriction fragment length polymorphism) analysis was used to determine the relation of Giardia duodenalis Groups A and B. Of these, 17 (85%) were found as Group A in symptomatic cases; 22 (92%) were Group B in asymptomatic cases by using PCR-RFLP (p < 0.001). Interestingly, 5 (83%) were Group A in examination of endoscopy aspirates of symptomatic cases, as 5 (83%) were Group B in asymptomatic cases.  相似文献   
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We report the treatment process of a pediatric patient with deformity and shortening in the arm after a recurrent aggressive aneurysmal bone cyst (ABC) in the proximal humerus. The patient was treated with curettage of the lesion and lengthening on an intramedullary nail following an osteotomy just distal to the ABC. The period of lengthening was approximately 50 days. At the end of the treatment the lengthening goal was achieved without any neurovascular complication. There was a minimal loss in shoulder hyperabduction due to the deformity of the humeral head.  相似文献   
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Alkaptonuria is a hereditary disorder of phenylalanine and tyrosine, with an incidence of approximately 1/200,000 to 1/1,000,000. Ochronosis is the accumulation of homogentisic acid and its metabolites in connective tissues such as the tendons, cartilage, and skin. In the present case study, a 50-year-old male presented with a nontraumatic calcaneal avulsion without a previous diagnosis of ochronosis. To the best of our knowledge, little information has been reported of this pathology in the Achilles tendon and the surgical management.  相似文献   
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Diabetic foot ulcer is a common chronic complication of diabetes mellitus. In addition to conventional primary therapy, there are adjuvant therapy methods such as hyperbaric oxygen therapy for the healing of diabetic foot ulcer wounds. The present study aimed to determine the efficacy of hyperbaric oxygen therapy in diabetic foot ulcers based on Wagner classification. It was performed retrospectively from prospectively collected data. One hundred thirty patients with diabetic foot ulcers were assessed in 2 groups: 1 group received hyperbaric oxygen therapy; the other group did not. Patients were examined according to age, sex, ulcer grade based on Wagner classification; ulcer healing status; whether hyperbaric oxygen therapy was received; duration of diabetes in years; HbA1C, sedimentation, C-reactive protein levels; and presence of accompanying diseases, including peripheral arterial disease, chronic obstructive pulmonary disease, hypertension, chronic kidney disease, neuropathy, and retinopathy. The mean follow-up period was 19.5?±?4.45 months (range 12 to 28 months). Seventy-one (54.6%) patients received hyperbaric oxygen therapy, and 59 (45.4%) patients did not. All patients in Wagner grade 2 healed in both groups. In the group that received hyperbaric oxygen therapy for grade 3 and 4 patients, 35 (87.5%) and 11 (84.6%) healed, respectively. In total, 60 (84.5%) patients in the group that received hyperbaric oxygen therapy healed. The subgroup comparison conducted according to Wagner classification revealed no differences between the 2 groups of grades 2 and 5 patients. It also revealed that treatment had higher levels of efficacy in the healing of ulcers in grade 3 and 4 patients.  相似文献   
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A Morgagni’s hernia is a congenital defect found in the anterior aspect of the diaphragm between the costal and the sternal portions of this muscle. This defect is also referred to as the space of Larrey. It has been reported that 70% of patients with Morgagni’s hernia are female, 90% of the hernias are right-sided, and 92% of the hernias have hernia sacs. This type of hernia is a rare clinical entity and accounts for 3% of all surgically treated diaphragmatic hernias. There are no large retrospective or prospective studies on this topic. This type of hernia is a rare type among adults without a well-described prevalence and without well-established definitive management strategies. There are also few clinical reports about this clinical entity and its surgical treatment. We treated 21 patients with Morgagni’s hernia in a 12-year period, and we report our experience while discussing the surgical treatment of this disease. We performed a retrospective review of the 21 patients who were operated between 2003 and 2015. These patients had undergone surgical repair of Morgagni’s hernia. For each subject, demographic data, symptoms of presentation, physical examination findings, preoperative imaging studies and diagnosis, and surgical procedures were documented. Location of the hernia sac and its contents, postoperative complications, and duration of hospital stay were recorded and evaluated. Twelve patients were females and nine were males. The mean age of patients was 63.85 years. Dyspnea was the most prominent symptom in our patients. Morgagni’s hernias were located on the right side in 19 patients and on the left side in 2 patients. Chest X-ray in 10 patients and abdominal computerized tomography in 17 patients were the major diagnostic tools. Four patients were operated as emergency while others underwent elective surgery (17 patients). Twelve patients were operated with laparoscopy and the remaining nine were operated with the conventional open abdominal technique. Hernia sacs were observed in all of the patients and removed except in four of them. The omentum and the transverse colon were the most commonly seen organs in hernia sacs. Hernia defects were repaired with primary sutures in four patients (all open cases) and primary closure supported with mesh in six patients (four laparoscopic, two open cases). In the remaining 11 patients, hernia defects were closed with synthetic meshes (eight laparoscopic, three open cases). Mean postoperative hospital stay was 9.8 days. No recurrence was observed in any patients. Only one of our patients died during follow-up. In Morgagni’s hernias, surgical intervention is necessary as the hernia may cause complications such as strangulation of the colon or intestines. A laparoscopic approach has increased its popularity in recent years because of the well-known advantages of laparoscopy.  相似文献   
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