Kimura's disease

An Asian man aged 42 had a pre-auricular swelling on the left with local skin itching. Owing to the highly probably benign nature at examination, surgical treatment was refrained from. One year later, the swelling had increased while the itching was unchanged, so that surgical excision was performed. Morbid-anatomical examination revealed angiolymphoid hyperplasia with eosinophilia, compatible with Kimura's disease. Kimura's disease is a chronic inflammatory disorder which is very rare in Europe, but which is frequently diagnosed in Asian countries and in immigrants from these countries. Tumours in the head and neck region, enlarged lymph nodes and increased eosinophil counts are typical signs. The IgE level is increased. The cause of this disease is so far not clear, although there are indications that an inflammatory reaction to an unknown allergen plays a part. It is essential that this disease should be distinguished in the diagnostic work-up from presence of a malignancy. Fine-needle aspiration cytology is often inconclusive; as a rule the diagnosis can only be made after surgical excision of the tumour. There is no consensus on the treatment of this disease, but symptomatic therapy usually suffices.     

Refining hollow obturator base using light-activated resin

Inclusion body myositis (IBM) is a separate class of the inflammatory myopathies with recently proposed clinical and pathological diagnostic criteria. An association between inflammatory myopathies and malignancy has been questioned in the literature. Recent reviews of the inflammatory myopathies suggest that only dermatomyositis is associated with malignancy. The largest study to date of patients with IBM found that 15% had a malignancy (6 of 40). We report the first documented case of IBM and concurrent transitional cell carcinoma of the bladder. We suggest that a causal relationship between IBM and malignancy may exist because of significantly improved functional strength gained after tumor removal.     

Staphylococcal vertebral osteomyelitis: case report

A 65-year old male Saudi patient presented with rapidly progressive quadriparesis. Lower cervical myelopathy was associated with radiological features of bone destruction, inflammatory disease of the spine and a paravertebral mass. Although tuberculosis and brucellosis are more commonly responsible for this clinical picture in our practice, Staphylococcus aureus was isolated from tissue recovered at surgery. He has been followed up for 12 months and has made an almost complete recovery after surgical decompression of spinal cord and a 2-month course of intravenous flucloxacillin. This case underscores the need for tissue diagnosis in patients presenting with inflammatory paravertebral swellings even in areas endemic for tuberculosis and brucellosis.     

Cutaneous malacoplakia: a pediatric case

INTRODUCTION: Cutaneous malakoplakia is an inflammatory disease characterized by granulomatous accumulation of distinctive phagocytic macrophages. It occurs mainly in visceral or orificial areas; the condition is rarely purely cutaneous, and appears to be extremely rare in childhood. CASE REPORT: A facial cutaneous crusted lesion was diagnosed as cutaneous malakoplakia in an immunocompetent child. The lesion had been excised twice and it had recurred, and the diagnosis was made possible only with a third biopsy, after a 2-year chronic expansion. This third biopsy revealed a dense granulomatous inflammation with numerous phagocytic histiocytes containing abundant fine granules and round Michaelis-Gutmann bodies, both staining with PAS, Perls and von Kossa. Biopsy cultures revealed only growth of two different streptococcus (group B) strains. The lesion resolved after a 4-month period of antibiotic therapy, including roxithromycin, ampicillin and trimethoprim-sulfamethoxasole. DISCUSSION: Diagnosis of malakoplakia is mainly made by histopathologic examination of tissue excision or biopsies. There are no specific clinical features. Most reported cases of this uncommon phagocytic reaction to common bacteria have developed in the genitourinary areas (71 p. 100); purely cutaneous localisation, as in our patient, are rare (4 p. 100). Intracytoplasmic granules may result from phagolysosomes and incomplete bacterial killing, with subsequent deposit of iron and calcium in the phagocytic macrophages. A number of reported cases have affected immunocompromised patients with either congenital immunodeficiency or secondary immunodeficiency. The most effective treatment option is based on a protracted antibiotherapy, using drugs that easily permeate the macrophages, e.g. quinolones and trimethoprim-sulfamethoxasole. Lesion may recur after surgical excision.     

Retroperitoneal ganglioneuroma. Report of a case

OBJECTIVE: To report an additional case of retroperitoneal ganglioneuroma. METHODS/RESULTS: A case of retroperitoneal ganglioneuroma that had been incidentally discovered in a 27-year-old male during abdominal US evaluation is described. The diagnosis was based on the histopathological findings after US-guided biopsy. The clinical features and the findings of the complementary tests, which included radiological assessment, intravenous urography, CT and cavography are presented, as well as the pathology findings. Treatment was by complete surgical excision of the tumor. CONCLUSION: Treatment of ganglioneuroma is by surgery, since the diagnosis is generally based on the histopathological analysis of the surgical specimen. For those cases with a preoperative diagnosis, some authors advocate surgery for patients with clinical evidence of neuroblastoma or another pathology arising from this disease. Although infrequent, ganglioneuroma coexisting with neuroblastoma can occur. For this reason, complete excision of the tumor is preferred.     

Aggressive fibromatosis (fibrosarcoma) of the facial nerve

Aggressive fibromatosis of the facial nerve is a very rare tumor. Three cases have been previously reported. The tumor is locally recurrent and often has a fatal outcome. This report is of a ten-year-old boy whose tumor originally developed in the parotid area with subsequent spread to the base of the skull, the neck and the cerebellopontine angle. Treatment was by wide surgical excision, radiation therapy and chemotherapy. Despite treatment, the patient died. The clinical features of this case will be described. Only wide surgical excision early in the course of the disease may offer a chance for cure.     

Acrodermatitis enteropathica-like rash and enterocolitis

Primary intracranial melanomas are rare and occur mainly in young adults. Originating from leptomeningeal melanoblasts and extending into the parenchyma, the tumours closely resemble meningiomas, from which they are radiologically difficult to distinguish despite progress in neuroimaging. Definitive diagnosis is usually made on histopathological examination, though confirmed only after post-mortem examination in some cases. Prolonged disease-free periods, and in rare cases long-term survival, are possible following successful total surgical excision. This case presented with typical clinical features but, at 79 years old, an unusual age.     

Primary angiosarcoma of the heart

Primary hemangioendotheliosarcoma is the least common form of sarcoma of the heart, itself an uncommon tumor. A review of most of the reported cases is presented. This study revealed that although the syndrome produced by cardiac sarcoma point to a strikingly characteristic picture, the diagnosis is almost always confused, especially with pericardial inflammatory diseases, and delayed. Successful treatment generally has not been available. This is the only case we know of that has been treated by resection utilizing cardiopulmonary bypass. The appearance of metastases in unusual locations in our patient, despite total excision of the primary, suggests tumor embolization during the bypass and the need for intra-operative chemotherapy, as well as such treatment before or after surgery.     

Intrapericardial teratoma. A case report and a literature review

A case of newborn intrapericardial teratoma is reported. The clinical, echocardiographic, tomographic and histologic features are described, and also, the therapeutic options. The newborn was submitted to surgical excision of the intrapericardial tumor and has a clinical follow-up greater than four years.     

Retroperitoneal Castleman's disease. Presentation of a clinical case

Castelman disease (CD) is a rare lymphadenopathy that usually presents as a solitary, slow growing mass; its etiology is still unknown. The authors described a case of CD localized in the retro-pancreatic space. A 33-year old patient, female, underwent abdominal ultrasonography because of upper right abdominal pain. The ultrasound showed gallbladder stones and a mass of 4 cm in diameter behind the pancreatic head, modifying the shape of the inferior vena cava. The patient underwent surgical excision and histological examination showed a hyaline type of CD. In the 70% of the cases, the disease is located in the mediastinum, only in 14% of the cases in the retroperitoneal space. The case we report is a hyaline type of CD that is the most frequent histological type, constitutes 90% of the cases, occurs usually in the young population and after surgical excision has a very good prognosis. Different is the clinical behaviour of the plasma cell type of CD that has an aggressive and often fatal clinical outcome. The patient we treated is still free of any symptoms one year after the operation.     

Penetration of cryotherapy in treatment after shoulder arthroscopy

The authors describe the case of a female patient affected with large symptomatic gastric leiomyoma, six centimeters in diameter, who presented to clinical observation referring gastric pain and melena. The first gastroscopy showed massive bleeding from a submucosal gastric lesion. The lesion was not endoscopically resectable and the injective endoscopic treatment of bleeding failed. The patient was surgically treated with laparotomic excision of the leiomyoma. She was back home in ten days. The authors describe this case to discuss the possibility to resect large gastric leiomyomas using endoscopic resection indeed surgical approach. They also enhance the validity of surgical treatment for its safety and radical approach to large lesions.     

Effect of hypoxia on the pressure developed by inspiratory muscles during airway occlusion

Patients with colitis and ileocolitis of unknown etiology from two previously reported series have been combined and the follow-up studies have been extended to compare the long term postoperative courses of ulcerative colitis (UC) and Crohn's disease of the colon (CDC). The combined and updated series of 176 patients, 99% of whom could be traced, provided a mean postoperative follow-up period for UC of 14 years (5 to 31) and CDC of 13.1 years (5 to 36). There were highly significant associations between generally accepted clinical and distributional features of UC and CDC and microscopic findings generally regarded as reliable for each. However, because of spectrum of features was found in each entity, neither clinical and distributional nor microscopic features alone are sufficient for diagnosis in every case. There were no differences in gross or disease-related mortality in UC and CDC whatever the method of diagnosis. After anastomotic procedures in CDC a recurrence rate of 73% was found. After proctocolectomy the ileostomy revision rate (considering all types of those which required further excision of ileum) was higher in CDC than UC whether the diagnoses were based on microscopic, clinical, or combined criteria, but the differences reached statistical significance only in the comparison of "clinical UC", with "clinical CDC". Moreover, after the first 2 postoperative years, the risk of having an ileostomy revision in UC and CDC (combined criteria) per patient year follow-up was virtually identical and there were no cases of short bowel syndrome. Differences in the clinical courses of UC and CDC after colectomy and ileostomy are of degree and do not reflect the ultimate course or potential for rehabilitation. Decisions regarding surgical therapy should be made independent of the diagnosis of UC or CDC.     

Actinomycosis in otorhinolaryngology. Apropos of a case with localization in the nasal cavity

The authors report an atypical case of actionomycosis implanted in the nasal cavity and occurring twenty years after septum surgery. Treatment by penicillin and surgical excision gave a good result. Cervicofacial actinomycosis is caused by actinomyctes which usually lives as a saprophyte in the oral cavity. A trauma is often found in the previous history. Diagnosis with tumors and abscess may be difficult. The histopathological examination shows typical aspects of the granuloma (gram+ and Grocott+) and the bacteriological isolation of the germ is difficult to obtain. Penicillin associated with surgical excision is the best therapy, but high doses must be used for a long time. The literature is reviewed without finding such a case.     

Treatment of chondrosarcoma. A retrospective study at the Norwegian Radium Hospital

The authors present a retrospective analysis of 59 chondrosarcoma patients treated at the Norwegian Radium Hospital during the period 1981 to 1993. 31 patients were admitted with untouched tumour, seven after fine needle cytology and 20 after open biopsy or partial excision. One patient had recurrent local disease. Only 20% of the tumours were of high grade malignancy. 51 patients were treated by surgery. Reconstructions were performed in 16 patients, using allografts or endoprostheses. Amputations were performed in six cases and wide excision in 12 cases. In these 18 patients local recurrence appeared in one case, and two developed lung metastases. Only one of the 18 patients operated by amputation or wide excision has since died from chondrosarcoma. Marginal excisions were performed in 26 cases. Nine of these patients developed a local recurrence, five developed metastases and three have died. Six patients had partial excisions. Postoperative radiotherapy was given to one patient only. Five of the six are alive. In one case, the quality of the margins could not be evaluated. A total of 45 of the 51 patients treated for the primary tumour by surgery are alive. The median observation time is four years. Treatment of nonmetastatic chondrosarcoma should be surgical. Chondrosarcoma patients show wider variations in age, localization of tumour and tumour growth rate than patients with other bone sarcomas. Although wide excisions provide the best local control of any grade of malignancy, the mutilation or risk involved may be so great that some patients may benefit from marginal or even partial excision.     

Coupling between single muscle spindle afferent and EMG in human wrist extensor muscles: physiological evidence of skeletofusimotor (beta) innervation

The present report of four cases highlights the potential for focal organizing pneumonia (FOP) to masquerade as a small peripheral lung adenocarcinoma on CT scans. Both entities may present the CT appearance of a peripheral spiculated lung nodule, often with an air bronchogram. A history suggestive of an infectious aetiology and the presence of other foci of inflammatory change on CT scan may be helpful clues to the diagnosis of FOP. Because FOP is comparatively rare, surgical excision will usually be required to exclude malignancy. In some cases, however, particularly after a negative percutaneous biopsy, conservative management with a follow-up CT scan at 3-4 weeks may be an alternative to immediate surgical intervention.     

Periodic health examinations. Why? What? When? How?

Pulmonary blastoma is now accepted as a distinctive neoplasm. It remains rare, and only 28 cases have been reliably recorded. A further two cases are now reported, and the previous literature is reviewed. There are no specific clinical or radiological features of pulmonary blastoma. The presentation can be that of any other pulmonary tumour although a peripheral situation is usual and a large size is often attained before detection. Pulmonary blastoma is a mixed tumour with malignant epithelial and connective tissue components with a distinctive resemblance to fetal lung. The treatment of choice is surgical excision but the overall prognosis is poor. It is doubtful whether the tumour has a true blastomatous origin.     

Tension pneumothorax precluding laparoscopic repair of diaphragmatic hernia

BACKGROUND: Central Europe and the Czech Republic are specific in the prevalence of obesity which has increased by 10-40% during the last 10 years. METHODS: In the Czech republic there is 30 years of experience of a comprehensive approach to obesity treatment which includes: dietary treatment; exercise; behavioral modification; drug treatment; and bariatric surgery. Each of these approaches has its place in complex obesity management. Since 1983 bariatric surgery has been established in the Czech Republic for the treatment of morbid obesity. Vertical banded gastroplasty (VBG), gastric banding, laparoscopic nonadjustable and adjustable gastric bandings have been used over the years. Since 1993 laparoscopic gastric banding has been the only method used in our department. RESULTS: The comprehensive approach for obesity treatment in the Czech Republic has resulted in the development of obesity management and research centers, regional obesity units, obesity out-patients clinics and weight reduction clubs. The surgical treatment is a well-established part of this system and the long-term results of surgical treatment are acceptable both in terms of weight loss and complication rate. There has been no statistical difference in weight loss results following VBG and laparoscopic gastric banding, but there is a significant decrease in morbidity, and shorter hospital stay associated with laparoscopic gastric banding. CONCLUSIONS: The surgical approach in obesity treatment has an important place in the comprehensive care of obese patients. Laparoscopic gastric banding in the hands of an experienced surgeon is a method with low morbidity, short hospital stay and long-term weight loss results which are fully comparable with the results of other surgical approaches.     

Emphysematous gastritis secondary to ingestion of large amounts of Coca Cola

Acute emphysematous gastritis (AEG) is a life-threatening disease in which gas-forming bacteria invade the gastric wall and cause acute inflammation of it. The clinical presentation of the patient with AEG is stormy: severe sepsis which usually leads to an early death. Presented herein is a case of a 16-yr-old boy with AEG. There were no predisposing factors to the condition in this case. Gas invaded the stomach wall and portal venous system, as well as the duodenal wall, a finding that has not been reported previously. The clinical course was very severe but, in contrast to previously reported cases, recovery was very rapid and left no sequelae.     

Hemobilia caused by a giant benign hemangioma of the liver: report of a case

We report herein the extremely unusual case of a 39-year-old woman in whom a giant cavernous hemangioma caused hemobilia. Cavernous hemangioma is the most common benign neoplasm of the liver and rarely causes any clinical symptoms or signs, while hemobilia usually occurs secondary to accidental operative or iatrogenic trauma, vascular disease, inflammatory disorders, gallstones, or tumors of the liver. Although invasive or malignant hepatic tumors often result in a communication between the biliary tract and the blood vessels, only one case of hemobilia caused by a benign cavernous hemangioma has ever been reported, but with no details about the patient. Our patient presented to a local hospital with severe melena as the initial main symptom, where ligation of the right hepatic artery was performed. This failed to relieve her symptoms, and she was subsequently referred to our department where a right hepatectomy was performed. Histopathological examination revealed no malignancy combined with the tumor; however, the hemangioma was exposed to the bile duct in segment VIII, which was presumably the cause of the hemobilia. This patient remains in good health almost 6 years after her operation. To the best of our knowledge this is the first case report of hemobilia caused by a cavernous hemangioma, and is accompanied by a detailed analysis.     

11 cases of anal Bowen's disease

Bowen's disease of the anal region is a rare, slow-growing, intraepidermal squamous-cell carcinoma (carcinoma in situ). If surgical excision is incomplete, there is a risk of subsequent development of malignancy and metastasis. Between 1980 and 1995 we treated 11 patients (8 female, 3 male) with anal Bowen's disease. The mean age was 55 (34-75) years. The main reason for excision was: pain (4), itching (3), bleeding (3) and a disturbing lump (3). The intraoperative findings were in all cases a lesion at the anocutaneous line: perianal or intra-anal tumor (6), erosion (2) or ulceration (2) as well as lichenoid lesion (4) or hyperpigmentation (3). The procedure was excision of the lesion in 10 cases. Only in one case was a biopsy taken. 3 patients had to be operated on a second time for reasons of radicality. 5 years after primary diagnosis, one patient developed a recurrent invasive squamous-cell carcinoma and had to undergo perineo-abdominal rectum amputation with postoperative radiotherapy (2 years after operation). Only one patient underwent a biopsy, which produced the diagnosis of invasive squamous-cell carcinoma. He underwent combined chemo-radiotherapy. The symptoms of anal Bowen's disease are unspecific and the clinical findings are uncharacteristic. The recommended therapy is complete surgical excision. With complete excision no recurrences do occur.