Monoclonal Gammopathy—a Diagnostic Challenge

ABSTRACT Four patients with IgG multiple myeloma (MM) and an unusually indolent course are presented. They had received no cytostatic therapy for 12–23 years. The M-component was initially 25–33 g/l, the bone marrow plasma cell percentage was 4–6%, but bone lesions were not demonstrated. A marked increase in size of the M-component and in the plasma cell percentage was noted in all patients, but a definitely neoplastic proliferation in only one patient. Spontaneous regression of the M-component was observed in two patients. In the light of the present cases we believe that neither a high level of the M-component nor an increase in its size should be used alone as indicators of a malignant course in MM patients. An indolent, non-progressive variant obviously occurs as one extreme in the clinical spectrum of this disorder with a course sometimes extended over 10–20 years.     

Parachute Mitral Valve in Adults—A Systematic Overview

Parachute mitral valve (PMV) is a rare congenital anomaly of the mitral valve apparatus seen in infants and young children. In most instances PMV is associated with other congenital anomalies of the heart, in particular obstructive lesions of the mitral inflow (mitral valve ring) and left ventricular outflow tract (subaortic stenosis), and coarctation of aorta and is referred to as Shone's complex or Shone's anomaly. PMV may also occur as an isolated lesion or in association with other congenital cardiac anomalies. Not much is known about PMV in adults as an isolated anomaly or in association with other congenital cardiac anomalies. We reviewed the literature to identify cases of PMV (isolated or associated with other lesions) in adults, to address prevalence, clinical presentation, diagnosis, treatment, and outcome of such patients. (Echocardiography 2010;27:581‐586)     

Splenic Abscess: A Diagnostic Challenge

Splenic abscess is uncommon and remains a diagnostic challenge. We present two cases. Both patients had predisposing factors that may have led to the splenic abscess. At admission, both patients presented clinical and roentgenographic signs, suggestive but nonspecific for splenic suppuration. Of particular interest was the isolation of Salmonella typhimurium in our first patient. The literature on splenic abscess is reviewed.     

Herpetic Esophagitis: A Diagnostic Challenge in Immunocompromised Patients

Viral esophageal infection is common in immunocompromised patients. Twelve patients with esophagitis secondary to herpes viruses are described. Odynophagia, dysphagia, and gastrointestinal bleeding were the most common symptoms. Multiple infections particularly with candida were present in three of the 12 cases (25%). Typical "volcano ulcers" at endoscopy and discrete diffusely scattered shallow ulcers seen on double contrast esophagram are highly suggestive of herpetic esophagitis. Single contrast esophagram plays no specific role in the diagnosis of herpetic esophagitis. An analysis of clinical, endoscopic, radiological, and pathological features is presented.     

A Case of Boerhaave's Syndrome —Evaluation of Diagnostic Value of Endoscopy—

Abstract: We experienced a case of Boerhaave's syndrome. The patient, a 47-year-old male, vomited after drinking a large quantity of alcohol and thereafter, complained of epigastralgia, back pain, and dyspnea. Leukocytosis, positive CRP, fever, tenderness of the upper abdomen and muscular defense were noted on admission. Endoscopy revealed an ulcer at the lower esophagus. Since a chest X-P and CT showed thoracic effusion and subcutaneous and mediastinal emphysema, rupture of the esophageal ulcer was suspected. The diagnosis of Boerhaave's syndrome was established by esophagography. A rupture along the longitudinal axis of about 3cm was found at the left posterior wall 3cm above the E-G junction, and was surgically sutured. Of many cases of Boerhaave's syndrome reported in the literature in Japan, endoscopy was performed in 70 cases. Recently, endoscopy has been adopted as the first choice for the diagnosis of this syndrome as well as for emergency examination of the esophagus and the diagnosis is established by endoscopy alone in many cases. Actually endoscopy was useful for the diagnosis of this disease in 88.2% of the above cases. These results as well as our case indicate diagnostic value of endoscopy, if performed carefully. Boerhaave's syndrome, Endoscopy     

Retrorectal Tumors: A Diagnostic and Therapeutic Challenge

PURPOSE Tumors occurring in the retrorectal space are heterogeneous and uncommon. The utility of newer imaging techniques has not been extensively described, and operative approach is variable. This study examined the diagnosis, treatment, and outcome of retrorectal tumors at a tertiary referral center.METHODS Patients with primary, extramucosal neoplasms occurring in the retrorectal space were identified using a prospectively maintained, procedural database of all adult colorectal surgical patients (1981–2003). Patients also were incorporated from the gynecologic oncology service. Exclusion criteria included inflammatory processes, locally advanced colorectal cancer, and metastatic malignancy. Medical records, radiology, and pathology reports were reviewed retrospectively.RESULTS Thirty-four patients with retrorectal tumors were treated. Malignant tumors comprised 21 percent. Older age, male gender, and pain were predictive of malignancy (P < 0.05). Sensitivity of proctoscopy was 53 percent; this increased to 100 percent with the use of transrectal ultrasound. Accuracy of magnetic resonance vs. computed tomographic imaging for specific histologic tumor type was 28 vs. 18 percent, respectively. Surgical approach was anterior (n = 14), posterior (n = 11), and combined abdominoperineal (n = 9). Eleven patients required en bloc proctectomy. Patients undergoing posterior resection had lower blood loss and required fewer transfusions (P < 0.05). All benign tumors were resected with normal histologic margins and none recurred (median follow-up, 22 months). All patients with malignancy had recurrence/recrudescence of their disease. For these patients, median disease-free and overall survivals were 38 and 61 months, respectively.CONCLUSIONS Retrorectal tumors remain a diagnostic and therapeutic challenge. Pain, male gender, and advanced age increase the likelihood of malignancy. Various imaging modalities are useful for planning resection but cannot establish a definitive diagnosis. Whereas benign retrorectal tumors can be completely resected, curative resection of malignant retrorectal tumors remains difficult.Poster presentation at the meeting of The American Society of Colon and Rectal Surgeons, Dallas, Texas, May 8 to 13, 2004.     

Obturator Hernia: A Diagnostic Challenge of Small-Bowel Obstruction

Obturator hernia is relatively rare and is a diagnostic challenge in the emergency department because the hernia mass is usually concealed beneath the pectineus muscle. We report the case of a 91-year-old emaciated woman with an incarcerated obturator hernia. The hernia was discovered early in the emergency department by computed tomography and was reduced by emergency laparotomy. The Howship-Romberg sign and pain from the ipsilateral thigh to the knee are important clinical manifestations raising suspicion of obturator hernia, but these did not occur in our patient. One of the clinical clues in our patient was small-bowel obstruction of unknown origin, diagnosed by computed tomography. We emphasize that emergency physicians should keep a high index of clinical suspicion for obturator hernia when encountering small-bowel obstruction in emaciated elderly women. Although we cannot shorten the time from onset of symptoms to hospital admission, we can make rapid evaluation and surgical intervention to reduce the morbidity and mortality of obturator hernia.     

Endomyocardial Fibrosis Mimicking Ebstein's Anomaly: A Diagnostic Challenge

Endomyocardial fibrosis is a rare disease that is seen most commonly in tropical countries. It usually presents with characteristics of right-heart failure. Herein, we report the case of a 14-year-old adolescent boy who experienced endomyocardial fibrosis. Upon transthoracic echocardiography, the condition was mistakenly diagnosed as Ebstein''s anomaly of the tricuspid valve. Sixteen months after undergoing tricuspid annuloplasty and receiving a bidirectional Glenn shunt, the patient showed no echocardiographic evidence of valvular regurgitation. We discuss imaging and surgical techniques that enable the diagnosis and treatment of endomyocardial fibrosis.Key words: Adolescent, arteriovenous shunt, surgical, diagnosis, differential, diagnostic imaging/methods, endomyocardial fibrosis/complications/diagnosis/pathology/radiology/surgery, heart ventricles/radiography, prognosis, treatment outcome, tricuspid valve insufficiency/diagnosis/surgery, ventricular dysfunction, right/etiologyEndomyocardial fibrosis (EMF) is a rare disease that is seen most commonly in tropical countries. It usually presents with characteristics of right-heart failure. A 14-year-old adolescent boy''s case of EMF was mistakenly diagnosed upon transthoracic echocardiography as Ebstein''s anomaly of the tricuspid valve. Here, we discuss the case of this patient, along with imaging and surgical techniques for the diagnosis and treatment of EMF.     

Inflammatory Myofibroblastic Tumor of the Liver: A Diagnostic Challenge

Inflammatory myofibroblastic tumor (IMT) is an uncommon myofibroblastic neoplasm that was formerly included within the broad category of inflammatory pseudotumor (IPT). IMT is rarely encountered in the liver. Similar to IMT of other organs, the interchangeable use of the terms IMT and IPT in liver has made the analysis of these lesions difficult. In this review, clinical and pathological features of IMT of the liver are reviewed and the differential diagnosis of IMT is discussed, with emphasis on IPT and the other entities included in this large category. IMT can mimic malignant tumors. There are no known unique diagnostic clinical, laboratory, or radiological features. The definitive diagnosis of IMT depends on careful pathological examination. The histopathological evaluation of hepatic IMT reveals that, the myxoid/vascular pattern is the most frequently observed, followed by, in decreasing frequency, fibrous histiocytoma-like pattern and hypocellular fibrous pattern. In IMT of the liver, anaplastic lymphoma kinase (ALK) expression reliably predicts the presence of an ALK gene rearrangement. The diagnosis of hepatic IMT depends on the dominant histopathological pattern, and the management of the disease is still controversial. IMT of the liver is a distinctive neoplasm of intermediate biological potential, and should be distinguished from the variety of lesions that are included under the broad category of IPT. Therefore, to avoid confusion regarding the true incidence and behavior of hepatic IMT, the term IPT should not be used interchangeably with IMT. The rarity of IMT in liver should not minimize its consideration in the differential diagnosis of liver tumors, especially in patients with tumor markers in normal range.